Peritoneovenous shunting in a patient with generalized congenital lymphangiectasis: a palliative option in refractory chylous ascites

Introduction: Non-immune hydrops fetalis (NIHF) with persistent chylothorax and chylous ascites is a severe manifestation of congenital generalised lymphangiectasis. The prognosis in general is poor. We report on peritoneovenous shunt placement as a feasible palliative treatment option. Case report: Female infant, born at gestational age 34 weeks, birth weight 2800g (>90th Perc.) with prenatal history of NIHF and bilateral hydrothorax. Continuous bilateral pleural drainage was initiated after birth. Chylous loss was continuously 200–400ml/kg/d. Therapeutic attempts (total parenteral nutrition, somatostatin infus…