Ann Thorac Surg 2006;82:2310-2311
© 2006 The Society of Thoracic Surgeons
How To Do It
Bridging the Cleft Over the Throbbing Heart
John Mathai, MCha,*,
Vijit Koshy Cherian, MChb,
Jacob Chacko, MCha,
Sudipta Sen, MCha,
Sampath Karl, MCha,
Murugu S. Pandyan, MChb,
Alpha K. Mathew, MChb
a Department of Pediatric Surgery, Christian Medical College & Hospital, Vellore, Tamil Nadu, India
b Department of Cardiothoracic Surgery, Christian Medical College & Hospital, Vellore, Tamil Nadu, India
Accepted for publication February 3, 2006.
* Address correspondence to Dr Mathai, Department of Pediatric Surgery, CMC & H, Vellore, Tamil Nadu, 632004 India (Email: paedsur{at}cmcvellore.ac.in).
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Abstract
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Primary repair of sternal cleft deformities are best achieved in the neonatal period. Conversion of a partial defect to a complete sternal cleft with sternal bar mobilization is able to achieve sternal approximation in most cases. We describe a new technique of fracturing the clavicles in unyielding cases as was our experience in a 6-week-old infant.
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Introduction
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Disorders of ventral midline thoracic fusion result in defects ranging from the lethal ectopia cordis to the benign sternal cleft. They find their first mention in the cuneiform tablets in the royal library at Nineveh [1]. Despite the interest this lesion evokes, it did not lend itself satisfactorily to classification on account of its rarity.
Cleft or bifid sternum was first recognized as a distinct entity by Shamberger and Welch [1]. They succinctly characterized the cleft or bifid sternum as being composed of an orthotopically placed heart, progressively enveloped by an intact pericardium and cleft sternum invested by skin [1, 2].
The exact incidence of this anomaly is difficult to reach, but the best measure of the frequency of its occurrence is about 0.15% of all chest deformities (5,182 patients) seen at a single institution during a 25-year period [2]. Close to 100 cases have been described in the English literature. It is established that repair during early infancy is ideal on account of its ease and good outcome [1–3, 4].
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Technique
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Herein we report a 6-week-old term infant weighing 4.7 kg. On examination of the precordium, a midline U-shaped defect of the sternum sparing the terminal gladiolus and xiphoid was noted. The defect was widest superiorly between the sternoclavicular joints and it measured 4 cm. The paradoxical respiratory excursions and cardiac impulse were readily visible through the normal skin over the defect. The abdomen and cardiorespiratory system were unremarkable on sonogram and echocardiography, respectively.
Intraoperatively a midsagittal incision was made, which extended well beyond the limits of the sternal cleft. The skin flaps were carefully raised without breaching the pericardium and reflected laterally until the appearance of the pectoralis fibers. The pectoralis muscle was then released from its sternal origins bilaterally. The endothoracic fascia deep to the sternal bars was entered by following the pericardium laterally and the pleura was progressively stripped away from the ribs. The periosteum on the anterior surface of the first five ribs and the sternal bars were opened, and sliding chondrotomies [5] were made at the costochondral junctions. A vertical wedge of bone (1 cm long) was removed converting the cleft into a complete one [4, 6].
Attempts to approximate the sternal bars were unsuccessful due to the unyielding sternoclavicular joints, which were beginning to tamponade the heart. This was overcome by fracturing the clavicles at the junction of the medial and lateral two thirds using up-angled Ruskin-Liston bone cutting forceps and co-apting the sternal bars with 2-0 Ethibond (Johnson & Johnson, New Brunswick, NJ) sutures while watching for changes in systemic and airway pressure [6]. The sutures were tied after leaving mediastinal and bilateral pleural drains. Sutures (5-0 Vicryl; Ethicon Sutures, Somerville, NJ) were used to approximate the periosteum and the pectoralis muscles in the midline over a vacuum drain. Interrupted Ethilon sutures (Ethicon Sutures, Somerville, NJ) were used for skin closure. The infant was electively ventilated for 4 days and was extubated after withdrawing supports.
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Comment
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Sternal clefts are classified as being complete or partial [1–2]. The partial deformity involving the upper sternum is the most common. It is frequently an isolated entity, and typically U-shaped, with the cleft extending until the fourth costal cartilage [3].
Although most cases remain asymptomatic and long-term survival has been reported in the uncorrected [1, 3], surgery is advocated to offer protection to the heart, for allaying parental anxiety, and for preventing recurrent respiratory infections due to the paradoxical respiratory movements. The first true bony repair is credited to Burton [1]. It is well established that primary repair is best accomplished in the neonatal period [1–3, 4] capitalizing on the pliability of the chest wall.
The essential steps of primary repair in infancy consist of conversion of a partial cleft into a total one [6] by the removal of a sagittal wedge of lower sternum preserving the xiphoid and gaining a fresh edge [4, 6] for direct approximation. If sternal approximation without cardiac compression is impossible, the transposition of the sternal bars toward the midline is facilitated by the sliding chondrotomies of Sabiston [5]. Trial juxtaposition of the sternal bars while observing the heart rate, blood pressure, central venous pressure, electrocardiogram, and oxygen saturation for cardiorespiratory compromise is recommended before finalizing the apposition [4].
We noticed that the sternoclavicular junctions continued to prevent the approximation of the upper sternum as was the case. This could be overcome by fracturing the clavicles just lateral to the protuberant medial third. We believe this achieves adequate sternal length superiorly, with no distraction, and it is possible to prevent herniation through the thoracic inlet without relying on the approximation of the sternal ends of the strap muscles [2]. Fracturing the clavicles did not significantly contribute to the duration or to the blood loss associated with the procedure, nor was any pseudo paralysis of the upper limbs noticed at discharge.
Release of the pectoralis muscle from the sternal bars facilitates sternal bar approximation. Retention of the periosteum around the rigid structures divided, possibly helps to maintain the reduction and later promotes union.
If at any stage cardiac embarrassment is imminent, primary repair must be abandoned for more extensive repairs using autologous free grafts, myocutaneous flaps, or prosthetic repairs, accepting the poor aesthetic appeal, limited scope for remodeling with growth and the inherent risk of infection and extrusion especially associated with the latter.
We believe that this technique of fracturing the clavicles, which extends the possibility of primary repair, has not been described thus far. Although disarticulation of sternoclavicular joints [2] has been described in a similar operative scenario, the late complication of disarticulation could be painful shoulder abduction with a propensity for recurrent dislocation and an unsightly deformity. Although Rockwood has proposed an operative repair for sternoclavicular dislocations, it is fraught with risk to the great vessels by implants, both at surgery and during the holding period. Worman, Leagus, and Omer suggest that the incidence of significant complication may approach 25% after sternoclavicular procedures [7].
Clavicular fractures readily unite in infancy, and the risk of painless nonunion is only 0.1%. Hence we believe that this new technique of fracturing the clavicles (Fig 1) to approximate the superior part of the sternal bars will extend the scope of primary repair in infancy without appreciably increasing the morbidity [8].
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Fig 1. Osteotomies in the sternal bar, clavicle, and ribs are depicted. Inset showing the approximation being achieved. (The roman numerals denote the order of ribs as they would appear.)
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References
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- Shamberger RC, Welch KJ. Sternal defects Paediatr Surg Int 1990;5:156-164.
- Acastello E, Majluf R, Garrido P, et al. Sternal cleft: a surgical opportunity J Paediatr Surg 2003;38:178-183.[Medline]
- Firmin RK, Fragomeni LS, Lennox SC. Complete cleft sternum Thorax 1980;35:303-306.[Abstract/Free Full Text]
- Greenberg BM, Becker JM, Pletcher BA. Congenital bifid sternum: repair in early infancy and literature review Plast Reconstr Surg 1991;88:886-889.[Medline]
- Sabiston Jr DC. The surgical management of congenital bifid sternum with partial ectopia cordis J Thorac Surg 1958;35:118-122.[Medline]
- Salley RK, Stewart S. Superior sternal cleft: repair in newborn Ann Thorac Surg 1985;39:582-583.[Abstract/Free Full Text]
- In: Dlabach JA, Corckarell JR, Canale ST, editors. 10th ed. Campbell’s operative orthopaedics. vol 3. Mosby Publications; 2003. pp. 3177.
- In: Rockwood CA, Bucholz RW, Heckman JD, Green DP, editors. 5th ed.. Fractures in adults. vol 2. Lippincott Williams & Wilkins; 2001. pp. 1282.
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Abstract
Introduction
