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Membranous esophageal obstruction simulating atresia with a double tracheoesophageal fistula in a neonate
Membranous esophageal obstruction simulating atresia with a double tracheoesophageal fistula in a neonate Robert J. Touloukian, M.D., New Haven, Conn.
The major esophageal anomalies fall readily into three anatomic categories: esoph(1)
ageal atresia alone, (2) esophageal atresia with an associated tracheoesophageal fistula, and (3) tracheoesophageal fistula alone. The common classifications proposed by Vogt,' Gross," and Swenson" fail to emphasize that on occasion the anomaly does not clearly belong in one of these three major categories. It is important to emphasize that such cases do exist, because recognition of a variant is vital to the successful treatment. The following case history describes a rarely reported anomaly in a neonate-membranous esophageal obstruction and a tracheoesophageal fistula. Case report A term Caucasian male infant, weighing 3,750 grams, was referred to the Newborn Special Care Unit of the Yale-New Haven Hospital when he was 24 hours old for evaluation of a heart murmur and cyanosis. The pregnancy was complicated by obesity and hypertension. Delivery was spontaneous, and the Apgar score was 8 at I minute. Shortly thereafter the infant became cyanotic, and a Grade 3 of 6 heart murmur was audible over the precordium. On admission the pulse was 150 beats per minute and regular. Respirations were 50 breaths per minute. Moist rales and rhonchi were heard in both lung fields.
From the Department of Surgery, Section of Pediatric Surgery, Yale University School of Medicine, and the Yale-New Haven Hospital, New Haven, Conn. 06510. Received for publication June 23, 1972.
An easily palpable thrill was present at the left sternal border. Peripheral pulses were intact. The remainder of the physical examination was unremarkable. Complete blood count and urinalysis were within normal limits. Blood gases on admission were as follows: pH 7.37, Pco, 45 mm. Hg, and Po, 37 in room air but increasing to 62 mm. Hg in 100 per cent oxygen. A chest x-ray film revealed a boot-shaped left cardiac silhouette with densities in the right lung field suggestive of a pneumonic process. A nasogastric tube was introduced but lodged in the midportion of the esophagus and could not be advanced. Air passed freely through the tube during inspiration. Oily Dionosil, injected through the catheter, entered the major bronchi through a communication between the esophagus and the trachea (Fig. 1). After a tube gastrostomy, the esophagus was exposed through an extrapleural right thoracotomy. The esophagus was grossly normal in appearance but completely filled with air during inspiration. A single tracheoesophageal fistula, 3 mm. in size, was identified in the upper esophagus corresponding to the site of the intraluminal obstruction. After the fistula had been divided, the trachea and esophagus were closed individually with 5-0 arterial silk sutures. The postoperative course was unremarkable. Pneumonia cleared within a few days on amphicillin and kanamycin therapy. The upper pouch seemed totally obstructed, requiring continuous suction through a Replogle tube. The feedings were begun through the gastrostomy. Blood gases remained unchanged: Cardiac catheterization on the third postoperative day revealed D-transposition of the great vessels with tricuspid atresia, probable single ventricle, and pulmonic stenosis. Because there was adequate mixing and fair oxygenation, no cardiac surgery was contemplated. On the ninth postoperative day, repeat esophagography confirmed total obstruction of
191
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Fig. 1. Preoperative study shows tip of catheter in upper esophageal pouch and contrast media entering trachea and major bronchi through the tracheoesophageal fistula.
Fig. :lA. The proximal esophagus is completely obstructed to antegrade flow of barium on the ninth day after division of the tracheoesophageal fistula.
the proximal esophagus to antegrade filling (Fig. 2A). The intraluminal web was clearly visible on retrograde filling of the esophagus through the gastrostomy (Fig. 2B). Two days later, esophageal continuity was restored by segmental resection of the esophagus, including the web. The anastomosis was performed with an inverting single layer of 5-0 arterial silk. A subsequent cineesophagogram revealed normal motility and adequate emptying of the esophagus (Fig . 3) . A schematic drawing of the original anomaly is shown in Fig. 4. During a 6 month follow-up period, the infant's growth and development have been normal, and his peripheral oxygenation has been adequate.
Discussion
Membranous esophageal obstruction with a tracheoesophageal fistula has anatomic similarities to the commonly observed form of esophageal atresia with a distal tracheoesophageal fistula. The obvious difference is the muscular continuity of the esophagus which belies the presence of an intraluminal obstruction. In a number of reported cases.v!' the proximal pouch actually overhung the distal esophagus , making location of the distal tracheoesophageal fistula more difficult; in some patients, the upper pouch extended to the diaphragm. Holcomb!' emphasized that a long tracheoesophageal fistula may enter the trachea just above the carina or extend to or above the thoracic inlet. In both cases, the esophagus is com-
Fig. :lB. The esophageal membrane is clearly visible on retrograde filling of the esophagus through the gastrostomy tube .
pletely separated by the membrane, and the fistula enters only the distal esophagus; thus infants with this anomaly are clearly analogous to their counterparts who have visibly separate esophageal segments. Holcomb! ' proposed that this anomaly be classified as Type G, according to Gross," or as ~IId, according to Vogt,' to give membranous obstruction of the esophagus wider recognition. Since the membrane only partially obstructed the esophagus in our patient, certain unique clinical differences existed. As the tracheoesophageal fistula lay just op-
Volume 65 Number 2
Esophageal obstruction with tracheoesophageal fistula
193
Februory . 1973
EsophaQeal Membrane
Fig. 3. Final result after resection of the obstructing membrane and direct esophageal anastomosis. Fig. 4. Schematic draw ing of the original anomaly.
posite the septum, allowing air to enter both the proximal and distal esophagus, the anomaly simulated atresia with a double fistulous communication. The true anatomic diagnosis was not apparent on the initial study, which demonstrated complete esophageal obstruction with prompt pulmonary aspiration through the tracheoesophageal fistula. Retrograde studies via the gastrostomy, performed after division of the tracheoesophageal fistula, indicated that obstruction was incomplete, with free reflux occurring around the web. The esophagus remained totally obstructed to antegrade passage of barium. A very similar case has been reported from our institution by Goldenberg.'" In addition to the septal obstruction and a tracheoesophageal fistula, a second fistula was identified at autopsy 1.5 cm. above the one which was recognized at operation. Death was attributed to right heart failure secondary to pneumonia, atrial and ventricular septal defects, bicuspid aortic valve, coarctation of the aorta, and a patent ductus arteriosus . Our patient also has severe congenital heart disease. The coexistent cardiac anomalies in these cases suggest that there was a major systemic insult early in gestation which had a widespread effect on the developing fetus. Aspiration through the proximal and distal esophageal segments requires prompt division of the tracheoesophageal fistula.
The urgency of this procedure is greater than with infants who have a blind upper pouch, since swallowed saliva directly enters the tracheobronchial tree through the fistula. Pharyngeal or indwelling sump suction is ineffective in retrieving all secretions before they reach the base of the pouch and , of course, gastric secretions intermittently reflux through the distal segment. Esophageal reconstruction may be delayed until pneumonia is treated and the obstruction clearly defined by contrast radiography. The membranous web may be excised, as reported by Goldenberg," and the esophagotomy incision closed transversely to minimize narrowing the lumen. We prefer to resect the web and perform an end-to-end anastomosis, since there is sufficient esophageal length to avoid tension and the risk of anastomotic stenosis. This approach is vindicated by the good result in our patient. Summary
A newborn infant with a partially obstructing esophageal web and a tracheoesophageal fistula was successfully treated by a twa-stage operation involving (1) division of the fistula and (2) subsequent esophageal reconstruction. The case is reported to emphasize that completely or partially obstructing esophageal webs ac-
1 94
The Journol of Thorocic ond Cordiovosculor
Touloukian
Surgery
companied by a tracheoesophageal fistula are rare variants of the common form of esophageal atresia which involves a distal tracheoesophageal fistula. REFERENCES
2 3
4
5
Vogt, E. C.: Congenital Esophageal Atresia, Am. I. RoentgenoI. Radium Ther. NucI. Med, 22: 463, 1929. Gross, R. E.: Surgery of Infancy and Childhood, Philadelphia, 1953, W. B. Saunders Company. Swenson, 0., Lipman, R., Fisher, I. H., and DeLuca, F.: Repair and Complications of Esophageal Atresia and Tracheoesophageal Fistula. N. Eng!. J. Med. 267: 960, 1962. Schwartz, S. I., and Dale, W. A.: Unusual Tracheoesophageal Fistulae With Membranous Obstruction of the Esophagus and Postoperative Hypertrophic Pyloric Stenosis, Ann. Surg. 142: 1002, 1955. Minnis, I. F., Jr., Burko, H., and Brevetti, G.: Segmental Duplication of the Esophagus Associated With Esophageal Atresia and Tra-
6
7 8 9
10 11
12
cheoesophageal Fistula, Ann. Surg. 156: 271, 1962. Wolf, R. Y., Duncan, L., and Pate, I. W.: Tracheoesophageal Fistula Associated With Esophageal Duplication, Surgery 58: 728, 1965. DaFoe, C. S., and Ross, C. A.: Tracheoesophageal Fistula and Esophageal Atresia. Dis. Chest 37: 42, 1960. Roe, B. B., and Nobis, P. D.: Congenital Tracheoesophageal Fistula: An Unusual Case, Dis. Child. 106: 489, 1963. Yahr, W. Z., Azzoni, A. A., and Santulli, T. V.: Congenital Atresia of the Esophagus With Tracheoesophageal Fistula: An Unusual Variant, Surgery 52: 937, 1962. Lister, I.: An Unusual Variation of Esophageal Atresia, Dis. Child. 38: 176, 1963. Holcomb, G. W., Jr., and Daniel, R. A., Jr.: Unusual Tracheoesophageal Fistulas With Membranous Atresia of the Middle or Distal Esophagus, Surgery 59: 1112, 1966. Goldenberg, I. S.: An Unusual Variation of Congenital Tracheoesophageal Fistula, I. THORAC. CARDIOVASC. SURG. 40: 114, 1960.
The major esophageal anomalies fall readily into three anatomic categories: esoph(1)
ageal atresia alone, (2) esophageal atresia with an associated tracheoesophageal fistula, and (3) tracheoesophageal fistula alone. The common classifications proposed by Vogt,' Gross," and Swenson" fail to emphasize that on occasion the anomaly does not clearly belong in one of these three major categories. It is important to emphasize that such cases do exist, because recognition of a variant is vital to the successful treatment. The following case history describes a rarely reported anomaly in a neonate-membranous esophageal obstruction and a tracheoesophageal fistula. Case report A term Caucasian male infant, weighing 3,750 grams, was referred to the Newborn Special Care Unit of the Yale-New Haven Hospital when he was 24 hours old for evaluation of a heart murmur and cyanosis. The pregnancy was complicated by obesity and hypertension. Delivery was spontaneous, and the Apgar score was 8 at I minute. Shortly thereafter the infant became cyanotic, and a Grade 3 of 6 heart murmur was audible over the precordium. On admission the pulse was 150 beats per minute and regular. Respirations were 50 breaths per minute. Moist rales and rhonchi were heard in both lung fields.
From the Department of Surgery, Section of Pediatric Surgery, Yale University School of Medicine, and the Yale-New Haven Hospital, New Haven, Conn. 06510. Received for publication June 23, 1972.
An easily palpable thrill was present at the left sternal border. Peripheral pulses were intact. The remainder of the physical examination was unremarkable. Complete blood count and urinalysis were within normal limits. Blood gases on admission were as follows: pH 7.37, Pco, 45 mm. Hg, and Po, 37 in room air but increasing to 62 mm. Hg in 100 per cent oxygen. A chest x-ray film revealed a boot-shaped left cardiac silhouette with densities in the right lung field suggestive of a pneumonic process. A nasogastric tube was introduced but lodged in the midportion of the esophagus and could not be advanced. Air passed freely through the tube during inspiration. Oily Dionosil, injected through the catheter, entered the major bronchi through a communication between the esophagus and the trachea (Fig. 1). After a tube gastrostomy, the esophagus was exposed through an extrapleural right thoracotomy. The esophagus was grossly normal in appearance but completely filled with air during inspiration. A single tracheoesophageal fistula, 3 mm. in size, was identified in the upper esophagus corresponding to the site of the intraluminal obstruction. After the fistula had been divided, the trachea and esophagus were closed individually with 5-0 arterial silk sutures. The postoperative course was unremarkable. Pneumonia cleared within a few days on amphicillin and kanamycin therapy. The upper pouch seemed totally obstructed, requiring continuous suction through a Replogle tube. The feedings were begun through the gastrostomy. Blood gases remained unchanged: Cardiac catheterization on the third postoperative day revealed D-transposition of the great vessels with tricuspid atresia, probable single ventricle, and pulmonic stenosis. Because there was adequate mixing and fair oxygenation, no cardiac surgery was contemplated. On the ninth postoperative day, repeat esophagography confirmed total obstruction of
191
1 92
Touloukian
The Journal of Tho racic and Ca rd iovas cular Surgery
Fig. 1. Preoperative study shows tip of catheter in upper esophageal pouch and contrast media entering trachea and major bronchi through the tracheoesophageal fistula.
Fig. :lA. The proximal esophagus is completely obstructed to antegrade flow of barium on the ninth day after division of the tracheoesophageal fistula.
the proximal esophagus to antegrade filling (Fig. 2A). The intraluminal web was clearly visible on retrograde filling of the esophagus through the gastrostomy (Fig. 2B). Two days later, esophageal continuity was restored by segmental resection of the esophagus, including the web. The anastomosis was performed with an inverting single layer of 5-0 arterial silk. A subsequent cineesophagogram revealed normal motility and adequate emptying of the esophagus (Fig . 3) . A schematic drawing of the original anomaly is shown in Fig. 4. During a 6 month follow-up period, the infant's growth and development have been normal, and his peripheral oxygenation has been adequate.
Discussion
Membranous esophageal obstruction with a tracheoesophageal fistula has anatomic similarities to the commonly observed form of esophageal atresia with a distal tracheoesophageal fistula. The obvious difference is the muscular continuity of the esophagus which belies the presence of an intraluminal obstruction. In a number of reported cases.v!' the proximal pouch actually overhung the distal esophagus , making location of the distal tracheoesophageal fistula more difficult; in some patients, the upper pouch extended to the diaphragm. Holcomb!' emphasized that a long tracheoesophageal fistula may enter the trachea just above the carina or extend to or above the thoracic inlet. In both cases, the esophagus is com-
Fig. :lB. The esophageal membrane is clearly visible on retrograde filling of the esophagus through the gastrostomy tube .
pletely separated by the membrane, and the fistula enters only the distal esophagus; thus infants with this anomaly are clearly analogous to their counterparts who have visibly separate esophageal segments. Holcomb! ' proposed that this anomaly be classified as Type G, according to Gross," or as ~IId, according to Vogt,' to give membranous obstruction of the esophagus wider recognition. Since the membrane only partially obstructed the esophagus in our patient, certain unique clinical differences existed. As the tracheoesophageal fistula lay just op-
Volume 65 Number 2
Esophageal obstruction with tracheoesophageal fistula
193
Februory . 1973
EsophaQeal Membrane
Fig. 3. Final result after resection of the obstructing membrane and direct esophageal anastomosis. Fig. 4. Schematic draw ing of the original anomaly.
posite the septum, allowing air to enter both the proximal and distal esophagus, the anomaly simulated atresia with a double fistulous communication. The true anatomic diagnosis was not apparent on the initial study, which demonstrated complete esophageal obstruction with prompt pulmonary aspiration through the tracheoesophageal fistula. Retrograde studies via the gastrostomy, performed after division of the tracheoesophageal fistula, indicated that obstruction was incomplete, with free reflux occurring around the web. The esophagus remained totally obstructed to antegrade passage of barium. A very similar case has been reported from our institution by Goldenberg.'" In addition to the septal obstruction and a tracheoesophageal fistula, a second fistula was identified at autopsy 1.5 cm. above the one which was recognized at operation. Death was attributed to right heart failure secondary to pneumonia, atrial and ventricular septal defects, bicuspid aortic valve, coarctation of the aorta, and a patent ductus arteriosus . Our patient also has severe congenital heart disease. The coexistent cardiac anomalies in these cases suggest that there was a major systemic insult early in gestation which had a widespread effect on the developing fetus. Aspiration through the proximal and distal esophageal segments requires prompt division of the tracheoesophageal fistula.
The urgency of this procedure is greater than with infants who have a blind upper pouch, since swallowed saliva directly enters the tracheobronchial tree through the fistula. Pharyngeal or indwelling sump suction is ineffective in retrieving all secretions before they reach the base of the pouch and , of course, gastric secretions intermittently reflux through the distal segment. Esophageal reconstruction may be delayed until pneumonia is treated and the obstruction clearly defined by contrast radiography. The membranous web may be excised, as reported by Goldenberg," and the esophagotomy incision closed transversely to minimize narrowing the lumen. We prefer to resect the web and perform an end-to-end anastomosis, since there is sufficient esophageal length to avoid tension and the risk of anastomotic stenosis. This approach is vindicated by the good result in our patient. Summary
A newborn infant with a partially obstructing esophageal web and a tracheoesophageal fistula was successfully treated by a twa-stage operation involving (1) division of the fistula and (2) subsequent esophageal reconstruction. The case is reported to emphasize that completely or partially obstructing esophageal webs ac-
1 94
The Journol of Thorocic ond Cordiovosculor
Touloukian
Surgery
companied by a tracheoesophageal fistula are rare variants of the common form of esophageal atresia which involves a distal tracheoesophageal fistula. REFERENCES
2 3
4
5
Vogt, E. C.: Congenital Esophageal Atresia, Am. I. RoentgenoI. Radium Ther. NucI. Med, 22: 463, 1929. Gross, R. E.: Surgery of Infancy and Childhood, Philadelphia, 1953, W. B. Saunders Company. Swenson, 0., Lipman, R., Fisher, I. H., and DeLuca, F.: Repair and Complications of Esophageal Atresia and Tracheoesophageal Fistula. N. Eng!. J. Med. 267: 960, 1962. Schwartz, S. I., and Dale, W. A.: Unusual Tracheoesophageal Fistulae With Membranous Obstruction of the Esophagus and Postoperative Hypertrophic Pyloric Stenosis, Ann. Surg. 142: 1002, 1955. Minnis, I. F., Jr., Burko, H., and Brevetti, G.: Segmental Duplication of the Esophagus Associated With Esophageal Atresia and Tra-
6
7 8 9
10 11
12
cheoesophageal Fistula, Ann. Surg. 156: 271, 1962. Wolf, R. Y., Duncan, L., and Pate, I. W.: Tracheoesophageal Fistula Associated With Esophageal Duplication, Surgery 58: 728, 1965. DaFoe, C. S., and Ross, C. A.: Tracheoesophageal Fistula and Esophageal Atresia. Dis. Chest 37: 42, 1960. Roe, B. B., and Nobis, P. D.: Congenital Tracheoesophageal Fistula: An Unusual Case, Dis. Child. 106: 489, 1963. Yahr, W. Z., Azzoni, A. A., and Santulli, T. V.: Congenital Atresia of the Esophagus With Tracheoesophageal Fistula: An Unusual Variant, Surgery 52: 937, 1962. Lister, I.: An Unusual Variation of Esophageal Atresia, Dis. Child. 38: 176, 1963. Holcomb, G. W., Jr., and Daniel, R. A., Jr.: Unusual Tracheoesophageal Fistulas With Membranous Atresia of the Middle or Distal Esophagus, Surgery 59: 1112, 1966. Goldenberg, I. S.: An Unusual Variation of Congenital Tracheoesophageal Fistula, I. THORAC. CARDIOVASC. SURG. 40: 114, 1960.