Malrotation is a congenital anomaly resulting from abnormal intestinal rotation during gestation. It can cause midgut volvulus and obstruction. Clinical presentation varies from asymptomatic to symptoms of obstruction. Diagnosis is made through imaging findings showing abnormal relationships of intestines and superior mesenteric vessels. Treatment is surgical correction through Ladd's procedure to unwind volvulus and fix intestines in normal position. Complications include recurrent volvulus and adhesive bowel obstruction.

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