title: Cyanotic breath-holding spell: a life-threatening complication after radical resection of a cervicomedullary ganglioglioma.
abstract: Cyanotic breath-holding spell is a benign and self-limiting disease of young children but occasionally associated with sudden, unexpected death. The authors report a rare case in a 2-year-old girl with a severe form that started after radical resection of a cervicomedullary ganglioglioma. She was admitted to our hospital because of delayed and unstable gait. Since magnetic resonance imaging showed a cervicomedullary tumor, she underwent a radical resection and histology showed the tumor to be a ganglioglioma. Postoperatively, the function of the lower cranial nerves and cerebellum deteriorated and hemiparesis on the left became apparent, but she returned to the preoperative state in a few months. In addition, mild sleep apnea (Ondine curse) and severe cyanotic breath-holding spells occurred. The former responded to medication but the latter failed and continued several times per day with a rapid onset and progression of hypoxemia, loss of consciousness, sweating and opisthotonos. Five months after the operation, the patient returned home with a portable oxygen saturation monitor equipped with an alarm. This case indicates that cyanotic breath-holding spell, as well as sleep apnea, is critical during the early postoperative period. This is the first report observing that such spells may occur as a complication of radical resection of a cervicomedullary tumor.
authors.affiliation: Department of Neurosurgery, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan. fujisawa@ns.m.kanazawa-u.ac.jp
authors.forename: Hironori
authors.initials: H
authors.lastname: Fujisawa
authors.forename: Yuya
authors.initials: Y
authors.lastname: Yoshida
authors.forename: Yo
authors.initials: Y
authors.lastname: Niida
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Hasegawa
authors.forename: Junkoh
authors.initials: J
authors.lastname: Yamashita
mesh_terms: D001049:Apnea / Q000150:complications / Q000209:etiology* / Q000517:prevention & control; D001932:Brain Neoplasms / Q000601:surgery*; D002675:Child, Preschool; D003490:Cyanosis / Q000209:etiology* / Q000517:prevention & control; D005260:Female; D018303:Ganglioglioma / Q000601:surgery*; D006801:Humans; D000860:Hypoxia / Q000209:etiology / Q000517:prevention & control; D010102:Oxygen Inhalation Therapy; D011183:Postoperative Complications; D013035:Spasm / Q000209:etiology / Q000517:prevention & control; D013546:Sweating; D014474:Unconsciousness / Q000209:etiology / Q000517:prevention & control