Prenatal intervention for the management of congenital diaphragmatic hernia

Pediatr Surg Int. 2018 Jun;34(6):579-587. doi: 10.1007/s00383-018-4270-0. Epub 2018 Apr 30.

Abstract

Congenital diaphragmatic hernia (CDH) is the result of incomplete formation of the diaphragm that occurs during embryogenesis. The defect in the diaphragm permits the herniation of abdominal organs into the thoracic cavity contributing to the impairment of normal growth and development of the fetal lung. In addition to the hypoplastic lung, anomalies of the pulmonary arterioles worsen the pulmonary hypertension that can have detrimental effects in severe cases. Most cases of CDH can be effectively managed postnatally. Advances in neonatal and surgical care have resulted in improved outcomes over the years. When available, extracorporeal membrane oxygenation can provide temporary cardiorespiratory support for those not effectively supported by mechanical ventilation. In spite of these advances, very severe cases of CDH still carry a very high mortality and morbidity rate. Advances in imaging and evaluation now allow for early and accurate prenatal diagnosis of CDH, thereby identifying those at greatest risk who may benefit from prenatal intervention. This review article discusses some of the surgical and non-surgical prenatal interventions in the management of isolated severe congenital diaphragmatic hernia.

Keywords: Congenital diaphragmatic hernia; Fetal endoscopic tracheal occlusion; Fetal intervention; Prenatal management.

Publication types

  • Review

MeSH terms

  • Female
  • Fetoscopy
  • Glucocorticoids / therapeutic use
  • Hernias, Diaphragmatic, Congenital / diagnosis*
  • Hernias, Diaphragmatic, Congenital / therapy*
  • Humans
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / prevention & control
  • Pregnancy
  • Prenatal Diagnosis
  • Prognosis
  • Sildenafil Citrate / therapeutic use
  • Trachea / surgery
  • Tretinoin / therapeutic use
  • Ultrasonography, Prenatal
  • Vasodilator Agents / therapeutic use

Substances

  • Glucocorticoids
  • Vasodilator Agents
  • Tretinoin
  • Sildenafil Citrate