Background

Alimentary tract duplications are rare anomalies and commonly diagnosed in the first few years of life [1]. Delayed diagnosis or misdiagnoses often leads to the occurrence of complications [2]. Gastric duplications cyst accounts for 4–7% of alimentary tract duplications. It may develop complications such as infection of the cyst, peritonitis secondary to its perforation, and hemorrhage [3, 4]. Herein, we present a unique presentation of complicated gastric duplications cyst that proved a diagnostic as well as management dilemma.

Case presentation

A 2.5-year-old girl presented in the outpatient department with progressive abdominal distension for the last 6 months, and abdominal pain (appreciated as inconsolable cry), and fever for the last 5 days. Past medical history showed that the girl initially presented at the age of 1 year with fever and abdominal pain in the Gastroenterology Department. Ultrasound followed by CT scan (Fig. 1) of the abdomen was performed which gave a suspicion of liver abscess. The abscess was aspirated under ultrasound guidance, and a pigtail catheter was inserted. The patient was discharged in good condition. However, a month later, the patient again presented with an epigastric swelling in addition to abdominal pain and fever. Examination revealed a fluctuant inflammatory epigastric swelling with upper abdominal distension. CT scan performed again and showed a residual abscess in the left lobe of the liver, tracking into the epigastrium. The patient was shifted to the Pediatric Surgery Department where open drainage of the abscess was done. About 150 ml frank pus was drained. Postoperatively, the fever did not settle; follow-up ultrasound revealed residual abscess which was aspirated (ultrasound-guided). The histopathology showed a pyogenic abscess, but no organism could be detected on cultures. The patient was then discharged in good condition.

Fig. 1
figure 1

Showing a well-circumscribed collection in the left lobe of the liver (*). Note a hypodense area in relation to stomach representing gastric duplication cyst (white arrow)

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During current admission, she was febrile with the following vitals: temp 101 °F, pulse 116/min, R/R 24/min, and BP 90/60. The abdomen was distended with mild tenderness in the upper abdomen; a cystic mass (14 × 10 cm) was palpable in the epigastrium and left hypochondrium. The lower abdomen was soft with normal bowel sounds. The rest of the systemic examination was unremarkable. The patient was admitted to the ward and an ultrasound was planned, but during admission, the patient became suddenly pale with accentuation of abdominal distension. The CBC which was initially 8 g/dl dropped to 3 g/dl when repeated. The patient also developed tachycardia and tachypnea. The patient was managed with IV fluids and packed red blood cells were transfused. After stabilization, an urgent CT scan was performed which showed a cystic intraperitoneal collection (Fig. 2).

Fig. 2
figure 2

Showing a huge cystic collection in the abdomen (*). Note a hypodense area in relation to stomach representing gastric duplication cyst (white arrow)

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The patient underwent exploratory laparotomy electively using a supraumbilical transverse abdominal incision. There was a huge cyst in the epigastrium and left hypochondrium, filled with hemorrhagic fluid. The cyst was aspirated to assist its dissection from surrounding viscera. The cyst had communication with another smaller cyst that was intimately attached to the greater curvature of the stomach (Fig. 3). Both cysts were excised leaving behind a small seromuscular defect of the greater curvature of the stomach which was repaired with a single layer of interrupted sutures. Postoperative recovery was uneventful, and the patient was discharged in good clinical condition on the 7th postoperative day. Figure 4 describes the timeline of the events. The histopathology of the bigger cyst divulged as a pseudocyst, whereas that of the smaller cyst as gastric duplication cyst. The patient is doing fine on a follow-up of 12 months.

Fig. 3
figure 3

Intraoperative photograph showing gastric duplication (D) intimately attached to the greater curvature of the stomach (S). The opened pseudocyst (P) is also visible

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figure 4

Fig. 4

Discussion

Alimentary tract duplications are characterized by intimate contact with any part of the gastrointestinal tract (GIT), smooth muscles in the wall, and lined by the mucosa of GIT. These are named after the part to which they are intimately attached [1]. Gastric duplication cysts are usually attached to the greater curvature of the stomach and often do not communicate with the gastric lumen [5]. In the present case, the gastric duplication cyst was not communicating with the gastric lumen and fulfilled all the criteria of a classical GIT duplication cyst.

Gastric duplication cyst often presents variably ranging from merely incidental finding to as severe as acute peritonitis or hemorrhagic shock. Those arising from the antropyloric region may impinge the gastric outlet and thus present with vomiting. Few communicating gastric duplication cysts may present with hematemesis [6]. Infection or perforation of the cyst may result in an acute abdomen, thus necessitating urgent surgical intervention [1]. Occasionally, the patient may develop anemia secondary to spontaneous bleeding within the cyst. In the present case, multiple complications were encountered accounting for delayed as well as inappropriate management. Most plausibly, a perforation of gastric duplication cyst brought about peritonitis which was initially simulated liver abscess; later, it might have developed a huge pseudocyst that masqueraded the associated gastric duplication cyst.

The diagnosis can be based on imaging studies. X-rays may show the mass effect of large duplication cyst but unable to confirm the diagnosis. Ultrasound abdomen may aid in diagnosis by showing gut signature sign of the cyst, but this is an operator-dependent modality. A contrast-enhanced CT scan is an investigation of choice for the diagnosis of GIT duplications. It will show relations of the cyst with the surrounding viscera. Any complications if present will also be delineated well by this modality [6,7,8].

The present case was a diagnostic as well as a management challenge. On initial presentation, it was reported as hepatic abscess and dealt with erroneously. Subsequently, the patient developed sudden accentuation of cyst size due to intracystic bleeding as depicted by the appearance of sudden pallor in the present case. CT scan again could not identify the gastric duplication though on the retrospective review of the CT films, a hypodense cystic structure could be seen in relation to the stomach (Fig. 1 and 2).

An extensive literature review was performed regarding the perforated gastric duplication cyst. About 22 cases of perforated gastric duplication could be retrieved (Table 1). The gastric duplication cyst after perforation may either cause peritonitis [1] or it may develop fistulous communication with the structures in the vicinity. The fistulous communications are reported with the colon, pancreas, spleen, anterior abdominal wall, and lower lobe of the left lung through the diaphragm [9,10,11,12,13,14,15]. Interestingly, 2 cases of pseudocysts formation are also reported as a complication of perforated gastric duplication cyst [4, 16].

Table 1 Showing the summary of cases of perforated gastric duplications cyst found on literature search
Full size table

The ultimate management is complete surgical excision as malignant transformations in gastric duplications have been reported. The excision may involve a partial gastrectomy if a small gastric area is involved [12]. In case of extensive gastric attachment, excision of the free part and mucosal stripping of the common wall is preferred [1]. In the present case, the gastric duplication was completely excised leaving behind a serosal defect which was repaired.

Conclusion

Complications in gastric duplication cyst may lead to misdiagnosis and maltreatment. Careful reporting of imaging investigations is required to identify these lesions as they can masquerade the actual pathology leading to diagnostic and management issues.