Strabismus, 17:75–77, 2009 Copyright  c 2009 Informa Healthcare USA, Inc. ISSN: 0927-3972 print / 1744-5132 online DOI: 10.1080/09273970902798490 PHACE Syndrome Associated With Congenital Oculomotor Nerve Palsy Ramesh Murthy, MD, FRCS Strabismus and Pediatric Ophthalmology, and Oculoplasty and Ocular Oncology Service, L V Prasad Eye Institute, Kallam Anji Reddy Campus, Hyderabad, India Milind N. Naik, MD, Savari Desai, MD, and Santosh G. Honavar, MD, FACS Oculoplasty and Ocular Oncology Service, L V Prasad Eye Institute, Kallam Anji Reddy Campus, Hyderabad, India ABSTRACT PHACE syndrome is a multisystem disorder presenting with facial hemangiomas, arterial anomalies, cardiac anomalies, posterior fossa malformations and eye abnormalities. The eye abnormalities include microph- thalmos, cataracts, optic atrophy and iris hypoplasia. Amongst the neurological anomalies, posterior fossa malformations are common. Fourth nerve palsy has been reported with PHACE syndrome. We report a child presenting with a triad of congenital third nerve palsy, cerebellar hypoplasia and facial capillary hemangioma. KEYWORDS oculomotor nerve palsy; cerebellar hypoplasia; facial capillary haeman- gioma; PHACE syndrome Oculomotor (third) nerve palsy in infancy and childhood is rare. The known causes in this age group include trauma, migraine, inflammation, neoplasm, and uncommonly neuromas of the oculomotor nerve (Kodsi and Young, 1992). The PHACE syndrome is a rare heterogeneous neurocutaneous disorder with a spectrum of clinical findings including posterior fossa anomalies (P), facial hemangiomas (H), arterial anomalies (A), cardiac anomalies/aortic coarctation (C), and eye abnormalities (E) (Frieden et al., 1996). We report a rare case of PHACE syndrome associated with congenital oculomotor nerve palsy. CASE REPORT A 2-month-old girl presented to the pediatric ophthalmology clinic at our institute with a dark red discoloration and papular elevated lesions on the right side of the face from birth. These were gradually increasing and associated with drooping of the right eyelid. The entire right hemi face, right side of the neck, right arm, and forearm were involved (Fig. 1). There was a large angle exotropia, with limitation of adduction, depression, and severe ptosis. There was anisocoria, with the right pupil being larger. Based on this, a diagnosis was made of capillary hemangioma with right third nerve palsy. The patient was given injection triamcinolone 0.2 ml (40 mg/ml) intralesionally into the right upper lid along with oral prednisolone 1mg/kg body weight on a tapering schedule over 4 weeks and topical beclamethasone ointment for the peribulbar component. Magnetic resonance imaging (MRI) was performed at the age of 6 months, which revealed the presence of right cerebellar hypoplasia, dilated fourth ventricle, and absence of vermis (Fig. 2). There was no enlargement or enhancement of the subarachnoid or intracavernous portion of the oculomotor nerve suggestive Correspondence: Ramesh Murthy, MD, FRCS, Consultant Pediatric Ophthalmology and Strabismus, Oculoplasty and Ocular Oncology, L V Prasad Eye Institute, Kallam Anji Reddy Campus, L V Prasad Marg, Banjara Hills, Hyderabad, India 500034. Tel: 0091 9949563100; Fax: 0091 20 23548271. E-mail: drrameshmurthy@gmail.com 75