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Biomedical subjects

A A Hidayat

Publications and source records attributed to A A Hidayat.

At least 19 recordsLinked to original sources

Lipofuscinosis of the cornea. A clinicopathologic study of three cases.

BACKGROUND: Lipofuscin pigments are the indigestible residue of lysosomal activity usually associated with normal aging. Abnormal amounts of lipofuscin also are associated with certain disease processes. The rarity of lipofuscin in the cornea and the similarities between its staining properties and those of intracellular micro-organisms caused great diagnostic problems in three cases. The correct diagnosis of corneal lipofuscinosis was made after extensive histochemical, autofluorescent, and ultrastructural studies. METHODS: Clinical histories of three patients are correlated with morphologic and histochemical findings on five corneal buttons with lipofuscinosis. RESULTS: The histopathologic features of one cornea with chronic keratitis and three corneas from two patients with bilateral opacities of undetermined origin were mostly similar. Large amounts of lipofuscin pigment were found within macrophages and stromal keratocytes. Other pathologic findings were nonspecific, including phagocytosis of degenerated collagen fibrils, scarring, and neovascularization of the stroma. CONCLUSION: The lipofuscin deposits are probably the consequence of a corneal degenerative process and not its cause, although their pathogenesis remains unclear. Familiarity with the morphologic appearance of corneal lipofuscinosis and its staining and autofluorescent properties is important because the small, 1- to 3-microns deposits may be mistaken for intracellular micro-organisms.

Adolescent

Squamous cell carcinoma of the cornea.

We treated two patients with primary squamous cell carcinoma of the cornea without involvement of the corneoscleral limbus. Superficial keratectomy and cryotherapy in one patient and penetrating keratoplasty in the other patient resulted in no recurrence of the tumor after 46 and nine months, respectively. Actinic damage and late manifestation caused by poor vision in both eyes of both patients may have been the risk factors for development of this tumor.

Adult

Dracunculiasis of the orbit and eyelid. Light and electron microscopic observations of two cases.

Dracunculiasis, an infection caused by the nematode parasite, Dracunculus medinensis, usually affects the skin and subcutaneous tissue. The authors studied two cases of dracunculiasis involving the orbit and eyelid in African children. In the first case, the patient presented with proptosis and the clinical diagnosis was Burkitt's lymphoma. In the second patient, the eyelid lesion was diagnosed as a dermoid cyst. Histopathologically, the orbital lesion showed a degenerated and partially calcified worm within a large intraconal abscess. The eyelid lesion contained a well-preserved gravid female worm filled with larvae. The results of transmission and scanning electron microscopic studies are discussed.

Animals

Climatic droplet keratopathy with corneal amyloidosis.

Climatic droplet keratopathy (CDK) is a degenerative disease of the cornea, highly prevalent in Saudi Arabia. It is characterized by aggregates of small, golden-yellow globules that accumulate in the subepithelial layers, Bowman's layer, and the superficial stroma of the cornea. The authors report on 22 patients (40 eyes) with CDK and lattice lines in the cornea. The condition occurred late in life (mean age, 67 years). It was bilateral in 82% of patients, with no family history of lattice dystrophy or systemic amyloidosis. These lines were found in all layers of the stroma. Results of histopathologic and electron microscopic examination of the lattice lines confirmed the presence of amyloid. Results of immunohistochemical examination showed evidence of deposition of AP protein. The cause of these deposits in the cornea is not known, but the authors speculate that environmental factors causing CDK also may play a role in the deposition of protein AP in the cornea. The contribution of trachoma to the degenerative changes in the cornea and secondary amyloidosis could not be ruled out, as all patients had inactive trachoma. Climatic droplet keratopathy with corneal amyloidosis, therefore, represents a form of "lattice degeneration" of the cornea.

Adult

Reticulohistiocytoma of the limbus and cornea. A clinicopathologic study of two cases.

Reticulohistiocytoma is a rare, benign histiocytic lesion usually occurring as an isolated skin nodule or as part of a systemic disorder known as "multicentric reticulohistiocytosis." The clinical and histopathologic findings of two women who presented with a single, painless mass localized to the cornea and limbus without skin lesions or systemic disease are reported. Histopathologically, the lesions were composed predominantly of large mononuclear and a few multinucleated cells with finely granular, "ground-glass" cytoplasm and large nuclei with prominent nucleoli. Immunohistochemical and electron microscopic studies conformed the histiocytic nature of these cells. Reticulohistiocytoma should be included in the differential diagnosis of epibulbar benign histiocytic lesions.

Adult

Spindle cell carcinoma of the conjunctiva. An immunohistochemical and ultrastructural study of six cases.

Six cases of conjunctival spindle cell carcinoma, a rare variant of squamous cell carcinoma, were studied. The median age of the three men and three women was 63.5 years. The tumors appeared as a single nodule in some patients or diffusely involved the conjunctiva in others. Two of the four individuals with intraocular extension presented with phthisis bulbi. Polyclonal antikeratin antibody was helpful and gave the most consistent results when compared with monoclonal antikeratin antibodies, AE1/3 and PKK1. The electron microscopic study of four lesions also established the epithelial nature of the tumor cells. Intracytoplasmic tonofilaments and a few desmosomes were present. Histopathologically, this variant of squamous cell carcinoma is difficult to distinguish from other spindle cell tumors, and this study demonstrates the value of immunohistochemistry and electron microscopy in supporting the correct diagnosis.

Adult

Amyloidosis of corneal stroma in patients with trachoma. A clinicopathologic study of 62 cases.

Sixty-two cases of corneal amyloidosis were studied. The median age of the 48 men and 14 women was 66 years. The patients had bilateral, diffuse corneal opacity extending to the limbus and severe visual impairment. The corneal condition was not familial. Advanced stages of trachoma were present in all individuals. Climatic droplet keratopathy (CDK) was also observed clinically in 19 patients. Histopathologically, the Congo red-positive, birefringent and dichroic amyloid deposits in the stroma were similar to that of lattice corneal dystrophy. Bowman's membrane was mostly absent, and peculiar microcystoid degeneration of the stroma was noted. Electron microscopic studies not only confirmed the diagnosis of amyloid but also showed a more diffuse amyloid pattern than did light microscopy. Trachoma is probably the cause of this corneal degeneration with secondary amyloidosis. The environmental conditions associated with CDK may be contributing factors in some cases.

Adult

Pseudoinflammatory macular dystrophy.

We studied a family with a dominantly inherited macular dystrophy resembling Sorsby's pseudoinflammatory dystrophy. Retinal pigment epithelial atrophy and varying degrees of pigment epithelial metaplasia were prominent fundus features in this pedigree. However, findings on electro-oculography were abnormal, unlike previous findings in patients with Sorsby's dystrophy. Histopathologic study of an eye from one patient demonstrated widespread atrophy of the retina, retinal pigment epithelium, and choroid. Although the pseudoinflammatory fundus appearance is common to several macular dystrophies and some eyes with end-stage, age-related macular degeneration, abnormal electro-oculograms and a dominant inheritance pattern distinguish the dystrophy in the present pedigree from other dystrophies and age-related macular degeneration.

Aged

Atypical cases of sinus histiocytosis (Rosai-Dorfman disease) with ophthalmological manifestations.

Sinus histiocytosis with massive lymphadenopathy is a non-neoplastic disease of unknown cause observed mainly in children with markedly enlarged cervical or other lymph nodes. We have reported two very atypical cases with extranodal manifestations that required ophthalmological consultation. Neither patient had significant lymphadenopathy. One patient, who had tumors of all four eyelids as well as lesions of the nose and larynx, was 68 years old when first examined, and 70 at the time of his last surgical procedure. The other patient, a 13-month-old child, had a unique corneoscleral lesion as his only clinical manifestation. The excised tissues obtained from the eyelids and larynx in case 1 and from the corneoscleral lesion in case 2 revealed characteristic histopathologic features of sinus histiocytosis (Rosai-Dorfman disease), including strongly positive immunoperoxidase staining for S-100 protein in the cytoplasm of most of the histiocytes.

Age Factors

The effect of rapid freezing on uveal melanomas.

We studied the immediate effects of rapid freezing on 19 variously sized uveal melanomas that were subjected to cryoenucleation using liquid nitrogen and a cryoring by light and electron microscopy and tissue culture. The freezing time and the temperature of 11 lesions were recorded. The light microscopic finding of an intranuclear clear center with peripheral displacement of clumped chromatin against the nuclear membrane was suggestive of intranuclear ice crystal formation but did not indicate cellular death of the tumors. The major ultrastructural changes, including plasmalemmal breaks, dissolution of cytoplasmic matrix, and damage to various organelles, however, suggested acute necrosis in tumors not exceeding 7 mm in elevation. Failure of the melanoma cells to grow in tissue culture and positive staining with trypan blue support the contention of tumor death. The late effects of rapid freezing were also evaluated in another case of uveal melanoma. The eye was enucleated six months after cryopexy. Histopathologic findings showed that the tumor was necrotic. Failure of the neoplasm to regress (noted clinically) was related to edema and inflammatory infiltrates.

Choroid Neoplasms

Ligneous conjunctivitis. A clinicopathologic study of 17 cases.

Seventeen cases of ligneous conjunctivitis were studied. Most of the patients were children; however, two elderly individuals were involved (range, birth to 85 years). The most common site was the tarsal conjunctiva, where firm sessile or pedunculated membranous lesions were seen. However, corneal involvement, as well as similar and recurrent lesions, were also present in the larynx, vocal cords, trachea, nose, vagina, cervix, and gingiva. Many recurrences (up to 10 times) rapidly followed simple surgical excision in all patients. Histopathologically, the lesions were characterized by subepithelial, eosinophilic, amorphous material admixed with acute and chronic inflammatory cells. The finding of amorphous material containing fibrin, immunoglobulins, and albumin suggested leakage from hyperpermeable blood vessels. Electron microscopic studies of the lesions disclosed few abnormal blood vessels with wide gaps between endothelial cells that were surrounded by a thick multilaminar basement membrane. The duration of the disease ranged from 4 months to 44 years. After several recurrences, spontaneous resolution occurred in six patients.

Adolescent

Metastatic eyelid disease.

The authors conducted a clinicopathologic study of 31 patients with metastatic disease to the eyelid at the Armed Forces Institute of Pathology. Metastasis was found predominantly in women (female/male = 4:1). The mean age was 69 years (range, 3-92). The most common primary sites were breast (35%), skin (16%), and gastrointestinal and urogenital tracts (each 10%). Aside from breast carcinoma, there was no sex predilection for the extramammary malignancies to metastasize to the eyelids. Metastasis was suspected in only 32% of the cases. Most common misdiagnoses were chalazion, cyst, granuloma, and xanthoma. The lid lesions were detected before the primary focus in 45% of the cases. The authors conclude that metastatic disease should be considered in the differential diagnosis of eyelid lesions.

Adolescent

Myxomas of the conjunctiva.

Fourteen cases of rare conjunctival myxoma were studied clinicopathologically. The median age of the eight men and six women was 50 years (range, 18 to 76 years). The tumors appeared as slowly growing, freely movable solitary lesions located primarily in the temporal bulbar conjunctiva. The clinical diagnosis was cyst in many cases. The lesions were removed by simple local excision and did not recur. Histologically, they were hypocellular and were composed of stellate and spindle-shaped cells; some had small intracytoplasmic and intranuclear vacuoles. The stroma contained abundant mucoid material and sparse reticulin and delicate collagen fibers. Scattered mast cells were found in many lesions. Ultrastructurally, the intracytoplasmic vacuoles appeared to be extremely dilated cisternae of rough-surfaced endoplasmic reticulum. Invaginations of the nuclear membrane by the cytoplasm correlated with intranuclear vacuoles seen by light microscopy. Conjunctival myxomas are benign tumors and should be differentiated from other benign and malignant soft-tissue tumors with myxomatous features.

Adolescent

Neoplastic angioendotheliomatosis. A variant of malignant lymphoma immunohistochemical and ultrastructural observations of three cases.

Neoplastic angioendotheliomatosis (NAE) is a rare, fatal disease characterized by widespread intravascular proliferations of malignant cells of putative endothelial origin. Clinically, dermatologic and bizarre neurological manifestations predominate, but review of the reported cases of NAE reveals ophthalmic involvement to be frequent. To our knowledge, no reports of NAE have appeared in the ophthalmic literature. We describe three cases of NAE with the ocular manifestations of visual loss, cells in the vitreous, retinal artery occlusion, retinal vascular and pigment epithelial alterations, nystagmus, and cortical blindness. Autopsies (including eyes and central nervous system) revealed pancorporal involvement by intravascular anaplastic cells in each patient. In two patients massive extravascular involvement was also present. The tumor cells lacked ultrastructural features of endothelial cells and failed to stain for factor-VIII-related antigen. Common leukocyte antigen, a maker for hematopoietic cells, particularly lymphocytes, was detected on tumor cells in all cases, indicating that NAE is probably an extranodal lymphoma. The dramatic response of the central nervous system lesions to radiotherapy in one case supports this contention. It is suggested that this disorder be treated as a malignant lymphoma.

Aged

A clinicopathologic study of 21 cases of adenoid squamous cell carcinoma of the eyelid and periorbital region.

We conducted a clinicopathologic study of adenoid squamous cell carcinoma involving the eyelid (15 cases) and periorbital region (six cases). The mean age of the 17 men and four women was 64.5 years (range, 43 to 85 years). Histopathologically, the lesions were characterized by acantholysis and a pseudoglandular pattern with many alveolar and tubular structures. Solar keratosis was also a prominent histologic feature, suggesting that exposure to the sun was a predisposing factor. Local recurrences occurred in five of 16 patients for whom follow-up information was available (mean follow-up period, 8.8 years). There was no regional lymph node or distant metastasis, and none of these patients died of their neoplastic disease. Surgical excision appears to be the best treatment.

Adenocarcinoma

The histopathology of involutional ectropion.

Twenty eyelid specimens from patients with involutional ectropion, obtained by full-thickness horizontal shortening procedures, were examined histopathologically by light and electron microscopy and compared with six normal eyelids from exenteration specimens. Paralytic, cicatricial, and congenital ectropions were excluded from the study. The main histopathologic features included: (1) collagen degeneration and elastosis of the tarsal plate; (2) increased amounts of adipose tissue in the distal tarsus and capsulopalpebral fascia; (3) subacute inflammation and epidermidalization of the tarsal conjunctiva; (4) focal degeneration, fibrosis and elastosis of pretarsal orbicularis, and occasionally minimal change in the muscle of Riolan; and (5) arteriosclerosis of the marginal artery. The combination of these histopathologic changes characterize and may contribute to the development of ectropion of the eyelid associated with aging.

Aged

Diabetic choroidopathy. Light and electron microscopic observations of seven cases.

The choroid of seven young patients (ages 20-29 years), who had had diabetes mellitus for many years (14-23 years) was studied by light and electron microscopy. The eight enucleated eyes were blind and painful as a complication of diabetes mellitus. Histopathologically, the choriocapillaris and other small choroidal blood vessels disclosed marked basement membrane thickening of their walls. Periodic acid-Schiff-positive homogeneous acellular nodules were present and resembled those of diabetic glomerulosclerosis (Kimmelsteil-Wilson disease). Some choroidal arteries were arteriosclerotic. Choroidal compromise was suggested by luminal narrowing of the capillaries, capillary dropout, and focal scarring. Choroidal neovascularization with subretinal fibrovascular membranes occurred in two patients at the midperiphery and periphery, and resembled those of retinitis proliferans. Leakage of proteinaceous fluid into the choroidal stroma and beneath the focally detached pigment epithelium was suggested by the electron microscopic observations. Choroidal vasculopathy in diabetes mellitus is similar to much of what has been described in other tissues of the eye and body, and suggests an important role in the pathogenesis of diabetic retinopathy since the outer retinal layers are largely dependent on the choroid for their nutrition and oxygenation.

Adult

Necrotizing scleritis. A clinico-pathologic study of 41 cases.

Scleritis is not a single clinical or pathologic entity. It has several distinct forms. A clinico-pathologic study of 41 cases of necrotizing scleritis suggests that these different histopathologic forms of disease may reflect different mechanisms of immunopathogenesis. These cases were divided into three main groups: (1) scleral inflammations associated with various systemic autoimmune diseases, including 11 cases of rheumatoid arthritis, three cases of Wegener's granulomatosis, one case of polychondritis, and one case of Goodpasture's syndrome; (2) infectious scleritis, consisting of four cases of herpes zoster ophthalmicus and two cases of pseudomonas scleritis; and (3) idiopathic scleritis, without evidence of systematic disorder, consisting of 19 cases. The first group exhibited predominantly necrosis of the sclera surrounded by granulomatous inflammation and vasculitis. None of these cases showed lymphoid follicles, or healing attempts manifested by proliferation of fibroblasts and blood vessels at the site of inflammation. The idiopathic group revealed few small foci of scleral necrosis and mainly non-granulomatous inflammation. In addition, there was evidence of proliferation of granulation tissue and lymphoid follicles in this group of eyes.

Adult