ESOPHAGEAL ATRESIA WITH DISTAL TRACHEOESOPHAGEAL FISTULA IN A PATIENTWITH FRONTO-METAPHYSEAL DYSPLASIA

Fronto-metaphyseal dysplasia (FMD) is an uncommon but clinically strik ing condition affecting bone and connective tissue. The terms used to define this syndrome fail to cover all the reported findings, the abno rmalities not being confined to the metaphyses and to the frontal bone s. We report on a patient who, in addition to the clinical manifestati ons characteristic of the syndrome, showed esophageal atresia with dis tal tracheoesophageal fistula. Particular emphasis is given to the ext raskeletal manifestations of the syndrome reported in the literature. (C) 1997 Wiley-Liss, Inc.