Operative Correction of Pectus Excavatum By George H. Humphreys, II, and Alfred Jaretzki, III H E D E F O R M I T Y now most frequently called "pectus excavatum" has been noted for centuries but was not described clearly until 1860. It is an u n c o m m o n anomaly, at first reported in isolated examples. By 1938 Ochsner and DeBakey I were able to collect records of only 32 corrective procedures, including their own. N o one surgeon had reported more than five cases, and the methods used were o f great variety. The next year Brown 2 presented his theory o f the anatomic cause due to shortened substernal ligament and described a method of surgical correction. This method depended on sternal mobilization by perichondrial resection, division of xiphosternal junction, wedge osteotomy of the sternum, and external traction support. Although his theory of cause remains as questionable as numerous other theories, his principle o f sternal mobilization has been generally accepted as the basis for corrective procedures. Sweet in 1944, 3 Lester in 1946, 4 and Ravitch in 19495 all modified and developed this principle. Further m e t h o d s continued to be reported. Lester in 19546 and Ravitch in 19657 published modifications of their earlier methods. Dailey 8 advocated a rib strut, which was used also in some cases by Lester and by Adkins and Gwathmey. 9 Later Adkins and Blades ~~ substituted a steel strut; Griffin and Minnis n used a removable steel pin, and this method was further used by M a y o and L o n g ; " Rehbein and Wernicke ~ originally used long crisscrossed metal blades; more recently Rehbein ~4 has used multiple steel struts. Willital, ~5 also working in Germany, uses a single steel strut, and a similar technique, but passing the strut through the sternum, has been used by Paltia 16 in Helsinki. Wire mesh has been used successfully in at least one case, reported by May. t7 The use of internal struts is advocated in order to avoid external traction. The latter, however, continues to be relied upon by Morris ~s as the principle corrective method, and is used by many other surgeons as an adjunct to internal correction, or to prevent paradoxical motion after extensive chest wall mobilization. Recently "sterno-turnover" has been advocated, in selected cases, by Wada et al.~9 as well as an original operation on costal cartilages alone, which they term "costoplasty." The fact that new methods of surgical correction continue to be advocated is an indication that no one procedure has gained universal acceptance. Even the indications for operation are not clear. Probably this uncertainty is related to the relative infrequency of the anomaly, its variation in severity in different individuals and at different ages, and the paucity of comparable long-term studies of results. Continuing experience, however, should make it possible to resolve
From the Department of Surgery, College of Physicians and Surgeons, Columbia University, New York. Address for reprint requests: George H. Humphreys, H, Department of Surgery. College of Physicians and Surgeons, Columbia University, 630 West 168th Street, New York, N. Y. 10032. 9 1974 by Grune & Stratton, lnc. Journal of Pediatric Surgery, Vol. 9, No. 6 (December), 1974
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some of these uncertainties and justifies description of a method which has now been used for more than 20 yr. PRINCIPLES OF REPAIR
A description of an earlier version of this method, based on Brown's principle, was published in 1960. 20 It has since been somewhat modified. It differs from Lester's radical operation 4 and Ravitch's 7 m e t h o d in that only small segments of cartilage are resected, and a forward-arched, semirigid thoracic cage is immediately restored. External support is rarely used in children under the age of 8, and when it is used in patients over that age, it is only to prevent paradoxical motion, not to achieve correction. Internal struts are never used. Adequate correction depends on complete mobilization of the sternum. In other radical procedures this is achieved by dividing the perichondrium and intercostal tissues, after resecting all of the cartilages, close to the sternum. This isolates it from its internal m a m m a r y blood supply and results in abnormal chest wall mobility in the early postoperative period. In the method described here, the sternum, adjacent anterior costal cartilages, and intercostal tissues DEFECT
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Fig. 1. Diagrams to show principles of repair. (A) Diagrammatic sagittal views. Left: Before correction: the anterior leaf of the diaphragm is short, the sternum angulated back at the manubriogladielar junction, and the xyphoid angled sharply forward. Right: After correction: the diaphragm is lengthened anteriorly, then attached to the joined rectus muscles in the sagittal plane and to the tip of the sternum from which the xyphoid has been detached and removed. The sternal angle is currected by wedge osteotomy at the
text, Step 6). (B) Diagrammatic anteroposterior views of the thoracic cage. Left: Before correction: showing segments to be excised or incised. Right: After correction: showing new cephalad position of the gladiolus in relation to the rib ends, widened costal angle, and "floating" lower ~ ~ ~ costalarch. (C) Diagrammaticsection of thorax at point of greatest depression (viewed from below). Left: Before correction: the sterum is tilted and heart displaced to the left of the spine. The fight costochondral junction is more angulated than the left. (Chondrectomies and chendrotemies are indicated.) Middle: After multiple chondrectomies, transverse sternotomy, and elevation of the sternum. (See text, Step 7). Right: after appropriate chondrotomies and resuture of cut ends of costal cartilages to the chest wall. An anterior arch is created and the heart tends to return to more nearly normal position. (See text, Step 7).
CORRECTION OF PECTUS EXCAVATUM
are elevated "en bloc." To do this it is necessary to enter the right pleural cavity deliberately, and usually also the left, to divide the superior epigastric vessels as they emerge under the costal margin to enter the rectus muscles, and to separate the attachment of the diaphragm not only from the sternum, but also from the costal arch as far laterally as the deformity extends. In this, the procedure resembles that described by Daniel 2~ except that he resected the cartilages completely. Wedge resection of the angulated sternum under a flap of elevated periosteum usually secures good correction without other support, and secondary division of sternal curvature as described in 19602o is rarely necessary. The extensive exposure of the anterior origin of the diaphragm permits lengthening of the central tendon when indicated and stiture of the transversus abdominis muscle to it, correcting "pot-belly" and creating, after repair of the rectus origins, a pull downward (toward the feet) on the costal margin and lower sternum rather than backward (toward the spine). At the same time, any diastasis of the rectus muscles or umbilical hernia is corrected (Fig. 1). OPERATIVE TECHNIQUE
After intratracheal anesthesia is established, the patient is placed supine with both arms at right angles to the body, supported on arm boards. A folded towel or thin sandbag is placed under the thoracic spine, so that the arms are in slight adduction and the neck extended. A strap is placed across the thighs and, with older patients, a footrest under the feet, to prevent the patient from sliding down on the table. The table is then tilted upward to bring the manubrium into a horizontal position (Fig. 2D). lncision
In most patients, especially in males, exposure is best obtained by a long midline incision, extending from the angle of Ludwig, at the level of the second chondrosternal junction, to a point slightly superior to the umbilicus. If an umbilical hernia is present, the incision should extend to the umbilicus. In females, in order to avoid an upper thoracic scar, a transverse incision may be used, running in the inframammary fold from the midclavicular line on each side to a short transverse incision over the junction of the gladiolus to the xiphoid. The incision is deepened through the subcutaneous tissue to the periosteum of the sternum superiorly and to the linea alba inferiorly. In a transverse incision, it is deepened to the fascia over the origins of the rectus abdominis muscles laterally on each side as far as the costochondral junction, or to the point of greatest backward angulation. Skin and subcutaneous tissue flaps are then dissected bilaterally (or superiorly and inferiorly in the case of a transverse incision) to expose a diamondshaped field of pectoralis major fascia superiorly and anterior rectus sheaths inferiorly from the level of the angle of Ludwig to just above the umbilicus. If an umbilical hernia is present, the skin of the umbilicus is dissected off the hernial protrusion, taking care to avoid opening the peritoneal cavity.
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Fig. 2. Exposure of thoracic cage. (D) Position of patient and incision. (E) Stage 1. Elevation and retraction of skin and subcutaneous tissue foll o w e d by elevation of anterior rectus sheaths and muscles. The linea a l b a is wide and thin, bulging through a diastasis of the rectus muscles. (Insert: Diagram to show elevation of rectus sheath and muscle.) (F) Stage 2. Elevation and retraction of the pectaralis major muscles from the anterior thoracic cage.
Exposure of Thoracic Cage Incisions are made in the anterior rectus sheaths along the medial edges of the rectus abdominis muscles on each side, from the xiphoid to just above the umbilicus (or to the midline defect if an umbilical hernia is present). Using a stay mattress suture of heavy silk held in a hemostat, placed through the rectus sheath at its attachment to the xiphoid, the rectus sheaths and muscles are then dissected transversely away from the anterior thoracic wall superiorly and from posterior rectus sheaths inferiorly. As the common costal cartilage is exposed, the internal mammary-superior epigastric vessels emerge from beneath the costal margin to enter the muscle. These vessels are divided between clamps and ligated to permit the muscles to be fully retracted as far as the inferior angle of the cartilage on each side (Fig. 2E). The plane between pectoralis major muscle and anterior thoracic wall is now developed by defining the inferior borders of the muscles on each side, lifting they away from the underlying ribs and cartilages and dividing their origins from the costal cartilages and sternum, from below upward, ligating perforating branches of the internal mammary artery as they are encountered. In this manner the costal cartilages and costochondral junctions are exposed on each side up to the second costal cartilage (Fig. 2F).
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Correction of Deformity Having thus exposed the entire deformity, including the diastasis of the rectus muscles and umbilical hernia if either are present, definitive correction is begun. Details of procedure will vary with the extent and form of the deformity, guided by the principles of sternal release and mobilization, followed by chest wall reconstruction, diaphragmatic reconstruction, and replacement of rectus and pectoral muscles. Step 1. Short segments of the third, fourth, and fifth costal cartilages at the point of greatest angulation (just medial to their costochondral junctions) are first resected subperichondrially, usually starting on the right where the angulation is greatest (Fig. 3G). Step 2. The xiphoid cartilage is resected, and the anterior mediastinum entered by passing a finger upward behind the sternum. The heart is often displaced to the left, and the distance between the posterior aspect to the most depressed point of the gladiolus and the anterior surface of the vertebral bodies of the spine is noted. If this is less than 3 cm, it will probably be necessary to lengthen the central tendon of the diaphragm (see Step 9) (Fig. 3H and I). Step 3. The right pleural cavity is next entered through the pleural reflec-
Fig. 3. Correction of deformity, I. (G) Step i. Subperichondrial resections. (H) Step 2-a. Excision of xyphoid. II) Step 2-b. Entry into anterier mediastinum.
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Fig. 4. Correction of deformity, Ih (J) Step 3. Separation of transversus abdomini~ muscle and diaphragm from the costal margin, entering the pleural cavity. (The illustration indicates entrance on the left first, although the reverse is usually the case.) (K) Step 4. Separation of sternum and cartilages from lateral chest wall by incision into the pleural cavity through perichondrium (after multiple chondrectomies, Step 1), intercostal muscle bundles and common costal cartilage.
tion onto the pericardium, and the diaphragmatic origin is cut from the anterior midline, laterally along the costal margin on the right as far as the lowest angle of the common costal cartilage (Fig. 4J). Step 4. The sixth and seventh (common costal) cartilages are now divided in the line of greatest deformity, corresponding to the resected segments above. This incision is carried directly into the pleural cavity, across the sixth and fifth intercostal muscles, ligating the intercostal vessels as encountered. The incision is then carried upward across intercostal muscles and posterior perichondrium as high as necessary to achieve sternal elevation without tension. Usually this will be at least across the perichondrium of the fourth costal cartilage. After transection and elevation of the sternum (step 6) further incision may be required to release tension (Fig. 4K). It will be noted that the sternum already tends to move upward exposing the cut edge of the diaphragm on the same plane as the transversus abdominis muscle under the posterior rectus sheath. This muscular envelope of the abdomen will later be repaired (Step 9). Step 5. On the left, especially if the heart is much displaced, it may be possible to avoid entering the pleural cavity by pushing the intact pleural reflection away from the under surface of the lower chest wall and upper medial aspect of the diaphragm. With good endotracheal anesthesia, however, there is
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less risk in deliberately opening the left pleural cavity than in making an unnoticed inadvertent small pleural opening, which may result in a postoperative pneumothorax. Whether or not the left pleural cavity is opened, the sixth and seventh (common costal) cartilages and intercostal bundles, at least as high as the perichondrium of the resected segment of fourth cartilage, are now divided as symmetrically as possible with the division on the opposite side. This must be done even when the angulation on this side is minimal, in order to allow the sternum to rise symmetrically. Step 6. A rectangular flap or "trap door" of periosteum is stripped from the sternum at the level of greatest backward angulation (usually at the level of the second interspace, just below the manubriogladiolar junction). The outer table of the sternum so exposed is now divided transversely with osteotome or Stryker saw, and a second transverse incision is made at an angle calculated so that the wedge removed will bring the anterior surface of the gladiolus up to, or a little above, the anterior surface of the manubrium (Fig. 5L). The posterior table of the sternum is now broken transversely, and the gladiolus held in the position of slight overcorrection. Care must be taken to avoid too great overcorrection, since the complete lack of tension in this procedure should obviate the tendency to recurrent depression that may follow less radical methods. This complete lack of tensions may require further upward
Fig. 5. Correction of deformity, IIh (L) Step 6-0. Elevation of sternum and attached cartilages after completion of transverse wedge osteotomy. (M) Step 6-b. Diagrams to show "trap door" periostoal elevation, wedge o~teotomy, and fixation in slight overcorrection by suture of periosteal flap. (a) Elevation of periosteum; (b) PeriosteaJ flap retracted; wedge cut and removed; (c) Posterior table broken, leaving periosteum intact; (d) Repair of sternum by overlapped suture of periosteal flap. (N) Step 7. Mu|tip|e chandratomies and backward breaking of the costal cartilages close to the sternum, to remold the anterior chest wall. (Insert: Excision of prominent cut edges of cartilages, if indicated.)
HUMPHREYS AND JARETZKI
division of perichondrium and intercostal muscles as described in Step 4. This should be done before securing the gladiolus in corrected position by overlapping the "trap door" of sternal periosteum with strong mattress sutures of heavy silk or other nonabsorbable material (Fig. 5M) Firm fixation of the transected sternum in good position and without tension is the key to successful repair. External or internal support is seldom needed in children. In adolescents and adults, wire sutures may be placed through the pectoral skin and muscles, passing under the corrected sternum, to be attached to an external metal "ladder" or "bridge" on the anterior chest after closure of the wound. These wires are to steady the mobilized sternum, preventing paradoxical chest wall motion in the early postoperative period. They are not for "'traction" which implies inadequate sternal mobilization. In some cases, extreme distortion of the sternum, with a twisting or S-shaped deformity, will require more than one sternal transection, sometimes vertically as well as horizontally, in order to achieve proper correction. Step 7. With the sternal deformity corrected, it will be found that the costal cartilages attached to it now project upward. By cutting halfway through them close to their sternal attachments and then breaking them back to meet the lateral chest wall edge, an anteriorly arched chest wall is restored. At times more than one cut is necessary, or additional resection of cartilage will be expedient. It should be noted, however, that if too much cartilage is resected, the arching of the anterior chest will be lost. The final reconstruction should be a cartilagenous arch, resting against the rib ends on each side, and with the sternum as its keystone. This results in a reasonable rigidity of the anterior chest in the early postoperative period that depends on reconstructing the cartilages rather than removing them (Fig. 5N). Step 8. Sutures are placed through perichondrium and intercostal muscles without reference to corresponding levels. Usually the common cartilage comes to lie one or two interspaces higher than it originally lay, with the angle of junction to the lower sternum now wide, in contrast to the usually very narrow preoperative angle. The cut ends of this cartilage should be trimmed to fit the chest wall, and the corresponding lateral cut ends, which now "float" in the hypochondrium, should be rounded off to obviate an unsightly "bump" later on (Fig. 60). Before completing closure of the chest, an intercostal catheter of medium size is placed through the posterior axillary line into the right pleural cavity, just above the diaphragm, with its tip in the paravertebral gutter. On the left, if the pleura has been opened, a small catheter is placed across the anterior pericardium with its tip in the pleural cavity, and brought out of the chest in the midline below the stump of the resected xiphoid. (This will be withdrawn at the close of the procedure: Step 10.) Step 9. The diaphragm is now repaired. If it is significantly shortened from xiphoid to spine, the central tendon can be lengthened by placing interrupted mattress sutures of strong, nonabsorbable suture material transversely across the V or U-shaped defect in the diaphragm where the depressed sternum was originally attached to it, so as to bring the cut edges of the defect together in the midline. The anterior muscular diaphragmatic edge, together with edges of
CORRECTION OF PECTUS EXCAVATUM
Fig. 6. Correction of deformity, IV. (0) Step 8. Suture of cartilageintercostal edge to chest wall. Sutures to lengthen central tendon if necessary. Suture of linea alba to diaphragm and sternum. Insert shows diagram of diaphragm from above to shQw anterior gap that is closed by suture if necessary. (P) Step 9-a. Suture of diaphragm to transversus abdominis muscle laterally. (Q) Step
9-b. Closure of pleural cavity by suture of the diaphragm to the costal margin. Steps (a) and (b) may be done together or separately, depending on where the costal margin comes to lie after correction.
the transversus abdominis muscles, are then sutured to the common costal cartilage in its new (elevated) position. This closes both pleural cavities (although they still communicate across the anterior mediastinum) and gives some support to the anterior abdominal wall, tending to control the associated " p o t belly." Any tension on the mobilized sternum is downward, toward the feet, which tends to neutralize overcorrection, rather than back toward the spine, which might cause a recurrent depression (Fig. 6P and Q). The right pleurai catheter is now attached to water seal drainage with slight suction. The left pleural catheter is clamped. Step 10. The rectus abdominis muscles are first brought together in the midline by suturing the cut edges of the anterior rectus sheaths together over the linea alba, bringing the left intrapleural catheter out between them just above the umbilicus. This corrects any diastasis and umbilical hernia, if present. The upper transversely cut rectus sheaths are now sutured to the anterior chest wall or costal margin with as little tension as possible (Fig. 7R). The pectoralis major muscles are brought back over the suture lines in the anterior chest wall by suturing the pectoral fascia to the periosteum of the sternum in the midline. Removal of the towel or sandbag under the thoracic spine will relieve tension at this stage. When completed, there should be no pleural leakage. The anesthesiologist now raises intratracheal pressure as the
HUMPHREYS AND JARETZKI
Fig. 7. Closure. (R) Step lO-a. Suture of the medial cut edges of the anterior rectus sheaths in the midline, bringing the muscles together in the midline over the linea alba, eliminating the diastasis. If an umbilical hernia is present, it is repaired at the same time. Suture of the superior cut edge of the left rectus sheath to the chest wall is shown. (S) Step lO-b. Rectus sheath closure is completed. The pectoralis major muscles are being sutured in place. (T) Step 10-c. Final fascial closures completed. Note that the pectoral muscles are brought together in the midline by suture to the sternal periosteum.
left pleural catheter is withdrawn under water seal and until no further bubbles appear in the water seal bottle on the right. Both lungs should now be fully reinflated (Fig. 7S and T). The closure is completed by subcutaneous and skin sutures to unite the skin flaps. Either a soft rubber drain brought out just above the umbilicus or small " H e m o v a c " tube drains should be used to prevent accumulation of serosanguineous fluid under the skin flaps. Usually blood replacement is necessary. Beginning with a saline infusion, blood is added at the start of correction o f the deformity, calculating the a m o u n t needed to replace blood estimated lost during the procedure. This method, although certainly more radical, time-consuming, and elaborate than others, does not result in greater morbidity or mortality. In over 70 operations there have been no operative deaths or serious complications. There was one superficial wound infection and one infection which required operation to remove a silk suture from a chronic sinus 10 mo after operation. The would then healed. This child also had congenital heart disease. He died of pneumonia and empyema 17 mo after removal o f the suture. There were no other late deaths. During the same 20 yr period an almost equal number of comparable patients have been observed who were operated upon by other methods. In ad-
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dition, the diagnosis was made on many other patients who were not operated u p o n . C o m p a r i s o n o f the l o n g - t e r m r e s u l t s in t h e s e p a t i e n t s will be r e p o r t e d in a n o t h e r p a p e r . F r o m t h i s c o m p a r i s o n t h e j u s t i f i c a t i o n f o r so r a d i c a l a " c o s metic" procedure should emerge. REFERENCES
1. Ochsner A, DeBakey M: Chone-chondrosternon, J Thorac Surg 8"469, 1939 2. Brown AL: Pectus excavatum (funnel chest). J Thorac Surg 9:164, 1940 3. Sweet RH: Pectus excavatum: Report of two cases successfully operated upon. Ann Surg 119:922, 1944 4. Lester CW: Surgical treatment of funnel chest. Ann Surg 123:1003, 1946 5. Ravitch MM: The operative treatment of pectus excavatum. Ann Surg 129:429, 1949 6. Lester CW: Pigeon breast, funnel chest and other congenital deformities of the chest. JAMA 156:1063, 1954 7. Ravitch MM: Technical problems in the operative correction of pectus excavatum. Ann Surg 162:29, 1965 8. Dailey JE: Repair of funnel chest using substernal osteoperiosteal rib graft strut: Report of case with 4-year follow-up. JAMA 150:1203, 1952 9. Adkins PC, Gwathmey O: Pectus excavaturn: An appraisal of surgical treatment. J Thorac Surg 36:714, 1958 10. Adkins PC, Blades B: A stainless steel strut for correction of pectus excavatum. Surg Gynecol Obstet 113:111, 1961 11. Grifl]n EH, Minnis JF: Pectus excavatum: A survey and a suggestion for maintenance of correction. J Thorac Surg 33:625, 1957
12. Mayo P. Long GA: Surgical repair of pectus excavatum by pin immobilization. J Thorac Cardiovasc Surg 44:53, 1962 13. Rebbein F, Wernicke H: The operative treatment of the funnel chest. Arch Dis Child 32:5, 1957 14. Rehbein F: The use of internal steel struts in the operative correction of funnel chest. J Pediatr Surg 1:80, 1966 15. Willital GH: Operative Korrektur der Trichterbrust und ihre Spatergebnisse, Monatsschr Kinderheilk i 18:633, 1970 16. Paltia V, Parkulainen KV, Sulamaa M, et al: Operative technique in funnel chest: Experience in 81 cases. Acta Chir Scand 116:90, 1959 17. May AM: Operation for pectus excavatum using stainless steel wire mesh. J Thorac Surg 42:122, 1961 18. Morris JD: Surgical correction of pectus excavatum. Surg Clin N Am 41:1271, 1961 19. Wada J, Ikeda 1, Ishida T et al: Results of 271 funnel chest operations. Ann Thorac Surg 10:526, 1970 20. Humphreys GH, Connolly JE: The surgical technique for the correction of pectus excavatum. J Thorac Cardiovasc Surg 40:194, 1960 21. Daniel RA: The surgical treatment of pectus excavatum. J Thorac Surg 35:719, 1958