Enteric Duplication Cysts in Children: A Clinicopathological Dilemma

Abstract

Aim: Enteric duplication cysts are rare and uncommon congenital malformations formed during the embryonic period of the development of human digestive system and are mainly encountered during infancy or early childhood, but seldom in adults. The clinical presentation is extremely variable depending upon its size, location and type. We present six cases of enteric duplication cysts with diverse clinico-pathological features.

Materials and methods: This study was carried out in the Department of Pathology and Department of Paediatric Surgery, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India for a period of 2 years (January 2013 - December 2014). We retrospectively analyzed six patients of enteric duplication cysts based on data obtained, which consisted of patient's age, sex, clinical presentation, radiological features, operative findings and histopathology report. The data collected was analyzed by descriptive statistics.

Results: Six children between age range of 3 days to 10 years had enteric duplication cysts. Two had ileal and one each were of pyloroduodenal, colonic and rectal duplication cyst. In one patient a presumptive diagnosis of enteric duplication cyst was made. Radiology played an important contributory role in diagnosis of these cysts in all the patients but histopathology proved to be gold standard for its confirmation. All these patients were managed by surgical excision. The postoperative and follow up period in all the cases was uneventful.

Conclusion: It is important to be aware and make a definitive diagnosis of this rare congenital anomaly as they can present in various clinical forms and can cause significant morbidity and even mortality if left untreated by causing life threatening complications.

Keywords: Congenital anomaly; Histopathology; Surgical excision.

[Table/Fig-2]:

Pyloroduodenal duplication cyst viewed on CT scan.

[Table/Fig-3]:

Intraoperative pyloroduodenal duplication cyst.

[Table/Fig-4]:

Histology of the pyloroduodenal duplication cyst wall showing mucosa, submucosa and smooth muscle. (H&E,10x)

[Table/Fig-5]:

Gross specimen of cystic mass adherent to sigmoid colon which is filled with mucoid material.

[Table/Fig-6]:

Colonic duplication cyst wall on histopathology. (H&E,10x).

[Table/Fig-7]:

Gross specimen of excised ileal duplication cyst.

[Table/Fig-8]:

Histology of the ileal duplication cyst showing two mucosal layers sharing a submucosal layer with muscular layer. (H&E,10x)