Surgical reconstruction for absence of sternum and pericardium in a newborn
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《美国医学杂志》
1 Pediatric Cardiology, Indraprastha Apollo Hospital, Sarita Vihar, New Delhi, India
2 Dept of Plastic Surgery, Indraprastha Apollo Hospital, Sarita Vihar, New Delhi, India
3 Cardiothoracic Surgery, Indraprastha Apollo Hospital, Sarita Vihar, New Delhi, India
4 Neonatology, Indraprastha Apollo Hospital, Sarita Vihar, New Delhi, India
5 Pediatrics (Prevat Hospital), Indraprastha Apollo Hospital, Sarita Vihar, New Delhi, India
Abstract
We report the surgical management of a 3 day old baby with absent sternum and pericardium with impending rupture of the skin cover. The repair described is innovative and may be used in place of primary repair by bringing the ribs together. Review of embryology and various modalities of closure are provided.
Keywords: Absent sternum; Pectoralis flap; Absent pericardium
Absent sternum is a potentially life threatening congenital midline defect. This anomaly may be associated with developmental anomaly of other midline visceral structures. Limited experience has been reported with successful management of this condition.[1] The management of this condition is challenging with respect to appropriate time for intervention and surgical reconstruction. We report the successful outcome after surgical reconstruction for complete absence of sternum associated with deficiency of pericardium in a newborn.
Case Report
Baby S was born via a lower segment caesarian section to a young primigravida mother after treatment for infertility. Antenatal sonography had failed to detect this anomaly. The attending pediatrician noted a midline defect in the form of complete absence of sternum, parchment like membranous abnormal skin covering an oval defect measuring 6 × 4 cm with intermittent herniation of cardiac structures on crying. The baby appeared normal apart from this defect Figure1. A chest X-ray appeared normal and an echocardiogram confirmed normal cardiac anatomy with absence of pericardium underlying this midline defect.
The child was transferred to our tertiary level hospital for further management. Cranial and abdominal ultrasounds were reported to be normal. A joint surgical evaluation by the plastic and cardiac surgical teams lead to the decision to undertake surgical reconstruction to prevent potential life threatening complication secondary to rupture of the membrane and evisceration of cardiac structures.
The excision of the membranous covering over the defect and dissection of the edges of the wound led to exposure of the edges of pericardium. The pericardium was mobilized all along the edges and sutured in midline without tension. The second layer was provided by a prolene mesh, which was hitched with prolene sutures to the proximal ends of the ribs on both sides thereby providing cover as well as some stability to the chest wall. Following this, bilateral pectoralis major myocutaneous units were mobilized. These were then brought to the midline and provided the final layer below the skin. Primary closure was not considered feasible by the operating surgeons due to the extent of the defect at the cranial end. The post-operative recovery was significantly smooth and the baby was discharged after a week. Three months after surgery, she remains well with a healed wound and stable anterior chest.
Discussion
The sternum is formed from paired lateral concentrations of mesenchymal cells in the thoracic region of the embryo, which can be identified after the fifth week. The process of fusion begins at the cephalic end and progresses caudally. The variable degrees of fusion occur in the cephalic portion of the thorax resulting clinically into bifid sternum. When there is total lack of fusion and the sternal anlages are widely separated, absence of sternum with associated mesodermal structures presents itself in a newborn baby. The antenatal diagnosis of this condition is possible, but was missed in the present case.
Isolated sternal defect is an extremely rare condition; the estimated incidence is unknown, although it is rarer than Cantrell's Pentology .The incidence of Cantrell's Pentology has been estimated to be around 1 in 100,000 live births[2] and includes defect of skin, sternum, diaphragm, pericardium and intracardiac defects.
The age at which this condition has been repaired has varied widely with complex repair techniques being defined in adolescent age group, when they present late.[3],[4],[5] The timing and indications for neonatal intervention have been changing with advancing level of neonatal critical care. The index patient is one of the youngest to have undergone successful repair as a newborn.
The principles of surgical reconstruction are based on the anatomy of the defect, associated structural abnormality, and the age of the patient.[6] It has generally been agreed that the correction of the sternal defect should be done at its earliest, to utilize the pliant nature of the neonatal rib cage. The size and nature of the defect along with the age of the patient governs the technique of closure.
A number of methods for repair of absent sternum have been reported, including primary approximation, sliding or rotating chondrotomies, and the use of prosthetic grafts or of flaps of bone, cartilage, autogenous tissue, or pectoralis major muscle.[7], [8], [9]
In neonates, the sternal bars can be easily approximated by simple suture, due to the flexibility of the cartilaginous thorax. This also enables approximation of the sternal bars with less danger of cardiac compression.[1]
The only other associated anomaly in this baby was a deficient pericardium, which could be easily closed in midline after adequate mobilization. The long-term outlook in the baby is promising in view of the absence of any other major associated anomaly. We recognize the need for close evaluation of pulmonary function as the baby grows. The successful surgical outcome in this baby is the result of continually evolving surgical and neonatal care in our part of the worldFigure2.
References
1. de Campos JR, Filomeno LT, Fernandez A, Ruiz RL, Minamoto H, Werebe Ede C, Jatene FB. Repair of congenital sternal cleft in infants and adolescents. Ann Thorac Surg 1998; 66 : 1151-1154.
2. nderoπlu L, Baykal C, Tulunay G, Talim B, Kale G. Prenatal diagnosis of Cantrell's pentalogy: a case report. Turk J Pediatr 2003; 45 : 357-358.
3. Alphonso N, Venugopal PS, Deshpande R, Anderson D. Complete thoracic ectopia cordis. Eur J Cardiothorac Surg 23(3) : 426-428.
4. Chaukar AP, Mandke NV, Mehta SS, Pandey SR, Parulkar GB, Sen PK. Surgical correction of absent sternum with homologous rib graft. J Postgrad Med 1980; 26 : 180-185.
5. Spitz L, Bloom R, Milner S, Levin SE. Combined anterior abdominal wall, sternal, diaphragmatic, pericardial, and intracardiac defects: a report of five cases and their management. J Pediatr Surg 1990; 10(4) : 491-496.
6. Ravitch MM. Disorders of the sternum and the thoracic wall. In Sabiston DC Jr, Spencer FC eds. Gibbon's Surgery of the Chest. 4th edn. Philadelphia; WB Saunders, 1983; 318-360.
7. Samarrai AA, Charmockly HA, Attra AA. Complete cleft sternum: classification and surgical repair. Int Surg 1985; 70 : 71-73.
8. Sarper A, Oz N, Arslan G, Demircan A. Complete Congenital Sternal Cleft Associated with Pectus Excavatum. Tex Heart Inst J 2002; 29 (3): 206-209.
9. 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.(Kohli Vikas, Nooreyazdan )
2 Dept of Plastic Surgery, Indraprastha Apollo Hospital, Sarita Vihar, New Delhi, India
3 Cardiothoracic Surgery, Indraprastha Apollo Hospital, Sarita Vihar, New Delhi, India
4 Neonatology, Indraprastha Apollo Hospital, Sarita Vihar, New Delhi, India
5 Pediatrics (Prevat Hospital), Indraprastha Apollo Hospital, Sarita Vihar, New Delhi, India
Abstract
We report the surgical management of a 3 day old baby with absent sternum and pericardium with impending rupture of the skin cover. The repair described is innovative and may be used in place of primary repair by bringing the ribs together. Review of embryology and various modalities of closure are provided.
Keywords: Absent sternum; Pectoralis flap; Absent pericardium
Absent sternum is a potentially life threatening congenital midline defect. This anomaly may be associated with developmental anomaly of other midline visceral structures. Limited experience has been reported with successful management of this condition.[1] The management of this condition is challenging with respect to appropriate time for intervention and surgical reconstruction. We report the successful outcome after surgical reconstruction for complete absence of sternum associated with deficiency of pericardium in a newborn.
Case Report
Baby S was born via a lower segment caesarian section to a young primigravida mother after treatment for infertility. Antenatal sonography had failed to detect this anomaly. The attending pediatrician noted a midline defect in the form of complete absence of sternum, parchment like membranous abnormal skin covering an oval defect measuring 6 × 4 cm with intermittent herniation of cardiac structures on crying. The baby appeared normal apart from this defect Figure1. A chest X-ray appeared normal and an echocardiogram confirmed normal cardiac anatomy with absence of pericardium underlying this midline defect.
The child was transferred to our tertiary level hospital for further management. Cranial and abdominal ultrasounds were reported to be normal. A joint surgical evaluation by the plastic and cardiac surgical teams lead to the decision to undertake surgical reconstruction to prevent potential life threatening complication secondary to rupture of the membrane and evisceration of cardiac structures.
The excision of the membranous covering over the defect and dissection of the edges of the wound led to exposure of the edges of pericardium. The pericardium was mobilized all along the edges and sutured in midline without tension. The second layer was provided by a prolene mesh, which was hitched with prolene sutures to the proximal ends of the ribs on both sides thereby providing cover as well as some stability to the chest wall. Following this, bilateral pectoralis major myocutaneous units were mobilized. These were then brought to the midline and provided the final layer below the skin. Primary closure was not considered feasible by the operating surgeons due to the extent of the defect at the cranial end. The post-operative recovery was significantly smooth and the baby was discharged after a week. Three months after surgery, she remains well with a healed wound and stable anterior chest.
Discussion
The sternum is formed from paired lateral concentrations of mesenchymal cells in the thoracic region of the embryo, which can be identified after the fifth week. The process of fusion begins at the cephalic end and progresses caudally. The variable degrees of fusion occur in the cephalic portion of the thorax resulting clinically into bifid sternum. When there is total lack of fusion and the sternal anlages are widely separated, absence of sternum with associated mesodermal structures presents itself in a newborn baby. The antenatal diagnosis of this condition is possible, but was missed in the present case.
Isolated sternal defect is an extremely rare condition; the estimated incidence is unknown, although it is rarer than Cantrell's Pentology .The incidence of Cantrell's Pentology has been estimated to be around 1 in 100,000 live births[2] and includes defect of skin, sternum, diaphragm, pericardium and intracardiac defects.
The age at which this condition has been repaired has varied widely with complex repair techniques being defined in adolescent age group, when they present late.[3],[4],[5] The timing and indications for neonatal intervention have been changing with advancing level of neonatal critical care. The index patient is one of the youngest to have undergone successful repair as a newborn.
The principles of surgical reconstruction are based on the anatomy of the defect, associated structural abnormality, and the age of the patient.[6] It has generally been agreed that the correction of the sternal defect should be done at its earliest, to utilize the pliant nature of the neonatal rib cage. The size and nature of the defect along with the age of the patient governs the technique of closure.
A number of methods for repair of absent sternum have been reported, including primary approximation, sliding or rotating chondrotomies, and the use of prosthetic grafts or of flaps of bone, cartilage, autogenous tissue, or pectoralis major muscle.[7], [8], [9]
In neonates, the sternal bars can be easily approximated by simple suture, due to the flexibility of the cartilaginous thorax. This also enables approximation of the sternal bars with less danger of cardiac compression.[1]
The only other associated anomaly in this baby was a deficient pericardium, which could be easily closed in midline after adequate mobilization. The long-term outlook in the baby is promising in view of the absence of any other major associated anomaly. We recognize the need for close evaluation of pulmonary function as the baby grows. The successful surgical outcome in this baby is the result of continually evolving surgical and neonatal care in our part of the worldFigure2.
References
1. de Campos JR, Filomeno LT, Fernandez A, Ruiz RL, Minamoto H, Werebe Ede C, Jatene FB. Repair of congenital sternal cleft in infants and adolescents. Ann Thorac Surg 1998; 66 : 1151-1154.
2. nderoπlu L, Baykal C, Tulunay G, Talim B, Kale G. Prenatal diagnosis of Cantrell's pentalogy: a case report. Turk J Pediatr 2003; 45 : 357-358.
3. Alphonso N, Venugopal PS, Deshpande R, Anderson D. Complete thoracic ectopia cordis. Eur J Cardiothorac Surg 23(3) : 426-428.
4. Chaukar AP, Mandke NV, Mehta SS, Pandey SR, Parulkar GB, Sen PK. Surgical correction of absent sternum with homologous rib graft. J Postgrad Med 1980; 26 : 180-185.
5. Spitz L, Bloom R, Milner S, Levin SE. Combined anterior abdominal wall, sternal, diaphragmatic, pericardial, and intracardiac defects: a report of five cases and their management. J Pediatr Surg 1990; 10(4) : 491-496.
6. Ravitch MM. Disorders of the sternum and the thoracic wall. In Sabiston DC Jr, Spencer FC eds. Gibbon's Surgery of the Chest. 4th edn. Philadelphia; WB Saunders, 1983; 318-360.
7. Samarrai AA, Charmockly HA, Attra AA. Complete cleft sternum: classification and surgical repair. Int Surg 1985; 70 : 71-73.
8. Sarper A, Oz N, Arslan G, Demircan A. Complete Congenital Sternal Cleft Associated with Pectus Excavatum. Tex Heart Inst J 2002; 29 (3): 206-209.
9. 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.(Kohli Vikas, Nooreyazdan )