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Letters to the Editor Indian Pediatrics 2004; 41:863-864 |
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Giant Occipital Meningocele as a Presenting Feature of Dandy-Walker Syndrome |
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A 10-month-old female infant, born of nonconsanguineous parents, full-term normal delivery presented with a large swelling over the occipital region since birth. The child could not hold the head. She was playful and recognized parents but could not sit or stand on her own. There were no other congenital abnormalities or family history of neural tube defects. On examination, there was frontal bossing, hypertelorism and mild ‘sun-setting’ sign. The anterior fontanelle was lax. Local examination revealed a large, pear shaped, pedunculated swelling in the occipital region. It was brilliantly transilluminant, nontender, fluctuant and non-pulsatile. There was no bruit. The physical and mental milestones were delayed. Neurological examination showed no gross motor or sensory deficit. Magnetic resonance (MR) imaging of the brain showed features suggestive of a Dandy-Walker malformation. The posterior fossa cyst formed a giant occipital meningocele with few incomplete septae within. A primary repair of the meningocele was performed. Cranioplasty was not performed. The child developed fulminant bacterial meningitis on the 10th postoperative day and later succumbed. In 1887, Sutton was the first to describe the association of Dandy-Walker syndrome with occipital meningoencephalocystocele on autopsy (1). The incidence varies between 11-16% in reported series. It might simply be an expression of increased intracystic pressure compensating for the raised intra-cranial pressure during fetal life. The elasticity and the compliance of the meningocele sac acts like a vent for CSF and is probably life saving preventing dangerous elevation of intracranial pressure. The grotesque external appearance is due to the centrifugal expansion of the occipital meningocele The presence of incomplete internal septae caused a ‘trap door’ effect and consequent further expansion of the meningocele sac. The difficulties of feeding, handling and ordinary care are great as well as the psychic trauma to parents in the presence of disfiguring and unconcealable mass, that earliest possible excision is highly desirable. The anterior fontanelle was lax and the sutures were wide open despite ventriculomegaly. It is a matter of specula-tion whether an initial cystoperitoneal shunt would have appreciably reduced the giant size of the meningocoele. There is a increased likelihood of developing decubital ulcers due to restricted head movement caused by the occipital lesion in addition to the high probability of shunt infection and frequent shunt malfunction(2). It is also postulated that resection of the cyst wall rarely gives. permanent relief since there is a generalized dysgenesis(3). A ventriculo-peritoneal shunt may be inserted for symptomatic progressive hydrocephalus and/or associated aqueductal stenosis. D.P. Muzumdar,
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