This document discusses intestinal malrotation, which occurs due to abnormal intestinal rotation and fixation during development. It describes the normal stages of intestinal rotation and fixation. Rotational disorders include nonrotation, incomplete rotation, and reverse rotation. Clinical manifestations include acute midgut volvulus in infants and chronic symptoms in older children. Radiologic imaging plays a key role in diagnosis. Treatment involves reducing recurrence of volvulus through the Ladd's procedure to divide abnormal bands and fix the intestine in normal positions.

1.  Paediatric Surgery Department,
Presented by:
Dr. Hussein Iman

2. 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

3. Normal rotation of
gut
Stages of Normal Rotation
Herniation
Rotation
Retraction
Fixation

4. 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

6. 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°)

10. 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.

14. Key points in embryology
• Intestinal rotation starts at 5th week and
completes by 11th 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.

15. Rotational disorders
Non rotation
Incomplete rotation
Reverse rotation

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

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

20. 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.

22. 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

23. • 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

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

25. • 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.

26. 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.

27. 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

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

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

31. 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.

32. • 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)

33. Normal position and fixation of
Duodenum

34. “Double bubble” in duodenal
obstruction

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

39. 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

41. Reversal of SMA and SMV Whirlpool sign

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

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

44. Surgery (Ladd’s procedure)

46. • 2nd 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

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

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

49. 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.