Congenital Esophageal Atresia With Tracheoesophageal Fistula in Identical Twins By R. Ohkuma • This is a report of the seventh case of esophageal atresia in both members of a pair of identical twins.
IN 0 EX WO R OS: Esophageal atresia; twins.
C
O N G EN IT A L esophageal atresia with or without tracheoesophageal fistula occurring in both members of a pair of identical twins is extremely rare. Only six similar cases have been reported in world literature through 1975.'-6 Although esophageal atresia in twins is thought to be a developm en tal defect rather than a genetically transmitted one, possible presence of genetic predisposition can not be excluded. CASE REPORT
were admitted to Shimonoseki National Hospital in Japan on July i, 1975, I hr after birth, with cyanosis and respiratory distress. At the time of delivery, 2 amnions, I chorion, and a single placenta with 2 umbilical cords originating adjacent to each other near tbe center of the placenta were noted. The weight of each infant was ]900 g. From birth, both infants had excessive oral secretions and respiratory distress. Feeding was attempted on the third day, but the infants promptly regurgitated. On the fourth day, a catheter inserted into the esophagus could not be passed, and on plain abdominal x-ray intestinal gas was demonstrated, suggesting the presence of a tracheoesophageal fistula (Fig. I). Iodized oil placed into the esophageal pouch showed the blind end at the level of the third dorsal vertebra (Fig. 2). On the basis of these radiologic findings, esophageal atresia with distal tracheoesophageal fistula was diagnosed. Although coarse respiratory murmurs were heard in both infants, surgical intervention was undertaken immediately.
Identical twin female infants born to a 33-yr-old woman, whose membranes had ruptured prematurely I mo earlier,
Fig. 2. Iodized oil shows the blind pouch of the upper esophagus near the third dorsal vertebra.
Fig. 1. Catheter is seen to be coiled in the upper blind esophageal pouch and intestinal gas is visible in the abdomen on the plain x-ray with a catheter inserted into the esophagus.
Journal of Pediatric Surgery, Vol. 13. No.4 (August), 1978
From Shimonoseki National Hospital, Department oj Surgery, Shimonoseki, Japon. Address reprint requests to Ryusuke Ohkuma, M. D., Shimonoseki National Hospital, Dept. oj Surgery, Shimonoseki, Japan 751. © 1978 by Grune & Strauon.Tnc. 0022-3468/78/1304-0002$01.00/0 361
R. OHKUMA
362
Infant 1 was explored through a right transpleural approach. After freeing the inferior esophageal segment, the tracheoesophageal fistula was divided at its fistulous opening and the tracheal defect was closed. Both esophageal segments were mobilized, and a Haight anastomosis was done with some difficulty because of a 2-cm initial gap. The chest was closed and a Stamm gastrostomy was done. A similar approach was used in infant 2. The distance between the two ends of the esophagus in this case was much greater than in the first case; therefore primary anastomosis could not be performed.
RESULTS
Hypothermia and respiratory distress persisted postoperatively in both infants. Infant 2 died on the second postoperative day and infant Ion the fourth postoperative day. Because of the early postoperative death, phenotypic confirmation of identical twins was not possible. However, these twins were of the same sex and same blood type, had a similar appearance with
identical anomalies, and had two amnions but one chorion and a single placenta. They were most likely identical. REFERENCES I. Panse FR, Gierlich J: Zur Pathogenese der Anencephalie. Virchows Arch 316:135,1949 2. Wooley MM, Chinnock RF, Paul RH: Premature twins with esophageal atresia and tracheoesophageal fistula. Acta Paediatrica 50:423,1961 3. Blank RH, Prillaman PE, Minor GR; Congenital esophageal atresia with tracheoesophageal fistula occurring in identical twins. J Thorac Cardiovasc Surg 53:192, 1967 4. Farha SJ, Diener CH, Block PR: Esophageal atresia with tracheoesophageal fistula occurring in multiple birth. J Kans Med Soc 72:386, 1971 5. David TJ, O'Caliaghan SE: Twinning and esophageal atresia. Arch Dis Child 49:660, 1974 6. Yamada K, Tsunoda A, Nishi T, et al: Esophageal atresia occurring in the both members of identical twins. Syusankiigaku 5:892, 1975
IN 0 EX WO R OS: Esophageal atresia; twins.
C
O N G EN IT A L esophageal atresia with or without tracheoesophageal fistula occurring in both members of a pair of identical twins is extremely rare. Only six similar cases have been reported in world literature through 1975.'-6 Although esophageal atresia in twins is thought to be a developm en tal defect rather than a genetically transmitted one, possible presence of genetic predisposition can not be excluded. CASE REPORT
were admitted to Shimonoseki National Hospital in Japan on July i, 1975, I hr after birth, with cyanosis and respiratory distress. At the time of delivery, 2 amnions, I chorion, and a single placenta with 2 umbilical cords originating adjacent to each other near tbe center of the placenta were noted. The weight of each infant was ]900 g. From birth, both infants had excessive oral secretions and respiratory distress. Feeding was attempted on the third day, but the infants promptly regurgitated. On the fourth day, a catheter inserted into the esophagus could not be passed, and on plain abdominal x-ray intestinal gas was demonstrated, suggesting the presence of a tracheoesophageal fistula (Fig. I). Iodized oil placed into the esophageal pouch showed the blind end at the level of the third dorsal vertebra (Fig. 2). On the basis of these radiologic findings, esophageal atresia with distal tracheoesophageal fistula was diagnosed. Although coarse respiratory murmurs were heard in both infants, surgical intervention was undertaken immediately.
Identical twin female infants born to a 33-yr-old woman, whose membranes had ruptured prematurely I mo earlier,
Fig. 2. Iodized oil shows the blind pouch of the upper esophagus near the third dorsal vertebra.
Fig. 1. Catheter is seen to be coiled in the upper blind esophageal pouch and intestinal gas is visible in the abdomen on the plain x-ray with a catheter inserted into the esophagus.
Journal of Pediatric Surgery, Vol. 13. No.4 (August), 1978
From Shimonoseki National Hospital, Department oj Surgery, Shimonoseki, Japon. Address reprint requests to Ryusuke Ohkuma, M. D., Shimonoseki National Hospital, Dept. oj Surgery, Shimonoseki, Japan 751. © 1978 by Grune & Strauon.Tnc. 0022-3468/78/1304-0002$01.00/0 361
R. OHKUMA
362
Infant 1 was explored through a right transpleural approach. After freeing the inferior esophageal segment, the tracheoesophageal fistula was divided at its fistulous opening and the tracheal defect was closed. Both esophageal segments were mobilized, and a Haight anastomosis was done with some difficulty because of a 2-cm initial gap. The chest was closed and a Stamm gastrostomy was done. A similar approach was used in infant 2. The distance between the two ends of the esophagus in this case was much greater than in the first case; therefore primary anastomosis could not be performed.
RESULTS
Hypothermia and respiratory distress persisted postoperatively in both infants. Infant 2 died on the second postoperative day and infant Ion the fourth postoperative day. Because of the early postoperative death, phenotypic confirmation of identical twins was not possible. However, these twins were of the same sex and same blood type, had a similar appearance with
identical anomalies, and had two amnions but one chorion and a single placenta. They were most likely identical. REFERENCES I. Panse FR, Gierlich J: Zur Pathogenese der Anencephalie. Virchows Arch 316:135,1949 2. Wooley MM, Chinnock RF, Paul RH: Premature twins with esophageal atresia and tracheoesophageal fistula. Acta Paediatrica 50:423,1961 3. Blank RH, Prillaman PE, Minor GR; Congenital esophageal atresia with tracheoesophageal fistula occurring in identical twins. J Thorac Cardiovasc Surg 53:192, 1967 4. Farha SJ, Diener CH, Block PR: Esophageal atresia with tracheoesophageal fistula occurring in multiple birth. J Kans Med Soc 72:386, 1971 5. David TJ, O'Caliaghan SE: Twinning and esophageal atresia. Arch Dis Child 49:660, 1974 6. Yamada K, Tsunoda A, Nishi T, et al: Esophageal atresia occurring in the both members of identical twins. Syusankiigaku 5:892, 1975
