[Anesthetic management of a patient with Maffucci syndrome for removal of a pituitary adenoma].
Maffucci syndrome is a rare syndrome characterized by multiple enchondromas and hemangiomas seen in various tissues and organs. We report anesthetic management of a 33-year-old man with multiple hemangiomas due to Maffucci syndrome undergoing removal of a pituitary adenoma. The preoperative examination revealed multiple hemangiomas in the head, neck, right upper and lower extremities, the tongue and the pharynx. In the operating room, we observed the locations of hemangiomas in the tongue and pharynx in detail using a fiberoptic bronchoscope before induction of anesthesia. Since there was a risk of rupture of a large hemangioma by using a laryngoscope, we planned fiberoptic bronchoscope-guided tracheal intubation without using a laryngoscope. After intravenous administration of propofol and rocuronium, a fiberoptic bronchoscope was orally introduced into the trachea and then the trachea was intubated with a flexible spiral endotracheal tube, preventing contact of the fiber or endotracheal tube with the hemangiomas. In addition, hemangiomas on the body surface were wrapped up with soft dressing to prevent rupture. Surgery was uneventfully completed. The trachea was carefully extubated, and bleeding from hemangiomas was not observed. Since patients with Maffucci syndrome have multiple hemangiomas, it is important to check for the presence of a hemangioma in the upper airway in anesthetic management.
Department of Anesthesiology and Resuscitology, Shinshu University School of Medicine, Matsumoto 390-8621.
Eriko
E
Imai
Tomoyuki
T
Kawamata
Katsumi
K
Yamamoto
Mariko
M
Mochidome
Mikito
M
Kawamata
D000236:Adenoma / Q000601:surgery*; D000328:Adult; D000769:Anesthesia, Inhalation / Q000379:methods*; D001999:Bronchoscopy; D004687:Enchondromatosis / Q000150:complications*; D006391:Hemangioma / Q000150:complications; D006801:Humans; D007442:Intubation, Intratracheal / Q000379:methods*; D008297:Male; D055100:Optical Fibers; D010911:Pituitary Neoplasms / Q000601:surgery*