A Simplified Approach to the Repair of Pediatric Pectus Deformities

Transcription

1 A Simplified Approach to the Repair of Pediatric Pectus Deformities Conrad W. Wesselhoeft, Jr., M.D., and Frank G. DeLuca, M.D. ABSTRACT For the past 14 years, a simplified operation utilizing a metal strut for internal fixation has been used to repair pectus anomalies in 123 children. Subperichondrial cartilage resection is performed through small incisions in the pectoral muscles. No sternal osteotomy required. A malleable strut is passed transstemally and removed in four to six months, frequently under local anesthesia. All children who underwent this procedure were discharged within five to six days, and no transfusions were necessary. The use of the technique has shortened operative time and decreased the necessity for extensive postoperative pulmonary physiotherapy. In 75 children followed for over 5 years, cosmetic results have been excellent, and self-image has improved substantially. In only 1 child was there a recurrence impressive enough to warrant reoperation. To wait, therefore, for severe progression of the deformity, or the development of obvious individual concern over appearance, or until unequivocal symptoms occur, is to deprive the child of his best chance for an optimal result and to require a large operation. Mark Ravitch [l] There have been many variations proposed for the operative repair of pectus deformities since Sauerbruch s first patient was operated upon in 1913 [2]. The modern era of surgical management was initiated by Brown in 1939 [3]. The most popular technique in use at the present time derives from the papers of Lester in 1946 [4], and Ravitch in 1949 [5]. This procedure requires extensive bilateral resection of the de- From the Department of Surgery, Section of Pediatric Surgery, Rhode Island Hospital and Brown University Program in Medicine, Providence, RI. Presented at the Eighteenth Annual Meeting of The Society of Thoracic Surgeons, Jan 11-13, 1982, New Orleans, LA. Address reprint requests to Dr. Wesselhoeft, 110 Lockwood St, Providence, RI formed cartilages, release of the intercostal bundles from the sternum, division of the xiphoid, and sternal osteotomy. Over the past fourteen years, we have utilized a simplified approach, wherein a metal strut is inserted for internal fixation. Patients and Methods From 1966 through 1980, 123 patients underwent repair of pectus deformities. This group included 28 girls and 95 boys. Thirty-eight of the children were less than 2 years of age at the time of operation, ard 30 were over 6 years of age (Table 1). Althoc<,h several studies have now documented improvement in cardiac and pulmonary performance after pectus surgery [6-81, all operations in this series were undertaken primarily for cosmetic reasons. One patient had an associated mild pulmonic stenosis with ventricular septa1 defect, while another had a preoperative diagnosis of mitral valve prolapse. Five children had a history of recurrent pulmonary infections. One patient had an associated collagen disorder with a fairly marked scoliosis. In 5 children, there was a very asymmetric, deep concavity with sternal rotation to the right. Five children were seen with a pectus carinatum defect, while the remainder demonstrated the typical excavatum depression. Operative Technique A transverse submammary incision is made over the deepest area of the defect (Fig 1). The xiphoid and rectus muscles are divided from the sternum. Next, small incisions are made in the pectoral muscles covering the lowermost three or four rib cartilages, and these muscles are resected subperichondrially from the sternal edge laterally to the costochondral junction. No sternal wedge osteotomy is performed. A drill is inserted through the sternum just above the highest resected cartilage. A malleable metal by The Society of Thoracic Surgeons

2 641 Wesselhoeft and DeLuca: Repair of Pediatric Pectus Deformities Table 1. Age at Time of Operation for 123 Patients Undergoing Surgical Repair of Pectus Deformities Age (yr) >6 30 No. of Patients Fig 2. (A) Following subperichondrial removal of the costal cartilages, a drill is inserted transsternally in preparation for the malleable strut. (B) After the strut is inserted, the ends are bent with pliers to elevate the sternum. Fig I. Operative procedurefor repair of pectus deformity. A submammary skin incision is made over the deepest portion of the defect. strut* is then passed transsternally and placed laterally beneath the pectoral muscles (Fig 2A, B). The ends of the strut are positioned so that one end is easily palpable beneath the skin, thus facilitating later removal (Fig 3). The incisions in the pectorals are sutured with interrupted catgut, a plastic suction catheter is inserted subcutaneously above the pectoral muscles, and the skin is closed with a subcuticular suture. A collodion dressing is applied. No antibiotics are used, and no external supports or chest plates are necessary. The strut is removed in four to six months, frequently under local anesthesia. No special instruments are required for strut removal. Results In our 123 patients, there was no mortality and minimum morbidity (Table 2). No child required a blood transfusion either intraoperatively or postoperatively. All patients were dis- John Tuzik & co., 82 Chickatawbut St, Dorchester, MA Recti Fig 3. The strut is positioned beneath the pectoral muscles. One end is placed far laterally, facilitating removal in six months. charged within five to six days. Five children developed an intraoperative pneumothorax requiring only needle aspiration; another child developed postoperative atelectasis, which cleared within three days after operation. Four patients had to have small subcutaneous wound infections opened, while 2 required needle aspiration of a small collection of subcutaneous fluid. Incisional keloid formation was fairly prominent in 5 patients. In two of these children, it was believed to be minimal and to require no treatment; in 2 others, local steroid injections appeared to suffice. In the fifth child, scar excision was performed with a

3 642 The Annals of Thoracic Surgery Vol 34 No 6 December 1982 Table 2. Complications of Pectus Repair Complication Pneumothorax 5 Atelectasis 1 Wound infection 4 Subcutaneous fluid collection 2 Incisional keloid 5 No. of Patients good cosmetic result. Forty-eight children had their struts removed under local outpatient anesthesia, while 75 required general anesthesia and hospitalization for six to eight hours. Seventy-five of the 123 patients have now been followed for over 5 years postoperatively; of these, 70 have had excellent cosmetic results (Figs 4A, 4B), defined as restoration of normal anterior chest contour, flattening of the abdomen, and a well-healed cutaneous scar. In 4 patients, the results have been classified as Fig 4. (A) Frontal and (B) lateral views of a 16-year-old boy, 12 years after pectus repair. fair to good. In only 1 child did we feel a recurrence of the defect was impressive enough to warrant reoperation. Comment We would agree with Randolph and associates [9] that performing this operation in children between 18 months and 3 years of age is technically easier, less time-consuming, and less debilitating than in older children. Physiological studies from the National Institutes of Health have shown that heart function in pectus patients is compromised by abnormally low stroke volumes during upright exercise [71. In addition, Majid and colleagues [lo] have demonstrated a postoperative decrease in right atrial pressure with exercise. The indication for operation in all our patients, however, was primarily cosmetic. Operations for pectus deformities have generally fallen into three groups: (1) procedures requiring no special fixation [3, 41; (2) those in which external support is used postoperatively [ll]; and (3) those using internal fixation at the time of repair [12, 131. The limited operation proposed by Brown [3] has since been aban- A B

4 643 Wesselhoeft and DeLuca: Repair of Pediatric Pectus Deformities doned. This consisted of simple sectioning of the so-called substernal ligament, or xiphisternal disarticulation. The most radical operation proposed at present is that of Wada and colleagues in which there is a complete turnover of the lower sternum [141. The majority of procedures currently in use are variations on the operation described by Ravitch [5,15]. A rather extensive dissection of the sternum is required to isolate it from its pectoral, intercostal, xiphoid, and mediastinal attachments. Mobilization or division of the lower sternum may cause devascularization and possible degenerative changes, which may also be secondary to occlusion of the internal mammary artery during the dissection [161. Bone sutures are used to fix the cuneiform osteotomy. The operation is rather time-consuming, and we have seen several children who required extensive pulmonary physiotherapy postoperatively because of the marked paradoxical respiration produced by the sternal dissection. External traction on the elevated sternum in an attempt to stabilize the chest wall postoperatively has largely been supplanted by various forms of internal fixation [ Rib or costal cartilage grafts have been used extensively to provide sternal support [ However, such grafts frequently lack sufficient size and curvature for proper fixation, and are very difficult to remove if infection occurs. Fonkalsrud and associates [231 utilized autologous perichondrial sheaths, which are sutured together posterior to the sternum, while Robicsek [241 places a piece of Marlex mesh beneath the sternum, and sutures it to the ends of the divided costal cartilages. Other than mesh [25], the most widely favored type of internal fixation is some sort of pin or metal strut [26]. Mayo and Long [271, Grob [281, and Peters and Johnson [29] all use a Kirschner wire placed transsternally. Rehbein and Werniche [12] use three pairs of metal struts fixed to the lateral ribs; the sternum is sutured to these anterior metal blades. Adkins and Blades [131 and Jensen and colleagues [301 have described metal "stribs" passed beneath the sternum between the pleura and endothoracic fascia. Catgut sutures attach the metal pieces to the ribs and the sternum. The technique that we have modified was first described by Paltia and co-workers in 1959 [31]. We do not extensively free the pectoral muscles from the sternum and costal cartilages; instead, we rely on small individual incisions made through the muscle over the cartilages to be resected. Only the three or four lowest cartilages are removed bilaterally. There is no division of the rectus muscles from their costal insertions, and no transverse sternal osteotomy is performed. However, in the five patients in whom there was a deep and highly asymmetric concavity with a sternal rotation to the right, a sternal osteotomy was required, as well as the placement of two struts. The metal struts we use are available in various lengths. They are chemically inert, and may be bent manually to elevate the sternum to the appropriate level. Care is taken to ensure placement of the strut above the level of the highest resected cartilage. No sutures are needed to fix the strut, as it is adequately stabilized by resting on the ribs laterally beneath the pectoral musculature. Internal strut fixation has also been used in our pectus carinatum patients, who may also require an osteotomy or excision of bony protuberances. We have had no cases of deep infection or osteomyelitis requiring strut removal. Moreover, the need for postoperative pulmonary therapy has diminished markedly because the stable anterior chest wall reduces the possibility of paradoxical respiration. We have not seen rib pressure necrosis or extensive callus formation produced by the strut. Many of these struts were removed under simple local anesthesia on an outpatient basis. The majority of struts were left in place for six months, but 4 patients had to have them removed within two months because of local discomfort. Of the 75 patients followed for over five years, only 1 patient has had a recurrence severe enough to warrant a second operative procedure. Singh [32] has had similar results in 85 patients in whom the chest was stabilized with a stainless steel plate. References 1. Ravitch MM: Congenital deformities of the chest wall and their operative correction. Philadelphia, Saunders, 1977, p 200

5 644 The Annals of Thoracic Surgery Vol 34 No 6 December Sauerbruch EF: Die Chirurgie der Brustorgane. Berlin, Springer, 1927, pp Brown AL: Pectus excavatum (funnel chest). J Thorac Surg 9:164, Lester CW: The surgical treatment of funnel chest. Ann Surg 123:1003, Ravitch MM: The operative treatment of pectus excavatum. Ann Surg 129:429, BevegHrd S: Postural circulatory changes at rest and during exercise in patients with funnel chest with special reference to factors affecting the stroke volume. Acta Med Scand 171:695, Beiser GD, Epstein SE, Stampfer M, et al: Impairment of cardiac function in patients with pectus excavatum, with improvement after operative correction. N Engl J Med 287:267, Liese W, Buhlmann AA: Work capacity and lung volumes before and after surgical correction of pectus excavatum. Schweiz Med Wochenschr 104:83, Randolph JG, Tunnel1 WP, Morton D Jr: Repair of pectus excavatum in children under 3 years of age: a twelve-year experience. Ann Thorac Surg 23:364, Majid PA, Zienkocicz BS, Roos JP: Pectus excavatum and cardiac dysfunction: a case report with pre- and post-operative haemodynamic studies. Thorax 34:74, Ochsner A, De Bakey ME: Chone-chondrosternon: report of a case and review of the literature. J Thorac Surg 8:469, Rehbein F, Wernicke HH: The operative treatment of the funnel chest. Arch Dis Child 32:5, Adkins PC, Blades B: A stainless steel strut for correction of pectus excavatum. Surg Gynecol Obstet 113:111, Wada J, Ikeda I, Ishida T, Hasegawa T: Results of 271 funnel chest operations. Ann Thorac Surg 10:526, Haller JA, Shermeta DW, Tepas JJ, et al: Correction of pectus excavatum without prostheses or splints: objective measurement of severity and management of asymmetrical deformities. Ann Thorac Surg 26:73, Arnold M: The surgical anatomy of sternal blood supply. J Thorac Cardiovasc Surg 64:596, Therkelsen F: Funnel chest. Acta Chir Scand 102:36, Effler DB: Pectus excavatum: surgical treatment. Cleve Clin Q 20:353, Holmes CL: Pectus excavatum. J Thorac Surg 33:321, Dailey JE: Repair of funnel chest using substernal osteoperiosteal rib graft strut. JAMA 150: 1203, Adams HD: Surgical treatment of pectus excavatum by costosternoplasty with rib strut. Surg Clin North Am 40:603, Brantigan OC: Pectus excavatum: simple autogenous tissue support to keep sternum elevated. Am Surg 33:253, Fonkalsrud EW, Follette D, Saiwat AK: Pectus excavatum repair using autologous perichondium for sternal support. Arch Surg 113:1433, Robicsek F: Marlex mesh support for the correction of very severe and recurrent pectus excavatum. Ann Thorac Surg 26:80, May AM: Operation for pectus excavatum using stainless steel wire mesh. J Thorac Cardiovasc Surg 42:122, Griffin EH, Minnis JF: Pectus excavatum: a survey and a suggestion for maintenance of correction. J Thorac Surg 33:625, Mayo P, Long GA: Surgical repair of pectus excavatum by pin immobilization. J Thorac Cardiovasc Surg 44:53, Grob M: Chirurgische Erkrankungen des Thorax. In Lehrbruch der Kinderchirurgie. Stuttgart, Georg Thieme, 1957, p Peters RM, Johnson G Jr: Stabilization of pectus deformity with wire strut. J Thorac Cardiovasc Surg 47:814, Jensen NK, Schmidt WR, Garamella JJ, et al: Pectus excavatum and carinatum: the how, when, and why of surgical correction. J Ped Surg 5:4, Paltia V, Parkkulainen KV, Sulamma M, et al: Operative technique in funnel chest. Acta Chir Scand 116:90, Singh SV: Surgical correction of pectus excavatum and carinatum. Thorax 35:700, 1980 Discussion DR. FRANCIS ROBICSEK (Charlotte, NC): The repair of pectus deformities requires three basic steps: mobilization, correction, and fixation. The method presented here seems to fulfill only two of these basic criteria. First of all, pectus is not a disease of the sternum but of the cartilages. To state the opposite is like saying that the reason the giraffe has such a long neck is to reach its head, which happens to be located a considerable distance from its body. I agree with the authors that removal of just the lowermost three or four cartilages is sufficient, but only if no other cartilages are involved. If the disease is more extensive, the operation should be more extensive. The authors have abandoned the conventional transverse sternal osteotomy approved by God and Ravitch. Again, this may work very well in the very young and in those with only a mild deformity. Using the method they describe, however, I would find it difficult to achieve proper correction of the

6 645 Wesselhoeft and DeLuca: Repair of Pediatric Pectus Deformities sternum in young adults or in patients with severe deformity. The fixation of the sternum with steel struts is not only effective but very patriotic, because it supports our ailing steel industry. To call it, however, a "simplified approach" may be a slight overstatement. My associates and I have had uniformly good results without using steel supports, simply by placing a Marlex mesh support in the substernal position. Finally, two minor points. First, we do not regard entering the pleural cavity as a complication but as an integral part of our operation. With a chest tube inserted into the right pleural space connected to the substernal area, one can expect very effective wound drainage and smooth healing. In addition, we do detach the pectoralis muscles to obtain a better exposure; also, by uniting them in front of the sternum, we achieve a better repair. I would like to congratulate Drs. Wesselhoeft and DeLuca on their very nice presentation and excellent long-range follow-up. DR. MILTON v. DAVIS (Dallas, TX): I think that this is a good paper; Drs. Wesselhoeft and DeLuca are probably doing very good work. I would just like to comment on a couple of the points they have made. I generally agree with the authors' assessment of the optimum age for repair. The sternum is a cartilaginous structure at birth. It has bone islands that grow outward like the ripples that form when a pebble is dropped into a still pond. When these bone islands coalesce, we say that the sternum is ossified. This occurs at different times in different children but typically around the age of 20 months, or between 18 and 22 months. In my opinion, the optimum time for repair is any time after the sternum has become completely ossified-but preferably not too long after this happens, because better results are achieved if operation is performed in younger patients. The reason for not doing the repair in very young children is because one can't always be sure that they are going to need it until the sternum undergoes ossification. I also agree that putting the struts through the sternum probably keeps them from migrating. If any surgeon still puts struts under the sternum, I would strongly advise him or her to anchor the struts with metal sutures rather than catgut or prolene, because metal sutures will be required to keep them in place. I am a little distressed that the authors, who have obviously had a successful and enlightening experience with this condition, still emphasize cosmetic results. I don't mean to suggest that cosmetic results are not important; they are very important. However, there are also major physiological and cardiovascular concerns to be considered. The depressed ster- num presses on the anterior wall of the right ventricle and interferes with the filling of the right ventricle, thereby impeding the ability of the individual to reach his or her full capacity under exercise stress. This should be recognized and understood by thoracic surgeons and other physiologists. About 13 years ago at a meeting of the Southern Thoracic Surgical Association, H. H. Shah and I reported on our results with our first 15 cases of sternal turnover, a procedure recommended by Juro Wada of Sapporo, Japan. I am still using this method, and it has stood the test of time. I recently amended the turnover procedure by placing an intramedullary grooved Steinmann pin, which can be molded to give a nice anterior contour to the sternum. I am now using this technique in lieu of inserting struts. DR. HOWARD s. BROWN (Atlanta, GA): I would like to ask Drs. Wesselhoeft and DeLuca if they do anything in particular to handle the perichondrium. I have had situations where I have done a beautiful straightening of the sternum, performed an osteotomy, and taken out the deformed cartilages only to have the retracted perichondrium form new cartilage, with a poor long-term cosmetic result. Also, I would like to ask if they use the same procedure in older patients, say, young adults. DR. WOLFGANG BIRCKS (Duesseldorf, W Germany) We almost always consider the correction of pectus defects a cosmetic procedure only. Do the authors have any objective functional results? DR. WESSELHOEFT: We owe a great debt to Dr. Robicsek for his continuing studies in the care of pectus anomalies. I think it is important to point out, however, that we are using this repair only in young pediatric patients. I do agree that older patients may require a more extensive operation; in our older patients, particularly those in the mid-teenage group, we would probably choose the standard Ravitch procedure, although we would still use the transsternal strut. However, I am afraid that I cannot agree with the use of a thoracostomy tube, as we have found that a small subcutaneous plastic catheter, which does not invade the pleural space, works just as well. Dr. Davis, we agree that there should be no substernal placement of the struts due to the migration problems that you mentioned. I would say that our pectus repairs were done primarily for cosmetic reasons. I think a sternal turnover operation is much too involved a procedure to use in the group of patients we are dealing with.

7 646 The Annals of Thoracic Surgery Vol 34 No 6 December 1982 Dr. Brown, we do not handle the perichondrium in any special way; we just leave it there following removal of the costal cartilages. I think I have already answered your other question on surgery in older individuals, who do require a somewhat different procedure. Again, we still would use the strut. In answer to Dr. Bircks, we did not do any sophis- ticated functional studies on these patients. We did not have any cases of Marfan s syndrome in our group, but we did have one patient who had some form of collagen disease, and that patient did not do well, as one would expect. Conditions of this nature would probably warrant a more extensive operation than we have described. Notice from the American Board of Thoracic Surgery The Part I (written) examination will be held at the Amfac Hotel, DallaslFort Worth Airport, Dallas, TX, in January, The closing date for registration is August 1, To be admissible for the Part I1 (oral) examination, a candidate must have successfully completed the Part I (written) examination. A candidate applying for admission to the certifying examination must fulfill all the requirements for the Board at the time the application is received. Please address all communications to the American Board of Thoracic Surgery, E Seven Mile Rd, Detroit, MI