Congenital diaphragmatic hernia and hydrops: A lethal association?

• Published in: Journal of pediatric surgery 2002-12, Vol.37 (12), p.1678-1680
• Main Authors: Sydorak, R.M., Goldstein, R., Hirose, S., Tsao, K., Farmer, D.L., Lee, H., Harrison, M.R., Albanese, C.T.
• Format: Article
• Language: English
• Subjects: Abnormalities, Multiple - epidemiology; Biological and medical sciences; Comorbidity; Congenital diaphragmatic hernia; Diseases of mother, fetus and pregnancy; fetal surgery; Follow-Up Studies; Gestational Age; Gynecology. Andrology. Obstetrics; Hernia, Diaphragmatic - epidemiology; Hernias, Diaphragmatic, Congenital; Humans; hydrops fetalis; Hydrops Fetalis - diagnostic imaging; Hydrops Fetalis - epidemiology; Infant, Newborn; Medical sciences; Pneumology; Pregnancy. Fetus. Placenta; prenatal diagnosis; Prognosis; Referral and Consultation - statistics & numerical data; Respiratory system : syndromes and miscellaneous diseases; Retrospective Studies; Survival Rate; Ultrasonography
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1053/jpsu.2002.36691

Background/Purpose: Nonimmune hydrops in the fetus is a finding that often portends death. The association and prognosis of fetuses with congenital diaphragmatic hernia (CDH) and hydrops is not known. Methods: A retrospective review of all prenatally diagnosed cases and referrals of CDH was performed. Variables analyzed included gestational age at diagnosis and delivery, side of hernia, presence of associated anomalies and hydrops, and neonatal outcome. Results: Since 1993, 474 prenatal referrals for CDH have been made. One hundred seventy-five were evaluated; 15 fetuses had hydrops (9%). Five patients had CDH, hydrops, and associated lethal anomalies. In the remaining 10 patients, 6 of the diaphragmatic defects were right-sided and 4 were left-sided. All except one had a major portion of the liver herniated into the chest. Six fetuses had prenatal intervention. Five neonates died shortly after birth. There were 5 long-term survivors; all received prenatal intervention. Conclusions: The association of CDH and hydrops is rare but often results in fatality. Hydrops appears to be associated with liver in the hernia, right-sided lesions, and lethal anomalies. Fetal intervention can be performed successfully in patients with CDH and hydrops, and may improve long-term survival rate in this group. J Pediatr Surg 37:1678-1680. Copyright 2002, Elsevier Science (USA). All rights reserved.