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Esophageal diverticulum in an infant with Down's syndrome and type III esophageal atresia
Esophageal Diverticulum in an Infant With Down's Syndrome and Type III Esophageal Atresia By Fernando L. Heinen, Pablo Vallone, and Gaston Elmo
Buenos Aires, Argentina
An 18-month-old infant with Down's syndrome presented with a symptomatic esophageal diverticulum (ED) located at the cervical esophagus. He had been operated on successfully for an esophageal atresia and distal tracheoesophageal fistula in the newborn period. Neither surgical maneuvers nor esophageal trauma could explain the ED, which was resected through a cervical approach.
J Pediatr Surg 38:E9. Copyright 2003, Elsevier Science (USA). All rights reserved.
SOPHAGEAL DIVERTICULA (ED) are uncommon in children. 1-3 The association with esophageal atresia has not been mentioned previously. We report herein on an 18-month-old infant with an ED located in the lower cervical esophagus. The ED became symptomatic at 17 months of age; it was not traumatic in origin and was not present in the newborn period, when a esophageal atresia with distal tracheoesophageal fistula (TEF) had been repaired successfully.
right recurrent laryngeal nerve, which was preserved. The ED was resected, and a transverse 2-layer suture closure of the esophagus was performed. A soft laminar rubber drain was left near the anastomosis for 2 days. A feeding tube was advanced intraoperatively and used for formula feeding for 7 days. A repeat contrast study showed an intact esophagus, and full oral alimentation has occurred over 6 months of follow-up. The specimen of the ED showed a complete esophageal wall without cartilage or respiratory epithelium.
CASE REPORT
Primary EDs are very rare in children and have been described in association with H-type TEF. 1,2 ED has no known association with Down's syndrome although duodenal diverticula have rarely been mentioned in these patients. 4 Intramural pseudodiverticulosis is a distinct entity characterized by multiple pinhole size dilatations of the submucosal glands or small saccular outpouching of the esophageal mucosa associated with reflux, mechanical obstruction of the esophagus, or infection (candidiasis)? Pseudodiverticular ballooning of the esophageal wall has been described after circular myotomies performed to permit anastomosis in the repair of a long gap esophageal atresia. Respiratory symptoms and dysphagia are common in these patients. 6 Infrequently, EDs have been mentioned after a traumatic esophageal instrumentation or foreign body impaction. 7-9 Pathological demonstration of 3 layers suggests that this was a true diverticula and not a diverticular duplication, which may contain carti-
E
An 18-month-old boy with Down's Syndrome had undergone surgery on his first day of life to correct esophageal atresia with a distal TEF through an extrapleural thoracotomy. The upper esophageal pouch had been easily dissected free from the surrounding tissues, and the distal fistula was divided and sutured. An end-to-end esophageal anastomosis was performed without undue tension. Neither a myotomy nor any other type of special surgical maneuver needed to be performed to approximate both esophageal ends. No postoperative complications were encountered, and oral feeding was started the seventh postoperative day. At the age of one month, mild choking during feeding prompted us to perform an esophageal contrast study. Anastomotic stenosis was detected, and a single balloon catheter radial dilatation under fluoroscopic and manometric control was performed. No traumatic or forceful maneuvers were exerted. No further stricture developed, and oral feeding pro-dressed uneventfully during the following 17 months. At this age, while the infant was thriving, coughing and choking episodes became evident during oral intake. A contrast x-ray study showed a lateral ED located at the level of the clavicle, several centimeters proximal to the anastomotic area (Fig 1). The esophageal lumen was widely patent without strictures, and the motility of the esophagus was normal without GE reflux. The above-mentioned occasional symptoms remained unchanged for several weeks. A hard milk curd the size of an almond, molded in the diverticulum, was expelled once during a coughing episode. Progressive enlargement of the ED seemed probable. Through a right oblique cervical incision along the anterior border of the sternocleidomastoid muscle, the cervical esophagus was exposed. After lateral retraction of the main carotid artery, the internal jugular vein, and the vagus nerve, the ED was clearly identified and dissected free. The insertion of a nasogastric tube facilitated the identification of the esophagus. The ED was in close relationship to the
Journal of Pediatric Surgery, Vol 38, No 4 (April), 2003:E9
INDEX WORDS: Esophageal diverticulum, esophageal atresia, Down's syndrome.
DISCUSSION
From the Department of Pediatric Surgery, Hospital Alem~n, Buenos Aires, Argentina. Address reprint requests to Fernando L. Heinen, MD, Chief of Pediatric Surgery, Hospital Alem~n (Deutsches Hospital), Av. Pueyrredhn 1640, (1118) Buenos Aires, Argentina. Copyright 2003, Elsevier Science (USA). All rights reserved. 1531-5037/03/3804-0033530. 00/0 doi: 10.1053/jpsu.2003.50147 9
10
HEINEN, VALLONE, AND ELMO
A f t e r repair o f e s o p h a g e a l atresia, c o u g h i n g a n d c h o k ing during f e e d i n g usually h a s b e e n a s s o c i a t e d w i t h e s o p h a g e a l stenosis, T E F r e c u r r e n c e , o n a tracheal div e r t i c u l u m at the p r e v i o u s TEF. 11,12 O u r c a s e r e p o r t s u g g e s t s that an E D also s h o u l d b e c o n s i d e r e d . REFERENCES
Fig 1. At 17 months of age, a contrast x-ray study in this patient showed a symptomatic lateral diverticulum at the distal cervical esophagus located at the level of the clavicle. Esophageal lumen was widely patent with a normal esophageal peristalsis.
lage and respiratory epithelium. 3 Esophageal dysmotility, achalasia, and gastroesophageal reflux often are associated with ED in adults, but no such functional disorders were detected in this infant. 1°
1. Borrie J, Wilson R: Oesophageal diverticula: Principles of management and appraisal of classification. Thorax 35:759-767, 1980 2. Cerro P, Fanucci E, letto F, et al: Diverticula of the pharyngoesophageal area. Radiol Med (Torino) 77:195-200, 1989 3. Ohbatake M, Muraji T, Yamazato M, et al: Congenital true diverticula of the esophagus: A case report. J Pediatr Surg 32:15921594, 1997 4. Sampliner J, Kollins SA, Hermann RE: Intraluminal Duodenal diverticulum associated with trisomy 21. Am J Roentgenol 127:677679, 1976 5. Lingaraj K, Prabhakaran K, Quak SH: Esophageal intramural pseudodiverticulosis associated with a web in a 12 year old boy. J Pediatr Surg 34:1573-1574, 1999 6. Otte JB, Gianello P, Wese FX, et al: Diverticulum formation after circular myotomy for esophageal atresia. J Pediatr Surg 19:68-71, 1984 7. Gorinati M, Dodero A, Semini V, et al: Traumatic pseudodiverticulum of the esophagus. Description of a case in a preterm newborn infant. Pediatr Med Chir 12:207-209, 1990 8. Wells SD, Leonidas JC, Conkle D, et al: Traumatic prevertebral pharyngoesophageal pseudodiverticulum in the newborn infant. J Pediatr Surg 9:217-222, 1974 9. Herman TE, McAlister WH: Esophageal diverticula in childhood associated with stricture from unsuspected foreign bodies of the esophagus. Pediatr Radiol 21:410-412, 1991 10. Castrucci G, Porziella V, Granone PL, et al: Tailored surgery for esophageal body diverticula. Eur J Coardiothorac Surg 14:380-387, 1998 11. Lequien P, Ponte C, Ribet M: Tracheal diverticulum after surgical correction of esophageal atresia. 2 cases. Arch Fr Pediatr 35:641-645, 1978 12. Bhatnagax V, Lal R, Agarwala S, et al: Endoscopic treatment of tracheal diverticulum after primary repair of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 33:1323-1324, 1998
Buenos Aires, Argentina
An 18-month-old infant with Down's syndrome presented with a symptomatic esophageal diverticulum (ED) located at the cervical esophagus. He had been operated on successfully for an esophageal atresia and distal tracheoesophageal fistula in the newborn period. Neither surgical maneuvers nor esophageal trauma could explain the ED, which was resected through a cervical approach.
J Pediatr Surg 38:E9. Copyright 2003, Elsevier Science (USA). All rights reserved.
SOPHAGEAL DIVERTICULA (ED) are uncommon in children. 1-3 The association with esophageal atresia has not been mentioned previously. We report herein on an 18-month-old infant with an ED located in the lower cervical esophagus. The ED became symptomatic at 17 months of age; it was not traumatic in origin and was not present in the newborn period, when a esophageal atresia with distal tracheoesophageal fistula (TEF) had been repaired successfully.
right recurrent laryngeal nerve, which was preserved. The ED was resected, and a transverse 2-layer suture closure of the esophagus was performed. A soft laminar rubber drain was left near the anastomosis for 2 days. A feeding tube was advanced intraoperatively and used for formula feeding for 7 days. A repeat contrast study showed an intact esophagus, and full oral alimentation has occurred over 6 months of follow-up. The specimen of the ED showed a complete esophageal wall without cartilage or respiratory epithelium.
CASE REPORT
Primary EDs are very rare in children and have been described in association with H-type TEF. 1,2 ED has no known association with Down's syndrome although duodenal diverticula have rarely been mentioned in these patients. 4 Intramural pseudodiverticulosis is a distinct entity characterized by multiple pinhole size dilatations of the submucosal glands or small saccular outpouching of the esophageal mucosa associated with reflux, mechanical obstruction of the esophagus, or infection (candidiasis)? Pseudodiverticular ballooning of the esophageal wall has been described after circular myotomies performed to permit anastomosis in the repair of a long gap esophageal atresia. Respiratory symptoms and dysphagia are common in these patients. 6 Infrequently, EDs have been mentioned after a traumatic esophageal instrumentation or foreign body impaction. 7-9 Pathological demonstration of 3 layers suggests that this was a true diverticula and not a diverticular duplication, which may contain carti-
E
An 18-month-old boy with Down's Syndrome had undergone surgery on his first day of life to correct esophageal atresia with a distal TEF through an extrapleural thoracotomy. The upper esophageal pouch had been easily dissected free from the surrounding tissues, and the distal fistula was divided and sutured. An end-to-end esophageal anastomosis was performed without undue tension. Neither a myotomy nor any other type of special surgical maneuver needed to be performed to approximate both esophageal ends. No postoperative complications were encountered, and oral feeding was started the seventh postoperative day. At the age of one month, mild choking during feeding prompted us to perform an esophageal contrast study. Anastomotic stenosis was detected, and a single balloon catheter radial dilatation under fluoroscopic and manometric control was performed. No traumatic or forceful maneuvers were exerted. No further stricture developed, and oral feeding pro-dressed uneventfully during the following 17 months. At this age, while the infant was thriving, coughing and choking episodes became evident during oral intake. A contrast x-ray study showed a lateral ED located at the level of the clavicle, several centimeters proximal to the anastomotic area (Fig 1). The esophageal lumen was widely patent without strictures, and the motility of the esophagus was normal without GE reflux. The above-mentioned occasional symptoms remained unchanged for several weeks. A hard milk curd the size of an almond, molded in the diverticulum, was expelled once during a coughing episode. Progressive enlargement of the ED seemed probable. Through a right oblique cervical incision along the anterior border of the sternocleidomastoid muscle, the cervical esophagus was exposed. After lateral retraction of the main carotid artery, the internal jugular vein, and the vagus nerve, the ED was clearly identified and dissected free. The insertion of a nasogastric tube facilitated the identification of the esophagus. The ED was in close relationship to the
Journal of Pediatric Surgery, Vol 38, No 4 (April), 2003:E9
INDEX WORDS: Esophageal diverticulum, esophageal atresia, Down's syndrome.
DISCUSSION
From the Department of Pediatric Surgery, Hospital Alem~n, Buenos Aires, Argentina. Address reprint requests to Fernando L. Heinen, MD, Chief of Pediatric Surgery, Hospital Alem~n (Deutsches Hospital), Av. Pueyrredhn 1640, (1118) Buenos Aires, Argentina. Copyright 2003, Elsevier Science (USA). All rights reserved. 1531-5037/03/3804-0033530. 00/0 doi: 10.1053/jpsu.2003.50147 9
10
HEINEN, VALLONE, AND ELMO
A f t e r repair o f e s o p h a g e a l atresia, c o u g h i n g a n d c h o k ing during f e e d i n g usually h a s b e e n a s s o c i a t e d w i t h e s o p h a g e a l stenosis, T E F r e c u r r e n c e , o n a tracheal div e r t i c u l u m at the p r e v i o u s TEF. 11,12 O u r c a s e r e p o r t s u g g e s t s that an E D also s h o u l d b e c o n s i d e r e d . REFERENCES
Fig 1. At 17 months of age, a contrast x-ray study in this patient showed a symptomatic lateral diverticulum at the distal cervical esophagus located at the level of the clavicle. Esophageal lumen was widely patent with a normal esophageal peristalsis.
lage and respiratory epithelium. 3 Esophageal dysmotility, achalasia, and gastroesophageal reflux often are associated with ED in adults, but no such functional disorders were detected in this infant. 1°
1. Borrie J, Wilson R: Oesophageal diverticula: Principles of management and appraisal of classification. Thorax 35:759-767, 1980 2. Cerro P, Fanucci E, letto F, et al: Diverticula of the pharyngoesophageal area. Radiol Med (Torino) 77:195-200, 1989 3. Ohbatake M, Muraji T, Yamazato M, et al: Congenital true diverticula of the esophagus: A case report. J Pediatr Surg 32:15921594, 1997 4. Sampliner J, Kollins SA, Hermann RE: Intraluminal Duodenal diverticulum associated with trisomy 21. Am J Roentgenol 127:677679, 1976 5. Lingaraj K, Prabhakaran K, Quak SH: Esophageal intramural pseudodiverticulosis associated with a web in a 12 year old boy. J Pediatr Surg 34:1573-1574, 1999 6. Otte JB, Gianello P, Wese FX, et al: Diverticulum formation after circular myotomy for esophageal atresia. J Pediatr Surg 19:68-71, 1984 7. Gorinati M, Dodero A, Semini V, et al: Traumatic pseudodiverticulum of the esophagus. Description of a case in a preterm newborn infant. Pediatr Med Chir 12:207-209, 1990 8. Wells SD, Leonidas JC, Conkle D, et al: Traumatic prevertebral pharyngoesophageal pseudodiverticulum in the newborn infant. J Pediatr Surg 9:217-222, 1974 9. Herman TE, McAlister WH: Esophageal diverticula in childhood associated with stricture from unsuspected foreign bodies of the esophagus. Pediatr Radiol 21:410-412, 1991 10. Castrucci G, Porziella V, Granone PL, et al: Tailored surgery for esophageal body diverticula. Eur J Coardiothorac Surg 14:380-387, 1998 11. Lequien P, Ponte C, Ribet M: Tracheal diverticulum after surgical correction of esophageal atresia. 2 cases. Arch Fr Pediatr 35:641-645, 1978 12. Bhatnagax V, Lal R, Agarwala S, et al: Endoscopic treatment of tracheal diverticulum after primary repair of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 33:1323-1324, 1998