Ann Thorac Surg 1995;60:1790-1791
© 1995 The Society of Thoracic Surgeons
Case Report
Bleeding Esophageal Duplication Detected in Utero
Matthias Peiper, MD,
Wolfgang Lambrecht, MD,
Dietrich Kluth, MD,
Bernd Hüneke, MD
Department of Pediatric Surgery and Department of Gynecology and Obstetrics, University Hospital, Hamburg, Germany
Accepted for publication May 22, 1995.
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Abstract
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Peptic complications in duplications of the alimentary tract may occur when the duplication is lined by gastric mucosa. These complications commonly develop in the first year of life. We report a case of a bleeding esophageal duplication in a fetus.
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Introduction
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Duplications of the gastrointestinal tract are unusual congenital anomalies. They may be found anywhere between the mouth and anus. Symptoms develop in most patients in the first year of life [1, 2]. Peptic complications may occur when ectopic gastric mucosa is present. We observed a fetus in which a bleeding peptic ulcer developed inside an intrathoracic foregut duplication in the 35th week of gestation.
A 26-year-old woman was evaluated routinely at 21 weeks of gestation. Maternal ultrasound revealed a large cystic mass in the right hemithorax of the fetus with mediastinal shift to the left and severe left lung compression (Fig 1
). A presumptive diagnosis of cystic adenomatoid malformation of the lung was made. In an attempt to decompress the mass and to permit adequate lung growth, fetal thoracocentesis under sonographic guidance was performed nine times during the next few months. Between 20 and 50 mL of clear fluid was drained at each thoracocentesis. After each procedure, the mediastinum shifted transiently almost into a normal position. During the last thoracocentesis at 35 weeks of gestation 40 mL of bright red blood was aspirated. Because of the unknown origin of the bleeding, the mother was given betamethasone, and a cesarean section was performed.
The birth weight of the child was 2,200 g, and Apgar scores at 1, 5, and 10 minutes were 5, 9, and 9, respectively. Chest roentgenograms showed the right hemithorax filled by a mass, which shifted the heart and mediastinum slightly to the left side (Fig 2). Furthermore, D1 through D3 hemivertebrae were seen. Therefore, a bleeding foregut duplication cyst was suspected. The child was operated on 5 hours after birth. The right side of the chest was entered through the fifth intercostal space. Most of the thoracic cavity was filled by a large cystic mediastinal tumor, compressing the right lung. There was no communication to the esophagus or to infradiaphragmatic intestine. The cyst was resected totally. After the specimen was opened, blood drained. An ulcerated lesion 8 mm in diameter was found in the lower part. Histologic examination showed gastric lining mucosa, beneath which were layers of smooth muscle and a deep peptic ulcer. The postoperative course was uneventful. Six years after the operation the child has developed normally.
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Fig 2. . Chest roentgenogram taken immediately after birth showing the right hemithorax filled by a mass.
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Comment
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Duplications of the alimentary tract are defined as cystic or tubular structures lined by normal gastrointestinal mucosa and having smooth muscle walls. They typically lie in close proximity to the alimentary tube and frequently share a common muscular wall and common blood supply. The mucosa may resemble that of the proximate alimentary viscus, but the appearance varies: cysts lined by colonic mucosa have been found at the base of the tongue, and sinuses lined by gastric mucosa are found at the anus. Therefore, duplications of the alimentary tract are named for the structure with which they are associated rather than for the mucosal lining within them. The most common anomalies associated with duplications of the alimentary tract are the vertebral anomalies [3].
Esophageal duplications, round or tube-like, occur in the posterior mediastinum. They represent about 20% of all alimentary tract duplications [3]. A number of these thoracic duplications communicate with the upper small intestine via an extension through the esophageal hiatus, to join the duodenum, jejunum, or ileum [2].
Duplications can be detected in utero. The ultrasonographic differential diagnosis of fetal thoracic masses includes diaphragmatic hernia, cystic adenomatoid malformation of the lung, bronchogenic or enteric cysts, mediastinal cystic teratoma, and bronchial atresia or stenosis. The prognosis of a prenatally diagnosed thoracic lesion depends on its size. A large mass causes mediastinal shift, hypoplasia of normal lung tissue, polyhydramnios, and cardiovascular compromise leading to fetal hydrops and death. Hydrops is a harbinger of fetal or neonatal demise and is probably the result of vena caval obstruction or cardiac compression from extreme mediastinal shift [4]. Although our fetus had no signs of hydrops, he was treated by serial thoracocenteses because of the massive mediastinal shift, to prevent pulmonary hypoplasia. According to the experience of Adzick [4], serial thoracocenteses are ineffective because of rapid reaccumulation of cyst fluid. Thoracoamniotic shunts seem to be more effective, but are difficult to place, can lead to premature rupture of membrane, and are prone to migration and occlusion [4].
Esophageal duplications present with different symptoms depending on localization, size, and mucosal characteristics. Large cysts usually present with respiratory distress. Small ones may be found as asymptomatic masses on chest roentgenograms. Peptic complications can occur if the mucosal lining of the esophageal duplication is gastric tissue. This is described in about one third of the cases [1]. The acid and pepsin secretions may digest the cyst's mucosal lining locally, causing peptic ulceration or producing mucosal necrosis. Among 81 cases of thoracic duplications collected by Ware and Conrad [2], peptic ulcers developed in 14, and 5 of these cases ended fatally. These ulcers may perforate into different organs: esophagus, tracheobronchial tree, and pleural space. Perforation into the esophagus causes hematemesis or melena [5, 6]. When the cyst ruptures into the tracheobronchial tree, hemoptysis becomes the prominent symptom [6, 7]. Fatal hemorrhage into the bronchial tree has been recorded [5], and partial pulmonary resection or pneumonectomy has been lifesaving in some cases [8]. Rupture into the pleural space causes chemical pleurisy, pneumothorax, or empyema [5].
Unlike unruptured esophageal duplication cysts, which should be easily identifiable mediastinal masses, ruptured cysts may be obscured by the adjacent pleural and pulmonary disease. The fluid content of the cyst may be partially evacuated and replaced by air, thus resembling a lung or mediastinal abscess [5, 7]. Peptic complications usually develop in newborns or infants [5]. Our case demonstrates that bleeding complications due to gastric mucosa in an esophageal duplication can occur even in the fetal period.
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Footnotes
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Address reprint requests to Dr Lambrecht, Department of Pediatric Surgery, University Hospital, Martinistraße 52, 20246 Hamburg, Germany.
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References
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- Holcomb GW, Gheissaryi A, O'Neill JA, Shorter NA, Bishop HC. Surgical management of alimentary tract duplications. Ann Surg 1989;209:167–74.[Medline]
- Ware GW, Conrad HA. Thoracic duplication of alimentary tract. Am J Surg 1953;86:264–72.[Medline]
- Bissler JJ, Klein RL. Alimentary tract duplications in children. Clin Pediatr 1988;27:152–7.[Abstract/Free Full Text]
- Adzick NS. Fetal thoracic lesions. Semin Pediatr Surg 1993;2:103–8.[Medline]
- Macpherson RI, Reed MH, Ferguson CC. Intrathoracic gastrogenic cysts: a cause of lethal pulmonary hemorrhage in infants. J Assoc Can Radiol 1973;24:362–9.
- Chitale AR. Gastric cyst of the mediastinum. J Pediatr 1969;75:104–10.[Medline]
- Burgner DP, Carachi R, Beattie TJ. Foregut duplication cysts presenting as neonatal respiratory distress and haemoptysis. Thorax 1994;49:287–8.[Abstract/Free Full Text]
- Page US, Bigelow JC. A mediastinal gastric duplication leading to pneumonectomy. J Thorac Cardiovasc Surg 1967;54:291–4.
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Abstract
Introduction
