Orbital IgG4-related disease, which can occur in adults of any age, is characterized by IgG4-positive lymphoplasmacytic infiltrations in ocular adnexal tissues. The signs and symptoms include chronic noninflammatory lid swelling and proptosis. Patients often have a history of allergic disease and elevated serum levels of IgG4 and IgE as well as hypergammaglobulinemia. Orbital IgG4-related disease must be differentiated from idiopathic orbital inflammation and ocular adnexal marginal zone B-cell lymphoma to ensure appropriate and effective treatment. Systemic steroid therapy decreases the size of the lesions, but relapse often occurs when systemic steroid therapy is discontinued. PMID:22778989
Kubota, Toshinobu; Moritani, Suzuko
Immunoglobulin4 (IgG4)-related disease is a systemic inflammatory disease characterized by elevation of serum IgG4. It involves various organs such as the pancreas (autoimmune pancreatitis), lacrimal gland (Mikulicz's disease), retroperitoneum (retroperitoneal fibrosis), aorta (aortic aneurysm and aortitis), heart (constrictive pericarditis), and pseudotumors around the coronary arteries. These disorders often coexist in accordance with progression of the disease. Because IgG4-related cardiovascular disorder affects the patient's prognosis, early detection and treatment is important. Coronary CT imaging and echocardiography accidentally detect IgG4-related disorders and (18)FDG-PET imaging can identify active inflammation in the lesions. Measurement of serum IgG4 levels and tissue biopsy are necessary for diagnosis. Minor salivary gland biopsy is recommended even though (18)FDG uptake is not detected when it is difficult to obtain a biopsy specimen from IgG4-related cardiovascular lesions. The first-line treatment is high-dose corticosteroid therapy, however, relapse is often reported. Corticosteroids suppress the development of active inflammatory diseases such as aortitis, pericarditis, and pseudotumors, but already-developed lesions do not respond. A large developed aneurysm can rupture even during or after corticosteroid therapy, therefore, additional surgical treatment may be needed. Treatment of IgG4-related cardiovascular disorders might require higher doses of corticosteroids than IgG4-related extracardiovascular disorders. The adequate dose of corticosteroid, type and dose of immunosuppressant, and surgical intervention should be carefully considered on a case-by-case basis. PMID:24898599
Tajima, Miyu; Nagai, Ryozo; Hiroi, Yukio
IgG4-related kidney disease (IgG4-RKD) is a comprehensive term for renal lesions associated with IgG4-related disease, which is a recently recognized clinical entity characterized by a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells with fibrosis affecting several organs. Tubulointerstitial nephritis with increased IgG4-positive plasma cells and fibrosis is the most dominant feature of IgG4-RKD and may cause acute or chronic renal dysfunction, although some glomerular lesions such as membranous nephropathy are sometimes evident. Radiologically, several characteristic abnormalities are often demonstrated, sometimes mimicking malignancies. IgG4-RKD predominantly affects middle-aged to elderly men, and most patients have accompanying IgG4-related extrarenal lesions such as sialadenitis, lymphadenopathy, or type 1 autoimmune pancreatitis. Serology usually demonstrates high levels of serum total IgG and IgG4, and high levels of serum IgE and hypocomplementemia are also frequent features. Corticosteroid therapy is usually quite effective, leading to amelioration of the renal dysfunction and radiological and serological abnormalities. However, as any delay in treatment may result in irreversible renal failure, early diagnosis and appropriate therapy are very important. Despite these distinctive clinicopathological features of IgG4-RKD, its pathogenesis remains poorly understood. Awareness of this condition and accumulation of more cases worldwide are necessary. PMID:24107849
Saeki, Takako; Kawano, Mitsuhiro
The term "IgG4-related disease" encompasses several disorders described many years ago under various designations depending on the organ or system involved (e.g., Mikulicz syndrome, Riedel's thyroiditis, and retroperitoneal fibrosis). The clinical presentation varies widely, as one or more organs may be affected, usually in the same region of the body and either synchronously or metachronously. The main targets are the pancreas, bile ducts, salivary glands, lachrymal glands, mediastinal lymph nodes, and retroperitoneum. IgG4-related disease is rare, with an estimated incidence of 0.2 to 1/100,000 in Japan and no available incidence data in occidental countries. Men older than 50 years are predominantly affected. Serum IgG4 levels are often greater than 1.35 g/L. Enlargement of the involved organs, which may be pseudotumoral, is due to a combination of infiltration by T cells and IgG4-expressing plasma cells, storiform fibrosis, and obliterative thrombophlebitis. Glucocorticoid therapy is effective but may be followed by relapses requiring the use of immunomodulating agents such as azathioprine, methotrexate and, more recently, rituximab. IgG4-related disease is not an autoimmune condition related to IgG4 autoantibodies, and neither does it involve immune complexes. Specific joint involvement has been reported in a very small number of patients. PMID:23849464
Palazzo, Elisabeth; Palazzo, Clémence; Palazzo, Maxime
Ocular adnexal non-Hodgkin’s lymphoma (NHL), the most common form of ophthalmic NHL, has a unique incidence pattern showing a steady and rapid increase in the past few decades, nearly equal rates among both genders, and predominance among Asians/Pacific Islanders. No major cause for ocular adnexal NHL has been identified, although infectious agents, immune disorders and genetic/epigenetic factors have all been implicated in its etiology. Identifying putative risk factors and biologic mechanisms leading to carcinogenesis in ocular adnexal NHL may enable implementation of effective preventive and/or therapeutic approaches for this malignancy. This article summarizes current knowledge on epidemiology of ocular adnexal NHL and the role of various potential risk factors in its etiology.
Moslehi, Roxana; Schymura, Maria J; Nayak, Seema; Coles, F Bruce
The objective of this study was to assess whether there is PCR evidence for C. psittaci DNA in ocular adnexal lymphoma specimens collected in an academic institution in the U.S. This was a retrospective, single-center study of patients from 1994 - 2004. We used 28 ocular adnexal lymphoma biopsy specimens from adult patients, 16 control lymphoma specimens from patients with systemic lymphomas not involving the ocular adnexa, and five control benign adnexal tissue samples. The presence of C. psittaci DNA was investigated by polymerase chain reaction (PCR) in each group. Two different assays were utilized: (1) conventional PCR/gel based assay targeting a 111-bp fragment of the 16S gene and (2) a real-time PCR assay amplifying a 148-bp portion of the 16S gene with detection via a specific fluorescent probe. Amplification was carried out to 60 cycles. Positive controls consisted of isolated DNA from C. psittaci strains VS1, CP3, and FP. A human DNA internal control was used to assess sample DNA quality and amplification success. Mean outcome measure was the presence of C. psittaci DNA. Using both assays, all patient samples in all categories yielded negative results. Both assays detected C. psittaci DNA from isolated strains. Internationally, Chlamydia psittaci has been associated with ocular adnexal lymphomas with great variability. Similar to several other recent studies in the USA, our study could not confirm the presence of C. psittaci in ocular adnexal lymphomas. Differences in the prevalence of C. psittaci infection in various geographic regions or technical differences in the application of the assays may underlie the variability in the association between C. psittaci and ocular adnexal lymphoma. PMID:17454602
Zhang, Grace S; Winter, Jane N; Variakojis, Daina; Reich, Steven; Lissner, Gary S; Bryar, Paul; Regner, Maryann; Mangold, Kathy; Kaul, Karen
Presently, 6 cases of IgG4-related pleuritis have been reported. We encountered a patient who developed chylothorax due to IgG4-related disease. To our knowledge, such patients have not been reported. This patient developed right-sided chylothorax and left-sided non-chylothorax lymphocyte-predominant pleuritis. Elevated serum and pleural IgG4 concentrations and histopathological analysis of pleural biopsy confirmed the diagnosis of IgG4-related pleuritis. Left-sided pleuritis improved with corticosteroid therapy, but right-sided chylothorax persists. IgG4-related disease can be one cause of chylothorax. PMID:25030570
Kato, Eisuke; Takayanagi, Noboru; Ishiguro, Takashi; Kagiyama, Naho; Shimizu, Yoshihiko; Sugita, Yutaka
Given the fact that infectious agents contribute to around 18% of human cancers worldwide, it would seem prudent to explore their role in neoplasms of the ocular adnexa: primary malignancies of the conjunctiva, lacrimal glands, eyelids, and orbit. By elucidating the mechanisms by which infectious agents contribute to oncogenesis, the management, treatment, and prevention of these neoplasms may one day parallel what is already in place for cancers such as cervical cancer, hepatocellular carcinoma, gastric mucosa-associated lymphoid tissue lymphoma and gastric adenocarcinoma. Antibiotic treatment and vaccines against infectious agents may herald a future with a curtailed role for traditional therapies of surgery, radiation, and chemotherapy. Unlike other malignancies for which large epidemiological studies are available, analyzing ocular adnexal neoplasms is challenging as they are relatively rare. Additionally, putative infectious agents seemingly display an immense geographic variation that has led to much debate regarding the relative importance of one organism versus another. This review discusses the pathogenetic role of several microorganisms in different ocular adnexal malignancies, including human papilloma virus in conjunctival papilloma and squamous cell carcinoma, human immunodeficiency virus in conjunctival squamous carcinoma, Kaposi sarcoma-associated herpes virus or human herpes simplex virus-8 (KSHV/HHV-8) in conjunctival Kaposi sarcoma, Helicobacter pylori (H. pylori,), Chlamydia, and hepatitis C virus in ocular adnexal mucosa-associated lymphoid tissue lymphomas. Unlike cervical cancer where a single infectious agent, human papilloma virus, is found in greater than 99% of lesions, multiple organisms may play a role in the etiology of certain ocular adnexal neoplasms by acting through similar mechanisms of oncogenesis, including chronic antigenic stimulation and the action of infectious oncogenes. However, similar to other human malignancies, ultimately the role of infectious agents in ocular adnexal neoplasms is most likely as a cofactor to genetic and environmental risk factors.
Verma, Varun; Shen, Defen; Sieving, Pamela C.; Chan, Chi-Chao
IgG4-related disease is a systemic fibro-inflammatory condition, which includes autoimmune pancreatitis as part of the disease spectrum. Imaging has been demonstrated to play a major role in the diagnosis of autoimmune pancreatitis. Recognizing the wide spectrum of extrapancreatic manifestations of IgG4-related disease coupled with a high clinical index of suspicion will allow for an accurate and timely diagnosis to be made, thus avoiding unnecessary invasive procedures and ensuring that early effective corticosteroid therapy is commenced. This review aims to serve as a concise reference tool for both clinicians and radiologists in the diagnosis of extrapancreatic IgG4-related disease. PMID:24290777
Tan, T J; Ng, Y L; Tan, D; Fong, W S; Low, A S C
Abstract IgG4-related disease is characterized by elevated serum IgG4 and tissue infiltration by IgG4-positive plasma cells. We herein report a case of lacrimal sac diverticulitis with marked IgG4-positive plasma cell infiltration. An 89-year-old woman presenting with right lower eyelid mass. Imaging modalities demonstrated a cystic orbital mass located beneath the globe and adjacent to enlarged lacrimal sac. Serological tests showed high IgG4 and normal IgG levels, measuring 242 and 1603?mg/dl, respectively. The orbital mass was surgically excised. Histologically, the excised tissue demonstrated marked inflammation with fibrosis surrounded by mononuclear epithelial cells. A variety of IgG and IgG4-positive plasma cells infiltrated the stroma. This patient was diagnosed as an IgG4-related lacrimal sac diverticulitis, based on current diagnostic criteria of IgG4-related disease. It is likely that IgG4-related inflammation occurs in a lacrimal sac diverticulum, which should be considered a differential diagnosis in inferior orbital tumors. PMID:24568363
Kase, Satoru; Suzuki, Yasuo; Shinohara, Toshiya; Kase, Manabu
IgG4-related lymphadenopathy with increased numbers of Epstein-Barr virus (EBV)-infected cells has been reported but not fully described. We analyzed 31 cases of IgG4-related lymphadenopathy and 24 cases of extranodal IgG4-related diseases for their possible relationship with EBV. Other types of reactive lymph nodes (22) and angioimmunoblastic T-cell lymphoma (AITL) (10) were also studied for comparison. EBV-encoded RNA (EBER) in situ hybridization revealed EBER cells in 18 of 31 cases (58%) of IgG4-related lymphadenopathy. Increased EBER cells were found in only 4 of 22 (18.1%) non-IgG4-related reactive lymphoid hyperplasia in patients of a similar age (P=0.002) and in only 5 of 24 (21%) extranodal IgG4-related biopsies (P=0.006). Interestingly, all patients with EBER progressively transformed germinal center-type IgG4-related lymphadenopathy had systemic lymphadenopathy and/or extranodal involvement. AITL also is associated with EBV, and IgG4-related lymphadenopathy sometimes mimics the morphology of AITL; however, the number of IgG4 cells in AITL was significantly less than that in IgG4-related lymphadenopathy (P<0.001). Increased numbers of regulatory T cells are seen in IgG4-related disease; however, there was not a significant difference between the EBER and EBER cases. In conclusion, the presence of increased numbers of EBV-infected cells in IgG4-related lymphadenopathy, compared with other reactive lymphadenopathy or extranodal IgG4-related disease, suggests that there may be a relationship at least between nodal IgG4-related disease and EBV. It is important to avoid overdiagnosing these cases as malignant lymphomas or EBV-related lymphoproliferative disorders. PMID:24705309
Takeuchi, Mai; Sato, Yasuharu; Yasui, Hiroshi; Ozawa, Hiroaki; Ohno, Kyotaro; Takata, Katsuyoshi; Gion, Yuka; Orita, Yorihisa; Tachibana, Tomoyasu; Itoh, Tomoo; Asano, Naoko; Nakamura, Shigeo; Swerdlow, Steven H; Yoshino, Tadashi
The paper describes a case of IgG4-related chronic periaortitis in a 56-year-old man. Computed tomography revealed stenosis of the abdominal aorta and both common iliac arteries due to calcified atherosclerotic plaques, in this connection, bifurcation aorta-common femoral bypass surgery was performed. Intraoperatively, a solid retroperitoneal mass intimately connected with the walls of vessels was revealed in the retroperitoneal space around the aorta and iliac arteries. Histological examination ascertained rough fibrous connective tissue growth, a marked lymphoplasmacytic infiltration with an admixture of eosinophils, and formation of lymphoid follicles with germinal centers. Immunohistochemical examination revealed the plasma cell expression of CD20, IgG, IgG4, and kappa and lambda light chains in the inflammatory infiltrate. Because of active immune inflammation, further conservative therapy was recommended. PMID:25051723
Recently, autoimmune pancreatitis (AIP) has been classified into two subtypes: type 1 as a pancreatic manifestation of IgG4-related disease (IgG4-RD), and type 2 related with a granulocytic epithelial lesion (GEL). Different from type 2 AIP, T helper type 2 (Th2) immune response is predominant over Th1 in type 1/IgG4-RD. Recent human and experimental animal studies have suggested a possible involvement of innate immunity in addition to acquired immunity, such as genetic background, bacterial/viral infections, complement activation via classical pathway, or IgG4-production of monocytes/basophils with TLR/NOD stimulation. Based on these findings, we have proposed a hypothesis for the development of type 1 AIP, one of the IgG4-RD. PMID:24598063
Okazaki, Kazuichi; Sumimoto, Kimi; Mitsuyama, Toshiyuki; Uchida, Kazushige
Purpose To analyze the incidence and severity of ocular and adnexal injuries in the Second Lebanon War among Israeli soldiers and\\u000a civilians.\\u000a \\u000a \\u000a \\u000a \\u000a Design Retrospective cohort study.\\u000a \\u000a \\u000a \\u000a Participants All patients recorded in the Israeli National Trauma Registry during the Second Lebanon War (July–August, 2006).\\u000a \\u000a \\u000a \\u000a Methods Retrieval of all data relevant to ocular and adnexal injuries sustained during the study period, and differentiation between\\u000a those associated
Adiel Barak; Amir Elhalel; Joseph Pikkel; Eli Krauss; Benjamin Miller
IgG4-related sclerosing cholangitis (IgG4-SC) is often associated with autoimmune pancreatitis. However, the diffuse cholangiographic abnormalities observed in IgG4-SC may resemble those observed in primary sclerosing cholangitis (PSC), and the presence of segmental stenosis suggests cholangiocarcinoma (CC). IgG4-SC responds well to steroid therapy, whereas PSC is only effectively treated with liver transplantation and CC requires surgical intervention. Since IgG4-SC was first described, it has become a third distinct clinical entity of sclerosing cholangitis. The aim of this review was to introduce the diagnostic methods for IgG4-SC. IgG4-SC should be carefully diagnosed based on a combination of characteristic clinical, serological, morphological, and histopathological features after cholangiographic classification and targeting of a disease for differential diagnosis. When intrapancreatic stenosis is detected, pancreatic cancer or CC should be ruled out. If multiple intrahepatic stenoses are evident, PSC should be distinguished on the basis of cholangiographic findings and liver biopsy with IgG4 immunostaining. Associated inflammatory bowel disease is suggestive of PSC. If stenosis is demonstrated in the hepatic hilar region, CC should be discriminated by ultrasonography, intraductal ultrasonography, bile duct biopsy, and a higher cutoff serum IgG4 level of 182 mg/dL.
Nakazawa, Takahiro; Naitoh, Itaru; Hayashi, Kazuki; Miyabe, Katsuyuki; Simizu, Shuya; Joh, Takashi
IgG4-related sclerosing cholangitis (IgG4-SC) is often associated with autoimmune pancreatitis. However, the diffuse cholangiographic abnormalities observed in IgG4-SC may resemble those observed in primary sclerosing cholangitis (PSC), and the presence of segmental stenosis suggests cholangiocarcinoma (CC). IgG4-SC responds well to steroid therapy, whereas PSC is only effectively treated with liver transplantation and CC requires surgical intervention. Since IgG4-SC was first described, it has become a third distinct clinical entity of sclerosing cholangitis. The aim of this review was to introduce the diagnostic methods for IgG4-SC. IgG4-SC should be carefully diagnosed based on a combination of characteristic clinical, serological, morphological, and histopathological features after cholangiographic classification and targeting of a disease for differential diagnosis. When intrapancreatic stenosis is detected, pancreatic cancer or CC should be ruled out. If multiple intrahepatic stenoses are evident, PSC should be distinguished on the basis of cholangiographic findings and liver biopsy with IgG4 immunostaining. Associated inflammatory bowel disease is suggestive of PSC. If stenosis is demonstrated in the hepatic hilar region, CC should be discriminated by ultrasonography, intraductal ultrasonography, bile duct biopsy, and a higher cutoff serum IgG4 level of 182 mg/dL. PMID:24282356
Nakazawa, Takahiro; Naitoh, Itaru; Hayashi, Kazuki; Miyabe, Katsuyuki; Simizu, Shuya; Joh, Takashi
Interstitial cystitis (IC) is a disease of undetermined etiology and pathogenesis. Inflammation is thought to play a key role in many patients, characteristically with an increase in mast cells within the detrusor muscle of the bladder. We observed that some patients with IC had prominent plasma cells in bladder tissue, which elicited our interest in their possible pathogenic role in patients with IC. A total of 44 cases of IC were collected, including 42 bladder biopsies and 2 cystectomies. Patient age ranged from 18 to 92 years (average age of 49.5 years) and included 7 male and 37 female patients. The histology and immunostains for IgG, IgG4 and tryptase were examined, and the results were correlated with clinical and cystoscopic findings. Four cases showed a significant increase in IgG4-positive plasma cells, with greater than 30 IgG4 plasma cells per high-power field and an IgG4/IgG ratio greater than 0.5. In addition, statistically significant differences were found between IC with IgG4-positive plasma cells vs IgG4-negative cases. The IgG4-positive patients were of older age and had increased severe inflammation and decreased bladder capacity as compared with the IgG4-negative patients. We propose that a subset of patients with IC may have an IgG4-related disease, and further study including serum IgG4 measurement is required to better define this relationship. PMID:23732167
Crumley, Suzanne; Ge, Yimin; Zhou, Haijun; Shen, Steven S; Ro, Jae Y
SUMMARY Extranodal marginal zone B-cell lymphoma is the most common orbital tumour. We conducted a retrospective analysis to examine: a) the impact of initial presentation and staging on outcome; b) response to various treatment modalities and the effect of the latter on recurrence. 90 patients with primary ocular adnexal marginal zone lymphoma (POAML) diagnosed at our institution between 1984 and 2009 were studied. POAML was associated with monoclonal gammopathy (13%) at presentation. Most POAML patients (86%) presented with Ann-Arbor stage I disease. Radiotherapy led to excellent local control, but relapses occurred in 18% of Ann-Arbor stage I patients during a median follow-up of 5 years. Local relapses, including secondary central nervous system (CNS) involvement, were observed in patients receiving radiation doses < 30.6 Gy. No differences in relapse rate and survival were observed between patients who did or did not undergo staging bone marrow biopsy. Ann-Arbor stage II-IV disease and high lactate dehydrogenase levels were associated with shorter freedom from progression. In conclusion, POAML is an indolent lymphoma with continuous risk for relapse. Radiation doses of at least 30.6 Gy should be given in Ann-Arbor stage I disease, since lower doses may be more frequently associated with relapses, including CNS relapses.
Bayraktar, S.; Bayraktar, U. D.; Stefanovic, A.; Lossos, I. S.
IgG4-related disease is a systemic disorder that can involve various abdominal organs. Abdominal manifestations include autoimmune pancreatitis, sclerosing cholangitis, gallbladder pseudotumours, multifocal renal abnormalities, retroperitoneal fibrosis, and sclerosing mesenteritis. Radiological appearances of the IgG4-related disease often resemble malignant tumors and other inflammatory processes, potentially leading to unnecessary invasive procedures. Moreover, a timely diagnosis of IgG4-related disease leads to an effective treatment with steroids. Therefore, it is important to understand the spectrum of imaging manifestations of IgG4-related disease and their differentiating features from other diagnoses. PMID:24929264
Al Zahrani, Hassan; Kyoung Kim, Tae; Khalili, Korosh; Vlachou, Paraskevi; Yu, Hojun; Jang, Hyun-Jung
Purpose To study the pattern of ocular adnexal lymphoproliferative disorders (OALD) in an ophthalmic referral center in Saudi Arabia and to review their, histopathological characteristics with clinical correlation. Methods Retrospective chart review of 40 cases of patients who underwent incisional biopsy with the suspected diagnosis of periocular and/or adnexal lymphoid lesions over the period: 2000–2012 at the King Khaled Eye Specialist Hospital (KKESH), Riyadh, Saudi Arabia. The routine histopathologic slides are reviewed by a single pathologist to identify cases of Benign Reactive Lymphoid Hyperplasia (RLH), Atypical Lymphoid Hyperplasia and probable lymphoma. The identification of the specific types of lymphoma is performed at a tertiary general hospital: King Faisal Specialist Hospital and Research Centre (KFSH&RC). Results Forty patients are included with an age range of 11–91 years and a median of 36 years. The males constitute 70% and females 30% of the cases. The right eye and/or orbit are involved in 48%. The left eye is involved in 45% while a bilateral disease is found in 7.5%. The median duration of symptoms is 5 months. The site distribution is conjunctiva (42.5%), orbit (25%), lacrimal gland (12.5%), eyelid (10%), lacrimal sac (7.5%) and caruncle (2.5%). One case is excluded after histopathologic diagnosis of malignant melanoma. Diagnosis in the remaining 39 cases includes: RLH in 14 cases (35%), atypical lymphoid hyperplasia in three cases (9%), and lymphoma in 22 cases (56%). Classification of the lymphoma group is: extranodal marginal zone lymphoma (EMZL) in 9/22 cases (41%), diffuse large B cell lymphoma (DLBCL) in 4/22 cases (18%), Castelman’s disease in 3/22 cases (14%), Burkitt’s lymphoma in 2/22 cases (9%), follicular lymphoma and T cell-rich B cell lymphoma: one case each (4.5%).Two cases remain unclassified. Conclusion We have a wide age range which is comparable to other studies. Our results show male predominance and the commonest site of involvement is conjunctival, however if RLH cases are excluded, the commonest site for lymphoma is orbit/lacrimal gland in 45% followed by conjunctival in 23%. The commonest type of lymphoma is: EMZL in 41% followed by DLBCL in 18% then other types of lymphoma including follicular lymphoma.
Alkatan, Hind M.; Alaraj, Ahmad; El-khani, Albarah; Al-Sheikh, Osama
Immunoglobulin G (IgG)4-related sclerosing disease is a recently described syndrome characterized by mass-forming lesions in various organs due to dense lymphoplasmacytic infiltrates and stromal sclerosis, elevated serum IgG4 titer, increased tissue IgG4 plasma cells, and favorable clinical outcome. We describe 4 patients with IgG4-related sclerosing mastitis, which represents a new member of this family of diseases. All patients were female with a mean age of 47.5 years, presenting with painless masses in 1 or both breasts. One patient had concurrent IgG4-related lymphadenopathy, and another had eyelid swelling of undetermined cause. The serum IgG4 titer was elevated in 1 tested patient, and circulating autoantibodies were found in 3 tested patients. All patients were well with no recurrence after excision or biopsy of the mass. Histologically, the breast masses featured dense lymphoplasmacytic infiltrates, prominent stromal sclerosis and loss of breast lobules. Phlebitis was present in 1 case. IgG4 cells ranged from 272 to 495 per high-power field, constituting 49% to 85% of all IgG cells. IgG4 cells were scarce in 9 of 9 cases of lymphocytic mastitis and 6 of 7 cases of granulomatous mastitis studied as controls. In summary, IgG4-related sclerosing mastitis appears to be a distinctive form of mastitis, sometimes accompanied by other components of IgG4-related sclerosing disease, and shows a favorable clinical outcome. PMID:19384187
Cheuk, Wah; Chan, Alexander C L; Lam, Wai-Lung; Chow, Sheung-Ming; Crowley, Peter; Lloydd, Richard; Campbell, Ian; Thorburn, Murray; Chan, John K C
We present the imaging findings in two cases of IgG4-related disease involving the sinonasal region in the pediatric age group. Imaging findings in IgG4-related disease affecting the nasal cavity and paranasal sinuses have been rarely reported in literature. The diagnosis is made by a combination of clinical, imaging, and histopathologic findings. Radiologists should be aware of the imaging findings of this condition to ensure early diagnosis and treatment.
Prabhu, Shailesh M; Yadav, Vikas; Irodi, Aparna; Mani, Sunithi; Varghese, Ajoy Mathew
We studied the relation between prostaglandin analogue use and ocular adnexal features. We used a prospective, cross-sectional study involving 157 current, 15 past, and 171 never users of prostaglandin analogues. Patients 50 years of age or older and without conditions affecting ocular adnexal anatomy underwent glaucoma medication use history, external digital photography and systematic external adnexal exam. Two masked readers assessed the digital photos for upper lid dermatochalasis and lower lid steatoblepharon using a validated grading scheme. Another masked clinical examiner also assessed upper lid ptosis, levator muscle function, and inferior scleral show. We performed ordinal logistic regression analysis accounting for multiple covariates to assess the relation between prostaglandin analogue use and adnexal features. Multivariable analyses indicated there was a 230-fold increased risk of incremental involution of dermatochalasis (odds ratio (OR) ?=? 2.30; 95% confidence interval (CI) 1.43–3.69; p?=?5.44E-04) and a 249-fold increased risk of incremental loss of lower lid steatoblepharon (OR ?=? 2.49; 95% CI, 1.54–4.03; p?=? 1.98E-04) associated with current prostaglandin analogue use (bimatoprost 0.03%, travoprost 0.005%, or latanoprost 0.004%) versus prostaglandin analogue never or past users. Upper lid ptosis (OR ?=? 4.04; 95% CI, 2.43–6.72; p?=?7.37E-08), levator dysfunction (OR?=? 7.51; 95% CI, 3.39–16.65; p?=?6.74E-07) and lower lid retraction (OR?=?2.60; 95% CI, 1.58–4.28; p?=?1.72E-04) were highly associated with current prostaglandin analogue use versus prostaglandin analogue never or past users. The associations between prostaglandin analogue use and deepening of the upper lid sulci and between prostaglandin analogue use and loss of inferior periorbital fat are confirmed in this multivariable analysis. The associations between prostaglandin analogue use and levator muscle dysfunction and between prostaglandin analogue use and upper lid ptosis represent significant side effects that could impact visual function in glaucoma patients.
Shah, Mamta; Lee, Grace; Lefebvre, Daniel R.; Kronberg, Benjamin; Loomis, Stephanie; Brauner, Stacey C.; Turalba, Angela; Rhee, Douglas J.; Freitag, Suzanne K.; Pasquale, Louis R.
IgG4-related disease is a recently recognized autoimmune systemic disorder that has been described in various organs. The disease is characterized histologically by a dense lymphoplasmocytic infiltrate of IgG4-positive cells, storiform fibrosis and can be associated with tumefactive lesions. IgG4-related disease involving the upper gastrointestinal tract is rare and only two previous case reports have reported IgG4-related esophageal disease. We report the case of a 63-year-old female patient with a long-standing history of severe dysphagia and odynophagia with an initial diagnosis of reflux esophagitis. Symptoms persisted despite anti-acid therapy and control esophagogastroduodenoscopy (EGD) revealed endoscopic images consistent with esophagitis dissecans superficialis (sloughing esophagitis). An underlying autoimmune process was suspected and immunosuppressant agents were tried to control her disease. The patient eventually developed disabling dysphagia secondary to multiple chronic esophageal strictures. A diagnosis of IgG4-related disease was eventually made after reviewing esophageal biopsies and performing an immunohistochemical study with an anti-IgG4 antibody. Treatment attempts with corticosteroids and rituximab was not associated with a significant improvement of the symptoms of dysphagia and odynophagia, possibly because of the chronic nature of the disease associated with a high fibrotic component. Our case report describes this unique case of IgG4-related esophageal disease presenting as chronic esophagitis dissecans with strictures. We also briefly review the main histopathological features and treatment options in IgG4-related disease.
Dumas-Campagna, Myriam; Bouchard, Simon; Soucy, Genevieve; Bouin, Mickael
IgG4-related disease (IgG4-RD) encompasses a wide range of extrapancreatic manifestations. Albeit some are relatively well known, others such as autoimmune prostatitis remain poorly described. We present a 61-year-old Latin-American male with autoimmune pancreatitis (AIP) who presented with lower urinary tract symptoms (LUTS), normal prostate specific antigen (PSA) test, and prostate enlargement attributed to benign prostatic hyperplasia (BPH). He underwent a transurethral resection of the prostate (TURP) after which symptoms were resolved. On histopathology, prostatic stroma had a dense inflammatory infiltrate rich in plasma cells and lymphocytes; immunohistochemical morphometric assessment showed >10 IgG4-positive plasma cells/high power field (HPF). The diagnosis of IgG4-related prostatitis was postoperatively. We compared the patient characteristics with those of previous reports on Asian patients. Shared findings included prostate enlargement, LUTS (symptoms that can be confused with BPH), and PSA within normal limits or mild elevations. IgG4-related prostatitis is rarely considered as a preprocedural diagnosis, even in patients with evidence of IgG4-RD. Involved prostate zones include mainly central and transitional zones and less frequently the peripheral. Currently, there is insufficient data about the natural history and outcome. Whether steroids, transurethral resection, or both are the treatment of choice needs to be elucidated.
Bourlon, Maria T.; Sanchez-Avila, Monica; Chable-Montero, Fredy
Background Immunoglobulin G4 (IgG4)-related disease is a systemic syndrome, characterized by sclerosing lesions and usually associated with a raised serum IgG4 level; the pancreas, salivary glands, and lacrimal glands are typically affected. Recently, it has been suggested that IgG4-related sclerosing disease represents a subset of cases previously diagnosed as idiopathic hypertrophic pachymeningitis. This rare inflammatory disorder causes localized or diffused thickening of intracranial dura mater. Headache, cranial nerve palsy, and ataxia are the most common clinical manifestations. Herein, we report the clinical and histopathological features of a rare case of IgG4-related intracranial hypertrophic pachymeningitis involving cranial hyperostosis. Case presentation A 52-year-old man presented with refractory generalized tonic-clonic seizure. Magnetic resonance imaging revealed thickening of the meninges with enhancement near the superior sagittal sinus; skull bone defect was also noted. Extensive excision of affected skull bone and dura was performed, providing the diagnosis of IgG4-related pachymeningitis. After the surgery, the patient’s seizure stopped and he was smoothly tapered off antiepileptic medication. Conclusion To our knowledge, this is the first reported case of IgG4-related pachymeningitis with concomitant skull hyperostosis.
Recognition of IgG4-related disease as an independent chronic inflammatory disorder is a relatively new concept; previously, the condition was thought to represent a subtype of Sjögren's syndrome. IgG4-related disease is characterized by elevated serum levels of IgG4 and inflammation of various organs, with abundant infiltration of IgG4-bearing plasma cells, storiform fibrosis and obliterative phlebitis representing the major histopathological features of the swollen organs. The aetiology and pathogenesis of this disorder remain unclear, but inflammation and subsequent fibrosis occur due to excess production of type 2 T-helper-cell and regulatory T-cell cytokines. The disease can comprise various organ manifestations, such as dacryoadenitis and sialadenitis (also called Mikulicz disease), type 1 autoimmune pancreatitis, kidney dysfunction and lung disease. Early intervention using glucocorticoids can improve IgG4-related organ dysfunction; however, patients often relapse when doses of these agents are tapered. The disease has also been associated with an increased incidence of certain malignancies. Increased awareness of IgG4-related disease might lead to consultation with rheumatologists owing to its clinical, and potentially pathogenetic, similarities with certain rheumatic disorders. With this in mind, we describe the pathogenic mechanisms of IgG4-related disease, and outline considerations for diagnosis and treatment of the condition. PMID:24296677
Yamamoto, Motohisa; Takahashi, Hiroki; Shinomura, Yasuhisa
We experienced severe asthmatic crisis during general anesthesia in a 45-year-old man with IgG4-related disease, COPD and athma undergoing removal of submandibular gland. The ventilatiory failure was caused by the stimulation of the operation, sputum, and neostigmine. His serum IgG4 level was extremely high. IgG4 related disease is a recently emerging entity characterized by a diffuse or mass forming inflammatory reaction rich in IgG4-positive plasma cells associated with fibrosclerosis and obliterative phlebitis. It is associated with an elevated serum level of IgG4 and an allergic disease. We must be careful in perioperative management of the patients with IgG4-related disease because general anesthesia can induce asthmatic crisis. PMID:24783608
Moriya, Machika; Oda, Shinya; Nakane, Masaki; Kawamae, Kaneyuki
IgG4-related disease is a recently recognized autoimmune systemic disorder that has been described in various organs. The disease is characterized histologically by a dense lymphoplasmocytic infiltrate of IgG4-positive cells, storiform fibrosis and can be associated with tumefactive lesions. IgG4-related disease involving the upper gastrointestinal tract is rare and only two previous case reports have reported IgG4-related esophageal disease. We report the case of a 63-year-old female patient with a long-standing history of severe dysphagia and odynophagia with an initial diagnosis of reflux esophagitis. Symptoms persisted despite anti-acid therapy and control esophagogastroduodenoscopy (EGD) revealed endoscopic images consistent with esophagitis dissecans superficialis (sloughing esophagitis). An underlying autoimmune process was suspected and immunosuppressant agents were tried to control her disease. The patient eventually developed disabling dysphagia secondary to multiple chronic esophageal strictures. A diagnosis of IgG4-related disease was eventually made after reviewing esophageal biopsies and performing an immunohistochemical study with an anti-IgG4 antibody. Treatment attempts with corticosteroids and rituximab was not associated with a significant improvement of the symptoms of dysphagia and odynophagia, possibly because of the chronic nature of the disease associated with a high fibrotic component. Our case report describes this unique case of IgG4-related esophageal disease presenting as chronic esophagitis dissecans with strictures. We also briefly review the main histopathological features and treatment options in IgG4-related disease. PMID:24883156
Dumas-Campagna, Myriam; Bouchard, Simon; Soucy, Genevieve; Bouin, Mickael
Immunoglobulin (Ig) G4-related disease is a recently recognized systemic fibroinflammatory condition characterized by a lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells with elevated circulating levels of IgG4. The disease can either be localized to one or two organs, or present as diffuse multi-organ disease. Furthermore, lesions in different organs can present simultaneously or metachronously. In the pulmonary manefestations, lesions associated with IgG4-related disease have been described in the lung parenchyma, airways and pleura, as well as the mediastinum. We report a case of IgG4-related disease presenting as massive pleural effusion and thrombophlebitis.
Choi, Jong Hyun; Sim, Jae Kyeom; Oh, Jee Youn; Lee, Eun Joo; Hur, Gyu Young; Lee, Seung Heon; Lee, Sung Yong; Kim, Je Hyeong; Lee, Sang Yeub; Shin, Chol; Shim, Jae Jeong; In, Kwang Ho; Kang, Kyung Ho
Immunoglobulin (Ig) G4-related disease is a recently recognized systemic fibroinflammatory condition characterized by a lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells with elevated circulating levels of IgG4. The disease can either be localized to one or two organs, or present as diffuse multi-organ disease. Furthermore, lesions in different organs can present simultaneously or metachronously. In the pulmonary manefestations, lesions associated with IgG4-related disease have been described in the lung parenchyma, airways and pleura, as well as the mediastinum. We report a case of IgG4-related disease presenting as massive pleural effusion and thrombophlebitis. PMID:24851132
Choi, Jong Hyun; Sim, Jae Kyeom; Oh, Jee Youn; Lee, Eun Joo; Hur, Gyu Young; Lee, Seung Heon; Lee, Sung Yong; Kim, Je Hyeong; Lee, Sang Yeub; Shin, Chol; Shim, Jae Jeong; In, Kwang Ho; Kang, Kyung Ho; Min, Kyung Hoon
IgG4-related sclerosing disease, a multiorgan system disease that has been identified in the last 10 years, is a fibroinflammatory condition with a marked propensity to manifest itself as mass forming lesions characterized by three main histological features (sclerosis, obliterative phlebitis and lymphoplasmacytic infiltrate) and by the presence of abundant IgG4+ plasma cells, frequent elevation of serum IgG4 and a dramatic initial response to steroid therapy. The aim of this mini-review is to increase the capacity to identify the characteristic features of IgG4-related sclerosing disease in specific organs and in two newly proposed entities (urethral caruncle and paratesticular fibrous pseudotumor) using biopsy specimens and methods of counting IgG4. In addition we examine the relationship between IgG4-related sclerosing disease and malignancy. In fact, an increased ability to recognize the characteristic features of IgG4-related sclerosing disease would play an extremely important role in avoiding unnecessary surgery in favor of initiating corticosteroid therapy. PMID:24341121
Mazzucchelli, Roberta; Racchini, Stefano; Barbisan, Francesca; Galosi, Andrea B; Giorgini, Sara
IgG4-related disease has evolved from originally being recognized as a form of pancreatitis to encompass diseases of numerous organs including the hypophysis and one reported case of dural involvement. A search of the University of Virginia, Division of Neuropathology files for 10 years identified ten cases of unexplained lymphoplasmacytic meningeal inflammation that we then evaluated using immunohistochemical stains for IgG4 and IgG. Ten control cases including sarcoidosis (4), tuberculosis (1), bacterial abscess (2), Langerhans cell histiocytosis (2), and foreign body reaction (1) were also examined. The number of IgG4-positive plasma cells was counted in five high power fields (HPFs) and an average per HPF was calculated. Cases that contained greater than ten IgG4-positive cells/HPF were considered to be IgG4-related. Five of the study cases met these criteria, including one case of leptomeningeal inflammation. All cases exhibited the typical histological features of IgG4-related disease including lymphoplasmacytic inflammation, fibrosis, and phlebitis. The dural-based lesions appear to represent a subset of the cases historically diagnosed as idiopathic hypertrophic pachymeningitis. While the leptomeningeal process most closely resembles non-vasculitic autoimmune inflammatory meningoencephalitis. Given these findings, IgG4-related meningitis should be considered in the differential diagnosis of meningeal inflammatory lesions after stringent clinical and histologic criteria are used to rule out other possible diagnoses. PMID:20844883
Lindstrom, Katherine M; Cousar, John B; Lopes, M Beatriz S
IMPORTANCE IgG4-related hypertrophic pachymeningitis (IgG4-RHP) is an increasingly recognized manifestation of IgG4-related disease, a fibroinflammatory condition that can affect virtually any organ. It is estimated that IgG4-RHP may account for a high proportion of cases of hypertrophic pachymeningitis once considered idiopathic. OBJECTIVE To summarize the current knowledge on IgG4-RHP including its pathological, clinical, and radiological presentations. Particular emphasis is placed on diagnostic and therapeutic implications. EVIDENCE REVIEW This review is based on 21 reports published in the English medical literature since 2009. PubMed was searched with the following terms: IgG4, pachymeningitis, IgG4-related pachymeningitis, IgG4-related disease, IgG4-related, and IgG4 meningitis. Only cases with biopsy-proven IgG4-RHP were considered and included in this review. FINDINGS Little is known with certainty regarding the pathogenesis of IgG4-RHP. The presence of oligoclonally restricted IgG4-positive plasma cells within inflammatory meningeal niches strongly suggests a specific response against a still unknown antigen. Clinical presentation of IgG4-RHP is not distinguishable from other forms of hypertrophic pachymeningitis and reflects mechanical compression of vascular or nerve structures, leading to functional deficits. Signs of systemic IgG4-related disease may concomitantly be present. Diagnostic process should rely primarily on magnetic resonance imaging, cerebrospinal fluid analysis, and meningeal biopsy. In particular, hallmark histopathological features of IgG4-RHP are a lymphoplasmacytic infiltration of IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. High-dose glucocorticoids are still the treatment of choice for IgG4-RHP because immunosuppressive agents have shown variable efficacy in reducing the meningeal hypertrophy. Rituximab is a promising therapeutic approach but experience with B-cell depletion strategies remains limited. CONCLUSIONS AND RELEVANCE IgG4-related disease accounts for an increasing proportion of cases of idiopathic hypertrophic pachymeningitis. Clinicians should become familiar with this alternative differential diagnosis because a prompt, specific therapeutic approach may avoid long-term neurological complications. PMID:24733677
Lu, Lucy X; Della-Torre, Emanuel; Stone, John H; Clark, Stephen W
Background An immunoglobulin G4 (IgG4)-related disease is important disease in differential diagnosis of tumors in kidney, pancreas, lung and other organs. The imaging findings of IgG4-related kidney diseases are usually expressed as defect contrast region, while cystic formation in kidney is extremely rare. Here, we report a case of IgG4-related tubulointerstitial nephritis with renal cystic change caused by the narrowing or obstruction of collecting duct in renal medulla. Case presentation Abdominal contrasted CT scan showed a 31 × 24 mm cystic tumor at the upper pole of the right kidney and multiple low-attenuation areas in the left kidney. 18?F-fluorodeoxyglucose (FDG)-PET/CT scan showed moderate FDG accumulation of cystic tumor in marginal lesion. In addition, FDG-PET/CT scan also showed moderate FDG accumulation in the pancreatic body. Laparoscopic right nephrectomy was performed. Histological examination was revealed lymphoplasmacytic infiltrate with focal fibrosis and severe narrowing or obstruction of lumen of collecting duct in renal medulla. Furthermore, the IgG4 positive plasma cells infiltrated exceeding 10 cells per one high-power field in renal medulla. The ratio of IgG4-plasma cells to IgG-positive plasma cells was about 50%. The serum level of IgG4 was also elevated (218 mg/dl). Based on these findings, we finally diagnosed IgG4-related tubulointerstitial nephritis with renal cystic change. Conclusion IgG4-related kidney disease might cause cystic formation by severe narrowing and obstruction of collecting duct.
A 70-year old man with a 14 year history of Sjögren syndrome, interstitial pneumonia, and autoimmune hepatitis (AIH) was admitted to our hospital due to hyponatremia with a one month history of fatigue, thirst, and nausea. Laboratory tests on admission revealed that this patient had a central adrenal insufficiency. Pituitary magnetic resonance imaging (MRI) further showed swelling of the stalk and posterior lobe of his pituitary, suggesting infundibulo-hypophysitis. Based on his past history of autoimmune disease, his serum IgG4 levels were measured and found to be remarkably high (924 mg/ dL). Previous biopsy specimens from his liver, lung, and parotid gland were immunostained for IgG4, which revealed a marked infiltration of IgG4-positive plasma cells. As a result of our tests, we made a diagnosis of IgG4-related systemic disease. Interestingly, a subsequent MRI scan at three weeks after the patient commenced glucocorticoid replacement therapy for adrenal insufficiency showed that the swelling of his pituitary stalk was reduced. This finding suggested that IgG4-related hypophysitis may improve either as a result of a supplemental dose of glucocorticoid or possibly spontaneously. Although six cases of IgG4-related hypophysitis have been reported in the scientific literature published in English, our current case is the first in which IgG4-related hypophysitis likely occurred as a result of a long-term history of IgG4-related systemic disease. We report this case herein and review the relevant literature. PMID:20371985
Hori, Michiko; Makita, Noriko; Andoh, Takahiro; Takiyama, Hirotoshi; Yajima, Yuki; Sakatani, Takashi; Fukumoto, Seiji; Iiri, Taroh; Fujita, Toshiro
Orbital and ocular andexal Mucosa-Associated Lymphoid Tissue Lymphoma (MALT) or ocular adnexal MALT lymphoma (OAML) is the most common of all eye non-Hodgkin lymphomas. Autoimmune inflammatory disorders and chronic infections are important etiological factors and CD5 and CD43 (sialophorin) tumor markers are significant negative prognostic factors. Disease signs and symptoms can occur a long time before diagnosis. Varieties of treatment options are available. The aim of this retrospective analysis was to compare the efficiency of different treatment options and to investigate disease outcome. Twenty OAML patients, diagnosed in the Clinic of Hematology, Clinical Centre of Serbia, between 2003 and 2013, were enrolled. In most cases, OAML developed in the eighth decade with greater incidence in the male population. Median age was 67.5 years. The median period between the appearance of local signs and symptoms and diagnosis was 7 months. The dominant sign at presentation was swelling of involved tissue (40%). The most common was orbital involvement (55%). All patients had localized disease. Observed laboratory parameters on presentation showed low disease activity. Sialophorin prognostic significance was not registered. Our patients were initially treated differently but there was no significant difference in progression-free survival (PFS) due to initial treatment option (p = 0.2957). Median PFS was 22 months (3-89), and 5-year PFS was 60%. Median overall survival (OS) was 43 months (1-105) and 5-year OS 95%. Eight patients (40%) relapsed and one patient died due to non-hematological complications. In our experience, most modern induction treatment options appear to result in the same, favorable outcome. PMID:24026660
Smiljanic, M; Milosevic, R; Antic, D; Andjelic, B; Djurasinovic, V; Todorovic, M; Bila, J; Bogdanovic, A; Mihaljevic, B
A 74-year-old woman with massive bilateral pleural effusion, which was exudative in nature, and with mononuclear cell predominance underwent a pleuroscopy. Parietal pleura were thickened and partly reddish in color. Biopsy specimens taken from the parietal pleura revealed lymphoplasmacytic inflammation with fibrosis. As her performance status rapidly worsened with thoracentesis, we performed bilateral pleurodesis using talc. Pathologic evaluation of the pleural biopsy specimen with immunohistochemical staining revealed 91 IgG4-positive plasma cells per high-power field and an IgG4/IgG ratio of 91%. Thus, the diagnosis of pleuritis from IgG4-related disease was established. Our case suggests that IgG4-related disease is one of the causes of pleural effusion, and it should be included in the differential diagnosis of unexplained pleuritis. PMID:24992134
Ishida, Atsuko; Furuya, Naoki; Nishisaka, Takashi; Mineshita, Masamichi; Miyazawa, Teruomi
IMPORTANCE IgG4-related disease (IgG4-RD) is characterized by an inflammatory reaction rich in IgG4-positive plasma cells. Head and brain involvement is rare in IgG4-RD, and brain parenchyma involvement has never been reported. OBSERVATION A man in his mid-50s with multiorgan IgG4-RD developed progressive spastic hemiparesis and dementia. Magnetic resonance imaging of the brain revealed several cortical and subcortical lesions. Pathologic findings in the brain were consistent with IgG4-related central nervous system involvement. The patient was treated with high-dose corticosteroids followed by rituximab, and his cognitive and motor functions improved significantly. CONCLUSIONS AND RELEVANCE IgG4-RD should be considered in patients with unusual neurologic manifestations suggestive of autoimmune disease. PMID:24781034
Regev, Keren; Nussbaum, Tami; Cagnano, Emanuela; Giladi, Nir; Karni, Arnon
(Case 1) A 63-year-old man was diagnosed as retroperitoneal fibrosis by the exploratory laparotomy for the pelvic mass with high IgG4 levels. (Case 2) A 64-year-old man had past medical history of autoimmune pancreatitis which was treated by steroid use. Three years later, he was diagnosed as IgG4-related gallbladder tumor by the cholecystectomy. And, then he was diagnosed as right hydronephrosis with high IgG4 levels. (Case 3) A 71-year-old man was diagnosed as left hydronephrosis and pelvic mass by computerized tomography with high IgG4 levels. We reported three cases of IgG4-related focal retroperitoneal fibrosis. All cases had pelvic mass with high IgG4 levels and were also treated effectively with steroid use. Those symptoms of the patients occurred in close association with IgG4 levels. It is necessary to acknowledge that retroperitoneal fibrosis may have aspects of IgG4-related systemic disease and that the measurement of serum IgG4 should be considered for diagnosing and treating the conditions. PMID:24908818
Yasuda, Yosuke; Iwai, Aki; Inadome, Yukinori; Okuno, Tetsuo
Context: Hypophysitis is a chronic inflammation of the pituitary gland that comprises an increasingly complex clinicopathological spectrum. Within this spectrum, lymphocytic and granulomatous hypophysitis are the most common forms, but newer variants have recently been reported. Objective: The aims of the study were to describe a new patient with IgG4-related hypophysitis, review the published literature, and provide diagnostic criteria. Setting: A 75-yr-old man presented with a 1-yr history of frontal headache. Initial studies revealed panhypopituitarism and a mass in both the sella turcica and the sphenoidal sinus. The patient underwent transphenoidal surgery, initiated high-dose prednisone followed by hormone replacement therapy, and was closely monitored for 3 yr. Results: Symptoms improved after prednisone, along with shrinkage of the pituitary and sphenoidal masses, but recurred when prednisone dose was lowered. Histopathology showed a marked mononuclear infiltrate in both the pituitary and sphenoidal specimens, mainly characterized by increased numbers of plasma cells. Many of the infiltrating plasma cells (>10 per high-power field) were IgG4-positive. Review of the literature identified 11 cases of IgG4-related hypophysitis (two diagnosed based on pituitary histopathology). Conclusions: We describe the first Caucasian patient with biopsy-proven IgG4-related hypophysitis and provide classification criteria for this disease.
Leporati, Paola; Landek-Salgado, Melissa A.; Lupi, Isabella; Chiovato, Luca
A 56-year-old man who had a 28mm renal mass on computed tomography (CT) pointed out at another hospital visited our department. The tumor was buried and located in the renal hilus, which was slightly enhanced in the early phase and washed out in the delayed phase. We diagnosed it as papillary renal cell carcinoma and conducted partial nephrectomy. The tumor was ocher and a solid nodule without capsule formation. Although there was no malignancy in histopathological examination, plasma cell infiltration was found and the IgG4/IgG-positive cell ratio was over 40%. Additionally, the serum IgG4 level was elevated postoperatively and the patient was diagnosed as having IgG4-related kidney disease. IgG4-related kidney disease is often found as multiple low-density lesions in CT. Because of the solitary nodule-like formation, it was difficult to distinguish from hypovascular renal cell carcinoma such as the papillary type. When a buried and solitary hypovascular tumor is detected, we must consider IgG4-related kidney disease as a differential diagnosis. Measuring the serum immunoglobulin and complement levels may be helpful for avoiding unnecessary surgery. PMID:23995528
Nofuji, Seisuke; Shindo, Tetsuya; Mizuno, Takahiro; Tanaka, Toshiaki; Kitamura, Hiroshi; Takahashi, Satoshi; Masumori, Naoya; Tsukamoto, Taiji
A 77-year-old man with jaundice and a pancreatic head tumor was referred to our hospital in August 2006. The initial laboratory tests, computed tomography (CT) scan, magnetic resonance imaging (MRI), and endoscopic retrograde cholangiopancreatography suggested IgG4-related cholangitis and autoimmune pancreatitis. Oral prednisolone (PSL) was then administered. This treatment reduced the size of the pancreatic parenchyma, and the lower common bile duct (CBD) returned to its normal size. Thus, the oral PSL was gradually tapered to a maintenance dose. In February 2010, a CT scan and MRI showed segmental wall thickening and stenosis of the middle CBD, the progression of which led to extrahepatic obstructive jaundice. We suspected the emergence of a cholangiocarcinoma rather than the exacerbation of the IgG4-related sclerosing cholangitis because the stricture of the CBD was short and localized. Then, a percutaneous transhepatic biliary drainage was performed. The biopsy specimens obtained via the percutaneous transhepatic tract indicated an abnormal glandular formation, suggesting the presence of a moderate, well-differencated adenocarcinoma. The gross examination, microscopic examination and immunohistochemical analysis of the pancreaticoduodenectomy specimen suggested that a cholangiocarcinoma developed from the IgG4-related sclerosing cholangitis.
Douhara, Akitoshi; Mitoro, Akira; Otani, Emi; Furukawa, Masanori; Kaji, Kosuke; Uejima, Masakazu; Sawai, Masayoshi; Yoshida, Motoyuki; Yoshiji, Hitoshi; Yamao, Junichi; Fukui, Hiroshi
A 77-year-old man with jaundice and a pancreatic head tumor was referred to our hospital in August 2006. The initial laboratory tests, computed tomography (CT) scan, magnetic resonance imaging (MRI), and endoscopic retrograde cholangiopancreatography suggested IgG4-related cholangitis and autoimmune pancreatitis. Oral prednisolone (PSL) was then administered. This treatment reduced the size of the pancreatic parenchyma, and the lower common bile duct (CBD) returned to its normal size. Thus, the oral PSL was gradually tapered to a maintenance dose. In February 2010, a CT scan and MRI showed segmental wall thickening and stenosis of the middle CBD, the progression of which led to extrahepatic obstructive jaundice. We suspected the emergence of a cholangiocarcinoma rather than the exacerbation of the IgG4-related sclerosing cholangitis because the stricture of the CBD was short and localized. Then, a percutaneous transhepatic biliary drainage was performed. The biopsy specimens obtained via the percutaneous transhepatic tract indicated an abnormal glandular formation, suggesting the presence of a moderate, well-differencated adenocarcinoma. The gross examination, microscopic examination and immunohistochemical analysis of the pancreaticoduodenectomy specimen suggested that a cholangiocarcinoma developed from the IgG4-related sclerosing cholangitis. PMID:24009815
Douhara, Akitoshi; Mitoro, Akira; Otani, Emi; Furukawa, Masanori; Kaji, Kosuke; Uejima, Masakazu; Sawai, Masayoshi; Yoshida, Motoyuki; Yoshiji, Hitoshi; Yamao, Junichi; Fukui, Hiroshi
IgG4-related lung disease is commonly associated with autoimmune pancreatitis. Recently, isolated IgG4-related interstitial lung disease (ILD) without other organ involvement has newly been reported in two cases with clinical features of nonspecific interstitial pneumonitis (NSIP).We report the first case of an isolated IgG4-related ILD in a 78-year-old man with dry cough and dyspnea, whose clinical findings proved to be different from NSIP. Serum IgG4 levels were increased. Chest CT scan revealed bilateral consolidations especially in the lower lobes, enlarged mediastinal and hilar lymph nodes and pleural effusions. Video-assisted thoracoscopic (VATS) lung biopsy revealed a pattern similar to usual interstitial pneumonia (UIP) and an abundant IgG4-positive plasma cell infiltration. He was effectively treated by steroid therapy.Increasing recognition of IgG4 related diseases has led to a growing number of new entities. The novel concept of isolated IgG4-related ILD as a pulmonary manifestation of a systemic IgG4-related disorder should be taken into account as a possible differential diagnosis of ILD and mass-forming lesions, even when no other organ manifestation is clinically apparent at the time of diagnosis. Lung specific diagnostic criteria and algorithms are required to enhance diagnostic accuracy in cases of possible IgG4-related ILD. PMID:23509921
Wibmer, Thomas; Kropf-Sanchen, Cornelia; Rüdiger, Stefan; Blanta, Ioanna; Stoiber, Kathrin M; Rottbauer, Wolfgang; Schumann, Christian
Purpose To compare genome-wide DNA methylation profiles according to Chlamydophila psittaci (Cp) infection status and the response to doxycycline treatment in Korean patients with ocular adnexal extranodal marginal zone B-cell lymphoma (EMZL). Methods Twelve ocular adnexal EMZL cases were classified into two groups (six Cp-positive cases and six Cp-negative cases). Among the 12 cases, eight were treated with doxycycline as first-line therapy, and they were divided into two groups according to their response to the treatment (four doxy-responders and four doxy-nonresponders). The differences in the DNA methylation states of 27,578 methylation sites in 14,000 genes were evaluated using Illumina bead assay technology. We also validated the top-ranking differentially methylated genes (DMGs) with bisulfite direct sequencing or pyrosequencing. Results The Infinium methylation chip assay revealed 180 DMGs in the Cp-positive group (74 hypermethylated genes and 106 hypomethylated genes) compared to the Cp-negative group. Among the 180 DMGs, DUSP22, which had two significantly hypomethylated loci, was validated, and the correlation was significant for one CpG site (Spearman coefficient=0.6478, p=0.0262). Regarding the response to doxycycline treatment, a total of 778 DMGs were revealed (389 hypermethylated genes and 336 hypomethylated genes in the doxy-responder group). In a subsequent replication study for representative hypomethylated (IRAK1) and hypermethylated (CXCL6) genes, the correlation between the bead chip analysis and pyrosequencing was significant (Spearman coefficient=0.8961 and 0.7619, respectively, p<0.05). Conclusions Ocular adnexal EMZL showed distinct methylation patterns according to Cp infection and the response to doxycycline treatment in this genome-wide methylation study. Among the candidate genes, DUSP22 has a methylation status that was likely attributable to Cp infection. Our data also suggest that the methylation statuses of IRAK1 and CXCL6 may reflect the response to doxycycline treatment.
Lee, Min Joung; Min, Byung-Joo; Choung, Ho-Kyung; Kim, Namju; Kim, Young A
IgG4-related inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT) share morphological features like a prominent fibroblastic/myofibroblastic proliferation and the presence of inflammatory cells. Since IPT is managed conservatively and IMT is treated by surgical excision, it is important to differentiate these two lesions. The aim of this study is to highlight morphological and immunohistochemical features that distinguish IPT and IMT. Clinicopathological characteristics of cases diagnosed as pulmonary IPT or IMT from 1997 to 2013 were reviewed. The histological features were studied on hematoxylin and eosin-stained sections. Immunohistochemistry was done for IgG, IgG4, ALK-1, SMA, desmin, and CD34 for classification into IPT and IMT. Of the ten patients, seven were male and the age ranged from 4 to 58 years. The tumor size ranged from 1.5 to 4.0 cm in diameter. Histologically, proliferation of bland-looking spindle cells along with fibrosis and an inflammatory infiltrate comprising of lymphocytes and plasma cells were the common morphological features of both lesions. The spindle cell proliferation was more marked in IMT whereas lymphoplasmacytic infiltrate was more prominent in IPT. Obstructive phlebitis was observed only in cases of IPT. IgG4 expression was noted in IPT, and the number of IgG4-positive plasma cells and the ratio of IgG4+/IgG+ plasma cells were significantly lower in IMT than in IgG4-related IPT. Expression of anaplastic lymphoma kinase (ALK) was observed only in IMT, but not in IgG4-related IPT. The proportion of proliferating spindle cells, lymphoplasmacytic infiltrate, obstructive phlebitis, IgG4+ plasma cells and the ratio of IgG4+/IgG+ plasma cells, and ALK expression are helpful in differentiating these morphologically similar but biologically different lesions, which require different treatment modalities. PMID:24100523
Bhagat, Priyanka; Bal, Amanjit; Das, Ashim; Singh, Navneet; Singh, Harkant
Recent studies have suggested the existence of two subtypes of autoimmune pancreatitis (AIP): type 1 AIP, related to IgG4 (lymphoplasmacytic sclerosing pancreatitis); and type 2 AIP, related to a granulocytic epithelial lesion (idiopathic duct-centric chronic pancreatitis). Compared with type 2 AIP, the clinicopathological features of type 1 AIP, with increased serum IgG4/IgE levels, abundant infiltration of IgG4 + plasmacytes and lymphocytes, autoantibodies, and steroid responsiveness, are more suggestive of abnormal immunity such as allergy or autoimmunity. Moreover, patients with type 1 AIP often have extrapancreatic lesions, such as sclerosing cholangitis, sclerosing sialadenitis, or retroperitoneal fibrosis, showing pathological features similar to those of the pancreatic lesions. Based on these findings, an international concept of and diagnostic criteria for AIP have been proposed recently. Of interest, many synonyms have been proposed for the conditions of AIP and extrapancreatic lesions associated with IgG4, such as "multifocal idiopathic fibrosclerosis," "IgG4-related autoimmune disease," "IgG4-related sclerosing disease," "systemic IgG4-related plasmacytic syndrome (SIPS)," and "IgG4-related multiorgan lymphoproliferative syndrome," all of which may refer to the same conditions. Therefore, the Japanese Research Committee for "Systemic IgG4-Related Sclerosing Disease" proposed a disease concept and clinical diagnostic criteria based on the concept of multifocal fibrosclerosing disease, in 2009, in which the term "IgG4-related disease" was agreed upon as a minimal consensus to cover these conditions. Although the significance of IgG4 in the development of "IgG4-related disease" remains unclear, we have proposed a hypothesis for the development of type 1 AIP, one of the IgG4-related diseases. The concept and diagnostic criteria of "IgG4-related disease" will be changed in accordance with future studies. PMID:21452084
Okazaki, Kazuichi; Uchida, Kazushige; Koyabu, Masanori; Miyoshi, Hideaki; Takaoka, Makoto
A 54-year-old woman with suspected low-grade B-cell lymphoma of mucosa-associated lymphoid tissue type of the eyelids underwent rituximab-containing chemotherapy. She initially responded to the rituximab therapy, but later experienced two recurrences over a 3-year period. Biopsy specimens and a review of her previous histology revealed that she had had immunoglobulin G4-related disease at the initial presentation. Although IgG4-related disease seems to respond well to rituximab therapy, long-term follow up, including disease monitoring, is needed to evaluate disease remission. PMID:22956241
Murakami, Jun; Matsui, Shoko; Ishizawa, Shin; Arita, Kotaro; Wada, Akinori; Miyazono, Takayoshi; Hounoki, Hiroyuki; Shinoda, Koichiro; Taki, Hirofumi; Sugiyama, Toshiro
The hallmarks of IgG4-related disease (IgG4-RD) are lymphoplasmacytic tissue infiltration with a predominance of IgG4-positive plasma cells, accompanied by fibrosis, obliterative phlebitis, dacryoadenitis, and elevated levels of IgG4. In a recent issue of Arthritis Research & Therapy, Tsuboi and colleagues demonstrated that regulatory T (Treg) cell-and T helper 2 (Th2) cell-derived cytokines contribute to the pathogenesis of Mikulicz's disease, an activation pathway that appears to be common for IgG4-RD. Additional organ-specific factors may account for the different organ involvement of IgG4-RD.
Immunoglobulin G4 (IgG4)-related disease (also known as hyper-IgG4 disease) is a recently defined emerging condition with highly heterogeneous clinicopathological features and variable disease manifestations. This disorder is characterized by unifocal or multifocal (multiorgan) involvement by tumefactive plasma cell-rich inflammatory infiltrates associated with prominent fibrosclerosis. This not uncommonly interferes with organ function resulting in diverse clinical symptoms. The autoimmune pancreatitis represents the prototype of this disease; however, to date almost all organs have been reported to be involved in this disorder. In the head and neck area several presentations of this disease may be encountered in salivary glands, lacrimal glands, thyroid gland, lymph nodes, soft tissue of the neck, ear and sinonasal tract. However, IgG4 positive plasma cells are occasionally prominent in non-specific chronic inflammatory conditions of the head and neck and the oral cavity unrelated to autoimmune diseases or systemic disorders, thus representing diagnostic pitfalls. The diagnosis of IgG4-related disease should be based on a combination of typical histological, clinical and serological findings. PMID:24619525
Agaimy, A; Ihrler, S
IgG4-related disease (IgG4-RD) is a systemic disease, mostly affecting the pancreas. It presents as accumulation of IgG4-producing plasma cells in various tissues. Other possible affected organs include the lacrimal glands, salivary glands, lungs, kidneys, liver, bile duct, retroperitoneum, breast, aorta, pituitary gland and prostate. A cutaneous presentation has also been described in the literature, and might be the initial presenting feature of IgG4-RD. We describe a 73-year-old white man who presented with two infiltrated, erythematous nodules on his abdomen. The histopathological characteristics were highly suggestive of IgG4-related cutaneous disease. Immunohistochemical stains were positive for IgG4. In the past, the patient's other organs had also been affected by IgG4-RD. Cutaneous presentation of IgG4-RD has been described previously in the literature but only in Asian patients (both East and South Asian). We also provide an overview of previously reported cutaneous manifestations of IgG4-RD. PMID:24758575
Kempeneers, D; Hauben, E; De Haes, P
Patients with autoimmune pancreatitis have a striking polyclonal elevation of total IgG4 in serum. This observation has been confirmed and extended to other fibrotic conditions (that are therefore called IgG4-related disease) but as yet remains unexplained. The affected tissue contains many IgG4-producing plasma cells embedded in a fibrotic matrix originating from activated mesenchymal (stellate) cells. We propose that the process results from an unusual interaction between two regulatory systems: the regulatory arm of the immune system (including Bregs) and the tissue repair regulatory components orchestrated by the activated stellate cell. This interaction results in ongoing mutual activation, generating TGFbeta, IL10, and vitamin D. This environment suppresses most immune reactions but stimulates the development of IgG4-producing plasma cells.
Lighaam, Laura C.; Aalberse, Rob C.; Rispens, Theo
IgG4-related disease (IgG4-RD) is associated with the infiltration of IgG4-positive plasma cells into various organs. Nephropathy of IgG4-RD is generally interstitial nephritis and glomerulonephritis is rare. We describe a case of membranous nephropathy (MN) without interstitial nephritis associated with IgG4-RD symptoms including lymphadenopathy and pulmonary and pleural lesions. Treatment with steroids improved these clinical symptoms, but withdrawal of steroids induced the repeated relapse of MN. Finally, flaring of MN was prevented by the combination of steroids and cyclosporine. This is the first report of the successful treatment of MN associated with IgG4-RD by this combination therapy.
Kanda, Hiroko; Koya, Junji; Uozaki, Hiroshi; Tateishi, Shoko; Sato, Kojiro; Hagino, Noboru; Sawada, Tetsuji; Yamamoto, Kazuhiko
Recently, autoimmune pancreatitis, a pancreatic manifestation of IgG4-related disease (IgG4-RD) has been recognized as a novel clinical entity associated with massive infiltration of IgG4-positive cells. The first international symposium on IgG4-RD endorsed the comprehensive nomenclature as IgG4-RD, which the Japanese research committee supported by the Ministry of Health, Labor and Welfare of Japan proposed in 2009, and proposed the individual nomenclatures for each organ system manifestations and the international pathologic consensus in 2011. In addition to the pathological consensus, the Japanese comprehensive diagnostic criteria (CDC) for IgG4-RD for general use, and several organ specific criteria for the organ specified physicians have been proposed; the International Consensus Diagnostic Criteria and the revised clinical diagnostic criteria in 2011 by Japan Pancreas Society (JPS-2011) for type1 AIP, the Clinical Diagnostic Criteria 2012 for IgG4-sclerosing cholangitis (IgG4-SC-2012), the diagnostic criteria for IgG4-positive Mikulicz's disease by the Japanse Society for Sjogren's syndrome, and Diagnostic criteria for IgG4-related kidney disease by the Japanese Society of Nephrology. Although the pathogenic mechanism still remains unclear, we have proposed a hypothesis of the pathogenic mechanism; abnormal innate and acquired immunity, regulatory T cells, and B cells on abnormal genetic backgrounds may be involved in the development of IgG4-cholangiopathy. Further studies are necessary to clarify the pathogenesis including genetic backgrounds, disease specific antigens, and the role of IgG4. PMID:24727875
IgG4-related sclerosing disease is an established disease entity with characteristic clinicopathological features. Some recent reports have demonstrated that this disease can occur in the respiratory system including the pleura. Herein, we describe the first documented case of concomitant occurrence of IgG4-related pleuritis and periaortitis. A 71-year-old Japanese female with a history of essential thrombocythemia presented with persistent cough and difficulty in breathing. Computed tomography demonstrated thickening of the right parietal pleura, pericardium, and periaortic tissue and pleural and cardiac effusions. Histopathological study of the surgical biopsy specimen of the parietal pleura revealed marked fibrous thickening with lymphoplasmacytic infiltration. Phlebitis was noted, however, only a few eosinophils had infiltrated. Immunohistochemical study revealed abundant IgG4-positive plasma cell infiltration and high ratio of IgG4-/IgG-positive plasma cells (84%). Therefore, a diagnosis of IgG4-related pleuritis was made with consideration of the elevated serum IgG4 level (684 mg/dL). Recently, the spectrum of IgG4-related sclerosing disease has expanded, and this disease can occur in the pleura, pericardium, and periaortic tissue. Although histopathological analysis of the pericardium and periaortic tissue was not performed in the present case, it was suspected that thickening of the pericardium and periaortic tissue was clinically due to IgG4-related sclerosing disease. Our clinicopathological analyses of IgG4-related pleuritis and pericarditis reveal that this disease can present as dyspnea and pleural and pericardial effusion as seen in the present case, therefore, it is important to recognize that IgG4-related sclerosing disease can occur in these organs for accurate diagnosis and treatment.
Ishida, Mitsuaki; Hodohara, Keiko; Furuya, Aya; Fujishiro, Aya; Okuno, Hiroko; Yoshii, Miyuki; Horinouchi, Akiko; Shirakawa, Ayaka; Harada, Ayumi; Iwai, Muneo; Yoshida, Keiko; Kagotani, Akiko; Yoshida, Takashi; Okabe, Hidetoshi
IgG4-related disease (IgG4-RD) is a newly recognized condition that is characterized by raised levels of serum IgG4, tissue infiltration of IgG4-positive plasma cells and presence of fibrosis. It affects multiple organs, including the pancreas, bile duct, and lacrimal and salivary glands. Skin lesions have rarely been reported, and those that have were described as distributed mainly in the head and neck region. We report a case of IgG4-RD with autoimmune pancreatitis and skin lesions on the trunk and limbs. The skin lesions responded well to oral prednisolone (PSL); however, tapering of PSL to 5 mg/day resulted in recurrence. At present, the skin disease is well controlled at a dose of 7 mg/day. Interestingly, IgG4 levels fluctuated with changes in the PSL dose but did not coincide with the severity of the skin disease, implying that the raised levels of IgG4 did not directly influence the skin symptoms. PMID:24986022
Ise, M; Yasuda, F; Suzaki, R; Kurihara, S; Konohana, I
Isolated IgG4 tubulointerstitial nephritis (TIN) is a rare disorder characterized by raised serum IgG4 levels and histological findings of dense lymphoplasmacytic infiltrates rich in IgG4 positive plasma cells. We report a case of isolated IgG4 TIN that presented with acute kidney injury in an 84 year old man with a polyclonal increase in his total IgG and a raised IgE of 381 kUA/L but without evidence of systemic autoimmunity. We draw a parallel with IgG4-related autoimmune pancreatitis and show raised levels of circulating regulatory T cells. Importantly the plasma levels of the T regulatory cell cytokine, IL10, the TH1 cytokines IL12 and IFN?, the proinflammatory TNF ? and immune regulatory IL27 were all highly raised. Furthermore, the level of IL21 that promotes IgG4 production was also very significantly elevated. These results suggest efforts of the immune system to reduce inflammation and suppress an exaggerated Th2 response. A raised serum IgG in the setting of acute kidney injury and in the absence of autoimmunity and chronic infection should encourage an assessment of the IgG subclasses. Prompt steroid treatment of those with a raised IgG4 may reduce ongoing renal damage. PMID:24789687
Lee, Lennard Y W; Yap, Hsiu; Sampson, Steve; Ford, Brian; Hayman, Grant; Marsh, James; Bansal, Amolak S
IgG4-related systemic disease is an emerging disease process that manifests with a constellation of features, most commonly but not exclusive to swelling and tuberous growth in the lacrimal and salivary glands, potentially involving many other organ systems. This condition often prompts investigations into malignancy or needless radical surgical procedures. A 58-year-old male was presented to a rheumatologist after several biopsies were done that were suspicious for neoplasia, involving the lacrimal gland and lung. The diagnosis was confirmed when tissue from the lacrimal gland biopsy was reviewed with special stains for IgG4, performed at the Mayo Clinic. This patient is interesting because his disease included bilateral lacrimal glands--at different intervals, the submandibular glands, the lung, and the thyroid gland. His disease responded to immunosuppression. Literature has shown resolution of the tumors upon starting glucocorticoids or rituximab. Our patient was given a course of prednisone and methotrexate with normal follow-up CT chest and physical exam. PMID:23370855
Masri, Karim Richard; Shaver, Timothy
Four patients with IgG4-related disease (IgG4-RD) showed increased percentages of RP105-negative B cells in the peripheral blood. Case 1: A 66-year-old man having retroperitoneal fibrosis had 18.8% of RP105-negative B cells. Oral prednisolone improved the affected lesions and the percentage of RP105-negative B cells decreased (3.2%) after the treatment. Case 2: A 53-year-old man with retroperitoneal fibrosis had 27.9% of RP105-negative B cells. Case 3: A 38-year-old man with follicular hyperplasia showed increased percentage of RP105-negative B cells (8.3%). Case 4: A 60-year-old man with interstitial nephritis had 27.5% of RP105-negative B cells. The treatment decreased the numbers of RP105-negative B cells. Increased numbers of RP105-negatvie B cells is possibly associated with disease activity of IgG4-RD. Analysis of expression of RP105 on B cells may be helpful in evaluation of disease activity of IgG4-RD.
Koarada, S; Tashiro, S; Nagao, N; Suematsu, R; Ohta, A; Tada, Y
Fibrosing mediastinitis (FM) and IgG4-related disease (IgG4-RD) are two fibroinflammatory disorders with potentially overlapping clinical and radiological features. In this paper, we looked for histopathologic features of IgG4-RD and enumerated infiltrating IgG4-positive plasma cells within mediastinal tissue biopsies from FM patients. We identified 15 consecutive FM surgical mediastinal tissue biopsies between 1985 and 2006. All patients satisfied the clinical and radiological diagnostic criteria for FM. All patients had either serological or radiological evidence of prior histoplasmosis or granulomatous disease, respectively. Formalin-fixed paraffin-embedded tissue sections of all patients were stained for H&E, IgG, and IgG4. Three samples met the predefined diagnostic criteria for IgG4-RD. In addition, characteristic histopathologic changes of IgG4-RD in the absence of diagnostic numbers of tissue infiltrating IgG4-positive plasma cells were seen in a number of additional cases (storiform cell-rich fibrosis in 11 cases, lymphoplasmacytic infiltrate in 7 cases, and obliterative phlebitis/arteritis in 2 cases). We conclude that up to one-third of histoplasmosis or granulomatous-disease-associated FM cases demonstrate histopathological features of IgG4-RD spectrum. Whether these changes occur as the host immune response against Histoplasma or represent a manifestation of IgG4-RD remains to be determined. Studies to prospectively identify these cases and evaluate their therapeutic responses to glucocorticoids and/or other immunosuppressive agents such as rituximab are warranted. PMID:22654916
Peikert, Tobias; Shrestha, Bijayee; Aubry, Marie Christine; Colby, Thomas V; Ryu, Jay H; Sekiguchi, Hiroshi; Smyrk, Thomas C; Specks, Ulrich; Yi, Eunhee S
Fibrosing mediastinitis (FM) and IgG4-related disease (IgG4-RD) are two fibroinflammatory disorders with potentially overlapping clinical and radiological features. In this paper, we looked for histopathologic features of IgG4-RD and enumerated infiltrating IgG4-positive plasma cells within mediastinal tissue biopsies from FM patients. We identified 15 consecutive FM surgical mediastinal tissue biopsies between 1985 and 2006. All patients satisfied the clinical and radiological diagnostic criteria for FM. All patients had either serological or radiological evidence of prior histoplasmosis or granulomatous disease, respectively. Formalin-fixed paraffin-embedded tissue sections of all patients were stained for H&E, IgG, and IgG4. Three samples met the predefined diagnostic criteria for IgG4-RD. In addition, characteristic histopathologic changes of IgG4-RD in the absence of diagnostic numbers of tissue infiltrating IgG4-positive plasma cells were seen in a number of additional cases (storiform cell-rich fibrosis in 11 cases, lymphoplasmacytic infiltrate in 7 cases, and obliterative phlebitis/arteritis in 2 cases). We conclude that up to one-third of histoplasmosis or granulomatous-disease-associated FM cases demonstrate histopathological features of IgG4-RD spectrum. Whether these changes occur as the host immune response against Histoplasma or represent a manifestation of IgG4-RD remains to be determined. Studies to prospectively identify these cases and evaluate their therapeutic responses to glucocorticoids and/or other immunosuppressive agents such as rituximab are warranted.
Peikert, Tobias; Shrestha, Bijayee; Aubry, Marie Christine; Colby, Thomas V.; Ryu, Jay H.; Sekiguchi, Hiroshi; Smyrk, Thomas C.; Specks, Ulrich; Yi, Eunhee S.
Extrahepatic bile ducts are the most commonly involved extrapancreatic organ site in patients with type 1 autoimmune pancreatitis. IgG4-related sclerosing cholangitis (IgG4-SC) alone, without evidence of pancreatic or other organ involvement, is uncommon and is difficult to distinguish from cholangiocarcinoma preoperatively. We describe 9 patients with isolated IgG4-SC over an approximate 10-year period, each clinically suspected to have cholangiocarcinoma. We examined the clinical, radiological, cytologic (including fluorescence in situ hybridization results), and histologic features. IgG and IgG4 immunohistochemistry were performed. All 9 patients were middle-aged men who presented with obstructive jaundice. The biliary strictures involved all parts of the extrahepatic biliary tree. Serum IgG4 was slightly elevated in three of eight patients. Cytologic findings were interpreted as negative in six, atypical in one, and suspicious for adenocarcinoma in one. Fluorescence in situ hybridization revealed aneuploidy in one and was equivocal (trisomy 7) in 2. Eight of 9 patients underwent radical resection for suspected cholangiocarcinoma. There was only one case diagnosed with IgG4-SC preoperatively based on biopsy. Histologic sections revealed a prominent lymphoplasmacytic infiltrate with storiform fibrosis and marked increased IgG4-positive plasma cells (?30/high-power field) in all specimens. Fifty percent of cases (4/8) had IgG4/IgG plasma cell ratio >40%. On median follow-up of 2.8 years, no relapse has occurred in any patient. Extrahepatic IgG4-SC may present as an isolated lesion mimicking cholangiocarcinoma. The diagnosis can be challenging. Clinicians and pathologists should recognize this to avoid major surgery. PMID:24890945
Graham, Rondell P D; Smyrk, Thomas C; Chari, Suresh T; Takahashi, Naoki; Zhang, Lizhi
Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis that is categorized as type 1 or type 2 according to the clinical profile. Type 1 AIP, which predominantly presents in a few Asian countries, is a hyper-IgG4-related disease. We report a case of IgG4-related AIP overlapping with Mikulicz's disease and lymphadenitis, which is rare and seldom reported in literature. A 63-year male from Northeast China was admitted for abdominal distension lasting for one year. He presented symmetric swelling of the parotid and submandibular glands with slight dysfunction of salivary secretion for 6 mo. He had a 2-year history of bilateral submandibular lymphadenopathy without pain. He underwent surgical excision of the right submandibular lymph node one year prior to admission. He denied any history of alcohol, tobacco, or illicit drug use. Serological examination revealed high fasting blood sugar level (8.8 mmol/L) and high level of IgG4 (15.2 g/L). Anti-SSA or anti-SSB were negative. Computed tomography of the abdomen showed a diffusely enlarged pancreas with loss of lobulation. Immunohistochemical stain for IgG4 demonstrated diffuse infiltration of IgG4-positive plasma cells in labial salivary gland and lymph node biopsy specimens. The patient received a dose of 30 mg/d of prednisone for three weeks. At this three-week follow-up, the patient reported no discomfort and his swollen salivary glands, neck lymph node and pancreas had returned to normal size. The patient received a maintenance dose of 10 mg/d of prednisone for 6 mo, after which his illness had not recurred. PMID:24409081
Qu, Li-Mei; Liu, Ya-Hui; Brigstock, David R; Wen, Xiao-Yu; Liu, Yong-Fang; Li, Ya-Jun; Gao, Run-Ping
IgG4-related disease has been recently defined as a distinct clinic-pathologic entity, characterized by dense IgG-4 plasmacytic infiltration of diverse organs, fibrosis, and tumefactive lesions. Salivary and lacrimal glands are a target of this disease and, when affected, may clinically resemble Küttner tumor, Mikulicz disease, or orbital inflammatory pseudotumor. In some patients, the disease is systemic, with metachronous involvement of multiple organs, including the pancreas, aorta, kidneys, and biliary tract. We report a 66-year old man who presented with salivary gland enlargement and severe salivary hypofunction and was diagnosed with IgG4-related disease on the basis of a labial salivary gland biopsy. Additional features of his illness included a marked peripheral eosinophilia, obstructive pulmonary disease, and lymphoplasmacytic aortitis. He was evaluated in the context of a research registry for Sjögren syndrome and was the only one of 2594 registrants with minor salivary gland histopathologic findings supportive of this diagnosis.
Baer, Alan N.; Gourin, Christine; Westra, William H.; Cox, Darren; Greenspan, John; Daniels, Troy E.
IgG4-related disease was first described in adults with autoimmune pancreatitis but is now known to affect multiple organs. Lung involvement has never been described in children to our knowledge. Here, we report an adolescent presenting with recurrent dry cough and hemoptysis who was found to have venous ectasia in the left upper lobe, and diffuse bronchiectasis. Sustained high levels of IgG4 (1,090 mg/dL) were found, and the endobronchial biopsy revealed a marked infiltration of plasma cells producing IgG4 (ratio of IgG4 plasma cells to IgG plasma cells >50%). This unique case highlights the occurrence of IgG4-related disease in a child and underscores the importance of careful scrutiny of all investigations in complex pediatric respiratory cases. PMID:23918112
Pifferi, Massimo; Di Cicco, Maria; Bush, Andrew; Caramella, Davide; Chilosi, Marco; Boner, Attilio L
Eight years after an episode of multiple IgG4-related disease, a pituitary mass with panhypopituitarism and a visual disturbance developed in a 70-year-old man under low-dose steroid therapy. A pituitary biopsy revealed findings of lymphocytic hypophysitis with the absence of IgG4-positive plasma cell infiltration. The serum IgG4 level was unremarkable. Although performing a pituitary biopsy and measuring the serum IgG4 level is crucial for making a diagnosis of IgG4-related hypophysitis, it is occasionally difficult to diagnose the disease in patients treated with steroid therapy, as observed in the present case. Based on a review of the diagnosis, conducting a careful assessment is required, especially in men and elderly patients thought to have solitary hypophysitis. PMID:24694491
Ohkubo, Yohsuke; Sekido, Takashi; Takeshige, Keiko; Ishi, Hiroaki; Takei, Masahiro; Nishio, Shin-ichi; Yamazaki, Masanori; Komatsu, Mitsuhisa; Kawa, Shigeyuki; Suzuki, Satoru
IgG4-related systemic disease encompasses multi-organ disorders, including tubulointerstitial nephritis. This disease is accompanied by a high serum IgG4 concentration and IgG4-positive plasma cell infiltration. We herein describe a 63-year-old woman with renal failure and dryness of the eyes and mouth, who had been treated with antituberculosis agents for urinary tract tuberculosis. She had a negative finding for a PCR analysis for Mycobacterium tuberculosis, a positive QuantiFERON-TB test, high serum IgG4 concentrations (2,660 mg/dl), and low serum IgM and IgA concentrations (34 and 82 mg/dl, respectively). Imaging tests revealed swelling in the submandibular glands, pancreas, and right kidney. A renal biopsy showed IgG4-positive plasma cell infiltration in the interstitium and tubular atrophy. This case was diagnosed as IgG4-related systemic disease. Corticosteroid therapy improved renal failure and swelling in the submandibular glands, pancreas, and right kidney. The case suggests that an abnormal reaction to tuberculosis may be associated with a predominance of type-2 helper T-cell immunity, thus resulting in IgG4-related systemic disease. PMID:22218636
Imai, Toshimi; Yumura, Wako; Takemoto, Fumi; Kotoda, Atsushi; Imai, Reika; Inoue, Makoto; Hironaka, Mitsugu; Muto, Shigeaki; Kusano, Eiji
Abstract We describe the case of a 75-year-old woman who presented with acute loss of vision. She experienced subacute headache, hearing loss on the left side, hoarseness, and dysphagia during the previous 10 months. On admission, she had bilateral loss of vision, without any ophthalmological abnormalities, and multiple cranial nerve palsies, including left hearing loss and right IX, X, and XI nerve palsies. Head magnetic resonance imaging with contrast enhancement revealed hypertrophic pachymeningitis. Laboratory findings showed no abnormalities except for an increased sedimentation rate and increased C-reactive protein levels. A biopsy of the dura mater was performed, and histopathological analysis revealed inflammatory thickening of the dura mater with plasma cell infiltration; the infiltrating cells were immunoreactive to an anti-IgG4 antibody, thereby indicating an IgG4-related disorder. Furthermore, the histopathological analysis revealed evidence of vasculitis in both veins and arteries. After corticosteroid treatment, her visual acuity dramatically improved. Acute loss of vision with multiple cranial nerve palsies may be an uncommon presentation of IgG4-related hypertrophic pachymeningitis. However, it should be recognized that these conditions might be underdiagnosed. The possibility of central nervous system involvement in IgG4-related disorders should be considered in patients with multiple cranial nerve neuropathies associated with hypertrophic pachymeningitis, even in the absence of systemic sclerosis symptoms. In our case, early treatment with corticosteroids showed immediate effectiveness in correcting the visual symptoms. (Received October 3, 2013; Accepted November 13, 2013; Published July 1, 2014). PMID:24998832
Tsugawa, Jun; Ouma, Shinji; Fukae, Jiro; Tsuboi, Yoshio; Sakata, Noriyuki; Inoue, Toru
We report a case of Henoch- Schönlein purpura nephritis (HSPN) associated with tubulointerstitial nephritis (TIN) and chronic sclerosing sialoadenitis. The patient is a 75-year-old Japanese woman who had bilateral submandibular gland swelling, palpable purpura on the lower legs, and decreased renal function with hematoproteinuria and marked hypocomplementemia, but no skin lesion suggestive of systemic lupus erythematosus (SLE), and did not fulfill the classification criteria for SLE. Her serum IgG4 level was high and immunostaining of renal biopsies revealed marked infiltration by IgG4-positive plasma cells in the interstitium, confirming the diagnosis of IgG4- related disease. On the other hand, glomeruli showed endocapillary proliferative glomerulonephritis with mesangial IgA and C3 deposition demonstrated by immunofluorescence staining, which were typical glomerular lesions for HSPN. The glomerular and tubulointerstitial lesions responded to steroid therapy dramatically, and her renal function recovered to within the normal range within a month. This case suggests a possible new association between HSPN and IgG4-related disease. PMID:22841022
Ito, Kiyoaki; Yamada, Kazunori; Mizushima, Ichiro; Aizu, Motohiko; Fujii, Hiroshi; Mizutomi, Kazuaki; Matsumura, Masami; Hayashi, Kenshi; Yamagishi, Masakazu; Umehara, Hisanori; Yamaguchi, Yutaka; Nagata, Michio; Kawano, Mitsuhiro
Purpose: To evaluate the long-term treatment outcome and disease behavior of primary ocular adnexal MALT (mucosa-associated lymphoid tissue) lymphoma (POAML) after treatment with radiotherapy. Methods and Materials: Seventy-eight patients (42 male, 36 female) diagnosed with stage I POAML between 1991 and 2010 at Kobe University Hospital were included. The median age was 60 years (range, 22-85 years). The median radiation dose administered was 30.6 Gy. Rituximab-based targeted therapy and/or chemotherapy was performed in 20 patients (25.6%). Local control (LC), recurrence-free survival (RFS), and overall survival (OS) rates were calculated using the Kaplan-Meier method. Results: The median follow-up duration was 66 months. Major tumor sites were conjunctiva in 37 patients (47.4%), orbita in 29 (37.2%), and lacrimal glands in 12 (15.4%). The 5- and 10-year OS rates were 98.1% and 95.3%, respectively. The 5- and 10-year LC rates were both 100%, and the 5- and 10-year RFS rates were 88.5% and 75.9%, respectively. Patients treated with a combination of radiotherapy and targeted therapy and/or chemotherapy had a trend for a better RFS compared with those treated with radiotherapy alone (p = 0.114). None developed greater than Grade 2 acute morbidity. There were 14 patients who experienced Grade 2 morbidities (cataract: 14; retinal disorders: 7; dry eye: 3), 23 patients who had Grade 3 morbidities (cataract: 23; dry eye: 1), and 1 patient who had Grade 4 glaucoma. Conclusions: Radiotherapy for POAML was shown to be highly effective and safe for LC and OS on the basis of long-term observation. The absence of systemic relapse in patients with combined-modality treatment suggests that lower doses of radiation combined with targeted therapy may be worth further study.
Hashimoto, Naoki [Division of Radiation Oncology, Kobe University Graduate School of Medicine, Hyogo (Japan); Sasaki, Ryohei, E-mail: email@example.com [Division of Radiation Oncology, Kobe University Graduate School of Medicine, Hyogo (Japan); Nishimura, Hideki; Yoshida, Kenji; Miyawaki, Daisuke; Nakayama, Masao; Uehara, Kazuyuki; Okamoto, Yoshiaki; Ejima, Yasuo [Division of Radiation Oncology, Kobe University Graduate School of Medicine, Hyogo (Japan); Azumi, Atsushi [Division of Ophthalmology, Kobe University Graduate School of Medicine, Hyogo (Japan); Matsui, Toshimitsu [Division of Hematology, Kobe University Graduate School of Medicine, Hyogo (Japan); Sugimura, Kazuro [Division of Radiation Oncology, Kobe University Graduate School of Medicine, Hyogo (Japan)
A 55-year-old woman presented with frequent episodes of syncope due to sinus pauses. During ambulatory Holter monitoring, atrial fibrillation and first-degree atrioventricular nodal block were observed. Magnetic resonance imaging and CT scans showed a tumor-like mass from the superior vena cava to the right atrial septum. Open chest cardiac biopsy was performed. The tumor was composed of proliferating IgG4-positive plasma cells and lymphocytes with surrounding sclerosis. The patient was diagnosed with IgG4-related sclerosing disease. Because of frequent sinus pauses and syncope, a permanent pacemaker was implanted. The cardiac mass was inoperable, but it did not progress during the one-year follow-up.
Song, Changho; Koh, Myoung Ju; Yoon, Yong-Nam; Joung, Boyoung
Immunoglobulin type gamma 4 (Ig)G4-related disease (IgG4-RD) is a relatively recently described clinical entity characterised by elevated levels of serum IgG4 and tissue infiltration of IgG4+ plasma cells in various organ systems. Cutaneous involvement is rare but is becoming increasingly appreciated; typically presenting as erythematous papules and/or nodules that are commonly pruritic. We report a case of IgG4-RD presenting with persistent pruritic papules and unilateral parotid swelling. His serum IgG4 level was elevated and a histological examination of his skin biopsies found a lymphoplasmacytic infiltration with an excess of IgG4+ non-clonal plasma cells. The patient was intolerant of oral prednisolone, however complete resolution of the cutaneous lesions was achieved with the anti-CD20 antibody, rituximab. PMID:24032760
Jalilian, Chris; Prince, H Miles; McCormack, Chris; Lade, Stephen; Cheah, Chan Y
IgG4-related sclerosing disease is an established disease entity with characteristic clinicopathological features. Recently, the association between IgG4-related sclerosing disease and the risk of malignancies has been suggested. IgG4-related autoimmune pancreatitis with pancreatic cancer has been reported. Further, a few cases of extraocular malignant lymphoma in patients with IgG4-related sclerosing disease have also been documented. Herein, we describe the first documented case of anaplastic large cell lymphoma (ALCL) following IgG4-related autoimmune pancreatitis and cholecystitis and diffuse large B-cell lymphoma (DLBCL). A 61-year-old Japanese male, with a past history of DLBCL, was detected with swelling of the pancreas and tumorous lesions in the gallbladder. Histopathological study of the resected gallbladder specimen revealed diffuse lymphoplasmacytic infiltration with fibrosclerosis in the entire gallbladder wall. Eosinophilic infiltration and obliterative phlebitis were also noted. Immunohistochemically, many IgG4-positive plasma cells had infiltrated into the lesion, and the ratio of IgG4/IgG-positive plasma cells was 71.6%. Accordingly, a diagnosis of IgG4-related cholecystitis was made. Seven months later, he presented with a painful tumor in his left parotid gland. Histopathological study demonstrated diffuse or cohesive sheet-like proliferation of large-sized lymphoid cells with rich slightly eosinophilic cytoplasm and irregular-shaped large nuclei. These lymphoid cells were positive for CD30, CD4, and cytotoxic markers, but negative for CD3 and ALK. Therefore, a diagnosis of ALK-negative ALCL was made. It has been suggested that the incidence of malignant lymphoma may be high in patients with IgG4-related sclerosing disease, therefore, intense medical follow-up is important in patients with this disorder.
Ishida, Mitsuaki; Hodohara, Keiko; Yoshida, Keiko; Kagotani, Akiko; Iwai, Muneo; Yoshii, Miyuki; Okuno, Hiroko; Horinouchi, Akiko; Nakanishi, Ryota; Harada, Ayumi; Yoshida, Takashi; Okabe, Hidetoshi
Ocular myositis frequently manifests with orbital pain and diplopia. The diagnosis of ocular myositis falls within the overall classification of idiopathic orbital inflammatory diseases, defined as non-infective non-specific orbital inflammation without identifiable local or systemic causes. Orbital myositis may form part of more widespread systemic inflammatory processes such as Crohn's disease and the more recently described IgG4-related disease. There is also a broad range of ophthalmic differential diagnoses. Diagnosis, assessment and management of ocular myositis requires the cooperation of ophthalmologists and rheumatologists/immunologists in order to achieve the best patient outcomes. The current literature and avenues of future research are reviewed. PMID:23093472
Fraser, Clare L; Skalicky, Simon E; Gurbaxani, Avinash; McCluskey, Peter
We report two cases of Japanese men who presented with proteinuria, eosinophilia, hypocomplementemia, and high serum immunoglobulin G4 (IgG4) concentration and were diagnosed with membranous nephropathy associated with IgG4-related tubulointerstitial nephritis on renal biopsy. The typical renal lesions of IgG4-related disease are tubulointerstitial nephritis, which improves remarkably with steroid therapy, and occasional glomerular changes. In our two cases, renal biopsy revealed IgG4-positive immune complex deposits in glomeruli in a pattern of membranous nephropathy and concurrent tubulointerstitial nephritis with IgG4 plasma cells. In both cases, proteinuria persisted with initial prednisolone treatment and was resolved only after the addition of mizoribine. We report the first two cases in which the combination of prednisolone and mizoribine was effective for treating membranous nephropathy associated with IgG4-related tubulointerstitial nephritis.
Kihira, Hiromi; Haneda, Manabu; Nishio, Yasuhide
We herein report a case of IgG4-related autoimmune pancreatitis (AIP). A 72-year-old male with jaundice visited our hospital complaining of epigastralgia. A blood chemistry analysis revealed elevated serum levels of total bilirubin and DUPAN-II. Computed tomography (CT) revealed irregularly shaped pancreatic masses with a stricture of the main pancreatic duct (MPD) in the head and tail that were interposed by marked atrophy with MPD dilation in the body. F-18 fluorodeoxyglucose (FDG)-positron emission tomography/CT revealed abnormally intense FDG uptake only at the masses. During surgery, another small tumor was also found in the atrophied body; therefore, a total pancreatectomy was performed under the diagnosis of multiple pancreatic cancers. The histological analysis revealed fibrosis with dense and diffuse infiltrations of lymphocytes and IgG4-positive plasma cells. The pancreatic parenchyma of the body was firmly replaced by fibrosis. AIP can lead to the formation of multiple pancreatic lesions, and thus the correct diagnosis is occasionally difficult to establish in atypical cases. PMID:23549930
Kimura, Akifumi; Yamamoto, Junji; Hatsuse, Kazuo; Aosasa, Suefumi; Nishiyama, Kiyoshi; Maejima, Tadashi; Ogata, Sho; Shimazaki, Hideyuki; Kawaguchi, Atsushi; Hase, Kazuo
Although considered essential for diagnosing IgG4-related disease (IgG4-RD), biopsy of target organs is often difficult to perform. Such was the case of a 56-year-old man admitted with general malaise and weight loss. Computed tomography revealed swelling of the submandibular gland, mild dilatation of the main pancreatic duct, renal involvement, periaortitis, and swelling of the lymph nodes in the abdominal cavity. Laboratory testing revealed elevated serum IgG4 level. These findings were suggestive of IgG4-RD; however, the patient refused consent for biopsy of the target organs for a definitive diagnosis for the invasiveness. Therefore, we tried to perform a biopsy from minor salivary gland, which revealed no sign of clinical abnormality because the biopsy is not an invasive diagnostic procedure. As a result, the biopsy revealed significant IgG4-positive plasma cell infiltration, allowing for definitive IgG4-RD diagnosis. Administration of oral prednisolone (30 mg/day) effectively improved all symptoms. These findings indicate that minor salivary gland biopsy is an effective means of IgG4-RD diagnosis in patients for whom biopsy of target organs is difficult even if there were no sign of clinical abnormality in appearance. PMID:24966985
Doe, Kentaro; Nozawa, Kazuhisa; Okada, Takashi; Tada, Kurisu; Yamaji, Ken; Tamura, Naoto; Takasaki, Yoshinari
Although considered essential for diagnosing IgG4-related disease (IgG4-RD), biopsy of target organs is often difficult to perform. Such was the case of a 56-year-old man admitted with general malaise and weight loss. Computed tomography revealed swelling of the submandibular gland, mild dilatation of the main pancreatic duct, renal involvement, periaortitis, and swelling of the lymph nodes in the abdominal cavity. Laboratory testing revealed elevated serum IgG4 level. These findings were suggestive of IgG4-RD; however, the patient refused consent for biopsy of the target organs for a definitive diagnosis for the invasiveness. Therefore, we tried to perform a biopsy from minor salivary gland, which revealed no sign of clinical abnormality because the biopsy is not an invasive diagnostic procedure. As a result, the biopsy revealed significant IgG4-positive plasma cell infiltration, allowing for definitive IgG4-RD diagnosis. Administration of oral prednisolone (30 mg/day) effectively improved all symptoms. These findings indicate that minor salivary gland biopsy is an effective means of IgG4-RD diagnosis in patients for whom biopsy of target organs is difficult even if there were no sign of clinical abnormality in appearance.
Doe, Kentaro; Nozawa, Kazuhisa; Okada, Takashi; Tada, Kurisu; Yamaji, Ken; Tamura, Naoto; Takasaki, Yoshinari
Hypocomplementaemia is frequently observed in IgG4-related diseases, however the clinical significance is unclear. We describe herein the clinical courses of 4 patients with IgG4-related disease with hypocomplementaemia. Our cases showed autoimmune pancreatitis, retroperitoneal fibrosis, Mikulicz's disease, interstitial lung disease, lymphadenopathy and mesenteric fibrosis around the aorta. A decrease in serum complement preceded deterioration of the disease and clinical improvement was observed in accordance with normalisation of serum complement. These clinical courses suggest that serum complement is a biomarker of the disease activity. PMID:24021494
Kihara, Mari; Sugihara, Takahiko; Hosoya, Tadashi; Miyasaka, Nobuyuki
Immunoglobulin G4-related systemic disease (IgG4-RSD) is a recently defined emerging entity characterized by a diffuse or mass forming inflammatory reaction rich in IgG4-positive plasma cells associated with fibrosclerosis and obliterative phlebitis. IgG4-RSD usually affects middle aged and elderly patients, with a male predominance. It is associated with an elevated serum titer of IgG4, which acts as a marker for this recently characterized entity. The prototype is IgG4-related sclerosing pancreatitis or autoimmune pancreatitis (AIP). Other common sites of involvement are the hepatobiliary tract, salivary gland, orbit, and lymph node, however practically any organ can be involved, including upper aerodigestive tract, lung, aorta, mediastinum, retroperitoneum, soft tissue, skin, central nervous system, breast, kidney, and prostate. Fever or constitutional symptoms usually do not comprise part of the clinical picture. Laboratory findings detected include raised serum globulin, IgG and IgG4. An association with autoantibody detection (such as antinuclear antibodies and rheumatoid factor) is seen in some cases. Steroid therapy comprises the mainstay of treatment. Disease progression with involvement of multiple organ-sites may be encountered in a subset of cases and may follow a relapsing-remitting course. The principal histopathologic findings in several extranodal sites include lymphoplasmacytic infiltration, lymphoid follicle formation, sclerosis and obliterative phlebitis, along with atrophy and destruction of tissues. Immunohistochemical staining shows increased IgG4+ cells in the involved tissues (>50 per high-power field, with IgG4/IgG ratio >40%). IgG4-RSD may potentially be rarely associated with the development of lymphoma and carcinoma. However, the nature and pathogenesis of IgG4-RSD are yet to be fully elucidated and provide immense scope for further studies.
Divatia, Mukul; Kim, Sun A
Immunoglobulin G4-related systemic disease (IgG4-RSD) is a recently defined emerging entity characterized by a diffuse or mass forming inflammatory reaction rich in IgG4-positive plasma cells associated with fibrosclerosis and obliterative phlebitis. IgG4-RSD usually affects middle aged and elderly patients, with a male predominance. It is associated with an elevated serum titer of IgG4, which acts as a marker for this recently characterized entity. The prototype is IgG4-related sclerosing pancreatitis or autoimmune pancreatitis (AIP). Other common sites of involvement are the hepatobiliary tract, salivary gland, orbit, and lymph node, however practically any organ can be involved, including upper aerodigestive tract, lung, aorta, mediastinum, retroperitoneum, soft tissue, skin, central nervous system, breast, kidney, and prostate. Fever or constitutional symptoms usually do not comprise part of the clinical picture. Laboratory findings detected include raised serum globulin, IgG and IgG4. An association with autoantibody detection (such as antinuclear antibodies and rheumatoid factor) is seen in some cases. Steroid therapy comprises the mainstay of treatment. Disease progression with involvement of multiple organ-sites may be encountered in a subset of cases and may follow a relapsing-remitting course. The principal histopathologic findings in several extranodal sites include lymphoplasmacytic infiltration, lymphoid follicle formation, sclerosis and obliterative phlebitis, along with atrophy and destruction of tissues. Immunohistochemical staining shows increased IgG4+ cells in the involved tissues (>50 per high-power field, with IgG4/IgG ratio >40%). IgG4-RSD may potentially be rarely associated with the development of lymphoma and carcinoma. However, the nature and pathogenesis of IgG4-RSD are yet to be fully elucidated and provide immense scope for further studies. PMID:22187229
Divatia, Mukul; Kim, Sun A; Ro, Jae Y
IgG4-related systemic disease (IgG4-RD) is an inflammatory condition of unknown etiology that has been identified as the cause of tumefactive lesions in a number of tissues and organs. The role of the IgG4 in the disease remains to be clarified fully, but the histopathologic diagnosis hinges upon the finding of IgG4-bearing plasma cells in addition to characteristic morphologic features, with
Zachary S. Wallace; Arezou Khosroshahi; Frederick A. Jakobiec; Vikram Deshpande; Mark P. Hatton; Jill Ritter; Judith A. Ferry; John H. Stone
We report the histopathological features of vertebral basilar system dolichoectasia (VBD) in a 68-year-old man who died as a result of accompanying infarction of the medulla oblongata on day 6 of admission. During hospitalization, the patient was also found to have an elevated serum IgG level and tumors of the renal pelvis. A possible clinical diagnosis of VBD associated with IgG4-related disease was considered postmortem. Autopsy examination, limited to the intracranial tissues, revealed marked infiltration of IgG4-containing plasma cells in the adventitia and media of the vertebral and basilar arteries. Multiple fibrous nodules forming pseudotumors were also evident on the outer surface of the affected arteries. These histological features were very similar to those of arteriopathy, such as inflammatory aortic aneurysm, which has been described in patients with IgG4-related disease, suggesting that autoimmune mechanisms, known to be involved in the pathogenesis of visceral lesions in the disease, also played a role in the etiology of VBD in the present patient. In conclusion, we consider that the present case may represent VBD as a manifestation of IgG4-related disease. PMID:21615518
Toyoshima, Yasuko; Emura, Iwao; Umeda, Yoshitake; Fujita, Nobuya; Kakita, Akiyoshi; Takahashi, Hitoshi
Clear cell sarcoma (CCS) is a distinctive soft tissue sarcoma that shows melanocytic differentiation. Primary gastrointestinal (GI) CCSs have been rarely reported, but to our knowledge, no association between GI CCSs and immunoglobulin G4 (IgG4)-related sclerosing disease has been described in the literature. We experienced 2 cases of CCS that arose in the small intestine and metastasized to the liver. Histologic features and immunophenotype were typical of CCS. One of them showed a unique peritumoral sclerosing inflammatory reaction, which was highly reminiscent of IgG4-related sclerosing inflammatory disease. Dense lymphoplasmacytic infiltration with extensive sclerosis and obliterative phlebitis was observed in the immediate vicinity of the primary and metastatic tumors, but not in the distant areas from the tumor. The average number of IgG4-positive plasma cells was more than 50 per high-power field. We report 2 cases of primary GI CCS with one case showing a unique peritumoral IgG4-related lymphoplasmacytic sclerosing inflammation. PMID:19118779
Joo, Mee; Chang, Sun Hee; Kim, Hanseong; Gardner, Jerad M; Ro, Jae Yun
IgG4-related disease is a newly recognized systemic condition characterized by high serum IgG4 levels and an inflammatory reaction rich in IgG4-positive plasma cells associated with fibrosis and obliterative phlebitis. It predominantly affects middle-aged or elderly males and was first described as autoimmune pancreatitis. The disease may also involve the hepatobiliary tract, salivary glands, orbit, lymph nodes, and the lungs, aorta, retroperitoneum, skin, nervous system, kidneys and thyroid gland. It may mimic infections, malignancies, and other immune-mediated conditions. Histopathology and immunohistochemistry form the basis of the diagnosis. Glucocorticoids are usually effective but relapses occur commonly. PMID:24660380
We report a case of Mikulicz's disease, IgG4-related plasmacytic syndrome, successfully treated with corticosteroid and mizoribine, followed by mizoribine alone. A 56-year-old woman experienced persistent bilateral swelling in the eyelids and submandibular region accompanied by lymphadenopathy without pain. The patient was diagnosed as having Mikulicz's disease since her bilateral lacrimal glands showed persistent swelling and biopsy specimens of the submandibular glands and lymph node showed diffuse infiltration of lymphocytes and plasma cells. Symptoms and laboratory data were improved with corticosteroids (30 mg). However, after tapering prednisolone to 5 mg, submandibular swelling developed. Adding mizoribine resulted in clinical improvement. Prednisolone was tapered and finally discontinued. Since the levels of IgG4 in peripheral blood were increased even after corticosteroid therapy and IgG4 was positive in a submandibular gland specimen obtained before the start of corticoid therapy. Thus, this case may be associated with IgG4. The present case is the first case in which mizoribine was effective for Mikulicz's disease and IgG4-related plasmacytic syndrome. PMID:20647666
Nanke, Yuki; Kobashigawa, Tsuyoshi; Yago, Toru; Kamatani, Naoyuki; Kotake, Shigeru
A 51-year-old woman received an ABO blood type-incompatible renal transplant. She was administered rituximab and basiliximab and underwent plasma exchanges for induction therapy, followed by administration of tacrolimus, mycophenolate mofetil and methylprednisolone as maintenance immunosupression therapy. A planned renal biopsy 2 years after transplantation revealed infiltration of plasma cells in the renal interstitium, although there was no 'storiform' fibrosis surrounding these cells. There were also no findings of rejection, BK virus nephropathy, or atypical plasma cells. Immunohistochemical stainings showed a large number of IgG4-positive plasma cells, most of which expressed kappa-type light chains. A CT scan showed a mass at the renal hilum. The serum IgG4 level was high. Based on these findings, the patient was suspected of having IgG4-related kidney disease. Nine months after the biopsy, her serum creatinine level increase to 1.56?mg/dL and the dose of methylprednisolone was therefore increased to 16?mg/day. Three months after this increase in steroid, a CT scan showed the hilum mass had disappeared. A follow-up biopsy 5 months later showed that infiltration of plasma cells in the renal interstitium had decreased markedly, although focal and segmental severely fibrotic lesions with IgG4-positive plasma cells were observed. Serum IgG4 levels decreased immediately after the increase in steroid dose and remained <100?mg/dL despite a reduction in methylprednisolone to 6?mg/day. Serum creatinine levels also remained stable at around 1.6?mg/dL. To our knowledge, this is the first report of IgG4-positive plasma cell-rich tubulointerstitial nephritis mimicking IgG4-related kidney disease after kidney transplantation. PMID:24842825
Nishikawa, Kouhei; Takeda, Asami; Masui, Satoru; Kanda, Hideki; Yamada, Yasushi; Arima, Kiminobu; Morozumi, Kunio; Sugimura, Yoshiki
Since first described in the mid 1990s, there has been burgeoning literature on IgG4-related sclerosing disease. The number of sites that may be involved is ever increasing, with the pancreas, salivary glands, and lymph nodes being the most commonly affected organs. There are no well-documented cases arising in the gastrointestinal tract. In this report, we present the first case to our knowledge of IgG4-related sclerosing disease involving the small bowel with a distinctly unusual clinicopathologic presentation. A previously well 46-year-old woman presented with a 2-year history of intermittent abdominal pain with recent worsening due to small bowel obstruction. Following imaging, which showed jejunitis with surrounding mesenteric inflammatory changes, she proceeded to a segmental small bowel resection. The resected jejunum revealed an isolated, stenosing chronic ulcer associated with a necrotizing mesenteric arteritis. A transmural inflammatory infiltrate rich in IgG4 plasma cells was seen in the wall of the bowel and mesenteric artery. Abundant IgG4 interfollicular plasma cells were also identified in a mesenteric lymph node. The serum IgG4 level was elevated at >800 mg/dL (reference range 8 to 140 mg/dL). Although phlebitis is an almost constant feature of this disease, arteritis is not described other than in the lung and aorta. In this report, we also discuss the diagnostic pitfalls and the differential diagnoses that should be considered when this condition arises in the gastrointestinal tract. PMID:22367294
Wong, Daniel D; Pillai, Sooraj R; Kumarasinghe, Marian Priyanthi; McGettigan, Ben; Thin, Lena W Y; Segarajasingam, Dev S; Hollingsworth, Peter N; Spagnolo, Dominic V
Cutaneous plasmacytosis is a rare condition affecting middle-aged individuals, characterized by multiple red-brown papules and plaques over the trunk. It has been reported mainly in Japan. The condition is accompanied by polyclonal hypergammaglobulinemia and superficial lymphadenopathy. Lung or retroperitoneal involvement occurs rarely. In the present study, 3 consecutive cases of cutaneous plasmacytosis were observed histologically to have abundant infiltration of IgG4-bearing plasma cells. All 3 were associated with superficial lymphadenopathy, one with interstitial lung involvement showing ground-glass opacity on computed tomography and the others with bone marrow plasmacytosis, showing histologic evidence of more IgG4-positive plasma cells. All 3 had polyclonal hypergammaglobulinemia, one had high serum concentration of IgG4, and all had elevated serum IL-6. The ratios of IgG4+ to IgG+ plasma cells were assessed using skin biopsy specimens with pemphigus (n = 7), discoid lupus erythematosus (n = 5), and morphea (n = 2) (mean ratios, 19%, 0%, and 0%, respectively); we noted the proportion of IgG4-positive plasma cells in cutaneous plasmacytosis (mean, 48%). IgG4-related sclerosing disease is a newly recognized systemic disorder characterized by lymphoplasmacytic infiltration and fibrosis and by a high serum IgG4 level and increased IgG4-positive plasma cells in the tissues. Skin manifestations of this disorder have not been described. Although cutaneous plasmacytosis could be a chronic allergic hypersensitivity reaction, our findings raise the possibility of a relationship in pathogenesis between cutaneous plasmacytosis and IgG4-related sclerosing disease. PMID:19386351
Miyagawa-Hayashino, Aya; Matsumura, Yumi; Kawakami, Fumi; Asada, Hideo; Tanioka, Miki; Yoshizawa, Akihiko; Mikami, Yoshiki; Kotani, Hirokazu; Nakashima, Yasuaki; Miyachi, Yoshiki; Manabe, Toshiaki
Malignant cutaneous adnexal neoplasms are one of the most challenging areas of dermatopathology. Tumors of the pilosebaceous apparatus can occur as single-lineage neoplasms or may manifest as complex proliferations with multilineal differentiation patterns including not only the germinative component of the hair bulb, the inner or outer root sheath epithelium and the sebaceous gland and duct, but also the sweat
A Neil Crowson; Cynthia M Magro; Martin C Mihm
IgG4-related systemic disease (IgG4-RD) is an inflammatory condition of unknown etiology that has been identified as the cause of tumefactive lesions in a number of tissues and organs. The role of the IgG4 remains to be clarified fully, but the histopathologic diagnosis hinges upon the finding of IgG4-bearing plasma cells in addition to characteristic morphologic features, with or without elevated seum IgG4. We present a 56-year-old man with orbital pseudotumor in whom, after 30 years of intractable disease, biopsy showed IgG4-RD involving the lacrimal gland, extraocular muscles, intraconal fat, and trigeminal nerve. Six months after initiating treatment with rituximab, his disease remained dormant, with improvement in his proptosis and normalization of serum IgG4 levels. We review the differential of idiopathic orbital inflammatory disease, including IgG4-RD, and emphasize the need for biopsy for accurate diagnosis and to guide appropriate treatment. PMID:22018678
Wallace, Zachary S; Khosroshahi, Arezou; Jakobiec, Frederick A; Deshpande, Vikram; Hatton, Mark P; Ritter, Jill; Ferry, Judith A; Stone, John H
IgG4-related disease (IgG4-RD) is characterized by a lymphoplasmacytic infiltrate composed of IgG4(+) plasma cells, tumefactive lesions, obliterative phlebitis, and mild to moderate eosinophilia. It has been suggested that IgG4-RD is characterized by allergic manifestations and is potentially driven by enhanced T-helper type 2 (Th2) responses. We aimed to investigate the potential contribution of atopy to enhanced Th2 responses in IgG4-RD. Peripheral blood mononuclear cells from 39 patients were isolated and subjected to in vitro mitogenic stimulation with PMA and ionomycin. Following stimulation, gated CD3(+) CD4(+) T cells were analyzed for production of the Th2 cytokines IL-4, IL-5, and IL-13. Among the 39 patients analyzed, only the 18 patients who had a history of atopy showed increases in circulating Th2 memory cells. Our results indicate that Th2 responses that have been reported in IgG4-RD may result from concomitant atopic manifestations in disease subjects. PMID:24382311
Mattoo, H; Della-Torre, E; Mahajan, V S; Stone, J H; Pillai, S
This book contains 20 chapters. Some of the chapter titles are: The Ruptured Globe: Primary Care; Corneal Trauma, Endophthalmitis; Antibiotic Usage; Radiology of Orbital Trauma; Maxillofacial Fractures; Orbital Infections; and Basic Management of Soft Tissue Injury.
Spoor, T.C.; Nesi, F.A.
Introduction IgG4-related disease (IgG4-RD) is a multisystem-involved autoimmune disease. Abnormally activated and differentiated B cells may play important roles. Regulatory B cells (Breg) are newly defined B cell subgroups with immunosuppressive functions. In this study, we investigated the differences of B cell subsets, the expressions of co-stimulatory molecules on B cells, and the function of Breg cells in patients with IgG4-RD, primary Sjögren’s syndrome (pSS) as well as in healthy controls (HC). Methods Newly diagnosed IgG4-RD patients (n?=?48) were enrolled, 38 untreated pSS patients and 30 healthy volunteers were recruited as disease and healthy controls. To analyze B cell subsets and B cell activity, PBMCs were surface stained and detected by flow cytometry. The function of Breg cells was tested by coculturing isolated CD19?+?CD24hiCD38hi Breg cells with purified CD4?+?CD25- T cells. Serum cytokines were measured by ELISA and cytometric bead array. Relationship between clinical data and laboratory findings were analyzed as well. Results Compared with pSS patients and HC, IgG4-RD patients had a lower frequency of peripheral Breg cells. Interestingly, CD19?+?CD24-CD38hi B cell subsets were significantly higher in peripheral B cells from IgG4-RD patients than in pSS patients and HC, which correlated with serum IgG4 levels. The expression of BAFF-R and CD40 on B cells was significantly lower in IgG4-RD patients compared with those in pSS patients and HC. Unlike HC, Breg cells from pSS patients lacked suppressive functions. Conclusions B cells in patients with IgG4-RD and pSS display a variety of abnormalities, including disturbed B cell subpopulations, abnormal expression of key signaling molecules, co-stimulatory molecules, and inflammatory cytokines. In addition, a significantly increased B cell subset, CD19?+?CD24-CD38hi B cells, may play an important role in the pathogenesis of IgG4-RD.
Adnexal skin tumors are rare and mostly not common. Their appearance are atypical and only histologically to save. 2 cases were demonstrated with impressing extension demanding a complex surgical approach. PMID:23129411
Halbach, E; La Forza, F; Schepler, H
The presence of adnexal masses during pregnancy is a rare situation but due to a broader use of sonography; its occurrence is increasing. Their management essentially relies on imagery. We report the case of an 18 weeks-pregnant women presenting bilateral teratomas visualized by RMI. She was successfully treated by laparoscopy. This paper approaches the management of adnexal masses during pregnancy. The epidemiology, possible complications, diagnostic approach and the therapeutic management are discussed. PMID:24908949
Liefferinckx, C; Hottat, N; Simon, P
Background Orbital tuberculosis (OTb) is rare and may be regarded as a manifestation of extrapulmonary tuberculosis. We report an interesting case series of six patients with varied presentations of orbital and adnexal tuberculosis in a South Indian patient population. Results A retrospective, interventional case series of six patients diagnosed with orbital and adnexal tuberculosis on the basis of clinical, radiological and histopathological evaluations between 2010 and 2013 was performed. Among the six patients with histopathologically proven OTb, five were women. The varied presentations included tubercular dacryoadenitis (two cases), classical periostitis (two cases), OTb with bone involvement (one case) and ocular adnexal tuberculosis (one case). Systemic involvement was seen in one case. All cases were treated with a regimen of antitubercular therapy (ATT). Conclusions OTb, though rare, should form a part of the differential diagnosis of orbital lesions in a high tuberculosis (TB) endemic country like ours. Biopsy still remains the mainstay of diagnosis.
Background and Objective: Although laparoscopic surgery for removal of adnexal masses is common, controversy exists about the safety and efficacy of this procedure for patients with malignancies. The aim of this study was to evaluate the effectiveness and safety of laparoscopic surgical treatment for patients with adnexal masses. Methods: This was a retrospective chart review of one surgeon's experience in managing patients diagnosed with adnexal masses at 2 urban referral teaching hospitals in New York City. We reviewed the charts for 100 consecutive patients who underwent operative laparoscopy for management of adnexal masses between March 4, 1996 and November 9, 1998. Conversion to laparotomy, malignancy rate, complications, length of stay, and blood loss were recorded for each patient. Results: Laparoscopic management was successfully completed for 81 of the 100 patients in this study; however, 19 required conversion to laparotomy. All 81 patients managed laparoscopically had a benign diagnosis, whereas 7 of the 19 patients who underwent laparotomy were diagnosed with malignancy. The median length of stay, estimated blood loss, and operating room time were significantly lower for those treated by laparoscopy alone compared with those converted to laparotomy (2 vs. 7 days; 100 vs. 500 ccs; 130 vs. 235 minutes, respectively; P < 0.05). Though few patients were in the laparotomy group, that data are presented for completeness. A total of 10 complications occurred, 4 in the group of patients managed laparoscopically (2 enterotomies, 1 pneumothorax, and 1 vaginal cuff cellulitis). Six complications occurred in those managed with laparotomy (2 enterotomies, 2 wound infections, 1 pneumonia, and 1 postoperative fever). The indications for conversion to laparotomy were: 7 malignancies (5 ovarian cancers and 2 uterine cancers), 7 dense adhesions, 2 small bowel enterotomies, 1 intraoperative bleeding, 1 secondary to a large uterus (880 grams), and 1 secondary to a large myoma (13 cm x 14.5 cm x 6 cm). Conclusions: The laparoscopic approach is effective and safe for managing patients with adnexal masses of unknown pathology. Malignancies can be diagnosed accurately, converted to laparotomy, and staged appropriately. Adequate surgical skills along with timely use of frozen sections are required for successful operative management.
Emeney, Pamela L.; Byrne, Daniel W.
Adnexal masses are common in women of all ages. A range of physiological and benign ovarian conditions that develop in women, especially in the reproductive age, and adnexal malignancies can be evaluated with magnetic resonance imaging (MRI). Management of women with adnexal masses is frequently guided by imaging findings; therefore, precise characterization of adnexal pathology should be performed whenever possible. Magnetic resonance imaging is useful in characterization of adnexal masses that are not completely evaluated by ultrasound because it can provide additional information on soft tissue composition of adnexal masses based on specific tissue relaxation times and allows multiplanar imaging at large field of view to define the origin and extent of pelvic pathology. The patients most likely to benefit from MRI are pregnant women and those who are premenopausal and have masses that have complex features on ultrasound but do not have raised cancer antigen 125 tumor marker levels. The overlap in imaging appearance among different cell type malignancies makes it difficult to predict exact histology based on MRI appearance; however, MRI has a high accuracy in differentiating benign from malignant masses. Teratomas, endometriomas, simple and hemorrhagic cysts, fibromas, exophytic or extrauterine fibroids, and hydrosalpinges can be diagnosed with high confidence. In this article, the authors review the histopathologic background and MRI features of adnexal masses and discuss the role of MRI in the differentiation of benign from malignant adnexal pathologies. PMID:17417086
Rajkotia, Kavita; Veeramani, Murugusundaram; Macura, Katarzyna J
We assessed 6 cases acquired during routine surgical sign-out for IgG4-related disease (IRD) according to criteria from a recent consensus meeting. These cases fulfilled the morphologic criteria-that is, dense lymphoplasmacytic infiltrates, IgG4:IgG ratio greater than or equal to 0.4, and fibrosis (storiform in 4 cases-but were associated with malignancy or did not fulfill the criteria for a new site. These criteria include increased serum IgG4 (normal in the majority of IRD) and a response to glucocorticoids, which is not appropriate treatment for resectable lesions as in our cases. Until more is known about the natural history of the disease, we propose that the possibility of an early, localized, or forme fruste of IRD should be considered and that cases associated with malignancy should at least be documented. Although we acknowledge the value of the consensus criteria, their strict application may result in missed opportunities to study the disease. PMID:24344155
Lee, Lik Hang; Shaffer, Eldon A; Falck, Vincent; Kelly, Margaret M
IMPORTANCE To report the clinical and histopathologic findings of ocular adnexal angiolymphoid hyperplasia with eosinophilia, an unusual but often misdiagnosed benign disorder. OBSERVATIONS The ophthalmologic findings of angiolymphoid hyperplasia with eosinophilia with ocular adnexal involvement are variable and include eyelid swelling, ptosis, proptosis, and loss of vision. Imaging studies typically reveal a well-circumscribed mass in the orbit. The condition may resemble other diseases that involve the orbit and ocular adnexal tissue, such as lymphoma, hemangioma, sarcoidosis, and dermoid cyst. Histopathologic analysis reveals marked vascular proliferation with an accompanying inflammation composed of numerous eosinophils, lymphocytes, and plasma cells. CONCLUSIONS AND RELEVANCE Angiolymphoid hyperplasia with eosinophilia is a rare disease that can affect the ocular adnexal tissue. The clinical presentation is often nonspecific; therefore, histopathologic studies are essential for diagnosis and subsequent management of this benign condition. PMID:24676051
Azari, Amir A; Kanavi, Mozhgan R; Lucarelli, Mark; Lee, Vivian; Lundin, Ashley M; Potter, Heather D; Albert, Daniel M
Infections of the eye can rapidly damage important functional structures and lead to permanent vision loss or blindness. Broad-spectrum antibiotics should be administered to the appropriate site of infection as soon as a diagnosis is made. Topical drops are preferred for corneal and conjunctival infections. Intravitreal antibiotics, and possibly subconjunctival and parenteral antibiotics, are preferred for endophthalmitis. Parenteral antibiotics are recommended for infection in deep adnexal structures. We review specific aspects of antibiotic therapy for ocular and periocular infection.
Snyder, R W; Glasser, D B
This review discusses in detail robotic tubal reanastomosis as one of the classic reproductive surgery procedures. Other applications of robotics in adnexal surgery are also reviewed, including adult and pediatric adnexectomy, resection of endometriosis, benign ovarian mass resection, early ovarian cancer resection and staging, ovarian transposition, and treatment of ovarian remnant syndrome and ovarian vein syndrome.
Tusheva, Olga A; Gargiulo, Antonio R; Einarsson, Jon I
Adnexal torsion is one of a few gynecologic surgical emergencies. Misdiagnosis or delay in treatment can have permanent sequelae including loss of an ovary with effect on future fertility, peritonitis, and even death. A PubMed search was performed between 1985 and 2012 for reviews, comparative studies, and case reports to provide a review of the epidemiology, risk factors, clinical presentation, common laboratory and imaging findings, and treatments of adnexal torsion. Common symptoms of torsion include pain, nausea, and vomiting, with associated abdominal or pelvic tenderness, and may differ in premenarchal and pregnant patients. Laboratory and imaging findings including ultrasound with Doppler analysis, computed tomography, and magnetic resonance imaging can assist in making the diagnosis but should not trump clinical judgment; normal Doppler flow can be observed in up to 60% of adnexal torsion cases. Treatment depends on the individual patient but commonly includes detorsion, even if the adnexae initially seem necrotic, with removal of any associated cysts or salpingo-oophorectomy, because recurrence rates are higher with detorsion alone or detorsion with only cyst aspiration. PMID:24126258
Sasaki, Kirsten J; Miller, Charles E
OBJECTIVE: Our purpose was to evaluate the pathologic features and outcome of pregnancy complicated by a persistent adnexal mass that was managed conservatively or with surgical intervention.STUDY DESIGN: A review was performed of patients who were seen with an adnexal mass in pregnancy from January 1988 to June 1994. We included patients with simple or complex masses >-6 cm that
Deborah N. Platek; Cassandra E. Henderson; Gary L. Goldberg
Background: Paraneoplastic syndromes are a heterogeneous collection of disease manifestations caused by underlying neoplasms, which can include adnexal masses. Case: We report a case of paraneoplastic limbic encephalitis consisting of confusion, agitation, and aggression, which subsequently progressed to a comatose state, in the presence of an adnexal mass. It was cured with resection of a mature teratoma with focal immature components. Conclusion: Female patients who present with symptoms of limbic encephalitis should be thoroughly screened for malignancy. Gynecologic consultation is an important component of the diagnostic workup when an adnexal mass has been identified. Paraneoplastic limbic encephalitis is a serious condition, yet it has a good prognosis if treated promptly with surgical resection of the adnexal mass.
Tillman, Julie A.
Objective To study the clinical and histological nature of benign adnexal masses managed surgically. Methods A retrospective descriptive study in a teaching hospital in Oman of all the women who had surgical management of benign adnexal masses from January 2008 to May 2012. Data pertaining to age, parity, presenting symptoms, imaging and tumor markers performed and the surgical intervention done on those women with benign adnexal masses was collected from the electronic health records of the patients. Results There were 198 women during this period operated for benign adnexal masses. The most common benign neoplasm was mature teratoma of the ovary followed by endometriosis. Conservative surgery in the form of ovarian cystectomy was necessary in three fourths of women and in about just less than 50% of women, the procedure was completed laparoscopically. Conclusion The most common benign tumor was teratoma but laparoscopic approach, which is the standard of care in these women, was possible only in just about 50% of the women.
Gowri, Vaidyanathan; Al Shukri, Maryam; Al Khaduri, Maha; Machado, Lovina
Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory condition associated with elevated serum IgG4 levels and tissue infiltration by IgG4-expressing plasma cells. Several inflammatory conditions associated with IgG4-RD have been reported. Warthin's tumor is a benign parotid gland tumor consisting of oncocytic epithelial cells and lymphoid stroma containing lymphoid follicles with reactive germinal centers. This is the first report describing a case of Warthin's tumor with possible involvement of IgG4-RD. The patient was a 71-year-old man presenting with swollen right parotid and bilateral submandibular glands. He had a history of a Warthin's tumor of the left parotid gland, autoimmune pancreatitis, and an inflammatory abdominal aortic aneurysm. Laboratory tests revealed a high serum IgG4 level. Histological examination of the resected parotid gland showed a Warthin's tumor and a nodule showing severe lymphocytic infiltration containing many IgG4-positive plasma cells. This case shows the possible involvement of Warthin's tumor with IgG4-RD. PMID:23246115
Aga, Mitsuharu; Kondo, Satoru; Yamada, Kazunori; Sawada-Kitamura, Seiko; Yagi-Nakanishi, Sayaka; Endo, Kazuhira; Wakisaka, Naohiro; Murono, Shigeyuki; Kawano, Mitsuhiro; Yoshizaki, Tomokazu
Background: This study evaluated the safety of laparoscopic management for persistent adnexal mass in the second trimester of pregnancy. Methods: Between April 1994 and March 2003, 67 consecutive women underwent laparoscopic removal of adnexal masses that had persisted into the second trimester of pregnancy in an academic tertiary referral center.Operative complications, pregnancy, and labor outcomes were evaluated. Results: The median
P. M. Yuen; P. S. Ng; P. L. Leung; M. S. Rogers
In clinical routine adnexal torsion tends to be subject to adnexectomy to prevent embolism of thrombosed ovarian veins and its sequelae. This therapeutic intervention can be derogatory especially for young women during the reproductive years. As seen in animal models with adnexal torsion, restitution of the adnexa next to reperfusion is possible until 36h after interruption of venous and arterial
A. Rody; C. Jackisch; W. Klockenbusch; J. Heinig; V. Coenen-Worch; H. P. G. Schneider
Microcystic adnexal carcinoma (MAC) is a relatively uncommon adnexal neoplasm that can display aggressive local invasion. MAC occurs most commonly on the central part of the face and can be clinically asymptomatic with a benign appearance. We describe the first reported case of MAC in an African American man who was treated by Mohs micrographic surgery. (J Am Acad Dermatol
Christopher M. Peterson; John L. Ratz; Omar P. Sangueza
Ocular traumas represent a major public health problem with poorly understood ramifications at both the individual and community levels. Any of the ocular structures can be damaged in the case of closed globe injury. These lesions, often multiple, may appear immediately or in a delayed fashion. Classifications have been developed recently in order to better inform the patient of the visual prognosis. However, significant efforts are still needed, on the one hand, to assess and develop new therapies, and on the other hand, to implement effective policies to prevent ocular trauma. PMID:22463853
Syphilis is a sexually transmitted disease caused by Treponema pallidum. Previously known as the "great imitator", this disease can have numerous and complex manifestations. The ophthalmologist should suspect the diagnosis in patients with uveitis or optic neuropathy and high-risk sexual behavior and/or another sexually transmitted disease (such as HIV) or those presenting with posterior placoid chorioretinitis or necrotising retinitis. Ocular involvement in acquired syphilis is rare, tending to occur during the secondary and tertiary stages of the disease. Syphilis may affect all the structures of the eye, but uveitis (accounting for 1-5% of the uveitis in a tertiary referral center) is the most common ocular finding. Granulomatous or non-granulomatous iridocyclitis (71%), panuveitis, posterior uveitis (8%) and keratouveitis (8%) are often described. In the secondary stage, the meninges and the central nervous system can be affected, sometimes with no symptoms, which justifies performing lumbar puncture in patients with uveitis and/or optic neuropathy. The diagnosis of ocular syphilis requires screening with a non-treponemal serology and confirmation with a treponemal-specific test. Parenterally administered penicillin G is considered first-line therapy for all stages of ocular syphilis. Systemic corticosteroids are an appropriate adjunct treatment for posterior uveitis, scleritis and optic neuritis if ocular inflammation is severe. Prolonged follow-up is necessary because of the possibility of relapse of the disease. With proper diagnosis and prompt antibiotic treatment, the majority of cases of ocular syphilis can be cured. PMID:24655791
Chiquet, C; Khayi, H; Puech, C; Tonini, M; Pavese, P; Aptel, F; Romanet, J-P
Sarcoidosis, a chronic multisystem disease, is a common cause of ocular inflammation. Even though clinical features are well-established, diagnosis requires histological confirmation, which remains difficult in patients with uveitis. Thus, the frequency of ocular sarcoidosis is overestimated. A set of criteria has been recently established in order to improve the diagnostic procedure. New imaging tools will enable the ophthalmologist to evaluate the level of ocular inflammation and to monitor its resolution after treatment initiation. Indocyanine green angiography and optical coherence tomography have dramatically improved our understanding of choroidal granulomas and macular edema. Treatment is based on topical and systemic corticosteroids in most of the cases, but immuno-suppressive agents may be necessary. The visual outcome remains favorable but severe complications, including glaucoma, cystoid macular edema and choroidal neovascularization, may need a prompt and aggressive management. PMID:22595776
Bodaghi, Bahram; Touitou, Valérie; Fardeau, Christine; Chapelon, Catherine; LeHoang, Phuc
Objective: Guidelines for referring women with pelvic masses suspicious for ovarian cancer to gynecologic oncologists have been developed by the American College of Obstetrician Gynecologists (ACOG). We set out to evaluate the negative predictive value of these guidelines and to assess a modified algorithm involving minimally invasive surgery in the treatment of women with masses suspected to be benign. Methods: 257 consecutive patients with adnexal masses of 8cm to 13cm on preoperative ultrasound examination meeting Triage Criteria set forth in ACOG Committee Opinion 280. Patients meeting the selection criteria were scheduled for operative laparoscopy, washings, adnexectomy, bagging, and colpotomy. A total of 240 patients successfully completed intended treatment (93.38%), and 234 of these did not require admission (97.5%). There was a low incidence of significant complications: 97.50% of women were successfully treated as outpatients, 97.92% of surgeries lasted <136 minutes, and 97.08% had blood loss <200mL. The negative predictive value of ACOG Committee Opinion 280 Triage Criteria as a deselector for having invasive ovarian malignancy in our population was 95.57% for premenopausal and 90.91% for postmenopausal women. Conclusions: Laparoscopic adnexectomy, bagging, and colpotomy is a desirable goal for patients with ovarian masses in the 8cm to 13cm range meeting selection criteria affording a minimally invasive approach with attendant benefits including outpatient treatment (97.5%), few complications, low likelihood of iatrogenic rupture of the ovarian capsule (1.25%), and low necessity for reoperation after final pathology is evaluated (6.03%). Negative predictive value of ACOG Committee Opinion 280 is confirmed in a community gynecology practice and is recommended to form the basis of a new treatment algorithm for women with adnexal masses.
Marchand, Greg J.
...HEALTH AND HUMAN SERVICES Food and Drug Administration 21 CFR Part 866 [Docket No. FDA-2010-N-0026] Medical Devices; Immunology and Microbiology Devices; Classification of Ovarian Adnexal Mass Assessment Score Test System; Correction AGENCY:...
...866 [Docket No. FDA-2011-N-0026] Medical Devices; Immunology and Microbiology Devices; Classification of Ovarian Adnexal...Drugs, 21 CFR part 866 is amended as follows: PART 866--IMMUNOLOGY AND MICROBIOLOGY DEVICES 0 1. The authority citation...
... Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES IMMUNOLOGY AND MICROBIOLOGY DEVICES Tumor Associated Antigen immunological Test Systems Â§ 866.6050 Ovarian adnexal mass...
Phototherapy can be translated to mean 'light or radiant energy-induced treatment.' Lasers have become the exclusive source of light or radiant energy for all applications of phototherapy. Depending on the wavelength, intensity, and duration of exposure, tissues can either absorb the energy (photocoagulation, thermotherapy, and photodynamic therapy (PDT)) or undergo ionization (photodisruption). For phototherapy to be effective, the energy has to be absorbed by tissues or more specifically by naturally occurring pigment (xanthophyll, haemoglobin, and melanin) within them. In tissues or tumours that lack natural pigment, dyes (verteporphin, Visudyne) with narrow absorption spectrum can be injected intravenously that act as focal absorbent of laser energy after they have preferentially localized within the tumour. Ocular phototherapy has broad applications in treatment of ocular tumours. Laser photocoagulation, thermotherapy, and PDT can be delivered with low rates of complications and with ease in the outpatient setting. Review of the current literature suggests excellent results when these treatments are applied for benign tumours, particularly for vascular tumours such as circumscribed choroidal haemangioma. For primary malignant tumours, such as choroidal melanoma, thermotherapy, and PDT do not offer local tumour control rates that are equivalent or higher than those achieved with plaque or proton radiation therapy. However, for secondary malignant tumours (choroidal metastases), thermotherapy and PDT can be applied as a palliative treatment. Greater experience is necessary to fully comprehend risks, comparative benefits, and complication of ocular phototherapy of ocular tumours. PMID:23238445
Singh, A D
Ocular melanomas comprise uveal and conjunctival sub-types, which are very different from each other. A large majority of uveal melanomas involve the choroid, with less than 10% being confined to the ciliary body and iris. They tend to metastasize haematogenously, almost always involving the liver. Therapeutic methods include various forms of radiotherapy, surgical resection and phototherapy, which are often used in combination. Conjunctival melanomas show many similarities to their cutaneous counterparts, often metastasizing by lymphatic spread. Treatment consists of excision of invasive melanoma with adjunctive radiotherapy and/or cryotherapy and topical chemotherapy for intra-epithelial disease. The management of patients with ocular melanomas demands a good understanding of the pathology of these tumours. Pathological examination of the tumour indicates the prognosis and hence the need for further investigation and treatment. The scope of the pathologist is enhanced thanks to advances in molecular biology.
Damato, Bertil E.; Coupland, Sarah E.
Well over 20 million people in the world are infected with Onchocerca volvulus and it is probable that 200 000-500 000 people are blind as a result of this infection, which is the most important cause of blindness in certain areas of Africa and Latin America. Treatment of the disease is difficult and often produces serious adverse reactions in the patient. Combined use of diethylcarbamazine citrate and suramin is still the most suitable form of treatment. Screening for the early detection of cases at high risk of ocular manifestations must be organized, and their treatment undertaken, if blindness is to be avoided. Prevention of ocular onchocerciasis is feasible, using vector control methods to reduce transmission, but the procedures are costly and may have to be maintained for many years. Research is needed to improve treatment and to find a chemoprophylactic agent or a preventive vaccine.
The eye is probably the most common site for the development of allergic inflammatory disorders, because it has no mechanical barrier to prevent the impact of allergens such as pollen on its surface. Physicians in various specialties and subspecialties who provide some form of primary care frequently encounter various forms of inflammation of the anterior surface of the eye that present as "red eye." However, the eye is rarely the only target for an immediate allergic-type response. Typically, many patients have other combinations of allergic disorders, such as rhinoconjunctivitis, rhinosinusitis, asthma, urticaria, or eczema. Even so, ocular signs and symptoms can frequently be the most prominent features of the entire allergic response for which a patient visits his or her physician. An improved differential diagnosis provides the basis for improved treatment algorithms. Over the past 20 years, we have witnessed an astonishing growth in therapeutic advances, ranging essentially from derivatives of simple aspirin to various newly developed biologic immunomodulatory agents, utilizing implantable drug-delivery devices that exceed the safety and efficacy of those available for other organ systems, and resorting to advanced surgical techniques for the correction of sight-threatening, disease-related complications. Overall, with the expanding knowledge base, the intricacy of ocular inflammation appears to be becoming ever more manageable and, with the team approach between the primary care physician, the ophthalmologist, and the clinical allergist/immunologist, the new "immuno-ophthalmology" approach improves patient outcomes. PMID:21913203
Spectrally resolved white-light interferometry was used to measure the wavelength dependence of refractive index (i.e., dispersion) for various ocular components. The accuracy of the technique was assessed by measurement of fused silica and water, the refractive indices of which have been measured at several different wavelengths. The dispersion of bovine and rabbit aqueous and vitreous humor was measured from 400 to 1100 nm. Also, the dispersion was measured from 400 to 700 nm for aqueous and vitreous humor extracted from goat and rhesus monkey eyes. For the humors, the dispersion did not deviate significantly from water. In an additional experiment, the dispersion of aqueous and vitreous humor that had aged up to a month was compared to freshly harvested material. No difference was found between the fresh and aged media. An unsuccessful attempt was also made to use the technique for dispersion measurement of bovine cornea and lens. Future refinement may allow measurement of the dispersion of cornea and lens across the entire visible and near-infrared wavelength band. The principles of white- light interferometry including image analysis, measurement accuracy, and limitations of the technique, are discussed. In addition, alternate techniques and previous measurements of ocular dispersion are reviewed.
Hammer, Daniel X.; Noojin, Gary D.; Thomas, Robert J.; Stolarski, David J.; Rockwell, Benjamin A.; Welch, Ashley J.
Tumours of cutaneous sweat glands are uncommon, with a wide histological spectrum, complex classification and many different terms often used to describe the same tumour. Furthermore, many eccrine/apocrine lesions coexist within hamartomas or within lesions with composite/mixed differentiation. In addition to the eccrine and apocrine glands, two other skin sweat glands have recently been described: the apoeccrine and the mammary?like glands of the anogenital area. In this review (the second of two articles on skin adnexal neoplasms), common as well as important benign and malignant lesions of cutaneous sweat glands are described, and a summary for differentiating primary adnexal neoplasms from metastatic carcinoma is outlined, striving to maintain a common and acceptable terminology in this complex subject. Composite/mixed adnexal tumours are also discussed briefly.
Obaidat, Nidal A; Alsaad, Khaled O; Ghazarian, Danny
Ocular pentastomiasis is a rare infection caused by the larval stage of pentastomids, an unusual group of crustacean-related parasites. Zoonotic pentastomids have a distinct geographical distribution and utilize reptiles or canids as final hosts. Recently, an increasing number of human abdominal infections have been reported in Africa, where pentastomiasis is an emerging, though severely neglected, tropical disease. Here we describe four ocular infections caused by pentastomids from the Democratic Republic of the Congo. Two cases underwent surgery and an Armillifer grandis infection was detected by morphological and molecular approaches. Thus far, 15 other cases of ocular pentastomiasis have been reported worldwide. Twelve cases were caused by Armillifer sp., recorded almost exclusively in Africa, where such infections occur as a consequence of hunting and consuming snakes, their final hosts. Seven further cases were caused by Linguatula serrata, a cosmopolitan pentastomid whose final hosts are usually canids. Intraocular infections caused permanent visual damage in 69% and a total loss of vision in 31% of reported cases. In contrast, ocular adnexal cases had a benign clinical course. Further research is required to estimate the burden, therapeutic options and pathogenesis of this neglected disease.
Sulyok, Mihaly; Rozsa, Lajos; Bodo, Imre
A prospective study was performed to characterize any change in the pattern of ocular injuries following the introduction of compulsory front seat belt wear on 1 February 1983. During a 24-week period in 1981 (1 February to 31 July), 24 patients with eye and adnexal injuries as a result of motor car road traffic accidents (RTAs) were seen at the Bristol Eye Hospital: 12 of these patients required emergency surgery. In the identical period two years later (1 February to 31 July 1983), only 6 patients suffered ocular injury from RTAs: 3 of these required emergency surgery. All patients in the latter series obeying the legislation on seat belt use were injured by flying glass, indicating a possible change in pattern of injury. The decrease in incidence of ocular injuries from RTAs between the two series was shown to be statistically significant. The compulsory fitting of laminated glass to all new cars in Great Britain and Europe is strongly advised.
Vernon, S A; Yorston, D B
Swollen eyelids are commonly ascribed to allergic conjunctivitis, contact dermatitis, eczema, angioedema, or acute sinusitis. The differential diagnosis extends to thyroid eye disease; blepharitis; Sjögren's syndrome; Churg-Strauss vasculitis; Wegener's granulomatosis; Gleich syndrome; orbital and ocular lymphoid hyperplasia or adnexal lymphoma; idiopathic orbital inflammatory disease/idiopathic sclerosing orbital inflammation; rarely, orbital parasitosis; and IgG4-related diseases. The likely diagnosis proceeds from the more to the less common in patients without a history of allergy or infection. Both ocular lymphoid hyperplasia and ocular adnexal lymphoma must be considered in the differential diagnosis of persistent disease, and neither of these entities can be recognized or differentiated from one another clinically or radiologically. Early diagnosis is essential because therapy may consist of frequent follow-up and/or active intervention. Outcomes in patients treated early and appropriately are often favorable. PMID:22525399
Ricketti, Anthony J; Cleri, Dennis J; Moser, Robert L; Bilyk, Jurij R; Vernaleo, John R; Unkle, David W
A correct diagnosis of any adnexal mass is essential to triage women to appropriate treatment pathways. Several imaging techniques are available that may be used to provide an assessment of a mass before treatment, such as transvaginal ultrasonography, magnetic resonance imaging, computed tomography, and positron emission tomography combined with computed tomography. In this chapter, we focus in depth on the role of transvaginal ultrasonography, as current evidence suggests it is the most appropriate initial imaging investigation to identify and characterise any mass if present in women suspected of having adnexal pathology. Subjective assessment by an experienced ultrasound examiner is the optimal approach to diagnose masses, followed by risk models and rules developed by the International Ovarian Tumor Analysis study. A group of tumours has proven difficult to classify with transvaginal ultrasound, and remain a diagnostic challenge for which accurate second-stage tests would be of value. Some studies suggest that magnetic resonance imaging (MRI), compared with other imaging modalities, may play a role in the assessment of this cohort of 'difficult to classify' adnexal masses. These studies, however, did not report quality of transvaginal ultrasonography (i.e. experience level of the examiner) and lacked uniformity in describing the criteria used to define such 'difficult' masses. On the basis of standardised terminology developed by the International Ovarian Tumor Analysis study to describe adnexal masses, as well as prediction models and rules developed in the course of the study, we propose new criteria that we can use to clearly define complex or 'difficult to classify' adnexal masses to focus the role for second-line imaging tests, such as conventional magnetic resonance imaging combined with dynamic contrast-enhanced or diffusion-weighted sequences on masses where further tests other than ultrasonography would be of value. PMID:24780415
Kaijser, Jeroen; Vandecaveye, Vincent; Deroose, Christophe M; Rockall, Andrea; Thomassin-Naggara, Isabelle; Bourne, Tom; Timmerman, Dirk
To our knowledge, patients with immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) associated with autoimmune hemolytic anemia (AIHA) have not been reported previously. Many patients with IgG4-SC have autoimmune pancreatitis (AIP) and respond to steroid treatment. However, isolated cases of IgG4-SC are difficult to diagnose. We describe our experience with a patient who had IgG4-SC without AIP in whom the presence of AIHA led to diagnosis. The patient was a 73-year-old man who was being treated for dementia. Liver dysfunction was diagnosed on blood tests at another hospital. Imaging studies suggested the presence of carcinoma of the hepatic hilus and primary sclerosing cholangitis, but a rapidly progressing anemia developed simultaneously. After the diagnosis of AIHA, steroid treatment was begun, and the biliary stricture improved. IgG4-SC without AIP was thus diagnosed. PMID:25024635
Masutani, Hironori; Okuwaki, Kosuke; Kida, Mitsuhiro; Yamauchi, Hiroshi; Imaizumi, Hiroshi; Miyazawa, Shiro; Iwai, Tomohisa; Takezawa, Miyoko; Koizumi, Wasaburo
We herein present a case of an overlap of immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) and primary biliary cirrhosis (PBC). A 61-year-old man was diagnosed with PBC due to abnormal liver biochemical tests and positivity for serum anti-M2 antibody. However, his response to bezafibrate and ursodeoxycholic acid was insufficient. Five years later, his serum IgG4 level was found to increase. His liver biopsy specimens showed features of nonsuppurative destructive cholangitis in some portal tracts and periductal fibrosis with dense infiltration of IgG4-positive cells in other portal tracts. This case demonstrates that the serum IgG4 level may be worth measuring in patients with PBC refractory to conventional treatment. PMID:24990335
Takemoto, Reika; Miyake, Yasuhiro; Harada, Kenichi; Nakanuma, Yasuni; Moriya, Akio; Ando, Masaharu; Hirohata, Mamoru; Yamamoto, Kazuhide
A 59-year-old man was admitted for evaluation of short of breath and bilateral leg edema that had progressively worsened over 3 months. Chest computed tomography revealed marked pericardial thickening. Cardiac echocardiography revealed right heart volume load and diastolic dysfunction. Cardiac catheterization revealed a dip-and-plateau pattern of the pressure curve of right ventricule (RV)without any coronary disease. On a diagnosis of heart failure due to pericardial constriction, he underwent pericardiectomy under cardiopulmonary bypass. Multiple longitudinal and transverse incisions like a waffle were made in the thickened epicardium and improved the hemodynamics. The symptoms improved after surgery. As serum level of immunoglobulin G(IgG)4 was elevated and IgG4-positive plasma cells were recognized in the resected pericardium, diagnosis of constrictive pericarditis of hyper-IgG4 disease was made. PMID:24322313
Matsuzaki, Yuichi; Naruse, Yoshihiro; Tanaka, Keita; Tabata, Aya
To our knowledge, patients with immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) associated with autoimmune hemolytic anemia (AIHA) have not been reported previously. Many patients with IgG4-SC have autoimmune pancreatitis (AIP) and respond to steroid treatment. However, isolated cases of IgG4-SC are difficult to diagnose. We describe our experience with a patient who had IgG4-SC without AIP in whom the presence of AIHA led to diagnosis. The patient was a 73-year-old man who was being treated for dementia. Liver dysfunction was diagnosed on blood tests at another hospital. Imaging studies suggested the presence of carcinoma of the hepatic hilus and primary sclerosing cholangitis, but a rapidly progressing anemia developed simultaneously. After the diagnosis of AIHA, steroid treatment was begun, and the biliary stricture improved. IgG4-SC without AIP was thus diagnosed.
Masutani, Hironori; Okuwaki, Kosuke; Kida, Mitsuhiro; Yamauchi, Hiroshi; Imaizumi, Hiroshi; Miyazawa, Shiro; Iwai, Tomohisa; Takezawa, Miyoko; Koizumi, Wasaburo
Background. To validate feasibility, efficacy, and safeness of laparoscopic treatment of benign adnexal diseases through a single transumbilical access (LESS) in a prospective series of patients. Methods. A prospective clinical trial including 30 women has been conducted at the Division of Gynecology of Catholic University of Sacred Hearth of Rome. Patients underwent different laparoscopic procedures by LESS utilizing a multiport trocar and conventional straight laparoscopic instrumentation. Intra and perioperative outcome has been reported. Results. Ten mono/bilateral adnexectomies and 20 cystectomies have been performed by LESS approach. Laparoscopic procedures were completed through a single access in 28 cases (93.4%). No major intra- or postoperative complications were observed. Mean hospital stay was 1.3 days. Conclusions. LESS approach is feasible to treat benign adnexal disease with a very low conversion rate and no early or late complications. More clinical data are needed to confirm these advantages compared to standard laparoscopic technique.
Fagotti, Anna; Fanfani, Francesco; Rossitto, Cristiano; Marocco, Francesco; Gallotta, Valerio; Romano, Federico; Scambia, Giovanni
Purpose: To assess the feasibility of making a diagnosis of adnexal and orbital diseases by Tele-ophthalmological means. Materials and Methods: Tele-consultation for eye diseases was done for 3497 patients from remote areas of Tamilnadu as part of the rural tele-ophthalmology project of a tertiary eye care hospital during a period of nine months from October 2004 to June 2005. These patients were comprehensively examined on-site by optometrists. Using digitized images sent by store and forward technique and videoconferencing, the ophthalmologist made a diagnosis and advised treatment. Results: Adnexal or orbital diseases were detected in 101 out of 3497 patients (2.88%). Medical treatment was advised to 13 of 101 patients (12.8%). Surgery was advised in 62 of 101 patients (61.28%) whereas 18 of 101 patients (17.8%) required further investigations at a tertiary center. Conclusion: It was feasible to apply the satellite based tele-ophthalmology set-up for making a presumptive diagnosis and planning further management of adnexal and orbital diseases based on live interaction and digital still images of the patients.
Verma, Malay; Raman, Rajiv; Mohan, Ravindra E
The histopathologic distinction between primary adnexal carcinomas and metastatic adenocarcinoma to the skin from sites such as the breast, lung, and others often presents a diagnostic dilemma. Current markers of diagnostic utility include p63 and cytokeratin 5/6; however, their expression has been demonstrated in 11% to 22% and 27% of cutaneous metastases, respectively. Furthermore, the immunoreactivity of p40 and GATA3 in various cutaneous adnexal carcinomas has not been previously reported. In the present study, we compared the expression of p40, p63, cytokeratin 5/6, and GATA3 in a total of 143 cases, including 67 primary adnexal carcinomas and 76 cutaneous metastases. p40, p63, cytokeratin 5/6, and GATA3 expression was observed in 80%, 84%, 86%, and 47% of primary adnexal carcinoma, respectively, and in 8%, 17%, 26%, and 40% of cutaneous metastases, respectively. ?(2) Analysis revealed statistically significant P values (<.0001) for p40, p63, and cytokeratin 5/6 in distinguishing primary adnexal carcinoma from cutaneous metastases. In summary, while p63 and cytokeratin 5/6 have similar sensitivity (84% and 86%, respectively) in detecting primary adnexal carcinomas, p40 appeared to be the most specific marker (92%) with the best positive predictive value (90%). Since breast and lung are the most common sites of origin for cutaneous metastases, p40 is the best distinguishing marker in these settings. None of the four studied markers (p40, p63, cytokeratin 5/6, and GATA3) are helpful in distinguishing between primary adnexal carcinomas from cutaneous metastases of salivary gland or bladder malignancies. PMID:24746214
Lee, Jonathan J; Mochel, Mark C; Piris, Adriano; Boussahmain, Chakib; Mahalingam, Meera; Hoang, Mai P
Purpose: To present the visual outcomes and ocular sequelae of victims of cluster bombs. Materials and Methods: This retrospective, multicenter case series of ocular injury due to cluster bombs was conducted for 3 years after the war in South Lebanon (July 2006). Data were gathered from the reports to the Information Management System for Mine Action. Results: There were 308 victims of clusters bombs; 36 individuals were killed, of which 2 received ocular lacerations and; 272 individuals were injured with 18 receiving ocular injury. These 18 surviving individuals were assessed by the authors. Ocular injury occurred in 6.5% (20/308) of cluster bomb victims. Trauma to multiple organs occurred in 12 of 18 cases (67%) with ocular injury. Ocular findings included corneal or scleral lacerations (16 eyes), corneal foreign bodies (9 eyes), corneal decompensation (2 eyes), ruptured cataract (6 eyes), and intravitreal foreign bodies (10 eyes). The corneas of one patient had extreme attenuation of the endothelium. Conclusions: Ocular injury occurred in 6.5% of cluster bomb victims and 67% of the patients with ocular injury sustained trauma to multiple organs. Visual morbidity in civilians is an additional reason for a global ban on the use of cluster bombs.
Mansour, Ahmad M.; Hamade, Haya; Ghaddar, Ayman; Mokadem, Ahmad Samih; El Hajj Ali, Mohamad; Awwad, Shady
Background and Objectives: The purpose of this study was to evaluate ocular inflammatory and non-inflammatory conditions using commercially available thermal camera. Materials and Methods: A non-contact thermographic camera (FLIR P 620) was used to take thermal pictures of seven cases of ocular inflammation, two cases of non-inflammatory ocular pathology, and one healthy subject with mild refractive error only. Ocular inflammatory cases included five cases of scleritis, one case of postoperative anterior uveitis, and a case of meibomian gland dysfunction with keratitis (MGD-keratitis). Non-inflammatory conditions included a case of conjunctival benign reactive lymphoid hyperplasia (BRLH) and a case of central serous chorio-retinopathy. Thermal and non-thermal photographs were taken, and using analyzing software, the ocular surface temperature was calculated. Results: Patient with fresh episode of scleritis revealed high temperature. Eyes with MGD-keratitis depicted lower temperature in clinically more affected eye. Conjunctival BRLH showed a cold lesion on thermography at the site of involvement, in contrast to cases of scleritis with similar clinical presentation. Conclusion: Ocular thermal imaging is an underutilized diagnostic tool which can be used to distinguish inflammatory ocular conditions from non-inflammatory conditions. It can also be utilized in the evaluation of tear film in dry eye syndrome. Its applications should be further explored in uveitis and other ocular disorders. Dedicated “ocular thermographic” camera is today's need of the hour.
Kawali, Ankush A
There exist ethnic differences in the prevalence of many ocular diseases. The ocular structures affected by these diseases can be imaged with devices that have increased in complexity over recent years. The purpose of this review is to explore what we mean by the term ‘ethnicity’ and what we know of ethnic differences in the structures of the eye that
R R A Bourne; RRA Bourne
Purpose: Our goal was to determine whether visual system responses to sildenafil accompany shifts in ocular perfusion.The human choroid, which supports the metabolic functionof the outer retina, is an erectile tissue, analogous in many respects to the corpus cavenosum. Methods: Right eyes of 12 normal adults were evaluated before and 2 hafter 50 mg oral dose of sildenafil. Pulsatile ocular
Gianmarco Paris; William E. Sponsel; Sal S. Sandoval; W. Rowe Elliott; Yolanda Trigo; Donald K. Sanford; Joseph M. Harrison
This white paper describes adnexal (ovarian and paraovarian) incidental findings found on CT and MRI in nonpregnant postmenarchal patients in whom no adnexal disorder is clinically known or suspected. This represents the first of 4 such papers from the ACR Incidental Findings Committee II, which used a consensus method based on repeated reviews and revisions and a collective review and interpretation of relevant literature. Recommendations for the management of incidental adnexal findings are organized into 4 main categories: benign-appearing cysts, probably benign cysts, adnexal masses with characteristic features, and all other adnexal masses, with pathways on the basis of patient menstrual status or age (when last menstrual period is unknown). A table and flowchart are provided for reference. PMID:24007607
Patel, Maitray D; Ascher, Susan M; Paspulati, Raj Mohan; Shanbhogue, Alampady K; Siegelman, Evan S; Stein, Marjorie W; Berland, Lincoln L
Patient: Female, 46 Final Diagnosis: Unilateral adneksial and renal agenesis Symptoms: Menometrorrhagia Medication: — Clinical Procedure: Total abdominal hysterectomy and unilateral salphingoopherectomy Specialty: Obstetrics and gynecology Objective: Rare disease Background: Unilateral renal and adnexal agenesis is quite rare. Absence of any uterine abnormality accompanying current urogenital abnormalities is even rarer. Case Report: We report on the case of a 46-year-old multiparous woman, incidentally diagnosed to have unilateral renal, ovarian, and tubal agenesis just before hysterectomy due to menometrorrhagia and myoma uteri. Conclusions: Any diagnosis of a urogenital abnormality necessitates investigation of comorbid renal or genital abnormalities.
Gursoy, Asl? Yarc?; Akdemir, Nermin; Hamurcu, Ugur; Gozukucuk, Murat
A 79-year-old female was referred to our Gynecologic Department presenting with a pelvic magnetic resonance imaging (MRI), showing an adnexal mass, later confirmed at the pelvic examination. The patient's routine laboratory tests were normal. A sonographic examination was performed with inconclusive results. Although the ultrasonography excluded the presence of vascularization and malignant degeneration, the adnexal localization appeared to be dubious. The laparoscopy and the subsequent histologic examination revealed the presence of a mucocele of the appendix. The following case report focuses the attention on a misdiagnosis of appendiceal mucocele. The misdiagnosis caused no negative impact on the treatment that in this case was adequate and successful.
Paladino, Elvira; Bellantone, Maria; Sesti, Francesco; Piccione, Emilio; Pietropolli, Adalgisa
A 79-year-old female was referred to our Gynecologic Department presenting with a pelvic magnetic resonance imaging (MRI), showing an adnexal mass, later confirmed at the pelvic examination. The patient's routine laboratory tests were normal. A sonographic examination was performed with inconclusive results. Although the ultrasonography excluded the presence of vascularization and malignant degeneration, the adnexal localization appeared to be dubious. The laparoscopy and the subsequent histologic examination revealed the presence of a mucocele of the appendix. The following case report focuses the attention on a misdiagnosis of appendiceal mucocele. The misdiagnosis caused no negative impact on the treatment that in this case was adequate and successful. PMID:24804128
Paladino, Elvira; Bellantone, Maria; Conway, Francesca; Sesti, Francesco; Piccione, Emilio; Pietropolli, Adalgisa
Purpose To analyze the characteristics and clinical manifestations of clinically diagnosed and serologically confirmed ocular toxocariasis\\u000a in patients.\\u000a \\u000a \\u000a \\u000a \\u000a Methods Thirty-three eyes of 33 patients diagnosed as having ocular toxocariasis at the Hallym University Medical Center between January\\u000a 1999 and December 2008 were included in the study. The diagnosis of ocular toxocariasis was based on clinical features and\\u000a confirmed serologically by enzyme-linked immunosorbent
Soon Il Kwon; Jung Pil Lee; Sung Pyo Park; Eun Kyung Lee; Sun Huh; In Won Park
An ocular defect may affect a patient psychologically. An ocular prosthesis is given to uplift the patient psychologically and improve the confidence. Ocular prosthesis can be custom made or a stock shell. To improve the comfort and matching of the prosthesis with that of the adjacent natural eye an custom made ocular prosthesis is preferred. Different techniques are available to fabricate a custom ocular prosthesis, here we have used paper iris disk technique. PMID:23066246
Gunaseelaraj, Rajkumar; Karthikeyan, Suma; Kumar, Mohan N; Balamurugan, T; Jagadeeshwaran, A R
Ocular myasthenia gravis, although clinically often characterized by typical classical features, can cause considerable diagnostic problems. This article aims to provide a pragmatic approach to the pathogenesis, clinical features, diagnostics and therapeutic strategies in the clinical routine. PMID:23670841
Pitz, S; Jordan, B; Zierz, S
Paroxysmal vertical ocular dyskinesia of a previously undescribed nature is reported in a 42-year old woman with demyelinating disease, who was affected with a myelopathy, bilateral amblyopia, and a modified sylvian aqueduct syndrome. Electroencephalograp...
J. B. MacLean J. F. Sassin
Environmental microbial assays of industrial microscope eyepieces were conducted following reports of multiple intershift ocular infections. Pathogenic Staphylococcus aureus was identified among the microorganisms cultured. This paper suggests that direct contact with industrial microscope eyepieces provides a potentially significant route of transmission of both bacterial and viral ocular infections. An industrial hygiene ocular health questionnaire was distributed to a first and second shift manufacturing operation to assess the incidence of ocular infections. These data were compared to the questionnaire responses of 122 control manufacturing workers who did not use microscopes. Based on self-reporting by employees, those who used microscopes were found to have statistically significant incidence of sites and conjunctivitis that was 8.3 times that of the control group. Sterilization of eyepieces by ethylene oxide, formaldehyde and isopropyl alcohol were considered, but ultimately rejected. These biocides were found respectively to damage ocular lens coatings, contribute to volatile organic emissions, or be ineffective against spore-forming bacteria. This article presents a detailed evaluation of a commercially available ultraviolet sanitization unit (manufactured by the King Bactostat Corp., 7115 Armistad Street, El Paso, TX 79912). This ultraviolet disinfection process proved to be rapid and emission free; it also yielded eyepieces free of residual chemical biocides that have the potential for ocular irritation. Field tests involving 60 eyepieces demonstrated effective disinfection by a Chi-Square statistical comparison, at values greater than 95% confidence level, as compared to unirradiated eyepieces. PMID:3591662
Olcerst, R B
Background The burden and pattern of ocular trauma in China are poorly known and not well studied. We aimed at studying the epidemiological characteristics of patients hospitalized for ocular trauma at major ophthalmology departments in the largest industrial base of plastic toys in China. Methods A retrospective study of ocular trauma cases admitted to 3 tertiary hospitals in China from 1st January 2001 to 31st December 2010 was performed. Results The study included a total of 3,644 injured eyes from 3,559 patients over the 10-year period: 2,008 (55.1%) open-globe injuries, 1,580 (43.4%) closed-globe injuries, 41 (1.1%) chemical injuries, 15 (0.4%) thermal injuries and 678 (18.6%) ocular adnexal injuries. The mean age of the patients was 29.0±16.8 years with a male-to-female ratio of 5.2?1 (P?=?0.007). The most frequent types of injury were work-related injuries (1,656, 46.5%) and home-related injuries (715, 20.1%). The majority of injuries in males (56.2%) and females (36.0%) occurred in the 15–44 age group and 0–14 age group, respectively. The final visual acuity correlated with the initial visual acuity (Spearman’s correlation coefficient?=?0.659; P<0.001). The Ocular Trauma Score also correlated with the final visual acuity (Spearman’s correlation coefficient?=?0.655; P<0.001). Conclusions This analysis provides an epidemiological study of patients who were hospitalized for ocular trauma. Preventive efforts are important for both work-related and home-related eye injuries.
Cao, He; Li, Liping; Zhang, Mingzhi
Objective Trichothiodystrophy (TTD) is a rare autosomal recessive disorder characterized by sulfur-deficient brittle hair and multisystem abnormalities. Many TTD patients have a defect in known DNA repair genes. This report systematically evaluates the ocular manifestations of the largest-to-date cohort of TTD patients and xeroderma pigmentosum (XP)/TTD patients. Design Case Series Participants Thirty-two participants, ages 1 to 30 years, referred to the National Eye Institute for examination from 2001 to 2010: Twenty-five had TTD, and 7 had XP/TTD. Methods Complete, age- and developmental stage-appropriate ophthalmic examination. Main Outcome Measures Visual acuity (VA), best corrected VA, ocular motility, state of the ocular surface and corneal endothelial cell density, corneal diameter, lens assessment. Results Developmental abnormalities included microcornea (44% TTD), microphthalmia (8% TTD, 14% XP/TTD), nystagmus (40% TTD), and infantile cataracts (56% TTD and 86% XP/TTD). Corrective lenses were required by 65% of the participants, and decreased best-corrected VA was present in 28% of TTD patients and 71% of XP/TTD patients. Degenerative changes included dry eye (32% TTD, 57% XP/TTD) and ocular surface disease identified by ocular surface staining with fluorescein (32% TTD), that usually are exhibited by much older patients in the general population. The two oldest TTD patients exhibited clinical signs of retinal/macular degeneration. Four XP/TTD patients presented with corneal neovascularization. Conclusions TTD and XP/TTD study participants had a wide variety of ocular findings including refractive error, infantile cataracts, microcornea, nystagmus, and dry eye/ocular surface disease. While many of these can be ascribed to abnormal development—likely due to abnormalities in basal transcription of critical genes—patients may also have a degenerative course.
Brooks, Brian P; Thompson, Amy H; Clayton, Janine A; Chan, Chi-Chao; Tamura, Deborah; Zein, Wadih M; Blain, Delphine; Hadsall, Casey; Rowan, John; Bowles, Kristen E; Khan, Sikandar G.; Ueda, Takahiro; Boyle, Jennifer; Oh, Kyu-Seon; DiGiovanna, John J; Kraemer, Kenneth H
The ocular toxicity of fluoroquinolones and the risks of their use in the treatment of ocular infection were reviewed. Systematic identification, selection, review and synthesis of published English-language studies relating to fluoroquinolone use and safety in animals and humans was conducted. Although not free of complications, fluoroquinolones are generally safe when used to treat ocular infection. Ocular toxicity appears to be dose-dependent and results from class-effects and specific fluoroquinolone structures. Phototoxicity and neurotoxicity have been reported, and toxic effects on ocular collagen may be associated with Achilles tendinopathy. Corneal precipitation may provide an advantageous drug depot but delay healing and result in corneal perforation in approximately 10% of cases. Although human toxicity studies are limited, the current recommended dose for intracameral injection of ciprofloxacin is less than 25 microg. Intravitreal injections of ciprofloxacin 100 microg, ofloxacin 50 microg/mL, trovafloxacin 25 microg or less, moxifloxacin 160 microg/0.1 mL or less and pefloxacin 200 microg/0.1 mL are considered safe. PMID:17760640
Thompson, Andrew M
Three cases of peritoneal benign cystic mesotheliomas in women 32-34 years of age and one case of peritoneal malignant mesothelioma in a 47-year-old woman are reported. All cases presented with abdominal discomfort and/or pain and the physical and radiological diagnostic methods showed adnexal tumors. The cystic mesotheliomas developed in the cul-de-sac and the right pelvic sidewall, presented as multiple small cysts or large multilocular cystic mass. The malignant mesothelioma showed extensive infiltration of the omentum the intestinal loops and the surface of the uterus and adnexa, with bilateral hydrosalpinx and ascites. All cases presented histological and immunohistochemical characteristics consistent with tumors of mesothelial origin. No history of asbestos exposure was reported. The correct diagnostic and therapeutic approaches to these neoplasms are discussed. PMID:22439416
Dellaportas, D; Kairi-Vassilatou, E; Lykoudis, P; Mavrigiannaki, P; Mellou, S; Kleanthis, C K; Kondi-Pafiti, A
A known HIV-positive male patient presented with a rapidly progressive ulcerative lesion involving the conjunctiva, lids, and anterior orbit along with a decrease in vision in the right eye. He was on anti retro-viral and anti-tubercular therapy. In view of the clinical features, our provisional clinical diagnosis was a malignant lesion of the eyelid with orbital involvement, possibly squamous cell carcinoma. However, incisional biopsy revealed absence of malignant cells. On Gomori Methenamine Silver staining, plenty of fungal filaments were seen, which was confirmed by culture as Candida albicans. Hence, it turned out to be a case of fungal adnexal infection mimicking carcinoma in a HIV-positive patient. The patient responded well to systemic anti-fungals. PMID:24104715
Mukherjee, Bipasha; Chatterjee, Raka; Biswas, Jyotirmay
Many ocular emergencies are difficult to diagnose in the emergency setting with conventional physical examination tools. Additionally, persistent efforts to re-examine the eye may be deleterious to a patient's overall condition. Ultrasound is an important tool because it affords physicians a rapid, portable, accurate, and dynamic tool for evaluation of a variety of ocular and orbital diseases. The importance of understanding orbital anatomy, with attention to the firm attachment points of the various layers of the eye, cannot be understated. This article describes the relevant eye anatomy, delves into the ultrasound technique, and illustrates a variety of orbital pathologies detectable by bedside ultrasound. PMID:24606775
Roque, Pedro J; Hatch, Nicholas; Barr, Laurel; Wu, Teresa S
The eye attempts to limit local immune and inflammatory responses to preserve vision. This phenomenon, known as ocular immune privilege, is mediated by a combination of local and systemic mechanisms. While immune privilege is believed to protect the eye from day-to-day inflammatory insults, it is not absolute and its mechanisms are still incompletely understood.
We performed a prospective multicentre study to evaluate the efficacy of therapeutic strategies currently used for ocular toxoplasmosis in a large number of patients (n=106). Treatment was given for at least four weeks and consisted of three triple drug combinations: group 1, pyrimethamine, sulphadiazine and corticosteroids (n=29); group 2. clindamycin, sulphadiazine and corticosteroids (n=37); and group 3. cotrimoxazole (trimethoprim and
A. Rothova; H. J. Buitenhuis; C. Meenken; G. S. Baarsma; T. N. Boen-Tan; P. T. V. M. de Jong; C. M. C. Schweitzer; Z. Timmerman; J. de Vries; M. J. W. Zaal; A. Kijlstra
An ocular screening system designed for safe, convenient screening of large groups was developed at Marshall Space Flight Center, leading to the formation of Medical Sciences Corporation. The system identifies visual defects accurately and inexpensively, and includes a photorefractor telephoto lens and an electronic flash. Medical Sciences Corporation is using the device to test at schools, industrial plants, etc.
This prospective observational study was conducted in the department of Ophthalmology Mymensingh Medical College Hospital during the period of November, 2009 to October, 2010. Two hundred & fifty (250) patients of both sexes and all ages with ocular trauma were selected randomly for this study. A detailed history of patients, duration of trauma, relation of trauma with work, visual status prior to injury, any surgery prior to injury & patients were alcoholic or not were taken. Male patients were 190(76%) and female patients were 60(24%). Majority of patients were 11-20 years group (39.2%). Most of patients (40%) attended into hospital within 60 hours of ocular trauma. Accidental occupational trauma were more common (51.2%) and assault injury were less common (12.8%). Greater number of ocular trauma was caused by sharp objects (59.2%) and less number of ocular trauma was caused by chemical injuries (2.4%). Open globe injuries were more common (62%) than closed globe injury (38%). Visual acuity on admission between 6/60 to PL comprises highest number (64%) and also on discharge between 6/60 to PL comprises highest number of cases (50%). Most of the patients came from poor socioeconomic group (60%). PMID:21804497
Hossain, M M; Mohiuddin, A A; Akhanda, A H; Hossain, M I; Islam, M F; Akonjee, A R; Ali, M
Normally light transmission through the eye is benign and serves to direct vision and circadian rhythm. However, with very intense light exposure, or with ambient light exposure to the aged eye and/or young or adult eye in the presence of light-activated (photosensitizing) drugs or dietary supplements, cosmetics, or diagnostic dyes, light can be hazardous, leading to blinding disorders. Light damage to the human eye is avoided because the eye is protected by a very efficient antioxidant system and the chromophores present absorb light and dissipate its energy. After middle age, there is a decrease in the production of antioxidants and antioxidant enzymes and an accumulation of endogenous chromophores that are phototoxic. The extent to which a particular exogenous photosensitizing substance is capable of producing phototoxic side effects in the eye depends on several parameters, including (1) the chemical structure; (2) the absorption spectra of the drug; (3) binding of the drug to ocular tissue (lens proteins, melanin, DNA); and (4) the ability to cross blood-ocular barriers (amphiphilic or lipophilic). For instance, compounds that have either a tricyclic, heterocyclic, or porphyrin ring structure and are incorporated into ocular tissues are potentially phototoxic agents in the eye. The extent to which these substances might damage the eye (photoefficiency) can be predicted using in vitro and photophysical techniques. With simple, inexpensive testing, compounds can be screened for their potential ocular phototoxicity at the developmental stage. It may be that a portion of the molecule can be modified to reduce phototoxicity while leaving the primary drug effect intact. Preclinical safety testing may prevent ocular side effects that can range from mild, reversible blurred vision to permanent blindness. PMID:12537645
Roberts, Joan E
The Food and Drug Administration (FDA) is classifying the ovarian adnexal mass assessment score test system into class II (special controls). The special control that will apply to these devices is the guidance document entitled "Guidance for Industry and FDA Staff; Class II Special Controls Guidance Document: Ovarian Adnexal Mass Assessment Score Test System." The Agency is classifying these devices into class II (special controls) because special controls, in addition to general controls, will provide a reasonable assurance of safety and effectiveness of these devices and there is sufficient information to establish special controls. Elsewhere in this issue of the Federal Register, FDA is announcing the availability of a guidance document that will serve as the special control for these devices PMID:21434456
Postmenopausal women with adnexal masses suspicious for malignancy must undergo surgery for histopathologic confirmation. The low positive predictive value for malignancy of the currently available preoperative examinations results in 5 to 220 surgeries performed for each case of pelvic malignancy detected, depending on the evaluation method and patient selection. Although extensively reviewed as an effective tool for the investigation and treatment of adnexal masses, laparoscopy is still underused for this purpose in postmenopausal women. Some reasons are uncertainty about the incidental diagnosis of a malignant lesion during laparoscopy, concern about the effect of laparoscopy over the course of a pelvic malignant lesion, and inadequate referral of patients at high risk to specialized centers with oncologic gynecologists. Identification of patients at low risk might also be inadequate, causing them to undergo unnecessary laparotomy. Herein we demonstrate through a comprehensive literature review that laparoscopy is a highly effective tool for investigation and treatment of suspected adnexal masses in postmenopausal women, both in general medical settings without oncologic backup and in specialized centers. The indications for laparoscopy in this context can be further expanded without oncologic harm if patients at low and high risk are appropriately selected for surgery at general and specialized settings, respectively. PMID:22818540
Zanatta, Alysson; Rosin, Mateus Moreira Santos; Gibran, Luciano
Patient: Female, 16 Final Diagnosis: Pelvic mass Symptoms: None Medication: None Clinical Procedure: CT • MRI Specialty: Diagnostic radiology • pediatrics Objective: Unusual presentation of unknown etiology, Rare disease, Mistake in diagnosis Background: Müllerian anomalies encompass a wide variety of malformations in the female genital tract, usually associated with renal and anorectal malformations. Of these anomalies, approximately 11% are uterus didelphys, which occurs when midline fusion of the müllerian ducts is arrested to a variable extent. Case Report: We report the case of a 16-year-old female with uterine didelphys, jejunal malrotation, hematometra, hematosalpinx, and bilateral subcentimeter homogenous circular cystic-like renal lesions, who initially presented with left lower quadrant abdominal pain, non-bloody vomiting, and a history of irregular menstrual periods. Initial CT was confusing for an adnexal cystic mass, but further imaging disclosed the above müllerian anomalies. Conclusions: Müllerian anomalies may mimic other, more common, adnexal lesions; thus, adequate evaluation of suspicious cystic adnexal masses with multiple and advanced imaging modalities such as MRI is essential for adequate diagnosis and management.
Santacana-Laffitte, Guido; Ruiz, Lorell; Pedrogo, Yasmin; Colon, Edgar
Background The purpose of this study was to investigate the correlation between ocular perfusion pressure and ocular pulse amplitude in glaucoma, ocular hypertension, and normal eyes. Methods Ninety eyes from 90 patients were included. Thirty patients had been recently diagnosed with glaucoma and had no previous history of treatment for ocular hypotension, 30 had elevated intraocular pressure (IOP) without evidence of glaucoma, and 30 had normal IOP (<21 mmHg) with no detectable glaucomatous damage. Goldmann applanation tonometry (GAT), dynamic contour tonometry (DCT), blood pressure measurement, pachymetry, Humphrey visual field, and routine ophthalmic examination was performed in each patient. Ocular perfusion pressure was calculated as the difference between mean arterial pressure and IOP. The ocular pulse amplitude was given by DCT. The Pearson correlation coefficient was used to compare the glaucomatous and ocular hypertensive groups, and comparisons with the normal IOP group were done using the Spearman’s rank correlation coefficient. Results Mean IOP by DCT was 22.7 ± 4.3 mmHg in the glaucoma group, 22.3 ± 2.8 mmHg in the ocular hypertension group, and 14.3 ± 1.6 mmHg in the control group. Mean IOP by GAT was 19.0 ± 5.1 mmHg for glaucoma, 22.4 ± 2.1 mmHg for ocular hypertension, and 12.9 ± 2.2 mmHg for controls. Mean ocular pulse amplitude was 3.4 ± 1.2 mmHg in the glaucoma group, 3.5 ± 1.2 mmHg in the ocular hypertension group, and 2.6 ± 0.9 mmHg in the control group. Mean ocular perfusion pressure was 46.3 ± 7.9 mmHg in the glaucoma group, 46.3 ± 7.9 mmHg in the ocular hypertension group, and 50.2 ± 7.0 mmHg in controls. No significant correlation between ocular perfusion pressure and ocular pulse amplitude was found in any of the groups (P = 0.865 and r = ?0.032, P = 0.403 and r = ?0.156, P = 0.082 and ? = ?0.307 for glaucoma, ocular hypertension, and normal eyes, respectively). Conclusion There is no significant correlation between ocular perfusion pressure and ocular pulse amplitude values in glaucoma, ocular hypertension, or normal eyes. IOP values measured by GAT correlate with those measured by DCT.
Figueiredo, Bruno P; Cronemberger, Sebastiao; Kanadani, Fabio N
In the 10-year period from 1977 to 1987, lawnmowers have been responsible for approximately 70,000 injuries annually with an estimated 5% (3300) involving the eyes. Hyphema, angle recession, and traumatic retinopathy are especially common forms of lawnmower ocular trauma. A series of 14 cases of lawnmower-induced eye injuries is presented along with demographics of the patient population involved. An analysis of various types of lawnmowers currently in use are also included, and preventive measures are suggested. PMID:3226684
John, G; Witherspoon, C D; Feist, R M; Morris, R
In a study of over 2,000 consecutive live births, 243 newborns (12%) suffered birth trauma to the eye and its adnexa. Two hundred twenty-one cases (11%) had multiple retinal hemorrhages. Severe ocular accidents by forceps delivery were seen in the form of hyphema, Purtscher's retinopathy, corneal edema, facial palsy, and corneal abscess. Purtscher's retinopathy and corneal abscess are rare birth injuries that have not been reported so far in newborn babies. PMID:7365642
Jain, I S; Singh, Y P; Grupta, S L; Gupta, A
The term "ocular bobbing" defines a distinctive class of abnormal spontaneous vertical eye movements which occur in a variety of clinicopathological settings. Four cardinal forms, which correspond to the predicted permutations of the two characteristic clinical variables, initial vertical excursion and phasic velocity, have now been described. Reverse ocular dipping, with directional reversal and phasic inversion from typical ocular bobbing, is the last link in this functional tetrad and is newly presented. The four pathological forms share several basic phenomenological features but exhibit clinical and aetiological diversity and significant differences in prognosis. An analysis of the clinical spectrum of disorders subsumed under the general heading of "ocular bobbing" is presented.
Mehler, M F
Abuse of drugs, including some used clinically and others with no legal or clinical use, can cause ocular injury and disease. Ocular manifestations of drug abuse may be due to the substances themselves, to invasive methods of administration, or to injury suffered during states of altered consciousness. Grouping the drugs into five categories (opiates, marijuana, stimulants, depressants and hallucinogens), the authors describe the pharmacologic, congenital, toxic, infectious, embolic, and psychological ocular manifestations of their abuse. PMID:2872731
McLane, N J; Carroll, D M
Malignant cutaneous adnexal tumors (MCATs) are rare neoplasms that do not have a well-studied treatment algorithm. They are generally treated by excision alone. Given its successful application in other cutaneous malignancies, sentinel lymph node biopsy (SLNB) has been advocated by some for use in MCATs. A retrospective chart review was performed. Clinicopathological factors, recurrence patterns, and long-term follow-up were documented. Survival analysis was performed. Forty-eight subjects were identified. Mean age was 69 years with locations on the face (52%), including periocular sites (16%), extremities (25%), and all other sites (7%). Histologic distribution was sebaceous carcinoma (56%), porocarcinoma (17%), eccrine carcinoma (13%), adenocarcinoma (10%), and hidradenocarcinoma (4%). Mean follow-up was 3.9 years (range, 0 to 17 years). Nine subjects (18.8%) recurred locally, and recurrence was inversely associated with age (P = 0.04). Four (8.3%) demonstrated lymph node involvement, none without first developing local recurrence. Five-year disease-specific survival was 97 per cent (confidence interval, 81.4 to 99.6%). Despite being one of the largest reported analyses of MCATs, this study is limited by the small number of subjects. MCATs seemingly develop nodal recurrence only after demonstrating aggressive local biology. SLNB may best be applied selectively, possibly only in patients with local recurrence. PMID:24666868
Barnes, Meredith; Hestley, Andrea; Murray, Douglas R; Carlson, Grant W; Parker, Doug; Delman, Keith A
The exact function of the sympathetic nervous system in the regulation of intraocular pressure remains unclear. Many observers have noted reduced intraocular pressure in eyes whose sympathetic supply has been interrupted. A case of ocular sympathetic denervation associated with ocular hypertension is reported. Reports on the relationship between intraocular pressure and sympathetic denervation are reviewed and their relevance to this case discussed. Images
Brazier, D J
Introduction Paratesticular fibrous pseudotumors represent benign new growths confined to intrascrotal structures. Both pathogenesis and clinical management are little understood due to the rarity of the lesion, with less than 200 cases reported to date. Recently, paratesticular fibrous pseudotumors have been postulated to be immunoglobulin G4-related, pathogenetically. Here we report two cases of patients with paratesticular fibrous pseudotumor to highlight the clinical features of this rare disease and we report the immunohistochemical examinations to support the theory of paratesticular fibrous pseudotumor being an immunoglobulin G4-related disease. Case presentations A 28-year-old white man presented with a painless intrascrotal mass. After a clinical examination, a malignant growth was suspected. His ultrasound results revealed a well-demarcated hypoechoic lesion of 1.5cm in diameter at the spermatic cord. Our patient underwent local excision. His follow-up has been uneventful for 12 years. The second case was an 18-year-old white man who presented with a painless scrotal mass suspicious of testicular tumor. A magnetic resonance imaging scan revealed a 3cm mass at the spermatic cord with very low signal density on T2-weighted imaging and a low and inhomogeneous uptake of gadolinium contrast agent on T1-weighted, fat-suppressed imaging. Following local excision, our patient has been well for 18 months. On histological examination, both of the lesions consisted of collagen-rich hyalinized fibrotic tissue with storiform features. There were lymphofollicular infiltrates and, sporadically, also venulitis. The immunoglobulin G4 staining (in case 2) showed an infiltrate of 10 to 15 positive cells per high-power field on average, corresponding to a proportion of 40% in evaluable hot spots. The two patients with paratesticular fibrous pseudotumor presented within a time span of 15 years. During that time, 400 patients with testicular germ cell tumors had been treated in our institution. Conclusions The specific histological features documented in our case lend support to the theory of paratesticular fibrous pseudotumor being an immunoglobulin G4-related sclerosing disorder. Paratesticular fibrous pseudotumors usually occur in young adulthood. Clinically, paratesticular fibrous pseudotumor can mimic testicular malignancy. Ultrasonographic findings are largely unspecific, however, scrotal magnetic resonance imaging may aid in discriminating the lesion from malignant tumors. Local excision, whenever technically feasible, is the preferred treatment of paratesticular fibrous pseudotumor.
Background Appreciating the utility of published diagnostic criteria for autoimmune pancreatitis, when compared to the characteristics of patients clinically managed as having disease, informs and refines ongoing clinical practice. Methods Comparative retrospective descriptive evaluation of patients with autoimmune pancreatitis including dedicated radiology review. Results 66 subjects with radiographic OR clinical features of autoimmune pancreatitis were initially identifiable (Male: n?=?50), with 55 confirmed for evaluation. The most common presentation included pain (67%), weight loss (65%), and jaundice (62%). Diffuse enlargement of the pancreas was evident in 38%, whilst multifocal, focal, or atrophic changes were seen in 7%, 33% and 9% respectively. 13% had no pancreatic parenchymal involvement. Peripheral rim enhancement was seen in 23 patients (42%). Where discernible, disease was a) Sclerosing pancreatitis and cholangitis, n?=?21; b) Sclerosing cholangitis, n?=?9; c) Sclerosing pancreatitis, n?=?4; d) Sclerosing pancreatitis and cholangitis with pancreatic pseudotumour, n?=?7; e) Sclerosing cholangitis with hepatic pseudotumour, n?=?3; f) Sclerosing pancreatitis with pancreatic pseudotumour, n?=?1. 56% of the patients had systemic manifestations and the median serum IgG4 at diagnosis was 5.12 g/L. The Korean criteria identified most patients (82%) compared to HISORt (55%) or the Japan Pancreas Society (56%). The majority (HISORt 60%; Japan Pancreas Society 55%; Korean 58%) met diagnostic criterion by radiological findings and elevated serum IgG4. Treatment and response did not differ when stratified by diagnostic criteria. Conclusion Our descriptive and retrospective dataset confirms that in non-expert practice settings, autoimmune pancreatitis scoring systems with a focus on radiology and serology capture most patients who are clinically felt to have disease.
In this activity, learners will perform various investigations to understand the vestibular-ocular reflex and learn about the importance of visual cues in maintaining balance. During the two-part activity, learners will compare the stability of a moving image under two conditions as well as compare the effects of rotation on the sensation of spinning under varying conditions. This lesson guide includes background information, review and critical thinking questions with answers, and handouts. Educators can also use this activity to discuss how the brain functions in space and how researchers study the vestibular function in space.
Marlene Y. Macleish, Ed D.; Bernice R. Mclean, M. E.
Ocular bobbing is a distinctive movement disorder occurring in a variety of related forms herein classified as `typical', `monocular', and `atypical'. `Typical' ocular bobbing occurs in patients with paralysis of horizontal conjugate eye movements and consists of abrupt, spontaneous downward jerks of the eyes with a slow return to the mid position. The `monocular' type reflects co-existing unilateral third nerve
John O. Susac; William F. Hoyt; Robert B. Daroff; William Lawrence
Conventional pharmacokinetic methods for studying ocular drug delivery are invasive and cannot be conveniently applied to humans. The advancement of MRI technology has provided new opportunities in ocular drug-delivery research. MRI provides a means to non-invasively and continuously monitor ocular drug-delivery systems with a contrast agent or compound labeled with a contrast agent. It is a useful technique in pharmacokinetic studies, evaluation of drug-delivery methods, and drug-delivery device testing. Although the current status of the technology presents some major challenges to pharmaceutical research using MRI, it has a lot of potential. In the past decade, MRI has been used to examine ocular drug delivery via the subconjunctival route, intravitreal injection, intrascleral injection to the suprachoroidal space, episcleral and intravitreal implants, periocular injections, and ocular iontophoresis. In this review, the advantages and limitations of MRI in the study of ocular drug delivery are discussed. Different MR contrast agents and MRI techniques for ocular drug-delivery research are compared. Ocular drug-delivery studies using MRI are reviewed.
Li, S. Kevin; Lizak, Martin J.; Jeong, Eun-Kee
Accurate and reliable staging methods are crucial for optimal care of patients with ocular and orbital malignancies. Positron emission tomography/computed tomography (PET/CT) has recently emerged as a staging tool in the field of ophthalmic oncology. For detecting primary ocular or orbital lesions, PET/CT does not seem to provide an advantage over clinical ophthalmologic examination or conventional imaging studies such as CT or magnetic resonance imaging of the orbit. However, PET/CT may detect distant metastatic lesions that conventional imaging studies miss. For orbital and ocular adnexal lymphoma, use of PET/CT has been proven to be feasible and is now accepted both as a standard part of the initial staging work-up and for the assessment of response to therapy. For other ophthalmic tumors, PET/CT seems most appropriate for advanced metastatic tumors of the orbit, eyelid, and eye, for which the detection of distant metastasis with 1 comprehensive study may be preferable to performing multiple CT scans with contrast.
Hui, Ka-Hoi; Pfeiffer, Margaret L.; Esmaeli, Bita
This chapter covers the very large number of possible disorders that can affect the three ocular motor nerves, the neuromuscular junction, or the extraocular muscles. Conditions affecting the nerves are discussed under two major headings: those in which the site of damage can be anatomically localized (e.g., fascicular lesions and lesions occurring in the subarachnoid space, the cavernous sinus, the superior orbital fissure, or the orbit) and those in which the site of the lesion is either nonspecific or variable (e.g., vascular lesions, tumors, "ophthalmoplegic migraine," and congenital disorders). Specific comments on the diagnosis and management of disorders of each of the three nerves follow. Ocular motor synkineses (including Duane's retraction syndrome and aberrant regeneration) and disorders resulting in paroxysms of excess activity (e.g., neuromyotonia) are then covered, followed by myasthenia gravis and other disorders that affect the neuromuscular junction. A final section discusses disorders of the extraocular muscles themselves, including thyroid disease, orbital myositis, mitochondrial disease, and the muscular dystrophies. PMID:21601071
Lueck, Christian J
Ocular drug delivery has been a major challenge to pharmacologists and drug delivery scientists due to its unique anatomy and physiology. Static barriers (different layers of cornea, sclera, and retina including blood aqueous and blood-retinal barriers), dynamic barriers (choroidal and conjunctival blood flow, lymphatic clearance, and tear dilution), and efflux pumps in conjunction pose a significant challenge for delivery of a drug alone or in a dosage form, especially to the posterior segment. Identification of influx transporters on various ocular tissues and designing a transporter-targeted delivery of a parent drug has gathered momentum in recent years. Parallelly, colloidal dosage forms such as nanoparticles, nanomicelles, liposomes, and microemulsions have been widely explored to overcome various static and dynamic barriers. Novel drug delivery strategies such as bioadhesive gels and fibrin sealant-based approaches were developed to sustain drug levels at the target site. Designing noninvasive sustained drug delivery systems and exploring the feasibility of topical application to deliver drugs to the posterior segment may drastically improve drug delivery in the years to come. Current developments in the field of ophthalmic drug delivery promise a significant improvement in overcoming the challenges posed by various anterior and posterior segment diseases. PMID:20437123
Gaudana, Ripal; Ananthula, Hari Krishna; Parenky, Ashwin; Mitra, Ashim K
A wide-field color-coded infra-red imaging device was applied to the measurement of i) the temperature profile across the ocular surface and ii) the temporal stability of central corneal temperature, on 21 subjects. The thermographs showed a pattern of ellipsoidal isotherms (major axis horizontal) approximately concentric about a temperature apex (coldest point) which was slightly inferior to the geometric center of the cornea (GCC). The GCC had a mean temperature (+/- SD) of 34.3 +/- 0.7 degrees C (range 32.8 to 35.4 degrees C). Temperature increased towards the periphery of the cornea with the limbus being 0.45 degrees C warmer than the GCC (p less than 0.0001). Following a blink, the GCC cooled at a mean (+/- SD) rate of 0.033 +/- 0.024 degrees C/s (p less than 0.0001) over the first 15s. Subjects whose corneas cooled more slowly following a blink demonstrated a greater capacity to avoid blinking for a prolonged period (p less than 0.05). This improved method of measuring ocular surface temperature has important applications in modeling corneal physiology and pathology. PMID:2791634
Efron, N; Young, G; Brennan, N A
IgG4-related disease is a recently proposed clinical entity with several unique clinicopathological features. A chronic inflammatory state with marked fibrosis, which can often be mistaken for malignancy, especially by clinical imaging analyses, unifies these features. Little is known about lymphomagenesis in the context of IgG4-related disease, we recently first reported the ocular adnexal marginal zone B-cell lymphomas arising from IgG4-related disease. To the best of our knowledge, no existing study has ever established the neoplastic potential of IgG4-producing cells. In the present report, we describe the first IgG4-producing lymphoma. The patient was a 72-year-old male who was being followed for an asbestos-related pleural plaque. During follow-up, computed tomography revealed bilateral renal masses and multiple swollen retroperitoneal lymph nodes. A retroperitoneal lymph node biopsy was performed. Histologically, the interfollicular areas were expanded by medium to large plasmacytoid cells. These plasmacytoid cells showed nuclear pleomorphism and had prominent Russell bodies. Immunohistochemistry and double immunofluorescence staining of these cells revealed IgG4 positivity and monotypic lambda-light chain predominance. A portion of these cells were partially positive for CD20, negative for CD3, and somewhat faintly positive for CD138. In addition, serum IgG4 was elevated. Southern blot analysis of the lymph node specimen detected immunoglobulin heavy chain gene rearrangement. The present study indicates that, not only can malignant lymphomas occur in the setting of IgG4-related disease, but IgG4-producing cells can also be neoplastic. PMID:18839275
Sato, Yasuharu; Takata, Katsuyoshi; Ichimura, Kouichi; Tanaka, Takehiro; Morito, Toshiaki; Tamura, Maiko; Yoshino, Tadashi
The eye is an easily accessible, highly compartmentalised and immune-privileged organ that offers unique advantages as a gene therapy target. Significant advancements have been made in understanding the genetic pathogenesis of ocular diseases, and gene replacement and gene silencing have been implicated as potentially efficacious therapies. Recent improvements have been made in the safety and specificity of vector-based ocular gene transfer methods. Proof-of-concept for vector-based gene therapies has also been established in several experimental models of human ocular diseases. After nearly two decades of ocular gene therapy research, preliminary successes are now being reported in phase 1 clinical trials for the treatment of Leber congenital amaurosis. This review describes current developments and future prospects for ocular gene therapy. Novel methods are being developed to enhance the performance and regulation of recombinant adeno-associated virus- and lentivirus-mediated ocular gene transfer. Gene therapy prospects have advanced for a variety of retinal disorders, including retinitis pigmentosa, retinoschisis, Stargardt disease and age-related macular degeneration. Advances have also been made using experimental models for non-retinal diseases, such as uveitis and glaucoma. These methodological advancements are critical for the implementation of additional gene-based therapies for human ocular diseases in the near future.
Liu, Melissa M; Tuo, Jingsheng; Chan, Chi-Chao
Pemphigus vulgaris (PV) is an autoimmune disorder affecting the skin and mucous membranes. Ocular involvement in PV has been reported but its prevalence and clinical characteristics are not well defined. This prospective cross-sectional study of 103 PV patients was designed to determine the prevalence, clinical types and epidemiological trends of ocular involvement in a population of Iranian patients with PV. Ocular involvement was present in 17 (16.5%) patients. Conjunctivitis was the most prevalent type of ocular involvement (9/17, 52.9%), followed by erosion of the palpebral conjunctiva (7/17, 41.2%). Erosion of the bulbar conjunctiva was noted in only one patient (5.9%). The most commonly reported symptoms were eye irritation (76.5%) and redness (76.5%). No significant relation was found between ocular involvement and disease activity (partial remission or relapse). Mucoid discharge was significantly more common in patients with conjunctival erosions as compared to patients with conjunctivitis (P = 0.038). We conclude that ocular involvement is not rare in PV; 16.5% of PV patients develop ocular disease independent of the disease activity and extension. Conjunctivitis is the most common type of involvement, however, palpebral conjunctival erosion is more frequent than previously realized. PMID:24985543
Akhyani, Maryam; Keshtkar-Jafari, Alireza; Chams-Davatchi, Cheyda; Lajevardi, Vahide; Beigi, Sara; Aghazadeh, Nessa; Rayati Damavandi, Maede; Arami, Shabnam
A method and apparatus for detecting human eye defects, particularly detection of refractive error is presented. Eye reflex is recorded on color film when the eyes are exposed to a flash of light. The photographs are compared with predetermined standards to detect eye defects. The base structure of the ocular screening system is a folding interconnect structure, comprising hinged sections. Attached to one end of the structure is a head positioning station which comprises vertical support, a head positioning bracket having one end attached to the top of the support, and two head positioning lamps to verify precise head positioning. At the opposite end of the interconnect structure is a camera station with camera, electronic flash unit, and blinking fixation lamp, for photographing the eyes of persons being evaluated.
Richardson, John R. (inventor); Kerr, Joseph H. (inventor)
Every year > 600,000 sports and recreation related eye injuries occur, out of which roughly 13,500 of these result in permanent loss of sight. Up to 90% of these sports related eye injuries are preventable by using adequate eye protection equipment. Protective eyewear is made of polycarbonate, a highly impact-resistant plastic which is now easily available as prescription and non-prescription eyewear and all players should be encouraged to use them. The medical officers by educating their patients regarding the risks of eye injuries in various sports and the confirmed benefits of using protective equipment have the potential to prevent injury to over thousands of eyes every year. The medical fraternity can also play a very important role in educating the coaches, parents, and children and thus put an end to unnecessary blindness and vision loss from sports related ocular injuries, therefore ensuring a lifetime of healthy vision.
Mishra, Avinash; Verma, Ashok K.
Cancer may affect the eye and orbit as a direct result of metastatic neoplastic infiltration, compression, or circulating antibodies involving paraneoplastic retinal degeneration. A metastatic tumor to the uvea is the most common form of an intraocular metastatic process. The choroid is the most common site for uveal metastasis; metastases to the ciliary body, iris, retina, optic disk, and vitreous are rare. Approximately one-third of patients have no history of primary cancer at the time of ocular diagnosis. Breast and lung carcinomas for women and lung and gastrointestinal carcinomas for men most commonly metastasize to the eye and orbit. The short-term prognosis for vision is usually good after an individualized therapeutic approach (chemotherapy, hormonal therapy, external beam radiotherapy, or plaque radiotherapy), but the systemic prognosis is poor. The visual paraneoplastic syndromes encompass several distinct clinical and pathological entities including carcinoma-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), and bilateral diffuse melanocytic uveal proliferation (BDUMP). The CAR syndrome affects photoreceptors, MAR is thought to affect bipolar cell function, and BDUMP targets the uveal tract. Identification of circulating antibodies against retinal proteins (recovering, 23-kDa retinal protein; 46-kDa and 60-kDa retinal proteins) serves to recognize the paraneoplastic nature of the patient's symptoms, which frequently develop before the cancer is diagnosed. Anecdotal therapeutic responses are described after systemic steroids, immunoglobulin injection, and plasmapheresis. Recognition of their visual symptoms and ocular findings should alert the ophthalmologist to the possibility of cancer and systemic evaluation should be pursued. PMID:11941243
De Potter, P; Disneur, D; Levecq, L; Snyers, B
Objectives The diagnosis of an adnexal mass is a prevalent issue among women in the United States while current methods of identifying those at high risk of malignancy remain insufficient. Ineffective triage of women with malignant masses is associated with delayed or inappropriate treatment and a negative effect on disease outcome. Methods We performed an evaluation of 65 ovarian cancer-related biomarkers in the circulation of women diagnosed with an adnexal mass. Our subject group consisted of women diagnosed with benign masses and early and late stage ovarian cancer. Results Over half of the biomarkers tested were found to differ significantly between benign and malignant cases. As individual markers, HE4 and CA 125 provided the greatest level of discrimination between benign and malignant cases and the combination of these two biomarkers provided a higher level of discriminatory power than either marker considered alone. Multivariate statistical analysis identified several multi-marker panels that could discriminate early stage, late stage, and combined ovarian cancers from benign cases with similar or slightly improved SN/SP levels to the CA 125/HE4 combination, however these larger panels could not outperform the 2-biomarker panel in an independent validation set. We also identified a 3-biomarker panel with particular utility in premenopausal women. Conclusions Our findings serve to advance the development of blood-based screening methods for the discrimination of benign and malignant ovarian masses by confirming and expanding upon the superior utility of the CA 125/HE4 combination.
Nolen, Brian; Velikokhatnaya, Liudmila; Marrangoni, Adele; De Geest, Koen; Lomakin, Aleksey; Bast, Robert C.; Lokshin, Anna
Granular cell tumor (GCT) is an uncommon tumor and is believed to be of schwannian origin. GCT is benign but rare malignant cases are recorded. GCT occurs in almost any part of the body. The common sites are the tongue, skin, and subcutaneous tissue. GCT of hand is an extremely rare. Till date only 17 cases are reported in the literature. Preoperative diagnosis of GCT is important, because GCT mimics dermal adnexal tumor in subcutaneous tissue, other soft tissue tumor or inflammatory lesions. GCT is composed of large polygonal cells with eosinophilic granular cytoplasm and these cells are often immunoreactive for the S-100 protein. Fine-needle aspiration cytology has been suggested to be diagnostic modality of choice and this would undoubtedly aid the correct diagnosis. Excision with wide surgical margins is curative for benign GCT. Recurrence and malignant transformation requires regular follow-up. Here, this communication documents a case of cytological diagnosis of the granular cell tumor of hand in a 21-year-old female, clinically suspected to be a dermal adnexal tumor.
Nasit, Jitendra G.; Chauhan, Sanjaykumar; Dhruva, Gauravi
The aim of this study is to evaluate the efficacy of qualitative analysis of contrast-enhanced ultrasound (CEUS) in discrimination of adnexal masses which were undetermined by conventional ultrasound (US). A total of 120 patients underwent transabdominal CEUS. The initial enhancement time and intensity compared with the uterine myometrium, contrast agent distribution patterns and dynamic changes of enhancement were assessed. The sensitivity (Sen), specificity (Spe), positive predictive value (PPV), negative predictive value (NPV), accuracy (ACC) and Youden’s index were calculated for contrast variables. The gold standard was the histological diagnosis. There were 48 malignant tumors and 72 benign tumors. The enhancement features of malignant masses were different from benign ones. Earlier or simultaneous enhancement with inhomogeneous enhancement yielded the highest capability in differential diagnosis, and Sen, Spe, PPV, NPV, ACC, Youden’s index was 89.6%, 97.2%, 93.2%, 95.6%, 93.3%, and 0.88, respectively. The qualitative evaluation of CEUS is useful in the differential diagnosis of adnexal masses where conventional US is indeterminate.
Zhang, Xinling; Mao, Yongjiang; Zheng, Rongqin; Zheng, Zhijuan; Huang, Zeping; Huang, Dongmei; Zhang, Jing; Dai, Qing; Zhou, Xiaodong; Wen, Yanling
SUMMARY Respiratory viruses (including adenovirus, influenza virus, respiratory syncytial virus, coronavirus, and rhinovirus) cause a broad spectrum of disease in humans, ranging from mild influenza-like symptoms to acute respiratory failure. While species D adenoviruses and subtype H7 influenza viruses are known to possess an ocular tropism, documented human ocular disease has been reported following infection with all principal respiratory viruses. In this review, we describe the anatomical proximity and cellular receptor distribution between ocular and respiratory tissues. All major respiratory viruses and their association with human ocular disease are discussed. Research utilizing in vitro and in vivo models to study the ability of respiratory viruses to use the eye as a portal of entry as well as a primary site of virus replication is highlighted. Identification of shared receptor-binding preferences, host responses, and laboratory modeling protocols among these viruses provides a needed bridge between clinical and laboratory studies of virus tropism.
Rota, Paul A.; Tumpey, Terrence M.
This review article discusses the relationship between ocular perfusion pressure and glaucoma, including its definition, factors that influence its calculation and epidemiological studies investigating the influence of ocular perfusion pressure on the prevalence, incidence and progression of glaucoma. We also list the possible mechanisms behind this association, and discuss whether it is secondary to changes in intraocular pressure, blood pressure or both. Finally, we describe the circadian variation of ocular perfusion pressure and the effects of systemic and topical medications on it. We believe that the balance between IOP and BP, influenced by the autoregulatory capacity of the eye, is part of what determines whether an individual will develop optic nerve damage. However, prospective, longitudinal studies are needed to better define the role of ocular perfusion pressure in the development and progression of glaucoma. PMID:24238296
Costa, Vital P; Harris, Alon; Anderson, Douglas; Stodtmeister, Richard; Cremasco, Fernanda; Kergoat, Helene; Lovasik, John; Stalmans, Ingborg; Zeitz, Oliver; Lanzl, Ines; Gugleta, Konstantin; Schmetterer, Leopold
Objective. To report our experience treating adnexal masses using a combination of the SILS port and straight nonroticulating laparoscopic instruments. Study Design. This prospective feasibility study included 14 women with symptomatic and persistent adnexal masses. Removal of adnexal masses via single-incision laparoscopic surgery using a combination of the SILS port and straight nonroticulating laparoscopic instruments was performed. Results. All of the patients had symptomatic complex adnexal masses. Mean age of the patients was 38.4 years (range: 21–61 years) and mean duration of surgery was 71?min (range: 45–130?min). All surgeries were performed using nonroticulating straight laparoscopic instruments. Mean tumor diameter was 6?cm (range: 5–12?cm). All patient pathology reports were benign. None of the patients converted to laparotomy. All the patients were discharged on postoperative d1. Postoperatively, all the patients were satisfied with their incision and cosmetic results. Conclusion. All 14 patients were successfully treated using standard, straight nonroticulating laparoscopic instruments via the SILS port. This procedure can reduce the cost of treatment, which may eventually lead to more widespread use of the SILS port approach. Furthermore, concomitant surgical procedures are possible using this approach. However, properly designed comparative studies with single port and classic laparoscopic surgery are urgently needed.
Dursun, Polat; Tezcaner, Tugan; Zeyneloglu, Hulusi B.; Alyaz?c?, Irem; Haberal, Ali; Ayhan, Ali
Ocular tumours present a therapeutic challenge because of the sensitive tissues involved and the necessity to destroy the tumour while minimising visual loss. Radiotherapy (RT) is one of several modalites used apart from surgery, laser, cryotherapy, and chemotherapy. Both external beam RT (EBRT) and brachytherapy are used. Tumours of the bulbar conjunctiva, squamous carcinoma and malignant melanoma, can be treated with a radioactive plaque: strontium-90, ruthenium-106 (Ru-106), or iodine-125 (I-125), after excision. If the tumour involves the fornix or tarsal conjunctiva, proton therapy can treat the conjunctiva and spare most of the eye. Alternatively, an I-125 interstitial implant can be used with shielding of the cornea and lens. Conjunctival mucosal-associated lymphoid tissue lymphoma can be treated with an anterior electron field with lens shielding and 25–30 Gray (Gy) in 2?Gy fractions. Discrete retinoblastoma (RB), too large for cryotherapy or thermolaser, or recurrent after these modalities, can be treated with plaque therapy, I-125, or Ru-106. For large RB, multiple tumours, or vitreous seeds the whole eye can be treated with an I-125 applicator, sparing the bony orbit, or with EBRT, under anaesthetic, using X-rays or proton therapy with vacuum contact lenses to fix the eyes in the required position. Post-enucleated orbits at risk for recurrent RB can be treated with an I-125 implant with shielding to reduce the dose to the bony orbit. Uveal malignant melanomas can be treated with plaque or proton therapy with excellent local control. Preservation of vision will depend on the initial size and location of the tumour.
Stannard, C; Sauerwein, W; Maree, G; Lecuona, K
Female adnexal tumor of probable Wolffian origin (FATWO) is a rare neoplasm which is usually considered as benign, although in some cases metastasis or recurrences have been reported even after a long interval following the initial diagnosis. Preoperative diagnosis of FATWO is very difficult because of the rarity of the disease and the limited literature available. In this case report, we present a case of FATWO arising from the ovary and review the literature based on the clinical characteristics and management of this rare condition. A 51- year- old postmenopausal woman was referred to our clinic for evaluation of an adnexal mass. After diagnostic evaluation, the patient underwent explorative laparotomy. Intra-operatively, a solid- cystic mass was found in the right ovary, the rest of the abdomen and the pelvis were normal. The ovarian mass was removed and examined with frozen-section (FS). When the frozen section proved negative for malignancy, total abdominal hysterectomy and bilateral adnexectomy were performed. The anatomic study revealed a well-capsulated mass which was 3.5×1.5 cm in diameter. Based on pathological and immunohistochemical results, the final diagnosis was concluded to be FATWO. Adjuvant therapy was not administered. Te patient was followed up after discharge from the hospital. One year after surgery she was asymptomatic. No evidences of recurrence were observed throughout this period. Although FATWOs are rare tumors, they should be kept in mind in women with an abdominal mass. They can present diagnostic difficulties and the diagnosis is based on the exclusion of other neoplasms. FATWO has malignant potential, after the initial surgical treatment patients should be appropriately followed up for possible recurrence and metastasis.
Turkcapar, Ayse Figen; Seckin, Berna; Gungor, Tayfun; Sirvan, Levent; Mollamahmutoglu, Leyla
Female adnexal tumor of probable Wolffian origin (FATWO) is a rare neoplasm which is usually considered as benign, although in some cases metastasis or recurrences have been reported even after a long interval following the initial diagnosis. Preoperative diagnosis of FATWO is very difficult because of the rarity of the disease and the limited literature available. In this case report, we present a case of FATWO arising from the ovary and review the literature based on the clinical characteristics and management of this rare condition. A 51- year- old postmenopausal woman was referred to our clinic for evaluation of an adnexal mass. After diagnostic evaluation, the patient underwent explorative laparotomy. Intra-operatively, a solid- cystic mass was found in the right ovary, the rest of the abdomen and the pelvis were normal. The ovarian mass was removed and examined with frozen-section (FS). When the frozen section proved negative for malignancy, total abdominal hysterectomy and bilateral adnexectomy were performed. The anatomic study revealed a well-capsulated mass which was 3.5×1.5 cm in diameter. Based on pathological and immunohistochemical results, the final diagnosis was concluded to be FATWO. Adjuvant therapy was not administered. Te patient was followed up after discharge from the hospital. One year after surgery she was asymptomatic. No evidences of recurrence were observed throughout this period. Although FATWOs are rare tumors, they should be kept in mind in women with an abdominal mass. They can present diagnostic difficulties and the diagnosis is based on the exclusion of other neoplasms. FATWO has malignant potential, after the initial surgical treatment patients should be appropriately followed up for possible recurrence and metastasis. PMID:24592074
Türkçapar, Ayse Figen; Seçkin, Berna; Güngör, Tayfun; Sirvan, Levent; Mollamahmuto?lu, Leyla
The invention relates to a method of treating ocular inflammatory diseases in a patient which comprises administering a therapeutically effective amount of phospholipase A2 inhibitory peptides to the patient for inhibiting or reducing ocular inflammation....
C. C. Chan
PURPOSE: This investigation retrospectively examined ocular injuries associated with air bag deployment to gain a better appreciation of potential risk factors in motor vehicle accidents. National statistics regarding the efficacy of air bags were reviewed. METHODS: Review of the literature from 1991 to 1998 identified 44 articles describing 97 patients with air-bag-induced ocular injuries. Variables extracted from each case were age, sex, height, position in the car, eye wear, vehicle impact speed, visual acuity, and specific ocular injuries. RESULTS: Corneal abrasions occurred in 49% of occupants, hyphemas in 43%, vitreous or retinal hemorrhages in 25%, and retinal tears or detachments in 15%. The globe was ruptured in 10 patients. Patients involved in higher-speed accidents (over 30 mph) sustained a greater percentage of vitreous or retinal hemorrhages and traumatic cataracts, while those at slower speeds were more prone to retinal tears or detachments. In a subset of 14 patients with serious ocular injuries, the impact speed of 11 patients was recorded at 30 mph or less. Slower speed may be a risk factor for some ocular injuries. Occupant height was not a significant factor. National statistics confirm that air bags reduce fatalities in motor vehicle accidents. However, children sitting in the front seat without a seat belt and infants in passenger-side rear-facing car seats are at risk for fatal injury. CONCLUSION: Air bags combined with seat belts are an effective means of reducing injury and death in adults during motor vehicle accidents. However, this study has documented a wide variety of ocular injuries associated with air bag deployment. It is hoped that researchers can develop modifications that continue to save lives while minimizing additional harm. Images FIGURE 1 FIGURE 2A FIGURE 2B FIGURE 2C FIGURE 2D FIGURE 3A FIGURE 3B FIGURE 4 FIGURE 5 FIGURE 7 FIGURE 8
Stein, J D; Jaeger, E A; Jeffers, J B
The intraocular patches were prepared using gelatin as the polymer. Ocular patch were prepared by solvent casting method. The patches were prepared for six formulations GP1, GP2, GP3, GP4, GP5 and GP6. Petri dishes were used for formulation of ocular patch. Gelatin was used as a polymer of choice. Glutaraldehyde used as cross linking agent and (DMSO) dimethylsulfoxide used as solubility enhancer. The elasticity depends upon the concentration of gelatin. 400 mg amount of polymer i.e gelatin gave the required elasticity for the formulation. PMID:23066196
Upreti, Kamalesh; Dabral, Prashant; Rana, Vinod; Upadhyaya, Kumud; Badhani, Anjali
Purpose. Various ocular and orbital tissues differ in their manifestations of inflammation, although the reasons for this are unclear. Such differences may be due to behaviors exhibited by resident cell types, including fibroblasts. Fibroblasts mediate immune function and produce inflammatory mediators. Chronic stimulation of ocular fibroblasts can lead to prolonged inflammation and, in turn, to impaired vision and blindness. Interleukin (IL)-1?, which is produced by various cells during inflammation, is a potent activator of fibroblasts and inducer of the expression of inflammatory mediators. The hypothesis for this study was that that human fibroblasts derived from distinct ocular tissues differ in their responses to IL-1? and that variations in the IL-1 signaling pathway account for these differences. Methods. Human fibroblasts were isolated from the lacrimal gland, cornea, and Tenon's capsule and treated with IL-1? in vitro. Cytokine and prostaglandin (PG)E2 production were measured by ELISA and EIA. Cyclooxygenase (Cox)-2 expression was detected by Western blot. Components of the IL-1 signaling pathway were detected by flow cytometry, ELISA, Western blot, and immunofluorescence. Results. Cytokine and PGE2 production and Cox-2 expression were greatest in corneal fibroblasts. VEGF production was greatest in Tenon's capsule fibroblasts. Variations in IL-1 receptor and receptor antagonist expression, I?B? degradation and p65 nuclear translocation, however, did not account for these differences, but overexpression of the NF-?B member RelB dampened Cox-2 expression in all three fibroblast types. Conclusions. The results highlight the inherent differences between ocular fibroblast strains and provide crucial insight into novel, tissue-specific treatments for ocular inflammation and disease, such as RelB overexpression.
Xi, Xia; McMillan, David H.; Lehmann, Geniece M.; Sime, Patricia J.; Libby, Richard T.; Huxlin, Krystel R.; Feldon, Steven E.
During 2005–2008, veterinary practitioners reported ocular infection by Thelazia spp. nematodes in 115 dogs and 2 cats in southwestern France. Most cases were detected in Dordogne, particularly in 3 counties with numerous strawberry farms, which may favor development of the fruit fly vector. Animal thelaziosis may lead to emergence of human cases.
Ruytoor, Perrine; Dean, Eric; Pennant, Olivier; Dorchies, Philippe; Chermette, Rene; Otranto, Domenico
AIM To report various ocular injuries caused by durian fruit. METHODS Three cases of ocular injuries were described in young patients, due to accidental fall of durian fruit on the forehead and face, while they were taking rest/sleeping /playing under the durian tree. RESULTS The ocular injuries observed were lacerating injury of cornea with iris incarceration, hyphema, superficial penetrating injury of sclera and angle recession glaucoma in the right eye of first patient; lacerating injury of cornea with iris prolapse in the left eye of second patient; subconjunctival haemorrhage, traumatic mydriasis and superficial penetrating injury of sclera, commotion retinopathy and macular edema in the left eye of third patient. Vision improved to normal in all the eyes following surgical/ medical/optical treatment. CONCLUSION Evidence of penetrating injury (because of thorns) and blunt injury (because of weight) can be seen in the eyes when durian fruit falls on the face. Vision can be recovered fully with immediate and appropriate treatment in these cases. The ocular injuries can be prevented by educating the public to wear protective metal frame wide goggles and not to sleep/take rest under the durian tree.
Reddy, Sagili Chandrasekhara
The National Eye Institute's Section on Epithelial and Retinal Physiology and Disease (SERPD) is seeking statements of capability or interest from parties interested in collaborative research to further develop, evaluate, or commercialize therapeutics for ocular diseases caused by accumulation of sub-retinal fluid.
Objective: To determine the most discriminating two-dimensional gray-scale and colour Doppler sonographic features that allow differentiation between malignant and benign adnexal masses, and to develop a scoring model that would enable more accurate diagnosis with those features. Methods: A cross sectional prospective study was conducted on patients scheduled for surgery due to presence of adnexal masses at Woman’s Health Center, Assiut University, Egypt between October 2012 and October 2013. All patients were evaluated by 2D ultrasound for morphological features of the masses combined with colour Doppler examination of their vessels. The final diagnosis, based on histopathological analysis, was used as a gold standard. Results: One hundred forty-six patients were recruited, 104 with benign masses, 42 with malignant masses. Features that allowed statistically significant discrimination of benignity from malignancy were; volume of mass, type of mass, presence and thickness of septae, presence and length of papillary projections, location of vessels at colour Doppler and colour score. A scoring model was formulated combining these features together; Assiut Scoring Model (ASM). The cut-off level with the highest accuracy in detection of malignancy, was ?6, had a sensitivity of 93.5% and specificity of 92.2%. Conclusion: Our Scoring Model; a multiparameter scoring using four gray-scale ultrasound and two colour Doppler features, had shown a high sensitivity and specificity for prediction of malignancy in adnexal masses compared with previous scoring systems.
OBJECTIVE: To evaluate the frequency of ocular complications and the clinical outcomes of these complications in patients with various stages of HIV infection. METHODS: Retrospective review of all HIV-infected patients seen in an AIDS ophthalmology clinic from November 1983 through December 31, 1992. RESULTS: Eleven-hundred sixty-three patients were seen for ophthalmologic evaluation. Of these, 781 had the acquired immune deficiency syndrome (AIDS), 226 had symptomatic HIV infection (AIDs-related complex [ARC]), and 156 had asymptomatic HIV infection. Non-infectious HIV retinopathy was the most common ocular complication, affecting 50% of the patients with AIDS, 34% of the patients with ARC, and 3% of the patients with asymptomatic HIV infection. Cytomegalovirus (CMV) retinitis was the most common opportunistic ocular infection, affecting 37% of the patients with AIDS. Other opportunistic ocular infections, including ocular toxoplasmosis, varicella zoster virus retinitis, and Pneumocystis choroidopathy were all much less common, each occurring in < or = 1% of the patients with AIDS. Treatment of CMV retinitis with either foscarnet or ganciclovir was successful in initially controlling the retinitis. However, relapse represented a significant problem and required frequent re-inductions. As a consequence of the retinal damage associated with relapse, loss of visual acuity occurred. The median time to a visual acuity of 20/200 or worse for all eyes with CMV retinitis was 13.4 months, and the median time to a visual acuity of 20/200 or worse in the better eye was 21.1 months. At last follow-up, 75% of the patients had a final visual acuity of 20/40 or better in at least one eye. Retinal detachments were a frequent ophthalmologic complication of CMV retinitis with a cumulative probability of a retinal detachment in at least one eye of 57% at 12 months after the diagnosis of CMV retinitis. Herpes zoster ophthalmicus developed in 3% of the overall series and was seen in all stages of HIV infection. Fifty-six percent of the cases of ocular toxoplasmosis had simultaneous toxoplasmic cerebritis. Ocular toxoplasmosis responded to standard anti-microbial therapy. Varicella zoster virus retinitis, when manifested by the acute retinal necrosis (ARN) syndrome, responded to intravenous acyclovir therapy. Conversely, in a limited number of patients with the progressive outer retinal necrosis syndrome, the disease responded poorly to intravenous acyclovir therapy, but appeared to respond to combination foscarnet and acyclovir therapy. Neuro-ophthalmic lesions were present in 6% of the patients with AIDS. The most common cause of a neuro-ophthalmic lesion was cryptococcal meningitis, and 25% of the patients with cryptococcal meningitis developed a neuro-ophthalmic complication. CONCLUSIONS: Ocular manifestations are common in patients with AIDS. CMV retinitis represented a major vision-threatening problem in these patients. While available therapy was successful in initially controlling the retinitis, the phenomenon of relapse resulted in some degree of long-term visual loss. Preservation of the patient's visual acuity in at least one eye was generally successful. Other opportunistic ocular infections were substantially less common than CMV retinitis but require aggressive therapy. Images FIGURE 1 FIGURE 2 FIGURE 3 FIGURE 4 FIGURE 5 FIGURE 6
Jabs, D A
The role of nutritional supplementation in prevention of onset or progression of ocular disease is of interest to health care professionals and patients. The aim of this review is to identify those antioxidants most appropriate for inclusion in an ideal ocular nutritional supplement, suitable for those with a family history of glaucoma, cataract, or age-related macular disease, or lifestyle factors predisposing onset of these conditions, such as smoking, poor nutritional status, or high levels of sunlight exposure. It would also be suitable for those with early stages of age-related ocular disease. Literature searches were carried out on Web of Science and PubMed for articles relating to the use of nutrients in ocular disease. Those highlighted for possible inclusion were vitamins A, B, C and E, carotenoids beta-carotene, lutein, and zeaxanthin, minerals selenium and zinc, and the herb, Ginkgo biloba. Conflicting evidence is presented for vitamins A and E in prevention of ocular disease; these vitamins have roles in the production of rhodopsin and prevention of lipid peroxidation respectively. B vitamins have been linked with a reduced risk of cataract and studies have provided evidence supporting a protective role of vitamin C in cataract prevention. Beta-carotene is active in the prevention of free radical formation, but has been linked with an increased risk of lung cancer in smokers. Improvements in visual function in patients with age-related macular disease have been noted with lutein and zeaxanthin supplementation. Selenium has been linked with a reduced risk of cataract and activates the antioxidant enzyme glutathione peroxidase, protecting cell membranes from oxidative damage while zinc, although an essential component of antioxidant enzymes, has been highlighted for risk of adverse effects. As well as reducing platelet aggregation and increasing vasodilation, Gingko biloba has been linked with improvements in pre-existing field damage in some patients with normal tension glaucoma. We advocate that vitamins C and E, and lutein/zeaxanthin should be included in our theoretically ideal ocular nutritional supplement. PMID:15228513
Bartlett, Hannah; Eperjesi, Frank
It is estimated that 5–10% of all cutaneous malignancies involve the periocular region and management of periocular skin cancers account for a significant proportion of the oculoplastic surgeon's workload. Epithelial tumours are most frequently encountered, including basal cell carcinoma, squamous cell carcinoma, and sebaceous gland carcinoma, in decreasing order of frequency. Non-epithelial tumours, such as cutaneous melanoma and Merkel cell carcinoma, rarely involve the ocular adnexae. Although non-surgical treatments for periocular malignancies are gaining in popularity, surgery remains the main treatment modality and has as its main aims tumour clearance, restoration of the eyelid function, protection of the ocular surface, and achieving a good cosmetic outcome. The purpose of this article is to review the management of malignant periocular tumours, with particular emphasis on surgical management.
This paper presents a series of six patients with ocular injuries resulting from magpie attacks. Five cases involved children. In two cases the penetration was overlooked initially. In one case the keratitis was caused by Bacillus cereus. Full ophthalmic examination, including indirect ophthalmoscopy and microbiological studies, must be undertaken initially to identify unrecognised eye injuries and to prevent the possible sight-threatening complications of vitreal fibrosis with subsequent retinal detachment or endophthalmitis. PMID:1454001
Horsburgh, B J; Stark, D J; Harrison, J D
Ocular pain and related symptoms are frequent manifestations of primary and secondary headache disorders. Neurologists are often the first physician to evaluate patients affected by these clinical features. The cause of eye pain may be attributed both to pathological disorders with abnormal neurologic and neuro-ophthalmologic findings and to diseases with no apparent eye disturbances. A thorough clinical approach is necessary for an appropriate diagnosis and a correct specific management. PMID:20464596
Colombo, Bruno; Dalla Libera, Dacia; Comi, Giancarlo
We describe 16 cases of ocular and, in some patients, associated CNS toxoplasmosis in AIDS patients. T gondii is commonly associated with infection in the immunocompromised host. The lesions are most often seen in the CNS and eyes; involvement in the brain, heart, lung, liver, spleen, and lymph nodes may be observed. CNS involvement by toxoplasmosis may be an initial manifestation of AIDS and may be associated with discrete or diffuse lesions. CT scan and MR imaging may demonstrate a multitude of lesions often displaying the characteristic ring-shaped enhancement after contrast injection. Ocular involvement by toxoplasmosis, though less common than CNS involvement, is characterized by several features. These may be manifested as single or multifocal retinal lesions in one or both eyes or massive areas of retinal necrosis. Invariably these lesions are unassociated with a pre-existing retinochoroidal scar suggesting that the lesions are a manifestation of acquired rather than congenital disease. Presence of IgM antibodies may support this observation although antibody levels in AIDS patients may not reflect the magnitude of disease. Vitreous reaction is often minimal. Anterior uveitis has been reported in one case. Treatment of the ocular infection with pyrimethamine, clindamycin and sulfadiazine is effective in over 75% of patients. Once resolution of the ocular infection is observed, maintenance therapy is continued as relapses occur in the absence of treatment. Corticosteroid treatment is unnecessary and its use has been associated with the development of CMV retinitis. Other retinal infections in AIDS patients which should be considered in the differential diagnosis include CMV, herpetic-associated ARN and syphilis. Concomitant CMV and toxoplasmosis in the same eye have been seen. Images FIGURE 1 FIGURE 2 A FIGURE 2 B FIGURE 3 A FIGURE 3 B FIGURE 4 A FIGURE 4 B FIGURE 5 A FIGURE 5 B FIGURE 6 FIGURE 7 FIGURE 8 A FIGURE 8 B FIGURE 9 A FIGURE 9 B FIGURE 9 C
Gagliuso, D J; Teich, S A; Friedman, A H; Orellana, J
Ocular drug therapy has always been considered as a major challenge in the field of drug delivery. The presence of blood ocular barriers and efflux pumps has imposed a great concern as well. Various vision threatening disorders require a long term therapy of drug molecules, especially for the diseases that affect the posterior segment. Pharmaceutical companies and other research institutes have adopted a multidisciplinary approach to meet the current challenges which is evidenced by the trends seen in the published and filed U.S. patents. Various strategies have been employed to achieve long term sustained and targeted delivery for both the anterior and the posterior segments of the ocular diseases. These strategies include formulating drugs into implant, micro or nanoparticulate systems and hydrogel-based systems. Transporter targeted approach has also allowed scientists to deliver drugs to both the segments of the eye. Recent developments such as delivery of drugs utilizing ultrasound, iontophoresis and microneedle based devices have been promising. Genebased therapeutics has opened a new avenue for vision threatening disorders. In all, the current developments in the entire field have been very exciting for finding out new strategies to treat vision threatening disorders. PMID:22493994
Gaudana, Ripal J; Gokulgandhi, Mitan R; Boddu, Sai H S; Mitra, Ashim K
Purpose To evaluate the outcomes of cyclophosphamide therapy for non-infectious ocular inflammation. Design Retrospective cohort study Participants Two hundred fifteen patients with non-infectious ocular inflammation observed from initiation of cyclophosphamide. Methods Patients initiating cyclophosphamide, without other immunosuppressive drugs (other than corticosteroids), were identified at four centers. Dose of cyclophosphamide, response to therapy, corticosteroid-sparing effects, frequency of discontinuation and reasons for discontinuation were obtained by medical record review of every visit. Main Outcome Measures Control of inflammation, corticosteroid-sparing effects, discontinuation of therapy. Results The 215 patients (381 involved eyes) meeting eligibility criteria carried diagnoses of uveitis (20.4%), scleritis (22.3%), ocular mucous membrane pemphigoid (45.6%) or other forms of ocular inflammation (11.6%). Overall, approximately 49.2% (95% confidence interval (CI): 41.7%-57.2%) gained sustained control of inflammation (for at least 28 days) within 6 months, and 76% (95% CI: 68.3%-83.7%) within 12 months. Corticosteroid-sparing success (sustained control of inflammation while tapering prednisone to 10 mg or less among those not meeting success criteria initially) was gained by 30.0% and 61.2% by six and 12 months respectively. Disease remission leading to discontinuation of cyclophosphamide occurred at the rate of 0.32/person-year (95% CI: 0.24 -0.41), and the estimated proportion with remission at or prior to 2 years was 63.1% (95% CI: 51.5%-74.8%). Cyclophosphamide was discontinued by 33.5% of patients within one year because of side effects-usually of a reversible nature. Conclusions Our data suggest that cyclophosphamide is effective for the majority of patients for controlling inflammation and allowing tapering of systemic corticosteroids to 10 mg of prednisone or less, although a year of therapy may be needed to achieve these goals. Unlike with most other immunosuppressive drugs, disease remission was induced by treatment in the majority of patients who were able to tolerate therapy. In order to titrate therapy properly and to minimize the risk of serious potential side effects, a systematic program of laboratory monitoring is required. Judicious use of cyclophosphamide appears beneficial for severe ocular inflammation cases where the potentially vision-saving benefits outweigh the substantial potential side effects of therapy, or when indicated for associated systemic inflammatory diseases.
Pujari, Siddharth S.; Kempen, John H.; Newcomb, Craig W.; Gangaputra, Sapna; Daniel, Ebenezer; Suhler, Eric B.; Thorne, Jennifer E.; Jabs, Douglas A.; Levy-Clarke, Grace A.; Nussenblatt, Robert B.; Rosenbaum, James T.; Foster, C. Stephen
...2010-04-01 2010-04-01 false Ocular surgery irrigation device. 886.4360 Section...Surgical Devices Â§ 886.4360 Ocular surgery irrigation device. (a) Identification. An ocular surgery irrigation device is a device...
...2013-04-01 2013-04-01 false Ocular surgery irrigation device. 886.4360 Section...Surgical Devices Â§ 886.4360 Ocular surgery irrigation device. (a) Identification. An ocular surgery irrigation device is a device...
Aim In this paper, we will try to highlight the importance of various investigations and their crucial role in identifying whether the defect is structural or functional. Case history A 24-year-old woman presented with ocular complications after bungee jumping. Subsequently, although all ophthalmic signs resolved, she complained of decreased vision in her left eye. Conclusion Initial ophthalmic injury was detected by optical coherence tomography scan showing a neurosensory detachment of the fovea. This was not initially detected on slit-lamp examination or fluorescein angiography. On later examination, although the optical coherence tomography scan showed no structural damage, electrodiagnostic tests showed a functional defect at the fovea.
Hassan, H Mohammed J; Mariatos, Georgios; Papanikolaou, Theocharis; Ranganath, Akshatha; Hassan, Hala
In this work, we consider the results of a study carried out in our unit about 65 dialysed chronic renal failure patients. The work is based on the study of medical records and the results of ophtalmoscopic examination and phosphocalcic balance evaluation. Ocular manifestations and their relation with initial nephropathy, dialysis and phosphocalcic disturbances are described. The following alterations were observed: - corneo conjunctival deposits (60%), - cataract (26%), - hypertensive retinopathy in 21(68%) cases among 31 (48%) who suffered from hypertension, - hypertensive choroïdopathy in one case, - vascular thrombosis in one case, - diabetic retinopathy in all patients (7) with diabetes. PMID:16370213
Jalel, Taktak; Faouzi, Haouala; Faten, Taktak; Abdellatif, Achour; Mahdouani, Kacem
We have undertaken a clinical ground study of proton-induced light flashes (phosphenes). Patients treated at the Institut Curie - Centre de Protonthérapie in Orsay, France, received radiation therapy to cure ocular and skull-base cancers. Sixty percent of the patients treated for choroidal melanomas using 73 MeV protons report anomalous phosphenes. Delivering a radiation dose on the retina only is not sufficient to trigger the light flash. The present study may be the first indication of phosphenes triggered by protons of few tens of MeV.
Khan, E.; Maréchal, F.; Dendale, R.; Mabit, C.; Calugaru, V.; Desjardin, L.; Narici, L.
YOUN, J., R. E. SALLIS, G. SMITH, and K. JONES. Ocular Injury Rates in College Sports. Med. Sci. Sports Exerc., Vol. 40, No. 3, pp. 428-432,2008. Purpose: To determine the rate of eye injury sustained in 12 college sports in order to assess whether there is a high risk of ocular eye injury in some sports. Methods: From the fall
JAMES YOUN; ROBERT E. SALLISI; GARY SMITH; KIRK JONES
Ocular manifestations of lupus are fairly common, may be the presenting feature of the disease and can be sight-threatening. Almost any part of the eye and visual pathway can be affected by inflammatory or thrombotic processes. Ocular pain and visual impairment require urgent assessment by an ophthalmologist. Infection should be excluded. Optic neuritis and ischaemic optic neuropathy may be difficult
R. R. Sivaraj; O. M. Durrani; A. K. Denniston; P. I. Murray; Caroline Gordon
Rosacea is a dermatologic condition that affects the midfacial region. Ocular rosacea is most frequently diagnosed when cutaneous signs and symptoms are also present. Ocular manifestations are essentially confined to the eyelids and ocular surface. Ocular involvement ranges from minor irritation, dryness, and blurry vision to potentially severe ocular surface disruption including corneal ulcers, vascularization and rarely perforation. We present a 49-year-old Saudi Arabian female with the diagnosis of rosacea who presented with a peripheral corneal performation. The perforation was successfully managed by surgical repair, oral doxycycline and topical steroid. The final best corrected visual acuity was 20/30 after treatment. Early referral to an ophthalmologist and careful long-term follow-up are recommended. PMID:20616930
Al Arfaj, Khalid; Al Zamil, Waseem
Rosacea is a dermatologic condition that affects the midfacial region. Ocular rosacea is most frequently diagnosed when cutaneous signs and symptoms are also present. Ocular manifestations are essentially confined to the eyelids and ocular surface. Ocular involvement ranges from minor irritation, dryness, and blurry vision to potentially severe ocular surface disruption including corneal ulcers, vascularization and rarely perforation. We present a 49-year-old Saudi Arabian female with the diagnosis of rosacea who presented with a peripheral corneal performation. The perforation was successfully managed by surgical repair, oral doxycycline and topical steroid. The final best corrected visual acuity was 20/30 after treatment. Early referral to an ophthalmologist and careful long-term follow-up are recommended.
Al Arfaj, Khalid; Al Zamil, Waseem
The purpose of this study was to investigate clinical and immunological responses to Demodex on the ocular surface. Thirteen eyes in 10 patients with Demodex blepharitis and chronic ocular surface disorders were included in this study and treated by lid scrubbing with tea tree oil for the eradication of Demodex. We evaluated ocular surface manifestations and Demodex counts, and analyzed IL-1?, IL-5, IL-7, IL-12, IL-13, IL-17, granulocyte colony-stimulating factor, and macrophage inflammatory protein-1? in tear samples before and after the treatment. All patients exhibited ocular surface manifestations including corneal nodular opacity, peripheral corneal vascularization, refractory corneal erosion and infiltration, or chronic conjunctival inflammatory signs before treatment. After treatment, Demodex was nearly eradicated, tear concentrations of IL-1? and IL-17 were significantly reduced and substantial clinical improvement was observed in all patients. In conclusion, we believe that Demodex plays an aggravating role in inflammatory ocular surface disorders.
Kim, Jae Hoon; Chun, Yeoun Sook
The goal of palliative care is the achievement of the best quality of life for patients and their families. Eyes are generally the first features of the face to be noticed. Loss of an eye is a traumatic event which has a crippling effect on the psychology of the patient. Several ocular and orbital disorders require surgical intervention that may result in ocular defects. An ocular prosthesis is fabricated to restore the structure, function, and cosmetics of the defects created by such conditions. Although an implant eye prosthesis has a superior outcome, due to economic factors it may not be a feasible option for all patients. Therefore, a custom-made ocular prosthesis is a good alternative. This case report presents a palliative treatment for a patient with an enucleated eye by fabricating a custom ocular prosthesis which improved his psychological, physical, social, functional, emotional and spiritual needs.
Thakkar, Prachi; Patel, JR; Sethuraman, Rajesh; Nirmal, Narendra
Heritability is the proportion of phenotypic variation in a population that is attributable to genetic variation among individuals. Many ophthalmic disorders and biometric traits are known to have a genetic basis and consequently much work has been published in the literature estimating the heritability of various ocular parameters. We collated and summarized the findings of heritability studies conducted in the field of ophthalmology. We grouped the various studies broadly by phenotype as follows: refraction, primary open-angle glaucoma, age-related macular degeneration (AMD), cataract, diabetic retinopathy, and others. A total of 82 articles were retrieved from the literature relating to estimation of heritability for an ocular disease or biometric trait; of these, 37 papers were concerned with glaucoma, 28 with refraction, 4 with AMD, 5 with diabetic retinopathy, and 4 with cataract. The highest reported heritability for an ophthalmic trait is 0.99 for the phenotype ? 20 small hard drusen, indicating that observed variation in this parameter is largely governed by genetic factors. Over 60% of the studies employed a twin study design and a similar percentage utilized variance components methods and structural equation modeling (SEM) to derive their heritability values. Using modern SEM techniques, heritability estimates derived from twin subjects were generally higher than those from family data. Many of the estimates are in the moderate to high range, but to date the majority of genetic variants accounting for these findings have not been uncovered, hence much work remains to be undertaken to elucidate fully their molecular etiology. PMID:20851442
Sanfilippo, Paul G; Hewitt, Alex W; Hammond, Chris J; Mackey, David A
There is an important role for ?-MSH and the melanocortin receptors in ocular immunity, development and health. This chapter will cover what is known about how ?-MSH is part of the mechanisms of ocular immune privilege, about the expression of melanocortin receptors and the implications of these findings on the role of ?-MSH in ocular physiology and its potential use to treat ocular pathologies.
Taylor, Andrew W.; Lee, Darren
The eye is an easily accessible, highly compartmentalised and immune-privileged organ that offers unique advantages as a gene therapy target. Significant advancements have been made in understanding the genetic pathogenesis of ocular diseases, and gene replacement and gene silencing have been implicated as potentially efficacious therapies. Recent improvements have been made in the safety and specificity of vector-based ocular gene transfer methods. Proof-of-concept for vector-based gene therapies has also been established in several experimental models of human ocular diseases. After nearly two decades of ocular gene therapy research, preliminary successes are now being reported in phase 1 clinical trials for the treatment of Leber congenital amaurosis. This review describes current developments and future prospects for ocular gene therapy. Novel methods are being developed to enhance the performance and regulation of recombinant adeno-associated virus- and lentivirus-mediated ocular gene transfer. Gene therapy prospects have advanced for a variety of retinal disorders, including retinitis pigmentosa, retinoschisis, Stargardt disease and age-related macular degeneration. Advances have also been made using experimental models for non-retinal diseases, such as uveitis and glaucoma. These methodological advancements are critical for the implementation of additional gene-based therapies for human ocular diseases in the near future.
Liu, Melissa M; Tuo, Jingsheng; Chan, Chi-Chao
Toxoplasmic retinochoroiditis is deemed a local event, which may fail to evoke a detectable systemic immune response. A correct diagnosis of the disease is a necessary basis for estimating its clinical burden. This is not so difficult in a typical clinical picture. In atypical cases, further diagnostic efforts are to be installed. Although the aqueous humor may be analyzed for specific antibodies or the presence of parasitic DNA, the DNA burden therein is low, and in rare instances a confirmation would necessitate vitreous sampling. A laboratory confirmation of the diagnosis is frustrated by individual differences in the time elapsing between clinical symptoms and activation of specific antibody production, which may result in false negatives. In congenital ocular toxoplasmosis, a delay in the onset of specific local antibody production could reflect immune tolerance. Herein, the authors attempt to provide a simple and practicable algorithm for a clinically tailored diagnostic approach in atypical instances. PMID:21770803
Garweg, Justus G; de Groot-Mijnes, Jolanda D F; Montoya, Jose G
The ocular disorders associated with occupations include asthenopia caused by VDT work and eye disorders from eye injuries or exposure to organic solvents or ionizing radiation. The factors associated with the eye disorders can be divided into two groups: mechanical factors and non-mechanical factors. Furthermore, the latter factors can be divided into two subgroups: chemical factors and physical factors. It is said that we can prevent most accidents that cause occupation-related eye disorders if all workers wear suitable protective gear. However, the compliance with wearing protective gear is relatively low because of inattention or discomfort. The industrial specialists have to educate the workers about the proper use of the protective gear. Then, the safety and health promoters have to remind the workers to frequently check their field site for potential hazards. PMID:24605525
Ocular involvement in Behçet disease (BD) is characterized by recurrent inflammatory attacks and spontaneous resolution of acute inflammatory signs. Both frequency and severity of uveitis attacks determine the magnitude of irreversible damage to intraocular structures and long-term visual prognosis. Recurrent attacks of occlusive retinal vasculitis lead to vision-threatening complications such as cystoid macular edema, retinal neovascularization, optic atrophy and retinal atrophy. This manuscript updates about the role of various drugs in the management of BD, discussing corticosteroids, disease modifying immunosuppressive drugs, and finally biologicals (anti-TNF-blocking agents and alpha interferon) which seem to be superior compared to all other available drugs in preventing loss of vision. Also recent findings from new biologicals will be summarized, and especially the role of these drugs in children will be discussed in detail. The authors suggest that at least moderate to severe retinal involvement should become treated with biologicals whenever available. PMID:24377434
Zierhut, Manfred; Abu El-Asrar, Ahmet M; Bodaghi, Bahram; Tugal-Tutkun, Ilknur
We report a case of Fisher syndrome accompanied by ocular flutter. A 19-year-old man presented with diplopia and vertigo, associated with preceding symptoms of common cold. Since symmetric weakness of abduction in both eyes, truncal ataxia, diminution of tendon reflexes, and gaze nystagmus were noted, he was diagnosed as having Fisher syndrome. Ocular flutter also was noticed during horizontal gaze. Serum anti-GQ1b antibody and anti-GM1 antibody were detected. He was followed without therapy and the symptoms resolved. The accompanying ocular flutter may suggest that a central nervous system disorder may also be present in Fisher syndrome. PMID:20593660
Nakayasu, Koki; Sakimoto, Tohru; Minami, Masayuki; Shigihara, Syuntaro; Ishikawa, Hiroshi
The use of point-of-care ultrasound in the emergency department has expanded considerably in recent years, allowing enhanced evaluation of the patient with an emergent eye or vision complaint. The technique is simple and quick to perform, and can yield clinical information that may not be readily obtainable through physical or slit-lamp exams. Ocular bedside sonography can aid in the diagnosis of retinal and vitreous hemorrhage, retinal and vitreous detachments, ocular infections, foreign bodies, retrobulbar hematoma, or ocular vascular pathology. Optic nerve sheath diameter can be measured in patients with a suspected intracranial process as a surrogate for intracranial pressure, and may aid emergency diagnosis and management. This article reviews common emergency ophthalmic pathologies diagnosed with ultrasound in the emergency setting and a mnemonic for the use of bedside ocular ultrasound is proposed to aid in thoroughly scanning the eye and its surrounding structures. PMID:24002686
Kilker, Bret A; Holst, John M; Hoffmann, Beatrice
The Collaborative Ocular Melanoma Study (COMS) is a multicenter investigation designed to evaluate therapeutic interventions for patients who have choroidal melanoma. Two randomized controlled trials are currently being conducted. Eligible patients for th...
A five year study on the ocular complications of diabetes compared results of eye examinations and biochemical analyses of diabetics with eye disease to nondiabetics with the same prognoses. For the same two populations, results of cataract surgery and po...
T. H. Kirmani
The human eye is subjected constantly to oxidative stress due to daily exposure to sunlight, high metabolic activities, and oxygen tension. Reactive oxygen species generated from environmental insults and pathological conditions render the human eye particularly vulnerable to oxidative damage. The ocular surface composed of the tear film, the cornea, and the aqueous humor forms the first physical and biochemical barrier of the eye and plays a pivotal role in combating free radicals. These ocular compartments are enriched in certain antioxidants in the form of metabolic enzymes or small molecules. Such an antioxidant defense system in the ocular surface is essential for the maintenance of redox homeostasis in the eye and protection against oxidative damage. Herein, we review the properties and functions of key constituent antioxidants of the ocular surface.
CHEN, YING; MEHTA, GAURAV; VASILIOU, VASILIS
Pupil behavior and ocular movements were studied in chronic cats with mesencephalic transection rostral to the oculomotor nuclei; these events were correlated to the behavioral arousal of the animal, the EEG recorded at mesencephalic and pontine reticular...
The ocular surface, in a strict sense, consists of the cornea and its major support tissue, the conjunctiva. In a wider anatomical, embryological, and also functional sense, the ocular mucosal adnexa (i.e. the lacrimal gland and the lacrimal drainage system) also belong to the ocular surface. This definition includes the source and the eventual drainage of the tears that are of utmost importance to ocular surface integrity. The ocular surface is directly exposed to the external environment, and therefore is endangered by a multitude of antigens and pathogenic microorganisms. As a mucosa, it is protected by the mucosal immune system that uses innate and adaptive effector mechanisms present in the tissue and tear film. Immune protection has two partly opposing tasks: the destruction of invading pathogens is counterbalanced by the limitation of inflammatory events that could be deleterious to the subtle structure of the eye. The immune system of the ocular surface forms an eye-associated lymphoid tissue (EALT) that is recognized as a new component of the mucosal immune system. The latter consists of the mucosa-associated lymphoid tissues in different organs of the body. Mucosa- and hence eye-associated lymphoid tissues have certain characteristics that discriminate them from the central immune system. The mechanisms applied are immunological ignorance, tolerance, or an immunosuppressive local microenvironment, all of which prefer non-reactivity and anti-inflammatory immunological responses. The interaction of these mechanisms results in immune privilege of the ocular surface. During eye closure, the ocular surface appears to have different requirements that make an innate pro-inflammatory environment more attractive for immune defense. The structural and functional components that contribute to this special immune regulation will be the focus of this chapter. PMID:17264481
Knop, Erich; Knop, Nadja
Sjogren-Larson syndrome is a rare autosomal recessive neurocutaneous disorder characterized by a triad of intellectual disability, spastic diplegia or tetraplegia and congenital ichthyosis with associated ocular features, which include pigmentary changes in the retina. The usual presentation of crystalline maculopathy is from the age of 1-2 years onwards. Herein, a case of Sjogren-Larson syndrome in a 25-year-old woman is presented to highlight the ocular findings. PMID:17300584
Aslam, Sher A; Sheth, Hiten G
The ocular findings in a father and two offspring with arteriohepatic dysplasia are reported in this paper. All three people had bilateral posterior embryotoxon, Axenfeld's anomaly and a pigmentary retinopathy. Other ocular findings were exotropia, an ectopic pupil, band keratopathy, choroidal folds, anomalous optic discs and infantile myopia. Persons with arteriohepatic dysplasia typically present with prolonged neonatal jaundice. Cognizance of the associated eye findings helps distinguish this syndrome from other types of familial intrahepatic cholestasis. PMID:6796244
Romanchuk, K G; Judisch, G F; LaBrecque, D R
PURPOSE:To review recent observations regarding the sources of Toxoplasma gondii infection and rates of ocular involvement in cases of infection acquired after birth, and to reconcile them with older observations and widely held beliefs about the pathogenesis of ocular toxoplasmosis.METHOD:A review of pertinent reports from the medical literature.RESULTS:There are several potential sources and routes of infection, including inhalation of spores
Gary N. Holland
Azelastine hydrochloride 0.05% and olopatadine hydrochloride 0.1% are topical ocular allergy treatments that have demonstrated\\u000a multiple pharmacologic actions, including antihistamine, mast cell stabilization, and inhibition of proinflammatory mediators.\\u000a In this article, the mechanisms of action, efficacy, and tolerability of these two agents on ocular signs and symptoms are\\u000a examined. By studying the various target sites of drug action, an enhanced
Leonard Bielory; Praveen Buddiga; Stephen Bigelson
Recent research into animal studies has contributed significantly to understanding the pathophysiology of some well-known teratogens, such as alcohol. Techniques, such as positron emission tomography (PET) and retinal synaptogenesis studies, have helped determine the specific areas in the developing brain and ocular structures, which are targeted by various teratogens. In this article, we also highlight a few newer agents, such as benzodiazepines, with potential for ocular malformation and morbidity in the developing foetus. PMID:19229271
Tandon, A; Mulvihill, A
Immunoglobulin G4-related disease (IgG4-RD) is a systemic disorder characterized by multiorgan fibrosis with IgG4-producing plasma cells, increased IgG4 serum concentration, and responsiveness to steroid therapy. Involvement of the pancreas, salivary glands, orbit, aorta, and other sites has been well documented in the literature; however, there have been limited reports of cases involving the coronary arteries. We report the case of a 53-year-old Hispanic man who was brought to the emergency center and diagnosed with sudden cardiac death. Autopsy was subsequently performed, revealing multiorgan involvement by IgG4-RD, including involvement of the coronary arteries. The inflammation and fibrosis, in combination with concomitant atherosclerotic disease, resulted in severe stenosis of the coronary arteries. Two of the coronary arteries were further occluded by thrombosis. These factors led to cardiac hypoperfusion, myocardial infarction and, ultimately, sudden cardiac death. Fatal involvement of the coronary arteries has not been previously reported, raising a new concern for a severe complication of IgG4-RD. PMID:24878025
Patel, Nimesh R; Anzalone, Mary L; Buja, L Maximilian; Elghetany, M Tarek
The nervous system contributes to the mechanisms of ocular immune privilege by the constitutive presence of the immunosuppressive neuropeptide alpha-melanocyte stimulating hormone (alpha-MSH) in the eye. Alpha-MSH through the melanocortin 5 receptor (MC5r) mediates induction of CD4+ regulatory T cells that suppress in an antigen specific manner autoimmune disease. We investigated whether there was a role for MC5r expression in ocular immunity and the natural induction of regulatory T cells that emerged following resolution of experimental autoimmune uveoretinitis (EAU). Unlike wild type mice, EAU in MC5r-/- mice caused severe retinal damage, did mice expressed a not induce the emergence of ocular autoantigen regulatory immunity in the spleen, and the MC5r-/- classical memory immune response when reimmunized with ocular autoantigen. There was expression of MC5r in retinal pigment epithelial cells, in the ganglion cell and neural outer plexiform layers of healthy wild type mice retinas. The recovery of the ocular microenvironment from EAU was not dependent on the expression of MC5r, nor was the recovery dependent on the induction of CD4+ regulatory T cells (Treg cells) in the spleen. However, protection of the retina from the inflammatory damage of EAU and the induction of ocular autoantigen-responsive CD4+ Treg cells in the post EAU spleen requires expression of MC5r. PMID:16914087
Taylor, A W; Kitaichi, N; Biros, D
Airbags indeed reduce the risk of injury and death in motor vehicle accidents, however, ocular injury induced by airbag deployment has been reported. From 1999 to 2001, medical records were retrospectively reviewed for patients with severe ocular injury related to airbag inflation at Taipei Veterans General Hospital. The ocular presentation, clinical course, management and visual outcome were recorded and studied in detail. Three cases of ocular posterior segment injury associated with airbag inflation were identified. Mean age was 37.3 years old (range 34-39 years). None of the patients was wearing a seat belt. All patients had periocular contusion, corneal edema, and hyphema. Vitreous hemorrhage was found in 3 cases, and there were 2 cases with severe macular injury, including traumatic maculopathy and hypotony maculopathy. Retinal detachment developed in 1 case. One patient presented with traumatic macular hole 6 weeks after injury. The initial visual acuity was hand motion only in all patients, the final visual outcomes recovered to 6/20, 1/60 and 2/60, respectively. The airbag-associated posterior segment ocular trauma was induced by impact with fully deployed airbag. Severe ocular posterior segment trauma with devastating visual sequelae might occur in drivers and passengers who have not fastened their seat belt. PMID:15553805
Yang, Chang-Sue; Chou, Tzu-Fang; Liu, Jorn-Hon; Hsu, Wen-Ming
The ocular pharmacokinetics of topical diclofenac sodium was studied in two experimental models of ocular inflammation and compared to physiological conditions. Keratitis or uveitis were induced by intrastromal injection of clove oil or by intravitreal lipopolysaccharide in rabbits. The control eyes were not inflamed. Simultaneously to the induction of inflammation, 30 ?l of 0.1% diclofenac were applied topically in the
M. Palmero; J. L. Bellot; N. Alcoriza; C. García-Cabanes; A. Orts
Chlamydia trachomatis, the causative agent of trachoma, affecting hundreds of millions of people, is now recognized as a major cause of sexually transmitted disease. In many countries chlamydial infection now outstrips gonorrhoea as the major cause of genital tract infection. Chlamydial urethritis and cervicitis are frequently complicated by ascending infection involving the endometrium, the fallopian tubes and epididymis. This often results in serious reproductive sequelae, e.g., infertility in the female and ectopic pregnancy. Extra-genital manifestations of chlamydial infection may occur involving the eyes (follicular conjunctivitis), joints (arthritis), and distal intestinal tract. Infection of the newborn child during birth may result in ocular or lung disease. There is need for further research on chlamydial infection, with the involvement of a number of different fields including medicine, epidemiology, microbiology, immunology, molecular genetics and operational research. The role of chlamydia has also to be defined in a variety of clinical syndromes for the development of improved diagnostic reagents and vaccine and the production of improved control and intervention strategies.
The records of 2000 blind or partially blind persons in the onchocerciasis areas of West Africa provided the background information for this report. The author has grouped his material in three sections. The first of these deals with diagnostic methods, and contains the results of skin and conjunctival biopsies, as well as a description of onchocercomas and an estimate of the life-span of Onchocerca adults. Next, the pathogenesis of ocular lesions is discussed in the light of evidence obtained from a series of animal experiments designed to test two theories—namely, the existence of an allergic state and damage by toxins. In the last section, which is devoted to clinical observations, the author demonstrates the existence of a relationship between the posterior segmental lesion and vitamin A deficiency, and shows that punctate corneal opacities result more often from certain virus diseases and malaria than from onchocerciasis. A description follows of various degenerations due to a local nutritional disorder combined with vitamin A deficiency in onchocercal limbitis and anterior uveitis.
Rodger, F. C.
The overall goal was the development of a Cockpit Ocular Recording System (CORS). Four tasks were used: (1) the development of the system; (2) the experimentation and improvement of the system; (3) demonstrations of the working system; and (4) system documentation. Overall, the prototype represents a workable and flexibly designed CORS system. For the most part, the hardware use for the prototype system is off-the-shelf. All of the following software was developed specifically: (1) setup software that the user specifies the cockpit configuration and identifies possible areas in which the pilot will look; (2) sensing software which integrates the 60 Hz data from the oculometer and heat orientation sensing unit; (3) processing software which applies a spatiotemporal filter to the lookpoint data to determine fixation/dwell positions; (4) data recording output routines; and (5) playback software which allows the user to retrieve and analyze the data. Several experiments were performed to verify the system accuracy and quantify system deficiencies. These tests resulted in recommendations for any future system that might be constructed.
Rothenheber, Edward; Stokes, James; Lagrossa, Charles; Arnold, William; Dick, A. O.
AIM To report ocular symptoms, funduscopic findings and demographic distribution of ocular toxoplasmosis in Iran METHODS In this cross-sectional study, a total of 40 patients with ocular toxoplasmosis (24 female, 16 male) were enrolled. The distribution of symptoms and funduscopic findings were studied. RESULTS The patients' age was in the range of 13-52 with the most common age of 19 years old. Twenty-four patients were female (60.0%). The most common presenting sign was visual loss. There was anterior chamber (AC) inflammation in 23 patients (57.5%). Vitritis was presented in 36 patients (90.0%). In 35 patients (87.5%), the retinal lesion was central. In patients with peripheral lesion, 3 patients (60.0%) had flashing vs 12.5% chance of flashing in all patients. Older patients had larger lesion (P=0.04). CONCLUSION Ocular toxoplasmosis substantially varies among patients with different age, gender, status of immunity, site of lesion and other undetermined factors. One of ocular symptoms, flashing, may necessitate a more precise peripheral fundus examination.
Tabatabaei, Seidali; Soleimani, Mohammad; Foroutan, Alireza; Ahmadabadi, Mehdinili; Zarei, Reza; Piri, Nilofar; Gordiz, Arzhang
Introduction The integrity of the cells/tissues in anterior and/or posterior segments of the eye plays a crucial role in biofunctions of the vision. To maintain ocular homeostasis, selective restrictiveness of the ophthalmic membranes and barriers control must act on shuttling of biomolecules. Thus, not all attempts to apply de novo nanotechnology approaches for ocular pharmacotherapy have met with the same successes as those cited here in this review, and sometimes these novel technologies tools provoke a great deal of challenges and hurdles mainly because of functional presence of these barriers. Methods Recent published articles related to applications of ocular nanomedicines were reviewed and highlighted in this review article. Results It seems the emergence of nanomedicines have arisen great hopes for ophthalmic pharmacotherapy, in which nanostructured medicines are expected to be able to cross the restrictive barriers of the eye. Although such fast inauguration of ocular nanomedicines will literally convey new challenges in the regulatory and translational processes, it will also grant a prolific platform from which many exciting, and yet unimagined, applications of biomedical nanotechnology will emerge for pharmacotherapy of the eye. Conclusion This review provides recent advancements on ocular nanomedicines.
Nakhlband, Ailar; Barar, Jaleh
Objective To describe the ocular signs and symptoms of patients complaining of eye irritation due to volcanic fog (vog). Methods The study utilized a non-comparative, retrospective chart review of 30 patients who had a chief complaint of eye irritation, which the subjects attributed to vog. Ocular signs and symptoms are described and related to the ambient concentration of sulfur dioxide (SO2), particulate matter sized 2.5 microns (PM2.5), and vog visibility in O‘ahu during the period of the study. Results Ocular signs noted were conjunctival injection (100%), clear mucous discharge (100%), papillary reaction (100%), punctal edema (80%), eyelid swelling (73.3%) and chemosis (63.3%). Ocular symptoms were itchiness (100%), foreign body sensation (100%), tearing (96.6%) and burning sensation (90%). All patients had concurrent respiratory symptoms. During the period of study, the highest 24-hour average concentration of particulate matter sized 2.5 microns (PM2.5) was 49.04 µg/m3 and vog was visually present. Conclusions Patients complaining of eye irritation due to vog have observable ocular signs and symptoms.
Lagunzad, John Kenneth D
Squirrel monkeys normally lack ocular dominance columns in V1. This study shows that squirrel monkeys can exhibit clear ocular dominance columns if they are made strabismic within a few weeks of birth. Columns were seen only in layer 4C beta and were coarser than the overlying blob pattern in the same animal. In physiological recordings from layer 4C of a normal squirrel monkey, single units were mostly monocular, but units driven by the two eyes were intermixed. These results suggest that in squirrel monkeys activity-dependent mechanisms do normally segregate geniculate inputs from the two eyes, but on a much finer scale than in Old World primates. Strabismic owl monkeys also showed ocular dominance columns; normal owl monkeys showed variable expression. Because ocular dominance columns, when present in New World monkeys, tend to occur in later-maturing parts of layer 4C, I hypothesize that a difference in the relative timing of the maturation of geniculocortical inputs and intracortical lateral connectivity explains the variability of ocular dominance column expression in New World monkeys. PMID:8604053
Livingstone, M S
Otolith function is studied by means of measurements of ocular torsion under various acceleration environments on earth and in weightlessness. Photographic measurements of ocular torsion as indicated by the rotation of landmarks on the iris with respect to head-fixed fiducial marks were obtained in subjects undergoing horizontal linear acceleration in a ground-based version of the space sled, lateral acceleration from weightlessness during pullout from the free-fall portion of parabolic flight, and optokinetic stimulation about the roll axis in the supine position in the laboratory and during weightlessness. The responses of ocular torsion to horizontal acceleration are in agreement with a simple low-order linear system with a dominant time constant of 0.33 sec, with a transfer function fit by a model with a pure delay of 0 to 400 msec and a first-order lag. In the pullout experiment, torsion was not observed in response to the onset of acceleration in the right-ear-down position, although it was present in response to the lateral stimulus. Results of the roll vection experiments indicate the independence of ocular torsion and visually induced tilt. In addition, an automatic video system using a soft contact lens target is presented which has been developed for ocular torsion measurements.
Young, L. R.; Lichtenberg, B. K.; Arrott, A. P.; Crites, T. A.; Oman, C. M.; Edelman, E. R.
This disclosure relates to methods of treating ocular diseases using polyamine analogs, particularly conformationally restricted polyamine analogs. The ocular diseases to be treated include a variety of ophthalmic disorders characterized by angiogenesis a...
L. J. Marton P. Campochiaro S. Haidt
Background Ocular dipping (OD), or inverse ocular bobbing, consists of slow, spontaneous downward eye movements with rapid return to the primary position. It has been mainly reported following hypoxic-ischemic encephalopathy, but has also been described in association with other types of diffuse or multifocal encephalopathies and structural brainstem damage. Case Report We report the case of a previously asymptomatic 66-year-old woman who presented with confusion, recent memory disturbances, and abnormal involuntary movements, followed by a coma. Abnormal spontaneous vertical eye movements consistent with OD developed from the fourth day after admission, and the patient died 20 days later. The pathological examination of the brain confirmed the diagnosis of Creutzfeldt-Jakob disease. Conclusions The precise location of damage causing OD is unknown. In contrast to ocular bobbing, OD has no localizing value itself, but structural brainstem damage is likely when it appears combined with other spontaneous vertical eye movements.
Llamas, Sara; Gonzalo, Juan Francisco; Sanchez Sanchez, Carmen
Nonpenetrating or blunt ocular trauma, orbital trauma and systemic trauma may cause a variety of posterior segment abnormalities. Blunt ocular trauma may cause damage to the retina (commotio retinae), retinal pigment epithelium (retinal pigment epithelial edema), choroid (choroidal rupture) and optic nerve (optic nerve evulsion) alone or in combination. Traumatic macular holes and retinal detachment or dialysis may also occur after blunt ocular trauma. Trauma to the orbital tissues adjacent to the globe can cause concussive forces with damage to multiple structures within the eye (chorioretinitis sclopetaria). Systemic trauma may result in diffuse retinopathy (Purtscher's retinopathy, shaken baby syndrome) or localized retinal abnormalities (whiplash retinopathy, fat embolism syndrome). Alterations in intravascular (Valsalva retinopathy) or intracranial pressure (Terson's syndrome) due to a variety of causes may result in preretinal or vitreous hemorrhage and associated visual loss. The purpose of this report is to review each of these entities of traumatic posterior segment abnormalities. PMID:2191381
Williams, D F; Mieler, W F; Williams, G A
Lymphocytes from individuals with inactive macular disciform lesions of presumed ocular histoplasmosis challenged with three histoplasmin antigens incorporated tritiated thymidine at a significantly higher rate than histoplasmin-stimulated lymphocytes of matched control and peripheral scar groups. This finding is consistent with the etiologic association of the disciform ocular syndrome and previous systemic infection with Histoplasma capsulatum. The disciform group had a higher mean response than the other two groups to pokeweed mitogen but not to phytohemagglutinin and had higher mean counts per minute to the specific antigens Toxoplasma gondii, Blastomyces dermatitidis, Cryptococcus neoformans, Mycobacterium tuberculosis, M battery, and M gaus, but not to Candida albicans. These data would suggest that individuals with the disciform lesion of presumed ocular histoplasmosis have a hyperreactive cellular immune response; this response may play an important role in the development of the disciform.
Ganley, J.P.; Nemo, G.J.; Comstock, G.W.; Brody, J.A.
Flurbiprofen, a nonsteroidal antiinflammatory agent which is not ocularly metabolized, was employed as a probe compound to investigate the drug kinetic relationship between systemic and ocular humoral circulation. The ocular and systemic bioavailabilities of topically applied flurbiprofen were also quantitated. Anesthetized albino female rabbits received flurbiprofen doses intracamerally, topically, and intravenously at 2 to 4 week intervals. Aqueous humor and plasma were used as the sampling compartments. Plasma clearance values of flurbiprofen were 6.77 and 7.87 ml/min, after 6-mg and 208-micrograms intravenous doses, respectively. These values were not significantly different and indicated no dose-dependent disposition kinetics over a 30-fold dose range. Both ocular and systemic flurbiprofen dispositions followed a biexponential pattern with a rapid distribution phase. The systemic and ocular distribution half-lives of flurbiprofen were 12 min and 15 min, respectively. The plasma elimination half-life was 74 min and the aqueous humor elimination half-life was 93 min. The latter approximated the turnover rate of aqueous humor and suggested that aqueous humor drainage was the major process of flurbiprofen elimination from the globe. About 99% of flurbiprofen is bound to plasma protein. At distribution equilibrium, the plasma and aqueous humor concentrations of flurbiprofen differed by a hundredfold, suggesting that only free drug entered the aqueous humor after the administration of a systemic dose. In the ophthalmic studies, right eyes were instilled with 50 microliters of 0.3% flurbiprofen in saline (dose = 150 micrograms), and left eyes were instilled with 50 microliters of 0.15% flurbiprofen in saline (dose = 75 micrograms). When the area of the aqueous humor concentration-versus-time curve values was normalized by the administration dose, the 75-micrograms dose was 30% more available to ocular tissues than was the 150-micrograms dose. This demonstrated a disproportionate relationship between the administered dose and the fraction absorbed. The intracameral dose was considered to be completely bioavailable for intraocular effects. The ocular bioavailability of the ophthalmic dose was defined by using intracameral administration as a standard measurement. The ocular bioavailabilities of the 75-micrograms and 150-micrograms topical flurbiprofen doses were 10% and 7%, respectively. Systemic bioavailability after topical administration of 225 micrograms of flurbiprofen was 74%. PMID:6533296
Tang-Liu, D D; Liu, S S; Weinkam, R J
Inflammation of the eye is often times seen in association with systemic inflammatory diseases. Understanding the various forms of ocular involvement in these conditions is important as untreated ophthalmic involvement can lead to severe vision loss. In addition to providing a basic framework for diagnosis and treatment, this review will highlight the ocular manifestations of the following systemic inflammatory conditions: rheumatoid arthritis, systemic lupus erythematosus, Wegener's granulomatosis, Sjögren's syndrome, polyarteritisnodosa, primary antiphospholipid syndrome, Behçet's syndrome, Kawasaki disease, Cogan's syndrome and relapsing polychondritis. PMID:23155672
Mohsenin, Amir; Huang, John J
A 6-year-old girl presented with signs of severe anterior uveitis. After initiating treatment, a cyst like lesion was observed in the anterior chamber that led to the diagnosis of ocular cysticercosis that was ultimately confirmed with histopathologic analysis. Ocular cysticercosis usually affects the extraocular muscle. Infection of the anterior chamber has been described, although to a lesser extent. Because of the usually poor visual prognosis and the controversy in treatment, physicians should be aware of this disease and its different variants of presentation. PMID:17920320
Cortez, Maria A; Giuliari, Gian P; Escaf, Luis; Escaf, Sonia; Vidal, Claudia
Objective Instability of ocular alignment may cause surgeons to delay surgical correction of childhood esotropia. We investigated the stability of ocular alignment over 18 weeks in children with infantile esotropia (IET), acquired non-accommodative esotropia (ANAET), or acquired partially-accommodative esotropia (APAET). Design Prospective observational study Participants Two hundred thirty-three children aged 2 months to <5 years with IET, ANAET, or APAET of less than 6 months duration Methods Ocular alignment was measured at baseline and at six-week intervals for 18 weeks. Main Outcome Measure Using definitions derived from a nested test-retest study and computer simulation modeling, ocular alignment was classified as ‘unstable’ if there was a change of ? 15 prism diopters (PD) between any two of the four measurements, as ‘stable’ if all four measurements were within ? 5 PD of one another, or as ‘uncertain’ if neither criteria was met. Results Of those who completed all three follow-up visits within time windows for analysis, 27 (46%) of 59 subjects with IET had ocular alignment classified as unstable (95% confidence interval (CI) = 33 to 59%), 20% as stable (95% CI = 11 to 33%), and 34% as uncertain (95% CI = 22 to 47%). Thirteen (22%) of 60 subjects with ANAET had ocular alignment classified as unstable (95% confidence interval (CI) = 12 to 34%), 37% as stable (95% CI = 25 to 50%), and 42% as uncertain (95% CI = 29 to 55%). Six (15%) of 41 subjects with APAET had ocular alignment classified as unstable (95% CI = 6 to 29%), 39% as stable (95% CI = 24 to 56%), and 46% as uncertain (95% CI = 31 to 63%). For IET, subjects who were older at presentation were less likely to have unstable angles than subjects who were younger at presentation (risk ratio for unstable vs. stable per additional month of age = 0.85, 99% CI = 0.74 to 0.99). Conclusions Ocular alignment instability is common in children with IET, ANAET and APAET. The impact of this finding on the optimal timing for strabismus surgery in childhood esotropia awaits further study.
The following findings in chronic precollicular cats in which ocular manifestations were correlated to behavior, EEG as recorded at mesencephalic and pontine reticular formation, EMG, EKG and respiration, are reported. (1) Pupils were mydriatic, nictitati...
Epigenetics is an emerging field in ophthalmology and has opened a new avenue for understanding ocular development and ocular diseases related to aging and environment. Epigenetic mechanisms, including DNA methylation, histone modifications, chromatin remodeling, and deployment of non-coding RNAs, result in the heritable silencing of gene expression without any change in DNA sequence. Accumulating evidence suggests a potential link between gene expression, chromatin structure, non-coding RNAs, and cellular differentiation during ocular development. Disruption of the balance of epigenetic networks could become the etiology of several ocular diseases. Here, we summarized the current knowledge about epigenetic regulatory mechanisms in ocular development and diseases. PMID:24318407
Yan, Biao; Yao, Jin; Tao, Zhi-Fu; Jiang, Qin
Purpose. Pulsatile ocular blood flow (POBF is influenced by well-known parameters, such as intraocular pressure (IOP), heart rate, scleral rigidity, blood pressure, and posture. Age is also likely to influence POBF strongly. The purpose of this study was to evaluate POBF in relation to age in normal subjects. Methods. Relevant data were collected from a sample of 105 normal subjects,
Giuseppe Ravalico; Giovanni Toffoli; Giulio Pastori; Maristella Croce; Sergio Calderini
Ocular fundus reflectometry is a technique aimed at the in-vivo measurement of the reflectance of the tissues of the ocular fundus. Studies have demonstrated a correlation between optical and physiological properties of such tissues in humans and the existence of a control mechanism, called neuro-vascular coupling (NC), which adjusts local blood perfusion to support vision-induced neural activity. We developed an instrument for functional imaging of the neural tissues of the ocular fundus based on reflectance measurements to study the NC. The images acquired with the instrument needed processing to work out reflectance time-courses. The algorithm exploited previously requires long computational time, provides poor discrimination of objects and need manual intervention. We have developed a fully automatic algorithm based on differential multiscale framework for the processing of the images of the ocular fundus with reduced computational time. This algorithm is reasonably efficient to determine relative translational displacement (translation and rotation) between the images and also to remove the geometric distortion. Simulation results performed on the fundus images show that differential multiscale framework based image registration reduces computational times up-to one fourth of the time required by the general purpose algorithm, and provides better alignment precision.
Palanisamy, Nithiyanantham; Bruschi, Ilaria; Bonaiuti, Matteo; Rovati, Luigi; Riva, Charles E.
The vast majority of diseases that cause catastrophic loss of vision do so as a result of ocular neovascularization. During normal retinal vascular development, vascular endothelial cells proliferate and migrate through the extracellular matrix in response to a variety of cytokines, leading to the formation of new blood vessels in a highly ordered fashion. During abnormal neovascularization of the iris,
Michael Dorrell; Hannele Uusitalo-Jarvinen; Edith Aguilar; Martin Friedlander
Late-onset of disturbed vision is a clinical feature of bovine GM1 gangliosidosis. Studies on eight affected calves showed that ocular lesions were confined to the retinae and optic nerves. Myriad tiny white spots were visible by ophthalmic examination of the fundus. These spots were related to protuberances on the vitreal surface caused by distended retinal ganglion cells. The perikaryons of
B. J. Sheahan; W. J. C. Donnelly; T. D. Grimes
Anisometropia represents a unique example of ocular development, where the two eyes of an individual, with an identical genetic background and seemingly subject to identical environmental influences, can grow asymmetrically to produce significantly different refractive errors. This review provides an overview of the research examining myopic anisometropia, the ocular characteristics underlying the condition and the potential aetiological factors involved. Various mechanical factors are discussed, including corneal structure, intraocular pressure and forces generated during near work that may contribute to development of anisomyopia. Potential visually guided mechanisms of unequal ocular growth are also explored, including the influence of astigmatism, accommodation, higher-order aberrations and the choroidal response to altered visual experience. The association between binocular vision, ocular dominance and asymmetric refraction is also considered, along with a review of the genetic contribution to the aetiology of myopic anisometropia. Despite a significant amount of research into the biomechanical, structural and optical characteristics of anisometropic eyes, there is still no unifying theory, which adequately explains how two eyes within the same visual system grow to different endpoints. PMID:24939167
Vincent, Stephen J; Collins, Michael J; Read, Scott A; Carney, Leo G
• Background: No internationally standardized classification of ocular trauma terminology has existed heretofore. Despite a growing interest in eye injuries, the absence of a common language continues to impede both clinical care and research. • Methods: A classification was initially developed based on the authors' extensive personal experience. It then underwent repeated reviews over a 3-year period by international ophthalmic
Ferenc Kuhn; Robert Morris; C. Douglas Witherspoon; Klaus Heimann; John B. Jeffers; Giora Treister
Rosacea is a prevalent disorder that may be disfiguring and cause significant ocular morbidity, if not diagnosed and managed appropriately. Ocular rosacea, in particular, is often left undiagnosed as no specific test is available to confirm the diagnosis. Accurate diagnosis is further complicated because symptoms of ocular rosacea are not always specific to the disorder alone. Other ophthalmic disorders may present with similar findings. Further challenges exist because the severity of ocular symptoms is often not related to the severity of cutaneous findings in rosacea. Isolating a disease marker may facilitate earlier diagnosis and treatment, and could also contribute to better understanding of disease pathogenesis. The glycomics of tear fluid and saliva in patients with rosacea shows promise as an initial step in the search for a biomarker specific to the disease. We have previously found potentially important disease biomarkers in roseatic tear and saliva samples. Further investigation should prove important in the early stages of developing a set of markers for accurate disease identification. PMID:24229635
Vieira, Ana Carolina; Mannis, Mark J
The evolutionary and developmental changes in the eye muscle innervation, the inner ear, and the vestibulo-ocular reflex are examined. Three eye muscle patterns, based on the innervation by distinct ocular motoneurons populations, can be identified: a lamprey, an elasmobranch, and a bony fish/tetrapod pattern. Four distinct patterns of variation in the vestibular system are described: a hagfish pattern, a lamprey pattern, an elasmobranch pattern, and a bony fish/tetrapod pattern. Developmental data suggest an influence of the hindbrain on ear pattern formation, thus potentially allowing a concomitant change of eye muscle innervation and ear variation. The connections between the ear and the vestibular nuclei and between the vestibular nuclei and ocular motoneurons are reviewed, and the role of neurotrophins for pattern specification is discussed. Three patterns are recognized in central projections: a hagfish pattern, a lamprey pattern, and a pattern for jawed vertebrates. Second-order connections show both similarities and differences between distantly related species such as lampreys and mammals. For example, elasmobranchs lack an internuclear system, which is at best poorly developed in lampreys. It is suggested that the vestibulo-ocular system shows only a limited degree of variation because of the pronounced functional constraints imposed on it.
For many academics and policy makers the concept of landscape is associated with 'land as it is seen'. In this paper I consider some of the literature which traces this association of landscape with the sense of sight. I focus on the suggestion that notions and concepts related to landscape are symptomatic of a pervasive 'ocular-centrism' within western thought. I
Psycho-visual problems associated with utilization of monocular, bi-ocular, and binocular visual systems are reviewed in the context of present knowledge. It is noted that simply because an instrument has been designed to be binocular, it is not necessari...
G. S. Harker
The aim of the present study was to formulate and evaluate controlled release polymeric ocular delivery of acyclovir. Reservoir-type ocular inserts were fabricated by sandwiching hydroxypropyl methylcellulose (HPMC) matrix film containing acyclovir between two rate controlling membranes of cellulose acetate phthalate (CAP). The solubility and dissolution rate of poorly soluble acyclovir was enhanced by preparing binary systems with beta-cyclodextrin and then incorporated into HPMC matrix. Nine formulations (AB-1 to AB-9) with varying ratio of HPMC (drug matrix) and CAP (rate controlling membrane) were developed and sterilized by gamma radiation. The formulations were subjected to various physico-chemical evaluations. The in vitro release profile of all the formulations showed a steady, controlled drug release up to 20 h with non-Fickian diffusion behavior. A high correlation coefficient found between in vitro/in vivo release rate studies. Formation of acyclovir complex was confirmed by differential scanning calorimetry. In addition, dissolution rate studies revealed improved solubility of acyclovir when complexed with beta-cyclodextrin. Stability studies showed that the ocular inserts could be stored safely at study storage conditions. In conclusion, the present study demonstrated controlled release formulation of acyclovir inserts for ocular delivery using biodegradable polymers. PMID:19772377
Deshpande, Praful Balavant; Dandagi, Panchaxari; Udupa, Nayanabhirama; Gopal, Shavi V; Jain, Samata S; Vasanth, Surenalli G
In a retrospective survey of patients taking medication for hyperlipidaemia, those taking niacin (nicotinic acid) were more likely (p < 0.05) to report sicca syndromes, blurred vision, eyelid oedema, and macular oedema compared with those who never took niacin. Additionally, 7% of those taking niacin discontinued the drug owing to adverse ocular side effects, while none of the other lipid
F W Fraunfelder; F T Fraunfelder; D R Illingworth
The diagnostic utility of immunohistochemistry in distinguishing primary skin adnexal carcinomas from metastatic adenocarcinoma to skin: an immunohistochemical reappraisal using cytokeratin 15, nestin, p63, D2-40, and calretinin
Often the distinction of primary adnexal carcinoma from metastatic adenocarcinoma to skin from breast, lung, and other sites can be a diagnostic dilemma. Current markers purportedly of utility as diagnostic adjuncts include p63 and D2-40; however, their expression has been demonstrated in 11–22% and 5% of metastatic cutaneous metastases, respectively. Both cytokeratin (CK) 15 and nestin have been reported as
Meera Mahalingam; Lisa P Nguyen; Joanna E Richards; Alona Muzikansky; Mai P Hoang
Normal vision depends on the optimal function of ocular barriers and intact membranes that selectively regulate the environment of ocular tissues. Novel pharmacotherapeutic modalities have aimed to overcome such biological barriers which impede efficient ocular drug delivery. To determine the impact of ocular barriers on research related to ophthalmic drug delivery and targeting, herein we provide a review of the literature on isolated primary or immortalized cell culture models which can be used for evaluation of ocular barriers. In vitro cell cultures are valuable tools which serve investigations on ocular barriers such as corneal and conjunctival epithelium, retinal pigment epithelium and retinal capillary endothelium, and can provide platforms for further investigations. Ocular barrier-based cell culture systems can be simply set up and used for drug delivery and targeting purposes as well as for pathological and toxicological research.
Barar, Jaleh; Asadi, Masoud; Mortazavi-Tabatabaei, Seyed Abdolreza; Omidi, Yadollah
Background Dust exposure is a well-known occupational hazard for terrestrial workers and astronauts alike and will continue to be a concern as humankind pursues exploration and habitation of objects beyond Earth. Humankind’s limited exploration experience with the Apollo Program indicates that exposure to dust will be unavoidable. Therefore, NASA must assess potential toxicity and recommend appropriate mitigation measures to ensure that explorers are adequately protected. Visual acuity is critical during exploration activities and operations aboard spacecraft. Therefore, the present research was performed to ascertain the ocular toxicity of authentic lunar dust. Methods Small (mean particle diameter?=?2.9?±?1.0 ?m), reactive lunar dust particles were produced by grinding bulk dust under ultrapure nitrogen conditions. Chemical reactivity and cytotoxicity testing were performed using the commercially available EpiOcularTM assay. Subsequent in vivo Draize testing utilized a larger size fraction of unground lunar dust that is more relevant to ocular exposures (particles <120 ?m; median particle diameter?=?50.9?±?19.8 ?m). Results In vitro testing indicated minimal irritancy potential based on the time required to reduce cell viability by 50% (ET50). Follow-up testing using the Draize standard protocol confirmed that the lunar dust was minimally irritating. Minor irritation of the upper eyelids was noted at the 1-hour observation point, but these effects resolved within 24 hours. In addition, no corneal scratching was observed using fluorescein stain. Conclusions Low-titanium mare lunar dust is minimally irritating to the eyes and is considered a nuisance dust for ocular exposure. No special precautions are recommended to protect against ocular exposures, but fully shielded goggles may be used if dust becomes a nuisance.
Amongst the various routes of drug delivery, the field of ocular drug delivery is one of the most interesting and challenging endeavors facing the pharmaceutical scientist. Recent research has focused on the characteristic advantages and limitations of the various drug delivery systems, and further research will be required before the ideal system can be developed. Administration of drugs to the ocular region with conventional delivery systems leads to short contact time of the formulations on the epithelium and fast elimination of drugs. This transient residence time involves poor bioavailability of drugs which can be explained by the tear production, non-productive absorption and impermeability of corneal epithelium. Anatomy of the eye is shortly presented and is connected with ophthalmic delivery and bioavailability of drugs. In the present update on ocular dosage forms, chemical delivery systems such as prodrugs, the use of cyclodextrins to increase solubility of various drugs, the concept of penetration enhancers and other ocular drug delivery systems such as polymeric gels, bioadhesive hydrogels, in-situ forming gels with temperature-, pH-, or osmotically induced gelation, combination of polymers and colloidal systems such as liposomes, niosomes, cubosomes, microemulsions, nanoemulsions and nanoparticles are discussed. Novel ophthalmic delivery systems propose the use of many excipients to increase the viscosity or the bioadhesion of the product. New formulations like gels or colloidal systems have been tested with numerous active substances by in vitro and in vivo studies. Sustained drug release and increase in drug bioavailability have been obtained, offering the promise of innovation in drug delivery systems for ocular administration. Combining different properties of pharmaceutical formulations appears to offer a genuine synergy in bioavailability and sustained release. Promising results are obtained with colloidal systems which present very comfortable conditions of use and prolonged action. PMID:23153114
Achouri, Djamila; Alhanout, Kamel; Piccerelle, Philippe; Andrieu, Véronique
Background: Azithromycin (AZT) is a macrolide antibiotic derived from and similar in structure to erythromycin. Oral administration of AZT is effective for the treatment of trachoma; however, topical formulations are difficult to develop because of the drug's hydrophobicity. The aim of this study is to formulate a novel topical ophthalmic delivery system of AZT. Materials and Methods: In the present study, ocular inserts of AZT are prepared using alginate, carbopol, and hydroxypropyl methylcellulose (HPMC) to solve the said formulation problem of drug and to facilitate ocular bioavailability. Ocular inserts were prepared by film casting method and the prepared films were subjected to investigations for their physical and mechanical properties, swelling behaviors, ex vivo bioadhesion, and in vitro drug release. Ocular irritation of the developed formulation was also checked by hen's egg chorioallantoic membrane test for ocular irritation potential. Results: The physicochemical, bioadhesive, and swelling properties of films were found to vary significantly depending on the type of polymers used and their combinations. The alginate films exhibited greater bioadhesion and showed higher tensile strength and elasticity than the carbopol films. HPMC addition to the films significantly affected the properties of ocular inserts. Carbopol:HPMC (30:70)-based ocular inserts sustained drug release for longest span of 6 h. The release profile of AZT showed that drug release was by both diffusion and swelling. The formulation was found to be practically nonirritant in ocular irritation studies. Conclusion: AZT can therefore be developed as an ocular insert delivery system for the treatment of ocular surface infections.
Gilhotra, Ritu Mehra; Nagpal, Kalpana; Mishra, Dina Nath
The treatment of ocular neovascular diseases is being revolutionized by intravitreal therapies targeting vascular endothelial growth factor (VEGF). Two agents are approved for treating neovascular age-related macular degeneration and are being evaluated for other retinal conditions: the RNA aptamer pegaptanib and the monoclonal antibody antigen-binding fragment ranibizumab. Bevacizumab, a related antibody, is being used similarly, although its use is off-label. Pegaptanib selectively binds to a VEGF isoform identified as being especially pathogenic in the eye and spares other isoforms, whereas the other two agents nonselectively bind all VEGF isoforms. Because VEGF is involved in a wide variety of physiologic processes, the ocular and systemic safety of anti-VEGF agents is of paramount concern. I provide an overview of safety data for intravitreal anti-VEGF therapies, focusing primarily on randomized, controlled trials. For pegaptanib, an accumulation of data from pivotal trials and a dedicated systemic safety study have revealed no ocular or systemic safety concerns. For ranibizumab, the principal ocular adverse event detected in clinical trials was a low frequency of ocular inflammation, and systemic adverse events included a slightly elevated risk of nonocular hemorrhage and stroke. Safety data from properly designed randomized controlled trials for bevacizumab are not available. PMID:21335144
This report presents an analysis of 507 abnormal retinal reflex images taken of Huntsville kindergarten and first grade students. The retinal reflex images were obtained by using an MSFC-developed Generated Retinal Reflex Image System (GRRIS) photorefractor. The system uses a 35 mm camera with a telephoto lens with an electronic flash attachment. Slide images of the eyes were examined for abnormalities. Of a total of 1835 students screened for ocular abnormalities, 507 were found to have abnormal retinal reflexes. The types of ocular abnormalities detected were hyperopia, myopia, astigmatism, esotropia, exotropia, strabismus, and lens obstuctions. The report shows that the use of the photorefractor screening system is an effective low-cost means of screening school children for abnormalities.
The amniotic membrane, composed of 3 layers, the epithelium, basement membrane, and the stroma, was first used along with the chorion as a biologic membrane to promote healing of skin burns in 1910. In ophthalmology, it was used in 1940 in the management of conjunctival defects. Its revival in the 1990s was due to its ability to reduce ocular surface inflammation and scarring, promote rapid epithelialization due to the presence of growth factors, and antimicrobial properties. This has resulted in its application in several ocular disorders. A review of the literature shows that amniotic membrane is definitely beneficial in some but not all pathology. The future of amniotic membrane transplantation is very exciting, especially in the field of limbal stem cell research. However, further work is needed to elucidate whether it functions merely as a biologic contact lens or whether it has additional benefits. PMID:16015081
Fernandes, Merle; Sridhar, Mittanamalli S; Sangwan, Virender S; Rao, Gullapalli N
This slide presentation reviews the use of ocular testing to determine the toxicity of lunar dust. The OECD recommendations are reviewed. With these recommendations in mind the test methodology was to use EpiOcular, tissues derived from normal human epidermal keratinocytes, the cells of which have been differentiated on cell culture inserts to form a multi-layered structure, which closely parallels the corneal epithelium and to dose the tissue with 100 mg dust from various sources. The in-vitro study provides evidence that lunar dust is not severely corrosive or irritating, however, in vitro tests have limitations, and in vivo tests provides a more complete scenario, and information, it is recommended that in vivo tests be performed.
Meyers, Valerie E.
Patients suffering from blunt ocular trauma pose several legal challenges for primary eye care practitioners. Although optometrists in private practice are under no legal duty to examine patients with ocular trauma, failure to do so breaches ethical considerations. When an optometrist undertakes examination of an injured patient, a medical standard of care will be applied, and "Good Samaritan" statutes afford no shield from liability for negligence committed during the course of examination and treatment. If specialized care is necessary, a timely referral must be arranged. Practitioners are liable for the acts or omissions of employees, and receptionists who schedule appointments should be able to triage patient complaints so that same-day appointments are scheduled for patients requiring urgent assessment and care. Documentation of the disposition of these patients is essential; forms are an ideal way to record and preserve essential information. PMID:8268690
Classé, J G
BACKGROUND—The influence of diabetes mellitus on ocular pulse amplitude (OPA), an indirect measure of choroidal perfusion, is unclear.?METHODS—OPA, using the Langham ocular blood flow (OBF) system, applanation intraocular pressure (IOP), systemic blood pressure (BP), heart rate, and haemoglobin (Hb) A1c were measured in patients with insulin dependent diabetes mellitus (IDDM) with no (DR-0, n = 22) non-proliferative (DR-1, n = 24), and proliferative (DR-2, n = 18) diabetic retinopathy.?RESULTS—Neither local (OPA, IOP) nor systemic perfusion parameters (BPs, HR) nor HbA1c were significantly altered in DR-0, DR-1, or DR-2 IDDM patients.?CONCLUSION—Choroidal circulation remains unaffected as diabetic retinopathy advances.??
Schmidt, K.; von Ruckmann, A.; Kemkes-Matthes, B.; Hammes, H.
Multiple sclerosis is the most common disabling neurological disease in young people. Many neuro-ophthalmological manifestations can occur during the course of the illness, and optic neuritis is both the most frequent and the best known. However, some ocular motility disorders can also occur, but since there may be no symptoms, they often remain underdiagnosed. These eye movement disorders are mostly related to brain-stem and cerebellum lesions. They can be acute, indicating a spatial dissemination of the demyelinating disease, and consequently may indicate the need for systemic treatment of multiple sclerosis. More often, they evolve slowly, progressing with the multiple sclerosis. In this case, they could provide a clue to the prognosis of the disease. In this survey, we discuss the two major symptoms of ocular motor disorders likely to occur during multiple sclerosis: oscillopsia and diplopia. PMID:18971858
Rougier, M-B; Tilikete, C
Human cysticercosis and tuberculosis are endemic diseases in developing countries. Both these diseases have certain common\\u000a factors of origin. We would like to present the co-existence of these infections in a 20-year-old female. She was a known\\u000a case of pulmonary and ocular tuberculosis and she acquired cysticercosis of the eye and brain.
A. Rani; N. Pushker; A. Kulkarni; Rajpal; R. Balasubramanya; M. S. Bajaj
A 55-year-old woman presented to the emergency department following an episode of severe visual impairment, headache, dizziness and confusion. The patient had been taking quinine sulfate as long-term medication for leg cramps. During an episode of sleepwalking, the patient had taken an overdose of quinine sulfate. Following a thorough investigation and assessment, a diagnosis of ocular quinine toxicity was made. We present this case and highlight the risks of quinine prescription. PMID:24287479
Sinha, Amit; Al Husainy, Sahar
Fifty consecutive patients with clinically definite multiple sclerosis were studied to assess the prevalence of concomitant uveitis. Asymptomatic ocular inflammatory changes were found in nine patients (18%) and appeared to show a positive correlation with severe and progressive disease. Conversely uveitis was uncommon in the presence of established optic atrophy which suggests a negative influence on its pathogenesis. In the absence of optic atrophy inflammatory changes in the eye may be a valuable index of disease activity. Images
Graham, E M; Francis, D A; Sanders, M D; Rudge, P
Purpose To document the characteristics, treatments, and anatomical and functional outcomes of patients with ocular trauma from improvised explosive devices (IEDs). Methods Retrospective review of ocular injuries caused by IEDs, admitted to our tertiary referral centre. Results In total, sixty-one eyes of the 39 patients with an average age of 24 years (range, 20–42 years) were included in the study. In total, 49 (80%) eyes of the patients had open-globe and 12 (20%) had closed-globe injury. In eyes with open-globe injury, intraocular foreign body (IOFB) injury was the most frequently encountered type of injury, observed in 76% of eyes. Evisceration or enucleation was required as a primary surgical intervention in 17 (28%) of the eyes. Twenty-two (36%) eyes had no light perception at presentation. Patients were followed up for an average of 6 months (range, 4–34 months). At the last follow-up, 26 (43%) of 61 eyes had no light perception. Postoperative proliferative vitreoretinopathy (PVR) developed in 12 (50%) of the 24 eyes that underwent vitreoretinal surgery, and four of these eyes became phthisical. There were no cases of endophthalmitis. The presence of open-globe injury and presenting visual acuity worse than 5/200 were significantly associated with poor visual outcome (<5/200, P<0.05). In eyes with open-globe injury, the presence of an IOFB was not associated with poor visual outcome (P>0.05). Conclusion Ocular injuries from IEDs are highly associated with severe ocular damage requiring extensive surgical repair or evisceration/enucleation. Postoperative PVR is a common cause of poor anatomical and visual outcome.
Erdurman, F C; Hurmeric, V; Gokce, G; Durukan, A H; Sobaci, G; Altinsoy, H I
This article briefly reviews the history and distribution of Onchocerca volvulus, and the disease for which it is responsible. The importance is stressed of ophthalmic examination, as opposed to clinical inspection, in determining the incidence of onchocerciasis in a region, such as the Sudan, where eye diseases are common. A description of the ocular manifestations of the infection is given, and their etiology is discussed.
Optical quality in barn owl eyes is presented in terms of measuring the ocular wavefront aberrations with a standard Tscherning-type wavefront aberrometer under natural viewing conditions. While accommodative state was uncontrolled, all eyes were focused within 0.4D with respect to the plane of the aberrometer. Total RMS wavefront error was between 0.06 and 0.15?m (mean: 0.10?m, STD: 0.03?m, defocus cancelled)
Wolf M. Harmening; Michael A. Vobig; Peter Walter; Hermann Wagner
Ocular allergy includes several clinical subtypes ranging from the mild seasonal allergic conjunctivitis to the potentially\\u000a sight-threatening atopic keratoconjunctivitis. Current therapies, particularly for the severe forms of disease, need to be\\u000a more localized and with fewer side effects. For this to be achieved, it requires a better understanding of the basic mechanisms\\u000a involved. In this chapter, recent findings are discussed
Virginia L. Calder; Peter M. Lackie
Diabetic retinopathy (DR) is a common complication of diabetes mellitus and a leading cause of new-onset vision loss in adults\\u000a worldwide. Current medical and surgical evidence-based care, including laser photocoagulation, is effective in preserving\\u000a vision. This care is most effective prior to the onset of ocular or visual symptoms, but many diabetic persons do not receive\\u000a the recommended annual eye
Paolo S. Silva; Jerry D. Cavallerano; Lloyd M. Aiello
The primary care physician is often called on to treat ocular emergencies. Two such injuries --chemical burns and traumatic central retinal artery occlusion --call for quick action and immediate referral. The evaluation of other injuries should proceed in logical order, beginning with the lids and proceeding to the pupils, globe, and retina and optic nerve. This systematic evaluation allows the examiner to identify patients who need referral to an ophthalmologist. Minor injuries can be safely treated by following the guidelines given. PMID:6401861
Born, C P
\\u000a The limitations of existing medical therapies for ocular disorders include low drug bioavailability, nonspecificity, side\\u000a effects, and poor treatment adherence to therapy. These limitations may be overcome through the use of sustained-release intraocular\\u000a drug delivery systems. Critical to the development of such systems has been the introduction of biocompatible polymers (biodegradable\\u000a and nonbiodegradable) that allow for drug release kinetics to
Susan S. Lee; Patrick Hughes; Aron D. Ross; Michael R. Robinson
ObjectiveLymphoproliferative lesions of the ocular adnexa were analyzed to examine (1) the suitability of the Revised European-American Lymphoma (REAL) classification for the subtyping of the lymphomas in these sites; (2) the predictive value of the REAL classification for the evolution of these tumors; and (3) the frequency and prognostic impact of tumor type, location, proliferation rate (Ki-67 index), p53, and
Sarah E Coupland; Lothar Krause; Henri-Jacques Delecluse; Ioannis Anagnostopoulos; Hans-Dieter Foss; Michael Hummel; Norbert Bornfeld; William R Lee; Harald Stein
Ocular inflammation is one of the leading causes of blindness and loss of vision. Human uveitis is a complex and heterogeneous group of diseases characterized by inflammation of intraocular tissues. The eye may be the only organ involved, or uveitis may be part of a systemic disease. A significant number of cases are of unknown etiology and are labeled idiopathic. Animal models have been developed to the study of the physiopathogenesis of autoimmune uveitis due to the difficulty in obtaining human eye inflamed tissues for experiments. Most of those models are induced by injection of specific photoreceptors proteins (e.g., S-antigen, interphotoreceptor retinoid-binding protein, rhodopsin, recoverin, phosducin). Non-retinal antigens, including melanin-associated proteins and myelin basic protein, are also good inducers of uveitis in animals. Understanding the basic mechanisms and pathogenesis of autoimmune ocular diseases are essential for the development of new treatment approaches and therapeutic agents. The present review describes the main experimental models of autoimmune ocular inflammatory diseases. PMID:22760810
Gasparin, Fabio; Takahashi, Beatriz Sayuri; Scolari, Mariana Ramos; Gasparin, Filipe; Pedral, Lycia Sampaio; Damico, Francisco Max
High-order monochromatic aberrations could potentially influence vision-dependent refractive development in a variety of ways. As a first step in understanding the effects of wave aberration on refractive development, we characterized the maturational changes that take place in the high-order aberrations of infant rhesus monkey eyes. Specifically, we compared the monochromatic wave aberrations of infant and adolescent animals and measured the longitudinal changes in the high-order aberrations of infant monkeys during the early period when emmetropization takes place. Our main findings were that (1) adolescent monkey eyes have excellent optical quality, exhibiting total RMS errors that were slightly better than those for adult human eyes that have the same numerical aperture and (2) shortly after birth, infant rhesus monkeys exhibited relatively larger magnitudes of high-order aberrations predominately spherical aberration, coma, and trefoil, which decreased rapidly to assume adolescent values by about 200 days of age. The results demonstrate that rhesus monkey eyes are a good model for studying the contribution of individual ocular components to the eye’s overall aberration structure, the mechanisms responsible for the improvements in optical quality that occur during early ocular development, and the effects of high-order aberrations on ocular growth and emmetropization.
Ramamirtham, Ramkumar; Kee, Chea-su; Hung, Li-Fang; Qiao-Grider, Ying; Roorda, Austin; Smith, Earl L.
Ocular fundus imaging plays a key role in monitoring the health status of the human eye. Currently, a large number of imaging modalities allow the assessment and/or quantification of ocular changes from a healthy status. This review focuses on the main digital fundus imaging modality, color fundus photography, with a brief overview of complementary techniques, such as fluorescein angiography. While focusing on two-dimensional color fundus photography, the authors address the evolution from nondigital to digital imaging and its impact on diagnosis. They also compare several studies performed along the transitional path of this technology. Retinal image processing and analysis, automated disease detection and identification of the stage of diabetic retinopathy (DR) are addressed as well. The authors emphasize the problems of image segmentation, focusing on the major landmark structures of the ocular fundus: the vascular network, optic disk and the fovea. Several proposed approaches for the automatic detection of signs of disease onset and progression, such as microaneurysms, are surveyed. A thorough comparison is conducted among different studies with regard to the number of eyes/subjects, imaging modality, fundus camera used, field of view and image resolution to identify the large variation in characteristics from one study to another. Similarly, the main features of the proposed classifications and algorithms for the automatic detection of DR are compared, thereby addressing computer-aided diagnosis and computer-aided detection for use in screening programs. PMID:21952522
Bernardes, Rui; Serranho, Pedro; Lobo, Conceição
The thin layer of tears covering the ocular surface are a complex body fluid containing thousands of molecules of varied form and function of several origins. In this review, we have discussed some key issues in the analysis of tears in the context of understanding and diagnosing eye disease using current technologies of proteomics and metabolomics, and for their potential for clinical application. In the last several years, advances in proteomics/metabolomics/lipidomics technologies have greatly expanded our knowledge of the chemical composition of tear fluid. The quickened pace of studies has shown that tears as a complex extra-cellular fluid of the ocular surface contains a great deal of molecular information useful for the diagnosis, prognosis, and treatment of ocular surface diseases that has the ability to addresses the emphasis on personalized medicine and biomarkers of disease. Future research directions will likely include (1) standardize tear collection, storage, extraction, and sample preparation; (2) quantitative proteomic analysis of tear proteins using multiple reaction monitoring (MRM)-based mass spectrometry; (3) population based studies of human tear proteomics/metabolomics; (4) tear proteomics/metabolomics for systemic diseases; and (5) functional studies of tear proteins. PMID:22732126
Zhou, Lei; Beuerman, Roger W
Objective To develop an animal model of intraocular tuberculosis (TB) with features of pulmonary TB and extrapulmonary dissemination to the eye. Methods Hartley strain guinea pigs were infected via an aerosol route with virulent Mycobacterium tuberculosis. One group of guinea pigs was infected with a relatively low bacterial inoculum and received no treatment. A second group of guinea pigs received high-dose infection and were treated with the first-line anti-TB drugs isoniazid, rifampin, and pyrazinamide. Development of ocular TB lesions was documented by histological analysis, acid-fast staining, and real-time polymerase chain reaction for M tuberculosis DNA. Results Untreated guinea pigs developed pulmonary and extrapulmonary TB. Ocular TB, primarily involving the uvea, developed in 5 of 12 eyes (42%). Uveal granulomatous lesions showed the presence of acid-fast organisms and M tuberculosis DNA. In treated animals, none of the 8 eyes examined revealed the presence of acid-fast organisms; however, there was mild nongranulomatous uveitis in 4 eyes. Conclusions Mycobacterium tuberculosis delivered via aerosol to guinea pigs results in extrapulmonary dissemination to the eye. Of significance, intraocular changes in this model include granulomatous inflammation and the presence of acid-fast organisms, as seen in human cases of ocular TB. Clinical Relevance The guinea pig model may provide greater insight into the pathogenesis of intraocular TB and assist in the development of novel modalities to treat this global infectious disease.
Rao, Narsing A.; Albini, Thomas A.; Kumaradas, Mirnalini; Pinn, Michael L.; Fraig, Mostafa M.; Karakousis, Petros C.
Ocular drug transport barriers pose a challenge for drug delivery comprising the ocular surface epithelium, the tear film and internal barriers of the blood-aqueous and blood-retina barriers. Ocular drug delivery efficiency depends on the barriers and the clearance from the choroidal, conjunctival vessels and lymphatic. Traditional drug administration reduces the clinical efficacy especially for poor water soluble molecules and for the posterior segment of the eye. Nanoparticles (NPs) have been designed to overcome the barriers, increase the drug penetration at the target site and prolong the drug levels by few internals of drug administrations in lower doses without any toxicity compared to the conventional eye drops. With the aid of high specificity and multifunctionality, DNA NPs can be resulted in higher transfection efficiency for gene therapy. NPs could target at cornea, retina and choroid by surficial applications and intravitreal injection. This review is concerned with recent findings and applications of NPs drug delivery systems for the treatment of different eye diseases.
Zhou, Hong-Yan; Hao, Ji-Long; Wang, Shuang; Zheng, Yu; Zhang, Wen-Song
Acute inflammation is a frequent, essential and beneficial response to maintain normal tissue function. PMN are the primary effector cells of acute inflammatory responses and their timely resolution by macrophages from an injured, stressed or infected tissues are required for the successful execution of this routine tissue response. Dysregulation of this fundamental program is a major factor in the global disease burden and contributes to many ocular diseases. Counter-regulatory signals are critical to the controlled activation of innate and adaptive immune responses in the eye and recent studies have identified two circuits in the cornea, uvea and/or retina, namely 15-lipoxygenase and heme-oxygenase, which control inflammation, promote resolution of PMN and afford neuroprotection. The role of these counter-regulator and pro-resolution circuits may provide insight into ocular inflammatory diseases and opportunities to restore stressed ocular tissue to a pre-inflammatory state, namely homeostasis, rather than limiting therapeutic options to palliative inhibition of pro-inflammatory circuits.
In a retrospective survey of patients taking medication for hyperlipidaemia, those taking niacin (nicotinic acid) were more likely (p < 0.05) to report sicca syndromes, blurred vision, eyelid oedema, and macular oedema compared with those who never took niacin. Additionally, 7% of those taking niacin discontinued the drug owing to adverse ocular side effects, while none of the other lipid lowering agents were found to cause these side effects (p = 0.016). Data from spontaneous reporting systems support a possible association of decreased vision, cystoid macular oedema, sicca-like symptoms, discoloration of the eyelids with or without periorbital or eyelid oedema, proptosis, loss of eyebrow or eyelashes, and superficial punctate keratitis with the use of niacin in high doses. Decreased vision may be marked, and if the drug is not discontinued, may progress to cystoid macular oedema. All ocular side effects listed above are reversible if the association with niacin is recognised and the drug is discontinued; both the incidence and severity of the ocular side effects seem to be dose dependent.
Fraunfelder, F W; Fraunfelder, F T; Illingworth, D R
Rhinosporidiosis, a granulomatous disease caused by Rhinosporidium seeberi, is widely prevalent in Sri Lanka and southern parts of India. Besides the eye, the lacrimal passage and nasal cavity are affected. The most common ocular involvement is a subconjunctival bleeding mass. Our case, a 9-year-old girl from a rural background with a history of pond water bathing, presented with a marginal chalazion involving the left lower lid. She had a history of chalazion involving the same site three months earlier which was managed by incision and curettage. A decision of surgical exploration was made and the evacuated mass was examined histologically showing evidence of rhinosporidiosis. This type of clinical presentation for ocular rhinosporidiosis (as recurrent chalazion) has not been reported earlier in the literature. This case emphasises that the clinicians from this part of the world must consider ocular rhinosporidiosis as differential diagnosis in cases with recurrent chalazion especially in a rural background with a habit of pond water bathing. PMID:22986579
Mukhopadhyay, Somnath; Shome, Subrata; Bar, Prasenjit Kumar; Chakrabarti, Amit; Mazumdar, Swati; De, Avisek; Sadhukhan, Kaushik; Bala, Bivas
The presence, frequency, and relationship of ocular and oral shedding of herpes simplex virus to previous herpes keratitis was investigated. This was to determine if a history of herpes keratitis predisposes to shedding of the herpes simplex virus into the tear film and/or mouth. Swabs were collected from the eyes and mouth of two groups of patients thrice weekly over a two- to four-month period. Group A comprised nine patients with a history of herpes labialis, group B 15 patients with a history of herpes keratitis. Herpes simplex virus type 1 was isolated from 1.33% of mouth specimens but was not identified in any of the ocular specimens. There was no significant difference between groups A and B in terms of ocular or oral shedding. Oral shedding appears to be independent of a previous herpes keratitis. The tear film is an unlikely source of virus in persons either with no history of herpes keratitis or between attacks in those patients with a history of previous herpes keratitis.
Kaye, S B; Madan, N; Dowd, T C; Hart, C A; McCarthy, K; Patterson, A
Ocular interferometry has potential value in a variety of ocular measurement applications, including measuring ocular thicknesses, topography of ocular surfaces or the wavefront of the eye. Of particular interest is using interferometry for characterizing corneal shape and irregular corneal features, making this technology attractive due to its inherent accuracy and spatial resolution. A particular challenge of designing an ocular interferometer is determining safe laser exposure levels to the eye, including both the retina and anterior segment. Described here are the laser exposure standards relevant in the interferometer design and the corresponding calculations and results. The results of this work can be used to aid in the design of similar laser-based systems for ocular evaluation.
Primeau, Brian C.; Goldstein, Goldie L.; Greivenkamp, John E.
There is no doubt that ocular migraine also known as retinal migraine or ophthalmic migraine should not to be confused with ophthalmoplegic migraine. The hallmark of ocular migraine is the unilateral visual loss or "monocular transient loss of vision" associated or followed by the headache. Better safe than sorry, therefore an ophthalmologic examination during the migraine attack is the most diagnostic method. age with typical history for ocular migraine. Importantly supportive data sustain that different neuro-ophthalmologic manifestations as amaurosis fugax, retinal vascular thrombosis and optic neuropathy, may be considered as the ocular hallmarks of the Hughes's syndrome. Clues for the evaluating of antiphospholipid antibodies include recurrent thrombosis especially in young people, recurrent fetal loss, and thrombocytopenia. There are no studies that focus exclusively on the prophylaxis of ocular migraine. Ocular features due to antiphospholipid antibodies - induced thrombosis or Hughes's syndrome should be treated with anticoagulant therapy. PMID:18064948
Coroi, Mihaela; Bontas, Ecaterina; Visan, Rodica; Defranceschi, Mladen; Cioranu, Cristiana Daniela
Abstract Research advancements in pharmaceutical sciences have led to the development of new strategies in drug delivery to anterior segment. Designing a new delivery system that can efficiently target the diseased anterior ocular tissue, generate high drug levels, and maintain prolonged and effective concentrations with no or minimal side effects is the major focus of current research. Drug delivery by traditional method of administration via topical dosing is impeded by ocular static and dynamic barriers. Various products have been introduced into the market that prolong drug retention in the precorneal pocket and to improve bioavailability. However, there is a need of a delivery system that can provide controlled release to treat chronic ocular diseases with a reduced dosing frequency without causing any visual disturbances. This review provides an overview of anterior ocular barriers along with strategies to overcome these ocular barriers and deliver therapeutic agents to the affected anterior ocular tissue with a special emphasis on nanotechnology-based drug delivery approaches.
Cholkar, Kishore; Patel, Sulabh P.; Vadlapudi, Aswani Dutt
There is an association between sarcoidosis and immune thrombocytopenia, which can be severe and even fatal if left untreated. Although ocular sarcoidosis is one of the most common extrapulmonary manifestations of sarcoidosis, immune thrombocytopenia has not been reported in isolated ocular sarcoidosis. We present a patient with a remote history of immune thrombocytopenic purpura who developed a sub-conjunctival mass consistent with isolated ocular sarcoidosis. PMID:24144263
Chen, John J; Allen, Richard C
Background Since there has been no conclusive evidence regarding the treatment of ocular myasthenia, treatment guidelines were recently issued by the European Federation of Neurological Societies/European Neurological Society (EFNS/ENS). However, the therapeutic outcomes concerning the quality-of-life (QOL) of patients with ocular myasthenia are not yet fully understood. Methods We investigated the therapeutic outcomes of patients with purely ocular myasthenia in a multicenter cross-sectional survey in Japan. To evaluate the severity of ocular symptoms, we used the ocular-quantitative MG (QMG) score advocated by Myasthenia Gravis Foundation of America. We used the Japanese translated version of the MG-QOL15, a self-appraised scoring system. Results Of 607 myasthenia gravis (MG) patients with an observation-duration of illness???2?years, the cases of 123 patients (20%) were limited to ocular muscles (purely ocular myasthenia). During the entire clinical course, 81 patients experienced both ptosis and diplopia, 36 had ptosis alone, and six had diplopia alone. Acetyl-cholinesterase inhibitors and prednisolone were used in 98 and 52 patients, respectively. Treatment improved ocular symptoms, with the mean reduction in ocular-QMG score of 2.3?±?1.8 points. However, 47 patients (38%) failed to gain minimal manifestation or a better status. Patients with unfavorable outcomes also self-reported severe QOL impairment. Multivariate analyses showed that the pretreatment ocular-QMG score was associated with unfavorable outcomes, but not associated with the patient’s QOL. Conclusion A treatment strategy designed in accord with a patient's ocular presentation must be considered in order to improve ocular symptoms and the patient's QOL.
Introduction. The objective of this work was to demonstrate a high spectral and spatial resolution fundus imager and to assess its utility in visualizing and characterizing normal anatomical and pathological tissue classes in the human ocular fundus. The ocular fundus (posterior portion of the eye) affords a unique opportunity to directly observe neural and vascular tissue in vivo. Many ocular and systemic diseases manifest changes in the normal fundus anatomy. Current examination techniques are not optimized to detect changes prior to the formation of damaging lesions. Spectral imaging may allow visualization of disease states before the onset of traditional clinical signs. Normal tissue in the eye has distinct spectral characteristics determined by specific structural organization and the presence of specific chemical substances and ocular pigments. Pathological states result in physical and chemical changes to the tissue. Spectral imaging exploits the differences in the spectral characteristics to separate different classes of material. When these spectral properties are combined with the spatial context of the image, improved visualization and detection is possible. Methods. Two independent spectral imaging devices were developed and integrated to a commercially available Zeiss fundus camera. Spectral data were collected in order to characterize the normal anatomical tissue classes and to assess the usefulness of spectral features for improved class discernment. Spectral images were collected for 14 subjects Diabetic Retinopathy were imaged. Mean spectral curves were produced for each class and for each subject. These spectral curves were normalized to remove the contribution from the pigment melanin (the major pigment associated with variation in fundus pigmentation) and modeled with a piece-wise linear function consisting of a DC offset and four slopes. Results. Differences in the shape of the spectral curve exist between macular edema and normal macular and between types of drusen. The mean spectral curves of the normal anatomical tissue showed a strong correlation with fundus pigmentation for classes greatly affected by melanin with significantly less correlation for classes not affected by melanin. Inter-subject variability was greatly reduced in the melanin-normalized curves. The simple piece-wise linear model captured the first order shape properties of the spectral curves and the first three principle components of the model coefficients produced good and consistent class separation. Conclusion. High-resolution spectral imaging of the ocular fundus produces features that are useful in separating fundus tissue classes for classification and visualization. Images at wavelengths in excess of 600 nm allow non-invasive visualization of the important choroidal circulation. The data further suggest spectral features that can be used to detect macular edema pre-clinically and spectral features that can be used to detect macular edema pre-clinically and quantitatively classify drusen sub-types.
Truitt, Paul Wiley
The eye movement response to earth vertical axis rotation in the dark, a semicircular canal stimulus, can be altered by prior exposure to combined visual-vestibular stimuli. Such plasticity of the vestibulo-ocular reflex has not been described for earth horizontal axis rotation, a dynamic otolith stimulus. Twenty normal human subjects underwent one of two types of adaptation paradigms designed either to attenuate or enhance the gain of the semicircular canal-ocular reflex prior to undergoing otolith-ocular reflex testing with horizontal axis rotation. The adaptation paradigm paired a 0.2 Hz sinusoidal rotation about a vertical axis with a 0.2 Hz optokinetic stripe pattern that was deliberately mismatched in peak velocity. Pre- and post-adaptation horizontal axis rotations were at 60 degrees/s in the dark and produced a modulation in the slow component velocity of nystagmus having a frequency of 0.17 Hz due to putative stimulation of the otolith organs. Results showed that the magnitude of this modulation component response was altered in a manner similar to the alteration in semicircular canal-ocular responses. These results suggest that physiologic alteration of the vestibulo-ocular reflex using deliberately mismatched visual and semicircular canal stimuli induces changes in both canal-ocular and otolith-ocular responses. We postulate, therefore, that central nervous system pathways responsible for controlling the gains of canal-ocular and otolith-ocular reflexes are shared.
Wall, C. 3rd; Smith, T. R.; Furman, J. M.
The eye movement response to earth vertical axis rotation in the dark, a semicircular canal stimulus, can be altered by prior exposure to combined visual-vestibular stimuli. Such plasticity of the vestibulo-ocular reflex has not been described for earth horizontal axis rotation, a dynamic otolith stimulus. Twenty normal human subjects underwent one of two types of adaptation paradigms designed either to attenuate or enhance the gain of the semicircular canal-ocular reflex prior to undergoing otolith-ocular reflex testing with horizontal axis rotation. The adaptation paradigm paired a 0.2 Hz sinusoidal rotation about a vertical axis with a 0.2 Hz optokinetic stripe pattern that was deliberately mismatched in peak velocity. Pre- and post-adaptation horizontal axis rotations were at 60 degrees/s in the dark and produced a modulation in the slow component velocity of nystagmus having a frequency of 0.17 Hz due to putative stimulation of the otolith organs. Results showed that the magnitude of this modulation component response was altered in a manner similar to the alteration in semicircular canal-ocular responses. These results suggest that physiologic alteration of the vestibulo-ocular reflex using deliberately mismatched visual and semicircular canal stimuli induces changes in both canal-ocular and otolith-ocular responses. We postulate, therefore, that central nervous system pathways responsible for controlling the gains of canal-ocular and otolith-ocular reflexes are shared. PMID:1441982
Wall, C; Smith, T R; Furman, J M
This is the first study of internal ocular shell structures of Cretaceous ostracodes. Internal molds from eight species of four genera from Santonian, Campanian, and Maastrichtian strata revealed a stalked ocular sinus constricted along its length and distally inflated. The latter part has anterior and posterior lobes and a subcentral concavity. These Cretaceous trachyleberids have ocular shell structures similar to Cenozoic taxa and it is presumed they functioned similarly. However, each taxon has a diagnostic ocular sinus and an inner surface of the eye tubercle that distinguishes each from all other forms examined.
Kontrovitz, M.; Puckett, T. M.
Vision threatening ocular inflammation may occur in patients with any of the acquired connective tissue disorders and vasculitic diseases. Additionally, the ocular inflammation may be the presenting manifestation of the disease, which leads the patient to seek medical care. Other manifestations of the potentially lethal disease may be subtle or absent, presenting the thoughtful ophthalmologist with the opportunity to make life saving discoveries. Necrotizing scleritis, peripheral ulcerative keratitis, and retinal vasculitis are the ocular findings which should prompt the ophthalmologist to initiate very aggressive measures aimed at discovering any evidence of extra-ocular abnormalities, laboratory or otherwise. Appropriate therapy will be sight saving and may be life saving. PMID:23688612
Foster, C Stephen
The current study investigates the role of regulatory T (Treg) cells in the pathogenesis of ocular attack in patients with\\u000a Behcet’s disease (BD). Nineteen BD patients with ocular complications (BDo), including 11 BD patients with ocular attack (BDa)\\u000a and eight BD patients with inactive ocular complications (BDi), were studied. Four BD patients without ocular complications\\u000a (BDwo) were also evaluated as
Yuki Nanke; Shigeru Kotake; Mari Goto; Hiroshi Ujihara; Masao Matsubara; Naoyuki Kamatani
Purpose. Fibrillin-2 (Fbn2) is the dominant fibrillin isoform expressed during development of the mouse eye. To test its role in morphogenesis, we examined the ocular phenotype of Fbn2?/? mice. Methods. Ocular morphology was assessed by confocal microscopy using antibodies against microfibril components. Results. Fbn2?/? mice had a high incidence of anterior segment dysgenesis. The iris was the most commonly affected tissue. Complete iridal coloboma was present in 37% of eyes. Dyscoria, corectopia and pseudopolycoria were also common (43% combined incidence). In wild-type (WT) mice, fibrillin-2-rich microfibrils are prominent in the pupillary membrane (PM) during development. In Fbn2-null mice, the absence of Fbn2 was partially compensated for by increased expression of fibrillin-1, although the resulting PM microfibrils were disorganized, compared with WTs. In colobomatous adult Fbn2?/? eyes, the PM failed to regress normally, especially beneath the notched region of the iris. Segments of the ciliary body were hypoplastic, and zonular fibers, although relatively plentiful, were unevenly distributed around the lens equator. In regions where the zonular fibers were particularly disturbed, the synchronous differentiation of the underlying lens fiber cells was affected. Conclusions. Fbn2 has an indispensable role in ocular morphogenesis in mice. The high incidence of iris coloboma in Fbn2-null animals implies a previously unsuspected role in optic fissure closure. The observation that fiber cell differentiation was disturbed in Fbn2?/? mice raises the possibility that the attachment of zonular fibers to the lens surface may help specify the equatorial margin of the lens epithelium.
Shi, Yanrong; Tu, Yidong; Mecham, Robert P.; Bassnett, Steven
To date, sildenafil citrate (Viagra) gives every evidence of being a safe drug for the eye despite a series of expressed concerns. A review of how its ocular safety profile has been identified offers insights into the strengths and weaknesses of present systems and resources for judging the ocular safety of Viagra or, for that matter, of any new drug. Such insights include: The great value of careful, informed assessment of preclinical information gleaned from laboratory experiments. By and large, such assessments point the way toward appropriate clinical evaluation. For Viagra, early in its development it was noted that besides exerting a major inhibitory effect on the intended target, the vascular-associated enzyme phosphodiesterase 5 (PDE5), the drug also exerts a lesser but definite inhibitory effect on the closely related PDE6, located in the retina. For this reason, preclinical evaluation of the drug included electroretinography plus postmortem histology. In addition, an extended eye examination was incorporated into clinical protocols. The often chaotic but invaluable information stream that becomes available once marketing approval has been gained and large populations begin to use a drug. False alarms, misattribution, and erroneous information are the order of the day. Nevertheless, as information accumulates, patterns of response clarify and the true nature of special susceptibility for subpopulations, if any, becomes apparent. A role for the astute clinician remains: Subtle changes or unusual risks for subpopulations can be missed entirely for long periods of time. A manifest need for improvement in evaluation of postmarketing side-effects. This need has led to the establishment of a new discipline: pharmacoepidemiology. In ophthalmology, the National Registry of Drug Induced Ocular Side-Effects maintains a constant and invaluable surveillance. Examples are supplied to illustrate each of these major points: Our presentation will include data gleaned from clinical trials plus postmarketing information on the incidence, duration, and type of color vision defects observed at different doses of Viagra.
Laties, A M; Fraunfelder, F T
OBJECTIVE: To evaluate the frequency and characteristics of ocular manifestations in outpatients with systemic sclerosis. METHODS: In this cross-sectional study, 45 patients with systemic sclerosis were enrolled. Data regarding demographics, disease duration and subtype, age at diagnosis, nailfold capillaroscopic pattern and autoantibody profile were collected, and a full ophthalmic examination was conducted. Parametric (Student's t-test) and nonparametric (Mann-Whitney U test) tests were used to compare continuous variables. Fisher's exact test was used to compare categorical data. P values < 0.05 were considered significant. RESULTS: Twenty-three subjects (51.1%) had eyelid skin changes; 22 (48.9%) had keratoconjunctivitis sicca, 19 (42.2%) had cataracts, 13 (28.9%) had retinal microvascular abnormalities and 6 (13.3%) had glaucoma. Eyelid skin changes were more frequent in patients with the diffuse subtype of systemic sclerosis and were associated with a younger age and an earlier age at diagnosis. Cataracts were presumed to be age-related and secondary to corticosteroid treatment. There was no association between demographic, clinical or serological data and keratoconjunctivitis sicca. The retinal microvascular abnormalities were indistinguishable from those related to systemic hypertension and were associated with an older age and a severe capillaroscopic pattern. CONCLUSIONS: Eyelid skin abnormalities and keratoconjunctivitis sicca were the most common ocular findings related to systemic sclerosis. Some demographic and clinical data were associated with some ophthalmic features and not with others, showing that the ocular manifestations of systemic sclerosis are characterized by heterogeneity and reflect the differences in the implicated pathophysiological mechanisms.
de A. F. Gomes, Beatriz; Santhiago, Marcony R; Magalhaes, Priscilla; Kara-Junior, Newton; de Azevedo, Mario N L; Moraes, Haroldo V
AIMS—To evaluate the efficacy of amniotic membrane transplantation (AMT) for ocular surface reconstruction.?METHODS—10 consecutive patients who underwent AMT were included. The indications were: group A, cases with persistent epithelial defect after corneal abscess (n=1), radiation (n=1), or chemical burn (n=3); group B, cases with epithelial defect and severe stromal thinning and impending or recent perforation, due to chemical burn (two patients, three eyes) or corneal abscess (n=2); group C, to promote corneal epithelium healing and prevent scarring after symblepharon surgery with extensive corneo-conjunctival adhesion (n=1). Under sterile conditions amniotic membrane was prepared from a fresh placenta of a seronegative pregnant woman and stored at ?70°C. This technique involved the use of amniotic membrane to cover the entire cornea and perilimbal area in groups A and B, and the epithelial defect only in group C.?RESULTS—The cornea healed satisfactorily in four of five patients in group A, but the epithelial defect recurred in one of these patients. After AMT three patients underwent limbal transplantation and one penetrating keratoplasty and cataract extraction. In group B amniotic membrane transplantation was not helpful, and all cases underwent an urgent tectonic corneal graft. Surgery successfully released the symblepharon, promoted epithelialisation and prevented adhesions in the case of group C.?CONCLUSION—AMT was effective to promote corneal healing in patients with persistent epithelial defect, and appeared to be helpful after surgery to release corneo-conjunctival adhesion. Most cases required further surgery for visual and ocular surface rehabilitation. Amniotic membrane used as a patch was not effective to prevent tectonic corneal graft in cases with severe stromal thinning and impending or recent perforation.?? Keywords: amniotic membrane; cornea; ocular surface; epithelial defect
Azuara-Blanco, A.; Pillai, C; Dua, H.
Purpose. Parstatin is a 41-mer peptide formed by proteolytic cleavage on activation of the PAR1 receptor. The authors recently showed that parstatin is a potent inhibitor of angiogenesis. The purpose of the present study was to evaluate the therapeutic effect of parstatin on ocular neovascularization. Methods. Choroidal neovascularization was generated in mice using laser-induced rupture of Bruch's membrane and was assessed after 14 days after perfusion of FITC-dextran. Oxygen-induced retinal neovascularization was established in neonatal mice by exposing them to 75% O2 at postnatal day (P)7 for 5 days and then placing them in room air for 5 days. Evaluation was performed on P17 after staining with anti-mouse PECAM-1. The effect of parstatin was tested after intravitreal administration. The effects of subconjunctival-injected parstatin on corneal neovascularization and inflammation in rats were assessed 7 days after chemical burn-induced corneal neovascularization. Retinal leukostasis in mice was assessed after perfusion with FITC-conjugated concanavalin A. Results. Parstatin potently inhibited choroidal neovascularization with an IC50 of approximately 3 ?g and a maximum inhibition of 59% at 10 ?g. Parstatin suppressed retinal neovascularization with maximum inhibition of 60% at 3 ?g. Ten-microgram and 30-?g doses appeared to be toxic to the neonatal retina. Subconjunctival parstatin inhibited corneal neovascularization, with 200 ?g the most effective dose (59% inhibition). In addition, parstatin significantly inhibited corneal inflammation and VEGF-induced retinal leukostasis. In all models tested, scrambled parstatin was without any significant effect. Conclusions. Parstatin is a potent antiangiogenic agent of ocular neovascularization and may have clinical potential in the treatment of angiogenesis-related ocular disorders.
Huang, Hu; Vasilakis, Panagiotis; Zhong, Xiufeng; Shen, Ji-Kui; Geronatsiou, Katerina; Papadaki, Helen; Maragoudakis, Michael E.; Gartaganis, Sotirios P.; Vinores, Stanley A.
Twenty-five patients were examined for ocular complaints following renal transplantation. Besides the expected complications of posterior subcapsular cataract and cytomegalovirus retinitis, other findings—such as focal depigmentation of the retinal pigment epithelium, a lack of hypertensive retinopathy, elevated intraocular tensions, microaneurysms, preretinal wrinkling, serous detachments of the retina, hemorrhages and exudates—were observed. A laboratory clue to the onset of cytomegalovirus retinitis was a rapid rise in cytomegalovirus (CMV) antibody titer and a positive CMV plaque count in tissue culture. ImagesFigure 1.Figure 2.
Oberman, Arthur E.; Chatterjee, Satya N.
We report the case of a 43 year old man who presented recurrent left abducens palsy. His medical history included arterial hypertension, ischemic cardiomiopathy, dyslipidemia, rhinitis, maxillary sinusitis. Physical examination revealed a overweight patient, horizontal gaze diplopia, left nerve VI paresis, mild left retro-orbital pain. The orbital MRI also didn’t offer new information: mild external edema on the left eye, with normal tendon aspect, no thickening or enhancement of the muscle belly and also normal aspect of the bony orbit. Recurrent palsy of EOMs can be caused in rare cases by ocular myositis.
ALBU, CARMEN VALERIA; CATALIN, B.; ZAHARIA, CORNELIA
Increasingly accurate phenotyping leads to better genetic evaluation. Genetic eye conditions may be due to a common cellar defect (eg, ciliopathies or RASopathies). Early-onset retinal dystrophies may be associated with renal disease. An understanding of genetic testing helps clinicians identify shortcomings in testing which may lead to a better understanding of the most appropriate test for a given ocular condition. Dedicated genetic counselors within ophthalmic and pediatric clinics are likely to improve the delivery of clinical care in these settings. PMID:24852152
Scanga, Hannah L; Nischal, Ken K
Optical quality in barn owl eyes is presented in terms of measuring the ocular wavefront aberrations with a standard Tscherning-type wavefront aberrometer under natural viewing conditions. While accommodative state was uncontrolled, all eyes were focused within 0.4D with respect to the plane of the aberrometer. Total RMS wavefront error was between 0.06 and 0.15 microm (mean: 0.10 microm, STD: 0.03 microm, defocus cancelled) for a 6 mm pupil. The results suggest that image quality in barn owl eyes is excellent. PMID:17845811
Harmening, Wolf M; Vobig, Michael A; Walter, Peter; Wagner, Hermann
Background Blank cartridge guns are generally regarded as being harmless and relative safe. However recent published articles demonstrated that the gas pressure from the exploding propellant of blank cartridge is powerful enough to penetrate the thoracic wall, abdominal muscle, small intestine and the skull. And there has been a limited number of case reports of ocular trauma associated with blank cartridge injury. In addition, no report on case with split extraocular muscle injury with traumatic cataract and penetrating corneoscleral wound associated with blank cartridge has been previously documented. This report describes the case of patient who sustained penetrating ocular injury with extraocular muscle injury by a close-distance blank cartridge that required surgical intervention. Case presentation A 20-year-old man sustained a penetrating globe injury in the right eye while cleaning a blank cartridge pistol. His uncorrected visual acuity at presentation was hand motion and he had a flame burn of his right upper and lower lid with multiple missile wounds. On slit-lamp examination, there was a 12-mm laceration of conjunctiva along the 9 o'clock position with two pinhole-like penetrating injuries of cornea and sclera. There was also a 3-mm corneal laceration between 9 o'clock and 12 o'clock and the exposed lateral rectus muscle was split. Severe Descemet's membrane folding with stromal edema was observed, and numerous yellow, powder-like foreign bodies were impacted in the cornea. Layered anterior chamber bleeding with traumatic cataract was also noted. Transverse view of ultrasonography showed hyperechoic foreign bodies with mild reduplication echoes and shadowing. However, a computed tomographic scan using thin section did not reveal a radiopaque foreign body within the right globe. Conclusion To our best knowledge, this is the first case report of split extraocular muscle injury with traumatic cataract and penetrating ocular injury caused by blank cartridge injury. Intraocular foreign bodies undetectable by CT were identified by B-scan ultrasonography in our patient. This case highlights the importance of additional ultrasonography when evaluating severe ocular trauma. And ophthalmologists should consider the possibility of penetrating injury caused by blank ammunition.
A high incidence of ocular rhinosporidiosis in Kanyakumari district of Tamil Nadu, India is reported. Among the four taluks (sub-division for administration), highest occurrence was found in Agastheeswaram taluk (51.2%) followed by Kalkulam (24.4%), Thovalai (22%) and Vilavancode (2.4%). The disease occurred among both sexes equally and preponderance of a particular sex was not observed. The young adolescents were found to be more susceptible. The disease was found in all socio-economic strata and among all communities and persons belonging to different religious groups. Most of the patients gave a history of bathing in muddy stagnant pools of water. PMID:2233980
Moses, J S; Balachandran, C; Sandhanam, S; Ratnasamy, N; Thanappan, S; Rajaswar, J; Moses, D
Background Interferon alpha, used in the treatment of different viral, autoimmune and malignant diseases, is known to induce a variety\\u000a of side effects. Recently, induction of sarcoidosis during interferon therapy has been reported. We analyzed patients for\\u000a uveitis, possibly induced by interferon alpha.\\u000a \\u000a \\u000a \\u000a Methods We report on three patients who had developed typical signs of ocular sarcoidosis under treatment with interferon alpha
Deshka Doycheva; Christoph Deuter; Nicole Stuebiger; Manfred Zierhut
The development of ocular dominance columns has served as a Rosetta stone for understanding the mechanisms that guide the construction of cortical circuits. Traditionally, the emergence of ocular dominance columns was thought to be closely tied to the critical period, during which columnar architecture is highly susceptible to alterations in visual input. However, recent findings in cats, monkeys and ferrets
Justin C. Crowley; Lawrence C. Katz
This study was performed to assess the in vivo ocular toxicity of benzalkonium chloride (BAK) homologs compared with commercially available BAK (BAK mixture) and to assess the ocular toxicity of BAK homolog after repeated ocular application. Rabbit eyes were examined by ophthalmology and scanning electron microscopy (SEM) after 10 applications of BAK homologs with C12 (C12-BAK) and C14 (C14-BAK) alkyl chain lengths and a BAK mixture at concentrations of 0.001% (w/v), 0.003% (w/v), 0.005% (w/v), 0.01% (w/v) and 0.03% (w/v). The ocular toxicity of C12-BAK to rabbit eyes was examined by ophthalmology and histopathology after repeated ocular application for 39 weeks. In addition, the antimicrobial activities of C12-BAK and C14-BAK against A. niger, S. aureus and P. aeruginosa were assessed. Ocular toxicity of C12-BAK was less than those of the BAK mixture and C14-BAK. No ocular toxicity was noted after ocular application of 0.01% C12-BAK to rabbits for 39 weeks. C12-BAK showed antimicrobial activities at a concentration of 0.003%. These results suggest that the use of C12-BAK to replace BAK mixture as a preservative in ophthalmic solutions should be considered in order to reduce the incidence of the corneal epithelial cell injury induced clinically by BAK.
Okahara, Akihiko; Tanioka, Hidetoshi; Takada, Koichi; Kawazu, Kouichi
PURPOSE. Ocular melanin synthesis modulates rod photorecep- tor production, because in albino eyes, rod numbers are re- duced by approximately 30%. In this study, rod numbers and ocular rhodopsin concentrations were measured in intermedi- ate pigmentation phenotypes to determine whether propor- tional reductions in melanin are correlated with proportional changes in rod numbers. Further, patterns of cell production and death
Philippe Donatien; Glen Jeffery
Delivery of drugs into eyes using conventional drug delivery systems, such as solutions, is a considerable challenge to the treatment of ocular diseases. Drug loss from the ocular surface by lachrymal fluid secretion, lachrymal fluid-eye barriers, and blood-ocular barriers are main obstacles. A number of ophthalmic drug delivery carriers have been made to improve the bioavailability and to prolong the residence time of drugs applied topically onto the eye. The potential use of microemulsions as an ocular drug delivery carrier offers several favorable pharmaceutical and biopharmaceutical properties such as their excellent thermodynamic stability, phase transition to liquid-crystal state, very low surface tension, and small droplet size, which may result in improved ocular drug retention, extended duration of action, high ocular absorption, and permeation of loaded drugs. Further, both lipophilic and hydrophilic characteristics are present in microemulsions, so that the loaded drugs can diffuse passively as well get significantly partitioned in the variable lipophilic-hydrophilic corneal barrier. This review will provide an insight into previous studies on microemulsions for ocular delivery of drugs using various nonionic surfactants, cosurfactants, and associated irritation potential on the ocular surface. The reported in vivo experiments have shown a delayed effect of drug incorporated in microemulsion and an increase in the corneal permeation of the drug.
Hegde, Rahul Rama; Verma, Anurag; Ghosh, Amitava
All major studies on human ocular absorption (the macula not included) are gathered. From every paper important data like wavelength interval, age, and a figure of the ocular absorption curve are copied. After a discussion on the age effect a curve is pro...
Tuberculosis and sarcoidosis share similarity in histopathologic findings and clinically occur in association with each other occasionally. Tuberculosis should always be ruled out before the diagnosis of sarcoidosis. But, the diagnosis is often complicated, especially in extrapulmonary cases. Here we present a case of bilateral vitreous hemorrhage with uveitis. Ocular sarcoidosis was initially diagnosed based on the characteristic ocular findings, negative results on chest radiography, tuberculosis culture, and polymerase chain reaction of aqueous, as well as simultaneous presence of panda and lambda sign on gallium-67 scans. The ocular condition improved after pars plana vitrectomy and systemic steroid therapy. However, TB lymphadenopathy but no recurrent ocular inflammation was found 6 years later. The patient received anti-TB treatment for 6 months thereafter. The eyes remained silent except cataract progression and glaucoma under two medications during this period. In conclusion, TB could occur coincidently or in association with sarcoidosis, continued follow-up is important for patients with ocular sarcoidosis. PMID:21484177
Lin, Jiun-Yo; Sheu, Shwu-Jiuan
BACKGROUND/AIMS—Although human ocular toxocariasis causes severe vision defect, little is known about its aetiology, diagnosis, and treatment. To develop a new animal model for human ocular toxocariasis, ophthalmological findings of fundi in Mongolian gerbils, Meriones unguiculatus, and BALB/c mice were investigated following infection with Toxocara canis.?METHODS—Using an ophthalmoscope, which was specifically developed to observe the fundi of small animals, ocular changes of fundi of 20 gerbils and 11 mice were monitored after oral infection with embryonated eggs of T canis.?RESULTS—Vitreous, choroidal, and retinal haemorrhages were consistently observed in Mongolian gerbils, but rarely in mice. Severe exudative lesions and vasculitis were often present in gerbils but not in mice. Migrating larvae were also frequently observed in gerbils.?CONCLUSION—Mongolian gerbils are more appropriate animal model for human ocular toxocariasis than previously used experimental animal such as mice, guinea pigs, rabbits, and monkeys because of its high susceptibility of ocular infection.??
Takayanagi, T. H.; Akao, N.; Suzuki, R.; Tomoda, M.; Tsukidate, S.; Fujita, K.
Although parasite-mediated host cell lysis is deemed to be an important cause of tissue destruction in ocular toxoplasmosis (OT), the severity of the disease is probably correlated with hypersensitivity and inflammation. Notwithstanding, the mechanisms that regulate the inflammatory process in recurrent OT are poorly understood. Recent evidence has identified interleukin (IL) 17 as a marker for disease severity. The ocular and cerebral presence of this cytokine is generally associated with the induction of autoimmune responses in the brain and the eye. Indeed, there are indications that autoimmunity may contribute to clinical variability in the activity of OT. IL-23, which induces the proliferation of IL-17-producing cells and IL-27, which is a counterplayer to IL-17, may regulate T(H)-1-cell-mediated responses in OT. The importance of these cytokines in experimental models of uveitis and encephalitis has been recently reported. CD25(+) regulatory T-cells may control the local inflammatory response and protect the host against collateral inflammatory tissue damage. The responses of these cells to OT may be suitably tailored to cope with either an acquired or a congenital aetiology. Knowledge relating to immunoreactivity in OT has grown impressively during the past few years. Its characteristic and variable features have been identified and the potential relevance of autoimmunity has been assessed. In light of this knowledge, potential future treatment options have been considered. PMID:19430646
Garweg, Justus G; Candolfi, Ermanno
To investigate the role of cellular immunodeficiency in recurrent toxoplasmic retinochoroiditis, six Cynomolgus monkeys (Macaca fascicularis) with healed toxoplasmic lesions of the retina were immunosuppressed by total lymphoid irradiation. Three months prior to irradiation 30,000 Toxoplasma gondii organisms of the Beverley strain had been inoculated onto the macula of eye in each monkey via a pars plana approach. Toxoplasmic retinochoroiditis developed in each animal, and lesions were allowed to heal without treatment. During total lymphoid irradiation animals received 2000 centigrays (cGy) over a 7-week period. Irradiation resulted in an immediate drop in total lymphocyte counts and decreased ability to stimulate lymphocytes by phytohemagglutinin. Weekly ophthalmoscopic examinations following irradiation failed to show evidence of recurrent ocular disease despite persistent immunodeficiency. Four months after irradiation live organisms were reinoculated onto the nasal retina of the same eye in each animal. Retinochoroidal lesions identical to those seen in primary disease developed in five of six animals. Toxoplasma organisms therefore were able to proliferate in ocular tissue following the administration of immunosuppressive therapy. This study fails to support the hypothesis that cellular immunodeficiency alone will initiate recurrent toxoplasmic retinochoroiditis. Results suggest that reactivation of disease from encysted organisms involves factors other than suppression of Toxoplasma proliferation. If reactivation occurs by other mechanisms, however, cellular immunodeficiency then may allow development of extensive disease.
Holland, G.N.; O'Connor, G.R.; Diaz, R.F.; Minasi, P.; Wara, W.M.
There have been many calls for preventive action against injuries caused by air-guns. Eye injuries are particularly serious, and we conducted a retrospective study to review their characteristics. Ophthalmic consultants in the south-west region of England and South Wales were asked to recollect any injuries that had resulted from air weapons. Information was recorded on the nature and circumstances of the injury, subsequent management, and long-term effects. 19 cases of ocular injury were identified, sustained at mean age 19.7 years, all in the past decade. 15 of the victims were male. At least 12 shootings were accidental but 2 were deliberate. The injury was self-inflicted in 5 cases, and in 6 the assailant was known to the victim. 9 sustained ruptured globes and 8 had severe contusions. Ultimately 4 individuals required enucleation and 2 evisceration. At last review, visual acuity was no perception of light in 10 (53%) and "counting fingers" or worse in 16. Victims spent an average of nearly 10 days as an inpatient. The characteristics of the incidents that lead to ocular air-weapon injuries are unchanged. Reform of the firearms laws is probably the best way to prevention. PMID:11461983
Shuttleworth, G N; Galloway, P H
There have been many calls for preventive action against injuries caused by air-guns. Eye injuries are particularly serious, and we conducted a retrospective study to review their characteristics. Ophthalmic consultants in the south-west region of England and South Wales were asked to recollect any injuries that had resulted from air weapons. Information was recorded on the nature and circumstances of the injury, subsequent management, and long-term effects. 19 cases of ocular injury were identified, sustained at mean age 19.7 years, all in the past decade. 15 of the victims were male. At least 12 shootings were accidental but 2 were deliberate. The injury was self-inflicted in 5 cases, and in 6 the assailant was known to the victim. 9 sustained ruptured globes and 8 had severe contusions. Ultimately 4 individuals required enucleation and 2 evisceration. At last review, visual acuity was no perception of light in 10 (53%) and ‘counting fingers’ or worse in 16. Victims spent an average of nearly 10 days as an inpatient. The characteristics of the incidents that lead to ocular air-weapon injuries are unchanged. Reform of the firearms laws is probably the best way to prevention.
Shuttleworth, G N; Galloway, P H
Aim. The aim of this study was to evaluate if patients with Behcet's disease who have ocular involvement have a more severe form of this disease as compared to patients with Behcet's disease alone. Methods. A total of 99 patients were included in the study. 76 patients were used as part of the examined group, and 23 patients formed a control group. Results. The following are the results of examined and control groups, respectively: recurrent oral aphthous ulcers 89.5%, 95.7%; genital ulcers 61.8%, 97.0%; articular involvement 72.4%, 65.2%; vasculitis 81.6%, 60.9%; positive pathergy test 25.0%, 47.8%. Higher frequency of genital ulcerations was noted in control group (P = 0.001). More than two major criteria were met in 100% of the cases. HLA B51 was present in 78.9% of the cases in the examined group and 43.5% of the cases in control group; thus there is significant difference between them (P = 0.001). Visual acuity >0.5 occurred in 76% (examined group). Most frequent ocular manifestations in the examined group were retinal periphlebitis 81.6%, periphlebitis and periarteritis 65%, and serofibrinous uveitis 63.2%. Macular edema as a complication was present in 63.2%. The majority of patients (55.3%) were treated with combined therapy consisting of cyclosporine A and systemic corticosteroids. In 38.2% of patients, laser photocoagulation was used on retinal periphery.
Ocular toxocariasis represents an organ manifestation of the systemic infection with the Nematode larva Toxocara canis. The infection can provoke very different changes in the eye. Especially posterior uveitis, vitreous body infiltrates, epiretinal membranes and suberetinal granulomas can occur. The diagnosis of these changes can be difficult due to the variety of the symptoms and on the other side by the absence of characteristic specific changes. Moreover unclear findings can occur when only serological examinations are performed. In unclear cases the antibody-detection by means of ELISA from the vitreous body fluid can be useful. The examination of vitreous body material can prove toxocara infection when no systemic signs of infection are present and no antibodies are detectable in the serum. Besides the systemic anti-helminthic therapy with Albendazol the pars plana vitrectomy is a substantial therapeutic option in ocular toxocariasis when vitreo-retinal complications are present. A well timed adequate therapy can stabilize the visual acuity in many cases. PMID:17219180
Bertelmann, E; Velhagen, K-H; Pleyer, U
Eyes of cutaneous melanoma-bearing miniature Sinclair swine were examined by light and electron microscopy during growth and maturation of animals. The histology of ocular depigmentation, which occurs during melanoma arrest and skin depigmentation, was studied in eyes of animals that had successful and unsuccessful tumor regression. In 12 animals one eye was removed at an early stage and the second eye at a later stage of disease or maturation. The histologic and clinical course of the ocular changes was correlated with changes in the cutaneous tumors. Animals showing rapid cutaneous tumor regression developed acute uveitis that was characterized by an influx of mononuclear cells into the stroma of the ciliary body; later this spread to the iris and choroid. In late stages the cornea sometimes developed a band of calcium precipitates beneath the basal epithelial cells (band keratopathy). Uveal melanocytes developed watery cytoplasm typical of cells with ruptured plasma membranes. Released melanin granules were taken up initially into the lysosomal compartment of mononuclear cells having the various morphologic features of lymphocytes and monocytes; later, melanin also appeared in fibrocyte lysosomes. The relationship of these processes to various cell-mediated immunity phenomena is being studied. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 Figure 8
Feeney-Burns, L.; Burns, R. P.; Gao, C. L.
Okamoto et al have developed an extremely useful and interesting model of retinal and subretinal neovascularization. Using molecular techniques, they have developed a transgenic model driven by overexpression of VEGF, a growth factor demonstrated to play an important role in neovascularization in many ocular diseases. They have been able to demonstrate that VEGF overexpression is sufficient to cause intraretinal and subretinal neovascularization. The mouse model is relatively cheap and reliable, does not require any exogenous agent, and has many characteristics of clinical intraocular neovascularization. The new vessels develop in the outer retina and subretinal space and have a characteristic histological appearance. They leak fluorescein on angiography, demonstrating their similarity to human disease and allowing identification and grading of neovascularization in vivo. The model can be used to investigate molecular mechanisms of VEGF-dependent neovascularization, with applications beyond ocular eye disease. The model can also be used to study anti-angiogenic agents that have the potential to treat common blinding diseases such as age-related macular degeneration. Okamoto et al have made a substantial contribution to the angiogenesis field with this work, and one looks forward to future investigations.
Miller, J. W.
Interleukin-6 (IL-6) is a key cytokine featuring redundancy and pleiotropic activity. It plays a central role in host defence against environmental stress such as infection and injury. Dysregulated, persistent interleukin (IL)-6 production has been implicated in the development of various autoimmune, chronic inflammatory diseases and even cancers. Significant elevation of IL-6 has been found in ocular fluids derived from refractory/chronic uveitis patients. In experimental autoimmune uveitis models with IL-6 knock-out mice, IL-6 has shown to be essential for inducing inflammation. IL-6 blockade can suppress acute T helper type 17 (Th17) responses via its differentiation and, importantly, can ameliorate chronic inflammation. Tocilizumab, a recombinant humanized anti-IL-6 receptor antibody, has been shown to be effective in several autoimmune diseases, including uveitis. Herein, we discuss the basic biology of IL-6 and its role in development of autoimmune conditions, focusing particularly on non-infectious uveitis. It also provides an overview of efficacy and safety of tocilizumab therapy for ocular inflammatory diseases. PMID:24528300
Mesquida, M; Leszczynska, A; Llorenç, V; Adán, A
The phrase “immune privilege” was coined by Peter Medawar to describe the absence of an immune response to allografts placed into the anterior chamber of the eye or brain. We now understand that immune privilege is more than a passive microenvironment with a distinctive anatomical structure that holds back immunity. The ocular microenvironment actively engages the immune system with immunosuppressive biochemical mechanisms. The unique characteristics of ocular immune privilege appear designed to protect the eye from damage while preserving foveal vision, thus providing the host with a definite survival advantage. However, the protection is not always sufficient and the eye becomes susceptible to uveitis. Uveitis is an intraocular inflammatory disorder that encompasses a wide range of underlying etiologies. It may be idiopathic or associated with systemic disease or infection. Understanding the biochemistry of immune privilege has the potential to identify its weaknesses that allow for immunity to break through.
Taylor, Andrew W.; Kaplan, Henry J.
BACKGROUND/AIMS—Landmines have long been used in conventional warfare. These are antipersonnel mines which continue to injure people long after a ceasefire without differentiating between friend or foe, soldier or civilian, women or children. This study focuses on Afghan non-combatants engaged in mine clearing operations in Afghanistan in the aftermath of the Russo-Afghan war. The patterns and types of injuries seen are described and experiences in their management, ways, and means to prevent them, and recommendations for the rehabilitation of the affected individuals are given.?METHODS—It is a retrospective and analytical study of 84 patients aged 19-56 years who sustained mine blast injuries during mine clearing operations in Afghanistan from November 1992 to January 1996. The study was carried out at a military hospital with tertiary care facilities. The patients were divided into three groups on the basis of their injuries. Group 1 required only general surgical attention, group 2 sustained only ocular injuries, while group 3 had combined ocular and general injuries. Patients in groups 2 and 3 were treated in two phases. The first phase aimed at immediate restoration of the anatomy, while restoration of function wherever possible was done in subsequent surgical procedures in the second phase.?RESULTS—It was observed that 51 out of 84 patients (60.7%) had sustained ocular trauma of a variable degree as a result of the blasts. The mean age of the victims was 29 years and they were all male. A total of 91 eyes of 51 patients (89.2%) had been damaged. Bilaterality of damage was seen in 40 (78.4%) patients. Most, 34 (37.3%), eyes became totally blind (NPL). Only a few escaped with injury mild enough not to impair vision. Foreign bodies, small and multiple, were found in the majority of eyes; most, however, were found in the anterior segment, and posterior segment injuries were proportionally less.?CONCLUSIONS—The prevalence of blindness caused by mine blast injuries is quite high. The resulting psychosocial trauma to the patients and their families is tremendous and has not been adequately highlighted. These injuries are a great drain on the country's resources. Enforcement of preventive measures and the use of protective gear and sophisticated equipment by the mine clearing personnel would prove to be far more economical in terms of human life as well as medical and economic resources. There is also need for greater attention towards the establishment of support groups and rehabilitation programmes for these individuals.??
Muzaffar, W.; Khan, M. D.; Akbar, M; Khan, M. D.; Malik, A. M.; Durrani, O.
Purpose Many studies suggest that alterations in ocular hemodynamics can significantly impact the development, progression, and incidence of glaucoma. This article discusses the role of mathematical modeling in studying the relationship between ocular hemodynamics and glaucoma pathophysiology. Methods Recent literature on glaucoma, ocular blood flow, autoregulation, the optic nerve head, and the use of mathematical modeling in ocular circulation was reviewed to summarize the current approaches used to investigate the relationship between vascular impairment and glaucoma progression. Results Mathematical models describing the mechanical, hemodynamic, and regulatory aspects of the ocular circulation have been developed. Preliminary modeling work shows the potential of mathematical models to elucidate the mechanisms that contribute most significantly to glaucoma progression. Conclusion Mathematical modeling is a useful tool when used synergistically with clinical and laboratory data in the study of ocular blood flow and glaucoma. The development of models to investigate the relationship between ocular hemodynamic alterations and glaucoma progression will provide a unique and useful method for studying the pathophysiology of glaucoma.
Harris, Alon; Guidoboni, Giovanna; Arciero, Julia C.; Amireskandari, Annahita; Tobe, Leslie A.; Siesky, Brent A.
. ?Recent experimental data indicate that both neurotrophic factors (NTFs) and intracortical inhibitory circuitry are implicated\\u000a in the development and plasticity of ocular dominance columns. We extend a neurotrophic model of developmental synaptic plasticity,\\u000a which previously failed to account correctly for the differences between monocular deprivation and binocular deprivation,\\u000a and show that the inclusion of lateral cortical inhibition is indeed necessary
Terry Elliott; Nigel R. Shadbolt
Glaucoma is an optic neuropathy of unknown origin. The most important risk factor for the disease is an increased intraocular pressure (IOP). Reducing IOP is associated with reduced progression in glaucoma. Several recent large scale trials have indicated that low ocular perfusion pressure (OPP) is a risk factor for the incidence, prevalence and progression of the disease. This is a strong indicator that vascular factors are involved in the pathogenesis of the disease, a hypothesis that was formulated 150 years ago. The relation between OPP and blood flow to the posterior pole of the eye is, however, complex, because of a phenomenon called autoregulation. Autoregulatory processes attempt to keep blood flow constant despite changes in OPP. Although autoregulation has been observed in many experiments in the ocular vasculature the mechanisms underlying the vasodilator and vasoconstrictor responses in face of changes in OPP remain largely unknown. There is, however, recent evidence that the human choroid regulates its blood flow better during changes in blood pressure induced by isometric exercise than during changes in IOP induced by a suction cup. This may have consequences for our understanding of glaucoma, because it indicates that blood flow regulation is strongly dependent not only on OPP, but also on the level of IOP itself. Indeed there is data indicating that reduction of IOP by pharmacological intervention improves optic nerve head blood flow regulation independently of an ocular vasodilator effect. PMID:20868686
Schmidl, Doreen; Garhofer, Gerhard; Schmetterer, Leopold
The emergency care and management of patients with ocular trauma can often be handled completely by the family physician, whose goal should be recognizing the nature and circumstance of the injury and subsequent decision making based on the initial management steps. Obtaining a careful and thorough patient history is vital, as unsuspected trauma may be looked for based on historical clues. The initial examination is similarly crucial for determining the extent of the injury and for formulating the treatment plan. Very often, early and effective first-aid care can prevent future complications, which could otherwise lead to subsequent deterioration of the eye. ImagesFigure 1Figure 2Figure 3Figure 4Figure 5Figure 6Figure 7Figure 8
Nichols, Bruce D.
A 68-year-old Caucasian man with a remote history of daily colloidal silver ingestion presented for ophthalmic examination in which he was noted to have a distinct slate gray skin discoloration. Funduscopy revealed confluent perimacular drusenoid deposits bilaterally, most of which localized at the level of or anterior to the inner segment ellipsoid band by optical coherence tomography (OCT) imaging. Enhanced depth imaging OCT demonstrated marked choroidal thinning. Fluorescein angiogram displayed a dark or silent choroid. Confirmatory serum silver levels were found to be markedly elevated. This report describes a unique geographic maculopathy with large drusenoid deposits anterior to the ellipsoid layer and severe choroidal thinning in association with ocular argyrosis. PMID:24548762
Rahimy, Ehsan; Beardsley, Robert; Ferrucci, Steven; Ilsen, Pauline; Sarraf, David
A 68-year-old Caucasian man with a remote history of daily colloidal silver ingestion presented for ophthalmic examination in which he was noted to have a distinct slate gray skin discoloration. Funduscopy revealed confluent perimacular drusenoid deposits bilaterally, most of which localized at the level of or anterior to the inner segment ellipsoid band by optical coherence tomography (OCT) imaging. Enhanced depth imaging OCT demonstrated marked choroidal thinning. Fluorescein angiogram displayed a dark or silent choroid. Confirmatory serum silver levels were found to be markedly elevated. This report describes a unique geographic maculopathy with large drusenoid deposits anterior to the ellipsoid layer and severe choroidal thinning in association with ocular argyrosis. PMID:24256711
Rahimy, Ehsan; Beardsley, Robert; Ferrucci, Steven; Ilsen, Pauline; Sarraf, David
Blindness is a major health concern worldwide that has a powerful impact on afflicted individuals and their families, and is associated with enormous socio-economical consequences. The Middle East is heavily impacted by blindness, and the problem there is augmented by an increasing incidence of diabetes in the population. An appropriate drug/gene delivery system that can sustain and deliver therapeutics to the target tissues and cells is a key need for ocular therapies. The application of nanotechnology in medicine is undergoing rapid progress, and the recent developments in nanomedicine-based therapeutic approaches may bring significant benefits to address the leading causes of blindness associated with cataract, glaucoma, diabetic retinopathy and retinal degeneration. In this brief review, we highlight some promising nanomedicine-based therapeutic approaches for drug and gene delivery to the anterior and posterior segments.
Xu, Qingguo; Kambhampati, Siva P.; Kannan, Rangaramanujam M.
PURPOSE: To evaluate the potential efficacy for autologous and allogeneic expanded corneal epithelial cell transplants derived from harvested limbal corneal epithelial stem cells cultured in vitro for the management of ocular surface disease. METHODS: Human Subjects. Of the 19 human subjects included, 18 (20 procedures) underwent in vitro cultured corneal epithelial cell transplants using various carriers for the epithelial cells to determine the most efficacious approach. Sixteen patients (18 procedures on 17 eyes) received autologous transplants, and 2 patients (1 procedure each) received allogeneic sibling grafts. The presumed corneal epithelial stem cells from 1 patient did not grow in vitro. The carriers for the expanded corneal epithelial cells included corneal stroma, type 1 collagen (Vitrogen), soft contact lenses, collagen shields, and amniotic membrane for the autologous grafts and only amniotic membrane for the allogeneic sibling grafts. Histologic confirmation was reviewed on selected donor grafts. Amniotic membrane as carrier. Further studies were made to determine whether amniotic membrane might be the best carrier for the expanding corneal epithelial cells. Seventeen different combinations of tryspinization, sonication, scraping, and washing were studied to find the simplest, most effective method for removing the amniotic epithelium while still preserving the histologic appearance of the basement membrane of the amnion. Presumed corneal epithelial stem cells were harvested and expanded in vitro and applied to the amniotic membrane to create a composite graft. Thus, the composite graft consisted of the amniotic membrane from which the original epithelium had been removed without significant histologic damage to the basement membrane, and the expanded corneal epithelial stem cells, which had been applied to and had successfully adhered to the denuded amniotic membrane. Animal model. Twelve rabbits had the ocular surface of 1 eye damaged in a standard manner with direct removal of the presumed limbal stem cells, corneal epithelium, and related epithelium, followed by the application of n-heptanol for 60 seconds. After 6 weeks, all damaged eyes were epithelialized and vascularized. Two such treated eyes were harvested without further treatment, to be used for histologic study as damaged controls. The remaining 10 rabbits received composite grafts (consisting of amniotic membrane with expanded allogeneic rabbit corneal epithelial cell transplants) applied to the ocular surface in a standard manner followed by the application of a contact lens. At 16 days following transplantation, 5 of the rabbits were sacrificed and the corneal rims were removed for histologic study. At 28 days, the remaining rabbits were sacrificed and the previously damaged eyes were harvested for histologic and immunohistochemical study. RESULTS: Human subjects. Of the 19 total patients admitted to the study, the presumed corneal epithelial stem cells of 1 patient did not grow in vitro. Of the remaining 18 patients (20 procedures, 19 eyes), 3 patients had unsuccessful results (3 autologous procedures), 1 patient had a partially successful procedure (allogeneic procedure), and 1 patient had a procedure with an undetermined result at present (allogeneic procedure). One unsuccessful patient had entropion/trichiasis and mechanically removed the graft and eventually went into phthisis. The other 2 unsuccessful patients suffered presumed loss of autologous donor epithelium and recurrence of the ocular surface disease (pterygium). The partially successful patient receiving an allogeneic transplant had infectious keratitis delay of his re-epithelialization; he has only minimal visual improvement but has re-epithelialized. The patient receiving the second allogeneic graft lost his donor epithelium at day 4. Additional donor epithelium was reapplied, but the result is undetermined at present. Amniotic membrane as carrier. The in vitro preparation of the amniotic membrane with corneal epithelial stem cell graft overlay was successful.Histology docume
Schwab, I R
A 40-year-old man presented with floaters and painless progressive blurring of vision in the right eye for one month duration. Visual acuity in the right eye was 6/24. There was mild anterior chamber reaction and vitritis. The optic disc was swollen and elevated with presence of granulomatous lesion in the optic disc head. Blood investigations were unremarkable. Serum angiotensin converting enzyme (ACE) was normal and conjunctival biopsy showed presence of inflammatory cells. B-Scan ultrasound revealed an echo-dense lesion in the optic nerve head. There was increased uptake of the right lacrimal gland and presence of ‘Panda sign’ with Gallium scan. A diagnosis of right ocular sarcoidosis was made base on the clinical features and Gallium scan.
Ismail, Shatriah; Embong, Zunaina; Hitam, Wan Hazabbah Wan
We describe complimentary imaging technologies in traumatic chorioretinal injury. Color and fundus autofluorescence (FAF) images were obtained with a non-mydriatic retinal camera. Optical coherence tomography (OCT) helped obtain detailed images of retinal structure. Microperimetry was used to evaluate the visual function. A 40-year-old man sustained blunt ocular trauma with a stone. Color fundus image showed a large chorioretinal scar in the macula. Software filters allowed detailed illustration of extensive macular fibrosis. A 58-year-old man presented with blunt force trauma with a tennis ball. Color fundus imaging showed a crescentric area of macular choroidal rupture with fibrosis. FAF imaging delineated an area of hypofluorescence greater on fundus imaging. OCT showed chorioretinal atrophy in the macula. Microperimetry delineated an absolute scotoma with no response to maximal stimuli. Fundus imaging with digital filters and FAF illustrated the full extent of chorioretinal injury, while OCT and microperimetry corroborated the structure and function correlations.
Kolomeyer, Anton M.; Szirth, Bernard C.; Nayak, Natasha V.; Khouri, Albert S.
More cost effective and robust designs of ocular adaptive optics systems could probably be derived from a thorough knowledge of ocular time-varying aberrations. This would in particular benefit to therapeutic systems where the problem of robustness is critical. Unfortunately, high frequency temporal statistical behavior of ocular aberrations remains poorly characterized. We set up an original high resolution custom-built Shack-Hartmann aberrometer running at a frequency of 236Hz additionally featuring pupil tracking and performedmeasurements on a 50-eye population. First analyses are carried out over 20 eyes. Qualitative correlation between dynamic aberrations and saccadic pupil movements is highlighted.
Jarosz, Jessica; Meimon, Serge; Conan, Jean-Marc; Paques, Michel
Conjunctivitis is often considered an innocuous condition which is self-limiting. This report describes misdiagnosis of ocular cicatricial pemphigoid as chronic conjunctivitis. Ocular cicatricial pemphigoid is a rare autoimmune condition. The clinical features which are useful to distinguish this condition from infective conjunctivitis are discussed. The investigation and treatment of ocular cicatricial pemphigoid is discussed. It is important to recognize nonocular symptoms and signs that may indicate the presence of a more serious underlying pathological condition necessitating specialized ophthalmic referral and subsequent investigation and treatment.
Ocular pain is most commonly associated with redness and inflammation; however, eye pain can also occur in the absence of grossly visible pathology. Pain in the quiet eye can be the first sign of a number of threatening conditions. Many of these conditions such as intermittent angle closure glaucoma, carotid artery dissection, idiopathic intracranial hypertension, and giant cell arteritis can lead to permanent vision loss or blindness. In this review, ocular history and examination techniques are summarized. The article also reviews pertinent ocular, orbital, referred, and other causes of eye pain in the quiet eye. The neurologist and headache specialist should recognize when consultation with an ophthalmologist is necessary. PMID:21128023
Ringeisen, Alexander L; Harrison, Andrew R; Lee, Michael S
Purpose Miller Fisher syndrome (MFS) is a rare immune-mediated neuropathy that commonly presents with diplopia following the acute onset of complete bilateral external ophthalmoplegia. Ophthalmoplegia is often accompanied by other neurological deficits such as ataxia and areflexia that characterize MFS. Although MFS is a clinical diagnosis, serological confirmation is possible by identifying the anti-GQ1b antibody found in a majority of affected patients. We report a patient with MFS who presented with clinical signs suggestive of ocular myasthenia gravis, but in whom the correct diagnosis was made on the basis of serological testing for the anti-GQ1b antibody. Case Report An 81-year-old white man presented with an acute onset of diplopia following a mild gastrointestinal illness. Clinical examination revealed complete bilateral external ophthalmoplegia and left-sided ptosis. He developed more marked bilateral ptosis, left greater than right, with prolonged attempted upgaze. He was also noted to have a Cogan’s lid twitch. Same day evaluation by a neuro-ophthalmologist revealed mild left-sided facial and bilateral orbicularis oculi weakness. He had no limb ataxia, but exhibited a slightly wide-based gait with difficulty walking heel-to-toe. A provisional diagnosis of ocular myasthenia gravis was made and anticholinesterase inhibitor therapy was initiated. However, his symptoms did not improve and serological testing was positive for the anti-GQ1b IgG antibody, supporting a diagnosis of MFS. Conclusions Although the predominant ophthalmic feature of MFS is complete bilateral external ophthalmoplegia, it should be recognized that MFS has variable associations with lid and pupillary dysfunction. Such confounding neuro-ophthalmic features require a thorough history, neurological examination, neuroimaging, and serological testing for the anti-GQ1b antibody to arrive at a diagnosis of MFS.
Anthony, Scott A.; Thurtell, Matthew J.; Leigh, R. John
PURPOSE.: Miller Fisher syndrome (MFS) is a rare immune-mediated neuropathy that commonly presents with diplopia after the acute onset of complete bilateral external ophthalmoplegia. Ophthalmoplegia is often accompanied by other neurological deficits such as ataxia and areflexia that characterize MFS. Although MFS is a clinical diagnosis, serological confirmation is possible by identifying the anti-GQ1b antibody found in most of the affected patients. We report a patient with MFS who presented with clinical signs suggestive of ocular myasthenia gravis but in whom the correct diagnosis was made on the basis of serological testing for the anti-GQ1b antibody. CASE REPORT.: An 81-year-old white man presented with an acute onset of diplopia after a mild gastrointestinal illness. Clinical examination revealed complete bilateral external ophthalmoplegia and left-sided ptosis. He developed more marked bilateral ptosis, left greater than right, with prolonged attempted upgaze. He was also noted to have a Cogan lid twitch. Same day evaluation by a neuro-ophthalmologist revealed mild left-sided facial and bilateral orbicularis oculi weakness. He had no limb ataxia but exhibited a slightly wide-based gait with difficulty walking heel-to-toe. A provisional diagnosis of ocular myasthenia gravis was made, and anticholinesterase inhibitor therapy was initiated. However, his symptoms did not improve, and serological testing was positive for the anti-GQ1b immunoglobulin G antibody, supporting a diagnosis of MFS. CONCLUSIONS.: Although the predominant ophthalmic feature of MFS is complete bilateral external ophthalmoplegia, it should be recognized that MFS has variable associations with lid and pupillary dysfunction. Such confounding neuro-ophthalmic features require a thorough history, neurological examination, neuroimaging, and serological testing for the anti-GQ1b antibody to arrive at a diagnosis of MFS. PMID:23190719
Anthony, Scott A; Thurtell, Matthew J; Leigh, R John
Introduction: Glaucoma is a multifactorial disease characterized by progressive optic nerve injury and visual field defects. Elevated intraocular pressure (IOP) is the most widely recognized risk factor for the onset and progression of open-angle glaucoma, and IOP-lowering medications comprise the primary treatment strategy. IOP elevation in glaucoma is associated with diminished or obstructed aqueous humor outflow. Pharmacotherapy reduces IOP by suppressing aqueous inflow and/or increasing aqueous outflow. Purpose: This review focuses on novel non-FDA approved ocular antihypertensive compounds being investigated for IOP reduction in ocular hypertensive and glaucoma patients in active clinical trials within approximately the past 2 years. Methods: The mode of IOP reduction, pharmacology, efficacy, and safety of these new agents were assessed. Relevant drug efficacy and safety trials were identified from searches of various scientific literature databases and clinical trial registries. Compounds with no specified drug class, insufficient background information, reformulations, and fixed-combinations of marketed drugs were not considered. Results: The investigational agents identified comprise those that act on the same targets of established drug classes approved by the FDA (ie, prostaglandin analogs and ?-adrenergic blockers) as well as agents belonging to novel drug classes with unique mechanisms of action. Novel targets and compounds evaluated in clinical trials include an actin polymerization inhibitor (ie, latrunculin), Rho-associated protein kinase inhibitors, adenosine receptor analogs, an angiotensin II type 1 receptor antagonist, cannabinoid receptor agonists, and a serotonin receptor antagonist. Conclusion: The clinical value of novel compounds for the treatment of glaucoma will depend ultimately on demonstrating favorable efficacy and benefit-to-risk ratios relative to currently approved prostaglandin analogs and ?-blockers and/or having complementary modes of action.
Chen, June; Runyan, Stephen A; Robinson, Michael R
Allergic diseases have greatly increased in industrialized countries. About 30% of people suffer from allergic symptoms and 40%–80% of them have symptoms in the eyes. Atopic conjunctivitis can be divided into seasonal allergic conjunctivitis (SAC) and perennial allergic conjunctivitis (PAC). The treatment of SAC is simple; antihistamines, anti-inflammatory agents, or chromoglycate. In severe cases of SAC, subcutaneous or sublingual immunotherapy is helpful. PAC needs longer therapy, often year round, with mast cell stabilizers, antihistamines, and sometimes local steroids. Atopic keratoconjunctivitis is a more severe disease showing chronic blepharitis often connected with severe keratitis. It needs, in many cases, continuous treatment of the lid eczema and keratoconjunctivitis. Blepharitis is treated with tacrolimus or pimecrolimus ointment. Conjunctivitis additionally needs corticosteroids and, if needed, cyclosporine A (CsA) drops are administered for longer periods. Basic conjunctival treatment is with mast cell-stabilizing agents and in addition, antihistamines are administered. Vernal keratoconjunctivitis is another chronic and serious allergic disease that mainly affects children and young people. It is a long-lasting disease which commonly subsides in puberty. It demands intensive therapy often for many years to avoid serious complicating corneal ulcers. Treatment is mast cell-stabilizing drops and additionally antihistamines. In relapses, corticosteroids are needed. When the use of corticosteroids is continuous, CsA drops should be used, and in relapses, corticosteroids should be used additionally. Nonallergic eosinophilic conjunctivitis (NAEC) is a less known, but rather common, ocular disease. It affects mostly middle-aged and older women. The eye symptoms of NAEC are largely similar to those seen in chronic allergic conjunctivitis. Basic therapy is mast cell-stabilizing drops. Eosinophilic inflammation needs additional corticosteroids. In severe cases, CsA drops are recommended. Antihistamines should be avoided. It is important to recognize the different forms of allergic ocular diseases and to start the treatment early and intensively enough to avoid chronicity of the disease and accompanying tissue destruction.
Kari, Osmo; Saari, K Matti
There is little information regarding ocular anatomy and histology in many of the rodent species. Histological analyses for morphologic features were performed in 31 globes from 18 rodent species submitted to and archived at the Comparative Ocular Pathology Laboratory of Wisconsin. The following measurements were taken: thickness of the cornea, corneal epithelium, corneal stroma, Descemet's membrane, and retina. H&E sections were evaluated for the following anatomical features: presence of pigmented epithelial cells in the peripheral cornea, presence and location of Schlemm's canal, presence of iridal sphincter and dilator and ciliary body muscles, presence of pars plicata and plana, presence of retinal vessels, presence of lamina cribrosa, and presence of tapetum lucidum. The springhaas was the only rodent in our collection that presented a well-developed tapetum lucidum fibrosum. The presence of retinal vessels was variable: vessels were observed in all of the members of the mouse-related clade, except the springhaas and the beaver, in all of the squirrel-related clade members, and in none of the Ctenohystrica. In the flying squirrels, blood vessels extended to the outer limiting membrane in the photoreceptor layer. Beavers, chinchillas, capybara, and guinea pigs lacked vessels within the retina; however, they had vessels within the optic nerve head. Ground squirrels have an optic nerve head, which is linear in the horizontal plane and an asymmetric retina. The tree-dwelling squirrels have a rounded but still elongated optic nerve, and the flying squirrel has a round optic nerve head like all the other rodents. PMID:23734597
Rodriguez-Ramos Fernandez, Julia; Dubielzig, Richard R
Background To evaluate the effect of human milk on ocular morbidity in infants who did not breast feed during the first 6 months of life. Material/Methods This retrospective randomized study included 154 subjects who were first or second grade elementary school students, 66–84 month of age. These children were randomly selected from 2080 students during health screenings. All of them were born at term and were normal birth weight. The study was composed of 3 age- and sex-matched groups. Group 1 included 52 subjects who had never been breast fed. Group 2 included 42 subjects who were breast fed and also received additional food (infant formula). Group 3 included 60 subjects who were only breast fed until 6 months old (no formula) except for the first month of life. All subjects underwent a complete ophthalmic examination and any morbidity was recorded. Frequencies of ocular morbidity were compared among the groups by using the chi-squared test. Results We found significant refractive errors in 12 (23%) subjects in Group 1 (no breast feeding). There was no significant refractive error in Group 2 (breast feeding and formula) and Group 3 (breast feeding only). The difference among the groups was statistically significant (p=0.014, chi-squared test). Allergic conjunctivitis was found in 5 subjects in Group 1, 3 in Group 2, and 2 in Group 3. There was no significant difference among the groups (p=0.395). Conclusions Refractive errors were more frequent in Group 1 (no breast feeding) than in Group 2 (breast feeding and formula) or Group 3 (breast feeding only). There is a need to confirm this finding by performing studies with larger sample sizes.
Aksoy, Adnan; Ozdemir, Murat; Aslan, Lokman; Aslankurt, Murat; Gul, Ozlem
This document is the study general handbook for The Studies of the Ocular Complications of AIDS (SOCA), which oversees the conduct of trials and observational studies regarding the ocular complications of AIDS. SOCA is a collaborative multi-center researc...
P. E. Scott
Three patients with hemoglobin S-C disease, all with ocular lesions, were studied and contrasted with four other patients who had predominantly either hemoglobin S or C but no ocular lesions. Attention is drawn to the diagnostic value of hemoglobin electrophoresis in such cases. With this technique a diagnosis of hemoglobin S-C disease was made in a patient previously thought to the “Eales' disease”; one sister was found to have asymptomatic hemoglobin S-C disease and unsuspected early ocular lesions. The number of persons in Canada with hemoglobinopathies is increasing. Awareness of an association between abnormal hemoglobins and ocular disease may lead to the recognition of an unsuspected blood disorder or of an unsuspected retinopathy. ImagesFig. 1Fig. 2Fig. 5
Speakman, J. S.; Crookston, J. H.; Mortimer, C. B.; Robertson, G. L.
A majority of cases of preventable and/or curable ocular morbidity and blindness are caused by ocular infections. They may account for 70 to 90% of all ocular morbidity seen by family doctors, general practitioners, health centers, and local ophthalmologists in both developed and developing countries. Unfortunately, most health authorities and doctors, including ophthalmologists, consider these diseases to be of little or no importance because they are not fully aware of the high prevalence of these infections and the blinding sequelae which may occur following incorrect diagnosis and treatment. Also, they are not aware of the social and economic impact of these infections in the absence of proper management and implementation of preventive measures. In this review, we examine present knowledge of chlamydial and common viral ocular infections. We discuss the problems of diagnosis, management, and prevention and propose solutions relevant to developed and developing countries. PMID:2539947
Darougar, S; Monnickendam, M A; Woodland, R M
The Collaborative Ocular Melanoma Study (COMS) is a multicenter investigation designed to evaluate therapeutic interventions for patients who have choroidal melanoma. Two randomized controlled trials are currently being conducted. Eligible patients for th...
The vestibulo-ocular reflex manifest by counterroll was used to determine the response dynamics of the vestibular system and alterations in these dynamics subsequent to +Gx exposure. Sic rhesus monkeys were tested before and after acceleration exposure to...
A. M. Junker C. R. Replogle K. A. Smiles R. Wheeler R. D. Brown
The report summarizes the following research areas: Molecular and perimacular vascular remodeling; natural course of sickle retinopathy; conjunctival angiography; sickle cell trait and retinopathy; therapy of sickle cell retinopathy; and related ocular fu...
M. F. Goldberg
Blastenogenic activity of ocular tissue extracts was elicited in lymphocyte cultures from peripheral blood of two of four patients with sympathetic opthalmia. Retinal pigment epithelium, retina, and lens were mitogenic, while choroidal antigen was usually...
F. P. Alepa G. E. Marak M. C. Johnson R. L. Font
Thirty patients with ocular hypertension were tested for contrast sensitivity loss. Seventeen were not on treatment, and thirteen were receiving some form of pressure reducing therapy. The contrast sensitivity results of 63% of ocular hypertensive eyes were abnormal (greater than 2 SDs from the age matched norm). Thus it appears that contrast sensitivity can detect early visual loss in patients who have normal visual fields and it is suggested that this test might be used as a criterion for therapy in ocular hypertension. There was no significant difference in the intraocular pressures between patients who gave abnormal contrast sensitivity results and those who did not in the untreated group (p greater than 0.05), suggesting that intraocular pressure level is a poor predictor of optic nerve fibre damage in patients with ocular hypertension.
Ross, J E; Bron, A J; Reeves, B C; Emmerson, P G
We report successful outcome of a huge post- trabeculectomy intra-bleb hematoma and hyphema that occurred following digital ocular compression. The patient was a 64-year-old lady suffering from bilateral primary angle closure glaucoma and cataract. She was on anti-platelet therapy. She underwent single-site phacoemulsification, intra-ocular lens implantation and trabeculectomy with mitomycin C in the right eye. The trabeculectomy was under-filtering. She was asked to perform digital ocular compression thrice daily. On 15th post-operative day, she presented with a huge intra-bleb hematoma and hyphema. The hematoma did not respond to conservative measures and was drained to prevent bleb failure. We recommend caution in the consideration of digital ocular compression in patients on prophylactic anti-coagulation.
Bhargava, Sagar; Choudhari, Nikhil S.; Vijaya, Lingam
Ocular surface temperature (OST) has been studied with numerous approach and Infrared (IR) thermography has proved to be the best way to capture temperature distribution over some surfaces. It is applied to a number of biomedical applications including studies in the field of ophthalmology. However, the analysis of an ocular thermogram is largely in nascent stage, and is usually achieved by first-order texture analysis. This current study conducted second-order texture analysis on ocular thermal images, mainly by cross co-occurrence matrix together with first-order texture analysis, moments and difference histogram. It was found that, for subjects aged above 35 years old their interocular difference in median, textural contrast, moment 2 and moment 3 (in absolute value) were significantly higher than younger peers. Several significant linear correlations among investigated features were observed. The features extracted from cross co-occurrence matrix may play an important role in the diagnosis of ocular diseases.
Tan, Jen-Hong; Ng, E. Y. K.; Rajendra Acharya, U.; Chee, C.
The goals of treatment with ocular prostheses are to restore facial aesthetics and self-esteem to anophthalmic patients. Fabricated in acrylic resin, artificial eyes should be aesthetically pleasing, scratch-resistant, and adequately polished. Use of a prosthesis without such characteristics can lead to psychological damage, as well as infection and inflammation due to the accumulation of microorganisms and other substances on an irregularly shaped prosthesis. The present literature review describes the different techniques for fabricating ocular prostheses. Reproduction of the iris color and color stability are important factors that promote adequate aesthetics. The fabrication of an individual ocular prosthesis in acrylic resin provides satisfactory aesthetic results because the impression process establishes the defect contour. Additionally, the iris and sclera can be individually characterized. As the techniques, materials, and manufacturing methods for ocular prostheses continue to evolve, the aesthetics and functionality of prostheses will also improve. PMID:24568127
Goiato, Marcelo Coelho; Bannwart, Lisiane Cristina; Haddad, Marcela Filié; dos Santos, Daniela Micheline; Pesqueira, Aldiéris Alves; Miyahara, Glauco Issamu
Introduction: Ocular trauma is a worldwide cause of visual morbidity, a significant proportion of which occurs in the industrial workplace and includes a spectrum of simple ocular surface foreign bodies, abrasions to devastating perforating injuries causing blindness. Being preventable is of social and medical concern. Aim: A prospective case series study, to know the profile of ocular trauma at a hospital caters exclusively to factory employees and their families, to co-relate their demographic and clinical profile and to identify the risk factors. Materials and Methods: Patients with ocular trauma who presented at ESIC Model hospital, Rajajinagar, Bangalore, from June 2010 to May 2011 were taken a detailed demographic data, nature and cause of injury, time interval between the time of injury and presentation along with any treatment received. Ocular evaluation including visual acuity, anterior and posterior segment findings, intra-ocular pressure and gonio-scopy in closed globe injuries, X-rays for intraocular foreign body, B-scan and CT scan were done. Data analyzed as per the ocular trauma classification group. The rehabilitation undertaken medically or surgically was analyzed. At follow-up, the final best corrected visual acuity was noted. Results: A total of 306 cases of ocular trauma were reported; predominantly in 20-40 year age group (72.2%) and in men (75%). The work place related cases were 50.7%and of these, fall of foreign bodies led the list. Visual prognosis was poorer in road traffic accidents rather than work place injuries owing to higher occurrence of open globe injuries and optic neuropathy. Finally, 11% of injured cases ended up with poor vision. Conclusion: Targeting groups most at risk, providing effective eye protection, and developing workplace safety cultures may together reduce occupational eye injuries.
Shashikala, P; Sadiqulla, Mohammed; Shivakumar, D; Prakash, K H
Bubbles in the ocular tear film have been observed following both dry-chamber, simulated compressed air dives and in-water, recreational compressed air dives. The current paper reports on the formation of tear film bubbles in a breath-hold diver following repeated, extended breath-hold excursions to a maximum depth of -28.5 m. It is believed that this is the first time that ocular tear film bubbles have been reported in breath-hold divers. PMID:18500071
Sheard, P W
Specific-pathogen-free chickens (two, four and ten weeks of age) which were inoculated via the air sac with Escherichia coli developed ocular lesions. Histologically, the main ocular lesions consisted of hyphema, hemorrhages of the iris, hypopyon, keratitis and uveitis. Hyphema was associated with hemorrhages of the iris, and hypopyon with keratitis and uveitis. Cyclophosphamide treatment enhanced the incidence and severity of hyphema and hemorrhages of the iris in the chickens. Images Fig. 1. Fig. 2. Fig. 3. Fig. 4.
Nakamura, K; Abe, F
In vitro organotypic models for testing ocular irritants have warranted sufficient interest as methods to replace in vivo ocular testing. The in vitro organotypic models claim to maintain short-term normal physiological and biochemical function of the mammalian cornea in an isolated system. In these test methods, damage by the test substance is assessed by quantitative measurements of changes in corneal opacity and permeability using opacitometry and spectrophotometry, respectively. Both measurements are used quantitatively for irritancy classification for prediction of the in vivo ocular irritation potential of a test substance. Examples of organotypic models that incorporate these criteria include: the bovine corneal opacity and permeability (BCOP) assay, the isolated chicken eye (ICE) test method and the isolated rabbit eye (IRE) assay. A fourth method, the hen's egg test-chorioallantoic membrane (HET-CAM) assay, differs in the evaluation criteria but is also normally included among this class of in vitro protocols. Each of these protocols is discussed in detail as representative candidate in vitro methods for assessing ocular irritation and corrosion. The methodologies, protocol details, applications, and their validation status are discussed. A brief historical perspective of the development of original in vitro ocular testing models is also mentioned. More importantly, improvement and troubleshooting the current techniques, in order to present the models as stand-alone in vitro tools for ocular toxicity assessment, is emphasized.
Barile, Frank A.
Background The aim of this study is to describe the type and severity of paintball-related ocular trauma and to determine the necessary precautions to minimize the risk of ocular injury regardless of whether adequate eye protection was used. Material/Methods A retrospective chart review identified patients treated for paintball-related ocular trauma at the Ataturk University Medical Hospital from June 2010 through March 2013. A descriptive analysis of data was performed. Results Ten patients with paintball-related ocular trauma were identified. At the time of their first examination, 7 of these patients had visual acuity (VA) of 20/200 or worse. One patient had a final VA of no light perception and 4 patients had a final VA of 20/200 or worse. Hyphema was noted in 7 patients, traumatic cataract in 2, iridodialysis in 2, retinal detachment in 3, and secondary glaucoma in 1. Six patients required surgery. Although all victims have used eye protection during the game, all patients were injured after they thought the game was over and had taken off their helmets or eye-protective devices. Conclusions Paintball-related accidents result in serious ocular trauma and most of the patients require surgery. These injuries result in severe loss of VA in some patients. Uninterrupted use of proper eye protection whenever a player is in the game field, even after they believe the game has ended, may reduce the incidence of severe ocular trauma in paintball players.
Keles, Sadullah; Ondas, Osman; Ekinci, Metin; Sener, Mustafa Talip; Erhan, Erim; Sirinkan, Ahmet; Salman, Ilknur Akyol; Kocer, Ibrahim; Baykal, Orhan
This study was conducted to evaluate the influence of alcohol consumption on the risk of ocular trauma. We retrospectively reviewed the medical records of 1,024 patients who visited emergency department and received ophthalmologic examination from January 1 to December 31, 2009. The patients were divided into 2 groups: those with ocular trauma (n = 494) and those without (n = 530); the influence of alcohol consumption was compared between these 2 groups. In the ocular trauma group, the association of the causes and types of ocular trauma with alcohol consumption was evaluated. One of 530 patients of no trauma group and 117 (23.7%) of 494 patients of trauma group were related with alcohol intake, and the difference was statistically significant (P < 0.001). Concerning the causes, physical assault was significantly more common in alcohol-associated injury (P < 0.001). Regarding the types of injury, orbital wall fracture and hyphema showed a significant association with alcohol consumption (P < 0.001). Older age and nighttime injury were significantly related to the increased risk of alcohol-associated ocular trauma (P = 0.018 and < 0.001, respectively). In conclusion, alcohol consumption significantly increases the risk of ocular trauma.
Han, Sang Beom; Yang, Hee Kyung; Woo, Se Joon; Hyon, Joon Young
Background Ophthalmologists often refer patients with suspected ocular sarcoidosis to pulmonologists for diagnostic examination of sarcoidosis. However, no recommendation has been proposed for managing such patients. This study aims to prospectively evaluate the diagnostic values of examinations and propose the management of patients with suspected ocular sarcoidosis. Methods Consecutive patients with suspected ocular sarcoidosis were prospectively investigated according to type of ocular lesions, measurement of serum ACE, and findings of chest radiography, chest CT, bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBLB). Diagnostic values were calculated on the basis of pathological results. Results Forty-two patients were included (female, 71.4%; mean age, 56.2±14.8 years), of whom 64.3% was diagnosed with sarcoidosis. Patient characteristics and ocular lesions did not differ significantly, regardless of the presence of sarcoidosis. Chest CT had low specificity and very high sensitivity for detecting sarcoidosis; in contrast, chest radiography and direct findings of bronchofiberscopy had high specificity and low sensitivity. Serum ACE and BAL did not have high diagnostic value. A flow chart was proposed to diagnose sarcoidosis, and this chart reduced the requirement of TBLB to 50% in our population. During the median follow-up of 51 months, 7 patients in the sarcoidosis group (25.9%) developed new lesions. Conclusions Application of our flow chart appears to detect avoidable TBLB. Development of a more comprehensive flow chart including survey of ocular findings is warranted.
Matsumoto, Shuuichi; Kojima, Eiji; Iwata, Susumu; Tanaka, Kentarou
Background Multiple congenital ocular anomalies (MCOA) syndrome is a hereditary congenital eye defect that was first described in Silver colored Rocky Mountain horses. The mutation causing this disease is located within a defined chromosomal interval, which also contains the gene and mutation that is associated with the Silver coat color (PMEL17, exon 11). Horses that are homozygous for the disease-causing allele have multiple defects (MCOA-phenotype), whilst the heterozygous horses predominantly have cysts of the iris, ciliary body or retina (Cyst-phenotype). It has been argued that these ocular defects are caused by a recent mutation that is restricted to horses that are related to the Rocky Mountain Horse breed. For that reason we have examined another horse breed, the Icelandic horse, which is historically quite divergent from Rocky Mountain horses. Results We examined 24 Icelandic horses and established that the MCOA syndrome is present in this breed. Four of these horses were categorised as having the MCOA-phenotype and were genotyped as being homozygous for the PMEL17 mutation. The most common clinical signs included megaloglobus, iris stromal hypoplasia, abnormal pectinate ligaments, iridociliary cysts occasionally extending into the peripheral retina and cataracts. The cysts and pectinate ligament abnormalities were observed in the temporal quadrant of the eyes. Fourteen horses were heterozygous for the PMEL17 mutation and were characterized as having the Cyst-phenotype with cysts and occasionally curvilinear streaks in the peripheral retina. Three additional horses were genotyped as PMEL17 heterozygotes, but in these horses we were unable to detect cysts or other forms of anomalies. One eye of a severely vision-impaired 18 month-old stallion, homozygous for the PMEL17 mutation was examined by light microscopy. Redundant duplication of non-pigmented ciliary body epithelium, sometimes forming cysts bulging into the posterior chamber and localized areas of atrophy in the peripheral retina were seen. Conclusions The MCOA syndrome is segregating with the PMEL17 mutation in the Icelandic Horse population. This needs to be taken into consideration in breeding decisions and highlights the fact that MCOA syndrome is present in a breed that are more ancient and not closely related to the Rocky Mountain Horse breed.
Is the concept of integrative, preventive and personalised medicine applicable to the relationship between retinitis pigmentosa (RP) and ocular blood flow (OBF)? RP encompasses a group of hereditary diseases of the posterior segment of the eye characterised by degeneration, atrophy and finally loss of photoreceptors and retinal pigment epithelium, leading to progressive visual loss. Many different mutations affecting different genes can lead to the clinical picture of RP. Even though the disease has a clear genetic background, there are obviously other factors influencing the manifestation and progression of RP. In this review, we focus on the role of OBF. There is evidence that, in PR patients, OBF is more reduced than one would expect secondary to the retinal atrophy. The main cause of this additional component seems to be primary vascular dysregulation (PVD) syndrome. As PVD syndrome is partly treatable, a vascular evaluation of RP patients is meaningful. Based on the outcome, a targeted individualised, preventive or supportive treatment might be introduced in selected RP patients.
The necessity of using animals to test whether new chemicals and products are eye irritants has been questioned with increasing frequency and fervor over the last 20 years. During this time many new nonanimal methods have been proposed as reliable alternatives to the traditional rabbit (Draize) test. To date, however, none of these nonanimal (in vitro) tests have become universally accepted as a complete replacement for the Draize test. To understand why a complete replacement has not been found, one has to first understand the reasonably complex structure of the eye, the standard Draize scoring scale--which is based on a qualitative evaluation of three different tissues--the differences between human and rabbit eyes, the intrinsic variability of the animal test, and the details of the different in vitro tests that have been proposed as replacements. The in vitro tests vary from relatively simple assays using single cells to more sophisticated assays that use discarded animal tissue or artificially constructed human tissue. It is clear that appropriately designed in vitro tests will eventually give more useful mechanistic information about ocular injury from which we can more comfortably predict the risk of human eye irritation from new products and ingredients. Images Figure 1 Figure 2
Curren, R D; Harbell, J W
The necessity of using animals to test whether new chemicals and products are eye irritants has been questioned with increasing frequency and fervor over the last 20 years. During this time many new nonanimal methods have been proposed as reliable alternatives to the traditional rabbit (Draize) test. To date, however, none of these nonanimal (in vitro) tests have become universally accepted as a complete replacement for the Draize test. To understand why a complete replacement has not been found, one has to first understand the reasonably complex structure of the eye, the standard Draize scoring scale--which is based on a qualitative evaluation of three different tissues--the differences between human and rabbit eyes, the intrinsic variability of the animal test, and the details of the different in vitro tests that have been proposed as replacements. The in vitro tests vary from relatively simple assays using single cells to more sophisticated assays that use discarded animal tissue or artificially constructed human tissue. It is clear that appropriately designed in vitro tests will eventually give more useful mechanistic information about ocular injury from which we can more comfortably predict the risk of human eye irritation from new products and ingredients. PMID:9599696
Curren, R D; Harbell, J W
New evidence for a central resolution of gravito-inertial signals has been recently obtained by analyzing the properties of the vestibulo-ocular reflex (VOR) in response to combined lateral translations and roll tilts of the head. It is found that the VOR generates robust compensatory horizontal eye movements independent of whether or not the interaural translatory acceleration component is canceled out by a gravitational acceleration component due to simultaneous roll-tilt. This response property of the VOR depends on functional semicircular canals, suggesting that the brain uses both otolith and semicircular canal signals to estimate head motion relative to inertial space. Vestibular information about dynamic head attitude relative to gravity is the basis for computing head (and body) angular velocity relative to inertial space. Available evidence suggests that the inertial vestibular system controls both head attitude and velocity with respect to a gravity-centered reference frame. The basic computational principles underlying the inertial processing of otolith and semicircular canal afferent signals are outlined.
Hess, B. J.; Angelaki, D. E.
Purpose. To determine ocular pain duration after routine in-office intravitreal injection and to determine whether topical eyedrops are beneficial in increasing patient comfort. Methods. Forty injection-naïve patients receiving routine intravitreal injections of bevacizumab for age-related macular degeneration were randomized into 3 groups: group 1 (control, no drops), group 2 (generic artificial tears), and group 3 (ketorolac tromethamine 0.4% eyedrops). Those who received topical medications were given a Visual Analog Pain score survey and asked to record their pain on a scale from 0 (no distress) to 10 (unbearable distress) daily until a score of 0 was achieved, at which point they were instructed to discontinue use of their given drops. Self-reported pain scores were assessed. Results. Pain after routine intravitreal injection lasts on average between 3 and 7 days. Patients receiving topical ketorolac eyedrops reported the fewest average number of pain days (2.25±1.22) vs patients receiving artificial tears (3.54±1.13) or those who received no postprocedure eyedrops (5.13±1.25); p<0.05. At most, patients receiving ketorolac eyedrops reported 3 days of recordable pain. Those who received artificial tears reported at most 5 days of recordable pain, and patients who did not receive any postprocedure eyedrops reported at most 7 days of recordable pain. Conclusions. Pain after intravitreal injection is generally mild, may be reduced by postinjection topical ketorolac eyedrops, and lasts less than 1 week. PMID:22562296
Rifkin, Lana; Schaal, Shlomit
We present our findings in a case of primary neuroendocrine carcinoma (NEC) of the lacrimal gland and a case of primary Merkel cell carcinoma (MCC) of the eyelid. An 86-year-old man noticed a swelling of the left upper eyelid three months earlier. We performed excision biopsy and histopathological examination indicated that he had a primary NEC of the left lacrimal gland. He underwent chemotherapy followed by excision including the clinically visible margins and 50?Gy radiotherapy of the surgical margins. He had neither recurrence nor metastasis for 6 months since the last radiotherapy. An 80-year-old man noticed a nodule in the right upper eyelid and was referred to our hospital because the size was increasing rapidly. A complete surgical excision of the margins of the tumor was performed with histopathological confirmation of negative margins. The final diagnosis was a primary MCC of the right upper eyelid. After surgery, he underwent 50?Gy radiotherapy on the neck to prevent metastasis. No recurrence or metastasis was found for two years. Although primary NEC of the ocular adnexa is extremely rare, the tumor has high malignancy and readily metastasizes. Thus, combined therapy including surgery, radiotherapy, and/or chemotherapy is needed for complete management of NEC.
Yamanouchi, Daisuke; Nakamura, Yosuke; Yotsukura, Jiro; Asanagi, Kaoru; Baba, Takayuki; Nizawa, Tomohiro; Kishimoto, Takashi; Yonemori, Yoko; Ota, Satoshi; Yamamoto, Shuichi
Background This study aims to evaluate the effect of oral statin medication use on the subsequent development of ocular inflammatory disease (OID). A retrospective nested case–control study was carried out on patient records from the Birmingham Veterans Affairs Medical Center. All male patients with a new diagnosis of OID over a 5-year period were included. Ten control subjects (without OID) were age-matched to each OID case. Prescription files of all subjects were queried for statin use. Information on selected comorbid medical conditions was also obtained. Conditional logistic regression was used to calculate odds ratios (ORs) and 95% confidence intervals (CIs) for risk of OID development in the context of statin use, controlling for comorbid conditions. Results Ninety-two incident cases of OID were identified. A trend toward a reduction in the risk of new OID development was found in patients that used statins compared to those that did not (OR 0.50, 95% CI 0.20 to 1.23, p = 0.13). The longer the duration of statin use, the greater is the effect. Conclusions Use of oral statins may be associated with a reduced risk for the development of OID. This reduced risk increases with increasing duration of use. Larger clinical studies would be required to definitively establish the effectiveness of statins in lowering the incidence of OID.
Behçet's disease (BD) is a chronic recurrent systemic inflammatory disorder of unknown etiology characterized by oral and genital ulcerations, skin lesions, and uveitis. The ocular involvement of BD, or Behçet's uveitis (BU), is characterized by panuveitis or posterior uveitis with occlusive retinal vasculitis and tends to be more recurrent and sight threatening than other endogenous autoimmune uveitides, despite aggressive immunosuppression. Although pathogenesis of BD is unclear, researches have revealed that immunological aberrations may be the cornerstone of BD development. General hypothesis of BD pathogenesis is that inflammatory response is initiated by infectious agents or autoantigens in patients with predisposing genetic factors and perpetuated by both innate and acquired immunity. In addition, a network of immune mediators plays a substantial role in the inflammatory cascade. Recently, we found that the immunopathogenesis of BU is distinct from other autoimmune uveitides regarding intraocular effector cell profiles, maturation markers of dendritic cells, and the cytokine/chemokine environment. In addition, accumulating evidence indicates the involvement of Th17 cells in BD and BU. Recent studies on genetics and biologics therapies in refractory BU also support the immunological association with the pathogenesis of BU. In this review, we provide an overview of novel findings regarding the immunopathogenesis of BU.
Park, Un Chul; Kim, Tae Wan; Yu, Hyeong Gon
There are two different types of ocular melanocytes and melanomas. Conjunctival melanocytes are located on the surface of the eye and are exposed to visible light and UV radiation. Recently, epidemiological studies demonstrated that sunlight plays a definite role in the occurrence of conjunctival melanoma, as it does in cutaneous melanoma. Uveal melanocytes consist of the iridal melanocytes, which are exposed to visible light and UV radiation; and the ciliary body melanocytes and choroidal melanocytes, which are not exposed to light radiation. Epidemiological studies demonstrated that sunlight may play a role in the occurrence of iridal melanoma, but may not be a major factor in the etiology of ciliary body and choroidal melanomas. Uveal melanocytes differ from epidermal melanocytes in that epidermal melanocytes respond to UV radiation and skin color becomes darker after exposure to sunlight; but uveal melanocytes do not respond to UV radiation and the iris color remains stable after exposure to sunlight. Recently, in vitro studies indicate that this phenomenon is determined both by cellular factors and environmental factors. PMID:15496134
Aims: To assess the aetiological factors associated with the occurrence of perforating ocular injuries in children in an urban setting and to assess the visual outcomes of such injuries. Methods: All cases of perforating ocular injury presenting to a single paediatric hospital (age less than 16 years) over a 17 year period were identified by a medical record search. All new cases of perforating ocular injury identified were included. All information was obtained retrospectively from the medical records. Results: There were 72 cases identified. The commonest causes of perforating ocular injury were sharp tools (knives/scissors) poked by the child into his/her own eye (17%), or objects thrown at the child (17%). Injuries were most likely to have occurred at home (58%). The age range for injuries was 8 months to 14 years 8 months. Perforating ocular injury was most frequent in the 3–6 year group (32%) followed by the 6–9 year group (25%). Males were more frequently involved than females (48–24). There was no correlation between the laterality of the eye, the time of day of the occurrence, or the day of the week of the occurrence. The final acuity achieved was better or equal to 6/12 in 36% and less than 6/60 in 31%. Injuries occurred more frequently on weekends than on weekdays. There were six enucleations (8%). Follow up was for an average period of 25 months. Conclusions: Penetrating ocular injury occurs most frequently in the home setting and mostly as the result of the use of sharp tools or by thrown objects. Prevention of penetrating ocular injury requires greater education of children and their carers especially on the potential dangers within the home.
Thompson, C G; Kumar, N; Billson, F A; Martin, F
To investigate the expression profiles of efflux transporters in human ocular tissues, quantitative real-time polymerase chain reaction, Western blotting, and immunohistochemistry were used to obtain the relative mRNA and protein expressions of various efflux transporters in human ocular tissues. The cornea, conjunctiva, iris-ciliary body (ICB), retina and choroid, human corneal epithelial cell line (HCEC), and human retinal pigment epithelial cell line (ARPE-19) were examined for the expressions of multidrug resistance-associated proteins 1-7 (MRP1-7), multidrug resistance 1 (MDR1) P-glycoprotein, lung resistance protein (LRP), and breast cancer-resistance protein (BCRP). The expression sites and patterns of efflux transporters were significantly different in ocular tissues, HCEC, and ARPE-19, as well as the expression profiles of efflux transporters in mRNA and protein levels in ocular tissues. At the protein level, MRP1-7, MDR1, and LRP were expressed in the corneal epithelium; MRP1-7, MDR1, LRP, and BCRP were expressed in the conjunctival epithelium; MRP1-2, MRP6-7, MDR1, and LRP were expressed in the ICB; MRP1-3, MRP6-7, MDR1, and LRP were expressed in the retina; MRP1-3, MRP6-7, MDR1, and LRP were expressed in the HCEC; and MRP7, MDR1, LRP, and BCRP were expressed in the ARPE-19. This quantitative and systematic study of efflux transporters in normal ocular tissues and cell lines provides evidence of cross-ocular tissue transporter expression differences, implying that efflux transporter expression variability should be taken into consideration for better understanding of ocular pharmacokinetic and pharmacodynamic data. PMID:23979916
Chen, Peng; Chen, Hao; Zang, Xinjie; Chen, Min; Jiang, Haoran; Han, Shasha; Wu, Xianggen
Topical anesthesia is a safe and cost-effective method considered as the first-choice in many procedures. The objective of the present study was to develop ocular inserts as a new form of lidocaine HCl to give a sufficient level of anesthetic. Ocuserts were prepared using HPMC and PVA in different ratios with lidocaine HCl alone and lidocaine HCl ?-cyclodextrins complex. Drug polymer interactions were studied by Fourier transform infrared spectroscopic studies. The prepared ocular inserts were characterized by means of ocusert thickness, weight variation, folding endurance, surface pH, moisture absorption, drug content and in-vitro drug release. Stability study was conducted on selected formulations, and in vivo evaluation of lidocaine HCl was also carried out. The results revealed that F7 formulations containing drug ?-cyclodextrins with 4 % HPMC and 2 % PVA were found to have good physical characteristics and appropriate flexibility. In addition to the highest initial and cumulative percentage of drug released in vitro. The selected F7 ocuserts retained their characteristics during the stability study. The results of in vivo study showed that the addition of ?-cyclodextrins in F7 significantly increase the drug content in the aqueous humor when compared with F3 ocuserts containing lidocaine HCl alone. PMID:24395530
Proper availability of drug on to corneal surface is a challenging task. However, due to ocular physiological barriers, conventional eye drops display poor ocular bioavailability of drugs (< 1%). To improve precorneal residence time and ocular penetration, earlier our group developed and evaluated in situ gel and nanoparticles for ocular delivery. In interest to evaluate the combined effect of in situ gel and nanoparticles on ocular retention, we combined them. We are the first to term this combination as "nanoparticle laden in situ gel", that is, poly lactic co glycolic acid nanoparticle incorporated in chitosan in situ gel for sparfloxacin ophthalmic delivery. The formulation was tested for various physicochemical properties. It showed gelation pH near pH 7.2. The observation of acquired gamma camera images showed good retention over the entire precorneal area for sparfloxacin nanoparticle laden in situ gel (SNG) as compared to marketed formulation. SNG formulation cleared at a very slow rate and remained at corneal surface for longer duration as no radioactivity was observed in systemic circulation. The developed formulation was found to be better in combination and can go up to the clinical evaluation and application. PMID:23833523
Gupta, Himanshu; Aqil, Mohammed; Khar, Roop K; Ali, Asgar; Bhatnagar, Aseem; Mittal, Gaurav
Proper availability of drug on to corneal surface is a challenging task. However, due to ocular physiological barriers, conventional eye drops display poor ocular bioavailability of drugs (< 1%). To improve precorneal residence time and ocular penetration, earlier our group developed and evaluated in situ gel and nanoparticles for ocular delivery. In interest to evaluate the combined effect of in situ gel and nanoparticles on ocular retention, we combined them. We are the first to term this combination as “nanoparticle laden in situ gel”, that is, poly lactic co glycolic acid nanoparticle incorporated in chitosan in situ gel for sparfloxacin ophthalmic delivery. The formulation was tested for various physicochemical properties. It showed gelation pH near pH 7.2. The observation of acquired gamma camera images showed good retention over the entire precorneal area for sparfloxacin nanoparticle laden in situ gel (SNG) as compared to marketed formulation. SNG formulation cleared at a very slow rate and remained at corneal surface for longer duration as no radioactivity was observed in systemic circulation. The developed formulation was found to be better in combination and can go up to the clinical evaluation and application.
Gupta, Himanshu; Aqil, Mohammed; Khar, Roop K.; Ali, Asgar; Bhatnagar, Aseem; Mittal, Gaurav
The term, ocular toxoplasmosis, refers to eye disease related to infection with the parasite, Toxoplasma gondii. Recurrent posterior uveitis is the typical form of this disease, characterized by unilateral, necrotizing retinitis with secondary choroiditis, occurring adjacent to a pigmented retinochoroidal scar and associated with retinal vasculitis and vitritis. Multiple atypical presentations are also described, and severe inflammation is observed in immunocompromised patients. Histopathological correlations demonstrate focal coagulative retinal necrosis, and early in the course of the disease, this inflammation is based in the inner retina. For typical ocular toxoplasmosis, a diagnosis is easily made on clinical examination. In atypical cases, ocular fluid testing to detect parasite DNA by polymerase chain reaction or to determine intraocular production of specific antibody may be extremely helpful for establishing aetiology. Given the high seroprevalence of toxoplasmosis in most communities, serological testing for T. gondii antibodies is generally not useful. Despite a lack of published evidence for effectiveness of current therapies, most ophthalmologists elect to treat patients with ocular toxoplasmosis that reduces or threatens to impact vision. Classic therapy consists of oral pyrimethamine and sulfadiazine, plus systemic corticosteroid. Substantial toxicity of this drug combination has spurred interest in alternative antimicrobials, as well as local forms of drug delivery. At this time, however, no therapeutic approach is curative of ocular toxoplasmosis.
Butler, Nicholas J; Furtado, Joao M; Winthrop, Kevin L; Smith, Justine R
In recent years, the atomic force microscope (AFM) has become an important tool in ophthalmic research. It has gained popularity largely because AFM is not restricted by the diffraction limits of light microscopy and can be applied to resolve images with molecular resolution. AFM is a minimally invasive technique and can be used to visualize molecular structures under near-physiological conditions. In addition, the AFM can be employed as a force apparatus to characterize the viscoelastic properties of biomaterials on the micron level and at the level of individual proteins. In this article, we summarize recent AFM studies of ocular tissues, while highlighting the great potential of AFM technology in ophthalmic research. Previous research demonstrates the versatility of the AFM as high resolution imaging technique and as a sensitive force apparatus for probing the mechanical properties of ocular tissues. The structural and mechanical properties of ocular tissues are of major importance to the understanding of the optomechanical functions of the human eye. In addition, AFM has played an important role in the development and characterization of ocular biomaterials, such as contact lenses and intraocular lenses. Studying ocular tissues using Atomic Force Microscopy has enabled several advances in ophthalmic research.
Ziebarth, Noël M.; Rico, Felix; Moy, Vincent T.
For the treatment of ocular keratitis acyclovir, as a highly specific inhibitor of herpes virus replication, is applied topically into the eye. The objective of this study was to design and evaluate freeze-dried, bioadhesive and biodegredable acyclovir ocular minitablets for prolonged local drug action. The sponge-like nature of the lyophilized ocular minitablets ensures rapid hydration and gelation of these tablets in the eye and thus would reduce the foreign body sensation. The polymers used were sodium carboxymethylcellulose (NaCMC), hydroxypropylmethylcellulose (HPMC), xanthan gum, chitosan and Carbopol 943P. The minitablets were evaluated for drug content, weight variation, bioadhesion, water uptake and in vitro drug release. In addition, the rheological characteristics of the polymers solutions were investigated. Rheological data revealed that all tested polymers exhibited pseudoplastic behaviour which is required to minimize interference with blinking. Drug release was found to be affected by the type and concentration of polymer. The order of sustainment was chitosan > xanthan > HPMC > Carbopol > NaCMC. Water uptake study, dissolution rate of the polymers and viscosity measurements could explain the different release profiles of the drug from the polymers. Chitosan minitablet was chosen for its significant sustained release and good bioadhesive property for in vivo study in rabbits. The tablet showed a good permeation into the cornea in comparison to the commercially available Zovirax(®) eye ointment. In conclusion, chitosan ocular minitablets containing acyclovir could be considered as a promising sustained drug delivery system for ocular keratitis treatment. PMID:20804440
Refai, Hanan; Tag, Randa
Purpose of review The oculomotor periphery was classically regarded as a simple mechanism executing complex behaviors specified explicitly by neural commands. A competing view has emerged that many important aspects of ocular motility are properties of the extraocular muscles and their associated connective tissue pulleys. This review considers current concepts regarding aspects of ocular motility that are mechanically determined versus those that are specified explicitly as innervation. Recent findings While it was established several years ago that the rectus extraocular muscles have connective tissue pulleys, recent functional imaging and histology has suggested that the rectus pulley array constitutes an inner mechanism, analogous to a gimbal, that is rotated torsionally around the orbital axis by an outer mechanism driven by the oblique extraocular muscles. This arrangement may account mechanically for several commutative aspects of ocular motor control, including Listing’s Law, yet permits implementation of non-commutative motility. Recent human behavioral studies, as well as neurophysiology in monkeys, are consistent with implementation of Listing’s Law in the oculomotor periphery, rather than centrally. Summary Varied evidence now strongly supports the conclusion that Listing’s Law and other important ocular kinematics are mechanically determined. This finding implies more limited possibilities for neural adaptation to some ocular motor pathologies, but indicates possibilities for surgical treatments.
Demer, Joseph L.
Sparganosis is an infection by the parasitic tapeworm larvae of Spirometra species. Ocular sparganosis is a rare disease that is easily misdiagnosed. We reported a rare case of ocular sparganosis mimicking orbital idiopathic inflammatory syndrome at initial presentation. A 34-year-old female presented with rapid progressive swelling of her left eyelid and mild proptosis for the duration of one month. The other ocular examinations were normal and the thyroid function was normal. Magnetic resonance imaging revealed a fusiform enlargement and mild heterogenous enhancement of the superior oblique muscle of the left orbit. First she received prednisolone therapy and the proptosis partially improved. Six months later, a white, flat and wrinkled string like worm wriggled out from the caruncular conjunctiva of the left eye. The pathology results confirmed that the worm was a Spirometra species larva. After removal of the larva and treatment with praziquantel, the proptosis was resolved without recurrence. Ocular sparganosis is a rare disease and only a few case reports have been reported. The drug therapy has not been effective and the surgical removal is the principal therapy. Despite its rarity, ocular sparganosis should be considered as a possible cause of orbital inflammation in patients. PMID:24215169
Ho, Tsai-Hsuan; Lin, Muh-Chiou; Yu, Wei-Wen; Lai, Ping-Hong; Sheu, Shwu-Jiuan; Bee, Youn-Shen
Treatment in glaucoma aims to lower intraocular pressure (IOP) to reduce the risk of progression and vision loss. The alpha2-adrenergic receptor agonist brimonidine effectively lowers IOP and is useful as monotherapy, adjunctive therapy, and replacement therapy in open-angle glaucoma and ocular hypertension. A fixed combination of brimonidine and timolol, available in some countries, reduces IOP as effectively as concomitant therapy with brimonidine and timolol and offers the convenience of 2 drugs in a single eyedrop. Brimonidine is safe and well tolerated. Its most common side-effects are conjunctival hyperemia, allergic conjunctivitis, and ocular pruritus. The newest formulation of brimonidine, brimonidine-Purite 0.1%, has a higher pH to improve the ocular bioavailability of brimonidine. This formulation contains the lowest effective concentration of brimonidine and is preserved with Purite(R) to enhance ocular tolerability. Brimonidine-Purite 0.1% is as effective in reducing IOP as the original brimonidine 0.2% solution preserved with benzalkonium chloride. Recent results from preclinical and clinical studies suggest that brimonidine may protect retinal ganglion cells and their projections from damage and death independently of its effects on IOP. The potential for neuroprotection with brimonidine is an added benefit of its use in glaucoma and ocular hypertension. PMID:18360646
Cantor, Louis B
Ocular surface defects represent one of the most common causes of impaired vision or even blindness. For treatment, keratoplasty represents the first choice. However, if corneal defects are more extensive and associated with a limbal stem cell (LSC) deficiency, corneal transplantation is not a sufficient therapeutic procedure and only viable approach to treatment is the transplantation of LSCs. When the LSC deficiency is a bilateral disorder, autologous LSCs are not available. The use of allogeneic LSCs requires strong immunosuppression, which leads to side-effects, and the treatment is not always effective. The alternative and perspective approach to the treatment of severe ocular surface injuries and LSC deficiency is offered by the transplantation of autologous mesenchymal stem cells (MSCs). These cells can be obtained from the bone marrow or adipose tissue of the particular patient, grow well in vitro and can be transferred, using an appropriate scaffold, onto the damaged ocular surface. Here they exert beneficial effects by possible direct differentiation into corneal epithelial cells, by immunomodulatory effects and by the production of numerous trophic and growth factors. Recent experiments utilizing the therapeutic properties of MSCs in animal models with a mechanically or chemically injured ocular surface have yielded promising results and demonstrated significant corneal regeneration, improved corneal transparency and a rapid healing process associated with the restoration of vision. The use of autologous MSCs thus represents a promising therapeutic approach and offers hope for patients with severe ocular surface injuries and LSC deficiency. PMID:23733704
Holan, Vladimir; Javorkova, Eliska
While airbags have saved many lives and are clearly beneficial overall, sodium hydroxide (NaOH) powder produced by the inflation reaction can cause significant alkali ocular injury if not irrigated promptly. Here we report a case of severe airbag related ocular alkali injury as a way to bring attention to the need for prompt ocular irrigation following motor vehicle accidents (MVA) with airbag deployment. A 47-year-old man was involved in a MVA with airbag deployment in a rural setting. Attention was paid to several other life-threatening traumatic injuries, however, ocular irrigation was not performed until some 6-7 hours after the MVA. Over the course of 6 months, airbag related alkali injury caused severe limbal ischemia, conjunctivalization of the cornea, corneal epithelial defects, cicatricial scarring, haze, and corneal/limbal vascularization despite amniotic membrane graft. Awareness of the importance of ocular irrigation following airbag deployment must be raised both in the ophthalmology and emergency medicine communities. PMID:22900239
Barnes, Shawn S; Wong, William; Affeldt, John C
An object of the invention is to provide a method for the treatment of various ocular diseases. Another object of the present invention are to provide a method for effectively treating ocular diseases while lowering the risks of undesirable physiological ...
R. B. Nussenblatt A. G. Palestine