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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Topics |
- Adolescent
- Adult
- Child
- Child, Preschool
- Dehydration
(prevention & control)
- Dysautonomia, Familial
(surgery)
- Esophageal Motility Disorders
(surgery)
- Failure to Thrive
(prevention & control)
- Female
- Follow-Up Studies
- Gastric Fundus
(surgery)
- Gastroesophageal Reflux
(surgery)
- Gastrostomy
(methods)
- Humans
- Infant
- Male
- Patient Admission
- Pneumonia, Aspiration
(prevention & control)
- Postoperative Complications
- Pulmonary Atelectasis
(etiology)
- Vomiting
(prevention & control)
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