Congenital diaphragmatic eventration in children

Authors

DOI:

https://doi.org/10.15574/PS.2025.2(87).8693

Keywords:

diaphragmatic eventration, diagnostic, management, mini-invasive surgery

Abstract

Diaphragmatic hernia and eventration represent the most common disorders of the diaphragm. Congenital diaphragmatic eventration is a relatively rare pathology characterized by the partial or whole bulging of one or both hemidiaphragms without interruption of its integrity.

The aim of this study was to analyze the data of etiology, clinical presentation, diagnosis, and methods of surgery in case of diaphragmatic eventration.

Diaphragmatic eventration is divided into congenital and acquired, total and partial. Congenital diaphragmatic eventration may be an isolated malformation or associated with chromosomal disorders, other congenital malformations, or be part of various congenital syndromes and developed due to the intrauterine infection. Clinical presentation varied from an asymptomatic course to a life-threatening distress syndrome. Chest radiographs, ultrasonography, computed and magnetic resonance tomography are applied for the eventration diagnosis. The different methods (open and mini-invasive) and approaches (thoracotomy and laparotomy) for the management of diaphragmatic eventration were analyzed.

Conclusions. Diaphragmatic eventration in children can cause severe respiratory disorders, which determines the necessity for surgery. In asymptomatic children, follow-up observation is possible, but in case of the presence of respiratory symptoms, the surgery is indicated. The choice of surgery (open or mini-invasive) depends on surgeons’ skills and the localization of the eventration.

No conflict of interests was declared by the authors.

Author Biography

P.S. Rusak, Shupyk National Healthcare University of Ukraine, Kyiv

Zhytomyr regional children clinical hospital, Ukraine

References

Alamo L, Gudinchet F, Meuli R. (2015). Imaging findings in fetal diaphragmatic abnormalities. Pediatr Radiol. 45(13): 1887-1900. https://doi.org/10.1007/s00247-015-3418-5; PMid:26255159

Aldrich TK, Herman JH, Rochester DF. (1980). Bilateral diaphragmatic paralysis in the newborn infant. J Pediatr. 97(6): 988-991. https://doi.org/10.1016/S0022-3476(80)80442-2; PMid:7441433

Al-Ebrahim KE, Elassal AA, Eldib OS et al. (2019). Diaphragmatic palsy after cardiac surgery in adult and pediatric patients. Asian Cardiovasc Thorac Ann. 27(6): 481-485. https://doi.org/10.1177/0218492319859806; PMid:31246505

Alshorbagy A, Mubarak Y. (2015). Open transthoracic plication of the diaphragm for unilateral diaphragmatic eventration in infants and children. Korean J Thorac Cardiovasc Surg. 48(5): 307-310. https://doi.org/10.5090/kjtcs.2015.48.5.307; PMid:26509123 PMCid:PMC4622030

Bawazir OA, Banaja AM. (2020). Thoracoscopic repair of diaphragmatic eventration in children: a comparison of two repair techniques. J Pediatr Surg. 55(6): 1152-1156. https://doi.org/10.1016/j.jpedsurg.2019.11.019; PMid:31937447

Bisgard JD. (1947). Congenital eventration of the diaphragm. J Thorac Surg. 16(5): 484-491. https://doi.org/10.1016/S0096-5588(20)31367-2; PMid:20267954

Bishara J, Burjonrappa S, Pirzada M, Halaby C. (2015). Diaphragmatic eventration misdiagnosed as diaphragmatic hernia in a preterm infant. Chest J. 148(4); Suppl: 780A. https://doi.org/10.1378/chest.2243317

Borruto FA, Ferreira CG, Kaselas C et al. (2014). Thoracoscopic treatment of congenital diaphragmatic eventration in children: lessons learned after 15 years of experience. Eur J Pediatr Surg. 24(4): 328-331. https://doi.org/10.1055/s-0033-1349054; PMid:23784749

Bowerson M, Nelson VS, Yang LJ. (2010). Diaphragmatic paralysis associated with neonatal brachial plexus palsy. Pediatr Neurol. 42(3): 234-236. https://doi.org/10.1016/j.pediatrneurol.2009.11.005; PMid:20159438

Briggs VA, Reilly BJ, Loewig K. (1973). Lung hypoplasia and membranous diaphragm in the congenital rubella syndrome - A rare case. J Can Assoc Radiol. 24(2): 126-127.

Chaudhary K, Anand R, Girdhar KK et al. (2011). Anesthetic management of a patient with congenital diaphragmatic eventration. J Anesth. 25(4): 585-588. https://doi.org/10.1007/s00540-011-1171-y; PMid:21626261

Chavhan GB, Babyn PS, Cohen RA, Langer JC. (2010). Multimodality imaging of the pediatric diaphragm: anatomy and pathologic conditions. Radiographics. 30(7): 1797-1817. https://doi.org/10.1148/rg.307105046; PMid:21057121

Clifton MS, Wulkan ML. (2017). Congenital diaphragmatic hernia and diaphragmatic eventration. Clin Perinatol. 44(4): 773-779. https://doi.org/10.1016/j.clp.2017.08.011; PMid:29127959

Demos DS, Berry MF, Backhus LM et al. (2017). Video-assisted thoracoscopic diaphragm plication using a running suture technique is durable and effective. J Thorac Cardiovasc Surg. 153(5): 1182-1188. https://doi.org/10.1016/j.jtcvs.2016.11.062; PMid:28087113

Di Buono G, Bonventre G, Amato G et al. (2020). Successful laparoscopic management of congenital diaphragmatic relaxation: A case report. Int J Surg Case Rep. 77; Suppl.: S25-S28. https://doi.org/10.1016/j.ijscr.2020.09.055; PMid:32972887 PMCid:PMC7876739

Di Giorgio A, Cardini CL, Sammartino P et al. (2006). Dual-layer sandwich mesh repair in the treatment of major diaphragmatic eventration in an adult. J Thorac Cardiovasc Surg. 132(1): 187-189. https://doi.org/10.1016/j.jtcvs.2006.02.033; PMid:16798335

El-Halaby H, Abdel-Hady H, Alsawah G et al. (2016). Sonographic evaluation of diaphragmatic excursion and thickness in healthy infants and children. J Ultrasound Med. 35(1): 167-175. https://doi.org/10.7863/ultra.15.01082; PMid:26679203

El-Magd EA, Elgeidie A, Abbas A et al. (2024). Laparoscopic approach in the management of diaphragmatic eventration in adults: gastrointestinal surgical perspective. Updates Surg. 76(2): 555-563. https://doi.org/10.1007/s13304-023-01665-7; PMid:37847484 PMCid:PMC10995002

Faheem M, Fayad A. (1999). Diaphragmatic rupture after epidural anesthesia in a patient with diaphragmatic eventration. Eur J Anaesthesiol. 16(8): 574-576. https://doi.org/10.1046/j.1365-2346.1999.00538.x; PMid:10500951

Fujishiro J, Ishimaru T, Sugiyama M et al. (2016). Minimally invasive surgery for diaphragmatic diseases in neonates and infants. Surg Today. 46(7): 757-763. https://doi.org/10.1007/s00595-015-1222-3; PMid:27246508

Fujishiro J, Ishimaru T, Sugiyama M et al. (2015). Thoracoscopic plication for diaphragmatic eventration after surgery for congenital heart disease in children. J Laparoendosc Adv Surg Tech A. 25(4): 348-351. https://doi.org/10.1089/lap.2014.0260; PMid:25536425

Gibson GJ. (1989). Diaphragmatic paresis: pathophysiology, clinical features, and investigation. Thorax. 44(11): 960-970. https://doi.org/10.1136/thx.44.11.960; PMid:2688182 PMCid:PMC462156

Graham DR, Kaplan D, Evans CC et al. (1990). Diaphragmatic plication for unilateral diaphragmatic paralysis: a 10-year experience. Ann Thorac Surg. 49(2): 248-252. https://doi.org/10.1016/0003-4975(90)90146-W; PMid:2306146

Gupta A, Sidler M, van Poll D et al. (2020). Thoracic versus abdominal approach to correct diaphragmatic eventration in children. J Pediatr Surg. 55(2): 245-248. https://doi.org/10.1016/j.jpedsurg.2019.10.040; PMid:31761454

Güvenç BH, Korkmaz M, Avtan L et al. (2004). Thoracoscopic diaphragm plication in children and indications for conversion to open thoracotomy. J Laparoendosc Adv Surg Tech A. 14(5): 302-305. https://doi.org/10.1089/lap.2004.14.302; PMid:15630946

Heiwegen K, van Heijst AF, Daniels-Scharbatke H et al. (2020). Congenital diaphragmatic eventration and hernia sac compared to CDH with true defects: a retrospective cohort study. Eur J Pediatr. 179(6): 855-863. https://doi.org/10.1007/s00431-020-03576-w; PMid:31965300 PMCid:PMC7220865

Heng L, Alzahrani K, Montalva L et al. (2024). Congenital diaphragmatic eventration: Should we maintain surgical treatment? A retrospective multicentric cohort study. J Pediatr Surg. 60(1): 161991. Online ahead of print. https://doi.org/10.1016/j.jpedsurg.2024.161991; PMid:39442326

Hoshino Y, Arai J. (2020). Diaphragm ultrasound examination for congenital diaphragmatic eventration in two premature neonates. BMJ Case Rep. 13(2): e232813. https://doi.org/10.1136/bcr-2019-232813; PMid:32019758 PMCid:PMC7035794

Hu J, Wu Y, Wang J et al. (2014). Thoracoscopic and laparoscopic plication of the hemidiaphragm is effective in the management of diaphragmatic eventration. Pediatr Surg Int. 30(1): 19-24. https://doi.org/10.1007/s00383-013-3418-1; PMid:24100524

Huang D, Tang J, Wang F et al. (2021). The feasibility and safety of same-day surgery for diaphragmatic eventration by minithoracotomy in children. Pediatr Surg Int. 37(9): 1215-1220. https://doi.org/10.1007/s00383-021-04907-0; PMid:33864497

Hüttl TP, Wichmann MW, Reichart B et al. (2004). Laparoscopic diaphragmatic plication: long-term results of a novel surgical technique for postoperative phrenic nerve palsy. Surg Endosc. 18(3): 547-551. https://doi.org/10.1007/s00464-003-8127-8; PMid:15108692

Hwang Z, Shin JS, Cho YH et al. (2003). A simple technique for the thoracoscopic plication of the diaphragm. Chest. 124(1): 376-378. https://doi.org/10.1378/chest.124.1.376; PMid:12853548

Izumi K, Hokuto I, Yamaguchi S et al. (2010). Diaphragm dysfunction with congenital cytomegalovirus infection. J Perinatol. 30(10): 691-694. https://doi.org/10.1038/jp.2010.65; PMid:20877363

Joho-Arreola AL, Bauersfeld U, Stauffer UG et al. (2005). Incidence and treatment of diaphragmatic paralysis after cardiac surgery in children. Eur J Cardiothorac Surg. 27(1): 53-57. https://doi.org/10.1016/j.ejcts.2004.10.002; PMid:15621471

Joshi A, Kumar M, Acharya A. (2018). Bilateral congenital eventration of diaphragm: keep in mind, the other side. BMJ Case Rep. 2018: bcr2018226051. https://doi.org/10.1136/bcr-2018-226051; PMid:30317202 PMCid:PMC6194440

Jurcak-Zaleski S, Comstock CH, Kirk JS. (1990) Eventration of the diaphragm. Prenatal diagnosis. J. Ultrasound Med. 9(6): 351-354. https://doi.org/10.7863/jum.1990.9.6.351; PMid:2192085

Kang H, Lee S, Park H et al. (2019). Ultrasound-guided perioperative management of 28-month-old patient with congenital diaphragmatic eventration. SAGE Open Med Case Rep. 7: 2050313X19827737. https://doi.org/10.1177/2050313X19827737; PMid:30800303 PMCid:PMC6378513

Kansal AP, Chopra V, Chahal AS et al. (2009). Right-sided diaphragmatic eventration: a rare entity. Lung India. 26(2): 48-50. https://doi.org/10.4103/0970-2113.48898; PMid:20440395 PMCid:PMC2860415

Kapoor V, Wright IM. (2010) Congenital myotonic dystrophy with cardiac conduction defect and eventration of the diaphragm. J Pediatr Int. 52(1): e6-8. https://doi.org/10.1111/j.1442-200X.2009.02998.x; PMid:20158653

Karmazyn B, Shold AJ, Delaney LR et al. (2019). Ultrasound evaluation of right diaphragmatic eventration and hernia. Pediatr Radiol. 49(8): 1010-1017. https://doi.org/10.1007/s00247-019-04417-1; PMid:31139880

Keyes S, Spouge RJ, Kennedy P et al. (2024). Approach to acute traumatic and nontraumatic diaphragmatic abnormalities. Radiographics. 44(6): e230110. https://doi.org/10.1148/rg.230110; PMid:38781091

Kim DH, Sung SC, Kim H et al. (2021). Is the pleating technique superior to the invaginating technique for plication of diaphragmatic eventration in infants? J Pediatr Surg. 56(5): 995-999. https://doi.org/10.1016/j.jpedsurg.2020.07.015; PMid:32792164

Konstantinidi A, Liakou P, Kopanou Taliaka P et al. (2023). Congenital diaphragmatic eventration in the neonatal period: Systematic review of the literature and report of a rare case presenting with gastrointestinal disorders. Pediatr Rep. 15(3): 442-451. https://doi.org/10.3390/pediatric15030041; PMid:37606445 PMCid:PMC10443257

Kulkarni ML, Sneharoopa B, Vani HN et al. (2007). Eventration of the diaphragm and associations. Indian J Pediatr. 74(2): 202-205. https://doi.org/10.1007/s12098-007-0018-x; PMid:17337837

Le UT, Titze L, Hundeshagen P et al. (2023). Robotic diaphragm plication: functional and surgical outcomes of a single-center experience. Surg Endosc. 37(6): 4795-4802. https://doi.org/10.1007/s00464-023-09942-7; PMid:36914782 PMCid:PMC10235138

Leo F, Girotti P, Tavecchio L et al. (2010). Anterior diaphragmatic plication in mediastinal surgery: the ''reefing the mainsail'' technique. Ann Thorac Surg. 90(6): 2065-2067. https://doi.org/10.1016/j.athoracsur.2010.02.043; PMid:21095375

Maish MS. (2010). The diaphragm. Surg Clin North Am. 90(5): 955-968. https://doi.org/10.1016/j.suc.2010.07.005; PMid:20955877

Mahalingam H, Babu B, Rangasami R et al. (2020). Fetal magnetic resonance imaging in the evaluation of congenital diaphragmatic anomalies. Egypt J Radiol Nucl Med. 51: 221. https://doi.org/10.1186/s43055-020-00340-w

Maltby JR, Beriault MT, Watson NC et al. (2003). LMA-Classic and LMA-ProSeal are effective alternatives to endotracheal intubation for gynecologic laparoscopy. Can J Anaesth. 50(1): 71-77. https://doi.org/10.1007/BF03020191; PMid:12514155

Mitsiakos G, Gavras C, Katsaras GN et al. (2022). Parvovirus B19 intrauterine infection and eventration of the diaphragm. Prague Med Rep. 123(1): 48-55. https://doi.org/10.14712/23362936.2022.6; PMid:35248165

Moreno-Galarraga L, Bardaji C, Herranz Aguirre M, Viguria N. (2021). Diaphragmatic pathology in children: Not always an easy diagnosis. Pediatr Emerg Care. 37(11): e767-e768. https://doi.org/10.1097/PEC.0000000000001765; PMid:30829839

Mouroux J, Padovani B, Poirier NC et al. (1996). Technique for the repair of diaphragmatic eventration. Ann Thorac Surg. 62(3): 905-907. https://doi.org/10.1016/S0003-4975(96)00530-9; PMid:8784039

Mouroux J, Venissac N, Leo F et al. (2005). Surgical treatment of diaphragmatic eventration using video-assisted thoracic surgery: a prospective study. Ann Thorac Surg. 79(1): 308-312. https://doi.org/10.1016/j.athoracsur.2004.06.050; PMid:15620964

Muller NL, Bryan AC. (1979). Chest wall mechanics and respiratory muscles in infants. Pediatr Clin North Am. 26(3): 503-516. https://doi.org/10.1016/S0031-3955(16)33745-2; PMid:386235

Narayana Swamy DK, Goplakrishna K, Anbareesha M. (2007). PONV: comparison of TIVA with propofol versus inhalational anaesthesia. J Anaesthesiol Clin Pharmacol. 23(4): 387-390.

Nardini M, Jayakumar S, Migliore M et al. (2021). Minimally invasive plication of the diaphragm: A single-center prospective study. Innovations (Phila). 16(4): 343-349. https://doi.org/10.1177/15569845211011583; PMid:34130535

Nason LK, Walker CM, McNeeley MF et al. (2012). Imaging of the diaphragm: anatomy and function. Radiographics. 32(2): E51-E70. https://doi.org/10.1148/rg.322115127; PMid:22411950

Podgaetz E, Garza-Castillon R Jr, Andrade RS. (2016). Best approach and benefit of plication for paralyzed diaphragm. Thorac Surg Clin. 26(3): 333-346. https://doi.org/10.1016/j.thorsurg.2016.04.009; PMid:27427528

Rana A, Manchanda S, Dadhwal V. (2021). Congenital focal eventration of the left hemidiaphragm: diagnostic dilemma resolved on prenatal MRI. BMJ Case Rep. 14(8): e245771. https://doi.org/10.1136/bcr-2021-245771; PMid:34344663 PMCid:PMC8336125

Reed JA, Borden DL. (1935). Eventration of the diaphragm: With a report of two cases. Arch. Surg. 31(1): 30-64. https://doi.org/10.1001/archsurg.1935.01180130033003

Rizeq YK, Many BT, Vacek JC et al. (2020). Diaphragmatic paralysis after phrenic nerve injury in newborns. Pediatr Surg. 55(2): 240-244. https://doi.org/10.1016/j.jpedsurg.2019.10.038; PMid:31757507

Robinson AE, Gooneratne NS, Blackburn WR, Brogdon BG. (1980). Bilateral anteromedial defect of the diaphragm in children. AJR. Am J Roentgenol. 135(2): 301-306. https://doi.org/10.2214/ajr.135.2.301; PMid:6773331

Sallout B, Alshebli D, Sallout L et al. (2021). Fetal diaphragmatic eventration: A case report. J Obstet Gynaecol Can. 43(8): 993-997. https://doi.org/10.1016/j.jogc.2020.12.014; PMid:33412306

Sandstrom CK, Stern EJ. (2011). Diaphragmatic hernias: a spectrum of radiographic appearances. Curr Probl Diagn Radiol. 40(3): 95-115. https://doi.org/10.1067/j.cpradiol.2009.11.001; PMid:21440192

Shah-Mirany J, Schmitz GL, Watson RR. (1968). Eventration of the diaphragm. Physiologic and surgical significance. Arch Surg. 96(5): 844-850. https://doi.org/10.1001/archsurg.1968.01330230152024; PMid:5653003

Simansky DA, Paley M, Refaely Y, Yellin A. (2002). Diaphragm plication following phrenic nerve injury: a comparison of paediatric and adult patients. Thorax. 57(7): 613-616. https://doi.org/10.1136/thorax.57.7.613; PMid:12096205 PMCid:PMC1746380

Singh G, Rai RK, Pant N, Wakhlu A. (2020). Study of thoracoscopic repair of diaphragmatic eventration in children: A case series. J Laparoendosc Adv Surg Tech A. 30(6): 692-694. https://doi.org/10.1089/lap.2019.0805; PMid:32155102

Sodhi KS, Narsimhan KL, Bhattacharya A, Khandelwal N. (2011). Bilateral congenital diaphragmatic eventration: an unusual cause of respiratory distress in an infant. Afr J Paediatr Surg. 8(2): 259-260. https://doi.org/10.4103/0189-6725.86082; PMid:22005383

Soni A, Singh P, Singh RJ, Sood V. (2005). Eventration of diaphragm - Embryological basis. J Anat Soc India. 54(1): 39-41.

Steimer D, DeBarros M, Marshall MB, Jaklitsch M. (2022). Thoracoscopic radial diaphragm plication. Ann Thorac Surg. 114(3): e227-e230. https://doi.org/10.1016/j.athoracsur.2021.11.037; PMid:34951968

Symbas PN, Hatcher CR, Waldo W. (1977). Diaphragmatic eventration in infancy and childhood. Ann Thorac Surg. 24(2): 113-119. https://doi.org/10.1016/S0003-4975(10)63718-6; PMid:879894

Takahashi T, Okazaki T, Ochi T et al. (2013). Thoracoscopic plication for diaphragmatic eventration in a neonate. Ann Thorac Cardiovasc Surg. 19(3): 243-246. https://doi.org/10.5761/atcs.cr.12.01907; PMid:22971706

Tepe M, Inan I, Kafadar S. (2024). Bi-parametric MRI of the diaphragm using dynamic and static images: The initial experience. Cureus. 16(5): e61446. https://doi.org/10.7759/cureus.61446

Thomas TV. (1970). Congenital eventration of the diaphragm. Ann Thorac Surg. 10(2): 180-192. https://doi.org/10.1016/S0003-4975(10)65584-1; PMid:4913762

Tiryaki T, Livanelioglu Z, Atayurt H. (2006). Eventration of the diaphragm. Asian J Surg. 29(1): 8-10. https://doi.org/10.1016/S1015-9584(09)60285-2; PMid:16428090

Versteegh MIM, Braun J, Voigt PG et al. (2007). Diaphragm plication in adult patients with diaphragm paralysis leads to long-term improvement of pulmonary function and level of dyspnea. Eur J Cardiothorac Surg. 32(3):449-456. https://doi.org/10.1016/j.ejcts.2007.05.031; PMid:17658265

Vieira Martins M, Martins DS, Oliveira G. (2021). Diaphragmatic eventration as a first sign of Scimitar syndrome. Cardiol Young. 31(11): 1870-1872. https://doi.org/10.1017/S1047951121001694; PMid:33941297

Wayne ER, Campbell JB, Burrington JD, Davis WS. (1974). Eventration of the diaphragm. J Pediatr Surg. 9(5): 643-651. https://doi.org/10.1016/0022-3468(74)90101-8; PMid:4424891

Wei S, Lu R, Zhang Z et al. (2022). MRI-assessed diaphragmatic function can predict frequent acute exacerbation of COPD: a prospective observational study based on telehealth-based monitoring system. BMC Pulm Med. 22: 438. https://doi.org/10.1186/s12890-022-02254-x; PMid:36424599 PMCid:PMC9685983

Wu S, Zang N, Zhu J et al. (2015). Congenital diaphragmatic eventration in children: 12 years' experience with 177 cases in a single institution. J Pediatr Surg. 50(7): 1088-1092. https://doi.org/10.1016/j.jpedsurg.2014.09.055; PMid:25783408

Xu PP, Chang XP, Tang ST et al. (2020). Robot-assisted thoracoscopic plication for diaphragmatic eventration. J Pediatr Surg. 55(12): 2787-2790. https://doi.org/10.1016/j.jpedsurg.2020.06.034; PMid:32711940

Yazici M, Karaca I, Arikan A et al. (2003). Congenital eventration of the diaphragm in children: 25 years' experience in three pediatric surgery centers. Eur J Pediatr Surg. 13(5): 298-301. https://doi.org/10.1055/s-2003-43573; PMid:14618518

Yongue C, Geraci TC, Chang SH. (2024). Management of diaphragm paralysis and eventration. Thorac Surg Clin. 34(2): 179-187. https://doi.org/10.1016/j.thorsurg.2024.01.006; PMid:38705666

Zhao S, Pan Z, Li Y et al. (2020). Surgical treatment of 125 cases of congenital diaphragmatic eventration in a single institution. BMC Surg. 20(1): 270. https://doi.org/10.1186/s12893-020-00928-z; PMid:33148241 PMCid:PMC7640684

Zhen L, Gu CM, Huang LY, Li DZ. (2019). Congenital cystic diaphragm with diaphragmatic eventration in a fetus: a case presentation. Fetal Pediatr Pathol. 38(4): 335-339. https://doi.org/10.1080/15513815.2019.1588440; PMid:30912683

Published

2025-06-28