Sánchez RJP, Guzmán NR, Rionda DE, Quintero GMA, Jiménez CAV

Language: Spanish
References: 10
Page: 50-53
PDF size: 99.66 Kb.

ABSTRACT

The Ehlers–Danlos Syndrome, is an hereditary disorder of the connective-tissue. It can affect skin, joints and blood vessels. It is characterized by joint hypermobility, cutaneous fragility and hyperextensibility, caused by a defect in collagen synthesis. Although, clinical manifestations can appear later on, it is a congenital defect. Affected individuals have a higher mortality risk, due to spontaneous organ and vessel rupture; during pregnancy, trauma, iatrogenic events, and during some procedures like arteriography, orotracheal intubation and endoscopy. The diagnosis is based on clinical manifestations and family history of the disease. We report a case of an eight year old male postoperated patient of spinal instrumentation and diaphragmatic lesion with Ehlers-Danlos Syndrome. He was brought to the ABC Medical Center for surgical treatment of a bowel obstruction. The general anesthesia technique, was balanced anesthesia with rapid sequence induction. Opioids and sevoflurane was used for maintenance of anesthesia as well as mechanic ventilation.

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