New aspects of colobomas and optic nerve anomalies.
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Biomedical subjects
Publications and source records attributed to D J Apple.
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A 47-year-old woman who had had a flat, hyperpigmented lesion of the optic disc and juxtapapillary choroid for at least 17 years experienced acute visual loss. This was caused by rapid growth of a pigmented tumour emanating from the original lesion. Histopathological examination of the enucleated globe revealed a large malignant melanoma originating from a melanocytoma (magnocellular nevus) within the optic nerve and peripapillary choroid. This case represents a very rare instance of malignant transformation of a normally benign tumour.
A 31-year-old woman lost central visual acuity in her left eye during scatter argon laser photocoagulation when her ophthalmologist treated the temporal peripapillary region. There was no evidence for a vascular explanation. Previous experimental work showed that the lateral spread of thermal energy into the optic nerve parenchyma from the peripapillary pigment epithelium and choroid can destroy adjacent nerve fibres. There is a risk of thermal optic neuropathy caused by peripapillary photocoagulation.
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A 79-year-old man presented with bilateral periorbital swelling of unknown duration. Diagnostic workup, including orbital and prostate biopsies processed with conventional hematoxylin-eosin and immunoperoxidase stains, revealed that the prostate was the origin of the orbital tumors. Our review of the literature revealed 17 previously reported cases of metastatic prostate carcinoma to the orbit. Salient clinical and histopathologic features of these cases are summarized. To the best of our knowledge, this is the first biopsy proven case of bilateral orbital metastasis of prostate carcinoma.
Light and electron-microscopic analysis of the enucleated eye of a 55-year-old man confirmed the diagnosis of Coats' syndrome. The histopathologic findings in this adult patient are identical to those seen in typical cases of Coats' syndrome of childhood. The characteristic thickening of vessel walls and the apparent deficiency of healthy, viable retinal vascular endothelial cells support the hypothesis that the ocular changes in this syndrome are directly related to a primary retinal vascular defect leading to abnormal permeability.
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An 8-year-old white female presented with retinal vascular changes of fibrovascular proliferation and avascularity of the peripheral retina that suggested a diagnosis of incontinentia pigmenti. These findings prompted a systemic evaluation, and the features of this disease were confirmed, including a history of recurring vesicles on the lower extremities of her sister in infancy. Associated systemic findings in the patient included alopecia, apigmented skin lesions, conical teeth, and a confirmatory biopsy. This case illustrates that a clinical recognition of eye findings can be of assistance in correctly diagnosing such forme fruste cases.
A 21-year-old man currently is well 12 years after his last treatment for metastatic retinoblastoma.
An 8-week-old infant had advanced Coats' disease. Six months after the Coats' disease was noted, a severe, secondary orbital cellulitis developed.
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We treated a patient with localized amyloidosis of the bulbar conjunctiva, which strongly resembled a neoplastic growth. Although the patient suffered from a long-standing, debilitating rheumatoid arthritis, suggesting the possibility of a secondary systemic amyloidosis caused by the chronic effusive process, no specific clinical evidence of systemic amyloidosis was noted. The ocular lesion was, therefore, classified as a primary localized amyloidosis. However, the simple recognition of amyloid in the ocular adnexa should not be considered a complete diagnosis. For such cases in which a prominent underlying systemic disease is present, a thorough medical evaluation and future follow-up should be ensured to rule out the possibility that this represents the initial manifestation of a potentially life-threatening secondary systemic amyloidosis caused by rheumatoid arthritis.
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Hypercalcemia occurs in approximately one of every five patients with thyrotoxicosis, and one of seven patients with hypercalcemia and thyrotoxicosis will have hyperparathyroidism as the cause of the serum calcium elevation. While there are no clinical features which permit easy identification of patients with hyperparathyroidism and thyrotoxicosis, determination of serum parathyroid hormone levels may help. Parathyroid hormone levels may be normal or suppressed if hypercalcemia is due to hyperthyroidism alone, and an elevated parathyroid hormone level suggest coexisting hyperparathyroidism.
The histology and ultrastructure of the hyaloid vascular system was studied in 16 monkeys, ages 85 days in utero to 3 years old. The well-developed hyaloid artery is identifiable ultrastructurally as an arteriole consisting of a nonfenestrated intima, a multilayered smooth muscular media, a connective tissue adventitia, and a perivascular sheath. By the fourth week after birth, the vessel walls appear hyalinized and acellular; the lumen is occluded by a thrombus. The time of the complete disappearance of the hyaloid artery varied considerably in individual monkeys; remnants of the hyaloid artery were often present on the disc (Bergmeister's papilla) in adult life. The vasa hyaloidea propria and tunica vasculosa lentis are small branches of the hyaloid artery which fill the primary vitreous. Ultrastructurally, they are Type A-1-alpha capillaries having a nonfenestrated endothelium, incomplete pericyte layer, and basement membranes surrounding each. Fluorescein angiography of several newborn monkeys revealed nonleakage of dye from the hyaloid artery and its branches, thus correlating with the presence of endothelial tight functions.
A 49-year-old man with chronic myelogenous leukemia developed dense posterior subcapsular cataracts and punctate cortical opacities after five years of busulfan (Myleran) therapy. We found that the histopathologic appearance of busulfan-induced cataracts is similar to other forms of human and experimental cataracts, regardless of cause. Ultrastructural examination revealed cortical liquefaction with formation of Morgagnian droplets, involving primarily the region from the equator to the posterior subcapsular space. In contrast to previous findings in rats, we observed posterior migration of equatorial nuclei and bladder cell formation. Also present were socalled crystalloid rays and abundant degenerate lens fiber membranes manifested as mutli-layered, irregularly folded configurations resembling myelin figures.