Abstract
The central tendon defect type of congenital diaphragmatic hernia (CDH) is extremely rare and usually associated with a significant pericardial effusion. Prenatal diagnostic ultrasound features of this quite rare entity remain often overlooked or misdiagnosed. There is a dearth of literature about the role of prenatal intervention, often through an elective pericardiocentesis, for the prevention of lung hypoplasia and to decrease the overall neonatal morbidity and mortality. To the best of our knowledge, till date, there is only one case that was subjected to a prenatal intervention. Here, we present a second case of a central tendon defect type of CDH with a large pericardial effusion that was subjected to a prenatal transthoracic pericardiocentesis. Although smooth intubation and ventilation were performed immediately after birth, the infant suffered for several months from respiratory instability. Laparoscopic central tendon hernia repair was performed, and neonate was discharged home at seven months of age. Although prenatal pericardiocentesis may facilitate smoother postnatal intubation and ventilation, its broader effect on respiratory function is uncertain and still remains elusive.
Highlights
Congenital diaphragmatic hernia (CDH) is a rare, major structural abnormality with a high mortality rate [1]. It is a defect of the diaphragm, which often leads to a herniation of the abdominal organs into the thoracic cavity
Anterior hernias have been further categorized into three subtypes: (1) retrosternal or parasternal hernias (Morgagni or Morgagni-Larrey hernias), the most anterior defect of the diaphragm; (2) anterior hernias, which are anteriorly localized with an extension into the anterior central tendon, due to the defect in septum transversum; and (3) central hernias, which are categorized by the defects in the central tendinous portion of the diaphragm [4]
We presented an uncommon, interesting case of a central tendon defect type of CDH
Summary
Congenital diaphragmatic hernia (CDH) is a rare, major structural abnormality with a high mortality rate [1]. It is a defect of the diaphragm, which often leads to a herniation of the abdominal organs into the thoracic cavity. CDH of central tendon defect is uncommon and is often associated with a large pericardial effusion and lung hypoplasia [5, 6]. Whether early prenatal management should regularly include pericardiocentesis for lung expansion and prevention of lung hypoplasia or to be deferred into postnatal period is not clearly defined in the. We report our experience of prenatal and postnatal management of a rare case of central tendon defect of CDH with a large pericardial effusion
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