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

Abstract

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.

Oculopharyngeal muscular dystrophy: A polyalanine myopathy

“…Surgery is recommended when ptosis interferes with vision or if the patient has cervical pain. Morbidity is infrequent after ptosis surgery in patients with OPMD [47]. Contraindications to blepharoplasty are marked ophthalmoplegia, a dry-eye syndrome, or poor orbicularis function.…”

“…Severe dysphonia and lower esophageal sphincter incompetence are contraindications to surgery. No mortality was observed in a recent retrospective study of 33 cricopharyngeal myotomies in OPMD cases [47]. Repetitive dilatations of the upper esophageal sphincter with bougies and botulinum toxin injections in the cricopharyngeal muscle are still at the experimental stage [48].…”

“…Two types of operations are used to correct ptosis with overall good results: resection of the levator palpebrae aponeurosis and frontal suspension of the eyelids [47]. Resection of aponeurosis is easily done but usually needs to be repeated once or twice.…”

Mapping the current evidence on the anesthetic management of adult patients with neuromuscular disorders—a scoping review

“…Other studies were on the anesthetic management of patients with myotonic dystrophy type I (DMI) 29 , 30 and II, 31 , 32 amyotrophic lateral sclerosis, 33 – 35 Duchenne muscular dystrophy, 36 glycogen storage disease, 37 inclusion body myositis, 38 Lambert–Eaton myasthenic syndrome, 39 mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes mitochondrial encephalomyopathy 40 and OPMD. 41 Opioid use was possibly associated with postoperative complications in a small retrospective study on the anesthetic management of DMI. 30 In this group of “other studies” the complication rates varied from 0% to 54%.…”

Oculopharyngeal muscular dystrophy or oculopharyngeal distal myopathy: case report

“…The degree of dysphagia presented by the patient is an important prognostic factor of the disease, as these patients progress to malnutrition. 6 …”