Ann Thorac Surg 1995;59:511-513
© 1995 The Society of Thoracic Surgeons
Case Reports
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,
James L. Talbert, MD
Department of Surgery, University of Florida College of Medicine, Gainesville, Florida
Accepted for publication May 19, 1994.
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Abstract
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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.
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Introduction
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The combination of unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula is a distinctly rare condition. We have found only 31 cases reported since 1874 [1–4]. Until recently, this combination of anomalies has been uniformly lethal. Hoffman and associates reported 2 long-term survivors in 1989 [1]. Both were full-term infants of uncomplicated deliveries and both weighed more than 2,000 g. We now report survival of a distressed, premature infant.
A 21-year-old woman with a twin pregnancy was referred to our institution when a prenatal ultrasound suggested a right diaphragmatic hernia in one fetus. The fetus with the anomaly had severe intrauterine growth retardation. At 36 weeks estimated gestational age, when poor heart rate variability and late decelerations developed in both fetuses, an emergency cesarean section was 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.
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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. An orogastric tube is curled in the proximal esophageal pouch. There is a normal gas pattern in the abdomen. An 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.
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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.
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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.
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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 4
months 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 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.
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Comment
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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 [1]. 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 [5–7]. 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 2B
). 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 [8]. 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.
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Footnotes
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Address reprint requests to Dr Langham, Department of Surgery, University of Florida, PO Box 100286, JHMHC, Gainesville, FL 32610-0286.
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References
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- Hoffman MA, Superina R, Wesson DE. Unilateral pulmonary agenesis with esophageal atresia and distal tracheoesophageal fistula: report of two cases. J Pediatr Surg 1989;24:1084–5.[Medline]
- Booth JB, Berry CL. Unilateral pulmonary agenesis. Arch Dis Child 1967;42:361–74.
- Black PR, Welch KJ. Pulmonary agenesis (aplasia), esophageal atresia, and tracheoesophageal fistula: a different treatment strategy. J Pediatr Surg 1986;21:936–8.[Medline]
- Brereton RJ, Rickwood AMK. Esophageal atresia with pulmonary agenesis. J Pediatr Surg 1983;18:618–20.[Medline]
- Waterston DJ, Bonham Carter RE, Aberdeen E. Oesophageal atresia: tracheo-oesophageal fistula, a study of survival in 218 infants. Lancet 1962;1:819–22.[Medline]
- Grosfeld JL, Ballantine TVN. Esophageal atresia and tracheoesophageal fistula: effect of delayed thoracotomy on survival. Surgery 1978;84:394–402.[Medline]
- 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.[Medline]
- 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.[Abstract/Free Full Text]
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Abstract
Introduction
