Isolated corneal pseudodendrites as the initial manifestation of tyrosinemia type II in monozygotic twins.

Fifteen-month-old twins presented with photophobia and bilateral corneal pseudodendrites, and tyrosinemia type II was suspected. Plasma tyrosine levels were elevated. After therapy with tyrosine-restricted diet, corneal lesions resolved. Bilateral pseudodendritic keratitis may be the initial or only manifestation of tyrosinemia type II.

Kaposi's Sarcoma of the Bulbar Conjunctiva in an Immunocompetent Patient.

Kaposi's sarcoma is an uncommon endothelial malignant tumor, first described by Moricz Kaposi in 1872. It is commonly encountered in patients with acquired immunodeficiency syndrome (AIDS), immunosuppression or organ transplantation. Conjunctival Kaposi's sarcoma is a rare tumor, mostly in patients with AIDS. We present a selected case with a superior bulbar conjunctival lesion in a patient who is HIV negative and immunocompetent, with an unremarkable general medical history. The lesion was surgically excised with no adjunctive treatment, and histological examination confirmed the diagnosis. There was no recurrence or metastasis 5 years after surgery.

Childhood orbitotemporal neurofibromatosis masked by congenital glaucoma and buphthalmos.

In newborns with unilateral buphthalmos and presumed congenital glaucoma, the differential diagnosis should include the rare condition of orbitotemporal neurofibromatosis. The authors present the diagnostic challenge of an unusual case of neurofibromatosis type 1 with unilateral congenital glaucoma, buphthalmos, and sphenoid-orbital dysplasia. The accompanying type of osseous orbital dysplasia has rarely been described in the literature, complicating the diagnostic and management process.

Unilateral optic disc edema in a paediatric patient: diagnostic dilemmas and management.

Introduction. We report a case of unilateral optic disc edema in a paediatric patient and discuss the concerns involved in diagnosis and management of similar cases. Materials and Methods. Female aged 10 years was referred to our clinic due to progressive visual loss of the LE over a few days. Her visual acuities (VA) were RE 10/10, LE 3/10, and she had a relative afferent pupillary defect and decreased colour vision in her LE and normal and painless eye movements. Fundoscopy showed a remarkably swollen disc of the LE, and visual field (VF) examination revealed enlargement of the blind spot and presence of horizontal inferior papillomacular scotoma. Neurological examination, CT of brain and orbits and blood tests were normal. Visual evoked potentials revealed an obstacle in the myelin substance before the optic chiasma of the LE. Results. The patient was treated with intravenous methylprednoslone for 3 days and with oral methylprednizole for 15 days in progressively diminished daily doses. This led to gradual improvement of VA, colour vision, and visual field and resolution of optic disc oedema. Discussion. Concerns that have to be taken into account regarding diagnosis and management of similar cases are related to lumbar puncture indications, treatment with corticosteroids, and appropriate followup.

Cryopreserved amniotic membrane transplantation for the management of symptomatic bullous keratopathy.

BACKGROUND: To report the results of cryopreserved human amniotic membrane transplantation for the management of symptomatic bullous keratopathy. METHODS: Prospective non-comparative interventional case series study. Consecutive cases with symptomatic bullous keratopathy for more than 12 months not amenable to conservative treatment were managed with amniotic membrane transplantation. They were recruited over a 5-year period (September 1999 to November 2004) in one referral centre. Only one eye of each patient (the worse affected eye in bilateral cases) was operated. A 360 degree conjunctival peritomy was followed by removal of the diseased corneal epithelium. Amniotic membrane was transplanted over the cornea as a patch and sutured to the free conjunctival edges. Primary outcome measures were ocular pain and epithelial defects; secondary measures were visual acuity and ocular surface inflammation. RESULTS: Four out of 85 recruited cases did not complete the minimum observation of 12 months and were excluded from the study. The mean follow-up period for the remaining 81 cases was 21 +/- 4.2 months (range 14-34 months). Seventy-one (87.6%) eyes became asymptomatic with healed epithelium, seven required repeated amniotic transplantation and three underwent penetrating keratoplasty. Visual acuity improved in 64 (79%) patients and remained unchanged in 14. No complications were recorded. CONCLUSIONS: Amniotic membrane transplantation is an efficient and safe treatment for symptomatic bullous kerato pathy, when penetrating keratoplasty is not available. It has been shown to alleviate pain, promote corneal epithelialization and reduce conjunctival inflammation whereas in some cases it may also improve visual acuity.