[Perioperative management of twins with prenatally diagnosed congenital diaphragmatic hernia]

Abstract

Congenital Diaphragmatic Hernia (CDH) occurs in one of every 2000-3000 births, and most of them are sporadic, and therefore recognized as a circumstantial event. But its occurrence in 85 children among the 40 families is also reported, and some reports suggest that an autosomal recessive gene may be responsible for this disease. We experienced identical twins (babies A and B) both with prenatally diagnosed CDH. They were delivered by emergent cesarean section at 33 weeks of gestation with birth weight of 1857 g and 1561 g, respectively. They were intubated immediately after birth, and ventilated with high frequency oscillation. Baby A presented persistent pulmonary hypertension of newborn, and received nitric oxide inhalation. At the age of 2 days, both of them were stabilized and underwent repair of CDH. After the repair, baby A developed perforation of ileum, airway bleeding and retinopathy of prematurity (ROP), and needed 28 days before extubation. Baby B also developed ROP, but had no other problem, and the trachea was extubated at the age of 12 days. They are the seventh pair reported in the world literature.