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INNOVATION Table of Contents   
Year : 2010  |  Volume : 7  |  Issue : 3  |  Page : 211-213
Sternal cleft repair: A report of two cases and review of literature


1 Department of Pediatric Surgery, SAIMS Medical College, Indore, India
2 Department of Pediatric Surgery, M. Y. Hospital, Indore, India
3 Department of Surgery, SAIMS Medical College, Indore, India

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Date of Web Publication18-Sep-2010
 

   Abstract 

A cleft sternum is a rare congenital anomaly often diagnosed as asymptomatic at birth. Clinical outcome may be unfavourable when an associated anomaly, particularly, an intra cardiac anomaly coexists with the defect. Primary repair should be employed in the neonatal period because the flexibility of the chest wall is maximal and thus the compression of underlying structures is minimal. However, patients with sternal cleft may even present late in the childhood or adolescence period. We herein report two cases of successful repair of sternal clefts with review of the available literature.

Keywords: Sternal cleft; surgery; congenital anomaly

How to cite this article:
Singh S, Lahoti B K, Garge S, Negi A, Jain V. Sternal cleft repair: A report of two cases and review of literature. Afr J Paediatr Surg 2010;7:211-3

How to cite this URL:
Singh S, Lahoti B K, Garge S, Negi A, Jain V. Sternal cleft repair: A report of two cases and review of literature. Afr J Paediatr Surg [serial online] 2010 [cited 2017 Mar 29];7:211-3. Available from: http://www.afrjpaedsurg.org/text.asp?2010/7/3/211/70432

   Introduction Top


Congenital midline cleft of sternum is a rare developmental anomaly, usually occurring as part of a defined syndrome, e.g., Cantrell's Pentalogy. Isolated sternal cleft without any associated anomaly is very uncommon. It results from failed ventral midline fusion of the sternal bands, which normally occurs during the first 3 months of embryonic life. [1],[2] Isolated sternal clefts present in early infancy because of the obvious cosmetic deformity. The anomaly is more common in females and the association with midline supraumblical raphe and facial haemangiomas is well known. Surgical treatment in the newborn period is preferred as the chest wall is pliable and the thoracic viscera accommodate easily to the closure of the chest wall. [3] We present our experience with neonatal surgical repair of subtotal isolated cleft sternum.


   Case Reports Top


Case 1

A 6-month-old male patient was admitted with h/o recurrent episodes of bronchopneumonia with a midline thoracic bulge, evident during expiration and crying. During inspiration, a depression appeared in the same area. On admission the neonate was asymptomatic. Observation and palpation allowed us to diagnose a 4.5-cm wide U-shaped, sternal cleft. The diagnostic image was consistent with the clinical evaluation: a chest roentgenogram showed the typically widely separated clavicles and absence of manubrium and of ossification centres of the sternum [Figure 1]. The electrocardiogram and an echocardiogram of the infant were normal.
Figure 1 :Showing U-shaped defect in the upper sternum

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Subsequently, primary closure of the defect was performed. Through a midline cevicothoracic incision, the sternal bars were dissected free from the insertion of pectoralis major muscles anteriorly and from endothoracic fascia. The resection of inferior sternal portion is done to get "V" transformation of the "U" defect. The pectoral muscles joined in the midline, and the edges of the bars were then sharply freshened, and multiple encircling non-absorbable sutures were passed through intercostal spaces [Figure 2]. A penrose drainage was left under the skin flaps and the skin closure was performed with absorbable subcuticular sutures. No per-operative complication occurred. The patient started feeding at 8 hours postoperatively. The antibiotic was stopped after taking out the drain. The patient was discharged 5 days after repair.
Figure 2 :Intra operative photographs

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Case 2

Our second patient was a 15 day-old, full-term male baby weighing 2.5 kg, who had normal Apgar scores at birth. The parents had a similar complaint of midline chest bulge on crying. He had a midline 'U'-shaped sternal cleft [Figure 3]. There was no other obvious congenital anomaly present. The Doppler echocardiography was normal. The patient underwent sternal repair on the 18 th day of life. The sternal bars were exposed and the pericardium and pleura separated from the undersurface. The edges of the sternal bars were mobilized and approximation was achieved by prolene sutures inserted through the intercoastal spaces. The patient's haemodynamic status was monitored for 5 min before tying the knots. The patient had a haemodynamically uneventful postoperative recovery. At a follow-up visit, the child was seen to develop facial haemangiomas. Oral betamethasone was begun. The patient has completed 1 year of follow-up and is doing well.
Figure 3 :Pre-operative photo of the second case

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   Discussion Top


The first case of sternal cleft was reported in 1740. [4] The first surgical correction was reported by Lannelongue in 1888, [3] but the first successful surgical repair of the anomaly was done by Burten in an 11-week-old child in 1943. [5]

The sternum is derived from paired concentrations of thoracic lateral bands that fuse in a craniocaudal direction by the ninth week of gestation. The superior part of the manubrium arises from three small mesenchymal primordiums, while the sternal bands fuse laterally with the costal cartilage. Disturbances of normal ventral midline thoracic fusion can present as a spectrum of abnormalities, including a prominent suprasternal notch, irregularities in shape of the xiphoid, ectopia cordis, superior sternal cleft or complete sternal cleft. [1],[2],[5]

The resultant anatomical defect produces a concave defect in the sternum covered by skin, with an orthotopic heart and an intact pericardium. The skin moves paradoxically with intrathoracic pressure changes [Figure 1]. The shape of the defect can vary from a narrow 'V' to a wider 'U'-shaped cleft. The sternal defect, if partial, involves the upper sternum and manubrium, in contrast to the sternal defect in thoracic and thoraco-abdominal ectopia cordis, in which the defects are mostly in the lower sternum.

The clinical presentation due to the defect is bulging of the skin with crying as seen in both of our patients. Mostly these infants with ectopia cordis are asymptomatic. Recurrent respiratory tract infection can occur due to reduced aeration of lungs due to associated decrease in the thoracic cavity space.

If the anomaly occurs as a part of upper midline syndrome, e.g., Cantrell's Pentalogy, then associated diaphragmatic and pericardial defects are also present. Fischer [7] reported an association with cervico-facial haemangiomas in 1879 and Ingelrans and Debeugny later reported occurrence of fatal post-operative haemorrhage from presumed haemangiomas of trachea. [8] The timing of appearance of haemangiomas is variable and parents should be informed before surgery. Other associated conditions are supraumblical raphe, band-like scars extending from umbilicus to the inferior aspect of the sternal defect, and rarely a split mandible (gnathoschisis). Intracardiac anomalies are rare in cases of sternal cleft, a distinction from the other sternal defects.

There is a general agreement that the earliest possible primary direct closure is the best treatment. [3],[9] There is a general agreement that the best treatment is primary closure as a neonate. As the infant grows, the cartilage becomes less malleable and the thoracic contents move anteriorly into the defect. [10] A V-shaped defect in the sternum can often be approximated by suture closure with relaxing osteotomy of the edges if needed. The U-shaped defects often require transection of the limbs of the U at the juncture with the normal sternum, followed by suture approximation. [1],[10]

The flexibility of the thoracic cage is inversely proportional to the age of the patient. Repairs done after the age of 3 months have always required more supportive postoperative care with higher incidence of cardiac complication. Beyond 1 year of age, autologous rib/coastal cartilage grafts/prosthetic materials are required. Good mobilization of the two sternal edges is the key to a secure approximation.

Wedge excision of the xiphoid process is required to approximate the lower edges. All the peristernal sutures are placed before tying the knots [Figure 2]. Hemodynamic stability is reassessed by the anaesthetist and after approximation the knots are tied after waiting for a brief period. The patient is to be watched in the early postoperative period. The risk of complication decreases with the early age of repair.

 
   References Top

1.Shamberger RC. Chest wall deformities. In: Ashcraft KW, Editor. Pediatric Surgery. Philadelphia, Pennsylvania: WB Saunders 2000. p. 248-51.  Back to cited text no. 1      
2.Greenberg BM, Becker JM, Pletcher BA. Congenital bifid sternum: Repair in earlyinfancy and literature review. Plast Recons Surg1991;88:886-9.  Back to cited text no. 2      
3.Mogilner J, Siplovich L, Bar-Ziv J, Mares AJ. Surgical Management of cleft sternum. J Pediatr Surg 1988;23:889-91.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]  
4.De Torres JI. Extract of a letter from Jos. Ignat. De Torres, MD to the Royal Society, containing the extraordinary case of the heart of a child turned upside down. London: Philosophical Transactions; 1739:41;776-8.  Back to cited text no. 4      
5.Burten JF. Method of correction of ectopia cordis. Arch Surg 1947;54:79-84.  Back to cited text no. 5      
6.Lannelongue M. De l'ectocardie et de sa cure par l'autoplastie. Ann Medico-Chirurgicales 1888;4:101-7.  Back to cited text no. 6      
7.Fischer H. Fissure sterno congenita mit partieller bauchspalte. Dtsch Z Chir 1879;12:367-9.  Back to cited text no. 7      
8.Ingelrans P, Debeugny P. Case of bifidity of sternum associated with tracheal angiomatosis.Ann Chir Infant 1965;6:123-8.  Back to cited text no. 8  [PUBMED]    
9.Shamberger RC, Welch KJ. Sternal defects. Pediatr Surg Int 1990;5:156-64.   Back to cited text no. 9      
10.Dςmini M, Cupaioli M, Rossi F, Fakhro A, Aquino A, Chiesa PL. Bifid sternum: Neonatal surgical treatment. Ann Thorac Surg 2000;69:267-9.  Back to cited text no. 10      

Top
Correspondence Address:
Saurabh Garge
Department of Pediatric Surgery, SAIMS Medical College, Indore
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0189-6725.70432

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    Figures

  [Figure 1], [Figure 2], [Figure 3]

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