SOC
Surgery on Children

Surgery on Children Journal aims to publish issues related to Pediatric Surgery, Pediatric Neurosurgery, Pediatric Plastic Surgery, Pediatric Cardiovascular Surgery, Pediatric Orthopedic Surgery, Pediatric Vascular Surgery, Pediatric Gynecology and Obstetrics, Pediatric Ear Nose Throat, Ophthalmology, Pediatric Anesthesiology and Reanimation, Pediatric Urology, Pediatric Surgical Intensive Care Clinic, and other clinical surgery fields on children of the highest scientific and clinical value at an international level and accepts articles on these topics.

eISSN: 3023-7289
Creative Commons CC BY-NC-ND 4.0 International License.
EndNote Style

Online Manuscript System

Index
Review
Chest wall deformities: diagnosis, classification, and treatment approaches
Abstract
Chest wall deformities encompass a range of structural abnormalities that may be congenital or acquired, impacting various aspects of the thoracic structure and function. This document provides an extensive review of primary chest wall deformities, including Pectus Excavatum (PE), Pectus Carinatum (PC), Poland Syndrome (PS), Jeune Syndrome (JS), and Sternal Cleft (SC). PE, the most prevalent deformity, is characterized by the depression of the anterior chest wall and is often linked to genetic syndromes such as Marfan and Noonan. Its clinical implications, such as exercise intolerance and cardiovascular effects, necessitate careful evaluation through physical examination, imaging, and pulmonary function tests. For severe cases, the Nuss and Ravitch surgical procedures offer corrective options, each with specific indications, benefits, and complications. PC is typified by the protrusion of the sternum and is generally considered a cosmetic issue. However, in severe cases, patients may experience functional impairments. Conservative treatment, including a dynamic compression system, is suitable for mild to moderate cases, while severe cases may require surgical intervention via the modified Ravitch or Abramson procedures. PS involves unilateral absence or hypoplasia of the pectoralis major muscle and may affect rib structures. Treatment focuses on both functional and cosmetic correction, with surgical options including custom implants and reconstructive techniques. JS, primarily congenital, leads to a restricted thoracic volume, posing a significant respiratory risk. Surgical expansion of the thorax is critical in severe cases, utilizing methods like rib grafting and vertical expandable titanium rib placement. SC, a rare condition with incomplete fusion of the sternum, requires early surgical intervention to prevent complications like respiratory distress and mediastinal shift. Across these conditions, individualized treatment plans are essential, balancing surgical benefits with risks, and considering each patient’s unique anatomical, functional, and psychosocial needs. The complexity of chest wall deformities necessitates multidisciplinary management to optimize outcomes and enhance quality of life for affected individuals.

Keywords : Pectus excavatum, pectus carinatum, poland syndrome, jeune syndrome, sternal cleft, thoracic surgery techniques

Download Article


1. Nuss D, Kelly RE. Chest wall deformities. In: Puri P, Höllwarth M, eds. Pediatric Surgery. Springer; 2009. doi:10.1007/978-3-540-69560-8_25
2. Shamberger RC, Welch KJ. Surgical repair of pectus excavatum. J Pediatr Surg. 1988;23(7):615-622. doi:10.1016/s0022-3468(88)80629-8
3. Croitoru DP, Kelly RE, Goretsky MJ, Lawson ML, Swoveland B, Nuss D. Experience and modification update for the minimally invasive Nuss technique for pectus excavatum repair in 303 patients. J Pediatr Surg. 2002;37(3):437-445. doi:10.1053/jpsu.2002.30851
4. Fokin AA, Steuerwald NM, Ahrens WA, Allen KE. Anatomical, histologic, and genetic characteristics of congenital chest wall deformities. Semin Thorac Cardiovasc Surg. 2009;21(1):44-57. doi:10. 1053/j.semtcvs.2009.03.001
5. Fonkalsrud EW. 912 open pectus excavatum repairs: changing trends, lessons learned: one surgeon’s experience. World J Surg. 2009;33(2):180-190. doi:10.1007/s00268-008-9793-4
6. Williams AM, Crabbe DCG. Pectus deformities of the anterior chest wall. Paediatr Respir Rev. 2003;4(3):237-242. doi:10.1016/s1526-0542(03) 00053-8
7. Obermeyer RJ, Goretsky MJ. Chest wall deformities in pediatric surgery. Surg Clin North Am. 2012;92(3):669-684. doi:10.1016/j.suc.2012.03.001
8. Park CH, Kim TH, Haam SJ, Lee S. Rib overgrowth may be a contributing factor for pectus excavatum: evaluation of prepubertal patients younger than 10 years old. J Pediatr Surg. 2015;50(11):1945-1948. doi:10.1016/j.jpedsurg.2015.07.010
9. Fokin AA, Steuerwald NM, Ahrens WA, Allen KE. Anatomical, histologic, and genetic characteristics of congenital chest wall deformities. In: Seminars in Thoracic and Cardiovascular Surgery. Vol 21. Elsevier; 2009. https://www.sciencedirect.com/science/article/pii/S1043067909000318
10. Leung AK, Hoo JJ. Familial congenital funnel chest. Am J Med Genet. 1987;26(4):887-890. doi:10.1002/ajmg.1320260416
11. Creswick HA, Stacey MW, Kelly RE, et al. Family study of the inheritance of pectus excavatum. J Pediatr Surg. 2006;41(10):1699-1703. doi:10.1016/j.jpedsurg.2006.05.071
12. Behr CA, Denning NL, Kallis MP, et al. The incidence of Marfan syndrome and cardiac anomalies in patients presenting with pectus deformities. J Pediatr Surg. 2019;54(9):1926-1928. doi:10.1016/j.jpedsurg. 2018.11.017
13. Billar RJ, Manoubi W, Kant SG, Wijnen RMH, Demirdas S, Schnater JM. Association between pectus excavatum and congenital genetic disorders: a systematic review and practical guide for the treating physician. J Pediatr Surg. 2021;56(12):2239-2252. doi:10.1016/j.jpedsurg.2021.04.016
14. Saxena AK, Willital GH. Valuable lessons from two decades of pectus repair with the Willital-Hegemann procedure. J Thorac Cardiovasc Surg. 2007;134(4):871-876. doi:10.1016/j.jtcvs.2007.06.008
15. Ewert F, Syed J, Kern S, Besendörfer M, Carbon RT, Schulz-Drost S. Symptoms in pectus deformities: a scoring system for subjective physical complaints. Thorac Cardiovasc Surg. 2017;65(1):43-49. doi:10.1055/s- 0036-1584355
16. Haller JA, Kramer SS, Lietman SA. Use of CT scans in selection of patients for pectus excavatum surgery: a preliminary report. J Pediatr Surg. 1987;22(10):904-906. doi:10.1016/s0022-3468(87)80585-7
17. Malek MH, Fonkalsrud EW, Cooper CB. Ventilatory and cardiovascular responses to exercise in patients with pectus excavatum. CHEST. 2003; 124(3):870-882. doi:10.1378/chest.124.3.870
18. St. Peter SD, Juang D, Garey CL, et al. A novel measure for pectus excavatum: the correction index. J Pediatr Surg. 2011;46(12):2270-2273. doi:10.1016/j.jpedsurg.2011.09.009
19. Bay V, Farthmann E, Naegele U. Unoperated funnel chest in middle and advances age: evaluation of indications for operation. J Pediatr Surg. 1970;5(6):606-609. doi:10.1016/s0022-3468(70)80004-5
20. Redding GJ, Kuo W, Swanson JO, et al. Upper thoracic shape in children with pectus excavatum: impact on lung function. Pediatr Pulmonol. 2013;48(8):817-823. doi:10.1002/ppul.22660
21. Malek MH, Berger DE, Marelich WD, Coburn JW, Beck TW, Housh TJ. Pulmonary function following surgical repair of pectus excavatum: a meta-analysis. Eur J Cardio-Thorac Surg Off J Eur Assoc Cardio-Thorac Surg. 2006;30(4):637-643. doi:10.1016/j.ejcts.2006.07.004
22. Nuss D, Obermeyer RJ, Kelly RE. Pectus excavatum from a pediatric surgeon’s perspective. Ann Cardiothorac Surg. 2016;5(5):493-500. doi:10. 21037/acs.2016.06.04
23. Nuss D, Kelly RE. Indications and technique of nuss procedure for pectus excavatum. Thorac Surg Clin. 2010;20(4):583-597. doi:10.1016/j.thorsurg.2010.07.002
24. Nuss D, Obermeyer RJ, Kelly RE. Nuss bar procedure: past, present and future. Ann Cardiothorac Surg. 2016;5(5):422-433. doi:10.21037/acs.2016. 08.05
25. Haecker FM, Sesia S. Vacuum bell therapy. Ann Cardiothorac Surg. 2016; 5(5):440-449. doi:10.21037/acs.2016.06.06
26. Haecker FM, Sesia S. Non-surgical treatment of pectus excavatum. J Vis Surg. 2016;2(3):63. doi:10.21037/jovs.2016.03.14
27. Obermeyer RJ, Cohen NS, Kelly RE, et al. Nonoperative management of pectus excavatum with vacuum bell therapy: a single center study. J Pediatr Surg. 2018;53(6):1221-1225. doi:10.1016/j.jpedsurg.2018.02.088
28. Kelly RE. Pectus excavatum: historical background, clinical picture, preoperative evaluation and criteria for operation. Semin Pediatr Surg. 2008;17(3):181-193. doi:10.1053/j.sempedsurg.2008.03.002
29. Papic JC, Finnell SME, Howenstein AM, Breckler F, Leys CM. Postoperative opioid analgesic use after Nuss versus Ravitch pectus excavatum repair. J Pediatr Surg. 2014;49(6):919-923. doi:10.1016/j.jpedsurg.2014.01.025
30. Haller JA, Colombani PM, Humphries CT, Azizkhan RG, Loughlin GM. Chest wall constriction after too extensive and too early operations for pectus excavatum. Ann Thorac Surg. 1996;61(6):1618-1624. doi:10.1016/ 0003-4975(96)00179-8
31. Antonoff MB, Erickson AE, Hess DJ, Acton RD, Saltzman DA. When patients choose: comparison of Nuss, Ravitch, and Leonard procedures for primary repair of pectus excavatum. J Pediatr Surg. 2009;44(6):1113-1119. doi:10.1016/j.jpedsurg.2009.02.017
32. Hebra A, Kelly RE, Ferro MM, Yüksel M, Campos JRM, Nuss D. Life-threatening complications and mortality of minimally invasive pectus surgery. J Pediatr Surg. 2018;53(4):728-732. doi:10.1016/j.jpedsurg.2017. 07.020
33. Park HJ, Lee SY, Lee CS, Youm W, Lee KR. The Nuss procedure for pectus excavatum: evolution of techniques and early results on 322 patients. Ann Thorac Surg. 2004;77(1):289-295. doi:10.1016/S0003-4975 (03)01330-4
34. Cheng YL, Lin CT, Wang HB, Chang H. Pleural effusion complicating after Nuss procedure for pectus excavatum. Ann Thorac Cardiovasc Surg Off J Assoc Thorac Cardiovasc Surg Asia. 2014;20(1):6-11. doi:10.5761/atcs.oa.12.02067
35. Park HJ, Lee SY, Lee CS. Complications associated with the nuss procedure: analysis of risk factors and suggested measures for prevention of complications. J Pediatr Surg. 2004;39(3):391-395. doi:10.1016/j.jpedsurg.2003.11.012
36. Moss RL, Albanese CT, Reynolds M. Major complications after minimally invasive repair of pectus excavatum: case reports. J Pediatr Surg. 2001;36(1):155-158. doi:10.1053/jpsu.2001.20039
37. Cw L. Pigeon breast (pectus carinatum) and other protrusion deformities of the chest of developmental origin. Ann Surg. 1953;137(4):482-489. doi: 10.1097/00000658-195304000-00008
38. Park CH, Kim TH, Haam SJ, Jeon I, Lee S. The etiology of pectus carinatum involves overgrowth of costal cartilage and undergrowth of ribs. J Pediatr Surg. 2014;49(8):1252-1258. doi:10.1016/j.jpedsurg.2014. 02.044
39. Haje SA, Harcke HT, Bowen JR. Growth disturbance of the sternum and pectus deformities: imaging studies and clinical correlation. Pediatr Radiol. 1999;29(5):334-341. doi:10.1007/s002470050602
40. Kotzot D, Schwabegger AH. Etiology of chest wall deformities—a genetic review for the treating physician. J Pediatr Surg. 2009;44(10):2004-2011. doi:10.1016/j.jpedsurg.2009.07.029
41. Fonkalsrud EW. Surgical correction of pectus carinatum: lessons learned from 260 patients. J Pediatr Surg. 2008;43(7):1235-1243. doi:10. 1016/j.jpedsurg.2008.02.007
42. Gritsiuta AI, Bracken A, Beebe K, Pechetov AA. Currarino-Silverman syndrome: diagnosis and treatment of rare chest wall deformity, a case series. J Thorac Dis. 2021;13(5):2968. doi:10.21037/jtd-20-3472
43. Fonkalsrud EW, Beanes S. Surgical management of pectus carinatom: 30 years’ experience. World J Surg. 2001;25(7):898-903. doi:10.1007/s00268-001-0048-x
44. Shamberger RC, Welch KJ. Surgical correction of pectus carinatum. J Pediatr Surg. 1987;22(1):48-53. doi:10.1016/s0022-3468(87)80014-3
45. Port E, Hunter CJ, Buonpane C, et al. Echocardiography reveals heart abnormalities in pediatric pectus carinatum. J Surg Res. 2020;256:364-367. doi:10.1016/j.jss.2020.06.061
46. Kravarusic D, Dicken BJ, Dewar R, et al. The Calgary protocol for bracing of pectus carinatum: a preliminary report. J Pediatr Surg. 2006; 41(5):923-926. doi:10.1016/j.jpedsurg.2006.01.058
47. Martinez-Ferro M, Fraire C, Bernard S. Dynamic compression system for the correction of pectus carinatum. Semin Pediatr Surg. 2008;17(3): 194-200. doi:10.1053/j.sempedsurg.2008.03.008
48. Abdellaoui S, Scalabre A, Piolat C, et al. Pectus arcuatum: a pectus unlike any other. J Pediatr Surg. 2023;58(9):1679-1685. doi:10.1016/j.jpedsurg.2023.03.013
49. Stephenson JT, Bois JD. Compressive orthotic bracing in the treatment of pectus carinatum: the use of radiographic markers to predict success. J Pediatr Surg. 2008;43(10):1776-1780. doi:10.1016/j.jpedsurg.2008.03.049
50. Robicsek F, Watts LT, Fokin AA. Surgical repair of pectus excavatum and carinatum. Semin Thorac Cardiovasc Surg. 2009;21(1):64-75. doi:10. 1053/j.semtcvs.2009.03.002
51. Abramson H, D’Agostino J, Wuscovi S. A 5-year experience with a minimally invasive technique for pectus carinatum repair. J Pediatr Surg. 2009;44(1):118-123. doi:10.1016/j.jpedsurg.2008.10.020
52. Yiyit N, Işıtmangil T, Öksüz S. Clinical analysis of 113 patients with Poland syndrome. Ann Thorac Surg. 2015;99(3):999-1004. doi:10.1016/j.athoracsur.2014.10.036
53. Fokin AA. Thoracic defects: cleft sternum and Poland syndrome. Thorac Surg Clin. 2010;20(4):575-582. doi:10.1016/j.thorsurg.2010.06.001
54. Bavinck JNB, Weaver DD, Opitz JM, Reynolds JF. Subclavian artery supply disruption sequence: hypothesis of a vascular etiology for Poland, Klippel-Feil, and Möbius anomalies. Am J Med Genet. 1986;23(4):903-918. doi:10.1002/ajmg.1320230405
55. Romanini MV, Calevo MG, Puliti A, et al. Poland syndrome: a proposed classification system and perspectives on diagnosis and treatment. Semin Pediatr Surg. 2018;27(3):189-199. doi:10.1053/j.sempedsurg.2018.05.007
56. Romanini MV, Torre M, Santi P, et al. Proposal of the TBN classification of thoracic anomalies and treatment algorithm for Poland syndrome. Plast Reconstr Surg. 2016;138(1):50. doi:10.1097/PRS.0000000000002256
57. Fokin AA, Robicsek F. Poland’s syndrome revisited. Ann Thorac Surg. 2002;74(6):2218-2225. doi:10.1016/S0003-4975(02)04161-9
58. Cochran JHJ, Pauly TJ, Edstrom LE, Dibbell DG. Hypoplasia of the latissimus dorsi muscle complicating breast reconstruction in Poland’s syndrome. Ann Plast Surg. 1981;6(5):402. doi:10.1097/00000637-198105000-00010
59. Baldelli I, Santi P, Dova L, et al. Body image disorders and surgical timing in patients affected by Poland syndrome: data analysis of 58 case studies. Plast Reconstr Surg. 2016;137(4):1273. doi:10.1097/PRS. 0000000000002018
60. Torre M, Palo F, Infant M. Open-surgery repair of congenital malformation of the chest: indications, technical tips and outcomes. Pediatr Med. 2019;2:48. doi:10.21037/pm.2019.08.04
61. Keppler-Noreuil KM, Adam MP, Welch J, Muilenburg A, Willing MC. Clinical insights gained from eight new cases and review of reported cases with Jeune syndrome (asphyxiating thoracic dystrophy). Am J Med Genet A. 2011;155A(5):1021-1032. doi:10.1002/ajmg.a.33892
62. de Vries J, Yntema JL, van Die CE, Crama N, Cornelissen EA, Hamel BCJ. Jeune syndrome: description of 13 cases and a proposal for follow-up protocol. Eur J Pediatr. 2010;169(1):77-88. doi:10.1007/s00431-009-0991-3
63. Phillips JD, van Aalst JA. Jeune’s syndrome (asphyxiating thoracic dystrophy): congenital and acquired. Semin Pediatr Surg. 2008;17(3):167-172. doi:10.1053/j.sempedsurg.2008.03.006
64. Conroy E, Eustace N, McCormack D. Sternoplasty and rib distraction in neonatal Jeune syndrome. J Pediatr Orthop. 2010;30(6):527-530. doi:10. 1097/BPO.0b013e3181e03a08
65. Davis JT, Ruberg RL, Leppink DM, McCoy KS, Wright CC. Lateral thoracic expansion for Jeune’s asphyxiating dystrophy: a new approach. Ann Thorac Surg. 1995;60(3):694-696. doi:10.1016/0003-4975(95)92703-O
66. Muthialu N, Mussa S, Owens CM, Bulstrode N, Elliott MJ. One-stage sequential bilateral thoracic expansion for asphyxiating thoracic dystrophy (Jeune syndrome). Eur J Cardio-Thorac Surg Off J Eur Assoc Cardio-Thorac Surg. 2014;46(4):643-647. doi:10.1093/ejcts/ezu074
67. Waldhausen JHT, Redding GJ, Song KM. Vertical expandable prosthetic titanium rib for thoracic insufficiency syndrome: a new method to treat an old problem. J Pediatr Surg. 2007;42(1):76-80. doi:10.1016/j.jpedsurg. 2006.09.059
68. Ashok Raja J,Mathevan G,Mathiarasan K,Ramasubramaniam P. Closing the cleft over a throbbing heart: neonatal sternal cleft. BMJ Case Rep. 2014;2014:bcr2014204529. doi:10.1136/bcr-2014-204529
69. Williams AP, Marayati R, Beierle EA. Pentalogy of cantrell. Semin Pediatr Surg. 2019;28(2):106-110. doi:10.1053/j.sempedsurg.2019.04.006
70. Shamberger RC, Welch KJ. Sternal defects. Pediatr Surg Int. 1990;5(3): 156-164. doi:10.1007/BF00179653
71. Fokin AA. Cleft sternum and sternal foramen. Chest Surg Clin N Am. 2000;10(2):261-276. doi:10.1016/S1052-3359(25)00495-8
72. Torre M, Rapuzzi G, Guida E, Costanzo S, Jasonni V. Thymectomy to achieve primary closure of total sternal cleft. J Pediatr Surg. 2008;43(12): e17-e20. doi:10.1016/j.jpedsurg.2008.09.011
73. Sabiston DC. The surgical management of congenital bifid sternum with partial ectopia cordis. J Thorac Surg. 1958;35(1):118-122. doi:10.1016/S0096-5588(20)30288-9
74. Biswas G, Khandelwal NK, Venkatramu NK, Chari PS. Congenital sternal cleft. Br J Plast Surg. 2001;54(3):259-261. doi:10.1054/bjps.2000. 3527
75. Laamiri R, Kechiche N, Hmidi N, et al. Isolated central sterna clefts: a rare congenital malformation. Afr J Paediatr Surg AJPS. 2021;18(2):117-118. doi:10.4103/ajps.AJPS_47_20
Article available in :
Volume 2, Issue 2, 2025
Page : 72-78
https://doi.org/10.51271/SOC-0039
Article Information
Received : 19 Kas 2024
Accepted : 18 Oca 2025
Published : 10 Nis 2025
Corresponding Author :

Cihan Genco Çetinkanat: Thoracic Surgery, Citrusmed Ltd. Co., Ankara, Turkiye

[email protected]
Citation : Uğurum Yücemen A, Yenigün BM, Çetinkanat CG. Chest wall deformities: diagnosis, classification, and treatment approaches. Surg Child. 2025;2(2):72-78.

Read: 739
Download : 994
Cited: 0
_Footer