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 infusion, thoracic duct ligation, and chemical pleurodesis) failed. The diagnosis congenital generalised lymphangiectasis was established by MRI as well as lung and skin histology.

At the age of 8 months (body weight 4.5kg) pleural effusions stopped and ascites occurred in the same quantity. Following drainage via Tenckhoff's catheter a peritoneovenous shunt (Denver ascites shunt, Denver (R) Biomedical Inc.) was placed in a palliative attempt. No perioperative complications occurred. External suction was stopped. A good control of ascites was achieved by peritoneovenous shunting (mechanically pumping the Denver shunt at 6 times/d). The patient's clinical condition stabilized temporally. Still on ventilation our patient died of severe pneumonia 24 days after the implantation.

Conclusion: Peritoneo- and pleurovenous shunting are established methods in the treatment of chronic ascites and pleural effusions in adults. In this pediatric case of intractable ascites peritoneovenous shunting offered an effective palliative treatment option.