Results of diaphragm agenesis repair

Authors

DOI:

https://doi.org/10.15574/SP.2024.7(143).4652

Keywords:

congenital diaphragmatic hernia, diaphragm agenesis, recurrence, thoracalization, polytetrafluoroethylene

Abstract

Aim: assessment of short-term outcomes in children after diaphragmatic agenesis repair and selection of the optimal method for defect repair.

Materials and methods. This study involved 10 newborns with congenital diaphragmatic hernia (type D) who underwent surgery in one hospital between 2000 and 2023, followed by an assessment of complications, mortality, and the effectiveness of the thoracalization concept of the abdominal cavity.

Results. There were 6 (60%) male patients and 4 (40%) female patients. Right-sided diaphragmatic dome agenesis was diagnosed in 2 (20%) children and left-sided in 8 (80%). Early total mortality in this group was 60%. The surgical approach for treatment was thoracotomy in 80% (n=8) of cases, and in 2 (20%) cases transverse subcostal laparotomy was chosen. All diaphragmatic agenesis repairs were performed using patches (mostly made of polytetrafluoroethylene), with 8 of them applying the principle of thoracalization of the abdominal cavity. Indications for reoperations in our group included hernia recurrence - 2 cases (in one patient at 6 and 15 months) and intestinal obstruction caused by malrotation - 1 case.

Conclusions. Patients with diaphragmatic agenesis have higher mortality compared to the group with small and medium defects. The size of the diaphragmatic defect is an independent prognostic factor and may serve as a marker for the degree of pulmonary hypoplasia and pulmonary hypertension. The optimal method of surgical treatment is diaphragmatic repair using a synthetic polytetrafluoroethylene patch with thoracalization of the abdominal cavity.

The study was carried out in accordance with the principles of the Declaration of Helsinki. The study protocol was approved by the Local Ethical Committee of these institutions. The informed consent of the children's parents was obtained for the research.

No conflict of interests was declared by the authors.

Author Biographies

Ye.O. Rudenko, Bogomolets National Medical University, Kyiv

National Children’s Specialized Hospital “OHMATDYT”, Kyiv, Ukraine

I.I. Shulzhyk, Bogomolets National Medical University, Kyiv

National Children’s Specialized Hospital “OHMATDYT”, Kyiv, Ukraine

References

Aydin E, Lim FY, Kingma P, Haberman B, Rymeski B et al. (2019, Mar). Congenital diaphragmatic hernia: the good, the bad, and the tough. Pediatr Surg Int. 35(3): 303-313. Epub 2019 Jan 24. https://doi.org/10.1007/s00383-019-04442-z; PMid:30680439

Burgos CM, Frenckner B, Luco M, Harting MT, Lally PA, Lally KP et al. (2019, Apr). Prenatally versus postnatally diagnosed congenital diaphragmatic hernia - Side, stage, and outcome. J Pediatr Surg. 54(4): 651-655. Epub 2018 Apr 14. https://doi.org/10.1016/j.jpedsurg.2018.04.008; PMid:29753526

Burgos CM, Modée A, Öst E, Frenckner B. (2017, Apr). Addressing the causes of late mortality in infants with congenital diaphragmatic hernia. J Pediatr Surg. 52(4): 526-529. Epub 2016 Sep 5. https://doi.org/10.1016/j.jpedsurg.2016.08.028; PMid:27624564

Chatterjee D, Ing RJ, Gien J. (2020, Sep). Update on Congenital Diaphragmatic Hernia. Anesth Analg. 131(3): 808-821. https://doi.org/10.1213/ANE.0000000000004324; PMid:31335403

Chock VY, Danzer E, Chung S, Noh CY, Ebanks AH, Harting MT et al. (2022, Jan). In-Hospital Morbidities for Neonates with Congenital Diaphragmatic Hernia: The Impact of Defect Size and Laterality. J Pediatr. 240: 94-101.e6. Epub 2021 Sep 7. https://doi.org/10.1016/j.jpeds.2021.09.001; PMid:34506854

Cioci AC, Urrechaga EM, Parreco J, Remer LF, Cowan M, Perez EA et al. (2021, Sep). One-year outcomes of congenital diaphragmatic hernia repair: Factors associated with recurrence and complications. J Pediatr Surg. 56(9): 1542-1546. Epub 2020 Oct 1. https://doi.org/10.1016/j.jpedsurg.2020.09.018; PMid:33268050

Cordier AG, Russo FM, Deprest J, Benachi A. (2020, Feb). Prenatal diagnosis, imaging, and prognosis in Congenital Diaphragmatic Hernia. Semin Perinatol. 44(1): 51163. Epub 2019 Jul 30. https://doi.org/10.1053/j.semperi.2019.07.002; PMid:31439324

Coughlin MA, Werner NL, Gajarski R, Gadepalli S, Hirschl R, Barks J et al. (2016, Jul). Prenatally diagnosed severe CDH: mortality and morbidity remain high. J Pediatr Surg. 51(7): 1091-1095. Epub 2015 Nov 10. https://doi.org/10.1016/j.jpedsurg.2015.10.082; PMid:26655216

Deprest JA, Nicolaides KH, Benachi A, Gratacos E, Ryan G, Persico N et al. (2021, Jul 8). Randomized Trial of Fetal Surgery for Severe Left Diaphragmatic Hernia. N Engl J Med. 385(2): 107-118. Epub 2021 Jun 8. https://doi.org/10.1056/NEJMoa2027030; PMid:34106556 PMCid:PMC7613453

Gupta VS, Harting MT, Lally PA, Miller CC, Hirschl RB, Davis CF et al. (2023, Mar 1). Mortality in Congenital Diaphragmatic Hernia: A Multicenter Registry Study of Over 5000 Patients Over 25 Years. Ann Surg. 277(3): 520-527. Epub 2021 Jul 29. https://doi.org/10.1097/SLA.0000000000005113; PMid:34334632

Harting MT, Lally KP. (2014, Dec). The Congenital Diaphragmatic Hernia Study Group registry update. Semin Fetal Neonatal Med. 19(6): 370-375. Epub 2014 Oct 11. https://doi.org/10.1016/j.siny.2014.09.004; PMid:25306471

Kirby E, Keijzer R. (2020, Apr). Congenital diaphragmatic hernia: current management strategies from antenatal diagnosis to long-term follow-up. Pediatr Surg Int. 36(4): 415-429. Epub 2020 Feb 18. https://doi.org/10.1007/s00383-020-04625-z; PMid:32072236

Kokhanov A, Lau C, Garg M, Jen H, Chu A. (2022, Jul 8). ECMO utilization in infants with congenital diaphragmatic hernia in the USA. World J Pediatr Surg. 5(4): e000393. https://doi.org/10.1136/wjps-2021-000393; PMid:36474734 PMCid:PMC9717354

Kryvchenya DYu, Benzar IM, Rudenko Ye O, Shulzhyk II. (2015). Reoperations in children with CDH from the high-risk group. Neonatolohiia, khirurhiia ta perynatalna medytsyna. 4(54): 75-81.

Kryvchenia DYu, Rudenko EO, Shulzhyk II. (2022). The main risk factors for reoperations in children with congenital diaphragmatic hernias. Paediatric Surgery (Ukraine). 1(74): 20-26. https://doi.org/10.15574/PS.2022.74.20

Macchini F, Raffaeli G, Amodeo I, Ichino M, Encinas JL, Martinez L et al. (2022, Feb 9). Recurrence of Congenital Diaphragmatic Hernia: Risk Factors, Management, and Future Perspectives. Front Pediatr. 10: 823180. https://doi.org/10.3389/fped.2022.823180; PMid:35223699 PMCid:PMC8864119

Mishalany HG, Nakada K, Woolley MM. (1979). Congenital diaphragmatic hernias: eleven years' experience. Arch Surg. 114: 1118-1123. https://doi.org/10.1001/archsurg.1979.01370340024003; PMid:485820

Morini F, Lally PA, Lally KP, Bagolan P. (2015, Dec). The Congenital Diaphragmatic Hernia Study Group Registry. Eur J Pediatr Surg. 25(6): 488-496. Epub 2015 Dec 7. https://doi.org/10.1055/s-0035-1569151; PMid:26642385

Morini F, Lally KP, Lally PA, Crisafulli RM, Capolupo I, Bagolan P. (2017, Sep 14). Treatment Strategies for Congenital Diaphragmatic Hernia: Change Sometimes Comes Bearing Gifts. Front Pediatr. 5: 195. https://doi.org/10.3389/fped.2017.00195; PMid:28959686 PMCid:PMC5603669

Paoletti M, Raffler G, Gaffi MS, Antounians L, Lauriti G, Zani A. (2020, Nov). Prevalence and risk factors for congenital diaphragmatic hernia: A global view. J Pediatr Surg. 55(11): 2297-2307. Epub 2020 Jun 20. https://doi.org/10.1016/j.jpedsurg.2020.06.022; PMid:32690291

Perrone EE, Deprest JA. (2021, May). Fetal endoscopic tracheal occlusion for congenital diaphragmatic hernia: a narrative review of the history, current practice, and future directions. Transl Pediatr. 10(5): 1448-1460. https://doi.org/10.21037/tp-20-130; PMid:34189104 PMCid:PMC8192998

Rafat N, Schaible T. (2019, Aug 8). Extracorporeal Membrane Oxygenation in Congenital Diaphragmatic Hernia. Front Pediatr. 7: 336. https://doi.org/10.3389/fped.2019.00336; PMid:31440491 PMCid:PMC6694279

Robertson JO, Criss CN, Hsieh LB, Matsuko N, Gish JS, Mon RA et al. (2018, Apr). Comparison of early versus delayed strategies for repair of congenital diaphragmatic hernia on extracorporeal membrane oxygenation. J Pediatr Surg. 53(4): 629-634. Epub 2017 Nov 22. https://doi.org/10.1016/j.jpedsurg.2017.10.056; PMid:29173775

Snoek KG, Reiss IK, Greenough A, Capolupo I, Urlesberger B, Wessel L et al. (2016). Standardized Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus - 2015 Update. Neonatology. 110(1): 66-74. Epub 2016 Apr 15. https://doi.org/10.1159/000444210; PMid:27077664

Vieira R, Pearse R, Rankin J. (2018, Oct 2). Mortality factors in infants with congenital diaphragmatic hernia: A systematic review. Birth Defects Res. 110(16): 1241-1249. Epub 2018 Sep 10. https://doi.org/10.1002/bdr2.1376; PMid:30198646

Yamoto M, Nagata K, Terui K, Hayakawa M, Okuyama H, Amari S et al. (2022, Jun 8). Long-Term Outcomes of Congenital Diaphragmatic Hernia: Report of a Multicenter Study in Japan. Children (Basel). 9(6): 856. https://doi.org/10.3390/children9060856; PMid:35740795 PMCid:PMC9222080

Yokota K, Uchida H, Kaneko K, Ono Y, Murase N et al. (2014, Sep). Surgical complications, especially gastroesophageal reflux disease, intestinal adhesion obstruction, and diaphragmatic hernia recurrence, are major sequelae in survivors of congenital diaphragmatic hernia. Pediatr Surg Int. 30(9): 895-899. Epub 2014 Aug 9. https://doi.org/10.1007/s00383-014-3575-x; PMid:25106890

Yu PT, Jen HC, Rice-Townsend S, Guner YS. (2020, Feb). The role of ECMO in the management of congenital diaphragmatic hernia. Semin Perinatol. 44(1): 151166. Epub 2019 Jul 30. https://doi.org/10.1053/j.semperi.2019.07.005; PMid:31472951

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Published

2024-11-28

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