Bilateral Congenital Diaphragmatic Hernia

Congenital diaphragmatic hernia (CDH) includes the anterior Morgagni hernia, hiatal hernia and the posterolateral Bochdalek hernia. Most CDH are Bochdalek type (85%). Bilateral CDH is extremely rare occurring in 1% of all CDH cases. Most of them die in utero while 25-35% of them survive. Antenatal diagnosis is possible with ultrasonography showing minimal mediastinal shift, bowel In thorax, small abdominal circumference and polyhydramnios. The prognosis of CDH depends on the degree of pulmonary hypoplasia and pulmonary hypertension caused by the intrinsic intrauterine pressure of the defect on the developing lung of the fetus. Furthermore a higher prevalence of major congenital anomalies (chromosomal and cardiac) has been reported for bilateral when compared with unilateral CDH. A prenatal diagnosis is more frequent among non-survivors compared to survivors due to the challenging nature of establishing the diagnosis of bilaterality. The patient with the largest defect and smaller lungs carries the worst prognosis. Most bilateral CDH usually have an acute presentation, but if the pulmonary impairment is subtle it can have a delayed presentation with an excellent prognosis. The rate of surgical repair of bilateral CDH is 50%. Most of these cases of bilateral CDH are repaired with a subcostal abdominal incision, though there has been scattered reports of staged bilateral thoracoscopic repair. Two-third of repaired cases will need a patch. Larger defect size, needing a patch repair, correlates with a more severe disease and mortality. The mortality of patients with bilateral CDH using ECMO is significantly larger that with unilateral CDH. 60% of the surviving patients are in need of pulmonary support at 30 days of life which would indicate a similar poor long-term prognosis of bilateral CDH patients at one and five years. Lower Apgar scores, prenatal diagnosis, the need for ECMO treatment, and patch repair have shown to be associated with higher mortality.

References:
1- Said SM, Moir CR, Ishitani MB, Zarroug AE: Successful thoracoscopic staged repair of bilateral congenital diaphragmatic hernia. J Pediatr Surg. 45(4):E5-8, 2010
2- Dhua AK, Aggarwal SK, Mathur N, Sethi G: Bilateral congenital diaphragmatic hernia. APSP J Case Rep. 3(3):20, 2012
3- James JP, Josephine JJ, Ponniah M: Late-presenting bilateral congenital diaphragmatic hernia: An extremely rare confluence of two rarities.Indian J Anaesth. 58(6):768-70, 2014
4- Botden SM, Heiwegen K, van Rooij IA, Scharbatke H, Lally PA, van Heijst A, de Blaauw I; Congenital Diaphragmatic Hernia Study Group. Bilateral congenital diaphragmatic hernia: prognostic evaluation of a large international cohort. J Pediatr Surg. 52(9):1475-1479, 2017
5- Oliveira PH, Piedade C, Conceiao V, Ramos M, Castro AO: Bilateral congenital diaphragmatic hernia with delayed diagnosis. Eur J Pediatr Surg. 23(4):322-4, 2013
6- Eroglu D, Yanik F, Sakallioglu AE, Arikan U, Varan B, Kuscu E: Prenatal diagnosis of bilateral diaphragmatic hernia by fetal sonography. J Obstet Gynaecol Res. 32(1):90-3, 2006



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