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Duodenal Atresia

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

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Duodenal Atresia

  1. 1. DUODENAL ATRESIA DIAGNOSTIC, THERAPY, PRE-OPERATIVE AND POST-OPERATIVE CARE DR. ISA BASUKI DEPARTMENT OF SURGERY, AWS GENERAL HOSPITAL
  2. 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. 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
  4. 4. 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
  5. 5. 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
  6. 6. 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
  7. 7. 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)
  8. 8. 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
  9. 9. 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
  10. 10. 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
  11. 11. 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
  12. 12. 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
  13. 13. 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
  14. 14. 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
  15. 15. 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%
  16. 16. 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
  17. 17. 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
  18. 18. 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
  19. 19. 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.
  20. 20. THANK YOU

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