Unusual case of incarcerated umbilical hernia

Umbilical hernias are a common surgical diagnosis in infants and young children. Spontaneous closure of umbilical hernias with a small ring diameter occurs in the majority of cases before the age of 4. Umbilical hernias are usually observed in patients with Down syndrome, trisomy 18 and 13, mucopolysaccharidosis and congenital hypothyroidism. This case report describes surgical treatment of an incarcerated umbilical hernia in an 11-year-old boy with mucopolysaccharidosis type I (MPS I). Surgical exploration revealed gangrenous appendicitis and peritonitis as the cause of incarceration in the umbilical hernia. Intestinal reduction, appendectomy, hernia repair and umbilicoplasty were done without perioperative complications, which are more frequent in this group of patients. In selected MPS I cases, it may be wise to perform elective hernia repair soon after diagnosis in order to prevent complications.

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