Paulo Gois Manso

Comparison of practical methods for urinary glycosaminoglycans and serum hyaluronan with clinical activity scores in patients with Graves' ophthalmopathy

objective  Immunosuppressive treatment of Graves’ opthalmopathy (GO) should be restricted to patients with active eye disease, but assessing disease activity is difficult. Several methods to evaluate GO activity have been introduced, but none of them is satisfactory. Glycosaminoglycans (GAGs) are complex polysaccharides that participate on the pathogenesis of GO and attempts to correlate its local increase to urinary GAGs (uGAGs) or serum hyaluronan (sHA) have been made, but the available techniques are labourious, time‐consuming and difficult for routine use. The aim of the present study is to develop practical and simple methods for uGAGs and sHA and compare them to the activity and severity of thyroid‐associated ophthalmopathy.

Exenteração: estudo retrospectivo

PURPOSE To review all cases of orbit exenteration performed at the Orbit Sector, Ophthalmology Department - Federal University of São Paulo, from 1998 to 2003. METHODS We reviewed conditions leading to orbital exenteration in 21 patients at the Orbit Sector of Unifesp-EPM from August 1998 to May 2003. Data regarding sex, age, race, primary lesion site, visual acuity at the moment of diagnosis, previous surgeries related to the exenteration, type of performed surgery, histopathologic diagnosis, postoperative complications and use of adjuvant treatment were collected. RESULTS 21 patient charts were retrospectively analyzed. Ages ranged from 5 to 91 years (mean of 58.5 years). Of these, 12 were male and 9 were female, most of them Caucasian. All lesions that led to exenteration were malignant neoplasias; however, none were metastatic. Lesions originated from eyelids in twelve patients, from bulbar conjunctiva in six and from the orbit in three. Cases were also classified as squamous cell carcinoma (eleven cases), basal cell carcinoma (four cases), sebaceous gland carcinoma (two cases), rhabdomyosarcoma (two cases), mucoepidermoid carcinoma (one case) and adnexal microcistic carcinoma (one case). Visual acuity at the moment of diagnosis ranged from 20/40 to no light perception. Only six patients had been submitted to previous surgeries related to the exenteration. After surgery, three patients suffered graft necrosis, one presented ethmoidal sinus fistula to the orbit and one presented orbital socket shrinkage. Six patients needed postoperative radiotherapy and two had been previously submitted to chemotherapy. CONCLUSION Most patients analyzed in our study presented lesions that are usually small in the beginning; however, they can disseminate to the orbit in the absence of adequate treatment.

Sarcoma granulocítico em órbita: relato de caso

Orbital granulocytic sarcoma is a localized tumor consisting of malignant cells of myeloid origin. This tumor may present in association with acute myelogenous leukemia. Granulocytic sarcoma may be found in a variety of locations throughout the body including the orbit and typically affects children and young adults. There is a slight male predominance in these cases. This is an uncommon case report of a 33-year-old Latin-American woman who was admitted to the Hospital for rapidly progressive orbital proptosis. There was no systemic manifestation of leukemia. The occurrence of orbital granulocytic sarcoma before the development of systemic leukemia in children and young adults is not uncommon and these cases frequently develop hematological evidence within 2 months after initial orbital disease. In this case report, there was no systemic manifestation of leukemia in the last 30 months, even in the presence of orbital tumors. Granulocytic sarcoma is most frequently confused with malignant lymphoma, rhabdomyosarcoma and neuroblastoma. The differential diagnosis of these cases can be challenging, particularly when there is no evidence of systemic leukemia, when imaging features are not sufficiently specific to distinguish granulocytic neoplasms from other tumors. To establish the diagnosis often a biopsy is required. The treatment in such cases (orbital granulocytic sarcoma) is not standardized. Orbital granulocytic sarcoma may be suspected in cases of orbital tumors even in the absence of systemic manifestations of leukemia at any age.

Metástase orbitária como único achado em paciente com hepatocarcinoma: relato de caso

Orbital metastasis of hepatocellular carcinoma is rare. We report an unusual orbital metastatic lesion as the only finding in a case of hepatocellular carcinoma. A 57-year-old man presented with a 6-month history of orbital painful right orbital mass, associated with proptosis. Computed tomography of the orbits showed an orbital soft tissue mass leading to bone erosion and intracranial invasion. Computed tomography of the abdomen showed a focal perfusion abnormality in the left lobe of the liver. Incisional biopsy was performed and the histopathologic examination of the specimen confirmed the diagnosis. The patient died 15 months after the initial presentation. COMENTS: This is a rare case of orbital metastasis of hepatocellular carcinoma. There was no another metastatic lesion and the patient reported only ophthalmological symptoms.

Correlation Between Clinical and Histological Analyses in Retroocular Connective Tissues and Extraocular Muscles from Patients with Graves' Ophthalmopathy.

Twenty-two patients with Graves’ ophthalmopathy underwent biopsy, and two patients had both eyes biopsied. The samples for the control group (n = 4) were obtained during routine non-thyroid-related corrective strabismus surgery. Ophthalmological evaluation with clinical activity score (CAS), endocrinological evaluation, and ultrasound were used in our study. Correlation between clinical and histological analyses in connective tissues and extraocular muscles from patients with Graves’ ophthalmopathy was done. The echography results disclosed an enlargement in all extraocular recti muscles with the exception of one patient. Periodic acid-Schiff and Giemsa stains revealed a moderate number of mast cells in the endomysial connective tissue, none of which displayed significant degranulation. There were no signs of muscle cell damage. Fifteen of the biopsies showed weak cellular reactions with only scattered inflammatory cells. Furthermore, the inflammatory process may be localized and not equally distributed throughout the muscle. Thus, the biopsies might not be representative for the whole muscle. Statistical significance analysis was found when sex and CAS were compared (p = 0.001683) using the Fisher test.In conclusion, our investigation indicates a pleomorphic pattern of histologic findings in connective tissue and extraocular muscles in Graves’ ophthalmopathy.

Corpo estranho orgânico intra-orbitário: avaliação tomográfica e conduta

Purpose: The clinical presentation of orbital foreign bodies is variable. The management and prognosis depends on the composition, location and if there is or not secondary infection. Metallic objects and glass are the most frequently encountered and well-tolerated, while organic foreign bodies can elicit an inflammatory reaction and leads to serious complications. It is frequently difficult to identify and localize organic intraorbital foreign bodies, despite modern imaging methods. Methods: Three patients with intraorbital organic foreign body after penetrating injury were evaluated. Results: All patients were submitted to removal of the foreign body presenting an improved clinical course. Computed Tomography (CT) was essential for evaluation, identification and localization of the foreign body. Conclusion: Preoperative identification of the foreign body in the orbit with the use of computed tomography was very helpful for patient management. The removal of the organic foreign body must be performed in order to avoid complications.

Case report of a metachronous multiple tumor: Mantle cell lymphoma in the orbital region associated with epithelial malignancies at other sites

Here we report the case of a 73-year-old man who was diagnosed with metachronous, multiple primary tumors with non-Hodgkin B-cell mantle cell lymphoma involving the orbit on the basis of biopsy and immunohistochemistry in 2012. The patient had been diagnosed with non-Hodgkin small cell lymphoma and basal cell skin carcinoma in 2010 and intestinal adenocarcinoma with metastasis to the regional lymph nodes in 2011, thus representing a typical case of metachronous, multiple primary tumors. Mantle cell lymphoma is a rare disease and its prognosis is quite poor, particularly when it is associated with other metachronous malignancies. Therefore, physicians should consider mantle cell lymphoma as a differential diagnosis for neoplasms of the orbit.

Atypical presentation of pilomatrixoma in the tarsal conjunctiva

Pilomatrixoma is a rare benign tumor, which usually affects young women in the head and neck region. The eyelid is a common site of the disease, though it is very rare in the tarsal conjunctiva.The pilomatrixoma has clinical pleomorphism, which confuses this disease with other similar conditions. The diagnosis is made by pathological examination in most cases. The treatment is surgical, performed by total excision of the lesion with clear margins and recurrence is very uncommon.We present an unusual case of probable recurrent pilomatrixoma in the left upper tarsal conjunctiva.

Multiple wedge-shaped retinal nerve fiber layer defects in a patient with optic nerve glioma in the contralateral eye: case report

This case report describes a young non-glaucomatous patient with neurofibromatosis and previous history of optic nerve glioma, which developed multiple wedge-shaped retinal nerve fiber layer defects close to a chorioretinal scar in the fellow eye. After discussing the different possible etiologies to the wedge-shaped defects, the disruption of the nerve fiber layer due to the chorioretinal lesion was considered the most plausible cause. However, further follow-up with visual field assessment, optic nerve head documentation and neuroimaging is mandatory in this case and may provide additional information to better understand it.