Search

Congenital chylous ascites (CCA) is a rare condition seen in the neonatal period. The pathogenesis is primarily related to congenital intestinal lymphangiectasis. Conservative treatment of chylous ascites involves paracentesis, total parenteral nutrition (TPN), medium-chain triglyceride (MCT)-based milk formula, use of somatostatin analogue and octreotide. Surgical treatment is considered when conservative treatment fails. We describe a laparoscopic treatment of CCA using the fibrin glue technique. A male infant, in whom foetal ascites was detected at 19 weeks of gestation, was born by caesarean section at 35 weeks of gestation weighing 3760 g. There was evidence of hydrops in the foetal scan. A diagnosis of chylous ascites was made by abdominal paracentesis. A magnetic resonance scan was suggestive of gross ascites, and no lymphatic malformation was identified. TPN and octreotide infusion was started and continued for 4 weeks, but the ascites persisted. The failure of conservative treatment led us to perform laparoscopic exploration. Intraoperatively, chylous ascites and multiple prominent lymphatic vessels around the root of the mesentery were noted. The fibrin glue was applied over the leaking mesenteric lymphatic vessels in the duodenopancreatic region. Oral feeding was started from post-operative day 7. After 2 weeks of the MCT formula, ascites progressed. Thus, laparoscopic exploration was necessary. We introduced an endoscopic applicator for fibrin glue and applied it into the place of leakage. The patient was doing well with no appearance of ascites reaccumulating and was discharged on the 45th post-operative day. Follow-up ultrasonography (1st, 3rd and 9th months after discharge) showed a small amount of ascitic fluid but with no clinical significance. Laparoscopic localisation and ligation of leakage sites could be difficult, especially in newborns and young infants due to the small size of lymphatic vessels. The use of fibrin glue to seal the lymphatic vessels is quite promising. more...

Abstract Background Lysosomal storage disorders (LSD) are a rare cause of non immunological hydrops fetalis (NIHF) and congenital ascites. The reported incidence is about 1%. The incidence of idiopathic NIHF is estimated to be about 18%. Patients and methods We report four cases with transient hydrops fetalis resulting from LSD and performed a literature review on LSD with NIHF and congenital ascites in combination. Results At present, 12 different LSDs are described to be associated with NIHF or congenital ascites. Most patients had a family history of NIHF, where the preceding sibling had not been examined. A diagnostic approach to the fetus with NIHF due to suspected LSD either in utero or postnatal is suggested. Transient forms of NIHF and/or ascites in association with MPS IVA, MPS VII and NPC are described for the first time in this publication. Conclusions LSD should be considered in transient hydrops. Enzymatic studies in chorionic villous sample or amniotic cultured cells, once the most common conditions associated with fetal ascites or hydrops have been ruled out, are important. This paper emphasizes the fact that LSD is significantly higher than the estimated 1% in previous studies, which is important for genetic counseling as there is a high risk of recurrence and the availability of enzyme replacement therapy for an increasing number of LSD. more...