Enteric duplication in children: clinical presentation and outcome

Abstract

RESULTSOf the nine patients who presented with ED, nearly two-thirds were infants (age range 3 months-5 years). Seven (77.8%) were male and two (22.2%) were female. The presenting symptoms were varied, with the most common being abdominal mass and bleeding per rectum. Descriptions of the patients' age and gender, clinical features, main investigations, types and sites of cysts, surgical procedures performed and complications observed are RESULTS A total of nine patients were managed during the study period. The patients' ages ranged from three months to five years. Four out of nine EDs were rectal duplications. Three EDs were of the cystic type, five were of the tubular type and one was a complex mixed anomaly. Patients presented with varied symptoms, with the two most common being the presence of an abdominal mass and bleeding per rectum. Diagnosis was mainly achieved based on magnetic resonance imaging and computed tomography, although Meckel's scan provided accurate diagnosis in three of the nine patients. All the cysts were resected without any major complications, and patients were event-free during the five-year follow-up. Table I. A total of four out of nine patients had rectal duplications, and a perineal mass was noted in one (11.1%) patient. One patient had infected wound discharge following drainage of a perineal abscess, while penoscrotal hypospadias was present in another. (2) Although ED may present at any age, a majority are also why EDs may pose as a diagnostic challenge. In the present study, none of the patients were diagnosed antenatally. CONCLUSIONSome patients, however, had unusual symptoms -one patient presented with a nonhealing wound in the perineum that had previously been incised and drained due to suspicions of it being a perineal abscess, although a closer look at the surface would have revealed the presence of mucosa in the deeper part of the wound. Only one patient in our series had associated penoscrotal hypospadias. Such anomalies have been reported in up to 50% of patients, with vertebral defects being the most common.(1)EDs may be cystic, tubular or mixed (cystic and/or tubular).Although EDs usually communicate with adjacent parts of the gut, instances of completely isolated duplication cysts have also been reported. (6) We found various types of duplication cysts in our patients, including the cystic, tubular and complex mixed varieties. According to the literature, the ileum is the most common site of ED, whereas gastric and colonic duplications are rare, (4) and thoracoabdominal duplications are the most challenging. (7)(8)(9) In our series, the ileum was the most commonly involved site (n = 5), presenting as either an isolated lesion or a combination. However, contrary to the literature, which does not describe a high incidence of rectal involvement, (7,9,10) our series saw four cases (out of a total of nine) of rectal involvement. Also, there was a higher incidence of tubular ED (n = 5) in our study. Interestingly, one patient had a rare, complex duplication cyst that s...

Cysts of Gastrointestinal Origin in Children: Varied Presentation

“…Abdominal examination may be unremarkable, but a mobile mass may be palpable in 50% of cases. Associated malformations are reported in 50% of patients, the most common being vertebral defects [151718]. …”

“…Though there is no consensus on the exact embryological origin of enteric duplication cysts, the most widely accepted theory is the split notochord theory [17]. Other theories suggested are incomplete twinning, phylogenetic reversal, persistent embryonic diverticula, entrapment of endodermal cells or persistent epithelial buds within the body wall and dysvacuolation [17].…”

“…Other theories suggested are incomplete twinning, phylogenetic reversal, persistent embryonic diverticula, entrapment of endodermal cells or persistent epithelial buds within the body wall and dysvacuolation [17]. As per Ladd, the term ‘enteric duplication cyst’ will be applied if a congenital lesion has (1) coat of smooth muscle, (2) GI type epithelial lining, and (3) intimate anatomical location with some part of gastro-intestinal tract (GIT) [13].…”

Congenital lymphangiomatosis and an enteric duplication cyst in a young dog

“…For the diagnosis of this type of lesions, ultrasonography is superior to CT in the demonstration of the internal nature of the cysts (Vargas-Serrano et al 1995). Therefore, ultrasound is considered as an initial diagnostic modality for enteric duplication cyst (Rasool et al 2013), although CT is still useful to define the characteristics, location, size and extent of lesions. Also, CT angiography using contrast material provides vascular anatomic information, and it facilitates investigation of the origin of the lesion.…”

Infected enteric duplication cyst

“…They are characterised by the following three features: a well-developed coat of smooth muscle, lined with a mucous membrane and found along (and often intimately attached to some portion of) the gastrointestinal tract 1. They are most frequently found in the small intestine (44%) and colon (15%), but can also be found in gastric (7%), duodenal (5%), rectal (5%), thoracic and thoracoabdominal (4%), cervical (rare) or pyloric (extremely rare) localisations 2. They arise during early embryonic development, but true aetiology remains unknown.…”