Presented at AW Sjahranie General Hospital, 28/08/2013, supervised by dr. Shanti SpBA and dr Slamet SpBA

1. DUODENAL ATRESIA
DIAGNOSTIC, THERAPY, PRE-OPERATIVE AND POST-OPERATIVE CARE
DR. ISA BASUKI
DEPARTMENT OF SURGERY, AWS GENERAL HOSPITAL

2. EPIDEMIOLOGY
• 1 PER 5000 TO 10,000 LIVE BIRTHS
• AFFECTING BOYS MORE COMMONLY THAN GIRLS
• MORE THAN 50% OF AFFECTED PATIENTS HAVE ASSOCIATED CONGENITAL
ANOMALIES
• TRISOMY 21  APPROXIMATELY 30% OF PATIENTS
• ISOLATED CARDIAC DEFECTS  30%
• OTHER GASTROINTESTINAL ANOMALIES  25%
• PREMATURE  45%
• GROWTH RETARDATION  33%

3. ETIOLOGY
• CONGENITAL DUODENAL OBSTRUCTION  INTRINSIC OR EXTRINSIC GASTROINTESTINAL LESION
• MOST COMMON CAUSE  ATRESIA
• INTRINSIC LESION  CAUSED BY A FAILURE OF RECANALIZATION OF THE FETAL DUODENUM
• EXTRINSIC FORM  DEFECTS IN THE DEVELOPMENT OF NEIGHBORING STRUCTURES
• ANNULAR PANCREAS IS AN UNCOMMON ETIOLOGY  THIS FORM OF OBSTRUCTION IS LIKELY DUE TO FAILURE OF
DUODENAL DEVELOPMENT RATHER THAN A TRUE CONSTRICTING LESION
• THE PRESENCE OF AN ANNULAR PANCREAS  VISIBLE INDICATOR FOR AN UNDERLYING STENOSIS OR ATRESIA
• OTHER:
• BILIARY ATRESIA,
• GALLBLADDER AGENESIS,
• STENOSIS OF THE COMMON BILE DUCT
• CHOLEDOCHAL CYST

5. CLASSIFICATION
• ANATOMICALLY  DUODENAL OBSTRUCTIONS ARE CLASSIFIED
AS:
• STENOSES 
• INCOMPLETE OBSTRUCTION DUE TO A FENESTRATED WEB OR DIAPHRAGM
• INVOLVE THE THIRD AND/OR FOURTH PART OF THE DUODENUM
• ATRESIA

6. CONT’D
• ATRESIAS, OR COMPLETE OBSTRUCTION ARE FURTHER CLASSIFIED INTO: (GRAY AND
SKANDALAKIS)
• TYPE I  92% OF CASES
• OBSTRUCTING SEPTUM (WEB) FORMED FROM MUCOSA AND SUBMUCOSA WITH NO DEFECT IN
SUBMUSCULARIS
• THE MESENTERY IS INTACT
• VARIANT  “WINDSOCK” DEFORMITY (THE MEMBRANE IS THIN AND ELONGATED)
• TYPE II  1% OF CASES
• A SHORT FIBROUS CORD CONNECTS THE TWO BLIND ENDS OF THE DUODENUM
• THE MESENTERY IS INTACT
• TYPE III  7% OF CASES
• THERE IS NO CONNECTIONS BETWEEN THE TWO BLIND ENDS OF THE DUODENUM
• V- SHAPED MESENTERY DEFECT

8. PATHOLOGY
• THE OBSTRUCTION CAN BE CLASSIFIED AS:
• PREAMPULLARY
• POSTAMPULLARY  APPROXIMATELY 85%
• THE PYLORUS IS USUALLY BOTH DISTENDED AND HYPERTROPHIC
• THE BOWEL DISTAL TO THE OBSTRUCTION IS COLLAPSED
• COMPLETE OBSTRUCTION OF THE DUODENUM  THE INCIDENCE OF
POLYHYDRAMNIOS 32% TO 81%.
• GROWTH RETARDATION IS ALSO COMMON

9. DIAGNOSIS
• HISTORY OF POLYHYDRAMNIOS
• PRENATAL ULTRASONOGRAPHY DETECT TWO FLUID-FILLED STRUCTURES CONSISTENT
WITH A DOUBLE BUBBLE IN UP TO 44% OF CASES
• MOST CASES OF DUODENAL ATRESIA ARE DETECTED AT BETWEEN 7 AND 8 MONTHS
OF GESTATION
• THE PRESENTATION OF THE NEONATE VARIES DEPENDING ON:
• OBSTRUCTION IS COMPLETE OR INCOMPLETE
• THE LOCATION OF THE AMPULLA OF VATER IN RELATION TO THE OBSTRUCTION
• CLASSIC PRESENTATION  BILIOUS EMESIS WITHIN THE FIRST HOURS OF LIFE IN AN
STABLE NEONATE (10% OF CASES THE EMESIS IS NONBILIOUS)

10. CONT’D
• ABDOMINAL DISTENTION MAY OR MAY NOT BE PRESENT, DUODENAL ATRESIA  SCAPHOID
• ASPIRATION VIA A NASOGASTRIC TUBE > 20 ML (N: < 5 ML)
• THE DIAGNOSTIC RADIOGRAPHIC PRESENTATION (UPRIGHT ABDOMINAL RADIOGRAPH) 
“DOUBLE BUBBLE” SIGN WITH NO DISTAL BOWEL GAS
• THE PROXIMAL LEFT-SIDED BUBBLE  AIR- AND FLUID-FILLED STOMACH
• THE SECOND BUBBLE TO THE RIGHT  THE DILATED PROXIMAL DUODENUM
• HOWEVER, THE PRESENCE OF DISTAL GAS DOES NOT EXCLUDE THE DIAGNOSIS OF ATRESIA
• LIMITED UPPER GASTROINTESTINAL CONTRAST STUDY  TO EXCLUDE MALROTATION AND
VOLVULUS

13. PRE-OPERATIVE CARE
• APPROPRIATE RESUSCITATION
• CORRECTION OF FLUID BALANCE AND ELECTROLYTE ABNORMALITIES
• GASTRIC DECOMPRESSION
• PERENTERAL NUTRITION VIA CENTRAL CATHETER LINE
• INVESTIGATIONS:
• COMPLETE METABOLIC PROFILE,
• COMPLETE BLOOD CELL COUNT,
• COAGULATION STUDIES,
• AN ABDOMINAL AND SPINAL ULTRASOUND EVALUATION,
• TWO-DIMENSIONAL ECHOCARDIOGRAPHY

14. THERAPY / OPERATION
• SURGICAL CORRECTION OF DUODENAL OBSTRUCTION IS NOT URGENT
• PRIOR TO THE MID 1970S, DUODENOJEJUNOSTOMY WAS THE PREFERRED TECHNIQUE
FOR CORRECTING DUODENAL ATRESIA OR STENOSIS
• VARIOUS TECHNIQUES:
• SIDE-TO-SIDE DUODENODUODENOSTOMY,
• DIAMOND-SHAPED DUODENODUODENOSTOMY,
• PARTIAL WEB RESECTION WITH HEINEKE-MIKULICZ–TYPE DUODENOPLASTY,
• TAPERING DUODENOPLASTY
• TODAY, THE PROCEDURE OF CHOICE IS EITHER LAPAROSCOPIC OR OPEN
DUODENODUODENOSTOMY

16. CON’T
• LONG SIDE-TO-SIDE DUODENODUODENOSTOMY, ALTHOUGH EFFECTIVE, IS
ASSOCIATED WITH A HIGH INCIDENCE OF ANASTOMOTIC DYSFUNCTION AND
PROLONGED OBSTRUCTION
• DUODENOJEJUNOSTOMY  BLIND-LOOP SYNDROME APPEARS TO BE MORE
COMMON
• GASTROJEJUNOSTOMY  HIGH INCIDENCE OF MARGINAL ULCERATION AND
BLEEDING
• FOR THE OPEN APPROACH  RIGHT UPPER QUADRANT SUPRAUMBILICAL TRANSVERSE
INCISION IS MADE
• AFTER MOBILIZING THE ASCENDING AND TRANSVERSE COLONS TO THE LEFT, THE
DUODENAL OBSTRUCTION IS READILY EXPOSED

17. CONT’D
• MALROTATION SHOULD BE EVALUATED BECAUSE IT CAN OCCUR IN ASSOCIATION WITH CONGENITAL
DUODENAL OBSTRUCTION IN UP TO 30% OF PATIENTS
• A SUFFICIENT LENGTH OF DUODENUM DISTAL TO THE ATRESIA IS MOBILIZED TO ALLOW FOR A TENSION-
FREE ANASTOMOSIS
• A TRANSVERSE DUODENOTOMY IS MADE IN THE ANTERIOR WALL OF THE DISTAL PORTION OF THE
DILATED PROXIMAL DUODENUM
• A DUODENOTOMY OF SIMILAR LENGTH IS MADE IN A VERTICAL ORIENTATION ON THE ANTIMESENTERIC
BORDER OF THE DISTAL DUODENUM

18. CONT’D
• THE ANASTOMOSIS IS THEN FASHIONED BY APPROXIMATING THE END OF EACH
INCISION TO THE APPROPRIATE MIDPORTION OF THE OTHER INCISION
• TAPERING DUODENOPLASTY IS USUALLY NOT NECESSARY AS THE PROXIMAL
DUODENAL DILATION FREQUENTLY RESOLVES AFTER RELIEF OF THE OBSTRUCTION
• THE LAPAROSCOPIC APPROACH WAS FIRST DESCRIBED BY ROTHENBERG
• STANDARD LAPAROSCOPIC APPROACH:
• PATIENT SUPINE,
• THE ABDOMEN IS INSUFFLATED THROUGH THE UMBILICUS

23. CONT’D
• THREE PORTS ARE USED:
• ONE AT THE UMBILICUS FOR THE CAMERA
• TWO WORKING PORTS IN THE LEFT/RIGHT MIDABDOMEN FOR SUTURING
• A LIVER RETRACTOR CAN BE PLACED IN THE RIGHT OR LEFT UPPER QUADRANT IF
NECESSARY
• ALTERNATIVELY, THE LIVER CAN BE ELEVATED BY PLACING A TRANSABDOMINAL WALL
SUTURE AROUND THE FALCIFORM LIGAMENT AND TYING IT OUTSIDE THE ABDOMEN
• THE DUODENUM IS MOBILIZED, AND THE LOCATION OF OBSTRUCTION IS IDENTIFIED
• USING THE SAME PRINCIPLES THAT HAVE BEEN DESCRIBED FOR THE OPEN
APPROACH, A STANDARD DIAMOND-SHAPED ANASTOMOSIS IS CREATED

27. RISK STRATIFICATION IN DUODENAL ATRESIA
Groups Mortality
Group A > 2.5 kg weight, no
additional.
congenital anomalies
11%
Group B 2-2.5kg with no anomaly OR
> 2.5 kg with additional.
serious anomaly
40%
Group C > 2 kg OR 2-2.5 kg with
additional serious anomaly
74%

28. POST-OPERATIVE CARE
• TOTAL PARENTERAL NUTRITION (TPN) IS CONTINUED
• NASOGASTRIC TUBE OUTPUT IS MONITORED
• FEEDINGS MAY BE STARTED WHEN THE VOLUME OF THE NASOGASTRIC OUTPUT HAS
DIMINISHED AND ITS COLOR HAS LIGHTENED AND IT BECOMES CLEAR  SEVERAL
DAYS TO A WEEK
• SMALL FEEDINGS ARE THEN INITIATED WITH VOLUME AND CONCENTRATION
ADVANCED AS TOLERATED
• THE MAJORITY MAY BE DISCHARGED WITHIN ONE TO SEVERAL WEEKS

29. COMPLICATIONS
• INTRAOPERATIVE
• INCORRECT IDENTIFICATION OF THE SITE OF OBSTRUCTION MOST COMMONLY
OCCURS WHEN A LONG, FLOPPY WEB (WINDSOCK DEFORMITY) IS PRESENT
• THE UNWARY SURGEON, NOT RECOGNIZING THE TRUE ATTACHMENT OF THE WEB,
MAY THEN CONSTRUCT A BYPASS ANASTOMOSIS ENTIRELY DISTAL TO IT
• MORE THAN ONE OBSTRUCTION PRESENT (RARE)
• THE CAREFUL PASSAGE AND WITHDRAWAL OF BALLOON CATHETERS BOTH
PROXIMALLY INTO THE STOMACH AND DISTALLY INTO THE JEJUNUMBEFORE
STARTING AN ANASTOMOSIS SHOULD PREVENT BOTH OF THESE SITUATIONS

30. CONT’D
• POSTOPERATIVE
• THE MOST COMMON  PROLONGED FEEDING INTOLERANCE
• IN GENERAL, IF NO SPECIFIC DIFFICULTIES WERE ENCOUNTERED AT THE INITIAL PROCEDURE, THERE
SHOULD BE CONCERN IF RELATIVELY NORMAL FUNCTION HAS NOT BEEN ACHIEVED BY 3 WEEKS
• UPPER GASTROINTESTINAL SERIES IS HELPFUL TO SEARCH FOR
• RESIDUAL ANATOMIC OBSTRUCTION,
• ANASTOMOTIC STENOSIS,
• PREVIOUSLY UNRECOGNIZED OBSTRUCTION AT A DIFFERENT LOCATION,
• POOR PERISTALSIS
• ADDITIONAL SIMPLE TAPERING OF THE PROXIMAL DUODENUM MAY SUFFICE TO PROVIDE
ADEQUATE ADDITIONAL MOTILITY

31. REFERENCES
1. HOLCOMB GW, MURPHY JP, M.D DJO. ASHCRAFT’S PEDIATRIC
SURGERY. 5TH ED. SAUNDERS/ELSEVIER; 2010.
2. CORAN AG, ADZICK NS, M.D TMK, M.D J-ML. PEDIATRIC SURGERY. 7TH
ED. ELSEVIER HEALTH SCIENCES; 2012.

32. THANK YOU