GASTROINTESTINAL ABNORMALITIES - A SIGNIFICANT CAUSE OF FEEDING DIFFICULTIES AND FAILURE-TO-THRIVE IN BRACHMANN-DELANGE SYNDROME

Gastroesophageal abnormalities occur with increased frequency in patie nts with Brachmann-de Lange syndrome (BDLS) and contribute to problems with feeding, emesis and failure to thrive. Comprehensive evaluation including longitudinal assessment of growth and development of 8 patie nts with BDLS was performed. Clinically significant feeding problems o ccurred in 6 of the 8 patients and the affected children were subseque ntly evaluated for gastrointestinal abnormalities. Findings in these p atients included tracheal aspiration, esophageal dysmotility, gastroes ophageal reflux, hiatal hernia, and esophagitis. Medical treatment was instituted where appropriate, and surgical treatment was performed if the problems did not resolve with medical treatment. Improvement in w eight centiles occurred in all patients fed by nasogastric or feeding gastrostomy tube but only one patient appeared to experience increase in rate of linear growth. Careful monitoring of symptoms and growth pa rameters, and prompt institution of appropriate medical and surgical m easures can improve the health and physical outcome of many patients w ith BDLS. (C) 1993 Wiley-Liss, Inc.