Chorioretinal lacuna in the amniotic band syndrome.

The malformations in the amniotic band syndrome (ABS) are due to entrapment of fetal parts by fibrous band in the amniotic sac. Limbs are most commonly affected followed by craniofacial defects in one third of patients. Ocular defects include corneal leukomas and lid colobomas often contiguous with facial clefts, strabismus, hypertelorism, and microphthalmos. Unilateral chorioretinal defects or lacunae are rare findings in the ABS. We report a female infant with such a lacunar defect along with central nervous abnormalities, and discuss the differential diagnosis and the embryopathic implications.

A unique epibulbar osseous choristoma.

The case of a 14-year-old girl found to have a unique epibulbar osseous choristoma attached to the sheath of the superior rectus muscle is recorded. The bony mass was palpable through the upper lid and by computerized tomography, it appeared densely calcified and noncystic. There was no ptosis or abnormality in ocular motility preoperatively or postoperatively.

Bilateral orbital involvement in fatal giant cell polymyositis.

The case of a young woman with giant cell polymyositis is described. She had bilateral, severe, midly painful proptosis and ophthalmoplegia. Extensive pharyngeal, laryngeal, and cardiac muscle involvement occurred 18 months later coincident with fatal cardiac arrhythmia. At autopsy, extensive muscle necrosis and giant cells were noted in extraocular, pharyngeal, laryngeal, and cardiac muscle with only minimal involvement of other striated muscles.

Conservative management of childhood orbital lymphangioma.

Repeated partial excisions are commonly performed for orbital lymphangiomas. Even with the advent of carbon dioxide (CO2) laser as an adjunct to subtotal excision, surgical complications remain common. The authors report six cases of childhood orbital lymphangioma, biopsy proven in five, in which conservative management of recurrent hemorrhage without additional surgery resulted in subsidence of proptosis and return of 20/20 visual acuity. Surgical aggressiveness for recurrent hemorrhages must be tempered by the knowledge that normal vision often results from cautious observation alone.

Early onset acute orbital involvement in childhood acute lymphoblastic leukemia.

A 5-year-old girl with acute lymphoblastic leukemia undergoing induction chemotherapy experienced acute proptosis while agranulocytotic and febrile. Orbital biopsy showed leukemic infiltration, and complete resolution was achieved with local irradiation and chemotherapy. Early onset orbital involvement is highly unusual in acute lymphoblastic leukemia. In the setting of agranulocytosis and fever, rapidly enlarging intraorbital masses require urgent ophthalmologic attention. Immediate biopsy is indicated to distinguish between several treatable conditions including opportunistic infection, hemorrhage, and neoplastic infiltration.

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.

Inaccuracy of fine needle aspiration biopsy.

Fine needle aspiration biopsy (FNAB) of orbital tumors has been recently advocated as a reliable diagnostic modality. An accuracy rate of 92% has been claimed by some authors. This figure remains unproven as most patients have not undergone simultaneous pathologic examination. We recently performed FNAB on 34 patients at the time of surgical biopsy. The procedure was performed with direct visualization and the tissue was obtained and prepared as described by Kennerdell. A definitive surgical biopsy was then obtained and submitted for routine pathologic examination. Cytologic diagnosis has been accurate in less than half of the cases biopsied. Reliance on fine needle aspiration biopsy alone in some patients would have resulted in inappropriate therapy. Fine needle aspiration biopsy should be limited to strongly suspected cases of orbital metastases or secondary tumors.

Wegener's granulomatosis presenting as orbital pseudotumor in children.

The case of a nine-year-old white female who presented initially with idiopathic inflammatory pseudotumor and was ultimately diagnosed (22 months later) as having Wegener's granulomatosis is presented. A review of the literature reveals that, to date, there are 21 well-documented cases of Wegener's granulomatosis occurring in children under the age of 16. Of these 21 cases, eight patients (39%) manifested ocular or orbital signs during the course of the disease. Four of these cases (50%) presented initially with idiopathic inflammatory pseudotumor. The diagnosis of Wegener's granulomatosis should be considered in children with orbital pseudotumor. Long-term follow-up may be necessary to exclude this possibility.

Reversible visual loss in pseudotumor cerebri.

A patient with chronic pseudotumor cerebri experienced precipitous visual loss as a result of a central retinal vein occlusion associated with an intracranial pressure elevation of 520 mm of CSF and a fall of mean systemic arterial pressure. Optic nerve sheath decompression was associated with substantial improvement of vision.

Lymphoid pseudotumour of the orbit.

A retrospective study of 55 patients with orbital lesions diagnosed on clinical grounds as cases of inflammatory pseudotumor was carried out to discover the relationship, if any, between the clinical and histological features and the response to treatment. It was found that few clinical parameters had a bearing on the subsequent progress of the lesion but B-scan ultrasonography was useful in identifying the lymphoproliferative mass. In 28 cases wherein histological material was available, the presence of follicles with germinal centres was found to indicate a good prognosis, whereas the presence of blast forms distributed randomly through the lymphoid tissue was associated with an inadequate response to corticosteroid therapy. Eosinophils were slightly more common in the corticosteroid-responsive lesions, but the presence of other leucocyte types was not helpful in determining prognosis.

Quantitative lacrimal scintillography.

In a study of 100 asymptomatic lacrimal systems, we have found that quantitative lacrimal scintillography provided a sensitive and reproduceable test of canalicular function. This technique, at the present time, appears to have no role in the clinical evaluation of tear passage through the nasolacrimal duct to the nose. A revision of the currently accepted theory of lacrimal drainage is proposed. Lacrimal drainage is best approached as a bimodel system. The first part, from conjunctiva to lacrimal sac, is dependent upon the "lacrimal pump." The second part, by which tears drain from the sac to the inferior meatus of the nose at basal tearflow levels, is dependent on gravity and the resistance of the nasolacrimal duct.

Inflammatory orbital pseudotumor. A clinicopathologic study.

Fifty-five patients, suspected on clinical grounds of having an inflammatory orbital pseudotumor, were studied retrospectively. Clinical groups based on natural history or response to corticosteroid therapy were correlated with available histopathological findings. These findings were interpreted without knowledge of clinical grouping or previous pathology reports. Although dogmatic statements are not possible, several conclusions are warranted. (1) Bilaterality was not significantly related to response to steroids or development of systemic disease. (2) B-scan ultrasonography, while not diagnostic, provided a worthwhile investigative technique. (3) Germinal follicles are associated with a good prognosis and indicate a reactive lesion, while diffusely distributed lymphoblasts are associated with steroid unresponsiveness and a probable neoplastic lymphoid lesions. (4) Eosinophils are more common in reactive lesions than in presumed lymphomas. Other cellular components showed no preferential distribution in either neoplastic or nonneoplastic groups.

Experimental inhibition of prostaglandin-like inflammatory response after cryotherapy.

To determine the effect of aspirin as an inhibitor of the prostaglandin-induced inflammatory reaction that follows cryotherapy, 12 rabbits were pretreated with aspirin and 12 were controls. The aspirin-pretreated animals showed less inflammatory reaction after cryotherapy and significantly less protein in the aqueous humor than did the control animals (P less than .01). We believe that the conjunctival hyperemia and chemosis, and the increase in protein concentrations in the aqueous humor that follow cryotherapy, are prostaglandin-related, because pretreatment with aspirin inhibited these phenomena.