Anesthetic management of a patient with Stickler's syndrome
Okuno, E.; Miyata, Y.; Uemura, T.; Saikawa, S.; Noguchi, N.; Fuchibe, M.; Sugahara, K.
Masui. Japanese Journal of Anesthesiology 59(5): 629-631
2010
ISSN/ISBN: 0021-4892 PMID: 20486578 Document Number: 646672
Stickler's syndrome is an autosomal multisystem disorder accompanying characteristic midface hypoplasia, retromicrognathia, and cleft palate. Mandibular hypoplasia causes difficulties in mask ventilation and endotracheal intubation, especially in infants. A 7-month-old girl diagnosed as Stickler's syndrome was scheduled for the laparoscopic inguinal hernia repair. However, during the direct laryngoscopy for endotracheal intubation, neither the vocal cords nor the epiglottis were visualized. At fifth intubation attempts, the part of the vocal cords was barely visualized, and the tracheal intubation was finaly successful. Anesthesia was maintained with sevoflurane and remifentanil. The patient had an uneventful recovery and was discharged on the second postoperative day without any complications. Sevoflurane and remifentanil allow faster recovery from anesthesia and both have been recommended for patients with difficult tracheal intubation in a patient such as with Stickler's syndrome.