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Ann Thorac Surg 2000;69:267-269
© 2000 The Society of Thoracic Surgeons

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Case Reports

Bifid sternum: neonatal surgical treatment

^a Department of Pediatric Surgery, Policlinico Sant’ Orsola, University of Bologna, Bologna, Italy
^b Department of Pediatric Surgery, University "G. D’Annunzio" of Chieti, Pescara, Italy

Address reprint requests to Dr Dòmini, Department of Pediatric Surgery, University of Bologna, Policlinico Sant’ Orsola, Via S. Domenico 11, 40124 Bologna, Italy
e-mail: m.domini{at}mail2.dex-net.com

	Abstract

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The sternal cleft is a rare congenital anomaly resulting from a fusion failure of the sternum, generally observed at birth and asymptomatic. Surgery is indicated to protect the heart and major vessels from trauma, to improve respiratory dynamics, and for aesthetic reasons. We observed 2 neonates for a superior and medial thoracic mass. The defect involved the upper two thirds of the sternum. The surgical operation for both patients consisted in the primary closure of the defect.

	Introduction

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Congenital anomalies of the sternum comprise a broad spectrum of deformities that is difficult to classify. Reviewing the world literature, Shamberger and Welch [1] in 1990 divided them into four groups: cervical ectopia cordis, thoracic ectopia cordis, thoracoabdominal ectopia cordis, and cleft sternum. There is a great difference between the first three groups and the last in terms of associated anomalies, surgical treatment, and prognosis.

The cleft sternum is a very rare congenital malformation—about 100 cases have been reported [1–7]—without a familial basis and with probable multifactorial etiology [2]. It can be complete (the rarest form) and incomplete (the upper cleft sternum or bifid sternum [BF]). The BF is usually isolated, with orthotopic normal heart and normal skin coverage [1]. It may be V-shaped, when the cleft reaches the xiphoid process, or broad and U-shaped, with a bony bridge joining the two edges, ending at the third or fourth costal cartilage. It is generally observed at birth and asymptomatic. In those rare symptomatic cases, cyanosis, dyspnea, and pulmonary infections are the main clinical features [3, 8]. Surgical correction is indicated to protect the heart and great vessels from injury, to improve respiratory dynamics, and to restore a good aesthetic appearance.

	Case reports

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Patient 1
E. C., a 6-day-old male neonate, 40 weeks’ gestation, birth weight 3,500 g, was transferred to the Department of Pediatric Surgery, University "G.D’Annunzio" of Chieti in Pescara for a midline thoracic bulge, evident during expiration and weeping. In inspiration a depression appeared in the same area. The anomaly was well tolerated without symptoms. Observation and palpation allowed us to diagnose a 4-cm-wide U-shaped, sternal cleft (Fig 1).

View larger version (143K): [in this window] [in a new window]   Fig 1. (Patient 1.) Aspect of the upper sternal midline bulge evident during expiration and weeping. In inspiration a depression appeared in the same area.

Patient 2
D.M., a full-term male neonate, 4,300 g weight, born from a dystocic delivery, was admitted to a neonatal intensive care unit (NICU) a few hours after birth because of a neonatal asphyxia (Apgar scores 1 in the 1st minute—immediate oxygen therapy—and 9 after 5 minutes). The clinical examination revealed a soft mass protruding from an upper thoracic defect, evident with every expiration. A chest roentgenogram showed the upper sternal cleft, with no other skeletal anomaly; a computed tomographic (CT) scan performed in the same hospital confirmed the diagnosis of agenesis of the manubrium. Echocardiography demonstrated a patent foramen ovalis. At the age of 20 days he was referred to the Department of Pediatric Surgery of the University of Bologna. The defect measured 3.5 cm at the widest point and reached the third intercostal space. On admission the neonate was asymptomatic.

At the age of 2 and 4 weeks, both patients were operated on by primary closure of the defect. Through a midline cervicothoracic incision, the sternal bars were dissected free from the insertion of pectoralis major muscles anteriorly, and from endothoracic fascia posteriorly. Because of the U-shaped defect, a wedge of cartilage had to be excised at the confluence of the sternal bars to approximate the two halves without tension, as advised by Ravitch [4]. The edges of the bars were then sharply freshened, and multiple encircling nonabsorbable sutures were passed through every intercostal space. A few minutes of trial juxtaposition of the sternal bars was required to evaluate hemodynamic status (heart rate, electrocardiogram, SaO₂, blood pressure, and central venous pressure), then the sutures were tied, the pectoralis major muscles were apposed in the midline, and the sternohyoid, sternothyroid, and sternocleidomastoid muscles were approximated medially to avoid lung herniation.

A Penrose drainage was left under the skin flaps and the skin closure was performed with absorbable subcuticular sutures. No intraoperative complications occurred except for two small lacerations of the parietal pleura, treated with intraoperative air aspiration without necessity of any thoracic drainage, in 1 patient. Both postoperative courses were uneventful. The first 2 postoperative days were spent in the NICU under mechanical ventilation. The patients were discharged after a smooth recovery on the 10th and 12th postoperative days. Both infants, after 18 and 15 months from intervention, respectively, show a good functional and cosmetic result (Fig 2).

View larger version (141K): [in this window] [in a new window]   Fig 2. (Patient 1.) Postoperative control 18 months after the operation showing the complete functional and aesthetic result.

	Comment

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Few associated anomalies are described [1]: a bandlike scar from the umbilicus to the sternal defect or from it to the chin, cervicofacial hemangiomas, and diastasis recti; none of these was found in our patients.

Until now no prenatal diagnosis has been made and in only one case has a prenatal ultrasonogram, reevaluated after birth, shown the presence of an upper cleft [5]. The diagnosis of BF is easily done at birth by inspection and palpation. Diagnostic investigations are therefore directed to exclude the infrequent associated anomalies. In our opinion, a CT scan (in one case performed elsewhere) is not advisable because it is invasive and expensive; it should be reserved only for differential diagnosis in difficult cases.

There is general agreement by now that the best treatment is the primary direct closure in neonatal age, when flexibility of the chest wall is maximal and compression of underlying structures is minimal [1–4, 6, 7]. After the first 3 months of age, the cardiopulmonary system progressively accommodates to the size of the thorax, the chest wall becomes firm, and other more complicated and less satisfying methods must be adopted: sliding or rotating chondrotomies, autologous grafts (costal cartilage, parietal skull, tibial periosteum), and use of prosthetic materials (stainless steel mesh, Marlex, acrylic, silicone elastomer, and Teflon) [1, 4, 6].

Our two cases confirm that the BF should be repaired early in life, when the thorax is compliant and the primary closure is generally safe and relatively easy.

	References

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1. Shamberger R.C., Welch K.J. Sternal defects. Pediatr Surg Int 1990;5:156-164.
2. Greenberg B.M., Becker J.M., Pletcher B.A. Congenital bifid sternum. Plast Reconstr Surg 1991;11:886-889.
3. Mogilner J., Siplovich L., Bar-Ziv J., Mares A.J. Surgical management of the cleft sternum. J Pediatr Surg 1988;10:889-891.
4. Ravitch M.M. The chest wall. In: Welch K.J., Randolph J.G., Ravitch M.M., O’Neill J.A., Rowe M.I., eds. Pediatric surgery. Chicago: Year Book, 1986:563-567.
5. Mahone P.R., Sherer D.M., D’Angio C., Woods J.R. Prenatal ultrasonographic findings associated with wide clefting of the upper two thirds of the fetal sternum. Am J Obstet Gynecol 1992;4:1219-1221.
6. Valla J.S., Bechraoui T., Belghith M., Daoud N., Grinda A. Fissure sternale congénitale. Fermeture par greffon périosté. Chir Pediatr 1989;30:219-221.[Medline]
7. Knox L., Tuggle D., Knott-Craig C.J. Repair of congenital sternal clefts in adolescence and infancy. J Pediatr Surg 1994;12:1513-1516.
8. Gray S.W., Skandalakis J.E. The anterior body wall. In: Gray S.W., Skandalakis J.E., eds. Embryology for surgeons. Philadelphia: WB Saunders, 1972:387-441.

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