Ophthalmological Aspects of Fronto-Ethmoidal Meningoencephaloceles from the South East Asian Region

Dr Remington Pyne MB, BS, DO(RC&S), FRACO, FRACS

Fronto-Ethmoidal Meningoencephaloceles for reasons unknown, are far more common in South East Asia than in most other regions of the world; and account for 25% of overseas referrals to the South Australian Cranio-Facial Unit. We reviewed twelve consecutive cases presenting to out Unit. All patients were of Malay or Indian origin, no cases were familial, but mean paternal age at the child’s birth was above normal. Cases were assessed by clinical examination, X-rays, with subsequent confirmation at surgery. In all cases the Encephalocele exited via the foramen caecum, with entry on to the face being one of three types; naso-frontal, naso-ethmoid, and naso-orbital. There was considerable medical canthal displacement, moderate globe displacement, and minimal lateral canthal displacement. All cases showed an elongated face. The Encephalocele appears to act as a foreign body displacing the eyes laterally, and the md-face downwards, acting quite differently to normal facial clefts. Ocular abnormalities included hypertelorism, telecanthus, non-functioning lacrimal apparatus, orbital dystopia, and squint. Binocular vision was usually present. Surgery involved shifting one or both orbits, or midial orbital walls only, to correctly position the eyes. Complications were minimal, but included convergent squint, which usually resolved without treatment.