Malrotation of gut

Malrotation of GUT

1. WELCOME

2. Date: 30.07.2016 Venue : Classroom, Paediatric Surgery Department, BSMMU Presented by: Dr. Mominul Haider Phase-A Resident MS (Urology) MALROTATION OF GUT

3. Objectives To discuss and understand - Normal rotation of gut Definition of Malrotation Presentation Investigation Treatment Complication

4. Introduction Malrotation refers to a group of congenital anomalies resulting from aberrant intestinal rotation and fixation. Incidence : 1/6000 live births No sex/race predilection

5. Normal rotation of gut Stages of Normal Rotation Herniation Rotation Retraction Fixation

6. Physiologícal Herniation Development of the primary intestinal loop is characterized by rapid elongation, particularly of the cephalic limb. The abdominal cavity temporarily becomes too small to contain all the intestinal loops, and they enter the extraembryonic cavity in the umbilical cord during the sixth week of development

7. Rotation The primary intestinal loop rotates around an axis formed by the superior mesenteric artery When viewed from the front, this rotation is counterclockwise, and it amounts to approximately 270° when it is complete Rotation occurs during herniation (about 90°) as well as during return of the intestinal loop into the abdomen (Remaining 180°)

8. Retraction During the 1Oth week, herniated intestinal loops begin to return to the abdominal cavity. The proximal portion of the jejunum, the first part to reenter the abdominal cavity, comes to be on the left side The caecal bud is the last part of the gut to reenter the abdominal cavity.

9. Key points in embryology Intestinal rotation starts at 5 th week and completes by 11 th week Midgut is supplied by SMA Rotation takes place around SMA axis 270 degree counterclock wise rotation of prearterial and post arterial limb. Ladds bands attach to the cecum irrespective of its postion at the end of rotation from right paracolic region.

10. Rotational disorders Non rotation Incomplete rotation Reverse rotation

11. Nonrotation Neither colon or duodenum undergo rotation Most common form of malrotation . M:F=2:1

12. Incomplete rotation Counter clock wise rotation of only 180  Caecum in the epigastrium overlying 3 rd part of duodenum. Most common form of surgically treated malrolation .

13. REVERSE ROTATION Rotates clockwise. DJ loop anterior to SMA and transverse colon posterior to SMA. Causes compression of colon by SMA - obstruction. Ileocecal volvulus- due to inadequate fixation of right colon.

14. CLINICAL MANIFESTATIONS Asymptomatic Acute Midgut Volvulus – First month of life Chronic Midgut volvulus - children older than 2 years Acute duodenal obstruction secondary to congenital bands - common in neonates and infants

15. Chronic duodenal obstruction secondary to congenital bands Reverse rotation with colonic obstruction – Rare, usually seen in adults Internal Hernia ( Mesocolic hernia) Volvulus of the Caecum – seen in old patients

16. Presentation Usually in early age, but may present later in life. 75% present during 1 st month of life. 15% present within the 1 st year. Bilious vomiting remains the cardinal sign of neonatal intestinal obstruction, and malrotation must be the presumed diagnosis until proven otherwise.

17. Other signs in the neonate include abdominal pain and distention. The inconsolable infant may rapidly deteriorate as metabolic acidosis quickly advances to hypovolemic shock. Late signs include abdominal wall erythema and hematemesis or melena from progressive mucosal ischemia.

18. Acute Midgut Volvulus Sudden onset of bilious vomiting in a previously healthy, growing infant. With the onset of proximal intestinal obstruction, the distal colon empties; lower abdomen may appear scaphoid . As vascular compromise progresses, intraluminal bleeding may occur and blood is often passed per rectum. Crampy abdominal pain is common.

19. Acute duodenal obstruction An infant or newborn usually presents with forceful, bilious vomiting. Abdominal distention may or may not be present The obstruction may be complete or incomplete, so meconium or stool may have been passed. Jaundice may be seen

20. Radiologic Diagnosis of Abnormalities of Rotation and Fixation Radiologic studies play a critical role in establishing a diagnosis of intestinal malrotation .

21. Plain radiograph Right-sided jejunal markings Absence colonic shadow in RIF Features of complications - Dilated bowel loops - Air fluids levels - Pneumoperitoneum

22. Contrast X Ray Delineation of the duodenojejunal junction remains the most important diagnostic tool. The duodenum should be seen traveling across the spine to the left. Additionally, the lateral film will show the duodenum obtaining a retroperitoneal, posterior position.

23. Abnormal findings include positioning of the duodenojejunal flexure to the right of the spine, obstruction of the duodenum The “coil spring,” “corkscrew,” or “beak” appearance of the obstructed proximal jejunum Double-bubble sign in acute duodenal obstruction. Gasless abdomen in midgut volvulus (gut is filled with fluid)

24. Normal position and fixation of Duodenum

25. “Double bubble” in duodenal obstruction

26. Ultrasound Reversal of the normal anatomic relationship between the SMA and “ whirlpool sign” - midgut volvulus.

27. CT Abdomen Anatomic location of small bowel on right and colon on left Relationship of the superior mesenteric vessels – “vertically placed or inverted sides” Aplasia of the uncinate process Features of volvulus / obstruction / gangrene Other associated anomalies

28. Reversal of SMA and SMV Whirlpool sign

29. Treatment Aim of treatment- Reduce the recurrence of volvulus , not the position.

30. Preoperative preparation Aggressive resuscitation with fluid & electrolyte. Intravenous broad spectrum antibiotics. Taken to the operating room for immediate exploration. Placement of a Nasogastric tube.

31. Surgery (Ladd’s procedure)

32. 2 nd look operation is usually performed when there are multiple areas of bowel of questionable viability, when the entire midgut appears nonviable, or when clinical signs & symptoms suggest progressive loss of intestine

33. Post operative care Nasogastric decompression Total parenteral nutrition until return of bowel function.

34. Complications Diarrhea & dehydration in short bowel syndrome Postoperative intussusception Postoperative adhesion Recurrent volvulus

35. References Pediatric surgery. —7th ed. / editor in chief, Arnold G. Coran ; associate editors, N. Scott Adzick . . [et al.] Ashcraft’s pediatric surgery / [edited by] George Whitfield Holcomb III, J. Patrick Murphy ; associate editor, Daniel J. Ostlie . — 5th ed.

36. Thank You