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Indian Journal of Surgery
Medknow Publications on behalf of Association of Surgeons of India
ISSN: 0972-2068
Vol. 67, No. 1, 2005, pp. 49-51
Bioline Code: is05010
Full paper language: English
Document type: Research Article
Document available free of charge

Indian Journal of Surgery, Vol. 67, No. 1, 2005, pp. 49-51

 en Obstructed umbilical hernia in a child with Hurler's syndrome
Gopinath Goutham, Nagaraj P, Kulkarni ML

Abstract

Case Report- Hurler's syndrome (MPS I-H) is a lysosomal storage disorder characterized by physical deformities and developmental anomalies and premature death. Deficiency of the enzyme alfa-L-Idronidase results in intralysosomal accumulation of dermatan sulfate and heparan sulfate resulting in cell dysfunction. It manifests as progressive hepatosplenomegaly, cardiac disease, severe skeletal abnormalities, hydrocephalus and mental retardation. Umbilical and inguinal hernias are common in this disorder. Obstruction of hernia in these children is rare because of wide hernial ring. This is a discussion on a child with Hurler's syndrome who presented with obstructed umbilical hernia. Surgery was performed under general anaesthesia after relieving the obstruction conservatively. Surgical and anaesthetic problems in this syndrome are discussed in the present communication.

Keywords
Hurler's syndrome, Mucopolysaccharidoses, Umbilical hernia, Mayo's repair

 
© Copyright 2005 Indian Journal of Surgery.

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