title: Nissen fundoplication in the treatment of children with familial dysautonomia.
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.
authors.affiliation: Department of Pediatric Surgery, Hadassah University Hospital, Mount Scopus, Jerusalem, Israel.
authors.forename: R
authors.initials: R
authors.lastname: Udassin
authors.forename: D
authors.initials: D
authors.lastname: Seror
authors.forename: I
authors.initials: I
authors.lastname: Vinograd
authors.forename: O
authors.initials: O
authors.lastname: Zamir
authors.forename: S
authors.initials: S
authors.lastname: Godfrey
authors.forename: S
authors.initials: S
authors.lastname: Nissan
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003681:Dehydration / Q000517:prevention & control; D004402:Dysautonomia, Familial / Q000601:surgery*; D015154:Esophageal Motility Disorders / Q000601:surgery*; D005183:Failure to Thrive / Q000517:prevention & control; D005260:Female; D005500:Follow-Up Studies; D005748:Gastric Fundus / Q000601:surgery; D005764:Gastroesophageal Reflux / Q000601:surgery*; D005774:Gastrostomy / Q000379:methods; D006801:Humans; D007223:Infant; D008297:Male; D010343:Patient Admission; D011015:Pneumonia, Aspiration / Q000517:prevention & control; D011183:Postoperative Complications; D001261:Pulmonary Atelectasis / Q000209:etiology; D014839:Vomiting / Q000517:prevention & control