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Postoperative complications in patients with oculopharyngeal muscular dystrophy: a retrospective study

[Complications postopératoires chez les patients souffrant de dystrophie musculaire oculo-pharyngée : une étude rétrospective]

From the Département d’anesthésie-réanimation, Centre hospitalier affilié universitaire de Québec, Université Laval, Québec, Québec, Canada.

Address correspondence to: Dr. Hélène G. Pellerin, Département d’anesthésie-réanimation, CHA (Hôpital de l’Enfant-Jésus), 1401, 18e rue, Québec, Québec G1J 1Z4, Canada. Phone: 418-649-5807; Fax: 418-649-5918; E-mail: helenpellerin{at}gmail.com

Purpose: Oculopharyngeal muscular dystrophy (OPMD) is a genetic disease with autosomal dominant transmission particularly common in Quebec where its prevalence is about 1:1000. The main features are bilateral ptosis of the eyelids and dysphagia. These symptoms are frequently treated surgically by levator palpebrae resection (LPR) and cricopharyngeal myotomy (CPM). The objectives of this retrospective chart review were to describe the postoperative complications in OPMD patients undergoing LPR or CPM and to determine their incidence.

Methods: Medical records of all OPMD patients who had either LPR or CPM between 1997 and 2002 were reviewed. The following complications were collected: death, pneumonia, aspiration pneumonitis, airway obstruction, reintubation, and severe coughing and choking caused by profuse secretions.

Results: One hundred and fourteen surgeries on 92 OPMD patients were studied. Fifty-nine were LPR under general anesthesia (LPR-GA) and 22 were LPR under local anesthesia (LPR-LA). Thirty-three surgeries were CPM, all under general anesthesia. There was no death or reintubation. Patients who had LPR-LA had shorter postanesthesia care unit (P < 0.001) and ambulatory surgery unit (P < 0.001) stays than those who had LPR-GA. Complications were more frequent after CPM than LPR-GA (P < 0.001).

Conclusion: The complication rate after LPR was low. Both local and general anesthesia are safe alternatives in OPMD patients operated for LPR. Patients having CPM presented more respiratory complications than those having LPR.