Nissen fundoplication in the treatment of children with familial dysautonomia.
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| Abstract |
Thirty-four children with familial dysautonomia (FD) underwent Nissen fundoplication and gastrostomy. The indications for operation were persistent cyclic vomiting that resulted in repeated aspiration pneumonia (94% of the patients), chronic dehydration (82%), failure to thrive (97%), and frequent hospitalizations (76%). There was no operative or early postoperative mortality. Long-term follow-up for up to 12 years was available. Eight patients died during this period from 7 months to 7.5 years postoperatively. In 5 patients (15% of the operated patients), the fundoplication ceased to function 16 months to 5 years postoperatively, which was attributed mainly to repeated severe dysautonomic crises with vigorous retching. Vomiting ceased in 85% of the symptomatic patients; pulmonary deterioration was halted, and the frequency of aspiration pneumonia was reduced in 68%; nutritional improvement was seen in 44%; the hydration status improved in 88%; and the frequency of hospital admissions decreased in 74%. These long-term findings resulted in a significant improvement in the quality of life for the majority of the patients. The absence of operative mortality and the low postoperative morbidity, together with the long-term beneficial results of this surgical procedure, should encourage early surgical intervention in selected FD patients.
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| Authors | R Udassin, D Seror, I Vinograd, O Zamir, S Godfrey, S Nissan |
| Journal | American journal of surgery (Am J Surg) Vol. 164 Issue 4 Pg. 332-6 (Oct 1992) ISSN: 0002-9610 [Print] United States |
| PMID | 1415939 (Publication Type: Journal Article) |
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