|Year : 2015 | Volume
| Issue : 4 | Page : 174-178
Enteric duplication in children: Experience from a tertiary center in South India
Susan Jehangir1, Pradeep Joseph Ninan1, Tarun John Jacob1, Anu Eapen2, John Mathai1, Reju Joseph Thomas1, Sampath Karl1
1 Department of Pediatric Surgery, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of Radiodiagnosis, Christian Medical College, Vellore, Tamil Nadu, India
|Date of Web Publication||2-Sep-2015|
Dr. Susan Jehangir
Department of Pediatric Surgery, Christian Medical College, Vellore - 632 002, Tamil Nadu
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Background: Enteric duplications (EDs) are rare aberrations of the embryonic gut. This study was undertaken to define the clinical characteristics and management challenges of this unusual entity in the Indian population. Materials and Methods: Hospital records of 35 children with 38 ED operated between 2003 and 2014 were analyzed and followed up. Results: The median age at presentation was 285 days (range 1-day to 16 years) with male preponderance (71%). Small bowel duplications were the most common (44%), and thoracoabdominal duplications were seen in 8% children compared to 2% in the literature. The median duration of symptoms was 18 days (interquartile range [IQR] 3-210 days). Associated anomalies were seen in 49% children with vertebral and spinal anomalies being the most common. Ultrasonogram (US) was done in 83% children and had a sensitivity of 55%. In the presence of a gastrointestinal bleed, Technetium 99m pertechnetate scintigraphy scan had a positive predictive value of 80%. Thirty-five lesions were completely removed. Mucosectomy was done in two children, and one total colonic duplication was left in situ after providing adequate internal drainage. There was no postoperative mortality. The follow-up was possible in 66% children. Conclusions: EDs are uncommon and have varied, nonspecific symptoms. Thoracoabdominal duplications are more common in the Indian population. The US is a good screening tool but requires a high index of suspicion where complete excision is not possible; the provision of adequate internal drainage is an acceptable alternative. The long-term prognosis of children with ED depends on the extent of physiological disturbance due to associated anomalies.
Keywords: Alimentary tract duplication, ectopic gastric mucosa, enteric duplication
|How to cite this article:|
Jehangir S, Ninan PJ, Jacob TJ, Eapen A, Mathai J, Thomas RJ, Karl S. Enteric duplication in children: Experience from a tertiary center in South India. J Indian Assoc Pediatr Surg 2015;20:174-8
|How to cite this URL:|
Jehangir S, Ninan PJ, Jacob TJ, Eapen A, Mathai J, Thomas RJ, Karl S. Enteric duplication in children: Experience from a tertiary center in South India. J Indian Assoc Pediatr Surg [serial online] 2015 [cited 2019 Sep 20];20:174-8. Available from: http://www.jiaps.com/text.asp?2015/20/4/174/164246
| Introduction|| |
Enteric duplication (ED) is defined as a congenital lesion that has an anatomical relationship with some part of the bowel is lined by the intestinal mucosa and has a well-developed coat of smooth muscle.  The incidence of ED is approximately, one in 18,000 live births.  Since, the first report by Calder in 1733 several case reports have been published.  However, there is a paucity of studies from the Indian subcontinent. Herein, we present our experience with 38 EDs treated between January 2003 and July 2014. To the best of our knowledge, this is the largest case series of this region and highlights some peculiar presentations, diagnostic, and management challenges.
| Materials and Methods|| |
An observational cohort study was conducted of 35 children with 38 ED-treated in the Department of Pediatric Surgery between January 2003 and July 2014. Children with ED were identified by an electronic search of radiology database and operation records. The hospital outpatient records, operation notes, radiological investigations, pathology reports, and discharge summaries were reviewed. The admission notes of one child were not traceable in the records. Based on hospital data the families were contacted by phone or followed up in the outpatient department. Statistical analysis was performed using SPSS Software version 10 (Chicago, Illinois, USA).
| Results|| |
Thirty-eight EDs in 35 children were identified during the study period. Twenty-four (71%) were boys. The median age at presentation was 285 days (IQR 30-720) with 22 (58%) presenting in the 1 st year of life. The clinical findings classified by site of involvement are summarized in [Table 1].
|Table 1: Clinical findings of enteric duplication classified by site of involvement|
Click here to view
Associated anomalies were seen in 17 of 35 children [Table 2]. Vertebral and spinal anomalies were the most common, which were seen most commonly with esophageal duplications. Associated gastrointestinal (GI) anomalies were malrotation, annular pancreas, and low anorectal malformation. One child each had solitary kidney, nonfunctioning kidney, undescended testis, incontinent epispadias, chordee, and caudal duplication syndrome.
A definite diagnosis of ED was made by preoperative imaging in 25 (71%) of 35. Twenty-nine (83%) of 35children had a ultrasonogram (US). A definite diagnosis of duplication was made in 13 (34%) children, and in a further 4 (11%) children a suspicion of cystic pathology was raised on US. The sensitivity of US for diagnosing duplication cysts was 55%. Seven (20%) children required a magnetic resonance imaging (MRI) or computed tomography (CT) for diagnosis [Figure 1]. A mass was detected on a plain chest radiograph in all six children with mediastinal duplication. Technetium 99m pertechnetate scintigraphy (Tc 99m scan) was diagnostic in 5 of 12 children [Figure 2]. Of the five positive scans four had GI bleed, whereas of seven negative scans, only one child had GI bleed. The positive predictive value of Tc 99m scan in the presence of GI bleed was 80% (95% CI: 28.81 % to 96.70 %).
Thirty-five cysts were completely removed. The site specific details are enlisted in [Table 1]. Simultaneous bowel resection was required in 13 abdominal duplications. Two cysts could not be excised completely, and a partial excision with mucosectomy was performed. Of the two total colonic duplications, one had a mucosectomy, and distal anastomosis to the normal bowel for internal drainage and the other was left in situ after providing adequate internal drainage. The retroperitoneal cyst communicating with a nonfunctioning kidney had cyst excision with nephrectomy. Six operations (three thoracic and three abdominal) were performed by minimal access.
Fifteen cysts had ectopic mucosa. Of these 11 had ectopic gastric mucosa, two had ectopic pseudostratified squamous epithelium, and one had ectopic small intestinal mucosa. Xanthogranulomatous mucosa was found in a gastric duplication. No ectopic pancreatic mucosa was seen in our series.
Follow-up and long-term prognosis
There were no immediate surgical complications. The follow-up was possible in 66% children with a mean follow-up of 21 months (1-month to 8 years). There were no bowel related complications.
The children with associated anomalies have persistent physiological disturbance caused by the defects requiring long-term follow-up. One child each with sacral agenesis, lumbar lipomeningocele, and incontinent epispadias required bladder augmentation for neurogenic bladder in the former two and small capacity bladder in the latter. These children are doing well with clean intermittent catheterization. A baby with thoracoabdominal duplication and L5 hemivertebra continues to have persistent spastic myoclonus. However, upper limb involvement in this child cannot be explained by the anatomical lesion. A child with thoracoabdominal duplication and developmental delay has recurrent respiratory infections postoperatively and is being investigated for gastroesophageal reflux disease.
| Discussion|| |
EDs are rare lesions with complex multifactorial etiology. It can be classified according to morphology into cystic and tubular or according to the site of involvement of the GI tract. The small bowel is most commonly involved in ED.  Eight percent children in our series were found to have thoracoabdominal lesions as compared to 2-3% in the Western population [Figure 2].  Bhat et al. in their series from North India also found a similar trend with 20% thoracoabdominal lesions.  We speculate that the thoracoabdominal preponderance in the Indian population could be attributed to its unique genetic variation and this observation warrants careful genetic and functional validation.  Retroperitoneal duplications are extremely rare with seven case reports in English literature including one case from our institution.  Uniquely, this cyst was communicating with a nonfunctioning right kidney and lined with gastric mucosa.
Symptoms were usually related to age, site, size, presence of ectopic mucosa, and communication with the native bowel. Obstructive intestinal complaints were the most common presentation in our series followed by GI bleed. Some peculiar presentations were seen in our series. A neonate with a perforated ilea duplication presented with unexplained persistent metabolic acidosis. The evaluation of chronic constipation in a teenager revealed a caudal duplication syndrome. An infant with retroperitoneal duplication and communication with the renal pelvis presented with dysuria hematuria syndrome attributed to the presence of gastric mucosa in the cyst. Other unusual presentations include recurrent meningitis in a thoracoabdominal duplication and vaginal bleeding in an unusual anterior tubular rectal duplication, which was also communicating with the vagina and lined by gastric mucosa [Figure 3].
|Figure 3: Line diagram of an unusual anterior rectal duplication with gastric mucosal lining communicating with the vagina (arrow)|
Click here to view
All neonates in our series presenting with acute symptoms required emergent operations. However, two antenatally diagnosed duplications were excised electively after 3 months of age. Laje et al. in their series of 18 abdominal duplication cysts detected antenatally found that only three became symptomatic in the first 2 weeks of life and required emergency surgery.  EDs are likely to be detected antenatally. The children who remain asymptomatic may have a planned resection later.
There was a delay in diagnosis in the majority of patients. This could be attributed to the nonspecific mild symptoms and possibly delay in seeking medical help. Though US is a good screening modality, it had a sensitivity of 55% in the diagnosis of ED in our experience. We recommend further investigations in the form of MRI or CT in children with persistent, nonspecific abdominal complaints.
The incidence of malignancy in duplication is rare and to date no malignancy has been reported in childhood.  Small bowel duplications are the most common site for malignant change followed by colon and rectum.  The mechanism of malignant change has been postulated to be a partial or complete stasis of secretions from the mucosa within the duplication.  The absence of case reports of malignancy in total colonic duplications that have undergone a distal re-entry procedure in childhood is corroborative evidence to this mechanism.  A total colonic duplication in our series was left in situ after providing adequate distal internal drainage as a dissection of the entire lesion posed an attendant risk of ischemia to the native bowel.
Complete excision is the treatment of choice for all EDs. Excision can be done using minimally invasive techniques. In our series, six cysts (three thoracics, three abdominal) were excised laparoscopically. Laparoscopy can also be used to localize the cyst and facilitate excision by a small incision.  The principles of management include bowel preservation, mucosectomy, internal drainage and staged operations where indicated in order to achieve the best possible outcome for each individual child.
The long-term prognosis in ED depends on the associated anomalies and the extent of the physiological disturbance caused by them. Our study was a retrospective chart review, and we were able to follow-up 66% of children. This is a drawback in our study.
| Conclusions|| |
EDs are rare congenital lesions of the gut, which may present with nonspecific and nonlocalizing symptoms. The preponderance of thoracoabdominal duplications in Indian population warrants further study. The US is a good screening tool when the index of suspicion is high. Appropriate guidelines for investigation of children with nonspecific abdominal pain are required to avoid delays in diagnosis. Management must be tailored to achieve the best possible result for each child. The long-term prognosis of isolated ED is excellent. However, those with associated anomalies especially of the spine have variable long-term outcomes depending on the extent of the anomaly.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Azzie G, Beasley S. Diagnosis and treatment of foregut duplications. Semin Pediatr Surg 2003;12:46-54.
Cavar S, Bogovic M, Luetic T, Antabak A, Batinica S. Intestinal duplications - Experience in 6 cases. Eur Surg Res 2006;38:329-32.
Ramakrishna HK. Intestinal duplication. Indian J Surg 2008;70:270-3.
Holcomb GW 3 rd
, Gheissari A, O′Neill JA Jr, Shorter NA, Bishop HC. Surgical management of alimentary tract duplications. Ann Surg 1989;209:167-74.
Bhat NA, Agarwala S, Mitra DK, Bhatnagar V. Duplications of the alimentary tract in children. Trop Gastroenterol 2001;22:33-5.
Adeyemo A, Rotimi C. Genetic variants associated with complex human diseases show wide variation across multiple populations. Public Health Genomics 2010;13:72-9.
Bal HS, Kisku S, Sen S, Masih D. A retroperitoneal enteric duplication cyst communicating with the right upper ureter in an infant. BMJ Case Rep 2014;2014.
Laje P, Flake AW, Adzick NS. Prenatal diagnosis and postnatal resection of intraabdominal enteric duplications. J Pediatr Surg 2010;45:1554-8.
Hsu H, Gueng MK, Tseng YH, Wu CC, Liu PH, Chen CC. Adenocarcinoma arising from colonic duplication cyst with metastasis to omentum: A case report. J Clin Ultrasound 2011;39:41-3.
Blank G, Königsrainer A, Sipos B, Ladurner R. Adenocarcinoma arising in a cystic duplication of the small bowel: Case report and review of literature. World J Surg Oncol 2012;10:55.
Orr MM, Edwards AJ. Neoplastic change in duplications of the alimentary tract. Br J Surg 1975;62:269-74.
Lima M, Molinaro F, Ruggeri G, Gargano T, Randi B. Role of mini-invasive surgery in the treatment of enteric duplications in paediatric age: A survey of 15 years. Pediatr Med Chir 2012;34:217-22.
[Figure 1], [Figure 2], [Figure 3]
[Table 1], [Table 2]