Congenital diaphragmatic hernia: a case series in tertiary care centre
Keywords:CDH, Pulmonary hypertension, Mechanical ventilation, Age of presentation
Congenital diaphragmatic hernia (CDH) is characterized by a defect in the diaphragm leading to the protrusion of abdominal contents into the thoracic cavity affecting the normal development of the lungs. The prevalence of CDH ranges between 1:2000 and 1:4000 live births; it accounts for 8% of all major congenital anomalies. Approximately 90% of diaphragmatic defects occur posterolaterally and 80% are left-sided. The severity of the condition varies widely, the degree of pulmonary hypoplasia and pulmonary hypertension largely determining outcome. Gross was the first person who reported a series of successful repairs in 1946 with 100% survival and over the next 40 years, CDH was considered the quintessential neonatal surgical emergency. Since then, despite advances in neonatal intensive care, anaesthesia, and surgery, the overall survival rate has remained just around 50%. The management of CDH patients should be multidisciplinary. Prompt mechanical ventilation and imaging ensured favorable outcome after operative repair. The prognosis of CDH patients depends on the age of presentation and the mode of transport of the newborn baby to the tertiary hospital with pediatric surgery set-up.
Chandrasekharan PK, Rawat M, Madappa R, Rothstein DH, Lakshminrusimha S. Congenital Diaphragmatic hernia - a review. Matern Health Neonatol Perinatol. 2017;3:6.
Dumpa V, Chandrasekharan P. Congenital Diaphragmatic Hernia. In: StatPearls. Treasure Island (FL): StatPearls Publishing. 2022.
Rygl M, Pycha K, Stranak Z, Melichar J, Krofta L, Tomasek L, Snajdauf J. Congenital diaphragmatic hernia: onset of respiratory distress and size of the defect: analysis of the outcome in 104 neonates. Pediatr Surg Int. 2007;23(1):27-31.
Matsuoka S, Takeuchi K, Yamanaka Y, Kaji Y, Sugimura K, Maruo T. Comparison of magnetic resonance imaging and ultrasonography in the prenatal diagnosis of congenital thoracic abnormalities. Fetal Diagn Ther. 2003;18:447-53.
Tonks A, Wyldes M, Somerset DA, Dent K, Abhyankar A, Bagchi I, et al. Congenital malformations of the diaphragm: findings of the West Midlands Congenital Anomaly Register 1995 to 2000. Prenat Diagn. 2004;24:596-604.
Lipshutz GS, Albanese CT, Feldstein VA, Jennings RW, Housley HT, Beech R, et al. Prospective analysis of lung-to-head ratio predicts survival for patients with prenatally diagnosed congenital diaphragmatic hernia. J Pediatr Surg. 1997;32:1634-6.
Moga M, Anastasiu C, Arvǎtescu C, Mironescu A. Congenital diaphragmatic hernia - A case report. Archives of the Balkan Medical Union. 2013;48(4):431-4.
Canadian Congenital Diaphragmatic Hernia Collaborative, Puligandla PS, Skarsgard ED, et al. Diagnosis and management of congenital diaphragmatic hernia: a clinical practice guideline. CMAJ. 2018;190(4):E103-12.
Noori S, Friedlich P, Wong P, Garingo A, Seri I. Cardiovascular effects of sildenafil in neonates and infants with congenital diaphragmatic hernia and pulmonary hypertension. Neonatology. 2007;91(2):92-100.
Patel N. Use of milrinone to treat cardiac dysfunction in infants with pulmonary hypertension secondary to congenital diaphragmatic hernia: a review of six patients. Neonatology. 2012;102(2):130-6.
Lawrence KM, Berger K, Herkert L, Franciscovich C, O'Dea CLH, Waqar LN, et al. Use of prostaglandin E1 to treat pulmonary hypertension in congenital diaphragmatic hernia. J Pediatr Surg. 2019;54(1):55-9.
Chatziioannidis I, Mouravas V, Babatseva E. When is the appropriate timing of surgical repair for congenital diaphragmatic hernia? Hippokratia. 2014;18(4):381.
Spinelli C, Ghionzoli M, Costanzo S, Pistolesi F, Massart F. Acute respiratory distress for late presenting congenital diaphragmatic hernia. Turk J Pediatr. 2008;50(5):480-4.
Kim DJ, Chung JH. Late-presenting congenital diaphragmatic hernia in children: the experience of single institution in Korea. Yonsei Med J. 2013;54(5):1143-8.