Congenital Diaphragmatic hernia – a review

Abstract

Congenital Diaphragmatic hernia (CDH) is a condition characterized by a defect in the diaphragm leading to protrusion of abdominal contents into the thoracic cavity interfering with normal development of the lungs. The defect may range from a small aperture in the posterior muscle rim to complete absence of diaphragm.The pathophysiology of CDH is a combination of lung hypoplasia and immaturity associated with persistent pulmonary hypertension of newborn (PPHN) and cardiac dysfunction. Prenatal assessment of lung to head ratio (LHR) and position of the liver by ultrasound are used to diagnose and predict outcomes. Delivery of infants with CDH is recommended close to term gestation. Immediate management at birth includes bowel decompression, avoidance of mask ventilation and endotracheal tube placement if required. The main focus of management includes gentle ventilation, hemodynamic monitoring and treatment of pulmonary hypertension followed by surgery. Although inhaled nitric oxide is not approved by FDA for the treatment of PPHN induced by CDH, it is commonly used.Extracorporeal membrane oxygenation (ECMO) is typically considered after failure of conventional medical management for infants ≥ 34 weeks’ gestation or with weight >2 kg with CDH and no associated major lethal anomalies. Multiple factors such as prematurity, associated abnormalities, severity of PPHN, type of repair and need for ECMO can affect the survival of an infant with CDH. With advances in the management of CDH, the overall survival has improved and has been reported to be 70-90% in non-ECMO infants and up to 50% in infants who undergo ECMO.

Orthopnea and fever in an elderly woman

“…[6][7][8][9][10] Environmental factors such as vitamin A deficiency or herbicide exposure (e.g., nitrofen) have also been associated with CDH. [11][12][13][14] Alcohol use and maternal pregestational diabetes are also significantly associated with CDH. 15 The diagnosis is usually achieved with imaging (ultrasound) in the prenatal period, 1,2 and the evolution of diagnostic techniques has dramatically decreased the number of cases found in post-natal period.…”

Fetal Torakal Anomaliler

“…Hemitoraks içinde safra kesesinin izlenmesi uyarıcı olabilir. Prognoz açısından sol taraf hernisinden farkı yoktur [2,3,13] Sol taraf hernisinde sıklıkla mide, barsaklar ve karaciğerin sol lobu herniyedir (Resim 9A, B). Bazen sol böbrek ve dalağın da hemitoraksa geçtiği izlenebilir.…”

“…Prognoz defektin boyutuna, eşlik eden anomali olup olmadığı-na, karaciğerin pozisyonuna ve karşı akciğerin hacmine bağlıdır [2,3,5,13].…”

“…Herniye segment genellikle toraksın anteriorunda lokalizedir ve portal triadı içeren tipik ekojenite paterni sayesinde kolayca tanı-nabilir. Eğer karaciğerin pozisyonu tam olarak anlaşılamıyorsa MRG yardımcı olabilir [2,3,5,6,13].…”

“…Anteromedial herni sternumun arkasından foramen Morgagniden gerçekleşir ve çok nadir görülür. Bilateral defekt sonucu oluşan herni de çok nadirdir ve prognozu oldukça kötüdür [2,13].…”