Pectus excavatum

Pectus excavatum

J THORAC CARDIOVASC SURG 80:686-695, 1980 Pectus excavatum Late results with and without operation The records were reviewed of 334 patients with a...

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J

THORAC CARDIOVASC SURG

80:686-695, 1980

Pectus excavatum Late results with and without operation The records were reviewed of 334 patients with a diagnosis of pectus excavatum at Presbyterian Hospital in New York, New York, from 1948 through 1977. No operation was done on 168; 174 operations were done on 166. In those not operated upon 18% of infants died of associated anomalies. The deformity improved or disappeared in half of the surviving infants and in some children up to the age of 6 years. Thereafter, it remained the same or worsened. Mild deformities were compatible with long life without symptoms; severe deformities were associated with chronic disability. In those operated upon, there was no surgical mortality. Results were satisfactory 5 plus years after operation in 68 of 102 patients. Late results deteriorated through adolescence and were more clearly related to method of correction than to sex, severity, age at operation, or time after operation. It is concluded that ( I) operation is justified in appropriate cases; (2) it is seldom indicated hefore the age of 3 years; (3) results are best following operations hetween ages 3 and 6 years, although they may he satisfactory at any age; (4) results of radical operations are superior to results of simpler ones; and (5) hetter objective methods of evaluation over many years are needed to judge the value of any procedure.

George H. Humphreys II, M.D., and Alfred Jaretzki lll, M.D., New York, N. Y.

W

hen the editor' of the Yearbook of Pediatrics writes, "We have worked hard to keep patients with funnel chest out of the hands of surgeons . . .. The lack of agreement on timing of operation confuses us and convinces us that surgeons don't know what they are doing," the challenge should be met. Surgeons who operate for correction of funnel chest are certain that they do know what they are doing. It is surprising, however, to find that reports of long-term results are few, even though it has been recognized for 40 years that the ultimate success of an operation depends on its evaluation. 2 Most series mix early with late results, and in many the results are known in too small a proportion of all operations done. Only nine of some 40 reports published since 1954 include results 5 years or more after operation (Table I). :I-II Because some children outgrow their deformity and others show no obvious handicap, there is widespread

From the Department of Surgery, College of Physicians and Surgeons, Columbia University, and the Surgical Services of the Babies and Presbyterian Hospitals, New York, N. Y. Read at the Sixtieth Annual Meeting of The American Association for Thoracic Surgery, San Francisco, Calif., April 28 to 30, 1980. Address for reprints: George H. Humphreys II, M.D., the Presbyterian Hospital, 622 W. 168th St., New York, N. Y. 10032

686

belief that operation is merely cosmetic. Several studies have concluded that operation is so often unsuccessful that it is of questionable value.": 12. 1:3 Yet, a real cardiopulmonary handicap is indeed present in some patients, and this condition is improved by operation.I" " If surgeons are to convince pediatricians, parents, and critics of "unnecessary surgery" that operation is justified, three questions must be answered: 1. What result can be expected without surgical correction? 2. What are the end results of corrective procedures? 3. If operation is justified, what are the indications for it? We undertook this study in the hope of finding answers to these questions. Method The records were reviewed of all 334 patients on whom the diagnosis of pectus excavatum was made at the Columbia-Presbyterian Medical Center in New York, New York, between Jan. 1, 1948, and Dec. 31, 1977. No corrective operation had been done on 168; 174 operations had been done on the other 166 patients. As many patients as possible were examined or contacted by telephone or letter to determine their present status. Results are known in 102 (84%) of the 122 patients operated upon in our hospital over 5 years ago.

0022-5223/80/110686+10$01.0010 © 1980 The C. V. Mosby Co.

Volume 80

Pectus excavatum

Number 5

687

November, 1980

Infants-less than 1 year IMPROVED

II,I!II!IPII---_

Number

1lp,I!I!I!I!............................

40%

Children-1 to 12 years

"'1IIIlII!I"'rrr-rlliffiIlllJ!!I!l)

::0:::

UNKNOWN (56%)

_ _ei~e::i~jr

75

old UNKNOWN 46% Total UNKNOWN 43%

Fig. 1. Results in patients not treated surgically.

Results Of the 334 patients, 244 were male and 90 female, a ratio of 2.7 to 1. This ratio did not differ between patients who were and those who were not treated surgically. Less than 10% of the malformations were familial. There was an incidence of 26% of other congenital anomalies, the most frequent being cardiac. All of the deaths in patients under 21 years of age were due to associated anomalies. Patients who were not operated upon. These were a heterogeneous group, falling into three subgroups. The first consists of 50 infants. The diagnosis was made at birth in 30 of them and during their first year in 20. Nine of them died. In the 41 who survived, the deformity disappeared or improved in 20, remained unchanged in five, and became worse in five. The remaining 11 patients were not seen after infancy. The second group consists of 75 children in whom the diagnosis was made between the ages of 1 and 12 years. Three died of other congenital disease. The deformity improved in 12 of the remaining 72, all of whom were given the diagnosis by the age of 6 years. It remained unchanged in 15 and worsened in three. The remaining 42 patients were seen only once, so the final result is unknown. None of the 43 patients in the third group improved. Seventeen of them had the deformity diagnosed between 13 and 21 years, and 26 were first seen as adults. One died of brain abscess at 20. Of the remaining 16 in whom the diagnosis was made in adolescence, five had no symptoms, but four of them were seen only once. Of the 11 with symptoms, six were seen only once, and in the other five the symptoms were observed to increase in severity over periods from 5 to 20 years. The 26 adults included 11 with no symptoms, five of whom were admitted only once; six were followed for many years, and ultimately one died of cancer. The

remaining 15 patients all had disabling psychological and probably physiological symptoms. Of these, five were admitted only once; 10 were followed for many years with progressively severe cardiopulmonary symptoms, although four died of unrelated disease (three cancer, one diabetic coma). The other three deaths appear to be related to the deformity: One died at age 66 of cardiac failure, one at age 65 following operation for constrictive pericarditis, and one at age 67 of cor pulmonale, which was directly attributed to the deformity (Fig. 1). In summary, among these 168 patients, 18% of the infants died of associated anomalies. In surviving infants the deformity disappeared or improved in about half, as it did in 21 % of children in whom the diagnosis was made up to the age of 6 years; no improvement occurred thereafter. Of 16 adolescents, 11 were symptomatic. Of 26 adults, 15 had symptoms which in 10 were known to be progressive over many years. Most deaths were due to associated anomalies or unrelated disease, but the deformity may have contributed to death in two patients, and it caused the death of one patient. Patients who were operated upon. A total of 166 patients underwent 174 operations. There were no operative deaths and no serious complications. Four late deaths were all related to complications of other anomalies. Age at operation varied from 17 months to 37 years; almost half of the patients were between 3 and 6 years of age and only nine were over 21 . Results were termed "excellent" when the chest appeared normal, the scar was inconspicuous, and the symptoms, if any, had gone. When there was some residual or recurrent sternal depression or if the scar was bothersome, but in general the patient and family were satisfied, the result was termed "good." Taken together, excellent and good results were considered

688

The Journal of Thoracic and Cardiovascular

Humphreys and Jaretzki

Surgery

Before operation MILD

31%

MODERATE 20%

SEVERE

37%

74

Up to 1 year after operation NORMAL

I

55%

33%

MILD

1 to 5 years after operation NORMAL

I

66%

MILD

Over 5 years after operation NORMAL 33%

MILO

40%

MODERATE 17 %

40

Fig. 2. Vertebral index.

"satisfactory. " In patients with unsightly scars, persistent pain, or sufficient asymmetry to cause embarrassment, but whose sternum was in better position than before operation, the results were termed "fair." If a second operation was done or considered indicated, the result was termed "poor." Together, fair and poor results were considered "unsatisfactory." These ratings are subjective, especially when based on the written response of the patient or parent. Physiological studies were not done because of the opinion that the deformity was not associated with measurable abnormalities.I"- 19 Other objective methods of evaluation have been advocated;" 10. 12. 20-26 among which we have found the vertebral index" most useful. We have obtained 224 vertebral index measurements on 94 patients; 74 before operation, 73 up to 1 year after operation, 37 between 1 and 5 years after operation, and 40 "late" readings. Those that fall within the limits of the standard diagram are "normal"; indices up to 5 points higher are "mild"; from 5 to 10 points higher, "moderate"; and more than 10 points higher, "severe." On this basis, preoperative indices were normal in 12%, mild in 31%, moderate in 20%, and severe in 37%. Up to 1 year after operation 55% were normal and 38% mild (93%); 7% of indices were persistently or recurrently moderate. Between 1 and 5 years, results were also good; 68% were normal and 24% mild (92%), but 8% of indices were moderate or severe indices. After 5 years only 33% were normal and 40% mild (73% satisfactory), whereas 17% of indices were moderate and 10% severe (Fig. 2). The key results, therefore, appear to us to be , 'late" - more than 5 years after operation. Since result is related to method of correction, the results of 13 operations done on nine patients in other hospitals are excluded. Results of 35 operations are excluded because the follow-up period was less than 5 years. There

remain for analysis 126 operations on 122 patients; results are known of 106 operations on 102 patients (84%). Of these, the results of 68 operations, or two thirds of the 102 patients, were satisfactory. In the hope of identifying factors resulting in unsatisfactory results in one third of the patients, we have explored the following: 1. Sex. There was no significant difference in result by sex. 2. Severity. If the presence of other anomalies or familial history be considered an indication of the severity of the genetic defect, it was not related to the late results. 3. Age at diagnosis. Although results appear to be better in patients whose diagnosis was made in infancy, the actual onset of the deformity is so unreliably reported in older groups that the significance of these data is questionable. 4. Age at operation. Late results were best in patients operated upon under the age of 6 years. Of the 14 patients treated before the age of 3 years, 10 (71 %) had satisfactory results, and of the 50 treated between 3 and 6 years, 35 had satisfactory results (70%). Of the 17 children operated upon between the ages of 6 and 12 years, only seven (41 %) had satisfactory results. Results were better in adolescents, among whom 12 of 18 (67%) had satisfactory results, and in the seven adults, among whom four (57%) had satisfactory results (Fig. 3). 5. Method of correction. Of the 161 operations done at our hospital in which the method is known, 74 were done by the method of the authors" and 87 by other methods. The latter were usually based on the methods of Lester'" and Ravitch.i" with a number of variations. Internal struts were used on none. Since late results cannot be known on 12 patients under 6 years of age when last seen, or on 23 patients

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68 9

Less than 3 years old 10 SATISFACTORY

71%

4 UNSATISFACT

29%

14

15 UNSATISFACT

30%

50

59%

17

33%

18

3 to 5 years old 35 SATISFACTORY

70% 6 to 12 years old

7 SATISFACTORY

41%

10 UNSATISFACTORY 13 to 20 years old

12 SATISFACTORY

67%

6 UNSATISFACTORY

Over 20 years old 57 %

4 SATISFACTORY

3 UNSATISFACTORY

43%

7

36%

106

Total 68 SATISFACTORY

64%

38 UNSATISFACTORY

Fig. 3. Known late results of operations according to patient's age at operation. Method

ALL

~ADICAL

Percent of Operations

No. of Ops.

SATISFACTORY

54 %

126

SATISFACTORY

73%

70

,----==1=··· ." ". .

56

Fig. 4. Late results of operations for pectus excavatum: Method.

operated upon during the most recent 5 years of the study period, 35 operations are excluded. Of the remaining 126 operations, 70 were done by the radical (our) method, and late results are known in 66 (94%); 56 were done by other methods, and late results are known in 40 (71%). Although the groups are not strictly comparable because of the larger number of unknown results in the other group (29%) than in the radical operation group (6%), the fact that 41% of the results in the other group were unsatisfactory as compared to 21% unsatisfactory in the radical group is significant (Fig. 4). 6. The factor of time. The vertebral index data ap-

pear to show that results deteriorate with time after operation. Is this because operations in the past were less effective than those done more recently? Grouping operations into four 5 year periods from 5 to more than 20 years after operation does not show any consistent relationship between the result and how long ago the operation was done. Some patients were not followed to the full extent of the time after operation, however. When grouped by time between operation and last visit by the patient, 42 patients were followed less than 10 years and only 10 for more than 20 years. Although those followed longest had poorer late results than those followed for

690

The Journal of

Humphreys and Iaretzki

Thoracic and Cardiovascular Surgery

5 to 9 years 26

SATISFACTORY

62 %

16 UNSATISFACTORY

38%

42

9 UNSATISFACT 26%

34

10 to 14 years 25 SATISFACTORY

74%

15 to 19 years 12 SATISFACTORY

60%

8 UNSATISFACTORY

40%

20

50%

10

38 UNSATISFACTORY 36%

106

Over 20 years 5 SATISFACTORY

50%

5 UNSATISFACTORY

Total 68 SATISFACTORY

64 %

Fig. 5. Known late results of operations: Follow-up time.

Table I. Other reports of late results Ref. No.

3 4 5 6 7 8 9 10 II

Year of report

Years patients followed

1964 1968 1970 1970 1971 1971 1977 1978 1978

5-10 5-15 7-8 5-11 7-10 5-15 5-16 8-28 11-27

Total No. of operations

54 43 25 86 186 319

Operations No. reported

58 33 41 55 18 81 100 108 278

shorter periods, the pattern is not consistent (Fig. 5). When results are grouped according to the age of the patient at last contact, they are more consistent. The result was satisfactory in 13 of the 18 children (72%), in 33 of the 48 adolescents (69%), and in 22 of the 40 adults (55%). This deterioration with age was true of both the radical and other operations, but results of radical operations were not only better than those of other operations in each age group, but also they deteriorated less rapidly following radical (89%, 79%, 71%) than following other operations (56%, 47%, 31 %) (Fig. 6).

Discussion The data obtained are suggestive rather than conclusive. Too many final results are unknown (43% of patients not treated surgically, 34% of those treated surgi-

I

Results known to be satisfactory

% of total

61 95 72

94 58 87

No.

27 29 28 52 8 59 78 89 149

I

% of reported

46% 88% 68% 94% 44% 73% 78% 82% 54%

I

% of total

54% 65% 32% 70% 58% 47%

cally), many of the subgroups are small in number, and results are perforce judged subjectively. Nevertheless, certain inferences can be drawn. What results can be expected without surgical correction? It seems clear that in about half of the infants who have the deformity at birth or develop it during their first year, and do not die of associated anomalies, the deformity will disappear or improve so much that surgical correction need not be considered. This has long been known'" and recently confirmed.P' This outcome becomes much less frequent after age 3, and was not observed in children in whom pectus excavatum was diagnosed after age 6. That many children, especially boys, are injured psychologically is widely known.s: 22. 28, 32. 33 In our records it was evident that many adults who denied symptoms had been through a difficult adjustment period and that some adults were

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Pectus excavatum

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6-12

13-19

Number

Radical Operations

Age

9

1 S AT I S FACT O R ~

====:33

SAJISFACIOR

20+

ATISFACTORYt Other Operations

6-12

jSAT ISFACTORY

20+

31%"

16 All Operations

~~~~~

ATISFACTORY

Fig. 6. Known late results by method and patients age at follow-up. permanently affected psychologically although living to advanced ages. All severely deformed adults had symptoms that were often considered neurotic or psychotic . One adult died of cor pulmonale directly attributed to his deformity, and in two others the deformity may have contributed to their deaths . There seems little doubt that the deformity was a serious problem for many of the adults for whom surgical correction had never been considered. Is surgical correction the answer? What are its ultimate results? Corrective operations have now been done for over 40 years," :34 and some of these patients must have reached advanced age, but neither this study nor, to our knowledge, any other study shows the results in later life of correction. * In this series there were no operative deaths or serious complications. The early results were generally good, but they deteriorated in our patients as they grew into adults . This is in agreement with others.": 11. :15 Nevertheless, the majority of the patients who had surgical correction had a satisfactory late result. What are the indications for operation? It has been shown that the deformity disappeared or became insignificant in about half of the surviving infants and in a small percent of children up to the age of 6 years. ·Our oldest patient was 53 years old when last examined in 1974. 21 years after correction of a severe deformity at the age of 32 . She is still happy with the result that we rated " good . " The patient whom we have followed longest was 34 years old when last heard from in 1973. 24'/2 years after correction of a moderate deformity at age 9. with an "excellent" result.

Although late results of operations on children under 3 years of age were as good as those between 3 and 5 years, they were no better. Since spontaneous improvement may occur, the decision to operate upon a patient under 3 should usually be deferred . If there has been no improvement, especially if attention has been directed to upper airway obstruction and exercises to encourage good posture and deep breathing, operation appears to be indicated by the age of 6 . If the deformity is deep and fixed when the patient is first seen, or if it is noted to be worsening, operation can be done earlier."! In this series, operations on patients under 6 years of age accounted for 64 of the 106 known late results (60%), and in 45 (70%) they were satisfactory (Fig. 3). Indications for operation in older patients are less clear. Although in no case did the deformity improve spontaneously after age 6, only seven of the 17 children undergoing correction between the ages of 6 and 12 years had satisfactory late results. One was reoperated upon successfully in adolescence . Among adolescents, 12 of the 18 known late results were satisfactory, but two patients were operated upon a second time for late recurrence, and both operations failed to achieve a satisfactory result. On the other hand , six of the 12 satisfactory results were rated excellent 10 to 23 years after operation . Results in the few adults were less good, but some satisfactory late results followed operations at all ages. These findings are of interest in relation to other surgeons' conclusions on choice of age for operation. Many prefer early operation." 10. 20. 21 .25 .26.28- 30. 32.33.36-39

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Humphreys and Jaretzki

Others' recommendations vary from a statement that late results are worst following operations in early childhood," through ages 6 to 16,40 ages 12 to 18,41 ages 12 to 33,2:1 "after puberty, "42 "after growth is complete, "4:1 to "any age. "44 Why our patients operated upon between the ages of 6 and 12 had worse late results than those older or younger is unexplained. Operations on symptomatic adults, though they cannot be expected to be as successful as those on young children, were nevertheless worthwhile. What is abundantly clear is the relationship of result to method of correction. There is no agreement among surgeons, and new methods, or modifications of older ones, continue to be advocated. It is easy to see why this is so. Surgeons who recognize the increasingly poor results as they deteriorate with the age of their patients tend to blame the method and to seek a better one.": 7. 11.29. :15. :16. :19. 41. 45-51 Since many methods gi ve good results in the first 5 years after operation, the temptation is strong to advocate the new one. However, unless it can be shown that results are superior to those of the older method through adolescence, the significance of the early better result is open to question. Ravitch'" has noted' 'the enormous variety of operative procedures devised for correction of funnel chest. " These may be grouped into four main types: I. Minimal operations for the very young have been discarded. 2. Operations using some form of external traction to bring a mobilized sternum into position and hold it during healing-a method attributed to Sauerbruch and early advocated by Brown," Ochner and Dellakey.>' Lester," and others-has been extensively used by some": H. 21. 25. 40. 50 but tried and discarded by others.": :16. :19. 51 Usually, some variation of internal support is used instead. 3. Internal support by rib, pin, wire, metal bar, or other rigid material, as advocated early by Dailey, 5:1 has a strong following. In 1968 Adkins, Groff, and Blades"described fifteen variations, and new ones have been developed since."": II. 1:1.20. :12. :13. 35. :16. :19. 41, 43. 44. 46-49. 54 None of the advocates of this method appear to have discarded it. 4. Operations have been employed which do not use traction or rigid support. Instead, by reshaping the sternum and removing or reconstructing the deformed cartilages after freeing the sternum from its mediastinal and diaphragmatic attachments, the surgeon strives to help natural forces restore normal contour and cardiopulmonary function. One variety, devised by Brunner and modified by Seling," Geroulanos;" Geisbe;" and others, depends on splitting the sternum sagittally

The Journal of Thoracic and Cardiovascular Surgery

to the point of angulation, then transecting, incising the cartilages from behind, and bringing them forward after removing a wedge of sternum. A more frequent variety in the United States is based on the method of Ravitch'": The sternum is completely freed not only from its mediastinal and diaphragmatic attachments, but also, after total resection of the deformed cartilages, from its intercostal and perichondrial attachments, so that it can rise freely after partial transection. Some surgeons omit the chest wall section, but this is essential for adequate sternal mobilization. The "sternal turnover" operation, most recently advocated by Wada and Ikeda"! and Davis and Shah.s" is the most extreme application of this principle. Reservations concerning destruction of blood supply to the sternum, originally expressed by Sutherland.s" resulted in the development by Daniel"? and by US 27 of modifications going wide of the internal mammary artery at the expense of deliberately opening one or both pleural cavities. We also do not remove all cartilage, but reconstruct to reform a forward arch and provide early thoracic stability. In older children, adolescents, and adults, others provide stability by adding internal support by strut," by "tripod suspension, "10 or by suspending the end of the gladiolus on the xiphoid!" or on a sling of perichondrium of the common costal cartilage. :15. :l7 Still others use various methods as circumstances dictate;": :12. :1:1 and a number have given up this type of procedure in favor of internal support. 5. 11, 20. :15. 41. 48 The results of the present study demonstrate the long-term effectiveness of full mobilization of the sternum. In judging the value of surgical correction, therefore, operative correction should not be condemned because of poor results of a faulty method. Finally, in order to answer the original questions, we must devise better objective methods of evaluating the deformity and its physiological sequelae. We must then use these methods in all of our patients, both those who have had surgically correction and those who have not, throughout the whole period of their growth to adults and thereafter periodically through their entire lives. Such a long-term undertaking is overdue. In the meantime, the evidence that we have indicates that adequate corrective procedures are indeed justified. REFERENCES Gellis SS: Footnote under review of Geisbe et al: Indications and choice of favorable time for operative correction. Yearbook of Pediatrics, Chicago, 1971, Year Book Medical Publishers, Inc., p 227 2 Brown AL: Pectus excavatum (funnel chest). J THORAC SURG 9:164-184, 1939

Volume 80 Number 5 November, 1980

3 Moghissi K: Long-term results in surgical correction of pectus excavatum and sternal prominence. Thorax 19: 350-354, 1964 4 Adkins PC, Groff DB III, Blades B: Experiences with metal struts for chest wall stabilization. Ann Thorac Surg 5:246-254, 1968 5 Sulamaa M, Wallgren El: Trichterbrust. Operations methode und Spatergebnisse. Z Kinderchir8:22-30, 1970 6 Jensen NK, Schmidt WR, Garamella 11, Lynch MF: Pectus excavatum and carinatum. The how, when, and why of surgical correction. J Pediatr Surg 5:4-11, 1970 7 Borgeskov S, Raahave D: Long-term results after operative correction of funnel chest. Thorax 26:74-76, 1971 8 Seling A, Marx E, Cardozo JM: Spatergebnisse der nach Brunneroperierten Trichterbrust. Langenbecks Arch Chir 328:139-152, 1971 9 Meissner F, Bennek J, Gottschalk M: Die Altersabhiingigkeit der Trichterbrust Operation. Zentralbl Chir 102:1432-1440, 1977 10 Haller JA Jr, Shermata DW, Tepas JJ, Bittner HR, Golladay ES: Correction of pectus excavatum without prosthesis or splints. Objective measurement of severity and management of asymmetrical deformities. Ann Thorac Surg 26:73-79, 1978 II Howard R: Funnel chest. Results and description of an improved operative technique. Med J Aust 2: 134-136, 1978 12 Bay V, Farthman E, Naegle U: Unoperated funnel chest in middle and advanced age. J Pediatr Surg 5:606-609, 1970 13 Gyllensward A, Irnell L, Michaelsson M, Qvist 0, Sahlstedt B: Funnel chest. A study before and ten years after operation. Acta Paediatr Scand Suppl 206:56-57, 1970 14 Beiser GD, Epstein SE, Stampfer M, Goldsteine R, Noland SP, Levitsky S: Impairment of cardiac function in patients with pectus excavatum, with improvement after correction. N Engl J Med 287:267-272, 1972 15 Hiasa Y, Sawada S, Fukuda N, Nakaya Y, Niki T, Mori H, Ochi T: Evaluation of cardiac function by means of the non-invasive techniques before and after operation of the funnel chest. Tokushima J Exp Med 25:127-133, 1978 16 Hansen JL, Jacoby 0: The respiratory function before and following surgery in cases of funnel chest. Acta Chir Scand III:226, 1956 17 Liese W, Biihlmann AA: Arbeitskapazitat und Lungenvolumina vor und nach Chirurgischer Korrektur einer Trichterbrust. Spiitresultate bei Erwachsenen. Schweiz Med Wochenschr 104:83-109, 1974 18 Majid PA, Zienkocicz BS, Roos JP: Pectus excavatum and cardiac dysfunction. A case report with pre- and postoperative hemodynamic studies. Thorax 34:74-78, 1979 19 Fishman AP, Turina GM, Bergofsky EH: Disorders of respiration and circulation in subjects with deformities of the thorax. Mod Conc Cardiovasc Dis 27:449-453, 1958

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20 Alnor PC, Jacquet GH: Das Spatergebnis nach Trichterbrustoperationen. Bruns Beitrag Klin Chir 203:171-195, 1961 21 Backer OG, Brunner S, Larsen V: The surgical treatment of funnel chest. Initial and follow-up results. Acta Chir Scand 121:253-261,1961 22 Clark JG, Grenville-Mathers R: Pectus excavatum. Br J Dis Chest 56:202-205, 1962 23 Geroulanos S, Hahnloser P, Senning A: Trichterbrustkorrektur. Indikation, operatives Vorgehen and Resultate nach einer vereinfachten und modifizierten Methode. Helv Chir Acta 41:101-108, 1974 24 Stucki HR: Bestimmung des Sagittalen Thoraxdurch rnessers bei Kindem. Z Kinderchir 11:21-31,1972 25 Svend-Hansen H: Senresultater efter Operative Korrektion af Tragtbryst. Ugeskr Laeger 136:2302-2304, 1974 26 Welch K: Satisfactory correction of pectus excavatum deformity in childhood. J THORAC SURG 36:697-713, 1958 27 Humphreys GH, Jaretzki A: Operative correction of pectus excavatum. J Pediatr Surg 9:899-909, 1974 28 Lester CW: Pigeon breast, funnel chest and other congenital deformities of the chest. JAMA 156:1063-1067,1954 29 Ravitch MM: Technical problems in the operative correction of pectus excavatum. Ann Surg 162:29-33, 1965 30 Hausmann PF: The surgical management of funnel chest. J THORAC SURG 29:636-648, 1955 31 Randolph JG, Tunnel WP, Morton D Jr: Repair of pectus excavatum in children under 3 years of age. Ann Thorac Surg 23:364-366, 1977 32 Hecker WC, Posch I U, Billinger W: Trichterbrust and Kielbrust. Munch Med Wochenschr 119:559-564, 1977 33 Zippel H, Lange ML: Ergebnisse der Operativen Behandlung der Trichterbrust. Beitrag Orthop Traumatol 25:481-488, 1978 34 Ochsner A, DeBakey M: Chone-chondrosternon. J THORAC SURG 8:469-508, 1939 35 Holcomb GW Jr: Surgical correction of pectus excavatum. J Pediatr Surg 12:295-30 I, 1977 36 Ekstrom G, Qvist 0: Surgical treatment of funnel chest. Acta Paediatr Scand 46:605-609, 1957 37 Fonkalsrud EW, Follette D, Sarwat AK: Pectus excavatum repair using autologous perichondrium for sternal support. Arch Surg 113: 1433-1437, 1978 38 Robicsek F, Daugherty HK, Mullen DC, Harbold NB, Hall DG, Jackson RD, Masters TN, Sanger PW: Technical considerations in the surgical management of pectus excavatum and carinatum. Ann Thorac Surg 18:549-562, 1974 39 Willital GH, Meier H, Schwandner R: Operative Korrekturrnoghlichkeiten der Trichter- und Keilbrust. Langenbecks Arch Chir 345:173-179, 1977 40 Hegemann G, Leutschaft R, Schoberth H: Erfahrungen Bei 100 Trichterbrustoperationen. Dtsch Med Wochenschr 87:774-784, 1962 41 Reismann B: Die Chirurgische Korrektur der Trichter und Hiihnerbrust. Dtsch Med Wochenschr 104:42-43, 1979

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The Journal of Thoracic and Cardiovascular Surgery

Humphreys and Jaretzki

42 Geisbe VH, Mildenberger H, Flach A: Indikationen zur operativen Korrektur und gunstige Zeitwahl fur die Operation. Z Kinderchir 7:343-356, 1969 43 de Vivie ER, Heisig B, Brunner L, Regensburger 0, Cottmann UP: Fruh- und Spatergbnisse von Trichterbrustoperationen nach der Methode von Jensen, Schmidt und Garamella, Bruns Beitrage Klin Chir 220:284-291, 1973 44 Heydorn WH, Zajtchuk R, Schuchmann OF, Strevey TE: Surgical management of pectus deformities. Ann Thorac Surg 23:417-420, 1977 45 Haller JA, Markatlic SA, Shermeta OW, Shaker IJ, White 11: Operative correction of pectus excavatum. An evolving perspective. Ann Surg 184:554-557, 1976 46 Johnson LP: Criteria for management of moderate funnel chest deformities in children. Am Surg 38:498-503, 1972 47 Naef AP: Surgical treatment of pectus excavatum. Ann Thorac Surg 21:63-66,1976 48 Paltia V, Parkulainen KV, Sulamaa M, Wallgren OR: Operative technique in funnel chest. Experience in 81 cases. Acta Chir Scand 116:90-98, 1959 49 Robicsek F: Marlex mesh support for the correction of very severe and recurrent pectus excavatum. Ann Thorac Surg 26:80-83, 1978 50 Wichern WA, Lester CW: Funnel chest. Arch Surg 84:170-173, 1962 51 Wada J, Ikeda K: Clinical experience with 306 funnel chest operations. Int Surg 57:707-710, 1972 52 Ravitch MM: Congenital Deformities of the Chest Wall and Their Operative Correction, Philadelphia, 1977, W. B. Saunders Company, p 146 53 Dailey JE: Repair of funnel chest using substernal osteoperiosteal rib graft strut. Report of case with 4-year follow-up. JAMA 150:1203-1204, 1952 54 Rehbein F: The use of internal steel struts in the operative correction of funnel chest. J Pediatr Surg 1:80-84, 1966 55 Davis MV, Shah HH: Sternal turnover operation for pectus excavatum. Ann Thorac Surg 17:268-272, 1974 56 Sutherland 10: Funnel chest. J Bone Joint Surg 408:244-251, 1958 57 Daniel RA: The surgical treatment of pectus excavatum. J THoRAc S URG 35:719-725, 1958

Discussion DR. LYMAN A. BREWER Pasadena. Calif.

Compliments are due Drs. Humphreys and Jaretzki on their excellent long-time follow-up study. Dr. Mulder and I have operated upon 97 patients with serious deformities at the Orthopaedic Hospital in Los Angeles, California. Most of the cases referred for repair have been turned down, for we operate only when the depression is severe, approximately the size of the fist of the patient. Complete repair consists of elevation and tailoring of the sternum and cartilages, lysis of the diaphragm, and Steinman pin or strut fixation up to 2 years following operation. Sternal support has practically eliminated recurrence.

A previously unreported complication during a secondary pectus excavatum operation in a 16-year-old girl with a recurrent deformity (by coincidence, 13 years after the primary repair by Dr. Humphreys) invites comment. During the secondary repair, on elevation of the sternum, profuse hemorrhage occurred, obviously from a heart chamber. A substernal pack and division of the right-sided cartilages facilitated digital control of the bleeding. Following rapid massive transfusions, the bleeding atrial appendage was repaired and the operation completed. Two years later the Steinman pin was removed, and she married at the age of 20. The lessons from this case are obvious: preoperative consideration of this complication; gentle and careful elevation of the sternum; plenty of blood available; and possible cardiac bypass standby support. DR. FRANCIS ROBICSEK Charlotte, N. C.

I want to congratulate Dr. Humphreys for this very nice presentation. He is one of the pioneers and the master surgeon of this field. However, I have a few bones to pick; namely, I really do not believe that once a pectus excavatum diagnosis has been made, the deformity can go away. The fact that 18 patients died below the age of I year suggests to me that the condition probably was really a retraction of the sternum due to respiratory or cardiorespiratory distress, rather than a true pectus excavatum. We usually do not wait until the patient passes the age of 3 years. I like to operate at the age of I year. I believe that if it looks like a pectus at the age of I year, it is a pectus. Also, I would like to caution against operating upon patients older than 20. In my experience, 50% of the patients requesting pectus repair after the age of 20 are psychologically unstable and are not likely to be pleased with the resuits. As far as the radical and nonradical procedures are concerned, Dr. Humphreys' procedure is indeed radical, but not as radical as Dr. Wadas "sternal turnover" operation. I think it is somewhat unfair to compare the results obtained by one excellent surgeon, an expert in the field, with the results of the rest of the institutions using different methods. Regarding the failure rate, I do not think that a 21% failure rate is acceptable in an operation which is more or less cosmetic. At the present time we use a procedure which we have developed in the footsteps of Angelo May, who used a somewhat simi lar technique in 196 I. We do a radical Ravitch procedure, and then we put a Marlex mesh under the sternum to support it. The cartilages are resected, a sternotomy is done, Marlex mesh is put under the sternum, and finally the freed-up pectoralis muscles are united above it. This gives a nice smooth surface. [Slide] This slide shows the operative site. The Marlex mesh is stretched like a drum. The sternum is elevated. It is left to fall back and then covered with the pectoralis muscle.

Volume 80 Number 5 November, 1980

In our experience with the follow-up period up to 4 years, the results were uniformly good. What the next 15 years are going to bring, naturally, will have to be seen. DR. ERIC W. FONKALSRUD Los Angeles. Calif.

I would also like to congratulate Drs. Humphreys and Jaretzki for their excellent presentation and for calling our attention to some of the pitfalls in the management of congenital chest wall deformities. It has become apparent that a final evaluation of the results following surgical repair must extend beyond 10 years, particularly when reconstruction is performed in the preschool age group. Growth during adolescence appears to be accompanied by an accentuation of the chest wall deformity, to some degree even if repair has been performed previously. In our experience, some type of support for the sternum after repair is particularly helpful, as has been emphasized by Drs. Brewer and Robicsek. We have used a stent of periosteum from the lower costal cartilages in younger children, placing this behind the sternum after the osteotomy has been made. This support becomes rigid within 4 weeks and has provided a permanent autologous stent. However, in older children the distance between the osteotomy and the tip of the sternum is longer than in young children, so that there is a much greater posterior force retracting the sternum backward toward the spine when the chest muscles are reapproximated over the repair. In this setting we have found it more feasible to use either a K-wire or other prosthetic material, as Dr. Brewer has indicated. I would like to ask the authors at what age they recommend repair and if they recommend using a prosthesis to minimize recurrences. DR. HUM PH R E Y S (Closing) I would like to thank the discussers for their interest and comments and support. The use of a pin, strut, stent, or wire is very widespread and very popular. As I pointed out in the paper, many surgeons have changed their operative technique from one which they consider unsatisfactory as the patients grow older to one that is more satisfactory, and usually the change is from either

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extemal support or no support to the use of a stent. It may well be that the long-term results of this procedure are better than those of the radical operation that we do without support. However, we have no records of the long-term results, and I am looking forward to hearing from those enthusiasts for internal support as to what their patients look like in their adult life. I would like to emphasize, as Dr. Fonkalsrud just did, that the recurrences usually appear in the rapid growth period of adolescence. I am sure that there are going to be some recurrences, regardless of the technique, in some of these patients because the genetic defect is there. Scoliosis and other complications develop. However, it may be that the internal support will be better than no support. I must confess that I objected to the use of a stent in the beginning because I do not like foreign bodies in the chest, and it does require a second operation to remove the support; especially in small children, this seems to be a poor idea if it can be avoided. Dr. Robiscek, I appreciate your comments. The 18% mortality in the infants was entirely due to associated anomalies, the commonest being congenital heart disease, as was brought out in the paper. The diagnosis at birth or in infancy of course is questionable. Is it really a pectus excavatum or is it simply retraction due to some other disease? This I am in no position to judge. When looking at a chart in which the diagnosis has been made, I simply have to accept it as a diagnosis. Some of these infants eventually required operation-relatively few. In most of them, about 50% of those diagnosed in infancy, the anomaly either disappeared completely or became so minimal as to not necessitate operation. I do believe that pectus excavatum does disappear, even in children past infancy. Actually, 16% did disappear between the ages of I and 6 years, but none disappeared thereafter. I believe the proper age for repair is between 3 and 6 years. However, we do get good repairs even in adults, so long as there is not already a fixed psychosis or psychoneurosis. This is a contraindication to operation, I agree. However, we have several very satisfied patients, the oldest patient now nearing the age of 60.