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Unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula
Ann Thorac Surg 1995;59:511-3
bin time [3]. In t r e a t m e n t of PVT, surgical intervention, either prosthetic valve r e p l a c e m e n t or thrombectomy, carries a high mortality of 37% to 55% I4]. Fibrinolytic t r e a t m e n t was an attractive alternative to solve the PVT p r o b l e m without surgical intervention. However, b e cause of the risk of systemic embolism, the use of thrombolytic t h e r a p y should be r e s e r v e d for tricupid valve t h r o m b o s i s or critically ill patients with mitral or aortic valve thrombosis [2]. Because this patient was in a p r o f o u n d shock state at the time of admission, we tried thrombolysis first. To decrease the risk of embolization, a less intense b u t m o r e p r o l o n g e d thrombolytic r e g i m e n than that u s e d for t r e a t m e n t of acute m y o c a r d i a l infarction has b e e n d e s c r i b e d [5]. In this patient, a p r o l o n g e d continuous infusion of low dose of tPA for up to 24 hours was performed. If thrombolytic t h e r a p y fails to lyse, o p e r a t i o n should be p e r f o r m e d . Because of a short p l a s m a half-life of a b o u t 5 m i n u t e s a n d the dosed e p e n d e n t clot selectivity of tPA, the systemic lyric effects are of short duration a n d are only mild to m o d e r a t e in severity [6]. Hence, w h e n thrombolytic t h e r a p y is unsuccessful, operation m a y not increase mortality. However, further studies are n e e d e d to s u p p o r t this. Echocardiography is a safe a n d convenient tool to detect abnormalities of prosthetic valves a n d the effect of thrombolytic t h e r a p y on PVT. A l t h o u g h various p a r a m eters of transthoracic e c h o c a r d i o g r a p h y were used, no studies have yet s h o w e d the sensitivity a n d specificity of the p a r a m e t e r s for diagnosis of PVT. The a b n o r m a l shortening of isovolumetric relaxation time a n d unexp l a i n e d p u l m o n a r y h y p e r t e n s i o n m a y raise suspicion of prosthetic mitral valve dysfunction [7]. After t h r o m b o lytic therapy, normalization of the interval of aortic valve closure to mitral valve o p e n i n g a n d the p u l m o n a r y artery p r e s s u r e were substantial indicators of the complete resolution of thrombus. But the difficulty in transthoracic e c h o c a r d i o g r a p h y is to detect detailed abnormalities in cases of mechanical prosthesis b e c a u s e of the echogenicity of the poppet, struts, a n d suture ring. Reverberation of the structures often m a s k e d n e i g h b o r i n g structure images. T r a n s e s o p h a g e a l echocardiography, avoiding mechanical interference, allows direct visualization of the t h r o m b u s associated with the mechanical prosthetic valve. In conclusion, thrombolytic t h e r a p y a p p e a r s to be an effective a n d relatively safe modality for treating PVT in selected patients. Echocardiography, both transthoracic a n d transesophageal, is a useful tool for diagnosis of PVT a n d therapeutic guidance to complete resolution after thrombolytic therapy. If thrombolysis fails, operation should be performed.
References 1. Edmunds LH. Thromboembolic complications of current cardiac valvular prostheses. Ann Thorac Surg 1982;34:96-106. 2. Rondaut R, Labbe T, Lorgnette-Readout FM, et al. Mechanical cardiac valve thrombosis. Circulation 1992;86(Suppl 2):I8-I15. 3. Opie LH. Drugs for the heart. 3rd ed. Philadelphia: Saunders, 1991:228. 4. Husebye DG, Pluth JR, Piehler JM, et al. Reoperation on © 1995 by The Society of Thoracic Surgeons
CASE REPORT STEADLANDET AL PULMONARY AGENESISAND TEF
511
prosthetic heart valves: an analysis of risk factors in 552 patients. J Thorac Cardiovasc Surg 1983;86:543-52. 5. Graver LM, Gelber PM, Tyras DH. The risks and benefits of thrombolytic therapy in acute aortic and mitral prosthetic valve dysfunction: report of a case and review of the literature. Ann Thorac Surg 1988;46:85-8. 6. Garabedian HD, Gold HK, Leinbach RC, et al. Dosedependent thrombolysis, pharmacokinetics and hemostatic effects of recombinant human tissue-type plasminogen activator for coronary thrombosis. Am J Cardiol 1986;58:673-9. 7. Cunha CL, Giuliani ER, Callahan LA, Pluth JR. Echophonocardiographic findings in patients with prosthetic heart valve malfunction. Mayo Clin Proc 1980;55:231-42.
Unilateral Pulmonary Agenesis, Esophageal Atresia, and Distal Tracheoesophageal Fistula Kevin M. Steadland, MD, Max R. Langham, Jr, MD, Michael A. Greene, MD, Charles E. Bagwell, MD, David W. Kays, MD, a n d James L. Talbert, MD Department of Surgery, University of Florida College of Medicine, Gainesville, Florida
A small-for-gestational-age, premature infant with the combination of unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula was treated successfully by early gastrostomy and delayed fistula division with esophagoesophagostomy. Only 2 other successfully treated cases have been reported previously. Both were full-term infants treated with early division of the tracheoesophageal fistula and esophagoesophagostomy. Gestational age, size, and associated medical problems need to be considered when planning operative therapy for these babies.
(Ann Thorac Surg 1995;59:511-3) he combination of unilateral p u l m o n a r y agenesis, e s o p h a g e a l atresia, a n d distal tracheoesophageal fistula is a distinctly rare condition. W e have found only 31 cases r e p o r t e d since 1874 [1-4]. Until recently, this combination of anomalies has b e e n uniformly lethal. Hoffman a n d associates r e p o r t e d 2 long-term survivors in 1989 [1]. Both were full-term infants of uncomplicated deliveries a n d both w e i g h e d m o r e than 2,000 g. W e now report survival of a distressed, p r e m a t u r e infant.
T
A 21-year-old w o m a n with a twin p r e g n a n c y was referred to our institution w h e n a p r e n a t a l u l t r a s o u n d s u g g e s t e d a right diaphragmatic hernia in one fetus. The fetus with the a n o m a l y h a d severe intrauterine growth retardation. At 36 weeks estimated gestational age, w h e n poor heart rate variability and late decelerations develo p e d in both fetuses, an e m e r g e n c y cesarean section was Accepted for publication May 19, 1994. Address reprint requests to Dr Langharn, Department of Surgery, University of Florida, PO Box 100286, JHMHC, Gainesville, FL 32610-0286. 0003-4975/95/$9.50 0003-4975(94)00383-I
512
CASEREPORT STEADLANDET AL PULMONARYAGENESISAND TEF
Fig 1. Anteroposterior chest roentgenogram obtained in the nursery on the first day of life, which shows a symmetric chest with dextrocardia and no evidence of a right lung. A n orogastric tube is curled in the proximal esophageal pouch. There is a normal gas pattern in the abdomen. A n endotracheal tube is in place with its tip at the level of the clavicles. The normal abdominal appearance of the liver and absence of any intestinal gas in the chest excludes the prenatal diagnosis of congenital diaphragmatic hernia.
Ann Thorac Surg 1995;59:511-3
performed. The infant girl with the anomaly weighed 1,890 g. She was intubated immediately. Apgar scores were 7 at 1 minute and 9 at 5 minutes. There were markedly decreased breath sounds on the right. A chest roentgenogram showed an opacified right hemithorax and dextrocardia (Fig 1). The orogastric tube was coiled in the proximal esophagus, with air in the gastrointestinal tract below the diaphragm. These findings suggested a right pulmonary agenesis, esophageal atresia, and tracheoesophageal fistula. An echocardiogram showed a left-sided aortic arch, a patent ductus arteriosus, and normal cardiac anatomy. The baby was immediately given famotidine (1.2 mg. kg -1. day 1 intravenously). A sump catheter was used to suction the proximal esophageal pouch continuously. A gastrostomy was performed 2 days later with extubation on postoperative day 1. Total parenteral nutrition allowed weight gain to 2,395 g on day of life 26, when we repaired the anomaly. Bronchoscopy showed a posterior takeoff of a distal tracheoesophageal fistula, with complete absence of the right main bronchus. Exploration of the right chest and mediastinum confirmed the dextrocardia, left-sided aortic arch, complete absence of the right lung, and the posterior takeoff of a distal tracheoesophageal fistula (Fig 2A). The heart was retracted anteromedially to allow division of the fistula and esophagoesophagostomy (Fig 2B). This retraction did not compromise cardiac function. The postoperative course was complicated by hypertrophic pyloric stenosis. A pyloromyotomy was performed on day of life 48. Persistent gastroesophageal reflux caused episodic apnea and bradycardia, which resolved after a Nissen fundoplication was performed on day of life 76. The patient was discharged home at 41/2months of age, tolerating gastrostomy tube feedings and weighing 4,048 g. Now, at 28 months of age, the patient weighs 9.6 kg (5th percentile). Her arterial blood gases are
Fig 2. (A) A right thoracotomy revealed a heart with a normal pericardium (P). There was no pleura or evidence of any lung tissue. (B) With the heart retracted anteromedially, the completed esophagoesophagostomy can be seen with the stay sutures still in place. The tracheal repair is not visible. Exposure for the operation was quite adequate, and this degree of retraction on the heart did not induce any arrhythmias or hemodynamic instability.
A
B
Ann Thorac 1995;59:513-5
Surg
normal on room air. She tolerates soft foods orally and is gaining weight without supplemental gastrostomy feedings. Developmental testing shows normal intelligence with some delay in motor ability, which is being treated with occupational and physical therapy. Comment Successfully treating infants with combinations of severe congenital anomalies is one of the most challenging aspects of pediatric surgery. The extremely rare infant with the combination of unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula presents such a problem. Including this report, there are only 3 known survivors with this combination of conditions [l]. The operative strategy Hoffman and colleagues used with the 2 previously reported survivors was early division of the tracheoesophageal fistula with esophagoesophagostomy. In small, premature infants without pulmonary agenesis and with esophageal atresia and distal tracheoesophageal fistula, early gastrostomy followed by delayed fistula division with esophagoesophagostomy has improved survival [5-71. The gastrostomy accomplishes the primary goal of protecting respiratory function by decreasing the incidence of reflux-induced pneumonia. Delaying thoracotomy and repair of the tracheoesophageal anomaly minimizes stress during the period of transitional circulation in a child with a small pulmonary arterial bed and makes the repair technically easier. A thoracotomy on the side of the pulmonary agenesis allows adequate ventilation of the single lung. This approach requires significant retraction on the heart to gain exposure of the posterior mediastinum (see Fig ZB). Black and Welch [3] reported that this retraction may cause bradycardia and hypotension severe enough to prevent repair of the tracheoesophageal anomaly. If this problem had been encountered, we planned to proceed with the operative repair of the anomaly using extracorporeal membrane oxygenation to support the patient. Prematurity and weight less than 2,000 g have been associated with a higher rate of intercranial hemorrhage on extracorporeal membrane oxygenation [S]. A delayed thoracotomy also minimizes this risk. This patient is the third reported successfully treated survivor with pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula, and illustrates that selected high risk infants may be successfully treated with an early gastrostomy and then delayed thoracotomy with repair of the anomaly after appropriate growth.
References 1. Hoffman MA, Superina R, Wesson DE. Unilateral pulmonary agenesis with esophageal atresia and distal tracheoesophageal fistula: report-of t&o cases. J Pediatr Surg 1989;24:1084-5. 2. Booth JB, Berry CL. Unilateral 1uulmonarv 1 agenesis. Arch Dis ” Child 1967$2&l-74. 3. Black PR, Welch KJ. Pulmonary agenesis (aplasia), esophageal atresia, and tracheoesophageal fistula: a different treatment strategy. J Pediatr Surg 1986;21:936-8. 0 1995 by The Society of Thoracic Surgeons
LEFT ATRIAL
THROMBUS
513
PHAM ET AL CASE REPORT AFTER LUNG TRANSPLANTATION
4. Brereton RJ, Rickwood AMK. Esophageal atresia with pulmonary agenesis. J Pediatr Surg 1983;18:618-20. 5. Waterston DJ, Bonham Carter RE, Aberdeen E. Oesophageal atresia: trachea-oesophageal fistula, a study of survival in 218 infants. Lancet 1962;1:819-22. 6. Grosfeld JL, Ballantine TVN. Esophageal atresia and tracheoesophageal fistula: effect of delayed thoracotomy on survival. Surgery 1978;84:394-402. 7. Randolph JG, Newman KD, Anderson KD. Current results in repair of esophageal atresia with tracheoesophageal fistula using physiologic status as a guide to therapy. Ann Surg 1989;209:526 -31. 8. Cilley RE, Zwischenberger JB, Andrews AF, Bowerman RA, Roloff DW, Bartlett RH. Intracranial hemorrhage during extracorporeal membrane oxygenation in neonates. Pediatrics 1986;78:699-704.
Left Atria1 Thrombus Transplantation
After Lung
Si M. Pham, MD, John M. Armitage, MD, William Katz, MD, and Bartley P. Griffith, MD
E.
Departments of Surgery and Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania
We report on a 16-year-old boy who developed a throma bus at the left atria1 suture line after undergoing bilateral sequential single lung transplantation. The diagnosis was made by transesophageal echocardiogram. (Ann Tharac Surg 2995;59:513-5) 16-year-old boy with cystic fibrosis underwent a bilateral sequential single lung transplantation. The operation was performed through a bilateral, anterolateral thoracotomy incision without cardiopulmonary bypass. The first chest radiograph immediately after the operation revealed bilateral patchy atelectasis and pulmonary edema, more extensive in the right lung than in the left (Fig 1). On postoperative day 1, the chest radiograph showed good aeration in the left lung field and in the periphery of the right lung. However, opacification in the perihilar area and in the middle portion of the right lung persisted. By postoperative day 3 there was marked opacification of the right lung and a radiologically normal left lung (Fig 1). In addition, the drainage from the right pleural tube started to increase (325 mL/day versus 125 mL on the previous day). Concomitantly, the patient’s arterial oxygen saturation had progressively deteriorated. A bronchoscopic examination revealed no airway obstruction; however, there was copious amount of clear fluid emanating from the right main bronchus. A transesophageal echocardiogram revealed a large mass consistent with a thrombus at the junction between the right pulmonary veins and the left atrium, partially
A
Accepted
for
publication May
27, 1994.
Address reprint requests to Dr Pham, Division of Cardiothoracic Surgery, University of Pittsburgh School of Medicine, Suite C-700, Presbyterian University Hospital, 200 Lothrop St, Pittsburgh, PA 15213.
0003-4975/95/$9.50 0003-4975(94)00587-W
bin time [3]. In t r e a t m e n t of PVT, surgical intervention, either prosthetic valve r e p l a c e m e n t or thrombectomy, carries a high mortality of 37% to 55% I4]. Fibrinolytic t r e a t m e n t was an attractive alternative to solve the PVT p r o b l e m without surgical intervention. However, b e cause of the risk of systemic embolism, the use of thrombolytic t h e r a p y should be r e s e r v e d for tricupid valve t h r o m b o s i s or critically ill patients with mitral or aortic valve thrombosis [2]. Because this patient was in a p r o f o u n d shock state at the time of admission, we tried thrombolysis first. To decrease the risk of embolization, a less intense b u t m o r e p r o l o n g e d thrombolytic r e g i m e n than that u s e d for t r e a t m e n t of acute m y o c a r d i a l infarction has b e e n d e s c r i b e d [5]. In this patient, a p r o l o n g e d continuous infusion of low dose of tPA for up to 24 hours was performed. If thrombolytic t h e r a p y fails to lyse, o p e r a t i o n should be p e r f o r m e d . Because of a short p l a s m a half-life of a b o u t 5 m i n u t e s a n d the dosed e p e n d e n t clot selectivity of tPA, the systemic lyric effects are of short duration a n d are only mild to m o d e r a t e in severity [6]. Hence, w h e n thrombolytic t h e r a p y is unsuccessful, operation m a y not increase mortality. However, further studies are n e e d e d to s u p p o r t this. Echocardiography is a safe a n d convenient tool to detect abnormalities of prosthetic valves a n d the effect of thrombolytic t h e r a p y on PVT. A l t h o u g h various p a r a m eters of transthoracic e c h o c a r d i o g r a p h y were used, no studies have yet s h o w e d the sensitivity a n d specificity of the p a r a m e t e r s for diagnosis of PVT. The a b n o r m a l shortening of isovolumetric relaxation time a n d unexp l a i n e d p u l m o n a r y h y p e r t e n s i o n m a y raise suspicion of prosthetic mitral valve dysfunction [7]. After t h r o m b o lytic therapy, normalization of the interval of aortic valve closure to mitral valve o p e n i n g a n d the p u l m o n a r y artery p r e s s u r e were substantial indicators of the complete resolution of thrombus. But the difficulty in transthoracic e c h o c a r d i o g r a p h y is to detect detailed abnormalities in cases of mechanical prosthesis b e c a u s e of the echogenicity of the poppet, struts, a n d suture ring. Reverberation of the structures often m a s k e d n e i g h b o r i n g structure images. T r a n s e s o p h a g e a l echocardiography, avoiding mechanical interference, allows direct visualization of the t h r o m b u s associated with the mechanical prosthetic valve. In conclusion, thrombolytic t h e r a p y a p p e a r s to be an effective a n d relatively safe modality for treating PVT in selected patients. Echocardiography, both transthoracic a n d transesophageal, is a useful tool for diagnosis of PVT a n d therapeutic guidance to complete resolution after thrombolytic therapy. If thrombolysis fails, operation should be performed.
References 1. Edmunds LH. Thromboembolic complications of current cardiac valvular prostheses. Ann Thorac Surg 1982;34:96-106. 2. Rondaut R, Labbe T, Lorgnette-Readout FM, et al. Mechanical cardiac valve thrombosis. Circulation 1992;86(Suppl 2):I8-I15. 3. Opie LH. Drugs for the heart. 3rd ed. Philadelphia: Saunders, 1991:228. 4. Husebye DG, Pluth JR, Piehler JM, et al. Reoperation on © 1995 by The Society of Thoracic Surgeons
CASE REPORT STEADLANDET AL PULMONARY AGENESISAND TEF
511
prosthetic heart valves: an analysis of risk factors in 552 patients. J Thorac Cardiovasc Surg 1983;86:543-52. 5. Graver LM, Gelber PM, Tyras DH. The risks and benefits of thrombolytic therapy in acute aortic and mitral prosthetic valve dysfunction: report of a case and review of the literature. Ann Thorac Surg 1988;46:85-8. 6. Garabedian HD, Gold HK, Leinbach RC, et al. Dosedependent thrombolysis, pharmacokinetics and hemostatic effects of recombinant human tissue-type plasminogen activator for coronary thrombosis. Am J Cardiol 1986;58:673-9. 7. Cunha CL, Giuliani ER, Callahan LA, Pluth JR. Echophonocardiographic findings in patients with prosthetic heart valve malfunction. Mayo Clin Proc 1980;55:231-42.
Unilateral Pulmonary Agenesis, Esophageal Atresia, and Distal Tracheoesophageal Fistula Kevin M. Steadland, MD, Max R. Langham, Jr, MD, Michael A. Greene, MD, Charles E. Bagwell, MD, David W. Kays, MD, a n d James L. Talbert, MD Department of Surgery, University of Florida College of Medicine, Gainesville, Florida
A small-for-gestational-age, premature infant with the combination of unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula was treated successfully by early gastrostomy and delayed fistula division with esophagoesophagostomy. Only 2 other successfully treated cases have been reported previously. Both were full-term infants treated with early division of the tracheoesophageal fistula and esophagoesophagostomy. Gestational age, size, and associated medical problems need to be considered when planning operative therapy for these babies.
(Ann Thorac Surg 1995;59:511-3) he combination of unilateral p u l m o n a r y agenesis, e s o p h a g e a l atresia, a n d distal tracheoesophageal fistula is a distinctly rare condition. W e have found only 31 cases r e p o r t e d since 1874 [1-4]. Until recently, this combination of anomalies has b e e n uniformly lethal. Hoffman a n d associates r e p o r t e d 2 long-term survivors in 1989 [1]. Both were full-term infants of uncomplicated deliveries a n d both w e i g h e d m o r e than 2,000 g. W e now report survival of a distressed, p r e m a t u r e infant.
T
A 21-year-old w o m a n with a twin p r e g n a n c y was referred to our institution w h e n a p r e n a t a l u l t r a s o u n d s u g g e s t e d a right diaphragmatic hernia in one fetus. The fetus with the a n o m a l y h a d severe intrauterine growth retardation. At 36 weeks estimated gestational age, w h e n poor heart rate variability and late decelerations develo p e d in both fetuses, an e m e r g e n c y cesarean section was Accepted for publication May 19, 1994. Address reprint requests to Dr Langharn, Department of Surgery, University of Florida, PO Box 100286, JHMHC, Gainesville, FL 32610-0286. 0003-4975/95/$9.50 0003-4975(94)00383-I
512
CASEREPORT STEADLANDET AL PULMONARYAGENESISAND TEF
Fig 1. Anteroposterior chest roentgenogram obtained in the nursery on the first day of life, which shows a symmetric chest with dextrocardia and no evidence of a right lung. A n orogastric tube is curled in the proximal esophageal pouch. There is a normal gas pattern in the abdomen. A n endotracheal tube is in place with its tip at the level of the clavicles. The normal abdominal appearance of the liver and absence of any intestinal gas in the chest excludes the prenatal diagnosis of congenital diaphragmatic hernia.
Ann Thorac Surg 1995;59:511-3
performed. The infant girl with the anomaly weighed 1,890 g. She was intubated immediately. Apgar scores were 7 at 1 minute and 9 at 5 minutes. There were markedly decreased breath sounds on the right. A chest roentgenogram showed an opacified right hemithorax and dextrocardia (Fig 1). The orogastric tube was coiled in the proximal esophagus, with air in the gastrointestinal tract below the diaphragm. These findings suggested a right pulmonary agenesis, esophageal atresia, and tracheoesophageal fistula. An echocardiogram showed a left-sided aortic arch, a patent ductus arteriosus, and normal cardiac anatomy. The baby was immediately given famotidine (1.2 mg. kg -1. day 1 intravenously). A sump catheter was used to suction the proximal esophageal pouch continuously. A gastrostomy was performed 2 days later with extubation on postoperative day 1. Total parenteral nutrition allowed weight gain to 2,395 g on day of life 26, when we repaired the anomaly. Bronchoscopy showed a posterior takeoff of a distal tracheoesophageal fistula, with complete absence of the right main bronchus. Exploration of the right chest and mediastinum confirmed the dextrocardia, left-sided aortic arch, complete absence of the right lung, and the posterior takeoff of a distal tracheoesophageal fistula (Fig 2A). The heart was retracted anteromedially to allow division of the fistula and esophagoesophagostomy (Fig 2B). This retraction did not compromise cardiac function. The postoperative course was complicated by hypertrophic pyloric stenosis. A pyloromyotomy was performed on day of life 48. Persistent gastroesophageal reflux caused episodic apnea and bradycardia, which resolved after a Nissen fundoplication was performed on day of life 76. The patient was discharged home at 41/2months of age, tolerating gastrostomy tube feedings and weighing 4,048 g. Now, at 28 months of age, the patient weighs 9.6 kg (5th percentile). Her arterial blood gases are
Fig 2. (A) A right thoracotomy revealed a heart with a normal pericardium (P). There was no pleura or evidence of any lung tissue. (B) With the heart retracted anteromedially, the completed esophagoesophagostomy can be seen with the stay sutures still in place. The tracheal repair is not visible. Exposure for the operation was quite adequate, and this degree of retraction on the heart did not induce any arrhythmias or hemodynamic instability.
A
B
Ann Thorac 1995;59:513-5
Surg
normal on room air. She tolerates soft foods orally and is gaining weight without supplemental gastrostomy feedings. Developmental testing shows normal intelligence with some delay in motor ability, which is being treated with occupational and physical therapy. Comment Successfully treating infants with combinations of severe congenital anomalies is one of the most challenging aspects of pediatric surgery. The extremely rare infant with the combination of unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula presents such a problem. Including this report, there are only 3 known survivors with this combination of conditions [l]. The operative strategy Hoffman and colleagues used with the 2 previously reported survivors was early division of the tracheoesophageal fistula with esophagoesophagostomy. In small, premature infants without pulmonary agenesis and with esophageal atresia and distal tracheoesophageal fistula, early gastrostomy followed by delayed fistula division with esophagoesophagostomy has improved survival [5-71. The gastrostomy accomplishes the primary goal of protecting respiratory function by decreasing the incidence of reflux-induced pneumonia. Delaying thoracotomy and repair of the tracheoesophageal anomaly minimizes stress during the period of transitional circulation in a child with a small pulmonary arterial bed and makes the repair technically easier. A thoracotomy on the side of the pulmonary agenesis allows adequate ventilation of the single lung. This approach requires significant retraction on the heart to gain exposure of the posterior mediastinum (see Fig ZB). Black and Welch [3] reported that this retraction may cause bradycardia and hypotension severe enough to prevent repair of the tracheoesophageal anomaly. If this problem had been encountered, we planned to proceed with the operative repair of the anomaly using extracorporeal membrane oxygenation to support the patient. Prematurity and weight less than 2,000 g have been associated with a higher rate of intercranial hemorrhage on extracorporeal membrane oxygenation [S]. A delayed thoracotomy also minimizes this risk. This patient is the third reported successfully treated survivor with pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula, and illustrates that selected high risk infants may be successfully treated with an early gastrostomy and then delayed thoracotomy with repair of the anomaly after appropriate growth.
References 1. Hoffman MA, Superina R, Wesson DE. Unilateral pulmonary agenesis with esophageal atresia and distal tracheoesophageal fistula: report-of t&o cases. J Pediatr Surg 1989;24:1084-5. 2. Booth JB, Berry CL. Unilateral 1uulmonarv 1 agenesis. Arch Dis ” Child 1967$2&l-74. 3. Black PR, Welch KJ. Pulmonary agenesis (aplasia), esophageal atresia, and tracheoesophageal fistula: a different treatment strategy. J Pediatr Surg 1986;21:936-8. 0 1995 by The Society of Thoracic Surgeons
LEFT ATRIAL
THROMBUS
513
PHAM ET AL CASE REPORT AFTER LUNG TRANSPLANTATION
4. Brereton RJ, Rickwood AMK. Esophageal atresia with pulmonary agenesis. J Pediatr Surg 1983;18:618-20. 5. Waterston DJ, Bonham Carter RE, Aberdeen E. Oesophageal atresia: trachea-oesophageal fistula, a study of survival in 218 infants. Lancet 1962;1:819-22. 6. Grosfeld JL, Ballantine TVN. Esophageal atresia and tracheoesophageal fistula: effect of delayed thoracotomy on survival. Surgery 1978;84:394-402. 7. Randolph JG, Newman KD, Anderson KD. Current results in repair of esophageal atresia with tracheoesophageal fistula using physiologic status as a guide to therapy. Ann Surg 1989;209:526 -31. 8. Cilley RE, Zwischenberger JB, Andrews AF, Bowerman RA, Roloff DW, Bartlett RH. Intracranial hemorrhage during extracorporeal membrane oxygenation in neonates. Pediatrics 1986;78:699-704.
Left Atria1 Thrombus Transplantation
After Lung
Si M. Pham, MD, John M. Armitage, MD, William Katz, MD, and Bartley P. Griffith, MD
E.
Departments of Surgery and Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania
We report on a 16-year-old boy who developed a throma bus at the left atria1 suture line after undergoing bilateral sequential single lung transplantation. The diagnosis was made by transesophageal echocardiogram. (Ann Tharac Surg 2995;59:513-5) 16-year-old boy with cystic fibrosis underwent a bilateral sequential single lung transplantation. The operation was performed through a bilateral, anterolateral thoracotomy incision without cardiopulmonary bypass. The first chest radiograph immediately after the operation revealed bilateral patchy atelectasis and pulmonary edema, more extensive in the right lung than in the left (Fig 1). On postoperative day 1, the chest radiograph showed good aeration in the left lung field and in the periphery of the right lung. However, opacification in the perihilar area and in the middle portion of the right lung persisted. By postoperative day 3 there was marked opacification of the right lung and a radiologically normal left lung (Fig 1). In addition, the drainage from the right pleural tube started to increase (325 mL/day versus 125 mL on the previous day). Concomitantly, the patient’s arterial oxygen saturation had progressively deteriorated. A bronchoscopic examination revealed no airway obstruction; however, there was copious amount of clear fluid emanating from the right main bronchus. A transesophageal echocardiogram revealed a large mass consistent with a thrombus at the junction between the right pulmonary veins and the left atrium, partially
A
Accepted
for
publication May
27, 1994.
Address reprint requests to Dr Pham, Division of Cardiothoracic Surgery, University of Pittsburgh School of Medicine, Suite C-700, Presbyterian University Hospital, 200 Lothrop St, Pittsburgh, PA 15213.
0003-4975/95/$9.50 0003-4975(94)00587-W