DUODENAL ATRESIADIAGNOSTIC, THERAPY, PRE-OPERATIVE AND POST-OPERATIVE

CARE

DR. ISA BASUKI

DEPARTMENT OF SURGERY, AWS GENERAL HOSPITAL

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%

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

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

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

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

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)

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

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

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

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

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

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

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

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%

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

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

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

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.

THANK YOU