Patients with postnatal manifestation of congenital diaphragmatic hernia: management specificities

Abstract

Treatment of congenital diaphragmatic hernia is one of the most critical neonatal surgery challenges, which is associated with high mortality rate. Despite the progress achieved in the treatment of congenital diaphragmatic hernia, the choice of surgical approach and time of hernial correction remains controversial.

Material and methods: From 2000 to 2018, 39 children with congenital false diaphragmatic hernia were hospitalized in the department of surgery of the Nizhny Novgorod Regional Children's Clinical Hospital. Of these, 26 (66.7%) were boys and 13 (33.3%) were girls. Cases of successful treatment of bilateral diaphragmatic hernia and correction of persistent right Bochdalek defect with underlying right tension pneumothorax with “late manifestation” of diaphragmatic hernia deserve a special presentation.

Results: Most typical set of symptoms includes respiratory failure, cardiovascular disorders, and intestinal pseudo-obstruction syndrome. Left-sided hernia was detected in 35 children (89.7%), while right-sided hernia was detected in 3 (7.7%), and a bilateral hernia was detected in one child. The mortality rate accounted for 25.6% (10 children) of children due to progression of cardiopulmonary complications.

Conclusions: Despite the extensive clinical experience in the management of children with diaphragmatic hernias, of the prenatal diagnostics potential and technical capacities of modern medicine, individual cases of diaphragmatic hernias are associated with challenges regarding timely detection of malformation in the postnatal period, since the diaphragmatic hernia with persistent diaphragmatic defect tend to demonstrate a late pattern of manifestation. Diaphragmatic hernia can develop at a later time—age 4–6 months, which could be explained by an intra-abdominal pressure increase when the child is becoming more active, while underlying Bochdalek defect is persisting.

Keywords

congenital false diaphragmatic hernia / bilateral diaphragmatic hernia / Bochdalek hernia

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