Congenital Chylothorax of the Newborn: A Systematic Analysis of Published Cases between 1990 and 2018

<b><i>Background:</i></b> Congenital chylothorax (CCT) of the newborn is a rare entity but the most common cause of pleural effusion in this age-group. We aimed to find the optimal treatment strategy. <b><i>Material and Methods:</i></b> A PubMed search was performed according to the PRISMA criteria. All cases were analyzed according to prenatal, perinatal, and postnatal treatment modalities and follow-ups. <b><i>Results:</i></b> We identified 753 cases from 157 studies published between 1990 and 2018. The all-cause mortality rate was 28%. Prematurity was present in 71%, male gender dominated 57%, mean gestational age was 34 weeks, and birth weight was 2,654 g. Seventy-nine percent of newborns had bilateral CCT, the most common associated congenital anomalies with CCT were pulmonary lymphangiectasia and pulmonary hypoplasia, and the most common chromosomal aberrations were Down, Noonan, and Turner syndromes, respectively. Mechanical ventilation was reported in 381 cases for mean 17 (range 1–120) days; pleural punctuations and drainages were performed in 32% and 64%, respectively. Forty-four percent received total parenteral nutrition (TPN) for mean 21 days, 46% medium-chain triglyceride (MCT) diet for mean 37 days, 20% octreotide, and 3% somatostatin; chemical pleurodesis was performed in 116 cases, and surgery was reported in 48 cases with a success rate of 69%. In 462 cases (68%), complete restitution was reported; in 34 of 44 cases (77%), intrauterine intervention was carried out. <b><i>Conclusion:</i></b> Respiratory support, pleural drainages, TPN, and MCT diet as octreotide remain to be the cornerstones of CCT management. Pleurodesis with OK-432 done prenatally and povidone-iodine postnatally might be discussed for use in life-threatening CCT.

A rare case of pulmonary lymphangiectasia associated with CHD

CHD may, at times, occur in the framework of other rare pathologies. These, having similar clinical manifestations, present a diagnostic dilemma for the clinician. The authors present the case of an infant with non-syndromic complete atrioventricular septal defect, whose post-operative period was surprisingly complicated by progressive pulmonary hypertension. Despite intensive care, the infant ultimately died. The diagnosis of unilateral primary pulmonary lymphangiectasia was only possible post mortem.

Case Report: A Relatively Mild Presentation of Unilateral Congenital Pulmonary Lymphangiectasia

Pulmonary lymphangiectasia (PL) is a rare congenital disorder of pulmonary lymphatic development. Although it was traditionally a fatal disorder of infancy, some cases in later childhood have been reported, suggesting a spectrum of severity. We present an unusual case of unilateral, congenital pulmonary lymphangiectasia. Our patient presented with neonatal respiratory distress, a chronic wet cough and recurrent episodes of bronchitis. Chest CT revealed thickening of the interlobular septae of the right lung. A lung biopsy confirmed the diagnosis of lymphangiectasia. His clinical course is characterized by chronic coughing and recurrent bronchitis but normal growth and development. This case illustrates a relatively mild presentation of unilateral PL, which, along with other reports, suggests variability in the presentation and severity of this disorder. In the absence of imaging and histological examination, mild presentations may go undiagnosed.

Primary pulmonary lymphangiectasia in Noonan syndrome: apropos of an extremely rare manifestation and a brief literature review

Noonan syndrome (NS) is a genetic multisystem disorder characterised by distinctive facial features, developmental delay, learning difficulties, short stature, congenital heart disease, renal anomalies, bleeding difficulties and lymphatic malformations. Although lymphatic dysplasias are present in 20% of patients with NS, however pulmonary lymphangiectasia has rarely been described. In this present paper, we report a 24-year-old male who was diagnosed with Noonan syndrome and primary pulmonary lymphangiectasia by using chest imaging modalities. A brief overview of the current literature is also provided laying emphasis on the clinical, pathogenetic and diagnostic aspects of this uncommon Noonan syndrome complication.

Congenital pulmonary lymphangiectasia in an extremely low birth weight: a case report

ObjectivesCongenital pulmonary lymphangiectasia (CPL) is a rare but fatal disorder of the lung.Case presentationWe describe the case of an extremely low birth weight (ELBW) infant who presented with severe respiratory distress and recurrent bilateral pneumothorax. He died on day 17. The post-mortem examination of the lungs showed numerous cystic spaces, ranging from 1 to 2 mm in size, in the visceral pleura, in the thickened interlobular septum and hilum. A diagnosis of primary CPL was made.ConclusionsWe intend to underline that CPL, albeit rare, must be one of the differential diagnoses in infants with severe neonatal respiratory distress not responding to intensive care. Chronic interstitial lung diseases are a challenging diagnostic clinical problem, which requires a multidisciplinary approach. Histological lung examination may be useful to demonstrate the presence of CPL.

Successful thoracic duct embolisation in a child with recurrent massive pericardial effusion diagnosed as a lymphatic anomaly

A 29-month-old girl had idiopathic massive pericardial effusion for over 6 months. Lymphangiography was performed for chronic and recurrent pericardial effusion and pulmonary lymphangiectasia, suspected based on CT findings. Magnetic resonance lymphangiography revealed chylolymphatic reflux from a tortuously dilated thoracic duct in the mediastinum to the pericardial space, suggesting primary chylopericardium with lymphangiectasia. Pericardial effusion resolved immediately after thoracic duct embolisation at the lower thoracic level. However, pericardial effusion recurred after 5 months, which resolved after additional embolisation of the abnormal lymphatic collateral vessels from the remnant upper thoracic duct. Here, we report an unusual case with chylous massive pericardial effusion diagnosed by magnetic resonance lymphangiography and treated with percutaneous embolisation.