• A rare case of association of muscle hypoplasia and diaphragm defect in a 5 month old child with congenital diaphragmatic hernia
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A rare case of association of muscle hypoplasia and diaphragm defect in a 5 month old child with congenital diaphragmatic hernia

PERINATOLOGIYA AND PEDIATRIYA.2019.2(78):67-71; doi 10.15574/PP.2019.78.67

Sliepov O. K., Ponomarenko O. P., Migur M. Yu., Gladishko O. P.
SI Institute of Pediatrics, Obstetrics and Gynecology named after academician O. Lukyanova of the National Academy of Medical Sciences of Ukraine, Kyiv

Late diagnosed congenital diaphragmatic hernia is a rare and highly dangerous developmental disorder in children and adults, in which a large polymorphism of clinical signs poses a risk of diagnostic and treatment mistakes, both at the pre-hospital stage and in the conditions of specialized centers.

The clinical case of a child with late-presenting left-sided congenital diaphragmatic hernia with unique anatomical features of a hernial sac was presented. The sac was represented by an eventrated diaphragm, with a non traumatic perforation on its apex. Reduction of hernial content, removal of the hypoplasized part of the diaphragm and its autologous reconstruction were performed during laparotomy. The result of surgical correction of the presented congenital malformation is good.

The described clinical case is unique, since it does not fall under any of the classifications and combines both the aperture of the diaphragm and its transverse defect. This case is an example of a late diagnosed diaphragmatic hernia with uncharacteristic clinical manifestations. The correct algorithm for diagnosis and treatment allowed to efficiently and promptly correct the life-threatening inborn diagnosed diaphragmatic hernia in the newborn child and save the life.

The described congenital malformation is rare, therefore, it has scientific and practical significance for children's surgeons, reanimatologists, neonatologists and other specialists.

Key words: congenital diaphragmatic hernia, late-presenting, association of hypoplasia of the diaphragm and perforation, surgical correction, infant.

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Article received: Feb 01, 2019. Accepted for publication: May 27, 2019.