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Complete foregut duplication
Complete Foregut Duplication C. M. Dresler, MD, G. A. Patterson, MD, FRCS(C), B. R. Taylor, MD, FRCS(C), and D. J. Moote, MD Division of Thoracic Surgery, University of Toronto, Toronto, Ontario, Canada
Alimentary tract duplications are rare congenital anomalies with the majority identified in the pediatric age group. However, duplications may be seen in the adult population and require operative excision. A 53-year-old man was seen with vague, nonspecific symptoms and
T
rue duplications of the structures within the alimentary tract are rare and usually are seen in the pediatric age group. When the duplications in adults are referred to clinical attention, the symptoms are usually vague; however, 85% [l]will require operative intervention to relieve their symptoms. Esophageal duplications predominantly occur in the lower third of the esophagus (60%)with symptoms of dysphagia or epigastric discomfort [2]. Gastric duplications will frequently contain parietal cells, and with secretion of hydrochloric acid may first be seen as peptic ulceration. In the two largest reviews of alimentary tract duplication in adults [l]and children [3], only 3% to 4% were thoracoabdominal duplications. We report a patient with complete esophageal duplication in continuity with a gastric duplication traversing the diaphragm through a separate hiatus. The patient is a 53-year-old man who had chronic iron deficiency anemia and a vague past history of lethargy, weakness, frequent "pneumonias," and occasional right upper quadrant pain. The patient had been followed up in the past with abdominal ultrasounds, barium swallow
was found to have a complete esophageal and gastric duplication with communication to the normal alimentary tract. Appropriate-for-structure epithelium was noted throughout the length of the duplication. (Ann Thorac Surg 1990;50:306-8) and enema, and endoscopy, all of which were nondiagnostic. Recently, the patient again was observed with marked lethargy and with a hemoglobin level of 76 g/L (7.6 g/dL). Physical examination was completely unremarkable except for intermittent hemoccult-positive stools. All laboratory examinations were normal except for low iron and ferritin levels consistent with chronic blood loss. A chest roentgenogram demonstrated a posterior mediastinal air fluid level (Fig 1). An extensive workup of the posterior mediastinal air fluid level and anemia was begun. Computed tomography of the chest and abdomen demonstrated a large fluid-filled tubular structure alongside the esophagus beginning high in the left posterior mediastinum, extending into the right posterior mediastinum, and entering the abdomen through a diaphragmatic hiatus separate from the true esophageal hiatus (Fig 2). In addition, a round cystic structure was identified under the left lobe of the liver along the lesser curve of the stomach. The remainder of the computed tomographic scan including the spine was normal. External and endoscopic ultrasound confirmed the presence of the thoracic tubular
Fig I. Chest roentgenogram, posteroanterior and lateral views, demonstrates an air fluid level (arrowheads) in the duplication located in the posterior mediastinum.
Accepted for publication Feb 23, 1990 Address reprint requests to Dr Patterson, Toronto General Hospital, Eaton Wing 10-230, 200 Elizabeth St, Toronto, Ont, M5G 2C4, Canada.
0
1990 by The Society of Thoracic Surgeons
0003-4975/90/$3.50
Ann Thorac Surg 1990;50:306-8
CASE REPORT DRESLER ET AL COMPLETE FOREGUT DUPLICATION
307
Fig 2 . Computed tomographic images outlining the extent of tlie dirplication in the chest and the abdomen. In the upper thorax (A), the air-containing duplication (black arrows) arises to the left of the esophagus (white arrow). I n the tnidthorax ( B ) , the duplication (white arrows) is larger (5 cm), contains a n air fluid leziel, and is located to the riglit of the esophagus (black arrow). The duplication tiarrozi~at the level of the diaphragniatic crura (white arrows, C) and enters the abdornen t h r o q h a seporute hintus. Posterior to the lobe of the liirer ( D ) the abdominal i e ~ (5 cni) and is completely fluid filled ioitll a soft tissue rim (white arrows). portion of the duplication b t ~ o i ~ larger
structure that appeared to be in continuity with the intraabdominal cystic structure. Barium study of the esophagus and stomach were normal. A pouch extended off the medial wall of the second portion of the duodenum (Fig 3) but did not fill the epigastric cystic structure. Endoscopic assessment of the esophagus and stomach was normal. In the duodenum, the ampulla was felt to be slightly more posterior than usual and an out-pouching of mucosa was seen. When the mucosa was invaginated and filled with contrast medium, a defect similar to that on the upper gastrointestinal study was seen. A normal pancreatic duct was visualized. Contrast medium studies of the small and large bowels and colonoscopy were normal. Celiac, superior mesenteric, and inferior mesenteric arteriograms were normal showing no neovascularity, varicosities, or arteriovenous malformations. A Meckel scan was negative for ectopic gastric mucosa either in the chest or the abdomen. It was assumed that the patient had a complete foregut duplication with intermittent hemorrhage into the duplication. Right posterolateral thoracotomy was performed.
The esophageal portion from thoracic inlet to diaphragmatic hiatus was excised first. Cephalad, the duplication was ligated as it narrowed to a 0.5 cm diameter at the thoracic inlet. Distally, the lesion was dissected from its separate hiatus and transected at that level. The diaphragmatic defect and thoracotomy were then closed. An upper midline laparotomy was performed, and the abdominal portion was excised after its identification in the retroperitoneum. The duplication was opened and found to be lined with hemorrhagic, ulcerated mucosa. A 3-mm biliary probe was passed through a small distal tract through the head of the pancreas and into the second portion of duodenum. The duplication was dissected down to the level of this tract and divided distally with a gastrointestinal anastomosis stapler. No abnormal vascular supply was encountered. The esophageal component contained normal squamous epithelium and muscular walls. The abdominal component had normal gastric mucosa with areas of ulceration and hemorrhage. Postoperatively, the patient did well and was dis-
308
CASE REPORT DRESLER ET AL COMPLETE FOREGUT DUPLICATION
Fig 3 . Barium fills the distal aspect of the duplication in a retrograde fashion (black arrow). Mucosal folds (white arrows) are identified where the duplication inserts into the second portion of the duodenum.
charged on postoperative day 10. His hemoglobin level has remained normal 1 year after resection.
Comment This report presents a case of a complete foregut duplication with the normal, mature mucosa belonging to the appropriate structure. Alimentary tract duplications are unusual even in the pediatric population. Only 10% to 15% of alimentary tract duplications involve the esophagus, with approximately 60% being in the distal portion [2]. The majority of alimentary tract duplications are first seen in the pediatric age group with symptoms of dyspnea, dysphagia, recurrent pneumonia, and failure to thrive. Approximately one third of esophageal duplications are identified in adults, and they are frequently asymptomatic. Duplication of the esophagus is a congenital anomaly
Ann Thorac Surg 1990;50:30&8
that may begin to develop as early as the fourth week of gestation. As the foregut epithelium develops, it elongates, develops a lumen, and undergoes dextro-rotation. Thus, the majority of esophageal duplications occur distally and on the right. In a recent review of the literature [2], only six tubular duplications of 44 esophageal duplications were identified. They varied in length from 2 to 36 cm in length, and no mention was made of continuity with a duplicated stomach. In another large review of alimentary tract duplications [3], 22%were intrathoracic, and all were cystic rather than tubular. Eight percent had cystic gastric duplications along the greater curvature and did not communicate with the stomach. Three percent had thoracoabdominal duplication associated with thoracic vertebral anomalies. There were no recorded esophageal-gastric duplications. Other rare thoracoabdominal duplications have been identified [3] as arising from various portions of small or large bowel and extending into the thorax through a separate diaphragmatic hiatus. All alimentary duplications extending into the thorax contain alimentary tract mucosa with the most common being gastric mucosa. The majority of these duplications are closed, and less than 10% communicate with the normal alimentary tract. None have been identified to include a normal stomach with gastric mucosa in continuity with an esophagus that had normal squamous epithelium. In the pediatric literature, ulceration of a thoracic esophageal duplication lined with gastric mucosa may occur with resultant hemorrhage. Although our patient is not within the pediatric age group, his symptoms had been present, by his estimation, since he was 18 years old. Recommended therapy for symptomatic lesions has been surgical excision. Initial attempts at marsupialization resulted in many complications making complete excision of the duplication a more favored option [3, 41. Operative morbidity and mortality are low, and recurrent symptoms have not been reported. Complete operative excision of alimentary duplications is the management of choice.
References 1. Anderson MC, Silberman WW, Shields TW. Duplications of the alimentary tract in the adult. Arch Surg 1962;85:110-24. 2. Arbona JL, Figueroa F, Mayoral J. Congenital esophageal cysts: case report and review of literature. Am J Gastroenterol 1984;79:177-82. 3. Holcomb GW, Gheissari A, O'Neill ]A, Shorter NA, Bishop HC. Surgical management of alimentary tract duplications. Ann Surg 1988;20:167-75. 4. Arnorsson T, Aberg C, Aberg T. Benign tumours of the oesophagus and oesophageal cysts. Scand J Thorac Cardiovasc Surg 1984;18:145-50.
Alimentary tract duplications are rare congenital anomalies with the majority identified in the pediatric age group. However, duplications may be seen in the adult population and require operative excision. A 53-year-old man was seen with vague, nonspecific symptoms and
T
rue duplications of the structures within the alimentary tract are rare and usually are seen in the pediatric age group. When the duplications in adults are referred to clinical attention, the symptoms are usually vague; however, 85% [l]will require operative intervention to relieve their symptoms. Esophageal duplications predominantly occur in the lower third of the esophagus (60%)with symptoms of dysphagia or epigastric discomfort [2]. Gastric duplications will frequently contain parietal cells, and with secretion of hydrochloric acid may first be seen as peptic ulceration. In the two largest reviews of alimentary tract duplication in adults [l]and children [3], only 3% to 4% were thoracoabdominal duplications. We report a patient with complete esophageal duplication in continuity with a gastric duplication traversing the diaphragm through a separate hiatus. The patient is a 53-year-old man who had chronic iron deficiency anemia and a vague past history of lethargy, weakness, frequent "pneumonias," and occasional right upper quadrant pain. The patient had been followed up in the past with abdominal ultrasounds, barium swallow
was found to have a complete esophageal and gastric duplication with communication to the normal alimentary tract. Appropriate-for-structure epithelium was noted throughout the length of the duplication. (Ann Thorac Surg 1990;50:306-8) and enema, and endoscopy, all of which were nondiagnostic. Recently, the patient again was observed with marked lethargy and with a hemoglobin level of 76 g/L (7.6 g/dL). Physical examination was completely unremarkable except for intermittent hemoccult-positive stools. All laboratory examinations were normal except for low iron and ferritin levels consistent with chronic blood loss. A chest roentgenogram demonstrated a posterior mediastinal air fluid level (Fig 1). An extensive workup of the posterior mediastinal air fluid level and anemia was begun. Computed tomography of the chest and abdomen demonstrated a large fluid-filled tubular structure alongside the esophagus beginning high in the left posterior mediastinum, extending into the right posterior mediastinum, and entering the abdomen through a diaphragmatic hiatus separate from the true esophageal hiatus (Fig 2). In addition, a round cystic structure was identified under the left lobe of the liver along the lesser curve of the stomach. The remainder of the computed tomographic scan including the spine was normal. External and endoscopic ultrasound confirmed the presence of the thoracic tubular
Fig I. Chest roentgenogram, posteroanterior and lateral views, demonstrates an air fluid level (arrowheads) in the duplication located in the posterior mediastinum.
Accepted for publication Feb 23, 1990 Address reprint requests to Dr Patterson, Toronto General Hospital, Eaton Wing 10-230, 200 Elizabeth St, Toronto, Ont, M5G 2C4, Canada.
0
1990 by The Society of Thoracic Surgeons
0003-4975/90/$3.50
Ann Thorac Surg 1990;50:306-8
CASE REPORT DRESLER ET AL COMPLETE FOREGUT DUPLICATION
307
Fig 2 . Computed tomographic images outlining the extent of tlie dirplication in the chest and the abdomen. In the upper thorax (A), the air-containing duplication (black arrows) arises to the left of the esophagus (white arrow). I n the tnidthorax ( B ) , the duplication (white arrows) is larger (5 cm), contains a n air fluid leziel, and is located to the riglit of the esophagus (black arrow). The duplication tiarrozi~at the level of the diaphragniatic crura (white arrows, C) and enters the abdornen t h r o q h a seporute hintus. Posterior to the lobe of the liirer ( D ) the abdominal i e ~ (5 cni) and is completely fluid filled ioitll a soft tissue rim (white arrows). portion of the duplication b t ~ o i ~ larger
structure that appeared to be in continuity with the intraabdominal cystic structure. Barium study of the esophagus and stomach were normal. A pouch extended off the medial wall of the second portion of the duodenum (Fig 3) but did not fill the epigastric cystic structure. Endoscopic assessment of the esophagus and stomach was normal. In the duodenum, the ampulla was felt to be slightly more posterior than usual and an out-pouching of mucosa was seen. When the mucosa was invaginated and filled with contrast medium, a defect similar to that on the upper gastrointestinal study was seen. A normal pancreatic duct was visualized. Contrast medium studies of the small and large bowels and colonoscopy were normal. Celiac, superior mesenteric, and inferior mesenteric arteriograms were normal showing no neovascularity, varicosities, or arteriovenous malformations. A Meckel scan was negative for ectopic gastric mucosa either in the chest or the abdomen. It was assumed that the patient had a complete foregut duplication with intermittent hemorrhage into the duplication. Right posterolateral thoracotomy was performed.
The esophageal portion from thoracic inlet to diaphragmatic hiatus was excised first. Cephalad, the duplication was ligated as it narrowed to a 0.5 cm diameter at the thoracic inlet. Distally, the lesion was dissected from its separate hiatus and transected at that level. The diaphragmatic defect and thoracotomy were then closed. An upper midline laparotomy was performed, and the abdominal portion was excised after its identification in the retroperitoneum. The duplication was opened and found to be lined with hemorrhagic, ulcerated mucosa. A 3-mm biliary probe was passed through a small distal tract through the head of the pancreas and into the second portion of duodenum. The duplication was dissected down to the level of this tract and divided distally with a gastrointestinal anastomosis stapler. No abnormal vascular supply was encountered. The esophageal component contained normal squamous epithelium and muscular walls. The abdominal component had normal gastric mucosa with areas of ulceration and hemorrhage. Postoperatively, the patient did well and was dis-
308
CASE REPORT DRESLER ET AL COMPLETE FOREGUT DUPLICATION
Fig 3 . Barium fills the distal aspect of the duplication in a retrograde fashion (black arrow). Mucosal folds (white arrows) are identified where the duplication inserts into the second portion of the duodenum.
charged on postoperative day 10. His hemoglobin level has remained normal 1 year after resection.
Comment This report presents a case of a complete foregut duplication with the normal, mature mucosa belonging to the appropriate structure. Alimentary tract duplications are unusual even in the pediatric population. Only 10% to 15% of alimentary tract duplications involve the esophagus, with approximately 60% being in the distal portion [2]. The majority of alimentary tract duplications are first seen in the pediatric age group with symptoms of dyspnea, dysphagia, recurrent pneumonia, and failure to thrive. Approximately one third of esophageal duplications are identified in adults, and they are frequently asymptomatic. Duplication of the esophagus is a congenital anomaly
Ann Thorac Surg 1990;50:30&8
that may begin to develop as early as the fourth week of gestation. As the foregut epithelium develops, it elongates, develops a lumen, and undergoes dextro-rotation. Thus, the majority of esophageal duplications occur distally and on the right. In a recent review of the literature [2], only six tubular duplications of 44 esophageal duplications were identified. They varied in length from 2 to 36 cm in length, and no mention was made of continuity with a duplicated stomach. In another large review of alimentary tract duplications [3], 22%were intrathoracic, and all were cystic rather than tubular. Eight percent had cystic gastric duplications along the greater curvature and did not communicate with the stomach. Three percent had thoracoabdominal duplication associated with thoracic vertebral anomalies. There were no recorded esophageal-gastric duplications. Other rare thoracoabdominal duplications have been identified [3] as arising from various portions of small or large bowel and extending into the thorax through a separate diaphragmatic hiatus. All alimentary duplications extending into the thorax contain alimentary tract mucosa with the most common being gastric mucosa. The majority of these duplications are closed, and less than 10% communicate with the normal alimentary tract. None have been identified to include a normal stomach with gastric mucosa in continuity with an esophagus that had normal squamous epithelium. In the pediatric literature, ulceration of a thoracic esophageal duplication lined with gastric mucosa may occur with resultant hemorrhage. Although our patient is not within the pediatric age group, his symptoms had been present, by his estimation, since he was 18 years old. Recommended therapy for symptomatic lesions has been surgical excision. Initial attempts at marsupialization resulted in many complications making complete excision of the duplication a more favored option [3, 41. Operative morbidity and mortality are low, and recurrent symptoms have not been reported. Complete operative excision of alimentary duplications is the management of choice.
References 1. Anderson MC, Silberman WW, Shields TW. Duplications of the alimentary tract in the adult. Arch Surg 1962;85:110-24. 2. Arbona JL, Figueroa F, Mayoral J. Congenital esophageal cysts: case report and review of literature. Am J Gastroenterol 1984;79:177-82. 3. Holcomb GW, Gheissari A, O'Neill ]A, Shorter NA, Bishop HC. Surgical management of alimentary tract duplications. Ann Surg 1988;20:167-75. 4. Arnorsson T, Aberg C, Aberg T. Benign tumours of the oesophagus and oesophageal cysts. Scand J Thorac Cardiovasc Surg 1984;18:145-50.