PHACE/S syndrome: A syndromic infantile segmental hemangioma.

PHACES syndrome is a neurocutaneous disorder characterized by posterior fossa brain malformations, hemangiomas, cardiac anomalies and coarctation of aorta, eye anomalies ± sternal clefts. All reported cases are sporadic and notably common in females. The underlying cause is unknown. Here is described, one of the twin baby with characteristic features of PHACE syndrome. The presence of large segmental hemangioma, especially on face should prompt the primary care provider to act early, to prevent complications related to facial hemangiomas and other associated anomalies.

Optic nerve sheath decompression for visual loss in intracranial hypertension: Report from a tertiary care center in South India.

Aim: Severe visual loss is the only serious complication of intracranial hypertension secondary to idiopathic intracranial hypertension (IIH) and some cases of cerebral venous thrombosis (CVT). Optic nerve sheath decompression (ONSD) has been shown to improve or stabilize visual function in patients with IIH, while its role in CVT is yet to be established. We report our experience with optic nerve sheath decompression for visual loss in IIH and CVT. Materials and Methods: In this prospective noncomparative, interventional study, 41 eyes of 21 patients with IIH and CVT and visual loss underwent ONSD. The main outcome measures included best-corrected visual acuity (BCVA), visual fields, pupillary light reflex, optic nerve sheath diameter on B-scan and resolution of papilledema which were evaluated preoperatively and at follow-up at four days, two weeks, one month, three months and final follow-up. In 7/41 eyes with absent light perception preoperatively, the functional outcome was analyzed separately. Results: Following ONSD BCVA and visual fields stabilized or improved in 32/34 (94%) eyes. Statistically significant improvement in BCVA, visual fields and pupillary light reflex occurred over the three month follow-up period. Surgical success was indicated by reduction in optic nerve diameter and papilledema resolution occurred in all patients. The outcome in the IIH and CVT groups was comparable. Four eyes with absent light perception showed marginal improvement in visual acuity. Four eyes had transient benign complications. Conclusion: Optic nerve sheath decompression is an effective and safe procedure to improve or stabilize vision in patients with visual loss caused by IIH and CVT.

Rhino-orbito-cerebral mucormycosis. A retrospective analysis of clinical features and treatment outcomes.

OBJECTIVE: The conventional management of rhino-orbito-cerebral (ROC) mucormycosis includes control of metabolic abnormality, administration of amphotericin B and surgery that spans simple sinus clearance, radical debridement and orbital exenteration. Recent literature includes anecdotal descriptions of successful treatment with conservative management of involved orbits. We evaluated the clinical features and outcome of treatment for the different stages of ROC mucormycosis. METHOD: In this retrospective case series, 34 case records of patients with a histopathological diagnosis of ROC mucormycosis treated between 1992 and 2000 were reviewed. Three clinical stages and three treatment groups were identified. Patients with limited sino-nasal disease (Clinical stage I) underwent sino-nasal debridement (Treatment group A). Patients with limited rhino-orbital disease (Clinical stage II) underwent either sino-nasal debridement alone (Treatment group A) or orbital exenteration in addition to sino-nasal debridement (Treatment group B). Patients with rhino-orbito-cerebral disease (Clinical stage III) did not undergo any surgical procedure (Treatment group C). Thirty-three patients received intravenous amphotericin B. Outcome for each group was measured as "Treatment success" (disease free, stable patient with metabolic abnormality under control) and "Treatment failure" (progression of disease with worsening general condition or mortality due to the disease). RESULTS: Uncontrolled diabetes in 30 (88.2%) of 34 patients was the commonest underlying disease and 16 (53.3%) of 30 diabetics had ketoacidosis. Chronic renal failure (n = 4), hepatic disease (n = 3) and idiopathic thrombocytopenia (n = 1) were the other underlying diseases. Eleven patients had stage I disease, 16 patients had stage II disease and seven patients had stage III disease. All 11 patients with stage I disease received treatment A; of 16 patients with stage II disease, 7 received treatment A and the remaining with stage III disease received treatment B; 7 patients with stage II disease received treatment C. Ten of 11 patients (91%) with stage I disease had treatment success. In patients with stage II disease, 7 of 7 (100%) with treatment A and 1 of 9 (11.1%) with treatment B had treatment success. All seven patients with stage III disease had treatment failure. CONCLUSION: Debridement of the sinuses is necessary in all cases of rhino-orbito-cerebral mucormycosis. Diagnosis in the early stage needs a high degree of suspicion. There is a definite role for retention of orbits in patients whose metabolic derangement is rapidly controlled and orbital involvement is non-progressive.