DUODENAL ATRESIA BY SHREOSI GHOSH INTERN MALDA MEDICAL COLLEGE AND HOSPITAL
INTRODUCTION DEFFINITION : Duodenal atresia is the congenital absence or complete closure of a portion of the lumen of the duodenum due to the defective fusion of foregut and midgut with failure of the recanalisation . EPIDEMIOLOGY: 1 Per 5000 to 10000 live births. Affecting boys more commonly than girls.
ETIOLOGY Intrinsic lesion : Caused by failure of recanalization of the fetal duodenum Extrinsic lesion : Defects in the development of the neighbouring structures Teratogens : Chlordiazepoxide Thalidomide Flurouracil
ASSOCIATIONS Prematurity (45%) Growth retardation (33%) Annular pancreas Down’s syndrome (30%) – Trisomy 21 Miller- Dieker syndrome More than 50% patients have associated congenital anomalies – Congenital heart disease (30%) Incomplete rotation of gut (20%) Anorectal malformations (10%)
CLASSIFICATION ANATOMICAL CLASSIFICATION : TYPE 1 (92%): Complete atresia TYPE 2 (1%): Fibrous cord separating two ends TYPE 3 (7%) :Incomplete or partial obstruction Stenosis Mucosal web with intact muscular wall- WINDSOCK DEFFORMITY
PATHOPHYSIOLOGY Failure of canalization of the duodenal lumen Improper formation of vacuoles Atresia or web may develop Accumulation of gases and secretions above the blockage
Pathophysiology contd... In intra luminal pressure , venous pressure Circulatory stasis or edema Gangrene, Peritonitis
CLINICAL FEATURES Bilious or nonbilious vomiting immediately after birth Jaundice Features of gastric outlet obstruction Dehydration and electrolytes changes Abdominal distension and tenderness Absence of flatus No passage of stool Respiratory distress Shock
DIAGNOSIS History : Maternal polyhydramnios Investigations: MATERNAL - Prenatal ultrasonography : Detects duodenal atresia between 7th and 8th month of gestation. Polyhydramnios and associated anomalies can also be detected.
2 . CHILD Plain X ray abdomen : DOUBLE BUBBLE SIGN with absence of air in the distal part
INVESTIGATION CONTD… Upper GI contrast study : Partial obstruction with the presence of air in the distal loop Other associated anomalies – Malrotation,Mid gut volvulus etc
MANAGEMENT GENERAL MANAGEMENT : IV fluids Oro or nasogastric aspiration Stomach wash NPM Antibiotics,antiemetics,vitamin K supplement,PPI
SURGICAL MANAGEMENT : PREOPERATIVE CARE : Appropriate resuscitation Correction of fluid imbalance and electrolyte abnormalities Monitoring of the complete metabolic profile Gastric decompression Perenteral nutrition via central catheter line Two dimensional echocardiographic monitoring
TREATMENT PROPER : Surgery can be done by : LAPAROTOMY : Supraumbilical transverse incision is given to the right upper quadrant of abdomen LAPAROSCOPY : Neonatal laparoscopic instruments (3mm ) and Trocars are used
TREATMENT CONTD… Different Methods are : DUODENODUODENOSTOMY : Surgical bypass for the duodenal obstruction is done by Side to side Duodenoduodenostomy.Associated malrotation should also be corrected ( LADD’S OPERATION ). Megaduodenum or anstomotic dysfunction can occur.
Kimura’s diamond shaped anstomosis : Done between transversly opened proximal pouch and longitudinally opened distal pouch.Presence of bile and concomittent distal atresia should be confirmed by saline irrigation. Less chances of the problems of anastomosis .
Treatment contd.. Tapering Duodenoplasty : Done with staples or sutures to reduce the Duodenal caliber when the proximal Duodenum is markedly dialated .
HEINEKE-MICULICZ DUODENOPLASTY : Partial web resection is done transduodenally in Duodenal mucosal web.
Treatment contd DUODENOJEJUNOSTOMY : Previously preferred technique. May cause blind loop problem. GASTROJEJUNOSTOMY: Done previously. High incidence of marginal ulceration and bleeding.
UPPER GI CONTRAST STUDY AFTER LAPAROSCOPY
POST OPERATIVE CARE Continuation of Total parenteral nutrition (TPN) Monitoring of Nasogastric tube output Feeding via transanastomotic nasojejunal or gastrostomy tube after diminution of nasogastric tube output Small feeding with volume and concentration advanced as tolerated Discharge within one to three weeks
COMPLICATIONS INTRAOPERATIVE : Windsock defformity (Floppy web) : Incorrect identification of site of obstruction Bypass anstomosis entirely distal to true web attachment Multiple obstructions POST OPERATIVE : Megaduodenum (30%) Prolonged feeding intolerance Poor peristalsis Residual obstruction Anastomotic stenosis
DIFFERENTIAL DIAGNOSIS Late appearing pyloric stenosis Incomplete diaphragm Other forms of intestinal atresia Duodenal duplication Midgut volvulus