Duodenal atresia is a congenital disease where the duodenum fails to form properly, occurring in 1 in 5,000-10,000 births. It is classified into three types depending on the severity of blockage of the duodenum. Type I involves a duodenal diaphragm, Type II is a complete blockage, and Type III is a complete separation of the duodenal ends. Ultrasound often reveals the "double bubble" sign of stomach dilation due to duodenal blockage. Treatment involves surgery to reconnect the duodenum. Prognosis is usually good though rare late complications can include megaduodenum or pancreatitis.

1. Duodenal atresia
20150410

2. Introduction
● congenital disease
● 1/5000-10000
● 20-40% : Down
● (but 8% in Down)
● 30-52% isolated anomaly
● 20-25% have cardiac anomalies
autosomal recessive

3. Cause
● annular pancreas
● duodenal growth failure failure of recanalization
● biliopancreatic tract or choledochal cysts

4. Recanalization of duodenum

5. Classification
Type I (duodenal diaphragm)
Type II
(complete a duodenal atresia)
Type III
(complete separation of the
two ends)
annular pancreas
92%
1%
7%

6. Ultrasound findings
● Polyhydramnios
● "double bubble"
Diagnosis 80-90%

7. Diagnosis
● Polyhydramnios
● abdominal distension
● Bilious vomiting
● "double bubble" sign

8. Diagnosis
ampuIa of vater
90% Bilious vomiting

9. Treatment
● nasogastric tube
● surgery (duodenoduodenostomy)
● 5% percent morbidity and mortality rate
● Prognosis is usually good
● rare late complications
megaduodenum
blind loop syndrome
duodenogastric reflux
esophagitis
pancreatitis
cholecystitis and cholelithiasis

10. Differential diagnoses
● late appearing pyloric stenosis
● incomplete diaphragm
● other forms of intestinal atresia
● common mesenteric intestinal volvulus
● duodenal duplication

11. Differential diagnoses
Small Bowel Obstruction
duodenal duplication
incomplete diaphragm

12. Syndrome
● Miller-Dieker Syndrome
● Trisomy 21
Teratogens
● Chlordiazepoxide
● Fluorouracil
● Thalidomide

13. Syndrome
● Miller-Dieker Syndrome
● Trisomy 21
Teratogens
● Chlordiazepoxide
● Fluorouracil
● Thalidomide
利眠寧 治療精神障礙藥物
Cardiac defects
Duodenal atresia
Microcephaly
服樂癌注射劑
Esophageal and Duodenal abnormalities
Radial aplasia
賽得膠囊(沙利竇邁) 治療痲瘋性結節紅斑
Cardiac defects Microtia
Duodenal atresia Spine malformations
Limb reduction abnormalities

14. Refference
http://www.orpha.net/consor/cgi-bin/OC_Exp.
php?Expert=1203
http://en.wikipedia.org/wiki/Duodenal_atresia
http://yibian.hopto.org/diag/ill/?illno=811