Spigelian hernia-cryptorchidism syndrome in a three-month-old male: A case report

Introduction: Spigelian hernia-cryptorchidism syndrome is an exceptionally rare congenital association, with fewer than 100 pediatric cases reported since it was first described by Schoofs in 1895 and an estimated incidence below 1: 1,000,000 live births. Case presentation: A three-month-old infant, born at term after an uneventful pregnancy, was referred with a right-sided abdominal swelling first noted at four weeks of age. Clinical examination revealed a soft mass in the right lower abdomen lateral to the rectus border. The ipsilateral testis was absent from the scrotum but was palpable in the cranial part of the swelling. Sonography demonstrated a hernial sac measuring 3.6 × 5.6 cm containing small-bowel loops and the right testis between the external and internal oblique muscles. Through a 3-cm transverse incision the sac was dissected, ligated and excised. The gubernaculum and superficial external inguinal ring were absent. Primary fascial repair and single-stage Dartos-pouch orchiopexy were performed. The postoperative course was uneventful and, at six-month of follow-up, the repair remained intact with the testis palpable in the scrotum. Conclusion: Spigelian hernia-cryptorchidism syndrome should be suspected in patients who have a palpable mass in the lower abdominal wall, ipsilateral to an absent testicle.