Bilateral congenital diaphragmatic hernia associated with brachyesophagus

Objectives: The authors report the case of an infant presenting with a bilateral, postero-internal diaphragmatic hernia associated with a brachyesophagus and vertebral malformation. The surgical care and the follow-up of this malformation complex are discussed. Methods: The antenatal examination identified only a left hernia; complete assessment of the malformation was performed at birth. Repair of the hernias was performed 20 h after birth by suturing the hernial orifices, involving neither prosthetic material nor transposition of the muscle. A Collis gastroplasty procedure was carried out 3 months later with repositioning of the hiatus and Toupet fundoplication. The existence of a thoracic vertebral hemiblock led to the development of scoliosis: the child was submitted to orthopedic treatment at the age of 24 months. Conclusion: This case demonstrates the difficulties involved in precise antenatal diagnosis of certain diaphragmatic malformations, and difficulties concerning surgical repair of wide muscular defects with aplasia of the pillars. This polymalformation syndrome requires multidisciplinary care and long-term follow-up.