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Disorders of the Pleura |

A Rare Cause of Chyluria and Recurrent Chylothorax: Lymphatic Dysplasia Syndrome

Santosh Jha, MBBS; Fatima Mamnoon, MBBS; Prahlad Prabhudesai, MD; Abhijeet Khandelwal, DTCD; Miti Maniar, MBBS; Ashish Prakash, DTCD
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Lilavati Hospital & Research Centre, Mumbai, Maharashtra, India


Chest. 2014;146(4_MeetingAbstracts):477A. doi:10.1378/chest.1992607
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Abstract

SESSION TITLE: Pleural Disease Global Case Reports

SESSION TYPE: Global Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Chylothorax,chyle in the pleural cavity though rare in incidence has significant morbidity&mortality.Chyle is the lymphatic fluid which is enriched with fat&its components triglycerides&chylomicrons.It is absorbed by intestinal epithelium&transported by thoracic duct to the circulation.The thoracic duct arises from cisterna chyle,enters thoracic cavity at T10 level of right hemi diaphragm & finally terminates in the vein at the junction of leftSubclavian&InternalJugular.Etiology of the Chylothorax is tumor (54%) trauma (25%) idiopathic (16%)& miscellaneous (6%) 1.

CASE PRESENTATION: This is a case of 44 year old gentleman,with history of hypertension who presented with complaint of minimally productive cough,&gradually progressive exertional breathlessness for last one month.Patient for the above said complaint had been admitted to some hospital outside where diagnostic&therapeutic tapping was done& ICD was also kept for some days.Pleural fluid withdrawn was suggestive of chylothorax.The patient has been referred to us for further evaluation and management.There was no history of trauma.Patient had chyluria about eight year back for which some surgery was done after which he had complication which led to shrunken of left kidney.He was asymptomatic for the last eight year.At admission his vitals were stable.Respiratory system examination was suggestive of left sided pleural effusion which was confirmed later on by chest radiography.We did baseline routine investigations which was suggestive of raised ESR, elevated liver enzymes(SGPT -57,SGOT- 146),Gamma interferon & montoux test was negative.USG-guided pleural tapping was done atleast three times at different time intervals &each time the quantity of pleural fluid showed a decreasing trend,& was consistent with chylothorax picture(milky fluid,Triglycerides-3583mg/dl, cholesterol-117). We further proceeded with advanced diagnostic modalities to know the cause of chylothorax like PET-CT(whole body) which was normal,MRI (chest) revealed findings of multiple cystic spaces in the mediastinum below carina,around the aorta&oesophagus,large left sided pleural effusion.Most likely represent lymphangiectasia (sec to obstruction at carina level)/ lymphatic dysplasia,MRI(abdomen&pelvis) was suggestive of Absent left kidney.Multiple cystic spaces in the retroperitoneum,retocrural region,in the liver,along the extrahepatic portal vein,with a cyst in left kidney&the renal hilum.These most likely represent dilated lymphatic channels/ lymphangiectasia / lymphatic dysplasia; Lympnangioscintigraphy- revealed dilated lymphatic channels in abdomen & lower part of thorax upto the level of carina,probably due to obstruction at that level &abnormal chylous leak seen in left pleural cavity.

DISCUSSION: Disorders of the lymphatic system that could be of various etiologies3.Following a modified Hilliard`s classification4 the disorder is mainly divided into lymphangioma, lymphangiomatosis lymphangiectasis& lymphatic dysplasia syndrome.Diagnosis of chylothorax can be confirmed by presence of chylomicrons and a triglyceride level of more than 110 mg /dl in the aspirated pleural fluid. Our conclusion based on above clinical picture &investigations was of an Idiopathic lymphatic dysplasia which was complicated initially as chyluria& later on presented as chylothorax.The management of chylothorax is both conservative&surgical.Total parenteral nutrition&mediumchain triglyceride(MCT) diet in conservative management.Thoracocentesis&ICD are also done for symptomatic relief & for complete lung expansion.Operative strategies are direct ligation of thoracic duct,mass ligation of supra-diaphragmatic thoracic duct,pleuro-peritoneal shunting,pleurectomy&pleurodesis.5

CONCLUSIONS: We approached surgically by thoracic duct ligation and pleurodesis by VATS&conservatively by MCT diet.The patient is better right now &on follow up with chest X-ray there was no evidence of pleural fluid.

Reference #1: 1.Richard w light;Pleural diseases,sixth edition. 2.John l. Faul et al;Thoracic lymphangiomas,lymphangiectasis,lymphangiomatosis& lymphatic dysplasia syndrome:am j respircrit care med vol 161.2000 3.Nutrition issues in gastroenterology,series #94 4.Hilliard, R. I., J. B. McKendry, and M. J. Phillips. 1990.Congenital ab-normalities of the lymphatic system: a new clinical classification. Pediatrics86. 5.Sukumaran K. Nair, Martin P. Hayward;Aetiology&management of chylothorax in adults;European Journal of Cardio-thoracic Surgery.

DISCLOSURE: The following authors have nothing to disclose: Santosh Jha, Fatima Mamnoon, Prahlad Prabhudesai, Abhijeet Khandelwal, Miti Maniar, Ashish Prakash

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