• Takayuki Yoshida, Kenta Furutani, Takeshi Hashimoto, Miki Taneoka, Toshiyuki Tobita, and Hiroshi Baba.
• Department of Anesthesiology, Niigata University Medical and Dental Hospital, Niigata 951-8520.
• Masui. 2010 Oct 1;59(10):1280-3.

AbstractWe report anesthetic management of a 6-month-old boy with Menkes disease who underwent three surgeries for vesicoureteral reflux, rupture of the bladder diverticulum, inguinal hernia, and gastroesophageal reflux. Menkes disease is a rare sex-linked disorder of copper absorption and metabolism. Anesthetic management of such patients is rather challenging because of high incidence of seizures, gastroesophageal reflux with the risk of aspiration, hypothermia, airway and vascular complications. In our patient general anesthesia was uneventfully maintained by sevoflurane combined with intravenous remifentanil and fentanyl. We experienced no major complications except some difficulties with intravenous and arterial cannulation. It was especially difficult to establish intravenous and invasive blood pressure lines because of tortuous blood vessels in this patient. We conclude that in patients with Menkes disease scheduled for surgery intravenous access should be established before the induction of general anesthesia. The necessity of invasive blood pressure monitoring should be also carefully considered beforehand.

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