Ann Thorac Surg 2007;84:673-674
© 2007 The Society of Thoracic Surgeons
Case Reports
Superior Sternal Cleft Repair Using Autologous Rib Grafts in an Infant with Complex Congenital Heart Disease
Charles A. Hill, BSa,
Louis C. Argenta, MDb,
Michael Hines, MDa,*
a Department of Cardiothoracic Surgery, Wake Forest University School of Medicine, Winston-Salem, North Carolina
b Department of Plastic and Reconstructive Surgery, Wake Forest University School of Medicine, Winston-Salem, North Carolina
Accepted for publication February 21, 2007.
* Address correspondence to Dr Hines, Wake Forest University School of Medicine, Medical Center Blvd, Winston-Salem, NC 27157-1096 (Email: mhines{at}wfubmc.edu).
 |
Abstract
|
|---|
We report the case of an infant with superior cleft sternum and obstructed type III (infra-diaphragmatic) total anomalous pulmonary venous return. A previously undescribed approach is presented using autologous rib grafts without division of the intact inferior sternum. This technique provides protection of the anterior and superior mediastinum without the use of foreign material, destabilization of the sternum, or compromise of respiratory mechanics.
|
Introduction
|
|---|
Failure of ventral fusion is a rare congenital anomaly with three subtypes: (1) sternal cleft, (2) thoracic ectopia cordis, and (3) thoracoabdominal ectopia cordis. Unlike forms of ectopia cordis, sternal clefts lack the abnormal displacement of the heart, and are most commonly subdivided into partial or complete clefts. Although the rare complete clefts have a total absence of midline fusion, partial clefts involve only a portion of the sternum, and are further subdivided by cleft location. Superior clefts ("bifid" sternum) are the most common clinically significant form [1], and these typically involve complete absence of the manubrium with a "V" or "U"-shaped defect. The inferior sternum is connected by a bony bridge of variable length. Isolated inferior clefts are less common and often combined with defects such as thoracoabdominal ectopia cordis, as seen in the Pentalogy of Cantrell [1]. Although cardiac anomalies are common with inferior clefts, they are unusual with superior clefts. However when present, they create a challenging situation, particularly when the cardiac defect is associated with unstable hemodynamics or pulmonary hypertension and where primary closure of the superior cleft is unfavorable. We report our experience with superior sternal cleft in an infant with complex congenital heart disease associated with severe pulmonary hypertension.
A newborn male was presented to our hospital with mild respiratory distress and cyanosis. Evaluation demonstrated obstructed type III total anomalous pulmonary venous return, a large superior sternal cleft, and a small non-ossified left and free floating hypoplastic right clavicle. The patient went directly to the operating room and under deep hypothermic circulatory arrest the pulmonary venous confluence was anastomosed directly to the posterior wall of the left atrium with a running absorbable suture. The atrial septum was closed with a small polytetrafluoroethylene patch augmenting the small left atrium. Although he was weaned successfully from cardiopulmonary bypass, persistent pulmonary hypertension prevented any sternal repair or closure. The chest was left open for several days, as the pulmonary hypertension resolved with nitric oxide therapy. Although significant improvement was observed, we did not think the child would tolerate primary closure of the cleft with the previously described techniques because of the potential problems with ventilation and worsening of the pulmonary hypertension. On postoperative day 3 the inferior sternal bridge was reapproximated with two number 1 sternal wires, and the muscle and skin were closed over the defect. After later requiring a Ladd procedure for malrotation, he was discharged home on postoperative day 33. The patient recovered from his cardiac operation with complete resolution of his pulmonary hypertension. Because of the significant defect in his upper chest, with his increased activity and ambulation we elected to repair the defect at 16 months of age.
A midline incision was carried down to the level of the inferior sternal bridge. After raising the skin flaps, the pectoralis muscles were mobilized and found to be of normal size. A separate incision was made laterally over the bony portion of the left seventh rib. The periosteum was elevated and the rib was removed and split longitudinally. The free-floating right clavicle was removed for the repair as well. The split rib segments were then sewn in place with absorbable suture to the edges of the sternal bars, nicely covering the defect. The right clavicular remnant was sewn across the most inferior part of the cleft, completing the coverage (Fig 1). The pectoralis muscles were brought over the repair and joined in the midline. Some free strap muscle and the right sternocleidomastoid, which crossed the midline to join the left first rib remnant, was also removed at its abnormal insertion and was brought to the midline and joined to the pectoralis flaps. The wound was then closed primarily leaving a small drain below the skin flaps, above the pectoralis muscles. At 4 years of age, the child is currently doing well without any restrictions on his activities. Physical examination demonstrates some bone absorption within the grafts, but the cleft is filled with dense, partially ossified fibrous tissue providing excellent coverage and protection for the mediastinum with normal respiratory excursion.
View larger version (142K):
[in this window]
[in a new window]
|
Fig 1. (A) Split autologous rib grafts and (B) clavicle remnant bridge the gap of the superior sternal cleft.
|
|
|
Comment
|
|---|
Many techniques have been described for the repair of sternal clefts. Although smaller defects may be closed primarily, larger defects may require the use of various materials to bridge the gap of the cleft. This is particularly true when primary closure may compromise either hemodynamics or ventilation. Mesh or sheets constructed from wire, polypropylene or polytetrofluoroethylene (PTFE) along with stainless steel plates and methyl methacrylate have all been described for these repairs [2]. Ravitch [3] had previously described a technique using rib grafts associated with a teflon covering; however more recently the use of foreign materials has been largely abandoned [4].
The use of synthetic materials can be avoided by making bilateral "sliding chondrotomies," allowing the sternal bars to be freed up and moved medially into position [2]. Knox and colleagues [5] used the autologous rib along with portions of the sternal bridge as grafts to fill the defect. Padalino and colleagues [6] used a similar technique, using portions of the sternal bridge in a "U"-shaped defect, and adding the lateral chondrotomies to complete the repair. However, these techniques necessitate destabilization of the sternum and reduce the thoracic volume that may interfere with respiratory mechanics [4].
Other methods of superior sternal cleft repair have been described; however few address the issue of cardiac defects and the potential hemodynamic instability associated with primary closure. Most authors agree that repair during the neonatal period is preferred in the case of an isolated sternal cleft defect, because a primary repair is usually possible due to the compliance of the cartilaginous rib cage. However, in older patients and patients with hemodynamic susceptibility to increasing thoracic pressure or reduced thoracic volumes, an immediate or primary closure may not be possible [6]. Reports of concomitant repair of a significant congenital heart defect all used bilateral chondrotomies [6, 7]. In our case, the severe pulmonary hypertension required delayed sternal closure and prohibited any concurrent repair, particularly any method that would potentially reduce intrathoracic volume and increase pulmonary vascular resistance.
Our technique bridges the superior defect and uses only autologous tissue, minimizing any negative effect on respiratory mechanics or cardiovascular function, and avoiding the potential complications of foreign materials while allowing for future growth. Bridging with split autologous rib grafts also provides adequate protection to the heart and great vessels, eliminating the need for more complicated and destabilizing techniques that may require 6 to 8 months for complete healing and rib regeneration [6]. This technique is particularly suited to situations in which early or concomitant repair is contraindicated by complicated congenital heart defects, particularly with associated pulmonary hypertension.
|
References
|
|---|
- Shamberger RC. Chest wall deformitiesIn: Shields TW, editor. General thoracic surgery. 4th ed.. Malvern, PA: Williams Wilkins; 1994. pp. 546-557.
- Groner JI. Ectopia cordis and sternal defectsIn: Ziegler MM, Azizkhan RG, Weber TR, editors. Operative pediatric surgery. New York, NY: McGraw-Hill; 2003. pp. 279-293.
- Ravitch MM. Disorders of the sternum and thoracic wallIn: Sabiston Jr DC, Spencer FC, editors. Gibbon’s surgery of the chest. 4th ed.. Philadelphia, PA: Saunders; 1983. pp. 318-360.
- Suri RK, Sharma RK, Jha NK, Sharma BK. Complete congenital sternal cleft in an adult: repair by autogenous tissues Ann Thorac Surg 1996;62:573-575.[Abstract/Free Full Text]
- Knox L, Tuggle D, Knott-Craig CJ. Repair of congenital sternal clefts in adolescence and infancy J Ped Surg 1994;29:1513-1516.[Medline]
- Padalino MA, Zanon GF, Migneco F, Rubino MG, Fusaro F, Stellin G. Surgical repair of incomplete cleft sternum and cardiac anomalies in early infancy Ann Thorac Surg 2006;81:2291-2294.[Abstract/Free Full Text]
- Bove T, Goldstein JP, Viart P, Deuvaert FE. Combined repair of upper sternal cleft and tetralogy of Fallot in an infant Ann Thorac Surg 1997;64:561-562.[Abstract/Free Full Text]
Top
Abstract
Introduction
