Indications for the surgical treatment of the funnel chest

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555 Indications for the surgical treatment of the funnel chest Clinic of Pediatric Surgery, Kaunas University of Medicine, Lithuania Key words: funnel chest, chest wall deformities. Summary. Aim of the study to establish the indications for surgical treatment of the funnel chest. Patients and methods. There was a retrospective and prospective analysis performed of the methods of investigation and treatment of 504 patients operated for the funnel chest during the period of 35 years (1968 2002). In order to make a right diagnosis and to establish the indications for surgical treatment a standard algorithm of investigations was prepared: examination, collecting the detailed life history, establishing a type and a degree of deformation, chest and column radiological examination, ECG, spirography and heart ultrasound examination. Results. On basis of physical examination and anamnestic data the most clinical signs and complaints were established. The heart compression was found in preoperative X-rays in 476 (94.4%) patients, the heart was rotated and dislocated in 441 (87.5%) patients. Postoperatively 96.1% of patients were free of the heart compression symptoms and for 71.8% the heart rotation has disappeared.the pathological ECG findings were recorded for 382 (71.8%) patients and abnormal spirograms were taken for 31 (8.5%) patients. Conclusions. The following indications for the funnel chest surgical treatment were established: obvious funnel chest diagnozed by physical examination, complaints and disturbances clear from anamnestic data, heart rotation and compression established by radiological examination, pathological findings in cardiorespiratory system (ECG, echocardiography, and spirography). An optimal age for operation was found out to be between 3 and 7 years. Introduction Chest wall deformities are not very common in children. E.Fonkalsrud (1, 2) claims that the chest wall deformity rate is 1 in 700 births, according to F.Robicsek it is 1:100. Assuming the material from different authors, we may suggest that the chest wall deformities rate might be from 0.2 to 2.3% (4-6). Such a variety of a statistical data depends not only on the contingent of the patients but also on the authors attitude at the pathology. The most common types among the chest wall deformities are a funnel chest (pectus excavatum) and a pigeon breast (pectus carinatum, arcuatum). Although the majority of the authors claim that the most common finding is a funnel chest, the rate of funnel and pigeon chest varies from 10:1 to 6:1 (1, 4-6). Our own investigations during the last decade make this statement doubtful. During the period of 35 years (1968-2002) we have operated on 853 patients for various types of the chest wall deformities, among them there were 504 (59.1%) with pectus excavatum, 342 (40.1%) with pectus carinatum or arcuatum and 7 (0.82%) affected by Poland s syndrome. The most complicated deformity causing the most pronounced complaints and disturbances of the cardiac and respiratory systems no doubt is a funnel chest. Therefore about 95% of works from different authors are directed to solve the problem of the pectus excavatum. Basing their knowledge on those investigations many authors have proven a negative impact of the funnel chest on the cardiorespiratory function, which was noted to recover after the operation. At the same time the new operating methods are being invented and the old ones are Correspondence to V. Barauskas, Clinic of Pediatric Surgery, Kaunas University of Medicine, Eivenių 2, 3007 Kaunas, Lithuania. E-mail: pedsurg@kmu.lt

556 being improved. During the last few decades the most popular was the procedure proposed by M.Ravitch (4) and from 1998 an overturn was performed by D.Nuss who offered a mini invasive technique. In spite of quite a successful treatment of the funnel chest there are still many surgeons, who disagree upon the indications for the surgical treatment (9-11). The aim of this study is to establish the relevant indications for the surgical treatment. Patients and methods We have examined 1112 patients with various chest wall deformities during the period of 35 years (1968 2002); 853 (76.7%) of them underwent the operation. There were 504 (59.1%) patients with pectus excavatum, 342 (40.1%) with pectus carinatum, arcuatum and 7 (0.82%) with Poland s syndrome. The object of present study is the group of 504 pectus excavatum operated patients. All these patients were examined using our united scheme of examination: 1. Physical examination. 2. Full patient s history (including the life history). 3. The establishment of the grade and type of funnel chest. 4. X-ray examination on the chest wall and spine. 5. Functional examination on cardiorespiratory system (ECG, echocardiography, spirography). 6. Analysis of early and long term postoperative results. These patients due to shape were classified (12, 13) into symmetric and asymmetric (with rotation of sternum and without) and described using 3 grades of deformities (Table 1). The grade of chest wall depression is established by using the method Fig. 1. Evaluation of sternum depression by I.Gizycka. offered by I.Gizycka (14). We take the lateral X ray picture of the chest and measure the shortest (a-b) and the longest (c-d) sternovertebral distance in cm (Fig.1). The relation (a-b): (c-d) shows the grade of deformity. If this relation is higher than 0.7 there is grade I deformity, if this relation various from 0.7 to 0.5 grade II, if it is lower than 0.5 grade III deformity must be diagnosed. Anamnestic data was collected very precisely using the special scheme, which consisted of 18 questions. All our 504 patients underwent X-ray (plain and lateral left picture) examination on the chest wall and spine and standard 12-lead electrocardiography Table 1. Classification of the funnel chest Grade of deformity Grade of deformity Asymmetric Symmetric Right Left with without with without rotation rotation rotation rotation Total I 97 7 3 107 II 291 23 6 320 III 67 7 3 77 Total 455 30 7 9 3 504

Indications for the surgical treatment of the funnel chest 557 (ECG) pre- and postoperatively. The postoperative examination was performed in about a month after the operation. Echocardiographic examination on Toshiba SSH-40 pre- and postoperatively was performed for 36 (7.1%) patients. Standard measurements of the left atrium, left ventricle, right ventricle, the aortic root, and the pulmonary artery were taken according to international standards. Contractility indices, such as fractional shortening (FS%), ejection fraction (EF%), and cardiac output (CO) were calculated using the Teichholz Formula. Spirography was used as a main method in order to investigate the respiratory function. Preoperatively it was performed on Pulma 1 for 413 (72%) patients. Pre-school children were not able to take part in it. We also haven t performed this examination in the early postoperative period, because at that time the patients used to feel certain discomfort and pains, so they couldn t perform spirography. For 52 (10.3%) patients the spirography was performed in a year after the operation and obtained data was compared with the preoperative. All subjective and objective data of examination were registered in the special questionnaire (128 questions). All the collected material underwent codification, processing by personal computer and statistical analysis Results It is quite easy to diagnose funnel chest by physical examination. The most common features of patient s appearance are presented in the Table 2. Anamnestic data is presented in the Table 3. Most of the children who had pectus excavatum also have had more serious complaints. The main complaints were shortness of breath, feeling fatigue, heartache after exercise, frequent respiratory tract infections and psychological problems. Our data shows that psychological problems appear in early childhood. X-ray examination was performed pre- and postoperatively for all 504 patients we carried study on. Pathological X-ray findings were presented in 94.4% patients preoperatively (Table 4). We can see typical heart displacement to the left and rotation in a clockwise direction in the plain X- ray picture and the heart compression and displacement towards the spinal column in the left lateral X-ray picture. Failure to thrive 412 81.7 Sharp epigastrial angle 244 48.4 Prominent ribs, Harrison groove 482 95.6 Protruding abdomen 425 84.3 Paradoxical sternum retraction 430 85.3 Wrong carriage 253 50.2 Table 2. Patient s appearance General weakness 31 6.1 Poorly eating 70 13.9 Increasing nervous 131 25.9 Heartache at rest 38 7.5 Heartache after exercise 78 15.4 Heartbeat 53 10.5 Feeling fatigue after exercise 442 87.7 Short of breath after exercise 434 86.1 Short of breath at rest 271 53.7 Psychological problems 323 64 Retarded physical development 441 87.5 Frequent respiratory tract infections 361 71.6 Sick child (parent s opinion) 452 89.6 Symptom Table 3. Anamnestic data Symptom Evaluating close-term postoperative X-ray pictures we could see that the symptoms of heart displacement and rotation have disappeared in 71.8%, there were no more compression signs in 96.1% cases. The mentioned symptoms were still present in 142 (28.2%) and 20 (3.9%) patients (Table 5). The sternovertebral distance is one of the most important parameters establishing the grade of chest wall deformity and also very important evaluating the efficiency of surgical procedure in close-term postoperative follow up. Preoperatively the shortest sternovertebral distance was 2.5 cm, the longest 11.2 cm, average 6.8 cm. Postoperatively the shortest distance was 5 cm, the longest 13 cm, average 8.5 cm. The biggest enlargement of

558 Table 4. Pathological X-ray findings preoperatively Heart compression 476 94.4 Heart displacement and rotation 441 87.5 Radiologic symptom Table 5. Pathological X-ray findings postoperatively Centimeters Rentgenologinis požymis Heart compression 20 3.9 Heart displacement and rotation 142 28.2 sternovertebral distance was 5 cm, the smallest 0.5 cm, the biggest sternum correction in percent was 100%, the smallest 5% (Fig.2). The pathological ECG changes were found in 382 (71.8%) our patients (Table 6). The most common finding was the overloaded right heart; the other findings were less common. We correlated pathological ECG findings with the data obtained by radiological examination and the correlation between them was statistically significant. The radiological findings of heart compression and displacement were usually (73.7+2.3 %, p<0.01) associated with pathological ECG findings. Postoperatively ECG findings were within normal limits in 330 (86.8%) cases; 52 (13.2%) patients still had some pathological ECG findings postoperatively, all they belonged to the age group over 10 years. Thirty six (7.1%) children with pectus excavatum underwent echocardiographic investigation. The standard measurements of the left atrium, left ventricle, right ventricle were close to normal for all 36 children and in one month after operation such parameters were the same, but contractility indices, such a fractional shortening (FS %), ejection fraction (EF%), and cardiac output (CO) have increased (Table 7) Fig. 2. Pectus excavatum changes of pre- and postoperative sternovertebral (SVD) distance The first line - biggest enlargement of SVD 100%; the second line smallest enlargement of SVD 5% Table 6. Preoperative pathological ECG changes Overloaded right heart 312 61.9 Incomplete right bundle branch 124 24.6 block Repolarization disturbances 72 14.2 Electrical axis deviation 68 13.3 Tachycardia 36 7.1 Bradycardia 14 2.8 Overloaded left heart 6 1.2 Changes In 52 (14.3 %) patients pulmonary function was compared at 1 year after operation (Table 8). The main aim of such an extended examination was to establish the indications for operation. As a matter of fact no one child with pectus excavatum underwent the operation just for cosmetic effect.

Indications for the surgical treatment of the funnel chest 559 All of them had some other complaints besides the psychological problems. Discussion The main aim of this study was to establish the diagnostic criteria and indications for surgical treatment of the funnel chest. I think that each patient deserves an individual approach and indications for surgical treatment should be applied rather to each separate patient than to a certain type of the deformity. Most of the authors (16-19) in their studies present heavy arguments proving that the funnel chest itself has a negative impact on the cardiorespiratory function, which has a tendency to improve after the operation. Performing this study I didn t aim to prove or deny any of those statements, but while analyzing quite a large group of the patients (504 individuals) who underwent surgical treatment I also noticed some regularity confirming the previous investigations results. Some authors (11) claim that the funnel chest itself is not causing any functional heart disturbance but due to the deformity the heart may change only its position and shape. Our study clearly disapproves this proposition. In case of the funnel chest, 71.8% of patients presented with pathological ECG findings. We compared the radiological and ECG changes and found a significant correlation between the parameters. It was proven that most of the patients got rid of the pathological symptoms postoperatively. We also took a note that the earlier an operation is performed, the less pathological findings are recorded in the postoperative period. This data was also confirmed by spirography, cardiac ultrasound and CT examinations. Therefore we disagree with M.Dahan (11) who looks at the problem of the funnel chest as if it is purely cosmetic. In the resent international literature there was very little discussion regarding the indications for surgical treatment and most of the authors (1, 9, 21, 22) focus their attention at Table 7. Pre- and postoperative echocardiography Indices Preoperative Postoperative Change (X) X ±S x X ±S x X ±S x p GSD (mm) 22.72 0.79 21.84 0.73 1.00 0.508 0.05 GDD (mm) 39.77 1.13 39.74 1.24 0.444 0.451 0.3 GST (ml) 17.81 1.27 16.28 1.13 1.73 0.862 0.05 GDT (ml) 67.31 4.07 67.83 4.01 0.515 1.54 0.7 ST (ml) 49.44 2.95 51.99 3.01 1.65 1.16 0.16 IF 0.739 0.01 0.764 0.008 0.022 0.01 0.02 FS 0.430 0.009 0.454 0.007 0.020 0.001 0.04 X middle; S x standard deviation; p probability. Table 8. Pulmonary function measurements before and 1 year after operation (n=52) Indices Preoperative Postoperative p VC L 4.16±1.3 4.63±1.2 <0.05 % predicted 81.6±4.2 86.31±6.6 <0.01 FEV 1 L 3.22±1.2 3.71±1.1 <0.02 % predicted 92.34±0.8 96.51±0.7 <0.05 FEV 1 /VC % 77.4±6.2 80.1±5.2 <0.02 % predicted 92.1±2.3 95.2±1.8 <0.01

560 functional cardiorespiratory disturbances and psychological problems for adolescents older than 12 years. Our study revealed that the psychological problems appear already among children of the early school age and the youngest patient who was very anxious about the shape of his chest was as young as 5 years of age. It is important to clear out that not a single child with a funnel chest was operated only for the cosmetic reasons, all of them had various complaints and the objective cardiorespiratory changes were recorded. The main indication for surgical treatment in our clinic was personal complaints of a functional and psychological origin, pathological radiological and ECG findings. Operation is indicated if at least one of the following symptoms is present or if there is a combination of the symptoms. On the contrary to other authors (18) our study shows that differing an operation till the end of puberty is not reasonable decision. The sur- gical treatment is indicated at preschool age. To our opinion the best age for the operative treatment is between 3 and 7 years while the surgery itself is easier and a patient is not so concerned about stay at the hospital. Conclusions 1. The following indications for the funnel chest surgical treatment were established: obvious chest wall deformity noted at the physical examination, complaints and disturbances clear from life history, cardiac compression and rotation confirmed by the chest X-rays, pathological findings in cardiac and respiratory systems (ECG, spirography). 2. An optimal age for the surgical treatment is between 3 and 7 years. Įdubusios krūtinės chirurginio gydymo indikacijos Kauno medicinos universiteto Vaikų chirurgijos klinika Raktažodžiai: įdubusi krūtinė, krūtinės ląstos deformacijos. Santrauka. Darbo tikslas. Nustatyti ir pagrįsti indikacijas įdubusios krūtinės chirurginiam gydymui. Ligoniai ir metodai. Atlikta retrospektyvioji ir prospektyvioji 504 ligonių, operuotų dėl įdubusios krūtinės ląstos per 35 metų laikotarpį (1968 2002 m.), tyrimo ir gydymo metodų analizė. Įdubusios krūtinės diagnostikai ir chirurginio gydymo indikacijoms nustatyti parengtas standartizuotas tyrimo planas: apžiūra, išsami ligos ir gyvenimo anamnezė, deformacijos rūšies bei laipsnio nustatymas; krūtinės ląstos ir stuburo rentgenografija, elektrokardiograma, spirografija, echokardioskopija. Rezultatai. Remiantis apžiūros ir anamnezės duomenimis, nustatyti būdingiausi įdubusios krūtinės klinikiniai požymiai, pacientų skundai, negalavimai. Krūtinės ląstos rentgenogramose, darytose prieš operaciją, 476 (94,4 proc.) tiriamiesiems nustatyta širdies kompresija, 441 (87,5 proc.) širdies dislokacija ir rotacija. Po operacijos 96,1 proc. pacientų išnyko širdies kompresija, 71,8 proc. rotacijos požymiai. Elektrokardiogramose patologinių pokyčių rasta 382 (71,8 proc.), spirogramose 31 (8,5 proc.) tiriamajam. Išvados. Nustatytos šios chirurginio gydymo indikacijos: apžiūros metu diagnozuotas akivaizdus krūtinės ląstos įdubimas; anamnestiškai aiškūs negalavimai, ligonio skundai; krūtinės ląstos rentgenogramose diagnozuota širdies kompresija, rotacija; rasta kardiorespiracinės sistemos patologinių pokyčių. Optimalus amžius daryti operaciją 3 7 metai. Adresas susirašinėjimui: V. Barauskas, KMU Vaikų chirurgijos klinika, Eivenių 2, 3007 Kaunas El. paštas: pedsurg @kmu.lt

Indications for the surgical treatment of the funnel chest 561 References 1. Fonkalsrud EW. Surgical management of chest wall malformations in childhood. Surg Childh Intern 1996;4:77-82. 2. Fonkalsrud EW, Dunn JCY, Atkinson JB. Repair of pectus excavatum deformities: 30 years of experience with 375 patients. Ann Surg 2000;231(3):443-8. 3. Robicsek F, Fokin A. Surgical correction of pectus excavatum and carinatum. J Cardiovasc Surg 1999;40:725-31. 4. Ravitch MM. Congenital deformities of the chest wall and their operative correction. Philadelphia, London, Toronto: WB Saunders Company; 1977. 5. Haller JA, Scherer LR, Turner CS, et al. Evolving management of pectus excavatum based on a single institutional experience of 664 patients. Ann Surg 1989;209:578-83. 6. Oelsnitz G. Die trichter und Kielbrust. Stuttgart; 1983. 7. Nuss D, Kelly RE, Croitoru DP, Katz ME. A 10-year review of a minimally invasive technique for the corresction of pectus excavatum. J Pediatr Surg 1998;33(4):545-52. 8. 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):437-45. 9. 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;61:1618-25. 10. Tjan TDT, Semik M, Rotering H, Rolf N, Scheld HH. Pectus excavatum: special surgical technique, perioperative management and long-term results. J Cardiovasc Surg 1999; 40:289-97. 11. Dahan M, Berjaud J, Vernhet JC. Technique operatoire du pectus excavatum methodes Radicales. Ann Chir Plast Esthet 1990;35:485-88. 12. Barauskas V. Krūtinės ląstos deformacijų diagnostika ir klasifikacija. (Diagnostics and clasification of chest wall deformities.) Medicina (Kaunas) 1998;34:437-45. 13. Barauskas V. Vaikų krūtinės ląstos deformacijos. (Chest wall deformities in children.) Kaunas; 2000. 14. Barauskas V, Urmonas V. Chest wall deformities in children: 29 years experience with 443 cases. Surg Childh Intern 1997; 5:21-6. 15. Wilekes CL, Backer CL, Mavroudis C. A 26-year review of pectus deformity repairs, including simultaneous intracardiac repair. Ann.Thorac Surg 1999;67:511-8. 16. Einsiedel E, Clausner A. Funnel chest. Psychological and psychosomatic aspects in children, youngsters, and young adults. J Cardiovasc Surg 1999;40:733-6. 17. Kowalewski J, Barcikowski S, Brocki M. Cardiorespiratory function before and after operation for pectus excavatum. Eur J Cardiothorac Surg 1998;13:275-9. 18. Haller JA, Loughlin GM. Cardiorespiratory function is significantly improved following corrective surgery for severe pectus excavatum. J Cardiovasc Surg 2000;41:125-30. 19. Xiao-ping J, Ting-ze H, Wen-ying L, Fu-kang W, Yu-ru Y, Jie-xiong F, et al. Pulmonary function for pectus excavatum at long-term follow-up. J Pediatr Surg 1999;13:1787-90. 20. Gotzen L, Dragojevic D. Funnel chest correction by use of AO implants. J Thorac Cardiovasc Surg 1979;5:4-13. 21. Clausner A, Clausner G, Basche M, et al. Importance of morphological findings in the progress and treatment of chest wall deformities with special reference to the value of computed tomography, echocardiography and stereophotogrammetry. Eur J Pediatr Surg 1991;2:291-7. 22. Lane-Smith DM, Gillis DA, Roy PD. Repair of pectus excavatumusing a dacron vascular graft strut. J Pediatr Surg 1994;29:1179-82. Received 1 April 2003, accepted 5 May 2003