Giant Umbilical Hernia in a Patient with Hurler’s Syndrome: Case Report and Management Principles

Abstract

The surgical and anesthetic management of patients with giant umbilical hernia associated with Hurler’s disease is a challenge due to the many problems. We present a recent case of giant umbilical hernia with skin necrosis accompanying Hurler’s syndrome. In addition, a literature review was conducted using the PubMed, Embase, Ovid, Scopus and Cochrane databases. The literature review identified 3 children documented in 3 publications throughout history. The observation we report proves that the umbilical hernia accompanying Hurler’s syndrome can reach an impressive size with a risk of complication, making surgical repair very difficult with a high risk of recurrence. Spinal anaesthesia is contraindicated due to the associated spinal malformations. Laryngoscopy and fibre-optic bronchoscopy can be used for intubation in difficult cases, and the laryngeal mask has proven to be a useful additional aid. Despite the risk, surgical repair is mandatory.