Peritoneovenous Shunting as Palliative Treatment in an Infant with Chylous Ascites due to Generalised Congenital Lymphangiectasia

Introduction

Congenital lymphangiectasia is a rare congenital disorder that is characterised by dilated lymphatic ducts in various tissues. Etiology is unclear, in primary forms a persistence of large immature lymphatic vessels after 20 weeks of gestation is suspected. The grade of pulmonary affection has a strong impact on survival. Advances in intensive neonatal care have changed the previously nearly fatal outcome (Bellini C et al., Orphanet J Rare Dis 2006; 1: 43). Early diagnostic signs in the antenatal ultrasound are bilateral hydrothorax, ascites and non-immune hydrops fetalis. Prenatal interventions such as repeated pleural punctions and chest drainage via pigtail catheter are performed to prevent pulmonary hypoplasia. Premature birth and primary respiratory insufficiency are frequently described. Assisted mechanical ventilation and continuous chest drain are initiated after birth and will be continued depending on the quantity of chylous effusion and the grade of respiratory distress.

In case of persistent ascites the peritoneovenous shunt, established in adult surgery, might be a feasible treatment option also in the pediatric patient. We describe a case of congenital generalised lymphangiectasia with an intractable loss of chylous ascites and its successful palliative treatment with a peritoneovenous shunt system.