Objective: The aim of this study is to share our experience about this rare disorder including clinical features, management and outcome perspectives. Study Design: Retrospective, cross sectional study. Place and Duration of Study: This study was conducted at the department of Gastroenterology, The Children’s Hospital & Institute of Child Health, Lahore, between the periods of January 2013 to December 2017. Patients and Methods: The database included age (months), gender, clinical presentation, laboratory parameters, management and outcome. The data was analyzed by SPSS version 22. Results: Twenty-four children were identified who met the criteria as chylous disorder. There were 15 (65.2%) females with interquartile range (IQR) of 144(1) months. Fourteen (58.3%) patients had primary etiology while rest 10 (41.6%) had secondary cause. Intestinal lymphangiectasia was the leading cause in 7 (50%) followed by idiopathic chylous ascites, effusion and syndromic chylous disorder. Among the secondary causes were post-surgical and tuberculosis in 4 (40%) each and two (20%) of malignancy (adenocarcinoma of colon, myxoma of heart and intestine). Majority of patients had lymphopenia and hypoalbuminemia. Acute management included stabilization with albumin, fluid aspirations to decompress, octreotides and dietary intervention. Eight children required ascitic tap and five pleurodesis for effusion and ascites. The majority of children did well on follow-up on medium chain triglycerides (MCT) oil and 3 (12.5%) children expired due to non-compliance issues, severe hypoalbuminemia and concurrent infections. Conclusion: Chylous disorder is a rare entity; symptoms control is good on dietary modification with MCT oil and supplemented deficient vitamins and required surgical intervention when medical measures have failed.
SARA BATOOL, HUMA ARSHAD CHEEMA, ZAFAR FAYYAZ, Anjum Saeed, Muhammad Arshad Alvi, Nadia Waheed, , Muhammad Nadeem Anjum. (2021) Chylous Disorders: A Rare Entity in Children, Pakistan Pediatric Journal, Volume 45, Issue 1.