Enteric Duplication Cyst Perforation in Elderly: A Rare Presentation

Introduction

Enteric duplication cysts are rare and usually manifest in the paediatric age group. It has an incidence of 0.2% in children, with a male predominance.¹ Enteric duplication is rare in adults and in elderly; only a few cases are reported so far. In adults, it is usually asymptomatic or may present with vague abdominal symptoms. Enteric cyst may arise anywhere in the alimentary tract from mouth to anus. They are found connected to the bowel, with a well-defined muscular coat and lined by the intestinal mucosa.^1,2

Most common site of enteric duplication in gastrointestinal tract is the ileum (33%), followed by oesophagus (20%) and colon (13%).^1,2 Enteric cyst may present clinically with features of intestinal obstruction, perforation, haemorrhage and malignancy, which may complicate the condition.³ Hereby, we present a rare case of enteric duplication cyst in an elderly patient who presented with perforation.

Case Report

A 62-year old male patient presented with a history of diffuse pain in the abdomen from two days, which was associated with four episodes of bilious vomiting and fever. He had not passed stools for two days. On examination, abdomen was distended with diffuse tenderness and guarding was present all over the abdomen. However, there was no rigidity. Laboratory parameters showed raised total leucocyte count (25,000 /mm3 ) (neutrophils 94%). Erect X- ray abdomen (Figure 1) showed no signs of gas under the diaphragm and ultrasound of abdomen and pelvis was normal.

CECT abdomen (Figure 2) showed mild free fluid in the peritoneal cavity and free air in the abdomen which was suggestive of perforation peritonitis. Preoperatively, the provisional diagnosis of sealed peptic/ enteric perforation was made.

Patient was prepared pre operatively with IV fluids and antibiotics. The patient was taken for emergency exploratory laparotomy. Intra-operatively, an enteric duplication cyst measuring 10×7×5 cm which was perforated and communicating with the lumen of jejunum (Figure 3 & 4) was observed.

Complete excision of the enteric duplication cyst with resection of the involved jejunum with end-to-end anastomosis was done. Postoperative period was uneventful. Histopathological study (Figure 5) revealed cyst with epithelial lining and wall containing smooth muscles arranged in circular and longitudinal fashion, the features suggestive of enteric duplication cyst.

Discussion

Duplication cysts were first described by Wendel in 1911.^2,4 Since then, a few cases were reported in adults. This condition is usually predominant in males. Unrecognized, asymptomatic cysts may present with adenocarcinoma during the 4th and 5th decades of life. The enteric duplication cyst can be associated with malrotation.^4,5 Duplication cysts are rare congenital anomalies presenting in paediatric patients, seen most commonly in the ileum.² In the present case, the enteric duplication cyst was connected with jejunum.

The aetiology of the enteric duplication cyst is unknown. However, the accepted theories include a persistence of foetal gut diverticula, intrauterine vascular occlusion, and a defect in the recanalization of the primitive gut.⁷ Any failure in the transition process of the intestinal tract from solid to tubular state between 6 and 8 weeks of intrauterine life may lead to formation of duplication cysts.^7,8 These cysts have an outer muscular layer with an inner gastrointestinal epithelium.⁴ True enteric duplication cyst is classically characterised by three criteria: attachment to any part of the gastrointestinal tract, lined by any gastrointestinal epithelium and containing smooth muscle in its wall. It may be cystic or tubular. Cystic types are more common. They do not communicate with the bowel, but may share a common wall and blood supply.

Tubular duplication cysts communicate with the intestinal lumen. They typically derive both their vascular supply and their smooth muscle wall from the adjacent intestinal segment, though they rarely have an independent blood supply.⁹ Duplication cysts are commonly detected in early life between 1-2 years of age with intestinal obstruction or a palpable mass. In adults, most enteric duplication cysts are asymptomatic. They may present with vague abdominal symptoms. However, they may also present acutely in a setting of obstruction, haemorrhage or perforation.³ Initial symptomatology depends upon the size and location of the lesion and most commonly includes obstruction, with or without vomiting, abdominal distension, dysphagia, palpable mass, or more infrequently, urinary symptoms.¹⁰ However, in our case, patient presented in the sixth decade of his life with features of perforation peritonitis.

Complications include perforation, intussusception, bowel obstruction from adjacent pressure or mass effect, volvulus, and associated malignancy. In rare conditions, malignancies arising within duplication cysts typically have a poor prognosis, as most of the cases are diagnosed at an advanced stage with widespread metastases. Reported cases have included squamous cell carcinoma, adenocarcinoma, and carcinoid tumors.^11,12 However, in our case, the patient presented with complication of enteric duplication cyst perforation.

Detection and diagnosis of enteric cysts can be difficult, as their presentation varies. Imaging can aid in the diagnosis. The preoperative diagnosis can be made with USG and CT abdomen. The cystic nature of the lesion is visible on USG. The diagnosis can be confirmed even on USG if the characteristic inner echogenic layer and the hypoechogenic outer muscular layer are well defined.^6,13 However in our case, the ultrasonogram failed to diagnose enteric duplication cyst and abdominal CT showed mild free fluid with free air in the peritoneal cavity suggestive of perforation peritonitis, but no enteric duplication cyst was detected preoperatively.

Surgery is recommended for symptomatic cases or when a complication arises. Till date, there is no consensus on the management of asymptomatic cases.^14,15 However, it is considered beneficial to surgically treat these cases due to risk of malignant change in duplication cysts. Surgery includes resection of the involved bowel due to the sharing of both muscular wall and vascular supply. In a completely isolated cyst, excision of enteric duplication cyst may be done without resecting the bowel.^14,15 In our case, the patient had a symptomatic duplication cyst with perforation in the enteric cyst and underwent surgical excision of the cyst along with connected jejunum. Overall, surgical excision is the standard treatment for symptomatic enteric duplications. When the segment of affected bowel is too large to resect, alternative treatment options include selective stripping of the affected intestinal mucosa.^4,11

Conclusion

A differential diagnosis of duplication cyst should be considered in adult patients with cystic lesions of the gastro-intestinal tract. Surgical treatment is necessary due to the probable risk of malignancy in duplication cysts.

Conflict of Interest

Nil