Patients with postnatal manifestation of congenital diaphragmatic hernia: management specificities

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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.

About the authors

Andrey S. Zheleznov

Privolzhsky Research Medical University

Author for correspondence.
Email: aszheleznov@mail.ru
ORCID iD: 0000-0002-8296-1213

MD, PhD

Russian Federation, 603005, Nizhny Novgorod

Natal'ya S. Ermolaeva

Privolzhsky Research Medical University

Email: ns12514@gmail.com
ORCID iD: 0000-0002-7928-8128
Russian Federation, 603005, Nizhny Novgorod

Vyacheslav V. Parshikov

Privolzhsky Research Medical University

Email: parshikovvv43@mail.ru
ORCID iD: 0000-0002-9827-6763

MD, PhD, DSc, Professor

Russian Federation, 603005, Nizhny Novgorod

Vadim O. Teplov

N.I. Pirogov Russian National Research Medical University

Email: teplov.vo@yandex.ru
ORCID iD: 0000-0002-7042-439X
Russian Federation, 117997, Moscow

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Supplementary files

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2. Figure: 1. Comparison of indicators of management of patients with congenital diaphragmatic hernias. The OY axis shows the proportion of the occurrence of the studied characters in the sample (%).

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3. Figure: 2. X-ray of the chest organs. The picture of a tense pneumothorax on the right, attention is drawn to the lability of the right dome of the diaphragm.

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4. Figure: 3. X-ray of the chest organs. The phenomena of pneumothorax were arrested.

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5. Figure: 4. X-ray of the chest organs. Local zone of enlightenment in the projection of the lower lobe of the right lung.

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6. Figure: 5. X-ray contrast study with contrast passage to exclude right-sided diaphragmatic hernia. Pictures for 10 min, 30 min, 1 h. There is no contrast in the shadow of the right half of the chest.

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7. Figure: 6. X-ray of the chest organs. Signs of a right-sided diaphragmatic hernia.

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8. Figure: 7. X-ray of the chest organs, performed on the 6th day after the operation.

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9. Figure: 8. X-ray of the chest organs. Signs of a left-sided diaphragmatic hernia.

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10. Figure: 9. Radiopaque study with contrast passage to confirm left-sided diaphragmatic hernia. Moving part of the stomach and intestinal loops to the anterior sections of the left chest cavity, darkening of the left

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