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Ann Thorac Surg 2006;81:2291-2294
© 2006 The Society of Thoracic Surgeons

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Case report

Surgical Repair of Incomplete Cleft Sternum and Cardiac Anomalies in Early Infancy

^a Division of Pediatric & Congenital Cardiac Surgery, Department of Pediatrics, University of Padova, Medical School, Padova, Italy
^b Division of Pediatric Surgery, Department of Pediatrics, University of Padova, Medical School, Padova, Italy

Accepted for publication July 5, 2005.

^_* Address correspondence to Dr Stellin, Pediatric & Congenital Cardiac Surgery Unit, University of Padova Medical School, Centro V. Gallucci, Via Giustiniani, 2, Padova, 35100 Italy (Email: giovanni.stellin{at}unipd.it).

	Abstract

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Congenital cleft sternum is a rare congenital anomaly due to lack of fusion of the lateral mesodermal plates. We report two cases of incomplete superior cleft sternum associated with cardiac anomalies that underwent successful complete surgical correction.

	Introduction

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Congenital cleft sternum (CS) is a congenital anomaly whose occurrence has been rarely reported [1]. It is commonly classified in two types: (1) complete, when the sternum is split from the manubrium to the xiphoid, and (2) incomplete, when a chondral bar bridges the midline [2].

We report on our surgical experience with two cases of incomplete superior CS that were associated with complex cardiac anomalies.

	Case Reports

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Patient 1
A newborn woman with a prenatal diagnosis of {S,D,D} double outlet right ventricle and subpulmonary ventricular septal defect (VSD) was found at birth to be affected by incomplete CS (ie, a thin skin membrane was covering the pericardium). In addition she presented with facial hemangiomas and supra-umbilical raphe (Fig 1). Genetic analysis was unremarkable. At birth, she was in good clinical condition showing only moderate dyspnea. Bi-dimensional echocardiography showed a large malalignment of the VSD, a bicuspid aortic valve, an aortic coarctation, and a patent ductus arteriosus.

View larger version (95K): [in this window] [in a new window]   Fig 1. Preoperative photograph. Note a thin pre-sternal membrane and a supra-umbilical raphe.

Her postoperative course was uneventful, and she was extubated 12 hours later and was transferred to the ward on postoperative day (POD) 3. Her sternal wound was cleaned daily. She was submitted to CS repair on POD 14. The sternal table was completely reconstructed using the following surgical technique.

After incising the skin and dissecting it free from the muscular–skeletal planes, the costal perichondrium was incised until the cartilage portion was exposed. This was incised longitudinally, so as to mobilize and approximate the sternal residuals (Fig 2A). The posterior perichondrial bed was left intact. This procedure was done bilaterally and from the insertion of the clavicles to the seventh or eighth pair of ribs. Both clavicles were interrupted in order to prevent constrictive thoracic outlet syndrome, once the lateral sternal bars were close together.

View larger version (39K): [in this window] [in a new window]   Fig 2. Cleft sternum repair technique. (A) Costal perichondrium is incised longitudinally so as to mobilize and approximate the sternal residuals. (B) The cartilage segments were positioned in between the lateral sternal bars. (C) The cartilage was secured to the bone with nonreabsorbable sutures so as to avoid dislocation.

Postoperatively she presented with a low cardiac output syndrome that required dopamine intravenous infusion (5 mcg/kg/min) for 6 days. She was finally discharged from the pediatric intensive care unit 14 days after CS repair (POD 28), and her subsequent clinical course was uneventful. She was discharged on POD 33, in good clinical condition, with good functional and aesthetic results of the sternal repair. Pre-discharge, bi-dimensional echocardiography showed good biventricular function, no residual VSD, good reconstruction of the left ventricular outflow tract, and no pericardial effusion. At 12-month follow-up, she underwent a successful balloon angioplasty for residual aortic coarctation. Currently she is in good clinical condition without medications; the sternal wound is well healed and solid.

Patient 2
A newborn female had postnatal diagnosis of CS, associated with diffuse hemangiomas and paradoxic movement of the pre-sternal soft tissues. Bi-dimensional echocardiography was unremarkable. She was discharged home and elective CS surgical repair was planned for the future after 1 year of age. However, after 9 months, she was urgently readmitted for severe respiratory distress. Bi-dimensional echocardiography showed a cephalic aortic arch with possible stenosis between the left carotid artery and the left subclavian artery, which was not visualized previously. Cardiac catheterization showed a double aortic arch and severe aortic arch coarctation, and she was listed for urgent surgery. After midline incision, pericardiotomy was done and cardiopulmonary bypass was started. During circulatory arrest, the double aortic arch was repaired by distal right aortic arch section, and the isthmic coarctation (distal to the double arch) was resected followed by a termino-lateral anastomosis to the ascending aorta. The sternal cleft was repaired in the same operative session using the previously mentioned technique. Because of residual coarctation diagnosed at routine 2-dimensional echocardiography in the intensive care unit on POD 1, she underwent emergent coarctation repair through the left lateral thoracotomy with reimplant of the aortic arch on the ascending aorta without cardiopulmonary bypass, and an enlargement of the proximal arch portion with an inverted left subclavian artery flap. Postoperative course was characterized by left pneumothorax and right chylothorax treated with chest drainage and medium chain fatty acid diet. She was finally discharged on POD 40 in good clinical and hemodynamic condition. Pre-discharge, bi-dimensional echocardiography showed good surgical results with trivial residual stenosis. Six months later, she had successful balloon dilation for residual coarctation. At a 12-month follow up, she was found in good satisfactory clinical condition. Bi-dimensional echocardiography suggests possible vascular occlusion at the right carotid artery site. The sternal site is well healed and solid.

	Comment

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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 single-stage 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

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1. Shamberger RC, Welch KJ. Sternal defects Pediatr Surg Int 1990;5:156-164.
2. Jose RM, De Campos L, Filomeno TB, et al. Repair of congenital sternal cleft in infants and adolescent Ann Thorac Surg 1998;66:1151-1153.[Abstract/Free Full Text]
3. Shalak L, Kaddoura I, Obeid M, Hashem H, Haidar R, Bitar FF. Complete cleft sternum and congenital heart diseasereview of the literature. Pediatr Int 2002;44:314-316.[Medline]
4. Kaplan LC, Matsuoka R, Gilbert EF, Opitz JM, Kurnit DM. Ectopia cordis and cleft sternumevidence for mechanical teratogenesis following rupture of the chorion or yolk sac. Am J Med Genet 1985;21:187-202.[Medline]
5. Frieden I, Reese V, Cohen D. PHACE syndromethe association of posterior fossa brain malformation, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Arch Dermatol 1996;132:307-311.[Abstract]
6. James PA, Mc Gaughran J. Complete overlap of PHACE syndrome and sternal malformation-vascular dysplasia association Am J Med Gen 2002;110:78-84.[Medline]
7. Metry DW, Dowd CF, Barkovich AJ, Frieden I. The many faces of PHACE syndrome J Ped 2001;139:117-123.[Medline]
8. Sabiston DC. The surgical management of congenital bifid sternum with partial ectopia cordis J Thorac Surg 1958;35:118-122.[Medline]
9. Domini M, Cupaioli M, Rossi F, Fakhro A, Aquino A, Chiesa PL. Bifid sternumneonatal surgical treatment. Ann Thorac Surg 2000;69:267-269.[Abstract/Free Full Text]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Massimo Antonio Padalino Francesco Migneco Maurizio G. Rubino Giovanni Stellin Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Padalino, M. A. Articles by Stellin, G. Search for Related Content PubMed PubMed Citation Articles by Padalino, M. A. Articles by Stellin, G. Related Collections Chest wall