Duodenal atresia20150410

Introduction

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

autosomal recessive

Cause● annular pancreas● duodenal growth failure failure of recanalization

● biliopancreatic tract or choledochal cysts

Recanalization of duodenum

ClassificationType I (duodenal diaphragm)

Type II (complete a duodenal atresia)

Type III(complete separation of the two ends)annular pancreas

92%

1%

7%

Ultrasound findings

● Polyhydramnios● "double bubble"

Diagnosis 80-90%

Diagnosis

● Polyhydramnios● abdominal distension● Bilious vomiting● "double bubble" sign

Diagnosis

ampuIa of vater

90% Bilious vomiting

Treatment

● nasogastric tube● surgery (duodenoduodenostomy)● 5% percent morbidity and mortality rate● Prognosis is usually good● rare late complicationsmegaduodenumblind loop syndromeduodenogastric refluxesophagitispancreatitischolecystitis and cholelithiasis

Differential diagnoses

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

Differential diagnoses

Small Bowel Obstructionduodenal duplication

incomplete diaphragm

Syndrome

● Miller-Dieker Syndrome● Trisomy 21

Teratogens● Chlordiazepoxide● Fluorouracil● Thalidomide

Syndrome

● Miller-Dieker Syndrome● Trisomy 21

Teratogens● Chlordiazepoxide● Fluorouracil● Thalidomide

利眠寧 治療精神障礙藥物Cardiac defectsDuodenal atresiaMicrocephaly

服樂癌注射劑Esophageal and Duodenal abnormalitiesRadial aplasia

賽得膠囊(沙利竇邁) 治療痲瘋性結節紅斑Cardiac defects MicrotiaDuodenal atresia Spine malformationsLimb reduction abnormalities

Refference

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