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Ann Thorac Surg 1997;64:561-562
© 1997 The Society of Thoracic Surgeons

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Case Reports

Combined Repair of Upper Sternal Cleft and Tetralogy of Fallot in an Infant

Departments of Cardiac Surgery and Pediatric Cardiology, Hôpital des Enfants Reine Fabiola, Brussels, Belgium

Accepted for publication March 31, 1997.

	Abstract

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We report on a 2-month-old infant with the unusual association of an upper sternal defect and tetralogy of Fallot. Surgical correction of the cardiac disease consisted of closing the ventricular septal defect and relief of the right ventricular outflow tract by infundibuloplasty. The already less compliant bony thorax of the infant made direct approximation of the upper sternal defect only possible with adjuvant bilateral chondrotomies. Sternal cleft repair is advised during the very first weeks of life, during which complete correction of the rarely associated tetralogy of Fallot can be successfully performed.

	Introduction

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Cleft sternum is a rare thoracic malformation, resulting from partial or total failure of sternal fusion at an early stage of embryonic development. Besides the isolated forms with good prognosis, cleft sternum can present in association with other life-threatening disorders as ectopia cordis or as a part of Cantrell's pentalogy, which comprises a lower sternal defect, a deficiency of the anterior diaphragm and pericardium, a midline supraumbilical abdominal wall defect, and an intracardiac disorder [1]. In these instances, the presence of intrinsic cardiac anomalies may influence the outcome negatively [2].

Here, we report an unusual association of an upper sternal cleft and tetralogy of Fallot in an infant who underwent succesful total repair at 2 months of age.

A 2-month-old girl was referred to our hospital for investigation of an asymptomatic cardiac disease and partial sternal cleft. Clinical examination showed a well-colored infant presenting a U-shaped upper sternal cleft, 5 cm wide at the cephalad level. The caudal portion of the sternum was fused at the level of the fifth rib. She also had a well-demarcated capillary facial hemangioma reaching to her neck basis. No associated internal vascular lesions were identified. Despite a normal neurologic and psychomotoric clinical status, further investigation revealed total agenesis of the corpus callosum and severe dysplasia of the left optical nerve, resulting in almost definite blindness of the left eye.

Echocardiography showed a heart in situs solitus, the apex slightly rotated in the cephalad direction. The intracardiac malformation consisted of a favorable form of tetralogy of Fallot, with a large perimembranous ventricular septal defect, aortic dextroposition, basoinfundibular stenosis, moderate dysplasia of a competent pulmonary valve, and good-sized pulmonary annulus (9 mm) and pulmonary artery branches (7 mm).

During the surgical procedure a ventral midline incision was made from the point corresponding with the virtual suprasternal notch to the epigastric region. The cartilaginous tissue bridge of the distal sternum was transected. The heart was located in a very thin pericardial sac and was slightly displaced upward. Cardiopulmonary bypass was instituted after heparin administration. After aortic cross-clamping and crystalloid cardioplegia, the ventricular septal defect was closed by a 0.4-mm polytetrafluoroethylene patch through a right atriotomy. The infundibular stenosis was relieved by excision of the muscular extensions of the supraventricular crest, and completed by subannular patching with bovine pericardium because the autologous pericardium was judged of poor quality. After removal of the aortic cross-clamp, spontaneous sinus rhythm resumed and cardiopulmonary bypass was discontinued at normothermia.

Second, the caudal borders of the sternum were resected while the superior edges were deperiostated. To overcome the gap of 5 cm, adjuvant sliding chondrotomies of the first four ribs of both sides were performed by oblique transection. The two thoracic flaps were then approximated with five interrupted stitches of Ticron 2/0 (Davis + Geck, Danbury, CT). But the shortness of both clavicles hindered firm closure of the cephalad sternal part and necessitated dissociation of the sternoclavicular junctions.

Closed suction drains were placed below the myocutaneous flaps, and the overlying tissue layers were sutured separately. There was no evidence of cardiac compression, and the patient was transferred to the intensive care unit in stable hemodynamic condition. Ventilatory support was deliberately maintained for 5 days to allow some stabilization of the thoracic wall. Nevertheless, respiratory weaning and hemodynamic recovery were uneventful.

Echocardiographic control showed a good result of the intracardiac repair without residual right outflow tract obstruction or valvular regurgitation. The child was discharged healthy on the 12th postoperative day. Follow-up examination at 2 months revealed a stable sternal repair and well-healed soft tissue coverage.

	Comment

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Disturbances of normal ventral midline thoracic fusion at the embryonic stage can present as a spectrum of abnormalities, varying from a prominent suprasternal notch to partial or complete sternal cleft, pentalogy of Cantrell, or ectopia cordis in its worst expression [1, 2].

Superior clefts are most often isolated, but association with vascular dysplasias has been reported. The presence of a craniofacial hemangioma is a well-described associated lesion and stresses the need for investigation of potentially lethal internal vascular anomalies [3]. Neither echocardiography nor angiography showed major vascular changes in this infant.

Conversely, inferior and complete clefts are usually associated with several other defects of the same developmental field, including various intracardiac malformations. Tetralogy of Fallot, for instance, has been described as an intrinsic part of Cantrell's pentalogy or true ectopia cordis [1, 2]. Therefore, this case can be considered rather exceptional as it combines uncommonly an upper sternal cleft and tetralogy of Fallot.

Although the embryology of the thoracic wall is well known, the etiology of cleft sternum remains unclear. Failure of fusion of the lateral sternal bands by an early disturbance affecting the midline mesodermal structures between the sixth and ninth gestational week is a possible mechanism, but no teratogen or familial basis has been identified as a potential cause [3]. Van Allen and Myhre [4] reported that early amnion rupture may result in multiple defects of the thorax, heart, face, and brain by pressure necrosis, incomplete morphogenesis, and tearing and tethering by amnion bands. The coexistance of an ocular defect and agenesis of the corpus callosum in this infant may eventually fit with these findings. Nevertheless, the presence of a sternal fusion defect in an otherwise asymptomatic patient should enhance the awareness and indicate a careful search for possible associated anomalies of other midline structures.

Numerous methods of surgical correction of the sternal cleft have been reported, including direct approximation of the sternal bands [5], sliding or rotating chondrotomies [6], interposition of bone, cartilage, or prosthetic grafts [7], and closure by pectoralis muscle flap advancement [8]. However, the determining factor in justifying the appropriate technique is the age of the patient. Presently, general consensus advocates early correction during the neonatal period, because the bony structures of the thorax are still compliant and permit direct sternal closure [5]. But the association of a congenital heart disease may occasionally delay the sternal repair until the age and weight for an elective operation on the cardiac malformation is reached.

Recently, advances in cardiopulmonary bypass techniques and the improved perioperative management of neonates and infants have allowed total correction of symptomatic tetralogy of Fallot in neonates. Di Donato and associates [9] even suggested that, if mortality is lower in the absence of symptoms, elective repair could be reasonably undertaken during the first months of life with a minor risk.

Despite the asymptomatic character of the cardiopathy, we preferred to perform a one-stage repair of both malformations to take advantage of the elasticity of the bony thoracic structures at 2 months of age. Primary sternal closure was possible without causing pressure on the enlarged right heart, but the wideness of the gap between the sternal edges of an already less compliant thorax presented the major surgical problem, necessitating bilateral sliding chondrotomies and sternoclavicular dislocation. This emphasizes the advisability of very early repair, preferably during the neonatal period.

In conclusion, this case is rather unusual as regards the association of an upper sternal cleft and an uncomplicated tetralogy of Fallot. A combined surgical repair of both anomalies should be attempted soon after birth, affording the obvious advantages of early complete correction of the cardiac malformation and optimal management of the thoracic malformation, which should provide the best long-term outcome of both diseases.

View larger version (135K): [in this window] [in a new window]   Fig 1. . Opening of the skin, subcutaneous layers, and superior pericardium shows the wide upper sternal cleft, fused at its caudal portion, and the prominent right ventricular mass in the notch of the cleft.

	Footnotes

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	References

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1. Ravitch M. Congenital deformities of the chest wall and their operative correction. Philadelphia: Saunders, 1977:23–53.
2. Cantrell JR, Haller JA, Ravitch M. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynecol Obstet 1958;107:602–14.[Medline]
3. Hersh JH, Waterfill D, Rutledge J, et al. Sternal malformation/vascular dysplasia association. Am J Med Genet 1985;21:177–86.[Medline]
4. Van Allen MI, Myhre S. Ectopia cordis thoracalis with craniofacial defects resulting from early amnion rupture. Teratology 1985;32:19–24.[Medline]
5. Salley RK, Stewart S. Superior sternal cleft: repair in the newborn. Ann Thorac Surg 1985;39:582–3.[Abstract/Free Full Text]
6. Sabiston DC. The surgical management of congenital bifid sternum with partial ectopia cordis. J Thorac Cardiovasc Surg 1958;35:118–22.
7. Verska JJ. Surgical repair of total cleft sternum. J Thorac Cardiovasc Surg 1975;69:301–5.[Abstract]
8. Snyder BJ, Robbins RC, Ramos D. Primary repair of complete sternal cleft with pectoralis major muscle flaps. Ann Thorac Surg 1996;61:983–4.[Abstract/Free Full Text]
9. Di Donato RM, Jonas RA, Lang P, Rome JJ, Mayer JE, Castañeda AR. Neonatal repair of tetralogy of Fallot with and without pulmonary atresia. J Thorac Cardiovasc Surg 1991;101:126–37.[Abstract]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map 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): Jacques P. Goldstein Frank E. Deuvaert Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Bové, T. Articles by Deuvaert, F. E. Search for Related Content PubMed PubMed Citation Articles by Bové, T. Articles by Deuvaert, F. E.