Anesthetic management of a child with acrocallosal syndrome

Anesthetic management of a child with acrocallosal syndrome S ir —Acrocallosal syndrome is an autosomal recessive condition, characterized by agenesis or hypoplasia of the corpus callosum, pre‐ and postaxial polydactyly, and, in most patients, moderate to severe psychomotor retardation with hypotonia ( 1 ), first described by Schinzel ( 2 ). Other features have also been described; minor craniofacial anomalies (prominent forehead, hypertelorism, short nose with anteverted nostrils, large anterior fontanelle), intracranial cysts and umbilical or inguinal hernia ( 3 ). At present, there is no test for the syndrome and the diagnosis is made from the physical characteristics. At least 39 patients with acrocallosal syndrome have been reported till date in the world literature ( 4 ). We describe the anesthetic management of a child with acrocallosal syndrome. An 8‐year‐old boy, 11 kg in weight (<3rd centile) was scheduled for surgical correction of syndactaly. He was born by Cesarian section at term following an uncomplicated pregnancy. Acrocallosal syndrome was diagnosed at 2 years of age and he had mental and growth retardation. Physical examination revealed forehead hypertelorism, depressed and broad nose, low set ears, high arched palate, preaxial polydactyly of the right hand, syndactyly of left feet and hypogenitalism. Preoperative laboratory evaluations were all within http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Pediatric Anesthesia Wiley

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Publisher
Wiley
Copyright
© 2008 The Authors. Journal compilation © 2008 Blackwell Publishing Ltd
ISSN
1155-5645
eISSN
1460-9592
DOI
10.1111/j.1460-9592.2008.02568.x
pmid
18811850
Publisher site
See Article on Publisher Site

Abstract

S ir —Acrocallosal syndrome is an autosomal recessive condition, characterized by agenesis or hypoplasia of the corpus callosum, pre‐ and postaxial polydactyly, and, in most patients, moderate to severe psychomotor retardation with hypotonia ( 1 ), first described by Schinzel ( 2 ). Other features have also been described; minor craniofacial anomalies (prominent forehead, hypertelorism, short nose with anteverted nostrils, large anterior fontanelle), intracranial cysts and umbilical or inguinal hernia ( 3 ). At present, there is no test for the syndrome and the diagnosis is made from the physical characteristics. At least 39 patients with acrocallosal syndrome have been reported till date in the world literature ( 4 ). We describe the anesthetic management of a child with acrocallosal syndrome. An 8‐year‐old boy, 11 kg in weight (<3rd centile) was scheduled for surgical correction of syndactaly. He was born by Cesarian section at term following an uncomplicated pregnancy. Acrocallosal syndrome was diagnosed at 2 years of age and he had mental and growth retardation. Physical examination revealed forehead hypertelorism, depressed and broad nose, low set ears, high arched palate, preaxial polydactyly of the right hand, syndactyly of left feet and hypogenitalism. Preoperative laboratory evaluations were all within

Journal

Pediatric AnesthesiaWiley

Published: Oct 1, 2008

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