Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI
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"We report the first observation of a patient with contgenital chylous ascites (CCA) and Ehlers-Danlos syndrome type VI due to primary lymphatic defect with additional vascular anomaly. CCA is a rare condition, and there is limited understanding of its pathophysiology and treatment options. We also review the patient’s treatment course mitigated with octreotide and total parenteral nutritional support, as there are no current established guidelines for CCA".
"There is no current standard treatment for CCA. For mild to moderate chylous ascites without secondary systemic symptoms, dietary management is the first-line modality. Using an MCT-based formula should slow chyle production. 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. We felt this clinical scenario justified immediate TPN and an attempt to slow chyle production with octreotide".
"Octreotide is a somatostatin analogue, and its mechanism of action is thought to affect vascular smooth-muscle contractility with resultant decreased flow through lymphatics.15 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 Our experience showed resolution of CCA after 5 days of octreotide at 1 μg/kg/hr with a slow dosage wean and discontinuation over 72 hours. Replacement of drained peritoneal fluid with IV albumin and IV immunoglobulin should be given after paracentesis to support intravascular circulation and especially considered in neonatal chylous ascites, due to their immature humoral immunologic system. Finally, laparoscopy should be used for intervention if ascites prove refractory after 4 to 8 weeks of medical and nutritional therapy. Surgical techniques can include drainage, shunting, pleurodesis, embolization, or direct surgery on the thoracic duct".