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4 Repair of pectus excavatum
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rest. Some with severe deformities may have mild restrictive
pulmonary function. Exercise pulmonary function studies in
older children (10–12 years old) who are very athletic have
demonstrated measurable decreases in cardiopulmonary
function. A computed tomographic scan is helpful in the
older patient to evaluate the degree of sternal rotation and
position of the heart. We no longer use preoperative echocardiography in asymptomatic patients. We reserve cardiac evaluation for those patients with elastic cartilage deformities
such as Marfan or Ehlers-Danlos syndrome.
ANESTHESIA
Chest wall reconstruction should be performed under general
anesthesia with or without a thoracic epidural. If an epidural
is used, a urinary bladder catheter is also recommended.
OPERATION
Modified Ravitch repair of pectus excavatum
The general concept of the modified Ravitch operation is
resection of all abnormal costal cartilages. The sternum is displaced by the overgrowth in length of the cartilages and,
therefore, needs only to be fractured on its anterior table to
restore a normal position once it has been freed from the
costal cartilages.
INCISION
A transverse, rather than vertical, incision through the
1
deepest portion of the defect is the most appealing from
a cosmetic perspective.
Sternal notch
Costal
cartilages
Sternum
2
Pectoralis major muscle
Xiphoid
Electrocautery
1
SKIN AND MUSCLE FLAPS
Superiorly and inferiorly based skin flaps at the level of,
2
but not including, the pectoralis major fascia are raised.
Pectoralis muscle flaps are created by dissecting from the
midline to a position lateral enough to expose the costochondral junction. The entire defect may involve ribs 3–8, but
most commonly the anomaly alters cartilages 5–8 bilaterally.
A minimum of four cartilages bilaterally should be excised.
SUBPERICHONDRIAL RESECTION OF THE DEFORMED
Manubrium
Sternum
Perichondrium
Periosteal elevator
Second rib
Sternum
Perichondrium
Costal
cartilage
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CARTILAGES
The perichondrium is incised anteriorly along
3a–c
are created to expose the defective cartilage (Figure 3a).
Meticulous care is needed for the posterior portion of the dissection to avoid entering the pleural space. Each deformed
cartilage is resected sharply from its junction with rib laterally
to the attachment with the sternum (Figure 3b). The perichondrium must be preserved in its entirety because it is from
this tissue that new cartilage and subsequent bone are generated (Figure 3c). Moreover, devascularization of the perichondrium may be a major cause for an acquired thoracodystrophy years later after pectus reconstruction.
each cartilage, and cephalad and caudad flaps
Operation 5
Division of
costal cartilage
3b
3a
3c
6 Repair of pectus excavatum
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MOBILIZATION OF THE STERNUM
The xiphoid is exposed and elevated, and a
4a, b
the finger (Figure 4a). The pleura and pericardium are freed
from the sternum using further finger dissection. The sternum is mobilized by dividing the intercostals and perichondrial bundles from their junction with the sternum (Figure
4b). Detachment begins at the xiphoid and proceeds just
above the highest involved perichondrial bundle.
retrosternal plane is bluntly developed with
Pleura and pericardium
freed from sternum
4a
Costal
cartilage
5
Clavicle
Pleura
45
Sternum
4b
STERNAL ‘TRIPOD’ SUPPORT
The sternum is then lifted into a more neutral position
and supported by the lowest of the normal costal carti-
5
lages. Subperichondrial exposure of this normal cartilage is
performed bilaterally. This normal cartilage is divided
obliquely (45 degrees) from medial to lateral so the medial
portion (anterior) sits atop the lateral (posterior). To complete the tripod support of the sternum, the medial segment
of cartilage is then sutured atop the lateral half.
A single oblique or transverse wedge osteotomy of the
Wedge osteotomy
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6
anterior table of the sternum facilitates resolving the
rotation of the sternum and adds to its support. Occasionally,
a second anterior table osteotomy is required. A wedge of
bone is removed, and the periosteum of the sternum is then
sutured to further secure the sternum in its neutral position.
Operation 7
7
Stainless steel strut
6
For children older than 10–12 years, or those with elastic
7
cartilage abnormalities (Marfan syndrome, EhlersDanlos syndrome), additional sternal support is recommended with a substernal stainless steel bar placed beneath
the distal third of the sternum and secured to the ribs.
Detached intercostal bundles need not be sutured to the sternum. If the distance between the sternum and resected
bundle is no more than 2–3 cm, however, the defect may be
closed with approximation of these tissues.
8 Repair of pectus excavatum
Pectoralis major
approximated
Substernal drain
Subcutaneous drain
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WOUND CLOSURE
A substernal drain is positioned to include decom-
8
pression of any violated pleural space. Pectoralis flaps are
closed over the sternum. A subcutaneous drain is inserted.
Finally, skin flaps are reapproximated with subcuticular
sutures.
POSTOPERATIVE CARE
Perioperative antibiotics are discontinued at 24 hours.
Incentive spirometry is vital to avoid atelectasis and possible
pneumonia. Urinary retention is common in the teenager
and older patient. Therefore, a bladder catheter is recommended for postoperative days 1 and 2. Costal cartilages
regenerate within 6–8 weeks. Contact sports should be
avoided during this regenerative interval. In children older
than 10–12 years who undergo strut placement via the modified Ravitch repair, the strut is usually removed in 6–8
months.
OUTCOME
All reconstructions should yield a satisfactory cosmetic result.
Modest physiological benefit can be documented in athletic
teenagers at the peak of their fitness. In other cases, additional
studies have demonstrated no physiological effect. Recurrence rates are reported as less than 5%. Recurrence
seems to be more prevalent if the operation was done at a very
early age (younger than 5–7 years) or during the older teen
years (15–17 years). An acquired form of Jeune syndrome
(thoracic chondrodystrophy) has been described in children
repaired younger than 3 years old. These children underwent
8
extensive resection of the chest wall such that cartilage and
perichondrium were devascularized. This problem is not a
recurrence of the defect but rather represents a failure of the
chest wall to grow. Over time, no growth occurred in the
resected areas, which led to the chondrodystrophy. A floating
sternum has also been described. In this condition, cartilage
is resorbed, which leaves a fibrous or unstable connection between the sternum and ribs. The unstable sternum
must be supported by struts to facilitate stable chest wall
dynamics.
OPERATION
Modified minimally invasive repair of pectus
excavatum (Nuss procedure)
A number of modifications have been made to the Nuss procedure since it was introduced in 1998. However, the basic
concept is unchanged. A substernal bar is used to elevate the
sternum and reposition the deformed costal cartilages. We
use a modified Nuss procedure at the Johns Hopkins
Children’s Center. Currently, with the exception of a large
bone hook and in situ bar benders, all equipment necessary
for this reconstruction is supplied by Walter Lorenz Surgical
in Jacksonville, Florida.
Operation 9
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INCISION AND SKIN FLAPS
The patient should be positioned with arms abducted to
9
allow access to the lateral chest wall. Placement of the
skin incisions depends on the geography of the defect. If the
defect is very low, care must be taken not to position the bar
under the xiphoid. Similarly, in the older teenager the defect
may extend to cartilages 3 and 4, which thus necessitates
more superior chest wall incisions. In general, two 2.5- to 4.0cm lateral thoracic incisions are made, each beginning below
the nipple and extending laterally. The distance below the
nipple is determined by which interspace the bar will traverse.
Skin flaps are mobilized to include one intercostal space
above and below the incision. Sufficient mobilization is necessary to allow for placement of bar stabilizers (see Figure 15).
Confirming preoperative measurements
using a Pectus Bar Template
Patient positioned with
both arms abducted
9
10
TEMPLATE MEASUREMENTS
A bar template is used to determine the appropriate
10
each of the lateral chest wall wounds and bent to the arc of the
desired repair. The templates are numbered to facilitate use of
the corresponding bar. We recommend overcorrection with a
more angular arc, because the sternum invariably rests atop
the bar in a position somewhat lower than that which the surgeon perceives. The segment of the bar directly underneath
the sternum should be kept as flat as possible, however, to
maintain as much sternal surface area in contact with the bar
as possible. This maneuver facilitates a sturdy substernal support. In addition, the left side of the bar is not bent to the
extent that the right side is conformed to the shape of the
template. This maneuver facilitates easier passage of the bar
through the substernal tunnel.
Lorenz bar. The template should be positioned in
10 Repair of pectus excavatum
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HEMITHORAX DISSECTION
The chest is entered, using electrocautery, in the
11
tion for sternal support. Typically, this position is at one
intercostal space above the deepest portion of the defect. Each
hemithorax must be entered in the area where the ribs begin
to move from anterior to lateral chest wall. This point usually
corresponds to the anterior axillary line. Next, a subxiphoid
skin puncture is made. A bone hook is inserted into the sternum or ribcage immediately adjacent to the xiphisternal
junction through this skin puncture. With the bone hook, the
sternum is elevated anteriorly. This maneuver increases the
distance between the sternum and pericardium, and facilitates safe substernal passage of the Crafoord clamp.
intercostal space corresponding to the optimal posi-
Sub-xiphoid skin puncture
Bone hook used to elevate sternum
and anterior chest
11
PASSAGE OF SUBSTERNAL CLAMP
Through the left-side incision in the intercostal
12
plane to a point as far medially as possible. The Crafoord
clamp is passed from the right hemithorax and advanced
until it is felt by the fingertip passed from the left hemithorax.
Throughout this maneuver, the sternum is elevated by using
the bone hook. Once the Crafoord clamp is advanced
through to the left hemithorax hole, two umbilical tapes are
caught and pulled retrograde through the tunnel. Some centers use a thoracoscope to doubly ensure safe passage of the
Crafoord clamp.
space, finger dissection can be used in a substernal
Crafoord clamp passed from right to left in asubsternal plane
12
INSERTION OF PECTUS BAR
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The appropriate, previously bent Lorenz bar is tied to
13
to left of the hemithorax in a concave-up position. Again, the
anterior chest is elevated using the bone hook to ensure safe
passage through the tunnel. Once the left side of the bar is
through the chest, it can be bent to conform to the left side of
the template.
one of the umbilical tapes and passed from the right
Operation 11
Lorenz bar passed from right to left
13
BAR FLIPPING AND APPLICATION OF STABILIZERS
The bar (concave up) is now flipped from a cranial to
14
the reverse direction because the xiphoid can be injured and a
less than optimal substernal support created. The bar is held
in this position (concave down), and the surgeon immediately observes the desired position of the sternum and anterior chest. Bilateral bar stabilizers should be fastened to the
bar.
caudal direction. The bar should never be flipped in
14
12 Repair of pectus excavatum
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Stabilizers
IN SITU
15
of the vertical stabilizers. This step is a very important in the
procedure because it ensures additional bar support.
BAR BENDING AND FIXATION
In situ bar bending now conforms the bar to the
ribcage. Bar bending is usually required on each side
In-situ bar bender used to contour bar to
shape of lateral chest wall, used medial
and lateral to bar stabilizer
15
16
Rib
Bar
Doyen
The bar is wired (No. 5 gauge) to the rib that it
16
the stabilizers using a tapered Doyen with a hole in the tip.
crossed over on each side of the chest just medial to
Further reading 13
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WOUND CLOSURE
Subcutaneous tissue is closed in the presence of 35–40 cm of
peak inspiratory pressure. This maneuver facilitates removal
of air contained in each hemithorax. A small red rubber
catheter can also be inserted into each hemithorax and
removed at the completion of wound closure. Last, the skin is
closed.
POSTOPERATIVE CARE
A chest radiograph is obtained in the recovery room.
Generally, 20–30% pneumothorax is well tolerated and does
not require tube decompression. As with the Ravitch repair,
older patients may require insertion of a Foley catheter.
Despite a more minimal chest wall dissection, duration of
narcotic requirement is longer than with the modified
Ravitch repair. No contact sports are allowed for 8 weeks.
Afterward, return to full activities is permitted. Children
younger than 12 years may undergo bar removal at 2 years,
whereas older teenagers, adolescents, and adults may require
bar stabilization for 3–4 years.
OUTCOME
Complications include flipped bar, pneumothorax, pleural
effusions, and chest wall asymmetry; one episode of cardiac
puncture has been reported. Long-term results have yet to be
determined. A significant learning curve is associated with the
introduction of this new technique. Thoracoscopy or finger
dissection and displacement of the sternum using a bone
hook such as described herein have facilitated bar insertion.
In situ bar bending and wiring have prevented flipping and
displacement. Although published series show that this procedure is best performed between the ages of 8 and 12, we
have used this modified technique (n = 65) at the Johns
Hopkins Children’s Hospital for adolescents and adults with
excellent early results (age range, 8–42 years). We have also
used the modified Nuss procedure for reoperation after a
Ravitch-type repair (n = 6) with similar excellent early results.
FURTHER READING
Haller J. History of the operative management of pectus deformities.
Surgical treatment of anterior chest wall deformities. Chest Surgery
Clinics of North America 2000; 10: 227–35.
Haller J. Complications of surgery for pectus excavatum. Surgical
treatment of anterior chest wall deformities. Chest Surgery Clinics of
North America 2000; 10(2): 415–26.
Haller JA, Loughlin GM. Cardiorespiratory function is significantly
improved following corrective surgery for severe pectus excavatum.
Journal of Cardiovascular Surgery 2000; 41: 125–30.
Hebra A, Swoveland B, Egbert M, et al. Outcome analysis of minimally
invasive repair of pectus excavatum: review of 251 cases. Journal of
Pediatric Surgery 2000; 35: 252–8.
Morshuis W, Folgering H, Barentsz J, Van Lier H, Lacquet L. Pulmonary
function before surgery for pectus excavatum and at long-term
follow-up. Chest 1994; 105: 1646–52.
Nuss D, Kelly RE, Croitoru DP, Katz ME. A 10-year review of a minimally
invasive technique for the correction of pectus excavatum. Journal
of Pediatric Surgery 1998; 33: 545–52.
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