Download Surgical Correction of Congenital Chest Wall Deformities

Surgical Correction of Congenital Chest Wall Deformities:
Technique & Early Experience in Kasr El Aini Hospital
Mahmoud El Battawy MD, Ayman Gado MD, Ahmed Abul Azm MD, Mohammed Swailam
MD, * Mohammed Khairy MD.
Departments of Cardiothoracic Surgery of Faculty of Medicine, Cairo University; and Benha University *.
Abstract
Background: We herewith describe the technique we used in the one-setting surgical correction
of different types of congenital chest wall (sternal or rib deformities): deficient ribs or sternum;
pectus excavatum, and the pectus carinatum defects.
Patients and Methods: Our study was prospectively-undertaken from 2003 till 2006, in Kasr El
Aini University Hospitals, Abul Reesh National Insurance Hospital for Students, and Benha
University. It enrolled 12 patients (7 males & 5 females) having 3 types of congenital sternal
deformities: Cleft or Deficient Sternum (DS) (5 patients, 41.6 %); Pectus excavatum (PE) (6
patients, 50%); and Pectus carinatum (PC) (1 patient, 8.3 %). Our youngest patient - at the time of
surgical correction - was a 4 months-old male baby with deficient sternum; while the oldest was a
15-years old young man with pectus carinatum. The mean age of our patients was 4 years ± 5.5
months (range 4 months - 15 years). The main presenting complaint in the pectus cases was
recurrent refractory episodes of chest infection; while in the deficient sternum patients, was fear of
possible trauma to the exposed heart. Follow-up period was done for all patients for a whole year
postoperatively by means of clinical examination and questionnaire questions to the patient (or his
or her relatives) asking them to share in the patient’s evaluation in comparison with the original
complaint stating their opinion in grades as : excellent, good, fair (or accepted), and poor.
Results: We had no mortality. No significant intraoperative morbidities were found. Blood loss
was minimal and no transfusion was required. Postoperatively, transient mild unilateral seroma
below the pectoralis muscle (needed a 5-days vacuum drainge) was noticed in 1 patient (8.3 %);
mild Chest wall pain (ameliorated by oral and local analgesia) in 1 patient (8.3 %); and lag of cartilage
regeneration leading to small anterolateral soft space (managed with increased oral calcium intake)
in another patient (8.3 %). No other types of morbidity occurred eg: blood loss needing transfusion;
Cardiac arrhythmias; or recurrence of sternal depression (pectus cases) . Postoperative hospital stay time
was relatively-short with a mean of 8 ± 3.5 days (range 4 -13 days). According to the patient’s
(and or child’s parents) words, the postoperative functional results were described as : excellent in
6 patients (50 %); good in 4 patients (33.3 %); and acceptable in 2 patients (16.6 %). Generallyspeaking, patients and or their parents accepted the surgical results quite well and reported an
obvious decline in the frequency of chest infections, and a favorable improvement in morals
together with an increased tolerance for prolonged physical activity.
Conclusion: Our immediate and short-term results showed that marked congenital sternal
deformities should be surgically-corrected once diagnosed after stabilization of the patient’s
clinical condition. The technique(s) we used achieved adequate stabilization with a sound degree
of patient safety. The postoperative results were satisfactory and acceptable for surgeons, patients,
and their relatives. Longer follow-up results are still awaited.
Abbreviations & Key Words: CSD: Congenital sternal deformities PE: Pectus Excavatum. DS: Deficient (Cleft) Sternum. PC:
Pectus Carinatum. ASD: Atrial Septal Defect. VSD: Ventricular Septal Defect..TGA: Transposition of Great Arteries. IHSS: Idiopathic
Hypertrophic Subaortic Stenosis. PS: Pulmonary Stenosis. TAPVD: Total Anomalous Pulmonary Venous Drainage. TOF:
Tetralogy Of Fallot.
Introduction :
The pectus deformity (pectus excavatum and pectus carinatum) are the two most common
congenital chest wall deformities in the pediatric age group (1), occurring in approximately 1:1000
and 1:5000 births, respectively (2),(3). Ravitch reported that pectus excavatum may occur as
frequently as 1 in 300-400 live births, and that it is rare in blacks (4). Pectus deformities tend to
occur more frequently in boys than girls, by almost a 4:1 ratio (5).
With an ambiguous etiology that has not been clearly elucidated, pectus deformities was claimed
to link to genetic factors with incidence rate reaching up to 37 % in predisposed families (6). A high
incidence was reported also in children with Marfan Syndrome (4), or those having developmental
anomalies of the diaphragm (which tethers the sternum posteriorly)(6). This theory was supported
by the occurrence of pectus excavatum in children after repair of agenesis of the diaphragm, and
the frequent association of pectus excavatum and congenital diaphragmatic hernia (6). Pectus
excavatum can be also seen in association with abdominal muscles’ deficiency “Prune-Belly
Syndrome”, Myopathies, certain Chromosomal Defects (eg: Turner Syndrome), or Congenital
Cardiac Diseases (ASD, VSD, Dextrocardia, TGA, IHSS, Aortic regurgitation, Ebstein Anomaly,
TAPVD, TOF, Tricuspid atresia, etc..) (7).
Sternal defects are rare compared with pectus excavatum and carinatum, yet they have received a
great deal of attention in the medical literature because of their dramatic presentation and often
lethal outcome. Deformities resulting from failure of ventral fusion of the sternum can be divided
into 4 groups: (1) Cleft Sternum (2) Thoracic Ectopia Cordis (3) Thoracoabdominal ectopia cordis
(4) Cervical Ectopia Cordis. The heart is in a normal position in cleft sternum but is displaced in
the other three entities. In the thoracic ectopia, the heart protrudes anteriorly and there are no
tissues covering the heart. In cervical ectopia, the protrusion is even more pronounced and the
heart is often fused with the head. In the thoracoabdominal type, the heart is covered, but often
displaced into the abdomen through a defect in the diaphragm (3).
Objective and aim of work:
We herewith describe the technique(s) used by members of our surgical team during the one-setting surgical
correction of different types of congenital chest wall (sternal deformities) namely : Cleft (deficient)
sternum; pectus excavatum, and the pectus carinatum defects.
Patients and Methods:
I. Study Design:
Our study was prospectively-undertaken from 2003 till 2006, in Kasr El Aini University Hospitals,
Abul Reesh National Insurance Hospital for Students, and Benha University.
II. The patient population: Our study enrolled 12 patients (7 males & 5 females) having 3
types of congenital sternal deformities: Deficient or Cleft Sternum (DS) in 5 patients (41.6 %);
Pectus excavatum (PE) in 6 patients (50%); and Pectus carinatum (PC) in 1 patient (8.3 %). Our
youngest patient - at the time of surgical correction - was a 4 months-old male baby with deficient
sternum; while the oldest was a 15-years old young man with pectus carinatum. The mean age of
our patients was 4 years ± 5.5 months (range 4 months-15 years) (Table 1).
Table 1: Preoperative Patient Data
Variable
*Patient Number (Total)
*Age (months/years)
- Mean
- Range
*Gender (Male/Female Ratio)
*Type of Deformity
» Deficient (Cleft) Sternum (DS)
» Pectus :
- Excavatum
- Carinatum
Data
12
4 years ± 5.5 months SD
4 months-15 years
7-5
5 (41.6 %)
7 (58.3 %)
6 (50 %)
1 (8.3 %)
III. Preoperative clinical examination:
The main presenting complaint in the pectus cases was “recurrent refractory episodes of chest
infection”; while in the deficient sternum patients, it was “fear of possible trauma to the exposed
heart”. Preoperative preparation consisted of a thorough clinical examination especially of the
heart and lungs.
IV. Preoperative special investigations :
Preoperative investigations were done in all cases to obtain a clear evaluation of the lesion’s extent
as: Plain Chest X-rays; CT Scanning; as well as any associated pathophysiology as
Echocardiographic study which revealed presence of mild mitral valve prolapse causing trivial
regurge in 1 of the pectus excavatum patients (16.6 %) due to the anterior compression of the heart
by the depressed sternum.
V. Indications for Surgical Correction:
For the pectus cases, the indication for surgical correction was the frequent recurrence of episodes of
chest infection. In female patients, the patient’s mother requested fast surgery for fear of future
disfigurement of her daughter’s chest. In the deficient sternum patients, surgery was done mainly as
prophylactic measure in order to prevent possible trauma to the inadequately-protected heart.
IV. Surgical Technique(s):
The surgical techniques used were following the basic fundamental concepts outlined by Ravitch
(4),(5).
With the only exclusion of separation of the perichondrium from the sternal edge as we
considered this to be further compromise to the perichondrial blood supply, and putting the nearby
internal thoracic vessels at high risk of trauma (injury or spasm). We performed subperichondrial
resection of all the offending pairs of costal cartilage, sternal remodeling by wedge osteotomy,
followed by final sternal stabilization.
(a) Corrective steps for Pectus Excavatum :
A vertical midline skin incision was started at 2nd. or 3rd. space extending till the xiphoid cartilage which
was dissected free of the upper part of the abdominal recti muscles. Using electrocautery, dissection was
then carried out from central to peripheral proceeding in two separate layers on both sides: the 1st. layer
consisting of skin-subcutaneous fascia; and the 2nd. layer taking the pectoralis major muscle. This was
followed by Subpericondrial dissection then resection of the deformed lower 6 cartilages stopping 1cm
short from the costochondral junction (growth plate). The previous step was achieved by passing a cottongauze strip behind the cartilage and applying traction to its ends pulling it up and facilitate opening its
perichondrial sheath and extraction of the inner offending cartilage prior to suturing its ends. A transverse
sternal osteotomy was then created above the level of the last deformed cartilage and the posterior
angulation of the sternum which is generally the 3rd. and occasionally the 2nd. cartilage. Punching few bites
from the anterior sternal table was done leaving around 1 cm between each 2 of them. We believed that
these small areas containing intact both anterior and posterior periosteal layers provides a better density of
bone that will cover and add solidity to the newly-formed callus (with a new corrected angle) over the
nearby punched areas. The upper and lower ends of the transverse osteotomy was then brought together by
few interrupted 2° prolene sutures. Retrosternal support-reinforcement was achieved by our innovative
technique consisting of implanting a “Single” nonabsorbable prosthetic bar formed from a merselene mesh
rolled over itself to form a tube, and then converted into a ribbon by passing a suture along its length. This
mesh-ribbon was sutured transversely “under tension” to the bony part of a rib from both sides behind the
middle of the sternal bone to bring the sternum anteriorly by around 30-35° thus abolishing the excavatum
deformity. Our modification which consisted of passing only a single mesh-ribbon intended to
render this step of the procedure less invasive as the multiple steps needed during passing multiple
substernal single mesh bands (as reported Karagounis et al (2004) (14)), means more chance for
injuring both mammary arteries. A single medium-sized haemovac drain was put under the
pectoalis muscles after bringing them together by midline continuous suturing, The chest cage was
then closed in layers after proper haemostasis and drainage of both pleural cavities. An elastic strip
was applied surrounding the chest cage for few days postoperatively until removal of the drains
was done. Postoperative regular analgesia and broad-spectrum antibiotics were prescribed in all
cases together with early ambulation and chest physiotherapy.
(b) Corrective steps for Pectus Carinatum :
In the carinatum subgroup, our technique was similar to that used in the excavatum cases being
performed through the same type of limited midline incision for dissecting similar layers. In our 2
cases, the carinatum defect was of the symmetric chondrogaldiolar type, as termed in 1949 by
Brodkin (15), consisting of anterior protrusion by the body of the sternum, with anterior protrusion
of the lower costal cartilages. The anterior osteotomy made was further pushed backwards by
inserting and fixing a bone graft taken from an adjacent rib. No retrosternal support mesh-band
was needed in both patients.
(c ) Corrective steps for Cleft (deficient) Sternum:
The skin was incised carefully just over the heart, and similar dissection of the pectoralis muscle layer.
Following pericardial opening, a piece of an appropriately-sized custom-made bone cement graft was
implanted “sandwitched” between 2 layers of merselene mesh to cover the sternal defect by suturing it all
around the adges of the defect using interrupted nonabsorbable 2° prolene sutures.
IIV.Postoperative patient follow-up and assessment of the surgical outcome::
Follow-up period was done for all patients for a whole year postoperatively by means of clinical
examination and questionnaire questions to the patient (or his or her relatives) asking them to share in rating
the patient’s evaluation and their satisfaction with the operative result in comparison to their original
complaint. Opinions were consequently graded as : excellent, good, fair (or accepted), and poor.
Table 2: Descriptors for the Rating Scale on the Patient Satisfaction (Davis & Weinstein, 2004) (16)
Survey Score
5
4
3
2
1
Descriptor
Completely satisfied with the repair = Excellent
Very pleased with the repair & notice considerable improvement = Very Good
Repair is just OK = Good
Result is somewhat disappointingt (cosmetically or functionally) = Acceptable
Total disappointment and dissatisfaction with the result = Unaccepted (Failure)
Results :
Mortality and Morbidity: We had no mortality. No significant intraoperative morbidities were
found. Blood loss was minimal and no transfusion was required. Postoperatively, transient mild
unilateral seroma below the pectoralis muscle (needed a 5-days vacuum drainge) was noticed in 1
patient (8.3 %); mild Chest wall pain (ameliorated by oral and local analgesia) in 1 patient (8.3 %); and
lag of cartilage regeneration leading to small anterolateral soft space (managed with increased oral
calcium intake) in another patient (8.3 %). No other types of morbidity occurred eg: blood loss
needing transfusion; Cardiac arrhythmias; or recurrence of sternal depression (pectus cases) (Table 3).
Table 3: Postoperative Morbidity and Mortality
Variable
Data
*Mortality
*Morbidity
- Ttransient mild unilateral seroma below the pectoralis muscle
- Mild Chest wall pain
- Lag of cartilage regeneration  small anterolateral soft space
- Blood loss needing transfusion
- Cardiac arrhythmias
- Recurrence of sternal depression (pectus cases)
None
3 (25 %)
1 (8.3 %)
1 (8.3 %)
1 (8.3 %)
None
None
None
Postoperative Course: Patients needed a relatively-short mean period of postoperative hospital
stay time of 8 ± 3.5 days (range 4 -13 days) (Table 4).
Postoperative Functional Outcome: According to the patient’s (and or child’s parents) words, the
postoperative functional results were described as : excellent in 6 patients (50 %); good in 4
patients (33.3 %); and acceptable in 2 patients (16.6 %). Generally-speaking, patients and or their
parents accepted the surgical results quite well and reported an obvious decline in the frequency of
chest infections, and a favorable improvement in morals together with an increased tolerance for
prolonged physical activity (Table 4).
Table 4: Postoperative Course and Functional Outcome
Variable
Data
*Postoperative Course:
(a) Hospital stay time (days):
- Mean
8 ± 3.5
- Range
4 -13
*Postoperative Functional Outcome (Davis & Weinstein, 2004) (16):
- Excellent
6 (50 %)
- Good
- Acceptable
4 (33.3 %)
2 (16.6 %)
Discussion:
A great variety of congenital abnormalities can occur in the chest wall. Their physiological
implications are also quite varied and span the spectrum from the rare entities of ectopia cordis and
asphyxiating thoracic dystrophy, which are often lethal, to the much more common pectus
excavatum and pectus carinatum with their limited physiological impact (1).
The basic pathophysiology in pectus deformities, is overgrowth of the costal cartilage. The
aberrant growth of these “Offending” Cartilages, displace the sternum either dorsally or ventrally.
The deformity may appear at birth or become more prominent during growth spurts. Pectus
excavatum tends to present at an earlier age than the carinatum defect. PE defect, is usually well
tolerated in infancy and childhood. However, the anterior chest depression in an infant with a
flexible chest may be accentuated by upper airway obstruction as from tonsillar or adenoidal
hypertrophy. Moreover, it was stated that both deformities will worsen with aging (1),(2),(4),(5). Most
of the defects will manifest before or during adolescence with some degree of aesthetic or
functional impairment (8). In our study, the clinical symptoms as told by either the patient or his or
her relatives (mostly parents) were the same as reported in the literature: the pectus cases
complained of recurrent chest infections with some palpitation and or arrhythmias in response to
exertion. A notice of cosmetic concern was a special complaint of the mothers of female
excavatum infants or children. Parents of underdeveloped or defective sternae were mainly asking
for a rapid surgical solution to cover the unprotected exposed heart.
Surgical correction is often necessary for moderate or severe chest wall deformities, because
spontaneous regression is rare (1). Repair is undertaken aiming to alleviate symptoms of pain,
cardiac or respiratory compromise (1), as well as to diminish significant psychological
consequences that prevent social activities in a child or adolescent (2). For the last half century the
golden classic or standard surgical approach to the pectus repair has been based on the techniques
described by Ravitch (4), consisting principally of subperichondrial removal of the offending costal
cartilage; remodeling of the sternum; followed by eventual sternal stabilization (5).
Many studies documented the results of the Ravitch approach (8-10). Recently, a two-stage,
minimally invasive approach “the Nuss procedure” has been described that avoids resection of the
costal cartilage. The 1st. stage involves small bilateral incisions through which a convex metal
plate is placed behind the sternum and then rotated 180 degrees to move the sternum ventrally. The
2nd. stage consists of removal of the bar, which is recommended at 2-to-4 years after the first stage
procedure. Advantages of this approach include the absence of an anterior scar and the avoidance
of cartilage resection (11),(12). A new innovative technique was designed in 2004 by Davis &,
Weinstein (13), consisting of implanting multiple polypropylene mesh bands (Marlex Mesh; Davol
Inc., Bard Cardiosurgery, Cranston, RI) 12 cms long X 1 cm wide being placed posterior to the
sternum and anterior to the pleura and pericardium. The mesh bands are anchored under tension to
the ribs with 2-0 polypropylene sutures for retrosternal reinforcement. In this study, we performed
repair of different types of congenital chest wall defects namely, Pectus excavatum, carinatum, as
well as deficient sternum.
once covered with muscle, subcutaneous tissue, and skin, this degree of deviation cannot be
noticed and only the flatness of the chest wall is apparent. We have found this technique yields a
stable repair
The second paper: As a relatively new technique, the Nuss procedure is still in evolution.
Technical modifications have been made to minimize some of the reported complications
including cardiac perforation and loss of bar position [9 –13]. As with all innovative surgical
procedures, the results of this new technique are reported and are compared with the standard
Ravitch procedure in terms of outcomes [14, 15]. Surgical results can be scientifically documented
by computed tomography (CT) measurements [16, 17] or subjectively evaluated by some
measurement of patient satisfaction. As the Nuss procedure continues to evolve, comparisons of
rates of complications, recurrences, lengths of stay, and cost will be necessary. Although in two
recent series the Ravitch and the Nuss approaches were compared at the same institution [11, 18],
lack of data about the criteria for procedure selection make the data difficult to interpret. During
the last decade we have continued to use the Ravitch approach and have made several technical
modifications in the operative procedure and perioperative management strategy. We present a
series of 69 consecutive patients with the pectus deformity who have undergone repair from 1991
to the present. We believe that these updated data regarding the efficacy, safety, and resource
utilization of the Ravitch approach will be useful for comparison as more results from the Nuss
procedure become available.
Some reported complications: We believe the seromas were related to larger flaps that attend the
smaller skin incision, and we have modified our postoperative drainage regimen as a result. Now,
Jackson-Pratt drains are left until drainage fully ceases. CT scan demonstrated considerable
ossification of the perichondrial beds. The other 2 patients had CT evidence of subluxation of
regenerated perichondrium underneath the sternum. In 2 of the 3 patients the pain was mild and did
not require therapy. Re-resection of the cartilage eliminated the pain. Two patients had recurrence
of sternal depression: 1 was mild and the other patient thought the deformity ended up unchanged
after several years. One patient had an area of nonregenerated cartilage that resulted in a small soft
space anterolaterally. The postoperative length of stay and costs were both slightly lower for the
carinatum subset compared with the excavatum subset, reflecting the slightly lower complexity of
the operative procedure.
Refs of paper 2:
1. Castile R, Staats B, Westbrook P. Symptomatic pectus deformities of the chest. Am Rev Respir
Dis 1982;126:564–8.
2. Ravitch MM. The operative treatment of pectus excavatum. Ann Thorac Surg 1949:429–44.
3. Ravitch MM. Congenital deformities of the chest wall and their operative correction.
Philadelphia: WB Saunders, 1977:145–58.
4. Haller JA, Scherer LR, Turner CS, Colombani PM. Evolving management of pectus excavatum
based on a single institutional experience of 664 patients. Ann Surg 1989;209:578–83.
5. Fonkalsrud EW, Salmon T, Guo W, Gregg JP. Repair of pectus deformities with sternal support.
J Thorac Cardiovasc Surg 1994;107:37–42.
6. Kowalewski J, Brocki M, Zolynski K. Long-term observation in 68 patients operated on for
pectus excavatum: surgical repair of funnel chest. Ann Thorac Surg 1999;67:821–4.
7. Nuss D, Kelly RE, Croitoru DP, Katz ME. A 10-year review of minimally invasive technique
for the correction of pectus excavatum. J Pediatr Surg 1998;33:545–52.
8. Hebra A, Swoveland B, Egbert M, et al. Outcome analysis of minimally invasive repair of
pectus excavatum: review of 251cases. J Pediatr Surg 2000;35:252–8.
9. Jacobs JP, Quintessenza JA, Morell VO, et al. Minimally invasive endoscopic repair of pectus
excavatum. Eur J Cardiothorac Surg 2002;21:869–73.
10. Moss RL, Albanese CT, Reynolds M. Major complications after minimally invasive repair of
pectus excavatum: case reports. J Pediatr Surg 2001;36:155–8.
11. Miller KA, Woods RK, Sharp RJ, et al. Minimally invasive repair of pectus excavatum: a
single institution’s experience. Surgery 2001;130:652–9.
12. Schaarschmidt K, Kolberg-Schwerdt A, Dimitrov G, Srauss J. Submuscular bar, multiple
pericostal bar fixation, bilateral thoracoscopy: a modified Nuss repair in adolescents. J Pediatr
Surg 2002;37:1276–80.
13. Nuss D, Croitoru DP, Kelly RE, et al. Review and discussion of the complications of
minimally invasive pectus excavatum repair. Eur J Pediatr Surg 2002;12:230–4.
14. Engum S, Rescorla F, West K, et al. Is the grass really greener? Early results of the Nuss
procedure. J Pediatr Surg 2000;35:246–51.
15. Hosie S, Sitkiewicz T, Petersen C, et al. Minimally invasive repair of pectus excavatum—the
Nuss procedure. A European multicentre experience. Eur J Pediatr Surg 2002;12:235–8.
16. 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;22:904–6.
17. Welch KJ. Chest wall deformities. In: Holder TM, Ashcraft KW, eds. Pediatric surgery.
Philadelphia: WB Saunders, 1980:162.
18. Wu PC, Knauer EM, McGowan GE, Hight DW. Repair of pectus excavatum deformities in
children. A new perspective of treatment using minimal access surgical technique. Arch Surg
2001;136:419–24.
19. Davis JT, Weinstein S. Repair of the Pectus Deformity: Results of the Ravitch Approach in the
Current Era Ann Thorac Surg 2004;78:421– 6
The Cleft Sternum paper by Antonio Cleft sternum is a rare congenital condition caused by
failed midline development and fusion of the mesodermal lateral plates at about 8 weeks of
intrauterine life [2]. The complete form is extremely rare and about 35 cases are currently reported
in the literature and are usually observed as isolated entities [3]. The incomplete clefts can be
subdivided into two further types: (1) inferior, when the chondral bridge joins the superior part of
the sternum; it is usually associated with other midline anomalies such as Cantrell pentalogy [2];
and (2) superior, when the chondral bridge is in the inferior part of the sternum; it can be isolated
[4] or associated with a neurocutaneous syndrome, which is more frequent in the female sex (87%
to 100% of cases) [5–7]. In about 10% of CS patients, a supra-umbilical raphe is observed, and
associated cardiac anomalies occur in about 30% [7]. Reported symptoms are dyspnea, cyanosis,
and lung infection [3]. This abnormality deserves early repair because of possible respiratory
impairment (ie, paradoxic movement of mediastinal structures) and potentially dangerous injuries
to the mediastinal organs, due to lack of the sternal shield [2, 3]. In early infancy, the sternum can
be closed easily thanks to the elasticity and the relative expandability of young cartilage versus the
bony chest wall of the adult. Whenever possible, surgical correction should be done without using
prosthetic material because of its inability to grow and its higher risk related to infection [2].
Simultaneous repair of cardiac abnormalities and CS is preferable. However, when cardiac repair
is technically complex, and the postoperative course is likely to be characterized by ventricular
dysfunction, sternal repair and subsequent chest closure soon after cardiac operation may not be
tolerated. Thus, CS repair may be better delayed and safely performed as clinical conditions
improve and myocardial edema is resolved.
Based on these concepts, we chose two different surgical strategies. In patient 1, due to the more
complex repair and a higher probability of myocardial dysfunction, we decided to postpone the
sternal reconstruction. In patient 2, since the repair was extracardiac, with a lower potential for
postoperative cardiac failure, we performed a singlestage correction. Cleft sternum surgical repair
is a safe technique that can be done using different techniques [2]; our current choice is the
modified Sabiston sliding chondrotomies technique [8] by which chondrotomies are performed
after dissecting the clavicles and costal periosteum free. Costal perichondrial posterior bed is
usually preserved incising only the anterior layer of it. This is to accomplish regrowth of the ribs,
which usually happens in 6 to 8 months. The two sternal bars can be approximated and the residual
gap is filled with cartilage graft obtained from the inferior cartilage bridge previously removed.
We believe the subperiosteal clavicular resection to be essential for good results with this
procedure; in fact, this maneuver prevents occurrence of a superior outlet thoracic syndrome as the
two sternal bars come close together. In addition, cartilage fragments have to be secured safely so
as to avoid dislocation caused by chest wall excursions during breathing or crying and the delaying
of cartilage and bone healing [9]. In conclusion, CS correction is a feasible and safe procedure
with good early outcome that should be performed as soon as possible, simultaneously or after
cardiac repair, depending on the patient’s clinical condition. This procedure is a long-lasting safe
repair when no prosthesis is adopted.
References
1. Shamberger RC, Welch KJ. Sternal defects. Pediatr Surg Int 1990;5:156–64.
2. Jose RM, De Campos L, Filomeno TB, et al. Repair of congenital sternal cleft in infants and
adolescent. Ann Thorac Surg 1998;66:1151–3.
3. Shalak L, Kaddoura I, Obeid M, Hashem H, Haidar R, Bitar FF. Complete cleft sternum and
congenital heart disease: review of the literature. Pediatr Int 2002;44:314–6.
4. Kaplan LC, Matsuoka R, Gilbert EF, Opitz JM, Kurnit DM. Ectopia cordis and cleft sternum:
evidence for mechanical teratogenesis following rupture of the chorion or yolk sac. Am J Med
Genet 1985;21:187–202.
5. Frieden I, Reese V, Cohen D. PHACE syndrome: the association of posterior fossa brain
malformation, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and
eye abnormalities. Arch Dermatol 1996;132:307–11.
6. James PA, Mc Gaughran J. Complete overlap of PHACE syndrome and sternal malformationvascular dysplasia association. Am J Med Gen 2002;110:78–84.
7. Metry DW, Dowd CF, Barkovich AJ, Frieden I. The many faces of PHACE syndrome. J Ped
2001;139:117–23.
8. Sabiston DC. The surgical management of congenital bifid sternum with partial ectopia cordis. J
Thorac Surg 1958;35:118–22.
9. Domini M, Cupaioli M, Rossi F, Fakhro A, Aquino A, Chiesa PL. Bifid sternum: neonatal
surgical treatment. Ann Thorac Surg 2000;69:267–9.
Final References of the whole paper :
(1) Castile R, Staats B, Westbrook P. Symptomatic pectus deformities of the chest. Am Rev Respir
Dis 1982;126:564–8.
(2) Robicsek F, Fokin A. Surgical correction of pectus excavatum and carinatum. J Cardiovasc
Surg 1999;40:725–31.
(3) Shamberger RC, Welch KJ. Surgical correction of pectus carinatum.J Pediatr Surg 1987;22:48–
53.
(4) Ravitch MM. The operative treatment of pectus excavatum. Ann Thorac Surg 1949:429–44.
(5) Ravitch MM. Congenital deformities of the chest wall and their operative correction.
Philadelphia: WB Saunders, 1977:145–58.
(6) Shamberger RC, Welch KJ. Surgical repair of pectus excavatum. J Pediatr Surg 1988;23:615–
22.
(7) Lester CW; The etiology and the pathogenesis of funnel chest, pigeon breast, and related
deformities of the anterior chest wall. J Thorac Surg 1957;34:1-10.
(8) Haller JA, Scherer LR, Turner CS, Colombani PM. Evolving management of pectus excavatum
based on a single institutional experience of 664 patients. Ann Surg 1989;209:578–83.
(9) Fonkalsrud EW, Bustorff-Silva J. Repair of pectus excavatum and carinatum in adults. Am J
Surg 1999;177:121–4.
(10) Kowalewski J, Brocki M, Zolynski K. Long-term observation in 68 patients operated on for
pectus excavatum: surgical repair of funnel chest. Ann Thorac Surg 1999;67:821–4.
(11) Nuss D, Kelly RE, Croitoru DP, Katz ME. A 10-year review of minimally invasive technique
for the correction of pectus excavatum. J Pediatr Surg 1998;33:545–52.
(12) Hebra A, Swoveland B, Egbert M, et al. Outcome analysis of minimally invasive repair of
pectus excavatum: review of 251cases. J Pediatr Surg 2000;35:252–8.
(13) Davis JT, Weinstein S. Repair of the Pectus Deformity: Results of the Ravitch Approach in
the Current Era Ann Thorac Surg 2004;78:421– 6.
(14) Karagounis VA, Wasnick J, Gold JP An Innovative Single-Stage Repair of Severe
Asymmetric Pectus Excavatum Defects Using Substernal Mesh Bands Ann Thorac Surg
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‫الملخص العربي‬
‫العالج الجراحى للتشوهات الخلقية لجدار الصددر الررققدة لالخةدرال ايلليدة لم تشدىى‬
‫القصر العينى‬
‫مقدمة شملةةهذهماةةرامة درة ة محةةت مالةةعام ةةدخلمةخاالنةةدامة ةةدجذ م ةخاالنةةدامة ةةدر م‬
‫جدةرمة صدر مكر كم دخلمةافع مالظه مة قص‪.‬‬
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‫الدم‪.‬‬
‫النتائجشم ممتك ماندكم دخلم فةدةمأننةدامة الالةقم قةدمأ اةتلمة نتةدفحمبةد فالصمةخكذينيكة م‬
‫ةخلن مة هقطني مةنماتدفحمة جتة مكداهممهتدزةمف م‪,%50‬م يةدةمفة م‪%33,3‬م مقالو ة م‬
‫ف م‪%16,6‬مم مة الدخل‪.‬‬
‫الخالصة شميسةت ذصممة ماةرامة درة ة مأنم ةدخلمة ت ةوادلمة ذقية م جةدةرمة صةدريجبم‬
‫الع ادم تة يدم يقمأنمة نتدفحمتكونممتضي م مقالو م كلمم مة جتةحم ة هتيض‪.‬‬