BILATERAL ULNAR AGENESIS - CASE-REPORT AND REVIEW OF THE LITERATURE

Bilateral ulnar agenesis is a rare abnormality. A total of 36 cases ar e analyzed: 35 of these are documented in the literature and 1 stillbo rn male is presented in this study. Most patients had one of the three conditions: Al-Awadi/Raas-Rothschild syndrome, syndrome of ulnar apla sia with split hand/split foot deformity, or the Brachmann-de Lange sy ndrome. Fifty percent of all cases with bilateral ulnar agenesis were associated with lower limb defects and these cases, for the most part, also belonged to the aforementioned syndromes. Nonskeletal, internal organ malformations were identified in 34% of aa patients. Nine patien ts presented With isolated bilateral ulnar agenesis. The Al-Awadi/Raas -Rothschild Syndrome and the split hand/split foot deformity are herit able disorders. There was no evidence for genetic etiology in most of the other cases. Bilateral ulnar agenesis in our fetus was part of the Brachmann-de Lange syndrome with associated cardiac defect, diaphragm atic hernia, and umbilical artery agenesis.