Foregut duplication cysts of the stomach with respiratory epithelium

Abstract

Gastrointestinal duplication is a congenital rare disease entity. Gastric duplication cysts seem to appear even more rarely. Herein, two duplications cysts of the stomach in a 46 year-old female patient are presented. Abdominal computed tomography demonstrated a cystic lesion attached to the posterior aspect of the gastric fundus, while upper gastrointestinal endoscopy was negative. An exploratory laparotomy revealed a non-communicating cyst and a smaller similar cyst embedded in the gastrosplenic ligament. Excision of both cysts along with the spleen was performed and pathology reported two smooth muscle coated cysts with a pseudostratified ciliated epithelial lining (respiratory type).

Key Words: Gastric, Cyst, Foregut, Duplication, Res-piratory

INTRODUCTION

Gastrointestinal duplications are rare and even more exceptional are those occurring in the stomach. As a general definition, a gastrointestinal duplication is a spherical hollow structure with a smooth muscle coat, lined by a mucous membrane and attached to any part of the gastrointestinal (GI) tract from the base of the tongue to the anus[1]. These malformations are believed to be congenital and are formed before differentiation of the lining epithelium and, therefore, are named for the organs with which they are associated[2]. Foregut duplications may or may not communicate with the GI tract and usually are diagnosed in a younger age; in adults non-specific symptoms delay diagnosis, which is established during surgical exploration[3]. Herein, two gastric duplication cysts with respiratory epithelium are reported.

CASE REPORT

A 46-year-old female patient was admitted to our Surgical Department from another medical center, where the patient was evaluated for an episode of loss of consciousness. Past medical history, present status and physical examination were non disease-specific. Diagnostic workup included an abdominal computed tomography (CT) which demonstrated a cystic lesion measuring 6 cm × 6 cm × 7 cm attached to the posterior wall of the gastric fundus, was well circumscribed and had no contrast enhancement (Figure 1). Interestingly CT coronary sections revealed a pulmonary sequestration in the basal segment of the left lower lobe (Figure 2). Moreover, upper GI endoscopy showed a bulging deformation of the gastric fundus by an extrinsic mass, without any mucosal abnormalities. An exploratory laparotomy was performed and revealed two cystic lesions; the first was attached to the fundus of the stomach and the second was embedded within the gastrosplenic ligament, close to the spleen. Therefore, intact excision of both cysts and splenectomy were carried out. Postoperative course was uneventful and the patient has been asymptomatic since then.

Figure 1 Abdominal CT scan demonstrating a cystic lesion attached to the posterior gastric wall (black arrow) and a pulmonary sequestration in the left pulmonary base (white arrow).

Figure 2 Coronary CT plate showing the pulmonary sequestration of the basal segment of the left lower lobe (arrow).

Pathologic examination of the surgical specimens reported a large cyst, measuring 8 cm × 5.5 cm in diameter and 0.3 cm thick and a smaller cyst (removed from the gastrosplenic ligament), measuring 3 cm in diameter and 0.2 cm thick. Both consisted of a smooth muscular wall, were lined by a pseudostratified, ciliated and columnar (respiratory type) epithelium and contained a clear, gelatinous fluid. Sub-epithelium seromucous glands were microscopically identified (Figure 3), a histologic appearance reminiscent of bronchial wall; however no cartilage was present.

Figure 3 Histological section of the cyst wall lined by a respiratory-type epithelium and containing seromucinous glands (HE, x 100).

DISCUSSION

Gastric duplications account for between 3% and 20% of gastrointestinal duplications[2,4,5] and occur twice as frequent in females as in males. Controversy exists over the embryological origin of these anomalies. Duplications of the stomach are usually single, less than 12 cm in diameter and located on the greater curvature or on the posterior or anterior gastric wall[2]. Wieczorek et al[6] reported that gastric duplications can be tubular or cystic; the cystic type does not communicate with the gastric lumen (about 80% of gastric duplication cysts[7]). The first case report of a gastric duplication was published in 1911 by Wendel[8] and approximately 150 cases have been reported since then[2].

Due to their position and mass effect, gastric duplications cysts are usually diagnosed in a younger age. However, in adults diagnosis may be difficult. A wide range of symptoms and signs have been reported and vary from asymptomatic to non disease-specific presentations, e.g. vague abdominal complaints, nausea, vomiting, epigastric fullness, weight loss, anemia, dysphagia, dyspepsia, etc. Vomiting usually occurs due to partial or complete gastric outlet obstruction, while even more emergent cases have been reported such as pancreatitis[9], hemoptysis[10] and gastrointestinal hemorrhage[11]. In our case, the female patient presented with loss of consciousness due to dehydration occurring as a result of excessive vomiting and concomitant anemia. Associated pathologic conditions including pulmonary sequestration[12], multicystic kidney[13] and neoplasias[14] have been reported. The presence of respiratory type mucosa and seromucinous glands along with the presence of pulmonary sequestration in the lower lobe of the ipsilateral lung in our case supports the theory of embryologic origin from supernumerary foregut buds.

Foregut duplications cysts of the stomach are usually incidentally diagnosed. Although upper gastrointestinal series demonstrate external pressure on the stomach, preoperative workup mainly includes abdominal ultrasonography and computed tomography scans, as well as endoscopic ultrasonography and magnetic resonance imaging[15,16].

Due to malignant transformation and the report of gastric cancer arising in duplications of the stomach[17] , surgical excision is considered to be the best treatment. Complete resection of the cyst is the ideal technique achieved with both open and laparoscopic[18] approaches. Unsuccessful approaches including percutaneous or endoscopic aspiration of cystic fluid have been reported[19], but are associated with complications, such as fistula formation and hemorrhage. When the recommended complete excision is not possible, converting both stomachs into one using a stapling device[20] or performing a segmental gastrectomy[21] are the possible alternatives.

In conclusion, foregut duplication cysts of the stomach are rare entities diagnosed incidentally and usually intraoperatively in adults and should be treated surgically by complete resection.