Duplication cysts
Key points
Definition
Congenital cystic or tubular lesions with a smooth muscle coat continuous with native bowel
Contain mucosa resembling part of the GI tract, occasionally heterotopic
Share a common wall and blood supply with adjacent bowel
Can occur anywhere along the alimentary canal
Pathophysiology theories
Split notochord (Bentley and Smith) - 4th week, the notochord and endoderm separate. During separation, an adhesion or neuroenteric band can form creating traction diverticulae that can induce the formation of duplications.
Aberrant recanalisation theory – failure of vacuolisation process during solid phase of gut development
Abortive twinning - split in the primitive streak – incomplete duplication of embryonic gut tube
Intrauterine vascular accident – local ischaemia or malrotation disrupting recanalisation
Epidemiology
Incidence around 1 in 4,500 live births
Slight male predominance
Two-thirds present before age 2 years
May be associated with vertebral, genitourinary or cardiac anomalies, particularly in neurenteric variants
Anatomy
Can occur from mouth to anus
Most common in ileum (30–40%), followed by oesophagus, stomach, duodenum, colon, rectum
Usually arise on the mesenteric border and share a common blood supply and wall of the gut, except in rare retroperitoneal cysts
Occasionally extramural or communicating with the gut lumen
Posterior mediastinal cysts will likely be foregut duplication cysts
Foregut cysts are associated with spinal and vertebral abnormalities - should always perform full neurological examination
Bronchogenic cysts - not usually connected by a tract to the trachea
Gastric duplications - usually on greater curvature or posterior wall
Lumen can communicate with pancreas, biliary ducts
Hindgut cysts may be long, and effectively run the entire length of the colon - may extend to perineum and have separate anal/vaginal opening
Classification
Cystic type – 80%, non-communicating, spherical or ovoid
Tubular type – 20%, may communicate with bowel lumen proximally or distally
Foregut cyst classification
Enteric (Lined by enteric mucosa - gastric or oesophageal)
Bronchogenic (Unilocular masses, mediastinal/paratracheal, lined by bronchial mucosa)
Neurenteric
Histopathology
Wall composed of mucosa, submucosa, muscularis and serosa identical to normal bowel
Ectopic mucosa (gastric or pancreatic) in up to one-third – may cause ulceration, bleeding or perforation
Epithelial lining corresponds to adjacent bowel, but foregut lesions may show respiratory or gastric mucosa
Clinical Features (BIOPIM)
Bleeding – ulceration due to ectopic gastric mucosa
Infection – secondary inflammation, abscess or perforation
Obstruction – intussusception, volvulus or extrinsic compression
Perforation – rare, may cause peritonitis
Incidental finding – increasingly on antenatal or abdominal imaging
Malignancy – rare, mainly colonic or rectal duplications
Investigation
'Double wall' sign on USS/CT is classical
Imaging to look for other cysts
Can do Technetium 99 scan for ectopic gastric mucosa
MRI for hindgut cysts/rectal duplications e.g., Currarino triad
Contrast via separate perineal openings for hindgut duplications
Endoscopy – useful in oesophageal or gastric duplications
May show compression or mucosal ulceration
Operative approaches
Operate around age 1
Attempt complete excision - bowel resection and anastomosis if necessary
If complete excision not possible e.g., due to long lesion - enterotomy and mucosal stripping (Wrenn procedure)
Site-Specific Notes
Oesophageal duplications
Often in lower third, posterior mediastinum
Present with dysphagia, respiratory distress or infection
Thoracoscopic or open excision via muscle-splitting approach
Care to preserve vagus and avoid oesophageal perforation
Neuroenteric cysts
Liaise with spinal surgeon to remove intraspinal component first or apply a clip at the vertebral foramen. Concern is for cord compression and meningitis if the extraspinal component is excised first
Thoracoabdominal duplication
Can remove in single or staged procedure
Gastric and duodenal duplications
May cause outlet obstruction, bleeding or pancreatitis
Surgery requires identification of biliary and pancreatic ducts
Cystogastrostomy or partial excision with mucosal stripping may be required
Ileal and jejunal duplications
Most common site
Often present with obstruction, intussusception or bleeding
Resection of involved segment with end-to-end anastomosis
Colonic and rectal duplications
May present late with constipation, infection or fistula
Malignant transformation reported
Excision + anastomosis if short
If long e.g., length of whole colon - enterotomy and stapled intermittent fenestrations to make communications in lumen + partial resections
Posterior sagittal or transanal approach for rectal lesions
Mucosal stripping used to preserve continence
If infected - common wall should be incised and allowed to drain into rectum
May need covering stoma if extensive
Prognosis
Excellent with complete excision
Recurrence rare
Outcome depends on location, mucosal type and associated anomalies
Worse prognosis if associated with spinal or thoracoabdominal defects
Standard scenario
Antenatal detection of an intraabdominal cyst
Questions to ask
Solid/cystic/mixed
Other abnormalities e.g., bowel dilatation
Liquor volume
Pulmonary hypoplasia
Hydrops
Differentials
Duplication
Lymphatic malformation
Choledochal
Mesenteric/Omental
Renal
Cystic neuroblastoma
Urachal cyst
Female - any of above + ovarian cyst, hydrocolpos
Investigations
Foetal MRI for more information
USS every 2 weeks to check for size changes
Check for maternal hypertension
Post natal management
Delivery in surgical centre with Level 3 neonatal unit
Resuscitation
Look for respiratory compromise if hydrops/pulmonary hypoplasia antenatal
USS for diagnosis
MRI (or CT) if unclear
Manage depending on diagnosis
Page edited by Prof. Ashok Daya Ram MBBS, FRCS, FRCPS, FEBPS, FRCS (Paed Surgery), Consultant Paediatric and Neonatal Surgeon, Norfolk and Norwich University Hospital, Norwich, UK. November 2025
Page edited by Mrs Charnjit Seehra BSc November 2024
References
Bradley Segura, David Schindel. "Gastrointestinal Duplications." Pediatric Surgery NaT, American Pediatric Surgical Association, 2020. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829466/all/Gastrointestinal_Duplications.
Ladd WE, Gross RE. Surgical management of alimentary tract duplications. Surgery. 1937.
Macpherson RI. Gastrointestinal tract duplications. Radiographics. 1993.
Bentley JF, Smith JR. Developmental origin of enteric duplications. Surg Gynecol Obstet. 1960.
Favara BE et al. Enteric duplication cysts: histogenesis review. 1971.
Strouse PJ et al. Pediatric gastrointestinal duplications: patterns. Radiology. 2002.
Holcomb GW, Murphy JP. Ashcraft’s Pediatric Surgery. 7th ed.
Wrenn EL. Mucosal stripping technique. Ann Surg. 1955.
Soper RT. Septostomy for colonic duplications. J Pediatr Surg. 1964.
Sinha CK et al. Duplication cyst epidemiology update. Pediatr Surg Int. 2011.