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眼咽型肌营养不良症患者行食管上括约肌肌切开术:长期临床结果

Upper esophageal sphincter myotomy in oculopharyngeal muscular dystrophy: long-term clinical results.

作者信息

Fradet G, Pouliot D, Robichaud R, St-Pierre S, Bouchard J P

机构信息

Department of Otolaryngology, Hôpital de l'Enfant-Jésus, Québec, Canada.

出版信息

Neuromuscul Disord. 1997 Oct;7 Suppl 1:S90-5. doi: 10.1016/s0960-8966(97)00090-4.

Abstract

From 1980 to 1995, 53 patients with oculopharyngeal muscular dystrophy (OPMD) underwent an upper esophageal sphincter (UES) myotomy for the control of marked dysphagia. From this number, a group of 21 patients had been evaluated for preoperative and postoperative symptoms in 1987. The same clinical assessment was performed in 1995 by an independent evaluator for a total of 37 patients including 12 patients from the first group. As a whole, after a mean follow-up of 6.2 years, surgery succeeded in 18 patients (49%), gave a partial improvement in 12 (32%) and failed in seven (19%). The 12 patients evaluated twice (in 1987 and 1995) have had very good early results, 8-69 months after UES myotomy: dysphagia was totally relieved in eight patients, occurred rarely in three and was moderate in one. Nevertheless, the very long-term follow-up (8 years later) has shown a recurrence of the swallowing and tracheobronchial symptoms in many cases.

摘要

1980年至1995年间,53例眼咽型肌营养不良症(OPMD)患者接受了食管上括约肌(UES)切开术以控制严重吞咽困难。其中,1987年对21例患者进行了术前和术后症状评估。1995年,由一名独立评估者对总共37例患者进行了同样的临床评估,其中包括第一组中的12例患者。总体而言,平均随访6.2年后,手术成功18例(49%),部分改善12例(32%),失败7例(19%)。12例接受两次评估(分别在1987年和1995年)的患者在UES切开术后8至69个月取得了非常好的早期效果:8例患者吞咽困难完全缓解,3例很少出现,1例为中度。然而,长期随访(8年后)显示,许多病例出现了吞咽和气管支气管症状复发的情况。

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