Giant Umbilical Hernia in a Patient with Hurler's Syndrome: Case Report and Management Principles
<p>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...
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Global Journal of Medical and Clinical Case Reports - Peertechz Publications,
2020-05-01.
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| Zusammenfassung: | <p>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.</p> |
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| DOI: | 10.17352/2455-5282.000088 |