malrotation of gut

1. Malrotation
Abhilash
11/09/2013

2. Introduction
• Malrotation –
• Group of congenital anomalies resulting from
aberrant intestinal rotation and fixation.
• Takes place during the first three months of
gestation.
• First reported by William Ladd in 1932.

3. Epidemiology
• Incidence : 1/6000 live births
• Most present < 1 month
• Incidence in general population – 0.2 to 0.5 %
• No sex/race predilection

4. Embryology
• 1. Herniation
• 2. Return to the abdomen.
• 3. Fixation.
• mutations in the forkhead box transcription
factor FOX - familial malrotation

5. Key points in embryology
• Intestinal rotation starts at 5th week.
• Midgut – 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.

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

14. • Duodenum not posterior to SMA
• Ligament of Treitz fails to reach its normal
position is right upper quadrant.
• Midgut mesentry is narrow and highly mobile.
• May cause
• Duodenal obstruction abnormal peritoneal
bands.
• Acute midgut volvulus.

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

17. 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.

18. Stringers classification
• I – Non rotation of colon and duodenum
• IIA – Pure duodenal nonrotation
• IIB- Reversed rotation of duodenum and colon
• IIC – Reversed rotation of duodenum only
• IIIA – Nonrotation of colon
• IIIB- Incomplete fixation of hepatic flexure
• IIIC- Incomplete attachment of cecum and
mesocecum

20. Associated anomalies(seen in 30%-
60%)
• Duodenal atresia / stenosis / web
• Congenital diaphragmatic hernia
• Gastroschisis
• Omphalocele
• Choanal atresia
• Polysplenia / asplenia
• Congenital megacolon

21. How does it present.
• Asymptomatic
• Midgut volvulus
• Mesocolic hernias.
• Duodenal and jejunal obstruction.
• Colonic obstruction.

22. Clinical features in adults
• Intermittent cramping or persistent aching pain.
• Severe abdominal cramping followed by diarrhea
- chronic volvulus.
• Vomiting - bilious /non bilious , variable in
duration and frequency.
• Malabsorption - diarrhea, nutritional deficiencies
• Rare - obstructive jaundice, chylous ascites and
superior mesenteric vein thrombosis

23. Plain radiograph
• No pathognomonic signs.
• Right-sided jejunal markings
• Absence colonic shadow in RIF
• Features of complications
- Dilated bowel loops
- Air fluids levels
- Pneumoperitoneum

24. Ultrasound
• Reversal of the normal anatomic relationship
between the SMA and
• “whirlpool sign” - midgut volvulus.
• “bird beak” appearance – duodenal
obstruction.
• false-positive rates of up to 21%

26. Upper GI contrast study
>incomplete duodenal obstruction, usually in
the third portion;
>ligament of Treitz not to the left of the midline
or at the level of the gastric antrum;
>abnormal position of the proximal jejunal loops
to the right of the midline; and
>deformity of the duodenum with a bird's beak,
corkscrew,•or coiled•configuration

28. 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

29. Reversal of SMA and SMV Whirlpool sign

30. Management
• Supportive management.
• Surgical management – Ladds procedure.

31. Ladd's procedure

32. Post operative care
• nasogastric decompression
• total parenteral nutrition until return of
bowel function.

33. • Mortality from midgut
• volvulus with severe bowel compromise may
exceed 30%.
• Long-term complications
1. adhesive small bowel obstruction (10%),
2. recurrent volvulus,
3. short gut syndrome.

34. References
• Ladd WE. Congenital obstruction of the
duodenum in children. N Engl J Med.
1932;206:277–83.
• Principles and Practice of Pediatric Surgery,
4th Edition. Keith T Oldam.
• Shackelford’s Surgery of the Alimentary tract
7th edition.
• Stringer Pediatric Gastrointestinal Imaging and
Intervention, 2nd edition