Duodenal atresia stenosis PRANAYA PPT

Duodenal, atresia, stenosis, PRANAYA, PPT, duodenal web, windsock, web, obstruction, double bubble, bilious, vomiting,

1. DUODENAL ATRESIA AND STENOSIS DR . PRANAYA PANIGRAHI BHU IMS, VARANASI

2. INTRODUCTION Congenital stenosis or atresia constitute one of the most common cause of neonatal intestinal obstruction.

3. PYLORIC ATRESIA DUODENAL ATRESIA AND STENOSIS – ANNULAR PANCREAS JEJUNOILEAL ATRESIA AND STENOSIS

4. PYLORIC ATRESIA Rare autosomal genetic defect. Lumen is completely obliterated by either a diaphragm or a solid core of tissue or compelete absence of pylorus. Nonbilious vomiting and upper abdominal distention. Xray : Single gas bubble and no distal gas in GI tract. T/t- Side-to-side Gastroduodenostomy .

5. DUODENAL ATRESIA AND STENOSIS Duodenum is one of the most common site of neonatal intestinal obstruction. Duodenal atresia , stenosis , and Annular P ancreas recognized as disease entities. First case reported by Calder in 1733. Cordes , in 1901 describe typical clinical finding. Association with trisomy 21 (Down syndrome) Incidence of duodenal atresia 1 in 6000 to 1 in 10,000 birth.

6. EMBRYOLOGY

7. The endoderm gives rise to the gut tube beginning in the fourth week of development. In the sixth week, the gut epithelium proliferates rapidly, resulting in obliteration of the intestinal lumen . The intestine is then gradually recanalized over the next several weeks of development. Errors in recanalization are thought to be the primary cause of duodenal atresia and stenosis.

8. If the ventral bud fails to rotate completely, it remains anterior to the duodenum, and fusion with the dorsal pancreatic primordium results in a ring of pancreatic tissue encircling the duodenum , creating an annular pancreas with concomitant partial or complete obstruction . More often, duodenal atresia or stenotic web underlies the annulus and is the actual cause of blockage.

9. Associated Anomalies Anomaly Percent Down syndrome 28.2 Annular pancreas 23.1 Congenital heart dis 22.6 Malrotation 19.7 EA/TEF 8.5 Genitourinary 8.0 Anorectal 4.4 Other bowel atresia 3.5 Other 10.9

10. Spectrum of Disorders Several varieties of intrinsic and extrinsic congenital lesions can cause complete (81%) or partial (19%) obstruction of the duodenum. Intrinsic obstruction includes IMPERFORATE and PERFORATE WEBS of variable thickness within continuous bowel as well as complete or almost complete bowel discontinuity.

11. CLASSIFICATION (Gray and Skandalakis ) Type 1 (92% of cases): There is an obstructing septum (web ) formed from mucosa and submucosa with no defect in the muscularis . mesentery is intact A variant of type 1 duodenal atresia, a “windsock deformity ,” can occur if the membrane is thin and elongated. Base of the membrane usually lies in the second portion of the duodenum BUT balloons out distally distending 3 rd and 4 th portion

12. TYPE 1

13. Type 2 (1% of cases): A short fibrous cord connects the two blind ends of the duodenum. The mesentery is intact Type 3 (7% of cases): There is no connection between the two blind ends of the duodenum. There is a V-shaped mesenteric defect A- TYPE 1 B- TYPE 2 C- TYPE 3

14. SITE OF ATRESIA AND STENOSIS Intrinsic blockage may occur in almost any portion of the duodenum. I t occurs near the junction of the first and second portions in 85% of cases.

15. EXTRINSIC OBSTRUCTION INCLUDE Annular Pancreas, Pre-duodenal Portal Vein, Ladd Bands Volvulus.

16. PREDUODENAL PORTAL VEIN crosses the anterior surface of the second portion of theduodenum , rather than running posterior to it, and may cause incomplete obstruction by compression. --------------------------------------------------------------------- Intestinal malrotation results in Ladd bands that usually give rise to an extrinsic partial obstruction of the second to third portion of the duodenum. ------------------------------------------------------------------- Varying degrees of midgut volvulus—acute , intermittent, or chronic—can occur in these infants , also resulting in obstruction.

17. Clinical Presentation Maternal polyhydramnios , noted in 30% to 65% of cases is an early clue. The classic “double-bubble ” obstructive pattern is usually identifiable on fetal ultrasonography

18. Repeated bilious emesis is the characteristic clinical feature of almost all newborns with duodenal obstruction. Because of the proximal level of intestinal blockage, the infant does not appear distended, although a subtle upper abdominal fullness may be noted .

19. DIAGNOSIS complete or high-grade duodenal obstruction , a plain film of the abdomen will generally confirm the diagnosis, with a finding of the “ double-bubble” sign . The lack of more distal intestinal gas is diagnostic of a complete obstruction.

20. presence of gas indicate partial obstruction. If diagnosis is suspected but the “double-bubble” sign is not clearly visible, injection of 30 to 60 mL of air through the nasogastric tube may demonstrate this characteristic imaging. Recognition of partial obstructions may be considerably delayed if the obstruction is of a relatively minor degree

21. MANAGEMENT Characteristic findings of duodenal obstruction on plain abdominal radiographs-- it is appropriate to proceed directly to operative intervention without obtaining contrast studies . Occasionally , an upper gastrointestinal study ( UGI) may be helpful in differentiating intrinsic duodenal obstruction from mid-gut volvulus.

22. Distal “ beaking ” effect in the third portion should arouse concern about volvulus and the need for an urgent operation. The UGI Series is the most useful study for the evaluation of older infants and children with symptoms of chronic partial obstruction. Gastroduodenoscopy may also be a useful diagnostic and sometimes therapeutic tool in these patients.

23. Treatment N asogastric or Orogastric tube decompression and I ntravenous fliuds . Gastrointestinal (GI) losses are replaced appropriately and placement of a peripherally inserted central catheter (PICC) line for parenteral nutrition. Surgery after the infant is optimized and associated anomalies have been appropriately studied.

24. Operative procedure Right upper quadrant transverse incision halfway between the liver edge and the umbilicus. Right colon and hepatic flexure are then mobilized medially to allow full exposure of the proximal duodenum. Access to the third and fourth portions of the duodenum

25. In type 1 cases, diminutive, gasless bowel distal to this point indicates that the obstruction is complete In type 2 and 3 cases, the discontinuity of the bowel will become evident during dissection In patients with annular pancreas, pancreatic tissue will be seen extending circumferentially around the second portion of the duodenum . Gross dilatation of the proximal segment, a tapering duodenoplasty , as the initial part of the procedure, may hasten the postoperative return of effective peristalsis

26. Tapering duodenoplasty – suture plication resection using a gastrointestinal anastomosis (GIA) stapler or needle-tip electrocautery and suture closure . Tapering is positioned on the anterior or anterolateral surface to avoid damage to the common bile duct, pancreas, and ampulla Hasten the postoperative return of effective peristalsis.

27. If there is continuity of the proximal and distal duodenum, it is best to open the distal bowel near the apparent point of obstruction, in a position and direction suitable for a potential bypass. filmy or thin webs- may sometimes be excised rather than bypassed. Then duodenum is closed as a duodenoplasty in a transverse manner, to shorten and widen the bowel overlying the resection to minimize the risk of stenosis.

28. Duodenoduodenostomy , joining the bowel just proximal and distal to the obstruction, is the best corrective option. When procedure is difficult because of patient anatomy, particularly in some small, premature infants , duodenojejunostomy is the next best choice . When performing a bypass anastomosis, a “ diamond anastomosis ” (proximal transverse and distal longitudinal incisions)

29. With type 3 deformities, the end of the distal segment is spatulated appropriately. Before making the incision in the distal segment, it is useful to inject saline into the lumen while occluding the bowel distally to slightly stretch the wall, thus enabling a technically easier anastomosis. 10-Fr Foley catheter should be passed proximally into the stomach and distally into the jejunum and pulled back with the balloon inflated, to ensure that no additional web or a windsock deformity is overlooked

30. DIAMOND ANASTOMOSIS Placing initial stay sutures joining the midpoints of each incision to the ends of the other. Placement should anticipate a posterior row of knots inside the lumen and an anterior row on the outside laparoscopic duodenoduodenostomy is another option

31. DUODENOJEJUNOSTOMY

32. When an annular pancreas is encountered in association with duodenal obstruction, bypass is always the procedure of choice. The ring of pancreatic tissue should never be transected because of the major ductal structures that traverse it. Damage to these structures will lead to leakage of pancreatic fluid and/or pancreatitis. An obstructing preduodenal portal vein is bypassed in a similar manner.

33. INTRAOPERATIVE COMPLICATIONS Incorrect identification of the site POSTOPERATIVE COMPLICATION Prolonged feeding intolerance is the most common complication following surgery to relieve duodenal obstruction.

34. Late duodenal obstruction may occasionally be noted in older infants and children. This may occur following a seemingly successful neonatal bypass procedure or excision of web, or de novo, when a diagnosis of partial duodenal obstruction was initially missed

35. CONCLUSION Survival of infants with duodenal obstruction has increased from 45% to 95% Almost all mortality is now related to associated anomalies of other organ systems, primarily those of the heart, lungs, and brain

36. JEJUNOILEAL ATRESIA AND STENOSIS