Congenital bilateral absence of the vas deferens

A 10-year-old boy with cystic fibrosis (CF) (DeltaF508/G551D mutation) underwent an uneventful elective interval laparoscopic appendectomy. During routine laparoscopic inspection of the abdomen and groins, congenital bilateral absence of the vas deferens was noted. Pictures of the patient's internal inguinal ring noted at time of laparoscopy are presented and compared with a similar-aged patient's internal ring with a normal vas deferens. The genetics of CF patients associated with congenital bilateral absence of the vas deferens is reviewed. The pediatric or general surgeon performing a herniorrhaphy should be aware of this anomaly in CF patients.