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Autologous repair of isolated complete sternal cleft in an adolescent
Journal of Pediatric Surgery (2009) 44, 2414–2416
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Autologous repair of isolated complete sternal cleft in an adolescent Vinay Jadhav, Sanjay Rao ⁎, Ashley D'Cruz Department of Pediatric Surgery, Narayana Hrudayalaya, Anekal Taluk, Bangalore 560099, India Received 12 August 2009; revised 12 September 2009; accepted 18 September 2009
Key words: Sternal clefts; Costal cartilage; Autologous repair; PHACE; Facial hemangioma
Abstract Isolated sternal clefts are rare congenital anomalies. Complete clefts are rarer still with only 23 cases being reported. We present an adolescent girl with a complete sternal cleft. The cleft was treated with autologous anterior perichondrial flaps and pectoralis advancement flaps. © 2009 Elsevier Inc. All rights reserved.
Isolated sternal clefts are rare anomalies. Complete clefts are rarer still with only 23 cases being reported [1]. We present an adolescent girl with a complete sternal cleft. The cleft was treated with autologous anterior perichondrial flaps and pectoralis advancement flaps.
1. Case report A 12-year-old girl presented with abnormal midline chest defect noted since birth. Physical examination revealed parchment-like skin covering a midline complete sternal cleft. Cardiac pulsations were visible in the defect. A vertical congenital scar extended from umbilicus to the root of the neck (Fig. 1). The rest of the chest wall and abdomen was normal. The child had a pale red skin lesion over the left side of the face and neck; this had appeared at 2 months of life and resolved spontaneously with scar at 3 years. This was most likely a superficial hemangioma. ⁎ Corresponding author. Tel.: +91 80 27835000x2139; fax: +91 80 27832648. E-mail address: [email protected] (S. Rao). 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2009.09.019
A computed tomography scan showed the left and right manubrium separated by a distance of 2.8 cm. The sternum below the level of the manubrium was completely absent, and the distance between the costochondral junctions was 3.1 cm. The lungs, heart, and great vessels were normal. An elliptical skin incision was made around the median scar. This scar was then excised, carefully separating it from the underlying pericardium. The sternal defect was exposed, and edges of the 2 hemisternum (sternal bars) were defined by separating it from the pericardium. Pectoralis major muscle flaps were raised to expose the costochondral junctions. The anterior perichondrium of the hemisternum was incised laterally, and the perichondrial flaps were raised. These perichondrial flaps were then reflected medially and sutured to each other in the midline. This effectively covered the pericardium and created a bed bridging the sternal cleft. Three peristernal stainless steel wires were placed. Rib grafts harvested from the chondral portion of the fifth rib on either side were tailored and laid on the perichondrial bed and fixed to the sternal edge with few interrupted sutures. Steel wires were then approximated and the 2 hemisternum brought together (Fig. 2). Cardiorespiratory function was monitored carefully during and after the sternal approximation. Previously mobilized pectoralis muscle flaps were sutured
Autologous repair of isolated complete sternal cleft
Fig. 1 Complete sternal cleft with a fibrous band extending up to the umbilicus. The sternal defect was covered by parchment-like skin, and the cardiac pulsations were visible.
to each other in the midline. Skin was closed over a suction drain. Postoperative recovery was uneventful. The postoperative appearance was satisfactory (Fig. 3).
2. Discussion Sternal clefts are rare clinical entities that may be an isolated disorder or a part of the pentalogy of Cantrell, which
2415
Fig. 3
Postoperative appearance.
includes anterior abdominal wall defect (omphalocele), sternal, pericardial, and diaphragmatic defects with intracardiac abnormalities. Isolated sternal cleft is a rare congenital anomaly, usually not associated with cardiac defects. Children in this group have an orthotopic heart, normal skin coverage, and an intact pericardium. These fusion abnormalities may be classified as complete or incomplete depending on the degree of separation. Partial clefts can be further classified into superior, inferior, and central clefts. Several distinct somatic
Fig. 2 Reconstruction steps. A, Incisions made in anterior perichondrium at the lateral edge of each hemisternum to create a medially based perichondrial flap. The anterior perichondrial flaps rotated medially and (B) sutured together in midline. (C) Fifth costal cartilage grafts harvested. (D) Costal cartilage grafts placed over perichondrial bed and held in place with few sutures. (E) Steel wires used to reinforce the sternum.
2416 associations are seen, including band-like scars extending from the umbilicus to the inferior aspect of the cleft or extending from superior sternal defect to the neck. Embryologically, both sternum and pectoralis muscle arise from lateral plate mesoderm. At 6 weeks, the migrating cells form 2 bands on either side of the anterior chest wall finally fusing by the 10th week [2]. Sternal clefts result from failure of fusion of lateral mesodermal sternal bars by the eighth week of gestation. Alcohol, methyl cobalamin deficiency, and disruption of the Hox-4 gene have been postulated as the causes for this event in murine models [3]. Fisher first reported an unexplained association of sternal clefts with cervicofacial hemangiomas in 1879. The acronym PHACE has been recently proposed to describe the association of Posterior fossa malformations, Hemangiomas, Arterial anomalies, Coarctation of the aorta, Eye abnormalities, and sternal nonunion [4]. These defects are asymptomatic and have a female preponderance. Uncorrected, a pectus excavatum–type defect may develop. Maier and Bortone [5] achieved the first primary closure of sternal cleft in 1949 in a 6-week-old infant:: and stressed the importance of early repair in infancy, when the chest is most flexible. The essential steps of primary repair in infancy consist of conversion of a partial cleft into a total one by the removal of a sagittal wedge of lower sternum preserving the xiphoid and gaining a fresh edge for direct approximation [6,7]. In children and adolescents, surgical repair is feasible but requires additional measures. These methods include sliding chondrotomies described by Sabiston [8], where bilateral oblique incisions are made through the costal cartilages to produce greater length and allow midline approximation of the sternal halves. Other methods include the door wing plasty of Meissner [9] where cartilages are divided laterally and swung medially to cover the defect. Acastello et al [10] have described primary closure of the defect with partial resection of the first 3 costal cartilages and disruption of the sternoclavicular junction to permit mobilization of the sternal bars for midline approximation. However, the late complication of disarticulation includes painful shoulder abduction with a propensity for recurrent dislocation and unsightly deformity. Mathai and Cherian [11] have reported successful repair of the defect by
V. Jadhav et al. primary approximation after fracturing the clavicles and hence avoiding the complications of disarticulation. Various prosthetic grafts such as Teflon, polypropylene, silicone, and titanium have been used in adults to cover a wider defect, but these materials provide a rigid frame and are associated with increased infection and synthetic reaction [12]. Autologous repair with rib grafts and anterior perichondrial flaps from the sternal bars is safe, easy, and effective and can be accomplished without the use of prosthetic material and its potential accompanying complications. It also provides sufficient strength and rigidity to the neosternum, with excellent cosmetic results [13,14].
References [1] Shamberger R, Welch KJ. Sternal defects. Pediatr Surg Int 1990;5: 156-64. [2] Firmin RK, Fragomeni LS, et al. Complete cleft sternum. Thorax 1980;35:303-6. [3] Ramirez-solis AR, et al. Hox-4 (Hox-2.6 ) mutant mice show homeotic transformation of a cervical vertebra and defects in the closure of the sternal rudiments. Cell 1993;73:279-94. [4] Frieden IJ, Resse V, Cohen D. PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of aorta and cardiac and eye defects. Arch Dermatol 1996;132:307. [5] Maier HC, Bortone F. Complete failure of sternal fusion with herniation of pericardium. J Thorac Surg 1949;18:851-9. [6] Greenberg BM, Becker JM, et al. Congenital bifid sternum: repair in early infancy and literature review. Plast Reconstr Surg 1991;88: 886-9. [7] Salley RK, Stewart S. Superior sternal cleft: repair in the newborn. Ann Thoracic Surg 1985;39:582-3. [8] Sabiston Jr DC. The surgical management of congenital bifid sternum with partial ectopia cordis. J Thorac Surg 1958;35:118-22. [9] Meissner F. Sterni congenita. Zentralbl Chir 1964;89:1832-9. [10] Acastello E, Majluf R, et al. Sternal cleft: a surgical opportunity. J Pediatr Surg 2003;38(2):178-83. [11] Mathai J, Cherian VK, et al. Bridging the cleft over the throbbing heart. Ann Thorac Surg 2006;82:2310-1. [12] Luthra S, et al. Sternal cleft—a natural absurdity or a surgical opportunity. J Pediatr Surg 2007;42:582-4. [13] De capos JR, Filomeno LT, et al. Repair of congenital sternal cleft in infants and adolescents. Ann Thorac Surg 1998;66(4):1151-4. [14] Knox L, Tuggle D, et al. Repair of congenital sternal defects in adolescence and infancy. J Pediatr Surg 1994;29:1513-6.
www.elsevier.com/locate/jpedsurg
Autologous repair of isolated complete sternal cleft in an adolescent Vinay Jadhav, Sanjay Rao ⁎, Ashley D'Cruz Department of Pediatric Surgery, Narayana Hrudayalaya, Anekal Taluk, Bangalore 560099, India Received 12 August 2009; revised 12 September 2009; accepted 18 September 2009
Key words: Sternal clefts; Costal cartilage; Autologous repair; PHACE; Facial hemangioma
Abstract Isolated sternal clefts are rare congenital anomalies. Complete clefts are rarer still with only 23 cases being reported. We present an adolescent girl with a complete sternal cleft. The cleft was treated with autologous anterior perichondrial flaps and pectoralis advancement flaps. © 2009 Elsevier Inc. All rights reserved.
Isolated sternal clefts are rare anomalies. Complete clefts are rarer still with only 23 cases being reported [1]. We present an adolescent girl with a complete sternal cleft. The cleft was treated with autologous anterior perichondrial flaps and pectoralis advancement flaps.
1. Case report A 12-year-old girl presented with abnormal midline chest defect noted since birth. Physical examination revealed parchment-like skin covering a midline complete sternal cleft. Cardiac pulsations were visible in the defect. A vertical congenital scar extended from umbilicus to the root of the neck (Fig. 1). The rest of the chest wall and abdomen was normal. The child had a pale red skin lesion over the left side of the face and neck; this had appeared at 2 months of life and resolved spontaneously with scar at 3 years. This was most likely a superficial hemangioma. ⁎ Corresponding author. Tel.: +91 80 27835000x2139; fax: +91 80 27832648. E-mail address: [email protected] (S. Rao). 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2009.09.019
A computed tomography scan showed the left and right manubrium separated by a distance of 2.8 cm. The sternum below the level of the manubrium was completely absent, and the distance between the costochondral junctions was 3.1 cm. The lungs, heart, and great vessels were normal. An elliptical skin incision was made around the median scar. This scar was then excised, carefully separating it from the underlying pericardium. The sternal defect was exposed, and edges of the 2 hemisternum (sternal bars) were defined by separating it from the pericardium. Pectoralis major muscle flaps were raised to expose the costochondral junctions. The anterior perichondrium of the hemisternum was incised laterally, and the perichondrial flaps were raised. These perichondrial flaps were then reflected medially and sutured to each other in the midline. This effectively covered the pericardium and created a bed bridging the sternal cleft. Three peristernal stainless steel wires were placed. Rib grafts harvested from the chondral portion of the fifth rib on either side were tailored and laid on the perichondrial bed and fixed to the sternal edge with few interrupted sutures. Steel wires were then approximated and the 2 hemisternum brought together (Fig. 2). Cardiorespiratory function was monitored carefully during and after the sternal approximation. Previously mobilized pectoralis muscle flaps were sutured
Autologous repair of isolated complete sternal cleft
Fig. 1 Complete sternal cleft with a fibrous band extending up to the umbilicus. The sternal defect was covered by parchment-like skin, and the cardiac pulsations were visible.
to each other in the midline. Skin was closed over a suction drain. Postoperative recovery was uneventful. The postoperative appearance was satisfactory (Fig. 3).
2. Discussion Sternal clefts are rare clinical entities that may be an isolated disorder or a part of the pentalogy of Cantrell, which
2415
Fig. 3
Postoperative appearance.
includes anterior abdominal wall defect (omphalocele), sternal, pericardial, and diaphragmatic defects with intracardiac abnormalities. Isolated sternal cleft is a rare congenital anomaly, usually not associated with cardiac defects. Children in this group have an orthotopic heart, normal skin coverage, and an intact pericardium. These fusion abnormalities may be classified as complete or incomplete depending on the degree of separation. Partial clefts can be further classified into superior, inferior, and central clefts. Several distinct somatic
Fig. 2 Reconstruction steps. A, Incisions made in anterior perichondrium at the lateral edge of each hemisternum to create a medially based perichondrial flap. The anterior perichondrial flaps rotated medially and (B) sutured together in midline. (C) Fifth costal cartilage grafts harvested. (D) Costal cartilage grafts placed over perichondrial bed and held in place with few sutures. (E) Steel wires used to reinforce the sternum.
2416 associations are seen, including band-like scars extending from the umbilicus to the inferior aspect of the cleft or extending from superior sternal defect to the neck. Embryologically, both sternum and pectoralis muscle arise from lateral plate mesoderm. At 6 weeks, the migrating cells form 2 bands on either side of the anterior chest wall finally fusing by the 10th week [2]. Sternal clefts result from failure of fusion of lateral mesodermal sternal bars by the eighth week of gestation. Alcohol, methyl cobalamin deficiency, and disruption of the Hox-4 gene have been postulated as the causes for this event in murine models [3]. Fisher first reported an unexplained association of sternal clefts with cervicofacial hemangiomas in 1879. The acronym PHACE has been recently proposed to describe the association of Posterior fossa malformations, Hemangiomas, Arterial anomalies, Coarctation of the aorta, Eye abnormalities, and sternal nonunion [4]. These defects are asymptomatic and have a female preponderance. Uncorrected, a pectus excavatum–type defect may develop. Maier and Bortone [5] achieved the first primary closure of sternal cleft in 1949 in a 6-week-old infant:: and stressed the importance of early repair in infancy, when the chest is most flexible. The essential steps of primary repair in infancy consist of conversion of a partial cleft into a total one by the removal of a sagittal wedge of lower sternum preserving the xiphoid and gaining a fresh edge for direct approximation [6,7]. In children and adolescents, surgical repair is feasible but requires additional measures. These methods include sliding chondrotomies described by Sabiston [8], where bilateral oblique incisions are made through the costal cartilages to produce greater length and allow midline approximation of the sternal halves. Other methods include the door wing plasty of Meissner [9] where cartilages are divided laterally and swung medially to cover the defect. Acastello et al [10] have described primary closure of the defect with partial resection of the first 3 costal cartilages and disruption of the sternoclavicular junction to permit mobilization of the sternal bars for midline approximation. However, the late complication of disarticulation includes painful shoulder abduction with a propensity for recurrent dislocation and unsightly deformity. Mathai and Cherian [11] have reported successful repair of the defect by
V. Jadhav et al. primary approximation after fracturing the clavicles and hence avoiding the complications of disarticulation. Various prosthetic grafts such as Teflon, polypropylene, silicone, and titanium have been used in adults to cover a wider defect, but these materials provide a rigid frame and are associated with increased infection and synthetic reaction [12]. Autologous repair with rib grafts and anterior perichondrial flaps from the sternal bars is safe, easy, and effective and can be accomplished without the use of prosthetic material and its potential accompanying complications. It also provides sufficient strength and rigidity to the neosternum, with excellent cosmetic results [13,14].
References [1] Shamberger R, Welch KJ. Sternal defects. Pediatr Surg Int 1990;5: 156-64. [2] Firmin RK, Fragomeni LS, et al. Complete cleft sternum. Thorax 1980;35:303-6. [3] Ramirez-solis AR, et al. Hox-4 (Hox-2.6 ) mutant mice show homeotic transformation of a cervical vertebra and defects in the closure of the sternal rudiments. Cell 1993;73:279-94. [4] Frieden IJ, Resse V, Cohen D. PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of aorta and cardiac and eye defects. Arch Dermatol 1996;132:307. [5] Maier HC, Bortone F. Complete failure of sternal fusion with herniation of pericardium. J Thorac Surg 1949;18:851-9. [6] Greenberg BM, Becker JM, et al. Congenital bifid sternum: repair in early infancy and literature review. Plast Reconstr Surg 1991;88: 886-9. [7] Salley RK, Stewart S. Superior sternal cleft: repair in the newborn. Ann Thoracic Surg 1985;39:582-3. [8] Sabiston Jr DC. The surgical management of congenital bifid sternum with partial ectopia cordis. J Thorac Surg 1958;35:118-22. [9] Meissner F. Sterni congenita. Zentralbl Chir 1964;89:1832-9. [10] Acastello E, Majluf R, et al. Sternal cleft: a surgical opportunity. J Pediatr Surg 2003;38(2):178-83. [11] Mathai J, Cherian VK, et al. Bridging the cleft over the throbbing heart. Ann Thorac Surg 2006;82:2310-1. [12] Luthra S, et al. Sternal cleft—a natural absurdity or a surgical opportunity. J Pediatr Surg 2007;42:582-4. [13] De capos JR, Filomeno LT, et al. Repair of congenital sternal cleft in infants and adolescents. Ann Thorac Surg 1998;66(4):1151-4. [14] Knox L, Tuggle D, et al. Repair of congenital sternal defects in adolescence and infancy. J Pediatr Surg 1994;29:1513-6.