Staged Management of Congenital Chylothorax With Hydrops Fetalis: An Insight Into EXIT Related Procedures

Abstract

Background: Idiopathic congenital chylothorax is a rare but serious disease. Advancement in perinatal care and the renovated treatment modalities have brought about significant improvement in patient outcome.Objective: To describe the clinical course of severe forms of idiopathic congenital chylothorax, focusing on the development of recent treatment modalities and their impacts.Design: A retrospective cohort by review of medical records in the NICU of a perinatal referral center in Taiwan. Study period was from January 2006 to June 2017. Neonates with the diagnosis of idiopathic congenital chylothorax with non-immune hydrops fetalis were enrolled. Clinical relevant including demographic data, perinatal interventions, post-natal course, and treatment outcome were described and analyzed.Results: Twenty-eight neonatal patients were included. The median gestational age at birth was 34 (range 27–36) weeks and median birth weight was 2,369 (range 1,430–3,608) g. Prenatal intervention was performed in 39.3% of the patients. Besides, 11 out of the 28 neonates developed tension pneumothorax in the first 24 h and 4 (36.4%) of them died. Sepsis was documented in two patients (7.1%). Overall survival rate was 71.4%. There were five patients enrolled during the last 2 years of the study period. EXIT with intubation was performed in two and octreotide was given to four of these most recent neonates, and all of them survived.Conclusion: Recent advances in the management of these neonates, specifically EXIT with intubation and use of octreotide. Both of them improved patient survival in our cohort, but the evidence of impact has yet to be validated.

Clinical features and outcomes of congenital chylothorax: a single tertiary medical center experience in China

“…It has been reported that survival is high in infants delivered after 32 weeks gestation [1]. The survival rate in infants with and without hydrops fetalis reported in our study was 78% and 88%, respectively, which was similar to that reported by Tai et al [9], but higher than previous reports of 30-70% [2]. In addition to gestational age, prenatal intervention is an important factor affecting the prognosis of infants with CC.…”

“…No combination of proven or suspected genetic syndromes were identified in our cases, which may be related to the improvement in prenatal diagnostic techniques in our geographic region in recent years. CC can occur at any stage of pregnancy, most often in the third trimester [3,9]. Once a fetal pleural effusion is detected, ultrasonic examination of fetal anatomic structures and fetal echocardiography should be performed to exclude other deformities.…”

“…Intrapartum extrauterine therapy (EXIT) has also been introduced in infants with CC. EXIT refers to respiratory management prior to severing the umbilical cord, including endotracheal intubation and thorax puncture, to leave sufficient space for lung expansion before the infant starts effective spontaneous breathing [9,15]; however, an early pneumothorax often occurs in infants who received intrapartum TC alone. An early pneumothorax is associated with a poor outcome in infants with CC [16].…”