Congenital chylothorax: a rare entity

Pleural effusions in a neonate are generally congenital in about one third of the cases and acquired in the remaining two thirds. Congenital isolated pleural effusion is rare. It has an incidence of approximately 1 in 12000 to 1 in 15000 pregnancies. Chylothorax is the most common cause of neonatal congenital pleural effusion. Incidence of congenital chylothorax is 1 in 8600 to 1 in 10000 deliveries with a male to female ratio of 2:1. It poses both a diagnostic as well as therapeutic challenge to the neonatologist. Authors hereby present a rare case of congenital chylothorax which was medically managed and discharged. The neonate responded well to octreotide and medium chain triglyceride (MCT)-diet and was discharged without any complications.

Recién nacido pretérmino con quilotórax congénito bilateral, una entidad infrecuente que amenaza la vida / Preterm newborn with bilateral congenital chylothorax, a rare life-threatening entity

RESUMEN El quilotórax corresponde a la acumulación de linfa en el espacio pleural. El diagnóstico se confirma por la presencia de quilomicrones en el líquido pleural, sin embargo en la práctica clínica se utilizan los criterios de Büttiker, para establecer el diagnóstico. El objetivo de esta publicación es presentar el caso de un recién nacido con quilotórax congénito bilateral, realizando una revisión actualizada del tema, que incentive la recolección de datos y la generación de guías nacionales para su abordaje estandarizado. Se trató de un recién nacido pretérmino, quien tras parto por cesárea presentó síndrome de distrés respiratorio, ameritó terapia temprana de surfactante logrando retiro de ventilación mecánica al día de vida, con posterior presentación de signos de dificultad respiratoria y hallazgo radiológico y ecográfico de derrame pleural bilateral. Se realizó toracentesis con citoquímico compatible con quilotórax. Nuevamente ameritó soporte ventilatorio invasivo, toracostomía cerrada bilateral y nutrición parenteral, con posterior evolución clínica satisfactoria. MÉD.UIS. 2017;30(1):87-92.

ABSTRACT Chylothorax refers to the accumulation of lymphatic fluid in the pleural space. Diagnosis is confirmed by the presence of chylomicrons in the pleural fluid, however in clinical practice Büttiker criteria are widely used. The aim of this publication is to present the case of a newborn with bilateral congenital chylothorax with an updated review of the literature that encourages data collection and generation of national guidelines for standardized approach. This was a newborn preterm, who after a cesarean delivery presented respiratory distress syndrome who required early surfactant therapy achieving withdrawal of mechanical ventilation on his first day, with subsequent presentation of signs of respiratory distress, and a radiological and ultrasound finding of bilateral pleural effusion. Thoracentesis was performed obtaining cytochemical compatible with chylothorax. Again required invasive ventilatory support, closed bilateral thoracostomy and parenteral nutrition, with a satisfactory clinical course. MÉD.UIS. 2017;30(1):87-92.

Congenital chylothorax - Successful management with chemical pleurodesis.

Congenital Chylothorax is a rare entity which is characterized by abnormal accumulation of chyle in pleural cavity. Chylothorax presenting as non-immune hydrops is even rarer. We report a case of congenital bilateral chylothorax presenting as non immune hydrops and managed successfully with chemical pleurodesis. A term male baby presented at birth with bilateral pleural effusions and subcutaneous edema. It was initially managed with ventilation and intercostals drainage (ICD). After the initiation of feeds, re-accumulation of pleural fluid led to the diagnosis of congenital chylothorax. Management with ICD and octreotide was unsuccessful but responded to chemical pleurodesis with 4% povidine iodine done on 3 separate occasions.

A clinical study of congenital chylothorax and octreotide therapy / 소아과

PURPOSE: Congenital chylothorax is an accumulation of lymphatic fluid within the pleural space. It is a common cause of unidentified hydrops fetalis. We examined the perinatal history, clinical manifestation, diagnosis, treatment, and outcome in 6 newborns diagnosed to have congenital chylothorax with hydrops fetalis. We also studied the effect of octreotide therapy for congenital chylothorax in relation to conservative treatment. METHODS: We retrospectively reviewed the medical records of 6 patients diagnosed to have congenital chylothorax with hydrops fetalis among 27,907 newborns who were born at the Cheil General Hospital and Womens Healthcare Center between January 2004 and July 2007 . The diagnosis of chylothorax is based on the analysis of pleural fluid before and after milk feeding. RESULTS: Incidence of congenital chylothorax in this study was 0.021%. All 6 cases were noted in over the 92% lymphocyte in pleural analysis. Transudate was changed into chyle with increasing triglyceride levels above 200 mg/dL after milk feeding. Three of 6 infants improved with conservative treatment, including thoracostomy and assisted ventilation. The others had persistent symptoms despite conservative treatment and responded to octreotide therapy. A complication, specifically vomiting was noted in 1 case during octreotide therapy. CONCLUSION: In this study, octreotide therapy resulted in a safe and excellent outcome. Therefore, octreotide therapy is considered in severe refractory congenital chylothorax in conservative treatment. Further studies are required to determine appropriate guidelines for octreotide therapy.

Spontaneous Chylothorax in Children / 소아알레르기및호흡기학회지

PURPOSE: The purpose of this study was to review the cases of spontaneous chylothorax and its diagnosis, treatment, outcome, and complications. METHODS: We retrospectively reviewed the medical records of 10 patients who were diagnosed as spontaneous chylothorax in Seoul National University Children's Hospital between January 1990 and August 2001. We reviewed demographic data, pleural fluid analysis, treatment modalities, complications, and outcomes. RESULTS: Spontaneous chylothorax was identified in 10 patients including 5 infants with congenital origin. Chylothorax was noted in left(3 cases), right(2 cases) and bilatelly in five cases. Three of five infants with congenital chylothorax were born with hydrops fetalis and two were born prematurely. Pleural fluid laboratory findings were as follows: WBC>1,000/microliter, lymphocytes 92.8+/-9.9%, protein 3.9+/-2.7 g/dL, triglyceride 994+/-825 mg/dL, cholesterol 46.6+/-26.7 mg/dL. All cases were treated initially with a combination of repeated thoracenteses and/or pleural drains, total parenteral nutrition, medium-chain triglyceride based formula or low fat diet. Seven cases responded to conservative treatment. One infant underwent successful thoracic duct ligation. Chylothorax persisted in 2 cases. There was an initial response to steroid treatment in 1 infant who was considered Noonan syndrome, but chylous effusion increased during the tapering of steroid. CONCLUSION: Conservative treatment was successful in 70% of the patients. A precise diagnosis, proper treatment, and prevention and treatment of complications are important in the management of chylothorax. Familiarity with the therapeutic options and appropriate timing for surgical intervention will be required.

A Case of Congenital Chylothorax in a Premature Infant

We experienced a case of isolated fetal pleural effusion diagnosed by antenatal ultrasonogram in the 33th week of gestational age. Chest PA at birth showed massive pleural effusion in both lungs. The serous pleural fluid changed to a milky nature after feeding so we diagnosed it as congenital chylothorax. The infant was managed by chest tube drainage, NPO & TPN with good response and was discharged on the 28th hospital day. We report the case with a brief review of its related literature.

Two Cases of Congenital Chylothorax Diagnosed by Prenatal Ultrasonography / 대한주산의학회잡지

Congenital chylothorax is a rare disorder and can be diagnosed by prenatal ultrasonography recently. Most cases of congenital chylothorax were characterized by different clinical courses of respiratory distress. We describe two female cases with congenital chylothorax observed by ultra- sonography prenatally. In the first case, left-sided pleural effusion was noted by prenatal ultrasonography taken at 34 weeks of gestation, and then pleural fluid was extracted by intrauterine thoracentesis under sonography guidance. After birth, this patient was managed by TPN(total parentral nutrition) and intermittent thoracentesis without surgical treatment. But, pleural fluid was accumulated recurrently and respiratory distress was aggravated. At 15th hospital day, shock state was developed and patient died. In the second case, bilateral pleural effusion and ascites were noted by prenatal ultrasonography, and then patient was delivered immediately without intrauterine thoracentesis. After birth, the second case received conservative therapy including mechanical ventilation, TPN, intermittent thoracentesis and paracentesis. The patient was discharged with complete regression of chylothorax. We report the two cases with brief review of related literatures.

A Case of Isolated Fetal Pleural Effusion Antenatally Diagnosed

The isolated fetal pleural effusion is rare and has no other signs of fetal hydrops. Its etiology is unknown but the most common cause is congenital chylothorax. We experienced a case of fetal pleural effusion diagnosed in the 35 weeks gestational age by antenatal ultrasonogram and antenatal diagnosis allowed early therapeutic intervention such as drainage of pleural fluid immediatly after birth. We diagnosed congenital chylothorax after neonate feeding changes the quality of pleural fluid from serous to milky So We report with a brief review and its related literatures.

Congenital Chylothorax

Chyle is the digestive product absorbed from intestinal lymphatics which the component have high concentration of triglyceride and protein. The various pathological circumstances which give rise to intrathoracic effusion of chyle are well documented and so the etiology is clearly defined in most instances. However, the occurrence of chylothorax in early infancy, in the absence of other demonstrable diseases and without apparent birth trauma, suggests the existence of congenital malformation of the thoracic ducts. We lately experienced a 20 day old new born who was proved to have congenital chylothorax by clinical history and laboratory findings. So we report the clinical findings and laboratory findings with the review of the literatures on congenital chylothorax.