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Biomedical subjects

C E Margo

Publications and source records attributed to C E Margo.

155 records · Page 9Linked to original sources

Retinoblastoma: the accuracy of clinical diagnosis in children treated by enucleation.

We correlated the pathologic diagnosis with the preoperative clinical diagnosis of retinoblastoma in children treated by enucleation within the United States and Canada between the years 1974 and 1980. In order to avoid inappropriate selectivity and institutional bias, we studied only those cases submitted directly to the Registry of Ophthalmic Pathology in which the enucleated eyes had not been examined initially at the local hospital. Of the 56 eyes removed because of suspected retinoblastoma, 15 (26.8%) did not contain a malignant tumor. In two cases enucleation was delayed because retinoblastoma was not considered initially in the differential diagnosis of spontaneous hyphema. During the same period, two of 268 eyes that were enucleated for reasons other than suspected retinoblastoma were found to contain a retinoblastoma. The diagnostic error rate in our study reflects the persistent difficulty in diagnosing retinoblastoma in eyes with opaque media and retinal detachment.

Child↗

Computed tomography with histopathologic correlation in children with leukokoria.

Computed tomography (CT) constitutes a major advance in noninvasive diagnostic investigation of children with leukokoria , especially where media opacities preclude adequate visualization of the posterior segment, and, performed in conjunction with other studies such as diagnostic ultrasonography, has significantly improved diagnostic accuracy. Although retinoblastoma, the commonest life-threatening cause of leukokoria , usually presents with characteristic CT findings of intraocular calcification, during extraocular extension, the orbital portions of the tumor are non-calcific and contrast dramatically with the intraocular portion. Currently CT is also the best available technique for diagnosis of trilateral retinoblastoma. Computed tomographic features of several types of pseudogliomas (a collective term for lesions commonly mistaken for retinoblastomas) are also discussed with histopathologic correlation. In persistent hyperplastic primary vitreous, a conical density in CT corresponded to detached and dysplastic retina. A high density layered opacity corresponded to subretinal blood. In Coats' disease and sclerosing endophthalmitis homogenous intraocular CT density corresponds to vitreous and subretinal exudates radiologically indistinguishable from non-calcific retinoblastoma. Long-standing retinal detachments in children occasionally presenting with leukokoria showed subtle homogenous intraocular densities on CT.

Child, Preschool↗

Periocular congenital melanocytic nevi.

Large congenital melanocytic nevi (CMN) are cutaneous malformations of neural crest origin that have a notorious reputation because of their reported propensity to undergo malignant degeneration. Traditionally, complete surgical excision has been advocated based on the premise that large CMN are a precursor to melanoma. Two cases of large periocular CMN are reported to emphasize the importance of distinguishing them from other congenital skin lesions. Data from the literature support the contention that these pigmented skin lesions spawn malignancies and that prophylactic therapy may be indicated.

Child, Preschool↗

Angiomatous malformation of the retrolaminar optic nerve.

A vascular abnormality of the optic nerve was found incidentally in two infants whose eyes were enucleated because of the clinical suspicion of retinoblastoma. The lesion consisted of numerous thin-walled vascular channels within pial septa of the retrolaminar optic nerve. Although it is unclear if this angiomatous malformation could disturb vision, the lesion may be more common than can be inferred from the small number of reported cases.

Hemangioma↗

Primary and secondary orbital teratomas.

Two patients with primary teratomas of the orbit and a third patient with a teratoma invading the orbit from the maxillary sinus are presented. The clinical presentation of each patient was spectacular. In the primary teratomas, the globe was displaced out of the orbit by the attached tumor, causing extreme proptosis. Computed tomography was virtually diagnostic, showing a variegated orbital mass with solid and cystic components. Histologic examination showed multiple tissues derived from two or three germinal layers. The surgical removal of the tumors and reasons for the poor visual outcome are discussed. The appearance of teratomas in the orbit and other cephalic structures appears to arise from the survival and proliferation of germ cells deposited there during embryogenesis.

Female↗

Adenocarcinoma of the ciliary epithelium in a 12-year-old black child.

Histopathologic examination of a blind eye from a 12-year-old black girl revealed an unsuspected adenocarcinoma of the nonpigmented ciliary epithelium and evidence of previous penetrating trauma. The association between adenocarcinoma of the ciliary epithelium and ocular trauma has been well documented in elderly patients but not in children. The histopathologic distinction between a pleomorphic adenocarcinoma of the ciliary epithelium and a uveal melanoma may be difficult. Signs of previous penetrating trauma should heighten suspicion of an adenocarcinoma, even in young children.

Adenocarcinoma↗

Borderline melanocytic tumor of the conjunctiva: diagnostic and therapeutic considerations.

A clinically benign appearing nevus was surgically excised from the conjunctiva of a 13-year-old girl because of complaints that it had recently enlarged. Histologically, the melanocytic lesion displayed considerable cytologic atypia and showed signs that were consistent with malignant transformation. Because of the rarity of conjunctival melanomas in children, the case was reviewed by several experienced pathologists and dermatopathologists whose diagnoses ranged from benign nevus with atypia to malignant melanoma. When the biologic potential of certain melanocytic lesions cannot be accurately predicted histologically, treatment and follow up must be individualized. Physicians must weigh the perceived risks and disadvantages of treating a histologically indeterminate tumor against the consequences of "under" treatment if the lesion is, in fact, biologically malignant.

Adolescent↗

Lenz syndrome in two sisters: clinicopathologic correlations of the ocular anomalies.

BACKGROUND: The Lenz syndrome (Mendelian inheritance in Man catalog number 309,800) is a presumed X-linked recessive disorder. Major diagnostic criteria include ocular, skeletal, and urogenital manifestations. We describe two sisters and the two sons of one of them with Lenz syndrome. The eye from one boy was removed because of pain and total loss of vision, allowing histopathologic documentation of the ocular malformations. METHODS: Clinicopathologic case report. RESULTS: Two sisters in this family displayed several of the major diagnostic criteria of the Lenz syndrome. The stunted growth of the eye, and the ocular and non-ocular anomalies defines the microphthalmos as monogenic, complex, and colobomatous. CONCLUSION: The pattern of inheritance of Lenz syndrome is best explained by X-linked dominant transmission. Future reports of familial cases with an excess of affected females are needed to confirm this hypothesis.

Abnormalities, Multiple↗

Eyes removed for primary ulcerative keratitis with endophthalmitis: microbial and histologic findings.

OBJECTIVES: To study the microbiological and histological findings in patients whose eyes are removed for primary ulcerative keratitis with suppurative endophthalmitis. METHODS: Survey of consecutive cases of ulcerative keratitis with histologically defined endophthalmitis treated by enucleation or evisceration. RESULTS: Eighteen cases of ulcerative keratitis with suppurative endophthalmitis were identified. Thirteen corneal ulcers were culture positive (72%). Microorganism were found in histologic sections of the anterior segment of 9 eyes (50%), but no microorganisms were identified in the vitreous. Two eyes (11%) had culture-positive endophthalmitis. Half of the patients in this study who had undergone elective intraocular surgery had done so within 4 months of enucleation. CONCLUSIONS: Considerable vitreous inflammation can occur with microbial keratitis in the absence of histologically demonstrable microorganisms in the vitreous. Microbial keratitis associated with suppurative endophthalmitis is usually caused by virulent organisms. The factors that contributed to the spread of purulent inflammation inside the eye could not be assessed in this morphologic study because of the lack of clinical data.

Adult↗

Anatomic and histopathologic findings following a failed ahmed glaucoma valve device.

Ahmed glaucoma valve implant (AGV) is one of the more commonly used implants in difficult glaucomas. The histology of a functioning bleb following AGV implantation and its anatomic relationship to the optic nerve when placed in the superionasal quadrant has been described. We report the histology of a failed bleb and the anatomic relationship between the optic nerve and the AGV end-plate when placed 9 mm from the limbus in a patient with neovascular glaucoma.

Aged↗

Light microscopic and ultrastructural findings in failed epikeratoplasty.

Corneal buttons from two patients with failed epikeratoplasties requiring penetrating keratoplasty were examined by light and transmission electron microscopy. Penetrating keratoplasty was performed for lenticule scarring in the first case (adult aphakia) and inadequate best corrected visual acuity in the second case (keratoconus). Findings included decreased lenticule keratocytes and electron-dense, fibrillogranular material associated with either lenticule or recipient keratocytes in both corneas and defects in Bowman's layer in one cornea. In addition, light and electron microscopic evidence consistent with recipient keratocyte migration into the lenticule was demonstrated in one case. These findings were compared with three previous cases.

Adult↗