Musculoskeletal deformities following repair of large congenital diaphragmatic hernias

Published:February 07, 2014DOI:



      Large congenital diaphragmatic hernias (CDH) can be repaired with either a muscle flap or prosthetic patch. The purpose of this study was to assess the frequency and severity of scoliosis, chest wall, and abdominal wall deformities following these repairs.


      Neonates who underwent CDH repair (1989–2012) were retrospectively reviewed. We then validated our retrospective review by comparing results of a focused radiologic evaluation and clinical examination of patients with large defects seen in prospective follow-up clinic. Tests for association were made using Fisher’s exact test.


      236 patients survived at least 1 year. Of these patients, 30 had a muscle flap, and 13 had a patch repair. Retrospectively, we identified pectus in 9% of primary repairs, 47% of flap repairs, and 54% of patch repairs. We identified scoliosis in 7% of primary repairs, 13% of flap repairs, and 15% of patch repairs. Prospectively, 75% of flap patients and 67% of patch patients had pectus and 13% of flap patients and 33% of patch patients had scoliosis. There was no significant difference between flap and patch patients.


      Scoliosis and pectus deformity were common in children with large CDH. The operative technique did not appear to affect the incidence of subsequent skeletal deformity.

      Key words

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of Pediatric Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Clark R.H.
        • Hardin W.D.
        • Hirschl R.B.
        • et al.
        Current surgical management of congenital diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study Group.
        J Pediatr Surg. 1998; 33: 1004-1009
        • Rajabi-Mashhadi M.T.
        • Ebrahimi M.
        • Mobarhan M.G.
        • et al.
        Prevalence of chest wall deformities in a large sample of Iranian children aged 7–14 years.
        Iran J Pediatr. 2010; 20: 221-224
        • Nasr A.
        • Struijs M.-C.
        • Ein S.H.
        • et al.
        Outcomes after muscle flap vs prosthetic patch repair for large congenital diaphragmatic hernias.
        J Pediatr Surg. 2010; 45: 151-154
        • Kuklová P.
        • Zemková D.
        • Kyncl M.
        • et al.
        Large diaphragmatic defect: are skeletal deformities preventable?.
        Pediatr Surg Int. 2011; 27: 1343-1349
        • Vanamo K.
        • Peltonen J.
        • Rintala R.
        • et al.
        Chest wall and spinal deformities in adults with congenital diaphragmatic defects.
        J Pediatr Surg. 1996; 31: 851-854
        • Jancelewicz T.
        • Chiang M.
        • Oliveira C.
        • et al.
        Late surgical outcomes among congenital diaphragmatic hernia (CDH) patients: why long-term follow-up with surgeons is recommended.
        J Pediatr Surg. 2013; 48: 935-941
        • Jaillard S.M.
        • Pierrat V.
        • Dubois A.
        • et al.
        Outcome at 2 years of infants with congenital diaphragmatic hernia: a population-based study.
        Ann Thorac Surg. 2003; 75: 250-256
        • Parot R.
        • Bouhafs A.
        • Garin C.
        • et al.
        Scoliosis and congenital diaphragmatic agenesis.
        Rev Chir Orthop Reparatrice Appar Mot. 2002; 88: 760-766
        • Barnhart D.C.
        • Jacques E.
        • Scaife E.R.
        • et al.
        Split abdominal wall muscle flap repair vs patch repair of large congenital diaphragmatic hernias.
        J Pediatr Surg. 2012; 47: 81-86
        • Scaife E.R.
        • Johnson D.G.
        • Meyers R.L.
        • et al.
        The split abdominal wall muscle flap—a simple, mesh-free approach to repair large diaphragmatic hernia.
        J Pediatr Surg. 2003; 38: 1748-1751
      1. Section on Surgery and the Committee on Fetus and Newborn. Postdischarge follow-up of infants with congenital diaphragmatic hernia.
        Pediatrics. 2008; 121: 627-632
        • De Boeck K.
        • Smith J.
        • Van Lierde S.
        • et al.
        Flat chest in survivors of bronchopulmonary dysplasia.
        Pediatr Pulmonol. 1994; 18: 104-107
        • Chiu P.P.L.
        • Sauer C.
        • Mihailovic A.
        • et al.
        The price of success in the management of congenital diaphragmatic hernia: is improved survival accompanied by an increase in long-term morbidity?.
        J Pediatr Surg. 2006; 41: 888-892
        • Losty P.D.
        • Pacheco B.A.
        • Manganaro T.F.
        • et al.
        Prenatal hormonal therapy improves pulmonary morphology in rats with congenital diaphragmatic hernia.
        J Surg Res. 1996; 65: 42-52
        • Hedrick H.L.
        • Kaban J.M.
        • Pacheco B.A.
        • et al.
        Prenatal glucocorticoids improve pulmonary morphometrics in fetal sheep with congenital diaphragmatic hernia.
        J Pediatr Surg. 1997; 32 ([discussion 221–2]): 217-221
        • Reamy B.V.
        • Slakey J.B.
        Adolescent idiopathic scoliosis: review and current concepts.
        Am Fam Physician. 2001; 64: 111-116
        • Goretsky M.
        • Kelly R.
        • Croitoru D.
        • et al.
        Chest wall anomalies: pectus excavatum and pectus carinatum.
        Adolesc Med. 2004; 15: 455-471
        • Hong J.Y.
        • Suh S.W.
        • Park H.J.
        • et al.
        Correlations of adolescent idiopathic scoliosis and pectus excavatum.
        J Pediatr Orthop. 2011; 31: 870-874
        • Lund D.P.
        • Mitchell J.
        • Kharasch V.
        • et al.
        Congenital diaphragmatic hernia: the hidden morbidity.
        J Pediatr Surg. 1994; 29 ([discussion 262–4]): 258-262
        • Jancelewicz T.
        • Vu L.T.
        • Keller R.L.
        • et al.
        Long-term surgical outcomes in congenital diaphragmatic hernia: observations from a single institution.
        J Pediatr Surg. 2010; 45: 155-160
        • Valfrè L.
        • Braguglia A.
        • Conforti A.
        • et al.
        Long term follow-up in high-risk congenital diaphragmatic hernia survivors: patching the diaphragm affects the outcome.
        J Pediatr Surg. 2011; 46: 52-56
        • Trachsel D.
        • Selvadurai H.
        • Bohn D.
        • et al.
        Long-term pulmonary morbidity in survivors of congenital diaphragmatic hernia.
        Pediatr Pulmonol. 2005; 39: 433-439
        • Tsang T.M.
        • Tam P.K.
        • Dudley N.E.
        • et al.
        Diaphragmatic agenesis as a distinct clinical entity.
        J Pediatr Surg. 1995; 30: 16-18
        • Stefanutti G.
        • Filippone M.
        • Tommasoni N.
        • et al.
        Cardiopulmonary anatomy and function in long-term survivors of mild to moderate congenital diaphragmatic hernia.
        J Pediatr Surg. 2004; 39: 526-531
        • Brant-Zawadzki P.B.
        • Fenton S.J.
        • Nichol P.F.
        • et al.
        The split abdominal wall muscle flap repair for large congenital diaphragmatic hernias on extracorporeal membrane oxygenation.
        J Pediatr Surg. 2007; 42: 1047-1051