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Letters to the Editor Indian Pediatrics 2002; 39:975-976 |
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Bilateral Congenital Chylothorax with Noonan Syndrome |
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Congenital chylothorax in neonates is not rare. More than 300 papers are available including case-series and reviews(1). Unilateral chylothorax has been reported earlier with Noonan’s Syndrome(2,3). We report a case of bilateral congenital chylothorax in a neonate with Noonan syndrome from Nepal. A term 8 hours old male baby weighing 2910 g delivered by cesarean section to a third gravida mother with uneventful antenatal period was admitted in NICU with complaint of fast breathing since birth. There was no perinatal asphyxia. The baby was alert, active, pink but dyspneic. The respiratory rate was 64/min, heart rate 156/min, with a CFT < 3 sec and SPO2 91-95%. The baby had facial dysmorphism with upturned nose, high arched palate, deeply grooved philtrum, low hair lines and webbed neck and had bilateral pitting edema of feet. Examination of chest revealed widely spaced nipples, sub-costal retractions, shield chest, stony dull note on percussion and decreased air entry bilaterally without any added sounds on auscultation. The left testis was not palpable in scrotal sac or inguinal canal. Cardiovascular, neurological and abdominal examinations were normal. The baby was admitted with a provisional diagnosis of Noonan syndrome with bilateral pleural effusion. The hemogram showed hemoglobin 15 g/dL, total leucocyte count 15 × 103/µL with 65% polymorphs and 35% lymphocytes. Total serum protein was 5.2 g/dL with albumin of 3.2 g/dL. Blood glucose and renal function tests were normal. Radiograph of chest showed bilateral radio-opaque homogenous opacity with obliteration of costophrenic angle and meniscus sign. Echocardiography and sonography of abdomen were normal except the presence of left testis in the left illiac fossa. Diagnostic pleural tap from both sides was yellow colored. The cytochemical analysis of right/left pleural fluid revealed albumin: 6.5/5.8 g/dL, glucose: 137/95 mg/dL, cholesterol: 43/45 mg/dL, triglycerides: 578/502 mg/dL and total counts: 1200/950/cu mm, predomi-nantly lymphocytes. Culture for pyogenic organisms was sterile. The Karyotype study revealed normal chromosomal pattern i.e., 46 XY by GTG banding. The baby was treated initially with intravenous fluids, oxygen administration by hood, anti-microbials (ampicillin + gentamicin) and thoracocentesis, first on right and then left side (8 hours later) under close cardiac monitoring. The amount of fluid aspirated from right and left side were 50 mL and 45 mL respectively. The patient improved clinically, respiratory rate decreased to 46/min and SPO2 varied from 90-95%. Feeds were subsequently started through nasogastric tube. On day-7 the patient became dyspneic again with features of reaccumulation of fluid. Radiograph of chest confirmed re-accumulation of fluid in pleural cavity bilaterally. Once again, 30 mL and 26 mL of fluid was aspirated from right and left side respectively. The repeat cytochemical analysis of pleural fluid did not show any significant change except increase in triglyceride level i.e., 640/695 mg/dL on right and left side respectively. The respiratory rate again declined to 52/min and concentration of feeds was modified to provide 150 Kcal of energy and 4 gm/kg/day proteins. Breast feeding was started on day 12. X-ray chest on day 17 did not show any evidence of fluid and patient was discharged. The child was followed up for 18 months in Well Baby Clinic. The growth and development of baby was normal and during this period, the child had one episode of bronchiolitis. Congenital chylothorax is an important differential diagnosis of pleural effusion in neonate. It may occur alone or in combination with other lymphatic anomalies. In Noonan Syndrome lymphatic dysplasia has been reported and may occur anywhere in the body including lung. Pulmonary lymphatic dysplasia may result in lymph flow obstruction resulting in development of fistulas between throacic duct or pleural space or in rupture of thoracic duct and chylothorax(2,3). Thoracentesis and lymphangiography is required for definitive diagnosis. In our patient, though lymphatic angiography was not done but thoracentesis and analysis of fluid confirmed chylothorax. The management involves repeated thoracentesis or intercostal tube drainage, surgical ligation and dietary manipulation which includes substitution of milk fat with medium chain triglycerides which are absorbed directly into portal venous system thus bypassing intestinal lymphatics(1). In majority, these measures result in complete resolution of the effusion without subsequent recurrence as happened in our case. In refractory cases however, total parenteral nutrition and surgery may be required(4). The early diagnosis and prompt management is essential for survival of such neonates. Rajniti Prasad, Kuldeep Singh, Rupa Singh, Department of Pediatrics, B.P. Koirala Institute of Health Sciences, Dharan, Nepal. E-mail: [email protected] . |
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