Duodenal atresia
Key points
1 in 10,000 births
Failure of epithelial apoptosis recanalisation in 2nd month, not vascular accidents later (as in other atresias)
Antenatal features
65% Polyhydramnios
Double bubble after 20 weeks
Signs of trisomy 21
2020 BAPS CASS - DA + Trisomy 21 - outcomes of DA repair are same as those without T21
Gray and Skandalakis classification
Type 1: Web/Windsock (92%)
Type 2: Fibrous Cord 1%
Type 3: V shaped mesenteric defect (7%)
80% atresia at or beyond ampulla
Annual pancreas - failure of rotation of ventral bud
Almost always has an intrinsic obstruction as well
Associations
~50% have associated abnormalities
Cardiac - endocardial cushion defects in approx 30%
Trisomy 21 in 30%
Malrotation in 25%
Differentials for double bubble with distal air
1. Malrotation (Ladds bands)/Volvulus
2. Wind sock
3. Annular pancreas
4. Pre-duodenal portal vein (associated with left atrial isomerism)
5. Biliary tree abnormalities
Ligament of Treitz: Double fold of peritoneum suspending duodenum - forms the DJ flexure
Duodenal atresia can form a pseudomalrotation by displacing the colon left
True malrotations will have absence of ligament of Treitz
Standard scenario
AXR showing double bubble
Concern is duodenal obstruction
Check for distal gas which may represent volvulus
State differentials:
Atresia/Windsock
Annular pancreas
Pre-duodenal portal vein
Ensure child is resuscitated by NICU team
Antenatal history - polyhydramnios, chromosomal analysis
Full examination - OA (pass NG), ARM, dysmorphic features, Echo
Consent for correction of atresia and malrotation if present
Urgent laparotomy (or UGI contrast) if distal gas
Planned laparotomy if not
RUQ transverse incision
Inspect for malrotation, duodenal and other atresias
Web/windsock suspected - pass OG tube - make first longitudinal incision below where wall is tugging
If large windsock - can resect part - but avoid medial wall due to ampulla
Make transverse incision above
Always flush distal bowel for atresias
Then diamond anastomosis
Some patients e.g. premature or long atresia may need duodenojejunostomy
Use of TAT:
2022 JPS Southampton retrospective study - TAT tubes
TAT: Feeds start 2 days, full 6 days, No TAT 3 days, full 10 days
Less need for central lines and less line complications in TAT group
1 perforation secondary to TAT
2024 Meta-analysis - probable benefit to TAT tube - reduced time to full feeds - but low quality evidence
Post op
NICU to place longline for PN
Can start feeds even if aspirates still bilious
Home when established on feeds
Early follow up to assess feeding
Regular follow up in childhood as risk of dilated dysmotile duodenum requiring tapering
References
Elizabeth Fialkowski et al. "Congenital Duodenal Obstruction." Pediatric Surgery NaT, American Pediatric Surgical Association, 2020. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829038/all/Congenital_Duodenal_Obstruction.
Bethell GS, Long AM, Knight M, Hall NJ; BAPS-CASS. The impact of trisomy 21 on epidemiology, management, and outcomes of congenital duodenal obstruction: a population-based study. Pediatr Surg Int. 2020 Apr;36(4):477-483. doi: 10.1007/s00383-020-04628-w. Epub 2020 Feb 29. PMID: 32114651; PMCID: PMC7069923.
Cresner R, Neville JJ, Drewett M, Hall NJ, Darwish AA. Use of trans-anastomotic tubes in congenital duodenal obstruction. J Pediatr Surg. 2022 Sep;57(9):45-48. doi: 10.1016/j.jpedsurg.2022.01.049. Epub 2022 Feb 6. PMID: 35307195.
Bethell GS, Neville JJ, Johnson MJ, Turnbull J, Hall NJ. Congenital duodenal obstruction repair with and without transanastomotic tube feeding: a systematic review and meta-analysis. Arch Dis Child Fetal Neonatal Ed. 2024 Feb 19;109(2):182-188. doi: 10.1136/archdischild-2023-325988. PMID: 37923385; PMCID: PMC10894825.