Successful management of congenital chylous ascites with early octreotide and total parenteral nutrition in a newborn

Abstract

Congenital chylous ascites (CCA) is a rare disease that results from maldevelopment of the intra-abdominal lymphatic system. Few cases have been described and no gold standard treatment has been defined so far. Octreotide, a somatostatin analogue, has been used for the treatment of CCA, but always after a failed conservative approach with fasting, total parenteral nutrition (TPN) or medium chain triglyceride (MCT) feeds. We report the case of a newborn with CCA treated by fasting, TPN and octreotide for a period of 15 days until the abdominal distension was successfully reduced at which point treatment was switched to an MCT formula. On day 25 the patient was breastfed and was discharged on day 33. No recurrence of chylous ascites was noted. Our experience highlights the successful treatment with TPN and octreotide as the first step for the conservative approach of CCA in a newborn, reducing the length of treatment and hospitalisation.

Japanese clinical practice guidelines for vascular anomalies 2017

“…MCT was used before, after, and during treatment . In the multicenter case series by Bellini et al ., MCT was reportedly performed in 14 patients without adverse effects.…”

Management of Chylous Ascites with Octreotide in a Bangladeshi Child: A Case Report

“…12,18 In our case we followed up the patient blood pressure, capillary blood glucose but there were no significant adverse effects. Therefore, it can be said that a relatively low dose of 0.5-1.0ìg/ kg/h of infusion seems to be sufficient for satisfactory outcome with minimal side effects.…”

“…Octreotide was first reported to significantly reduce chylous effusion in an adult with chylothorax 13 and subsequently in several other patients with various etiology. [14][15][16][17][18] Octreotide is a somatostatin analogue that inhibits the secretion of some pituitary and gastrointestinal hormones, reduce secretion of fluids from the intestine and pancreas, causes vasoconstriction in the blood vessels& increases splanchnic arteriolar resistance, reducing gastrointestinal flow and consequently the lymph flow. 17 It is a pharmacologic agent that also augments the closure of any leakage in the lymphatic system.…”

Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI

“…A notable decrease in ascites, however, can take 3–8 weeks on dietary changes alone 11-13. If the ascites cause respiratory distress, abdominal compartment syndrome, vascular or perfusion deficits, or peritonitis, then a combination of paracentesis, discontinuation of oral feeds, and initiation of octreotide should be considered 13,14. Our patient experienced hypoxia and worsening respiratory distress due to chylous abdominal accumulation despite MCT formula.…”

“…Use of parenteral octreotide has been shown to successfully decrease lymph output and chyle leak in both pediatric and adult patients 11-13,16. No treatment guideline currently exists for octreotide use 17.…”