Pectus Excavatum Example Journal PDF

Download as pdf or txt
Download as pdf or txt
You are on page 1of 6

ANNALS OF SURGERY

Vol. 231, No. 3, 443 448


2000 Lippincott Williams & Wilkins, Inc.

Repair of Pectus Excavatum Deformities:


30 Years of Experience With 375 Patients
Eric W. Fonkalsrud, MD, James C.Y. Dunn, MD, and James B. Atkinson, MD
From the Department of Surgery, UCLA School of Medicine, Los Angeles, California

To review the surgical experience with pectus excavatum


chest deformities at UCLA Medical Center during a 30-year
period.

lages, transverse wedge osteotomy of the anterior sternum,


and internal support with a steel strut for 6 months. Repair
was performed on 177 children before age 11 years; 38
adults with severe symptoms underwent repair.

Background

Results

Objective

Pectus excavatum is a relatively common malformation that is


often symptomatic; however, childrens physicians often do
not refer patients for surgical correction.

Methods
Hospital records from 375 patients who underwent repair of
pectus excavatum deformities between 1969 and 1999 were
reviewed. Decrease in stamina and endurance during exercise was reported by 67%; 32% had frequent respiratory infections, 8% had chest pain, and 7% had asthma. The mean
pectus severity score (width of chest divided by distance between posterior surface of sternum and anterior surface of
spine) was 4.65 (normal chest 2.56). All patients had
marked cardiac deviation into the left chest. Repair was performed with subperiosteal resection of the abnormal carti-

Congenital chest wall deformities have long been considered as curious and essentially unimportant anomalies of
thoracic contour. During the past several years, however,
pectus malformations have come to be recognized as fairly
common and much more symptomatic than previously believed. Pectus excavatum is by far the most common congenital chest deformity, occurring in approximately one in
every 700 births (personal communication, March of Dimes
Birth Defects Foundation, March 1995), but the recognition
of symptoms and the recommendation for surgical correction remain highly controversial: less than 15% of patients
undergo surgery. Few medical conditions arouse such opin-

Correspondence: Eric W. Fonkalsrud, MD, Dept. of Surgery, UCLA Medical Center, Los Angeles, CA 90095.
Accepted for publication June 25, 1999.

The mean hospital stay was 3.1 days. With a mean follow-up
of 12.6 years, all patients with preoperative respiratory symptoms, exercise limitation, and chest pain experienced improvement. Vital capacity increased 11% (mean) within 9
months in 35 patients evaluated. There were no deaths.
Complications included hypertrophic scar formation (35), atelectasis (12), pleural effusion (13), recurrent sternal depression
(5), and pericarditis (3). More than 97% had a very good or
excellent result.

Conclusion
Pectus excavatum deformities can be repaired with a low rate
of complications, a short hospital stay, and excellent longterm physiologic and cosmetic results.

ionated views from physicians as does the discussion regarding whether pectus excavatum is primarily a cosmetic
disorder, or whether it causes physiologic impairment and
limitations to the patient. The timeliness of this controversy
has been accentuated in recent years as the insurance coverage for many medical conditions has been restricted by
managed health care.
Compounding the decision of whether to correct pectus
excavatum disorders by surgery is the lack of reliable and
consistent standard investigative studies that clearly indicate
the physiologic limitations on a specific patient by pectus
excavatum, or the improvement that occurs after surgery.
Because only a few hospitals have compiled a large surgical
experience, and because most surgeons perform only a few
pectus operations each year using a variety of surgical
techniques, the reported results have been inconsistent, fur443

444

Fonkalsrud and Others

Table 1.

Ann. Surg. March 2000

AGE AT PECTUS EXCAVATUM


REPAIR

Age Range
26
611
1116
1620
20

No. of Patients
102
75
87
73
38

47%

375

ther confusing the decision of whether to correct the deformity.


The present report reviews the surgical experience with
pectus excavatum chest deformities at UCLA Medical Center during a 30-year period.

PATIENTS AND METHODS


The medical records of all patients who underwent surgical correction of pectus excavatum deformities at UCLA
Medical Center from January 1969 through November 1999
were reviewed. Three hundred seventy-five patients were
identified. During the same period, an additional 76 patients
with pectus carinatum and 4 with Polands syndrome underwent surgical repair. There were 298 male patients and
77 female patients (21%). The deformity was evident during
the first few months of life in 88% of patients. A family
history of pectus deformity was present in 42% of patients.
The age at surgery ranged from 2.5 to 53 years (Table 1).
Surgical repair was performed on 224 patients during the
past 10 years. Three hundred thirty-six of the operations
were performed by one surgeon. The period of follow-up
has extended from 3 months to 30 years (mean 12.6 years).
The most common complaint for young patients was
related to the unattractive physical appearance of the deformity. Mild to severe exercise limitation with decreased
stamina and endurance and inability to keep up with peers in
strenuous athletic activities was reported by 251 patients
(67%), most frequently in those older than 6 years; it was
most severe in those older than 16 years. Many had been
able to participate in competitive athletic activities during
early adolescence but then found it progressively more
difficult to keep up with peers. One hundred twenty patients
(32%) had frequent respiratory infections; 26 (7%) had
asthmatic symptoms, which were often severe after exercise. Functional heart murmurs were present in 90 patients
(24%); however, only 13 patients had cardiac anomalies
(Table 2). Two adult patients had mild to moderate angina.
Forty-eight of the older patients experienced aching or
compression-type pain in the lower anterior chest after
exercise, with three having pain even at rest. Sixty patients
(16%) had mild or moderate scoliosis; three patients had

severe spinal deformities. Two patients had Marfan syndrome. Other anomalies were present in 26 patients (7%).
Almost all patients showed a narrow anterior-posterior
diameter of the chest; only two patients were slightly overweight. Thirty-nine of the 55 patients tested had electrocardiographic evidence of right ventricular strain, and 30 (8%)
had mitral valve prolapse. Almost all patients had displacement of the heart into the left chest. The mean pectus
severity score as determined on chest x-ray or computed
tomography scan by measuring the internal width of the
lower chest and dividing by the distance between the posterior surface of the sternum and the anterior surface of the
spine, was 4.65 (range 3.54 9.50); the normal chest is 2.56
(Fig. 1).1 There was a tendency for patients with a high
pectus severity score to have the most severe symptoms.
Approximately 15% of the patients, largely those younger
than 6 years, had minimal measurable physiologic impairment.
The surgical technique used for each of the 375 patients
was a modification of that described by Ravitch2 and
Welch,3 and has been extensively detailed in previous reports.4,5 The repair of pectus excavatum includes the following essential features:
1. A transverse curvilinear incision is made midway
between nipples and costal margin, extending from
mid-nipple line bilaterally.
2. Limited skin flaps are elevated over the pectoralis
muscles using needle-point electrocautery to minimize blood loss.
3. The pectoralis muscles are reflected laterally over a
short distance from attachments to the sternum and
deformed costal cartilages; the abdominal muscles
are mobilized from the lower costal cartilages.
4. The perichondrium is incised on the midanterior surface of the lower four to five costal cartilages bilaterally, extending from the costochondral junction to
the sternum.
5. Abnormal costal cartilages are resected subperichondrially, carefully preserving the perichondrium.
6. The xiphoid is detached from the sternum.
7. The intercostal muscles and perichondrial sheaths of
the resected cartilages are transected from the sternum.
8. The lower retrosternal space is mobilized.

Table 2.

ASSOCIATED DISORDERS

Functional heart murmur


Scoliosis
Mitral valve prolapse
Congenital heart disease
Marfan syndrome
Other anomalies
Total 375 patients.

90 (24%)
60 (16%)
30 (8%)
13 (3%)
2 (0.5%)
26 (7%)

Repair of Pectus Excavatum Deformities

Vol. 231 No. 3

445

Figure 1. Chest x-rays from a 15year-old girl with severe symptoms


from pectus excavatum. Note the
short distance between the posterior surface of the sternum and the
anterior surface of the spine. The
pectus severity score was 9.5 (normal chest 2.56).

9. The pleura is incised on the right side of the mediastinum and a small chest tube is inserted.
10. A transverse anterior wedge osteotomy of the sternum is made at the level where the sternum depresses
posteriorly.
11. The posterior table of the sternum is gently fractured
without displacement and then elevated and twisted
to the desired position.
12. Nonabsorbable sutures are placed through the anterior table of the sternum across the osteotomy.
13. A stainless-steel (Adkins) strut (Baxter Healthcare
Corp., Operating Room Division, McGaw Park, IL)
is placed across the lower anterior chest to support
the tip of the sternum and is wired to the appropriate
rib on each side (fifth or sixth).6
14. The xiphoid and perichondrial sheaths are sutured
back to the sternum. The perichondrial sheaths are
closed loosely.
15. The pectoralis and abdominal muscles are sutured
together over the sternum.
16. Thorough hemostasis is achieved with needle-point
electrocautery, and the wound is copiously irrigated
with cefazolin solution.
17. The skin is closed with subcuticular absorbable sutures and Steri-Strips or staples.
The endotracheal tube is removed in the recovery room
within 2 hours. The chest tube is removed within 24 hours
after surgery. Intravenous cefazolin is given for 3 days, and
oral cephalexin is given for 3 additional days. Postoperative
pain was remarkably mild in all patients and was controlled
with intravenous analgesics for the first 2 postoperative days
and by oral medications thereafter. Epidural analgesia was
not used.
During the past 15 years, the mean duration of the operation has been 2.7 hours (2.2 hours for patients younger than
12 years, 3.2 hours for older patients). Only the infrequent
patient with cardiac anomalies has been placed in an intensive care unit after surgery during the past 18 years. The

total hospital stay rarely exceeded 4 days (mean 3.1 days);


many children younger than 10 years were discharged
within 48 hours. Blood loss rarely exceeded 100 mL (mean
94 mL). The steel strut was removed on an outpatient basis
approximately 6 months after repair with the patient under
light general anesthesia (mean operating time 22 minutes).
The majority of patients returned to school or work within
2 weeks. Full physical activity, including strenuous exercise
but not body contact sports, was resumed by almost all
patients within 10 weeks.

RESULTS
Each of the 251 patients with preoperative limitation in
stamina and endurance with exercise experienced marked
improvement within 4 months after surgery, and most were
able to participate in vigorous exercise, including running,
swimming, hiking, basketball, and tennis, before removal of
the sternal bar. Body contact sports, including football, were
resumed after removal of the sternal bar. Of the 120 patients
with preoperative respiratory symptoms, 115 had a decrease
in frequency and severity of pulmonary infections after
repair. Twenty-four of the 26 patients with asthmatic symptoms showed clinical improvement after surgery, as evidenced by fewer episodes of wheezing and a 25% to 40%
decrease in requirement for medications. Each of the 48
patients with chest pain reported considerable improvement
within 3 months. Thirty-three of the 35 patients who underwent preoperative measurement of vital capacity with an
incentive spirometer experienced improvement within 6
months (mean improvement 11%). Although objective measurements of physiologic improvement after surgery are not
available for all patients, almost all showed a shifting of the
heart from the left chest to a normal position on chest
radiograph within a few weeks (Figs. 2 and 3). Functional
heart murmurs were no longer audible in 74 of the 90
patients.
Postoperative complications included wound seroma in

446

Fonkalsrud and Others

Ann. Surg. March 2000

Figure 2. (A) Chest of a 16-yearold boy with symptomatic pectus


excavatum. (B) Nine months after
surgery.

12 patients, pleural effusion in 13 patients, atelectasis or


pneumonitis in 12 patients, and unintentional pneumothorax
in 6 patients. Pericardial effusion occurred in three older
patients with severe depression (severity index 6.0);
pericarditis developed in two of them, and they were given
indomethacin. Recurrent depression occurred in five of the
early patients in whom a sternal support bar was not used;
three of them underwent a secondary repair. Protrusion of
the second or third costal cartilage developed in 21 preadolescent patients within 6 years after surgery at the time of
pubescent skeletal growth; however, only 5 patients underwent late resection of additional cartilage on an outpatient
basis. Mild to moderate hypertrophy of the cutaneous scar
occurred in the wounds of 35 patients. It is our current
practice to inject triamcinolone solution (10 mg/mL) into
the dermis at the time of pectus repair, or into the scar when

hypertrophy becomes apparent. Only seven patients reported mild or moderate discomfort from the sternal bar.
There were no deaths within 12 months after surgery.

DISCUSSION
Symptoms from pectus excavatum are recognized infrequently during early childhood, apart from an unwillingness
to go without a shirt while swimming or to participate in
other athletic or social activities. Most patients are therefore
advised by well-meaning family physicians or pediatricians
that the deformity will improve with age, that it will not
affect heart or lung performance, that it is primarily a
cosmetic problem, and that surgical repair is dangerous,
minimally effective, and unnecessary. It is clear that each of
these views is incorrect with our present knowledge of

Figure 3. (A) Chest of an 8-yearold girl with symptomatic pectus


excavatum. (B) Fourteen months
after surgery.

Vol. 231 No. 3

pectus excavatum deformities and the current techniques for


surgical repair based on a large clinical experience with
long-term follow-up.4,7 The severe compression of the heart
noted in the majority of patients with a pectus severity index
of more than 4.4 is clearly more than a cosmetic deformity.
Easy fatigability and decreased stamina and endurance
became apparent during early adolescence when the child
participated in more vigorous physical activities. Many adolescent patients chose sedentary activities, such as working
with computers, rather than athletics. For all patients undergoing repair, the heart was considerably displaced into the
left chest and pulmonary expansion during inspiration was
confined. Some very competitive children have been able to
compensate for the pectus-induced limitations in chest expansion in short-duration athletic activities by wider diaphragmatic excursions, at a greater expense of energy.
Phrenic excursions during respiration, particularly while
exercising, were often much greater than in normal children
to compensate for the limited movements of the bony thorax. Most of the patients had an asthenic habitus, a narrow
anterior-posterior chest diameter, and a slouching posture.
Deep inspirations accentuated the severity of the depression
in almost all patients. None of the patients who were followed up before surgery for more than 3 years showed any
evidence of spontaneous regression of the deformity; on the
contrary, almost all patients showed a progressive worsening. Younger children commonly had a potbelly appearance, accentuated by flaring of the lowest costal cartilages.
Approximately 35% of patients, most often in the preadolescent age group, had a symmetric depression involving
the lower 70% of the sternum. The remaining patients had
an asymmetric depression, with the sternum tilted posteriorly on the right and the involved costal cartilages being
more depressed on the right, occasionally extending almost
up to the clavicle. Some of these patients had protrusion of
a few costal cartilages on the left side.
During the past 12 years, we have been consulted by
several patients older than 20 years who have severe untreated pectus excavatum deformities with worsening symptoms and limitations, and who are very desirous of having
surgical correction. Thirty-eight of these patients underwent
successful repair.8 Although the technical aspects of repair
were more tedious than in children, the postoperative recovery and the long-term results have been similar to those
in younger patients.
Objective measurements to document the severity of the
physical limitations caused by pectus excavatum are often
difficult to obtain in children, imprecise, and confusing. No
consensus has been achieved during the past few decades
regarding the degree of cardiopulmonary impairment that
occurs with this common deformity, and how much improvement results after surgical repair.9 Studies conducted
almost three decades ago using cardiac catheterization before and after pectus repair showed significant improvement
in stroke volume and cardiac output.10 Using cycle ergometry testing, Cahill et al11 showed that children with severe

Repair of Pectus Excavatum Deformities

447

pectus deformities had an abnormally elevated work of


breathing, and that there was a significant improvement in
maximum oxygen uptake and total exercise time in 16
children tested before and after repair. A comprehensive
review of many published reports indicating improvement
in cardiac or respiratory function after pectus repair is
provided by Shamberger and Welch.12 Because of the high
expense and often invasive nature of the procedures used for
physiologic evaluation before and after pectus excavatum
repair, we have not favored the routine use of such tests for
many years. The severity of the pectus depression can be
easily measured by calculating the pectus severity index
from computed tomography scans, as recommended by
Haller et al.1 In the present study, 186 patients had the
pectus severity index calculated from chest x-rays, which
appears to be accurate and less expensive than computed
tomography scans.
In our early experience, for children younger than 6
years, we used an internal autologous tissue substernal
support by suturing the perichondrium of the fifth ribs from
each side together posterior to the sternum.13 We discontinued this procedure 16 years ago when we observed mild
recurrent depression in three patients, one of whom experienced mild constriction of the fifth ribs bilaterally. We
have not observed the appearance of the constrictive asphyxiating thoracic dystrophy reported by Haller et al,14 and
more recently by Weber and Kurkchubasche,15 in any of the
375 patients who underwent surgery.
Although we have used the substernal support bar for
adolescent patients for 30 years, we have included the use of
the strut for patients of all ages during the past 12 years. The
sternal support bar eliminates postoperative flail chest and
paradoxical respiration, which reduces pain and permits
early ambulation and deeper respiratory excursions, ultimately reducing the length of hospital stay and overall cost.
The bar is easily removed and ensures a good long-term
results: recurrent depression is extremely uncommon, even
when repair is performed at an early age. Costal cartilage
regeneration from the perichondrial sheaths is remarkably
rapid in children, with the chest becoming very stable within
4 weeks. For adults, the costal cartilages are usually brittle
and thick and often must be scooped out from the perichondrial sheaths with a rongeur rather than being stripped out
with a smaller elevator. Cartilage regeneration in adults has
been slightly slower than in children, although all patients
had a stable chest within 8 weeks. Removal of the support
bar approximately 6 months after repair has not resulted in
recurrent depression in any of the patients. We prefer to
perform surgical repair in the preadolescent age group in
patients who have moderate symptoms and a high pectus
severity index because the operation is technically easier at
this age, taking approximately 1 hour less than in adolescents.
Although the surgical technique used in the present review
has provided excellent results in more than 97% of patients
with a short hospital stay (mean 3.1 days), we are awaiting

448

Fonkalsrud and Others

with enthusiasm the long-term results with the revolutionary


new concept of minimally invasive pectus excavatum repair
recently described by Nuss et al.16 The current experience of
these authors with 40 patients who underwent bar removal 2 or
more years after the repair with relatively short follow-up is
very gratifying (personal communication, Kelly RE Jr, Nuss
D). Longer follow-up, however, with a larger group of patients
progressing through the adolescent period of rapid skeletal
growth will be necessary to determine the frequency of complications and the recurrence rate. Whether the minimally
invasive technique will be effective in correcting deformities in
older patients or in those with combined depression and protrusion deformities remains to be determined. Pain appears to
be more severe and prolonged, complications are more frequent and severe, the hospital stay is longer, and the limitation
of physical activity longer than for the patients reviewed in the
present report.
To obtain optimal results, repair of pectus excavatum requires attention to several technical details, with small alterations made on the basis of different anatomic features in a
specific patient. As noted with other major operations, the
number of repairs performed by a surgical team may have a
close relation with the complication rate and the outcome.17
This retrospective clinical study confirms that pectus
excavatum deformities can be repaired with a low rate of
complications and a short hospital stay. The improvement in
respiratory symptoms, exercise tolerance, and endurance as
well as cosmetic appearance of more than 97% of the
patients in this study support the view that symptomatic
patients of all ages should undergo repair, preferably during
preadolescent years. Routine use of substernal support with
minimal preoperative testing has provided excellent longterm clinical results at low cost.

References
1. Haller JA Jr, Kramer SS, Lietman SA. Use of CT scans in selection of
patients for pectus excavatum surgery: a preliminary report. J Pediatr
Surg 1987; 22:904 906.

Ann. Surg. March 2000

2. Ravitch MM. Operative technique of pectus excavatum repair. Ann


Surg 1949; 129:429 444.
3. Welch KJ. Satisfactory surgical correction of pectus excavatum deformity in childhood: a limited opportunity. J Thorac Surg 1958; 36:697
713.
4. Fonkalsrud EW, Salman T, Guo W, et al. Repair of pectus deformities with sternal support. J Thorac Cardiovasc Surg 1994; 107:
37 42.
5. Fonkalsrud EW. Chest wall deformities. In: Rowe MI, ONeill JA,
Grosfeld JL, Fonkalsrud EW, Coran AG, eds. Essentials of Pediatric
Surgery. St. Louis: CV Mosby; 1995:383391.
6. Adkins PC, Blades B. A stainless steel strut for correction of pectus
excavatum. Surg Gynecol Obstet 1961; 113:111113.
7. Haller JA Jr, Scherer LR, Turner CS, et al. Evolving management of
pectus excavatum based on a single institutional experience of 664
patients. Ann Surg 1989; 209:578 583.
8. Fonkalsrud EW, Bustorff-Silva J. Repair of pectus excavatum and
carinatum in adults. Am J Surg 1999; 177:121124.
9. Shamberger RC. Congenital chest wall deformities. Curr Prob Surg
1996; 33:469 552.
10. Beiser GD, Epstein SE, Stampfer M, et al. Impairment of cardiac
function in patients with pectus excavatum, with improvement after
operative correction. N Engl J Med 1972; 287:267272.
11. Cahill JL, Lees GM, Robertson HT. A summary of preoperative and
postoperative cardiorespiratory performance in patients undergoing
pectus excavatum and carinatum repair. J Pediatr Surg 1984; 19:430
433.
12. Shamberger RC, Welch KJ. Cardiopulmonary function in pectus excavatum. Surg Gynecol Obstet 1988; 166:383391.
13. Fonkalsrud EW, Follette D, Sarwat AK. Pectus excavatum repair using
autologous perichondrium for sternal support. Arch Surg 1978; 113:
14331437.
14. Haller JA, Colombani P, Humphries C, et al. Chest wall constriction
after too extensive and too early operations for pectus excavatum. Ann
Thorac Surg 1996; 61:1618 1625.
15. Weber TR, Kurkchubasche AG. Operative management of asphyxiating thoracic dystrophy after pectus repair. J Pediatr Surg 1998; 33:
262265.
16. Nuss D, Kelly RE Jr, Croitoru DP, et al. A 10-year review of a
minimally invasive technique for the correction of pectus excavatum.
J Pediatr Surg 1998; 33:545552.
17. Harmon JW, Tang DG, Gordon TA, et al. Hospital volume can
serve as a surrogate for surgeon volume for achieving excellent
outcomes in colorectal resection. Ann Surg 1999; 230:404 413.

You might also like