Duodenal atresia is a common cause of intestinal obstruction in neonates, occurring in about 1 in 5,000-10,000 live births. It results from a failure of recanalization of the fetal duodenum, leading to a complete obstruction. Clinically, it presents with bilious vomiting within the first few hours of life. Diagnosis involves finding the classic "double bubble" sign on abdominal x-ray. Surgical treatment involves reconstructing bowel continuity, usually via a diamond-shaped duodenoduodenostomy. With proper management, over 95% of patients will recover successfully from duodenal atresia.

1. Duodenal Atresia
Dr Shambhavi Sharma

2. Duodenal atresia - Introduction
• Duodenal atresia and stenosis – common cause for intestinal
obstruction
• 1/5000-10000 live births
• Boys commonly affected than girls
• associated with other congenital anomalies (45-65%)
• Down’s syndrome (50%)
• Gut malrotatation (30%)
• Congenital heart diseases (25-65%)
• Anorectal and genitourinary anomalies

3. Embryology
• Fourth week of development
• Endoderm gives rise to the gut tube
• Sixth week
• Gut epithelium proliferates rapidly, resulting in obliteration of the intestinal
lumen
• Over the next several weeks
• Intestine then gradually recanalize
• Errors in recanalization primary cause of duodenal atresia and stenosis.

4. Duodenal atresia - Etiology
• Intrinsic lesion
• Failure of recanalization of fetal duodenum resulting in complete obstruction
• Extrinsic lesion
• Annular pancreas
• Preduodenal portal vein
• Malrotation
• Ladd’s band

5. Classification(Gray and Skandalakis)
• Type I
• More than 90%
• Obstructing septum (web) formed from
mucosa and submucosa with no defect
in the muscularis.
• Mesentery intact
• Variant: “windsock deformity”
• Membrane is thin and elongated
• Base of the membrane usually lies in the
second portion of the duodenum
• balloons out distally distending 3rd and
4th portion
Windsock deformity

6. • Type II
• Dilated proximal and collapsed distal segments connected by fibrous cord
• Type III
• Obvious gap separating proximal and distal segment

7. Duodenal atresia - pathology
• Obstruction level – pre-ampullary or post-ampullary
• More than 85% distal to ampulla
• Stomach and proximal bowel dilated
• Pylorus hypertrophic and distended
• Distal bowel collapsed
• Windsock deformity : distal bowel dilated

8. Clinical presentation
Depends on
• Whether obstruction is complete or incomplete
• Location of ampulla in relation to obstruction
Classic presentation
• Bilious vomiting within first few hours of life in otherwise healthy neonates
• In less than 15 % non bilious vomiting seen (preampullary obstruction)
• Partial obstruction: symptoms may present later in the 1st year of life with the
introduction of solid foods
In rare cases,
• diagnosis may even be delayed into later childhood or adulthood usually in
association with GERD, esophagitis, duodenal ulcer

9. Physical Examination :
• Upper abdominal distention that resolves with placement of a NG
tube
• With complete obstruction, placement of a NG tube may result in a
scaphoid abdomen due to the absence of distal gas GI tract
• Aspiration via NG tube of >20 mL of gastric contents suggests
intestinal obstruction

10. Duodenal atresia – diagnosis
• Antenatal diagnosis
• Detected between 7 and 8 months
• Poly-hydraminos
• Two fluid filled structures –
consistent with double bubble in
up to 44%

11. Duodenal atresia – diagnosis
• Postnatal diagnosis
• Double bubble sign with no distal bowel gas
• Proximal left sided bubble – air and fluid filled stomach
• Second bubble to right – dilated proximal duodenum
• In doubtful cases
• 40/60ml of air instilled in stomach – reproduce sign

12. Duodenal atresia – diagnosis
Role of Upper GI contrast study
• Differentiating intrinsic duodenal
obstruction from midgut volvulus
Intrinsinc duodenal obstruction
 Smooth rounded end seen at level of obstruction
in second portion of duodenum
Midgut volvulus
 Distal beaking effect in third part of duodenum
Web Distal part of duodenum

13. Duodenal atresia –preoperative preparation
• Work up
• Complete blood counts
• Electrolytes
• Abdominal USG
extrinsic causes of compression: anomalies of the pancreas or biliary tree ,preduodenal
portal vein or duodenal duplication.
• Echocardiography to rule out cardiac anomalies

14. • Emergency intervention only in cases of suspected midgut volvulus
• Gastric decompression using NG tube
• Maintenance intravenous fluids , with replacement fluid added to account for
gastric losses
• Prophylactic preoperative antibiotic (ampicillin and gentamicin in neonates)
• 1 mg of intramuscular vitamin K
• In premature infants with extremely low birth weight (<1,000 g) delayed
operative intervention for several weeks with nasogastric decompression and
parenteral nutrition to allow for growth of the infant

15. Surgical techniques
• Diamond shaped duodenoduodenostomy (preferred)
• Side-to-side duodenoduodenostomy
• Duodenojejunostomy
• Web resection with duodenoplasty

16. Side-to-side duodenoduodenostomy:
associated with a high incidence of anastomotic dysfunction and
prolonged obstruction
Duodenojejunostomy: associated with Blind-loop syndrome
Gastrojejunostomy should not be performed as it is associated with a
high incidence of marginal ulceration and bleeding

17. Diamond shaped Duodenoduodenostomy
•Approach :
 Open
 Laparoscopic
• Steps
Open approach
 Incicion : RUQ supraumbilical transverse incision or an umbilical crease incision
 After mobilizing the ascending and transverse colon to the left, the duodenal
obstruction is readily exposed

18. • Malrotation should be evaluated (can occur in 30% of
cases )
• Sufficient length of duodenum distal to the atresia is
mobilized to allow for a tension-free anastomosis
• transverse duodenotomy is made in the anterior wall
of the distal portion of the dilated proximal
duodenum
• Similar length duodenotomy made in a vertical
orientation on the antimesenteric border of the
distal duodenum

19. • Small red rubber catheter placed distally into the jejunum to
assess for a rare second duodenal atresia.
• Saline injected to distend the distal bowel to rule out any
jejunoileal atresia.
• Anastomosis approximating the end of each incision to the
appropriate mid-portion of the other incision
• Single-layered technique with silk or absorbable suture
• Use of a transanastomotic feeding tube is left the surgeon’s
preference

20. • When the proximal duodenum is markedly dilated, tapering
duodenoplasty with staples or sutures should be considered to narrow
the duodenal caliber to lessen dysmotility.

21. Windsock deformity :
• identification of the constricting
ring from which the web originates
by passage of a nasogastric tube
• longitudinal dodenotomy on the
antimesenteric wall is made, which
centers on the constricting ring
• Web resected laterally, anteriorly,
and posteriorly,
• Avoid and preserve the medial
third of the web
• Minimizes the risk of injury to the
ampulla
• Hemostatic, running absorbable
suture is placed along the cut edge
of the web,
• medial portion is left to hang
distally into the lumen
• Doudenoplasty is then completed
by closing the duodenotomy
transversely in a single layer with
silk or absorbable suture to further
open the constricting ring

22. Laparoscopic approach
• first described by Rothenberg in
2002
• patient supine
• abdomen insufflated through
the umbilicus
• Two other ports:
• Baby’s RLQ
• Right mid-epigastric region
cranial

23. Duodenal atresia – surgery
Duodenojejunostomy
• Only done if duodenoduodenostomy not possible
• existence of large gap between the proximal and distal
duodenal segments
• in patients with normal rotation and a normal ligament of
Treitz
• infants less than 1,000 g
• Equivalent outcomes but more post operative
complications

24. Post operative care
• Monitor NG effluent
• Feeding
 NPO till return of normal bowel function and no evidence of anastomotic leak
 May require TPN depending on neonate status
 Started once NG effluent diminished and becomes clear
 Started with small volume feed and gradually increase if tolerated

25. Duodenal atresia - prognosis
• Outcomes
• More than 95% will recover successfully
• Mortality – associated with other associated anomalies
• Long-term complications
• Delayed gastric emptying
• Severe gastroesophageal reflux and bleeding peptic ulcer
• Megaduodenum
• Duodenogastric reflux
• Intestinal obstruction related to adhesions

26. References
• Ashcraft’s Pediatric Surgery 7th edition
• Fischer’s mastery of surgery 7th edition
• Grosfeld Pediatric Surgery 6th edition

27. Take Home Messages
Duodenal atresia – common causes for intestinal obstruction in
neonates
• Presents with classical features of intestinal obstruction
• Diagnosis is often clinical with radiological investigations to support
for diagnosis
• Operative treatment has good outcome

28. THANK YOU