Document Type : case report


1 Department of Anesthesiology, Intensive Care, Rescue and Pain Medicine, Cantonal Hospital St. Gallen, Rorschacher Strasse 95, 9007 St. Gallen, Switzerland

2 Department of Anesthesiology, Intensive Care, Rescue and Pain Medicine, Cantonal Hospital St. Gallen, Rorschacher Strasse 95, 9007 St. Gallen, Switzerland Department of Anesthesiology, Children’s Hospital of Eastern Switzerland,


Background: Congenital Diaphragmatic Hernia (CDH) is a rare developmental abnormality of the diaphragm with an incidence of approximately 2.5 cases per 10,000 births. Despite advances in treatment, mortality and long-term morbidity among survivors remain high.
Case Presentation: A newborn was delivered by planned caesarean section due to prenatal diagnosis of CDH, diagnosed at 18 weeks and 5 days (Correction 3.1) during routine ultrasound. The Observed-to-Expected (O/E) Lung area-to-Head circumference Ratio (LHR) was 52%. After birth, the newborn was in respiratory distress and required mechanical invasive ventilation. Surgical repair was planned after stabilisation for the second day of life. A subcostal laparotomy was performed, and an anteromedial hernia with mostly small intestine, the left colon flexure and the spleen as content was found. The abdominal contents were reduced, and the hernia was repaired with a suture. Due to the development of a severe pulmonary hypertension, extubation was only possible on the fifth postoperative day, but reintubation was needed. The patient remained in the intensive care unit for a total of 25 days and could be discharged 46 days after birth in a good condition.
Discussion: When treating patients with CDH, the most feared complication is pulmonary hypertension, which can be life-threatening and refractory to treatment. Surgical repair should be postponed until the newborn is medically stable.
Conclusion: CDH presents a diagnostic and therapeutic challenge. When diagnosed prenatally, patients should be referred to a tertiary centre for a multidisciplinary approach.


  1. McGivern MR, Best KE, Rankin J, Wellesley D, Greenlees R, Addor MC, Arriola L, Walle Hd, Barisic I, Beres J, Bianchi F, Calzolari E, Doray B, Draper ES, Garne E, Gatt M, Haeusler M, Khoshnood B, Klungsoyr K, Latos-Bielenska A, O'Mahony M, Braz P, McDonnell B, Mullaney C, Nelen V, Queisser-Luft A, Randrianaivo H, Rissmann A, Rounding C, Sipek A, Thompson R, Tucker D, Wertelecki W, Martos C. Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study. Arch Dis Child Fetal Neonatal Ed 2015 Mar; 100(2):F137–44.
  2. Deprest J, Brady P, Nicolaides K, Benachi A, Berg C, Vermeesch J, Gardener G, Gratacos E. Prenatal management of the fetus with isolated congenital diaphragmatic hernia in the era of the TOTAL trial. Semin Fetal Neonatal Med 2014 Dec; 19(6):338–48.
  3. Chatterjee D, Ing RJ, Gien J. Update on Congenital Diaphragmatic Hernia. Anesth Analg 2020 Sep; 131(3):808–21.
  4. Salas GL, Otaño JC, Cannizzaro CM, Mazzucchelli MT, Goldsmit GS. Congenital diaphragmatic hernia: postnatal predictors of mortality. Arch Argent Pediatr 2020 Jun; 118(3):173–9.
  5. Moore KL, Persaud TVN, Torchia MG. The developing human: clinically oriented embryology. 8th Ed. New York: Saunders/Elsevier; 2008.
  6. Veenma DC, de Klein A, Tibboel D. Developmental and genetic aspects of congenital diaphragmatic hernia. Pediatr Pulmonol 2012 Jun; 47(6):534–45.
  7. Blumenfeld YJ, Belfort MA. New approaches to congenital diaphragmatic hernia. Curr Opin Obstet Gynecol 2020 Apr; 32(2):121–7.
  8. Skari H, Bjornland K, Haugen G, Egeland T, Emblem R. Congenital diaphragmatic hernia: a meta-analysis of mortality factors. J Pediatr Surg 2000 Aug; 35(8):1187–97.
  9. Kirby E, Keijzer R. Congenital diaphragmatic hernia: current management strategies from antenatal diagnosis to long-term follow-up. Pediatr Surg Int 2020 Apr; 36(4):415–29.
  10. Metkus AP, Filly RA, Stringer MD, Harrison MR, Adzick NS. Sonographic predictors of survival in fetal diaphragmatic hernia. J Pediatr Surg 1996 Jan; 31(1):148–51; discussion 151–2.
  11. Cordier AG, Russo FM, Deprest J, Benachi A. Prenatal diagnosis, imaging, and prognosis in Congenital Diaphragmatic Hernia. Semin Perinatol 2020 Feb; 44(1):51163.
  12. Werneck Britto IS, Olutoye OO, Cass DL, Zamora IJ, Lee TC, Cassady CI, Mehollin-Ray A, Welty S, Fernandes C, Belfort MA, Lee W, Ruano R. Quantification of liver herniation in fetuses with isolated congenital diaphragmatic hernia using two-dimensional ultrasonography. Ultrasound Obstet Gynecol 2015 Aug; 46(2):150–4.
  13. Puligandla PS, Skarsgard ED, Offringa M, Adatia I, Baird R, Bailey M, Brindle M, Chiu P, Cogswell A, Dakshinamurti S, Flageole H, Keijzer R, McMillan D, Oluyomi-Obi T, Pennaforte T, Perreault T, Piedboeuf B, Patricia Riley S, Ryan G, Synnes A, Traynor M. Diagnosis and management of congenital diaphragmatic hernia: a clinical practice guideline. CMAJ 2018 Jan 29; 190(4):E103–12.
  14. Snoek KG, Reiss IK, Greenough A, Capolupo I, Urlesberger B, Wessel L, Storme L, Deprest J, Schaible T, Heijst Av, Tibboel D; CDH EURO Consortium. Standardised Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus - 2015 Update. Neonatology 2016; 110(1):66–74.
  15. 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.
  16. 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.
  17. Barbaro RP, Paden ML, Guner YS, Raman L, Ryerson LM, Alexander P, Nasr VG, Bembea MM, Rycus PT, Thiagarajan RR; ELSO member centers. Pediatric Extracorporeal Life Support Organization Registry International Report 2016. ASAIO J 2017 Jul/Aug; 63(4):456–63.
  18. Guner YS, Harting MT, Fairbairn K, Delaplain PT, Zhang L, Chen Y, Kabeer MH, Yu P, Cleary JP, Stein JE, Stolar C, Nguyen DV. Outcomes of infants with congenital diaphragmatic hernia treated with venovenous versus venoarterial extracorporeal membrane oxygenation: A propensity score approach. J Pediatr Surg 2018 Nov; 53(11):2092–9.
  19. Dingeldein M. Congenital Diaphragmatic Hernia: Management & Outcomes. Adv Pediatr 2018 Aug; 65(1):241–7.