Eur J Pediatr Surg 2020; 30(05): 434-439
DOI: 10.1055/s-0039-1692165
Original Article

Malrotation in Congenital Diaphragmatic Hernia: Is It Really a Problem?

Kim Heiwegen
1   Department of Pediatric Surgery, Radboudumc-Amalia Children's Hospital, Nijmegen, The Netherlands
,
Ivo de Blaauw
1   Department of Pediatric Surgery, Radboudumc-Amalia Children's Hospital, Nijmegen, The Netherlands
,
Julia van Ling
1   Department of Pediatric Surgery, Radboudumc-Amalia Children's Hospital, Nijmegen, The Netherlands
,
Sanne M. B. I. Botden
1   Department of Pediatric Surgery, Radboudumc-Amalia Children's Hospital, Nijmegen, The Netherlands
› Author Affiliations

Abstract

Introduction Congenital diaphragmatic hernia (CDH) has been associated with other congenital malformations, such as intestinal rotation abnormalities. However, there is no standard evaluation and treatment of intestinal rotation during initial CDH repair. This study evaluates intestinal malrotation in CDH patients.

Materials and Methods All patients with a CDH treated in a high-volume center between 2000 and 2015 were retrospectively evaluated. Demographics, gastrointestinal characteristics, surgical treatment, and abdominal outcomes (acute surgery, small bowel obstruction, and volvulus) were described.

Results A total of 197 CDH patients were surgically repaired. In 76 (39%) patients, a malrotation was described at initial CDH repair, in 39 (20%) patients, a normal rotation, but 82 patients (42%) had no report on intestinal rotation. During follow-up (range: 2–16 years), 12 additional malrotations were diagnosed, leading to a prevalence of at least 45% (n = 88). These were mostly diagnosed due to acute small bowel obstruction, of which three had a volvulus. Of the 12 later diagnosed malrotations, 58% required acute surgery, compared to 3% of the 76 initially diagnosed patients (p < 0.001).

Conclusion Malrotation is associated with CDH, with a prevalence of at least 45% and in 21% a normal intestinal rotation. “Missed” malrotations have a higher risk on need for acute surgery later in life.



Publication History

Received: 17 January 2019

Accepted: 07 April 2019

Article published online:
14 June 2019

© 2020. Thieme. All rights reserved.

Georg Thieme Verlag KG
Stuttgart · New York

 
  • References

  • 1 Pérez-Egido L, Parente A, Cerdá JA. Acute gastric volvulus and congenital diaphragmatic hernia, case report and review. Afr J Paediatr Surg 2015; 12 (03) 200-202
  • 2 Fox C, Stewart M, King SK, Patel N. Acute gastrointestinal compromise in neonates with congenital diaphragmatic hernia prior to repair. J Pediatr Surg 2016; 51 (12) 1917-1920
  • 3 Baoquan Q, Diez-Pardo JA, Tovar JA. Intestinal rotation in experimental congenital diaphragmatic hernia. J Pediatr Surg 1995; 30 (10) 1457-1462
  • 4 Hosgor M, Karaca I, Karkiner A. , et al. Associated malformations in delayed presentation of congenital diaphragmatic hernia. J Pediatr Surg 2004; 39 (07) 1073-1076
  • 5 Basani L, Aepala R, Reddy BM. Congenital diaphragmatic hernia, Meckel's diverticulum and malrotation in a 3-month-old infant. Afr J Paediatr Surg 2016; 13 (01) 47-49
  • 6 Heiwegen K, de Blaauw I, Janssen S, van Rooij I, van Heijst A, Botden S. Short term surgical outcomes in the treatment of congenital diaphragmatic hernia: an overview of 15 years experience. Ann Emerg Surg 2017; 2 (01) 1005
  • 7 Peetsold MG, Heij HA, Kneepkens CM, Nagelkerke AF, Huisman J, Gemke RJ. The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity. Pediatr Surg Int 2009; 25 (01) 1-17
  • 8 Schoenwolf GC, Bleyl SB, Brauer PR, Francis-West PH. Larsen's Human Embryology. 4th ed. Philadelphia: 2009
  • 9 Zani A, Eaton S, Puri P. , et al; EUPSA Network Office. International Survey on the management of congenital diaphragmatic hernia. Eur J Pediatr Surg 2016; 26 (01) 38-46
  • 10 Graziano K, Islam S, Dasgupta R. , et al. Asymptomatic malrotation: diagnosis and surgical management: An American Pediatric Surgical Association outcomes and evidence based practice committee systematic review. J Pediatr Surg 2015; 50 (10) 1783-1790
  • 11 Al-Salem AH. Congenital hernia of Morgagni in infants and children. J Pediatr Surg 2007; 42 (09) 1539-1543
  • 12 Elhalaby EA, Abo Sikeena MH. Delayed presentation of congenital diaphragmatic hernia. Pediatr Surg Int 2002; 18 (5-6): 480-485
  • 13 Rescorla FJ, Shedd FJ, Grosfeld JL, Vane DW, West KW. Anomalies of intestinal rotation in childhood: analysis of 447 cases. Surgery 1990; 108 (04) 710-715
  • 14 Levin TL, Liebling MS, Ruzal-Shapiro C, Berdon WE, Stolar CJ. Midgut malfixation in patients with congenital diaphragmatic hernia: what is the risk of midgut volvulus?. Pediatr Radiol 1995; 25 (04) 259-261
  • 15 Askegard-Giesmann JR, Amah CC, Kenney BD. Intestinal malrotation and midgut volvulus. Paediatric Surgery: A Comprehensive Textbook for Africa,-ed Seattle. Global-HELP Organization; 2010: 393-396 Available at: https://global-help-publications.storage.googleapis.com/books/help_pedsurgeryafrica65.pdf. Accessed June 4, 2019
  • 16 Al-Salem AH, Nawaz A, Matta H, Jacobsz A. Herniation through the foramen of Morgagni: early diagnosis and treatment. Pediatr Surg Int 2002; 18 (2-3): 93-97