oa Southern African Journal of Anaesthesia and Analgesia - Alternative anaesthetic management of an infant with Wolf-Hirschhorn syndrome during repair of an atrial septal defect using total intravenous anaesthesia : letter to the editor

Volume 16, Issue 5
  • ISSN : 2220-1181
  • E-ISSN: 2220-1173



Wolf-Hirschhorn syndrome (WHS) is a rare chromosomal disorder caused by a deletion of the short arm of chromosome 4 with an incidence of 1:50 000 births and a 2:1 female preponderance. Characteristic features of WHS are craniofacial dysmorphisms such as microcephaly, micrognathia, prominent glabella ("Greek helmet facies"), dysplastic ears, preauricular tags, hypertelorism, broad and/or beaked nose, short philtrum, cleft palate, down-turned corners of the mouth and dental anomalies. Further clinical features are low birth weight and developmental delay, mental and motoric retardation, muscular hypotonia, congenital heart defects, feeding, genital and renal anomalies, and seizures. However, many children with this disease require general anaesthesia for diagnostic or therapeutic procedures such as magnetic resonance imaging (MRI), anti-reflux surgery, dental surgery, repair of a cleft palate or urological surgery.

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