Obstructed umbilical hernia in a child with Hurler's syndrome

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