Congenital rib absence with liver herniation: A case report

Abstract

Introduction

Congenital rib absence with liver herniation is an extremely rare condition. Only a few cases have been reported, and most of them have been associated with Poland syndrome.

Case presentation

A full-term female newborn, born at 38 weeks of gestation with a birth weight of 3100 g, delivered vaginally following a normal pregnancy was brought to our clinic at 11 days of life for a right lower thoracic bulge. There was no relevant family history. The physical exam was completely normal except for a 4 × 5 cm round protrusion in the right lower anterolateral chest. The ribs in the area were clearly absent. The skin, the subcutaneous tissue and the muscles appeared normal. He had normal vital signs and breath sounds. The abdominal exam was normal. A plain chest X-ray showed normal lung fields, absence of the right lower ribs, a right diaphragmatic eventration, and a soft tissue herniation. A three-dimensional computed tomography (3D-CT) reconstruction confirmed the diagnosis of congenital hypoplastic 6th and 7th right ribs, absent 8th and 9th right ribs, and a foramen in the right 10th rib. It also showed that the visible bulge was the liver protruding through the weakened chest wall. The only other anatomical anomaly was 6th to 9th thoracic butterfly vertebrae. The patient is being managed conservatively and is doing well.

Conclusion

Congenital intercostal liver herniation with absent ribs is an extremely rare condition and can be managed conservatively in asymptomatic cases. We recommend 3D-CT reconstruction as an effective way to confirm the diagnosis and rule out associated malformations.