The Management of Pectus Excavatum C. EVERETT KOOP, M.D., Sc.D.(MED.), F.A.C.S., F.A.A.P.*
IN SPITE of the number of recent additions to the surgical literature on pectus excavatum, it would seem that there is no agreement concerning the etiology of this thoracic defect, no uniformity of opinion concerning the physiological deficits produced by the funnel chest, no established indication for operation, no critical cardiac or pulmonary function tests to establish such indications, and no little confusion concerning the age for operation if and when surgical treatment is indicated. There is less variation in recommended techniques than one might suppose. The different methods of management that are suggested, however, may indicate that no method is eminently satisfactory. We have operated upon a number of infants with pectus excavatum, performing a minor procedure before the age of four months, and, on a larger series of older children, carrying out a major reconstruction of the thoracic cage. In addition, we have followed a group of nonsurgically treated children larger than the two surgically treated series combined, and it is on the basis of these experiences that we venture an opinion concerning the management of pectus excavatum. Fortunately, we have had no mortality and very little morbidity, but in spite of this good record, the potential mortality from the major procedure leads us to take and to recommend a most conservative attitude toward this lesion. ETIOLOGY
Many who have written about pectus excavatum call attention to the abnormal pull of the diaphragm as a factor in etiology. Much is said about the thick ligament behind the sternum holding the sternum in its depressed position, yet at a recent meeting representative of pediatric surgeons in America, most had not encountered such a ligament in the From the Surgical Clinic of the Children's Hospital of Philadelphia and the Harrison Department of Surgical Research, School of Medicine, University of Pennsylvania, Philadelphia. '" Associate Professor of Pediatric Surgery, University of Pennsylvania Schools of Medicine; Surgeon-in-Chief, Children's Hospital of Philadelphia; Assistant Surgeon, University of Pennsylvania Hospital.
C. Everett Koop
course of their operations for the correction of pectus excavatum. This does not mean that the ligament does not exist, but it does mean that it is certainly not the etiological factor in all patients. Hausmanl has reported 13 such ligaments in an operative series of 51 patients. Other authors have stressed musculoskeletal imbalance. We have seen evidence in enough patients with severe funnel chests and severe, operable craniostenosis to suggest that there may be a fundamental bone and cartilage defect. There is no doubt that hereditary tendencies are frequent. PECTUS EXCAVATUM AND PHYSIOLOGIC DEFICITS
Pectus excavatum certainly causes displacement of the heart to the left and usually upward. The heart is enlarged in its transverse diameter but the anteroposterior diameter is decreased. Sometimes the heart is rotated if the depression is asymmetrical. Edeiken and Wolferth2 showed that in spite of this, electrocardiograms fail to exhibit consistent electrical axis deviation. They were of the opinion that uncomplicated funnel chests have no clearly defined effect on the functional capacity of the heart unless the deformity is traumatic or of rapid development. On the other hand, Fisher and Dolehide,3 in studying fatal cardiac failure in persons with thoracic deformities, reported 11 cases in over 30,000 necropsies. In many of these patients the deformity was so marked that it had hindered general development so that the appearance of the patient suggested dwarfism. This is not the usual type of patient seen in childhood with pectus excavatum, although heart failure allegedly due to a funnel chest has been reported. Rydell and Jennings4 report having seen a number of adults with funnel chest and believe that surgical treatment of these patients is rarely indicated. They state firmly that as yet they have not examined an adult who had serious enough cardiac or pulmonary difficulties to justify operation, although they felt that one would be seen eventually. They thought that for persons under puberty the main reasons for operation were for appearance and posture and because of the possible development of psychoneuroses. To counteract this strong statement, there is the report of Bill5 on questionnaires sent to a group of American surgeons asking what proportion of patients with some degree of funnel chest develop cardiac, pulmonary or psychological disabilities. The answers to these questionnaires surprised us. A majority of the 25 surgeons' who replied thought that funnel chest produced cardiac, pulmonary and psychologic difficulties. Twenty-three of these 25 surgeons were of the opinion that funnel chest should be repaired in childhood. We find it difficult to reconcile the number of children to whom operation is recommended with the number of adults with pectus excavatum who have no physiologic deficit. Evans'6 study of patients with funnel chest and a
anagement of Pectus Excavatum
suspicion of heart disease indicated, to him that these patients were invalids because of medical advice rather than because of true impairment of their hearts. There seems little doubt that in some patients with funnel chest, forcible displacement of the heart produces some torsion of the great vessels and may be responsible for diminished exercise tolerance. We have seen several patients with an apparent cardiac deficit who have been improved by operation for funnel chest. We have also seen two patients with the concomitant lesions of pectus excavatum and tetralogy of Fallot. There are sufficient reports in the literature by competent observers to indicate that there is, on occasion, the definite association of cardiac symptomatology with funnel chest. Cardiorespiratory studies upon infants are rather impractical and one wonders whether they would be critical enough to separate the patients requiring surgical intervention from those who could get along without it. The respiratory history in some children is suggestive of difficulties associated with funnel chest. It is not unusual to obtain a history of repeated bouts of pneumonia in successive winters which is improved or eliminated following operation for funnel chest. In our opinion, the respiratory infections are not due to diminished lung volume, but rather to the difficulty which these children have in producing an effective and a productive cough. INDICATIONS FOR OPERATION
The standard indications which are listed by most authors are physiologic, orthopedic and cosmetic, or psychological. Physiologic Indications
The difficulties in assessing the cardiac deficit have already been mentioned. Exercise tolerance is usually considerable in a child and may not be a critical enough test to pick up minor changes in cardiac physiology which can be progressive and detrimental to the child's future. We have, however, seen patients whose exercise tolerance has decreased over several years of observation in whom we thought operation for the correction of pectus excavatum was beneficial. Certainly patients who have a cardiac deficit not due to intrinsic cardiac disease or who have a diminishing exercise tolerance are candidates for operation. The indications for operative treatment in the respiratory group are much less clear. Even the child who has repeated respiratory infections, including pneumonia, during successive winters may have his respiratory infections on some basis completely unrelated to a concomitant thoracic cage deformity. The approach to surgical intervention in such patients should be ultraconservative, and operation recommended on a respiratory basis only after every avenue of investigation has been exhausted.
c. Everett Koop
There are patients with such a respiratory history who are below par in their growth and development, and who have a deep funnel chest. It is very tempting to assume that the physical retardation and the thoracic deformity are associated in a causal relationship. This temptation is even greater when repeated observations indicate that the funnel is deepening with the passage of time. In our own experience, several patients operated upon for repeated bouts of pneumonia and the associated retardation of growth and development have improved without question following operation. However, in the same patients, one cannot say unequivocally that the passage of time alone might not have brought about a similar beneficial change. On the other hand, certain patients stand out in one's experience as respiratory problems that were alleviated without question by operation. One such patient in our series had been treated as an asthmatic for several years, had severe respiratory infections in the winter and during the pollen season was in considerable distress. These facts plus his apparent retardation in school led us to operate upon him at the age of eight. Six months after the operation and in spite of sensitivities to the same allergens, this boy had no respiratory problem during the pollen season, and during the following several winters had nothing more serious than several upper respiratory infections. During the next year he did two years' school work in one and caught up with his class. The benefits of operation in this child seemed to us to be unmistakable. It would seem that the conservative surgeon would have no difficulty in deciding which patient to operate upon for cardiac or respiratory causes. However, when one gets into the field of cosmetic and psychic indications for operation, we believe that the indications for surgical treatment are less definite. Cosmetic and Psychic Indications
We are in favor of numerous operative procedures which correct cosmetic defects and improve the child's adjustment to his problem. However, a review of most of these surgical procedures indicate that they are obviously associated with employability, frequently have functional as well as cosmetic benefits, and uniformly carry a small risk in comparison to the benefits to be derived from operation. On the other hand, the cosmetic defect of a pectus excavatum can always be covered with a shirt. Employability is rarely dependent upon having a cosmetically· acceptable thoracic cage. And finally, countless adults with serious cosmetical alterations in the thoracic cage have no functional impairment in spite of rigorous activity. If the operation for the correction of a funnel chest carried with it little or no risk, we would be as anxious to recommend surgical intervention as we are where a child has a single undescended testicle. However, if one lists the potential complications he
The Management of Pectus Excavatum
must think a second time before exposing his patient to these hazards: pneumothorax, hemothorax, empyema, pericarditis, pneumonitis, osteomyelitis, serum collection, and wound infection. A wound infection in such a patient would rarely be confined to the wound itself, but would spread to the adjacent tissues, dead space, and body cavities. Certainly some cosmetic defects produced by a funnel chest are not much worse than the postoperative results in some patients whom I have examined. The cosmetic indications should be ignored in the female where eventual breast development will effectively hide the thoracic cage deformity. More will be said about some of the deterring factors regarding the indications for operation in a discussion of the age of election for these operative procedures. Other Considerations
Correctly or incorrectly, we have used anteroposterior thoracic measurements with a pair of calipers in some instances as an indication for operation. Most youngsters who have a pectus excavatum also have a shorter anteroposterior diameter of the chest at the level of the manubrium than do children of comparable age without the deformity. If, on repeated examinations at intervals of six months, the anteroposterior diameter at the level of the manubrium is increasing while the anteroposterior diameter at the depth of the funnel is decreasing in actual measurement, it seems to us that there is relatively good evidence that the deformity will be progressive and that operation can be advised. Again, it is only fair to state that we have not followed a series of patients who showed this progression to see what their natural course in development might be without operative interference. Because of our disinclination to carry out a major thoracic cage reconstruction, we tend to be liberal in our indications for surgical intervention in young infants with a funnel chest. Infants under the age of four months with a funnel chest and nonrigid cartilages are benefited by an operation which removes the xiphoid process, detaches the rectus sheath from the lower thoracic cage, and perhaps divides the lowermost cartilages attached to the sternum. This operative procedure, unlike the one carried out in older children, is practically without risk. Because of this, we feel that it can be undertaken in the sincere hope that the later, more major procedure can be avoided. Even though we find it difficult to obtain statistics on it, we would rather carry out this simple operation on several children even without the guarantee of success than to do one major thoracic reconstruction on an older child. In approximately 20 patients operated upon under the age of four months, only one has been reoperated upon for his deformity at a later date. Another is probably a candidate for eventual intervention. We would do more of these operations if we saw patients early enough but most of the young patients we
C. Everett Koop
see with this deformity are brought to us at the age of 10 to 15 months for the first visit and we conclude at that time that the minor .procedure does not carry enough hope for success to make it worthwhile. ROENTGEN STUDIES
Roentgen studies of the chest should include anteroposterior, lateral and oblique views. An especially helpful study is the lateral view showing the sternovertebral distance. This can also be dramatically shown by streaking the depression in the anterior thorax with some barium. Figure 548 shows the anteroposterior as well as the la teral view in a child
Fig. 548. Anteroposterior and lateral roentgenograms of a 10 year old with pectus excavatum.
of two years. Figure 549 shows the extremely narrow sternovertebral distance in a 55 year old man who had carried on an active life as a school teacher without any cardiac or respiratory difficulty. It is the occurrence of such depressions in normal, healthy adults that strengthens our belief in a conservative course of action in many children. AGE OF ELECTION OF OPERATION
Except for the rare patient who may be encountered in cardiac failure, the operation for pectus excavatum is a purely elective procedure. It has already been stated that we favor a minor operative procedure on infants under the age of four months (Fig. 550) and have no hesitancy in operating upon such infants if the need for a major thoracic reconstruction can be prevented in later life. After the age of four months, especially in robust infants, the excision of the xiphoid process, the division of the
The Management of Pectus Excavatum
rectus attachments, and the digital elevation of the sternum does not seem to be followed by much change in the external configuration of the thoracic cage. When patients with a funnel chest are first seen by us after the age of four months, it is our practice to follow them at intervals of six months, measuring the anteroposterior diameter of the chest at the level of the
Fig. 549. Lateral roentgenogram of a 55 year old school teacher who has led an active life without physiologic deficit. Note narrow sternovertebral distance.
manubrium and in the depth of the funnel as has been described. Approximately two-thirds of our patients so observed tend to improve, insofar as the funnel chest deformity is concerned, with the passage of time. Although it is true that they never completely lose the sternal depression, the costal cartilages seem to be less acutely angulated, and the funnel is wide and shallow rather than narrow and deep. None of these patients shows any physiologic deficit. If a youngster under observation is obviously undergoing a deepening of his deformity and we believe operation is indicated, we prefer to
c. Everett Koop
postpone the procedure until at least the age of 20 months in order that the child may have as comfortable a postoperative course as is possible. It has been our experience that when a child under the age of 20 months is operated upon for a major thoracic reconstruction, a sufficient degree of thoracic instability occurs to make breathing difficult for a matter of several days at least. With the subperichondral resection of costal cartilages and a sternal osteotomy, we have seen children at or
Fig. 550. Three and a half month old male infant with pectus excavatum.
about a year of age breathe over 60 times per minute in oxygen. This is an exhausting experience for children, leads to their agitation, and one wonders how long such respiratory effort can be continued. Anesthesiologists consider the postoperative respiratory problems of these patients to be major ones. In several instances reported to us, anesthesiologists have had to breathe with bag and mask for small infants following major thoracic reconstruction when, in their exhaustion, they could no longer breathe for themselves.
The Management of Pectus Excavatum
Little has been written by surgeons concerning this phase of postoperative management, but it would seem that inasmuch as the indications are not urgent and the operative procedure is no more difficult after two years than before, the age of election should be beyond two years if one is to carry out a major reconstruction. Another reason for postponing the age of election is that we have seen youngsters at two who we believed would ultimately require operation, whose defects have grown more shallow with the passage of time. It would seem that when one adds the postoperative respiratory problems of the young child to the possible spontaneous alteration in the deformity, there is every reason to postpone the operation. Most of the patients upon whom we operate are not only over two, but usually past the age of four. OPERATIVE TECHNIQUE
Our technique for the minor procedure in infants has been described in part. A transverse incision approximately an inch and a quarter long is made over the sternoxiphoid junction to excise the xiphoid, separate the rectus sheaths from the lower costal cage, and occasionally take a small segment of the lowermost costal cartilage bilaterally. The procedure should not take more than 20 to 30 minutes skin to skin. Major thoracic reconstructions have usually followed the principles originally outlined by Brown,1 and later by Ravitch 8 and Lester.9 The technique consists essentially of division of the xiphisternal articulation, division of the posterior ligament if present, and resection of all the deformed costal cartilages for the length of their deformity. A transverse cuneiform osteotomy is carried out at the sternomanubrial junction and the corrected position is maintained by nonabsorbable sutures. Some authors recommend the use of a bone graft bridging the gap left by the resected cartilages. Other writers describe the use of external traction applied to the sternum to maintain its new, elevated position, while some describe the use of removable struts or wires to maintain the new position. We favor a technique which resects all the deformed cartilages and the sternum to which they are attached (Fig. 551) with the subsequent 180 degree rotation of the resected sternum with attached cartilages after which the entire anterior thoracic plate is wired into its new position. Notched lines of division of the cartilages aid greatly in wiring the breast plate in its new position. The great advantage of this technique is the immediate postoperative stability of the chest. An asymmetrical deformity does not lend itself well to this procedure and occasionally, even with a symmetrical defect, alterations in the costal cartilages may be necessary in order to complete the procedure without too much of a pigeon-breast deformity. All of our patients so treated appear to have been overcorrected in the immediate postoperative period, but with the passage of
several weeks the pigeon-breast deformity flattens out and the cosmetic result is good. For patients not suitable for this procedure, we prefer the technique described by Ravitch without the use of external support or traction,
Fig. 551. Breast plate before reconstruction with 180 degrees of rotation.
Fig. 552. Seven year old boy before and after operation for the correction of pectus excavatum.
believing that these techniques add an increased risk for the entrance of infection. For any procedure we prefer a transverse incision through the depth of the funnel, believing that the postoperative cosmetic result is better than with a vertical incision. Attention has already been called to the
The 1Management of Pectus Excavatum
fact that some postoperative scars are not much improvement, cosmetically, over the previously existing funnel. Figure 552 shows the preoperative and postoperative photographs of a child of seven treated by the method described. SUMMARY Pectus excavatum is an uncommon deformity of the anterior chest wall found in persons who usually have a narrow anteroposterior diameter of the thorax at the level of the manubrium and frequently come from families in which other members have a similar deformity. Rarely, patients so deformed have alterations in cardiac and respiratory physiology which can be benefited by reconstruction of the thoracic cage. Pectus excavatum is also a cosmetic deformity but, because of the potential danger in major thoracic reconstruction, we believe that cosmetic surgery for funnel chest should be confined to those patients who show a psychic disturbance and that operation upon patients in the hope of preventing psychological problems is never indicated. There is no doubt that many adults with deep funnel chests have no physiologic incapacity. It is also true that innumerable children show a decreasing depth of the deformity with the passage of time. All of these factors together lead us to take a conservative view toward recommending major thoracic reconstruction for the correction of pectus excavatum. On the other hand, a minor procedure undertaken in infants for the correction of funnel chests is relatively innocuous and should be undertaken more frequently, we believe, in the hope that later more serious procedures can be avoided. Attention is called to the opening statement of this clinic indicating that there are still many unknowns in the management of pectus excavatum. REFERENCES 1. Hausman, P. F.: The Surgical Management of Funnel Chest. J. Thoracic Surg.
29: 636, 1955. 2. Edeiken, J. and Wolferth, C. C.: The Heart in Funnel Chest. Am. J. Med. Sc 184: 445, 1932. 3. Fisher, J. W. and Dolehide, R. A.: Fatal Cardiac Failure in Persons with Thoracic Deformities. A.M.A. Arch. Int. Med. 93: 687, 1954. 4. Rydell, J. R. and Jennings, W. K.: The Surgical Treatment of Funnel Chest Deformity. Am. J. Surg. 88: 69, 1954. 5. Bill, A. H. Jr.: Funnel Chest: Indications for Surgery and the Time of Choice for Operation. Pediatrics 11: 582, 1952. 6. Evans, W.: Heart in Sternal Depression. Brit. Heart J. 8: 162, 1946. 7. Brown, A. L.: Pectus Excavatum (Funnel Chest): Anatomic Basis; Surgical Treatment of Incipient Stage in Infancy; and Correction of Deformity in Fully Developed Stage. J. Thoracic Surg. 9: 164, 1939. 8. Ravitch, M. M.: Operative Treatment of Pectus Excavatum. Ann. Surg. 129: 429, 1949. 9. Lester, C. W.: Funnel Chest: Cause, Effect, and Treatment. J. Pediat. 37: 224, 1950. 1740 Bainbridge Street Philadelphia 46, Penns ylvani1\