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

Abstract

<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.

Extending the prenatal Noonan's phenotype by review of ultrasound and autopsy data

“…Successful cases of fetal surgery have been described in NS 48,49 . In this study, only one fetus benefited from pleuro‐amniotic drainage.…”

Congenital Chylothorax of the Newborn

“…Dear Editor, We wish to congratulate the authors for the important work of revision of the congenital chylothorax of the newborn (CCT) [1], a subject still much debated. We respectfully wish to make a few brief comments on the article.…”

“Open Questions” and Role of Lung Ultrasound in the Management of Congenital Chylothorax

“…As neonatologists we read with interest the systematic analysis by Resch et al [1] about the treatment strategies available for congenital chylothorax (CCT), finding this topic clinically relevant. In the last 3 years, we admitted 10 patients with a diagnosis of CCT in our neonatal intensive care unit (NICU), and we strongly believe that a standardized protocol can be useful and safe for CCT management.…”

“…Resch et al [1] propose to continue the MCT diet until full enteral feeding (FEF) is reached, while other authors suggest different durations (from 2 to 6 weeks from the removal of chest drains [2-5]). Furthermore, lung ultrasound (LUS) is a useful technique in the diagnosis, the grading and the treatment choices (invasive versus noninvasive) of pleural effusions, as recently described by Rocha et al [5].…”

“…Other authors introduce octreotide as a second-line treatment [6]. The algorithm of Resch et al [1] considers octreotide treatment starting from week 5 or 6 in case of failure of the MCT diet. This is in line with the findings of a recent systematic review [6].…”