MALROTATION WITH VOLVULUS

Pathology

foregut = to ampulla of vater (ie stomach + duodenum)

midgut = from ampulla of vater → 1st half of transverse colon (small bowel & R colon)
hindgut = remainder of colon to upper anal canal
 organogenesis in GIT is complete by 12/40

 normally
o gut begins as a long straight tube from stomach to rectum
o as the midgut elongates it protrudes out of the umbilicus out of the abdomen
 remnants = Meckles diverticulum/ vitelline cysts
o midgut loops 270˚ anticlockwise around superior mesenteric artery
o proximal part of jejunum is 1st part to return
o duodenum moves to the site of ligament of Treitz
o colon moves to LUQ
 failure to return = omphalocoele or gastroshcisis
o at 8 – 10 weeks the caecum rotates and moves from left to right
o before doing this the duodenum fixes to the posterior abdominal wall
o caecum & rest of large bowel then fix to posterior abdominal wall by its
mesentery along a line running from DJ flexure to the caecum (a broad band)
o colon is fixed in each flank and the transverse colon is fixed to the stomach by
the greater omentum
 
 in malrotation
o due to only 90˚ of twisting → means colon returns 1st and is on the left with
small bowel on the right (1st and 2nd parts of duodenum are normal)
o caecum remains positioned left of the midline - attached to right lateral
abdominal wall and subhepatic region by ‘Ladd’s bands’
o predisposes to twisting
  only 1 attachment to the posterior abdominal wall at the duodenum
(which is shortened) & proximal colon = narrow band
o entire midgut can twist on this axis resulting in duodenal obstruction with or
without mid-gut ischaemia (remember the SMA is in this mesentery)
o terminal ileum and caecum are drawn into the volvulus and are wrapped
around the stalk of the mesentery in 2 – 3 tight coils
o not surprisingly midgut malrotation and volvulus is associated with abdominal
heterotaxia and asplenia/polysplenia

Clinical
age
o within 1st year of life - usually within 1st week
vomiting
obile stained vomit with soft abdo early
ooften have recurrent symptoms with twisting and untwisting
PR bleeding is a late sign
abdo distension is not a constant sign

Investigations
 AXR
o double bubble with duodenal obstruction
o absence of air beyond obstruction
 barium meal
o DJ flexure is pulled down lower than second part
o cork screw appearance of duodenum
o malposition of the caecum
o normal in 10%

Treatment
 urgent surgery
 untwist (2-3x)
 broaden the narrow root of the small bowel mesentery
  remove appendix
 +/- remove necrotic tissue

Outcome
 low recurrence rate