CASE REPORT
Immediate Operation for Ectopia Cordis A. F. Jones, M.D., R. L. McGrath, M.D., S. M. Edwards, M.D., and J. R. Lilly, M.D.
ABSTRACT Forty-one infants with thoracoabdominal ectopia cordis have been reported to date; 9 of them survived. Among the patients with an omphalocele as the abdominal wall defect, however, only 2 survived. Death in this group of patients occurred almost exclusively as a consequence of either attempted coverage of the heart or secondary to the associated intracardiac lesion. We report here the case of a patient recently treated in whom coverage with a temporary Silastic prosthesis protected the exposed heart from infection and rupture of the sac, thus permitting full cardiac investigation before operation.
Thoracoabdominal ectopia cordis is a constellation of five malformations: midline supraumbilical abdominal wall defect, defect of the lower sternum, deficiency of the anterior diaphragm, deficiency of the diaphragmatic pericardium, and intracardiac defect [l]. The syndrome was reviewed in 1972 by Toyama [ 6 ] , who called attention to the poor prognosis. Of 41 patients reported, only 9 experienced longterm survival [5-71.* When the abdominal wall defect was an omphalocele, the outlook was even more dismal because of the additional technical problems imposed by providing coverage for the exposed heart. If the technical difficulties can be surmounted, the ultimate prognosis depends on the severity of the cardiac malformation. Consequently, early and precise delineation of the intracardiac lesion and careful consideration to its repair are of crucial importance. In the past, the urgency to provide coverage of the exposed heart resulted in postponement of the cardiac workup. In a recently treated patient with ecFrom the Departments of Surgery and Pediatrics, University of Colorado School of Medicine, Denver, CO. Accepted for publication Oct 4, 1978. Address reprint requests to Dr. Lilly, Pediatric Surgery, University of Colorado Medical Center, 4200 E Ninth Ave, Denver, CO 80262. *Koop CE: Personal communication, 1978.
484
0003-4975/79/110484-03$01.25@ 1977 by A. F. Jones
topia cordis and omphalocele, the time required for cardiac investigation was provided by covering the heart with a temporary Silastic prosthesis. With the risk of infection and rupture of the sac thus avoided, a thorough and unhurried evaluation of the inlxacardiac lesion was realized, and the parents were permitted to participate in a meaningful operative decision. A 3,300 gm male infant was born to a gravida I para 1 20-year-old woman after an uncomplicated 40-week pregnancy and spontaneous delivery. There was a 5 X 5 cm midline thoracoabdominal wall defect extending from the lower sternal border to the umbilicus. The defect was covered by a thin, transparent membrane, which was torn in onle spot. Cardiac ventricles were seen beating in the sac and protruded 3 cm above the abdominal wall (Fig 1). The infant was vigorous but cyanotic with crying. There was a normal single first heart sound and a single second heart sound at the base of the heart with a grade 2/6 holosystolic murmur. Chest roentgenogram showed intrathoracic enlargement of the right atrial border and decreased pulmonary vascularity. Operation was performed on the first day of life. Skin flaps, which were widely mobilized over the entire abdomen and approximated over the heart, were created. Unacceptable systemic hypotension from compression of the ventricles resulted. Therefore, t~Silastic pouch was fashioned over the exposed heart and attached to the fasciocutaneous defect (Fig 2). This was well tolerated. Diagnostic workup of the intracardiac defect was begun postoperatively. A frontal plane electrocardiogram showed a mean QRS axis of +120 degrees. Echocardiograni demonstrated both atrioventricular valves in the chest and one great vessel. Pulmonary valve atresia, ventricular septa1 defect, and a strikingly elongated ascending aorta (Fig 3) were found at cardiac
485
Case Report: Jones et al: Ectopia Cordis
Fig 2 . Thoracoabdominal ectopia cordis with protrusion of both ventricles into the omphalocele. There is a clip on the umbilical cord; the infant‘s head is to the right.
Fig 3 , Lateral view of the left ventricular angiogram. Most of the left ventricle is shown outside the thoracic and abdominal cavities (arrows). Note the strikingly elongated ascending aorta and the relationship of the diaphragm to the heart. Relocation of the heart to a normal position, therefore, would be impossible. The large patent ductus arteriosus is indicated by an arrow.
When the baby was 2% months old, an ulcer developed in the pseudomembrane overlying the left ventricle. Because of concern that penetration of the ulcer would erode through the ventricular wall and because of the stable cardiac situation, operative construction of a permanent closure over the exposed heart was attempted. During the initial dissection of the inflammatory tissue from the underlying ventricle, a rent was made in the heart. Bleeding was controlled, but effective cardiac output Fig 2 . Temporary coverage of the heart achieved with a was never restored and the infant died. Postmortem examination confirmed the cliniSilastic prosthesis. The infant’s head is toward the top of the photograph. cal diagnosis of pulmonary valve atresia and ventricular septa1 defect. The patent ductus arcatheterization. A patent ductus arteriosus teriosus was approximately ten times the filled the pulmonary arteries. diameter of the hypoplastic main pulmonary The cardiac defect, combined with the prob- artery. Most of the long tubular ventricles prolem of ectopia cordis, was considered formida- truded out of the chest. No malformations sepable, and the parents decided against an heroic rate from this syndrome were identified. operation. Consequently, the Silastic pouch was replaced with sterile dressings and the in- Comment fant was discharged home at the parents’ re- Forty-two cases of the complete syndrome of quest. During the next two months, the child thoracoabdominal ectopia cordis have now did surprisingly well. Cyanosis with crying was been reported [5-7].* Toyama [7] reported 17 only rarely present. A pseudomembrane of inflammatory tissue covered the heart. “Koop CE: Personal communication, 1978.
486 The Annals of Thoracic Surgery Vol 28
No 5 November 1979
additional patients in whom the syndrome was suspected but for whom adequate information was lacking. Cantrell [ll, Mulder [31, and their co-workers called attention to the intracardiac defect as the prime determinant of prognosis. Ventricular septal defect was the most common malformation and has occurred in 31 of the 41 patients [5-71.* Atrial septal defect was present in 20 [5-71. However, more complex lesions, less amenable to repair, were not unusual. As we mentioned previously, the presence of an omphalocele also has an important effect on survival. Of 13 patients with umbilical hernia or diastasis recti, 6 survived at least 2 years [5, 61. On the other hand, of 28 with omphalocele, only 2 were reported alive at 2 years [31.* The high mortality in patients with an omphalocele has been almost exclusively a consequence of attempts at operative coverage of the heart. At least 7 patients were reported to have died of cardiac or respiratory failure in the immediate postnatal period after either reduction of the heart or coverage with skin flaps [6, 7].* Recently, temporary closure of omphaloceles with synthetic material has protected the abdominal viscera from infection while allowing their gradual reduction 141. Separation of the prosthesis and increased risk of infection occur after ten days. Therefore, definitive closure should be done before that time. In our patient, similar coverage of the heart with a Silastic pouch was successful in preventing infection and permitting a careful diagnostic approach to the underlying intracardiac lesion. Because the severity of the cardiac malformation was overestimated, however, definitive repair was not attempted during the infection-free, ten-day period. Consequently, we would recommend the following overall ‘Koop CE: Personal communication, 1978.
surgical approach for infants with ectopia cordis and omphalocele: (1) Temporary coverage of the heart with a Silastic prosthesis. (2) Complete diagnostic workup and, when feasible, consideration for repair of associated intracardiac malformations. (3) Permanent reconstruction of the thoracoabdominal defect. We planned to use an acrylic prosthesis. Koop* suggested detaching the anterior diaphragm from the thorax and reattaching it to the albdominal wall. Eraklis and co-workers [2] used pericardial and diaphragmatic remnants. Whatever surgical repair is elected, it should be accomplished during the first ten days of life.
References Cantrell JR, Haller JA, Ravitch MM: A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet 107:602, 1958 Eraklis AJ, Trump DS, Longino LS: Omphalocele with diaphragmatic and pericardial defects: diagnosis and repair. J Pediatr Surg :2:354, 1967 Mulder DG, Crittenden IH, Adarns FH: Complete repair of a syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart; excision of left ventricular diverticulum. Ann Surg 151:113, 1960 Schuster SR: A new method for the staged repair of large omphaloceles. Surg Gynecol Obstet 125:837, 1967 Spitz L, Bloon KR, Milner S, et al: Combined anterior abdominal wall, sternal, diaphragmatic, pericardial and intracardiac defects. J Pediatr Surg 10:491, 1975 Toyama WM: Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium, and heart. Pediatrics 50:778, 1972 Van der Horst RL, Milha AS, Chiesler E: Ectopia cordis with single ventricle and i i diverticulum. S Afr Med J 49:109, 1975 “Koop CE: Personal communication, 1978.
Immediate Operation for Ectopia Cordis A. F. Jones, M.D., R. L. McGrath, M.D., S. M. Edwards, M.D., and J. R. Lilly, M.D.
ABSTRACT Forty-one infants with thoracoabdominal ectopia cordis have been reported to date; 9 of them survived. Among the patients with an omphalocele as the abdominal wall defect, however, only 2 survived. Death in this group of patients occurred almost exclusively as a consequence of either attempted coverage of the heart or secondary to the associated intracardiac lesion. We report here the case of a patient recently treated in whom coverage with a temporary Silastic prosthesis protected the exposed heart from infection and rupture of the sac, thus permitting full cardiac investigation before operation.
Thoracoabdominal ectopia cordis is a constellation of five malformations: midline supraumbilical abdominal wall defect, defect of the lower sternum, deficiency of the anterior diaphragm, deficiency of the diaphragmatic pericardium, and intracardiac defect [l]. The syndrome was reviewed in 1972 by Toyama [ 6 ] , who called attention to the poor prognosis. Of 41 patients reported, only 9 experienced longterm survival [5-71.* When the abdominal wall defect was an omphalocele, the outlook was even more dismal because of the additional technical problems imposed by providing coverage for the exposed heart. If the technical difficulties can be surmounted, the ultimate prognosis depends on the severity of the cardiac malformation. Consequently, early and precise delineation of the intracardiac lesion and careful consideration to its repair are of crucial importance. In the past, the urgency to provide coverage of the exposed heart resulted in postponement of the cardiac workup. In a recently treated patient with ecFrom the Departments of Surgery and Pediatrics, University of Colorado School of Medicine, Denver, CO. Accepted for publication Oct 4, 1978. Address reprint requests to Dr. Lilly, Pediatric Surgery, University of Colorado Medical Center, 4200 E Ninth Ave, Denver, CO 80262. *Koop CE: Personal communication, 1978.
484
0003-4975/79/110484-03$01.25@ 1977 by A. F. Jones
topia cordis and omphalocele, the time required for cardiac investigation was provided by covering the heart with a temporary Silastic prosthesis. With the risk of infection and rupture of the sac thus avoided, a thorough and unhurried evaluation of the inlxacardiac lesion was realized, and the parents were permitted to participate in a meaningful operative decision. A 3,300 gm male infant was born to a gravida I para 1 20-year-old woman after an uncomplicated 40-week pregnancy and spontaneous delivery. There was a 5 X 5 cm midline thoracoabdominal wall defect extending from the lower sternal border to the umbilicus. The defect was covered by a thin, transparent membrane, which was torn in onle spot. Cardiac ventricles were seen beating in the sac and protruded 3 cm above the abdominal wall (Fig 1). The infant was vigorous but cyanotic with crying. There was a normal single first heart sound and a single second heart sound at the base of the heart with a grade 2/6 holosystolic murmur. Chest roentgenogram showed intrathoracic enlargement of the right atrial border and decreased pulmonary vascularity. Operation was performed on the first day of life. Skin flaps, which were widely mobilized over the entire abdomen and approximated over the heart, were created. Unacceptable systemic hypotension from compression of the ventricles resulted. Therefore, t~Silastic pouch was fashioned over the exposed heart and attached to the fasciocutaneous defect (Fig 2). This was well tolerated. Diagnostic workup of the intracardiac defect was begun postoperatively. A frontal plane electrocardiogram showed a mean QRS axis of +120 degrees. Echocardiograni demonstrated both atrioventricular valves in the chest and one great vessel. Pulmonary valve atresia, ventricular septa1 defect, and a strikingly elongated ascending aorta (Fig 3) were found at cardiac
485
Case Report: Jones et al: Ectopia Cordis
Fig 2 . Thoracoabdominal ectopia cordis with protrusion of both ventricles into the omphalocele. There is a clip on the umbilical cord; the infant‘s head is to the right.
Fig 3 , Lateral view of the left ventricular angiogram. Most of the left ventricle is shown outside the thoracic and abdominal cavities (arrows). Note the strikingly elongated ascending aorta and the relationship of the diaphragm to the heart. Relocation of the heart to a normal position, therefore, would be impossible. The large patent ductus arteriosus is indicated by an arrow.
When the baby was 2% months old, an ulcer developed in the pseudomembrane overlying the left ventricle. Because of concern that penetration of the ulcer would erode through the ventricular wall and because of the stable cardiac situation, operative construction of a permanent closure over the exposed heart was attempted. During the initial dissection of the inflammatory tissue from the underlying ventricle, a rent was made in the heart. Bleeding was controlled, but effective cardiac output Fig 2 . Temporary coverage of the heart achieved with a was never restored and the infant died. Postmortem examination confirmed the cliniSilastic prosthesis. The infant’s head is toward the top of the photograph. cal diagnosis of pulmonary valve atresia and ventricular septa1 defect. The patent ductus arcatheterization. A patent ductus arteriosus teriosus was approximately ten times the filled the pulmonary arteries. diameter of the hypoplastic main pulmonary The cardiac defect, combined with the prob- artery. Most of the long tubular ventricles prolem of ectopia cordis, was considered formida- truded out of the chest. No malformations sepable, and the parents decided against an heroic rate from this syndrome were identified. operation. Consequently, the Silastic pouch was replaced with sterile dressings and the in- Comment fant was discharged home at the parents’ re- Forty-two cases of the complete syndrome of quest. During the next two months, the child thoracoabdominal ectopia cordis have now did surprisingly well. Cyanosis with crying was been reported [5-7].* Toyama [7] reported 17 only rarely present. A pseudomembrane of inflammatory tissue covered the heart. “Koop CE: Personal communication, 1978.
486 The Annals of Thoracic Surgery Vol 28
No 5 November 1979
additional patients in whom the syndrome was suspected but for whom adequate information was lacking. Cantrell [ll, Mulder [31, and their co-workers called attention to the intracardiac defect as the prime determinant of prognosis. Ventricular septal defect was the most common malformation and has occurred in 31 of the 41 patients [5-71.* Atrial septal defect was present in 20 [5-71. However, more complex lesions, less amenable to repair, were not unusual. As we mentioned previously, the presence of an omphalocele also has an important effect on survival. Of 13 patients with umbilical hernia or diastasis recti, 6 survived at least 2 years [5, 61. On the other hand, of 28 with omphalocele, only 2 were reported alive at 2 years [31.* The high mortality in patients with an omphalocele has been almost exclusively a consequence of attempts at operative coverage of the heart. At least 7 patients were reported to have died of cardiac or respiratory failure in the immediate postnatal period after either reduction of the heart or coverage with skin flaps [6, 7].* Recently, temporary closure of omphaloceles with synthetic material has protected the abdominal viscera from infection while allowing their gradual reduction 141. Separation of the prosthesis and increased risk of infection occur after ten days. Therefore, definitive closure should be done before that time. In our patient, similar coverage of the heart with a Silastic pouch was successful in preventing infection and permitting a careful diagnostic approach to the underlying intracardiac lesion. Because the severity of the cardiac malformation was overestimated, however, definitive repair was not attempted during the infection-free, ten-day period. Consequently, we would recommend the following overall ‘Koop CE: Personal communication, 1978.
surgical approach for infants with ectopia cordis and omphalocele: (1) Temporary coverage of the heart with a Silastic prosthesis. (2) Complete diagnostic workup and, when feasible, consideration for repair of associated intracardiac malformations. (3) Permanent reconstruction of the thoracoabdominal defect. We planned to use an acrylic prosthesis. Koop* suggested detaching the anterior diaphragm from the thorax and reattaching it to the albdominal wall. Eraklis and co-workers [2] used pericardial and diaphragmatic remnants. Whatever surgical repair is elected, it should be accomplished during the first ten days of life.
References Cantrell JR, Haller JA, Ravitch MM: A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet 107:602, 1958 Eraklis AJ, Trump DS, Longino LS: Omphalocele with diaphragmatic and pericardial defects: diagnosis and repair. J Pediatr Surg :2:354, 1967 Mulder DG, Crittenden IH, Adarns FH: Complete repair of a syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart; excision of left ventricular diverticulum. Ann Surg 151:113, 1960 Schuster SR: A new method for the staged repair of large omphaloceles. Surg Gynecol Obstet 125:837, 1967 Spitz L, Bloon KR, Milner S, et al: Combined anterior abdominal wall, sternal, diaphragmatic, pericardial and intracardiac defects. J Pediatr Surg 10:491, 1975 Toyama WM: Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium, and heart. Pediatrics 50:778, 1972 Van der Horst RL, Milha AS, Chiesler E: Ectopia cordis with single ventricle and i i diverticulum. S Afr Med J 49:109, 1975 “Koop CE: Personal communication, 1978.
