Gastroesophageal fundoplication and gastrostomy in familial dysautonomia

Gastric and esophageal dysfunction are components of familial dysautonomia. The limited success of various medical management programs, has led to two types of surgical intervention. Experience with nine patients who had gastrostomy alone and 12 patients who had gastroesophageal fundoplication is reviewed. Both surgical procedures decreased frequency of vomiting and pneumonias and had positive effects on weight gain. Although "dysautonomic crises" are not eliminated, sufficient modification in character occurs so that associated risks are lessened. It is suggested that if medical management cannot control recurrent pneumonia, postprandial vomiting, esophageal bleeding, and/or inadequate weight gain, then the patient should be evaluated for fundoplication and/or gastrostomy.