Intestinal malrotation and
volvulus
Introduction
refers to any variation of abnormality in rotation and fixation of the GI tract
during the fetal development and this condition leads to various acute and
chronic presentations of disease.
Epidemiology
1/200-1/500 live birth: most of patients are asymptomatic.
Only 1/6000 live birth are symptomatic and diagnosis may occur at any
age, even prenatal.
30-62% of children with IM have associated congenital anomaly.
All children with CDH, omphalocele, gastroschisis have
malrotation by definition.
17% of duodenal atresia and 33% of patients with jejunal atresia have
malrotation.
Embryology
development of the alimentary tract:
Develops from the embryonic foregut, midgut and hindgut.
Midgut maturation involves 4 stages: herniation, rotation,
retraction and fixation.
Normal rotation of the proximal duodenojejunal loop and the loop take
place around the superior mesenteric artery(SMA) as the axis, both make
270 degree conterclockwise
Counter-clockwise rotation
Counter-clockwise rotation of the gut occurs through 270 degree
concomitantly with herniation of the small intestinal loops followed by return of
the gut to the abdominal cavity during the third month of gestation.
Intestinal malrotation and volvulus 1
Counter-clockwise Rotation 90 X 3 times ⭐️
1. Primary intestinal loop before rotation
2. 1st rotation in 90degree : coecum is left side
3. 2 nd rotation in 180degree : is to duodenum, easy to get vulvolus
4. 3 rd rotation in 270degree : coecum is in normal position
Duodenal obstrunction can occur due to
extrinsic compression from bands leading from the caecum to the lateral
abdominal wall (Ladd’s bands)or from small bowel volvulus.
which also leads to ischaemia of the midgut from superior mesenteric
artery occlusion
associated anomalies
Clinical feature
Most of these in the newborn period
30% : 3-7% days of life
50-75% : before 1 month
Intestinal malrotation and volvulus 2
At greatest risk of life-threatening midgut volvulus ,although this
complication can occur at any age
Acute onset of volvulus is a true emergency and must be
diagnosed quickly
Bilious vomiting
passed meconium or normal stools
Rapid deterioration with abdominal distress
Rectal bleeding may occur.
Billous vomiting in the newborn is a sign of intestinal obstruction unti proved
otherwise
Abdominal distention
progressive midgut ischaemia
Intestinal necrosis
Peritonitis ,skin discoloration
Prenatal diagnosis
Ultrasound
first detected as dilated bowel during fetal ultrasound assessment.
compression of the duodenum by Ladd’s bands in utero may also
explain the association between malrotation and some cases of duodenal
atresia or stenosis.
Diagnosis during childhood beyond infancy,the
child with malrotation may present
recurrent abdominal pain and
chronic or intermittent vomiting,(which may or may not be bilious)
confirmed by other investigations.
Investigations
X-RAY :Plain Abdominal Radiograph
Intestinal malrotation and volvulus 3
Double-bubble sign and some air beyond the double bubble
A relatively airless abdomen is highly suggestive of volvulus with
malrotation
Upper gastrointestinal contrast
Lateral show duodenum in the retroperitoneal
Low-lying ligament of Treitz, or failur to be located left of the spine.
In case of volvulus: coil spring or cork screw sign, the « beak » in
case of incomplete obstruction.
Lower GI contrast enema
May be helpful to identify the position of the cecum and whole colon.
Ultrasound
Doppler US: dilated duodenum with inversion of the SMA and vein (
the whirlpool sign).
CT scan
Not recommended as principal diagnostic tool suitable for acute
volvulus.
Treatment
Neonates with malrotation and midgut volvulus with the finding of acute
abdomen require emergency laparotpmy.
Intestinal malrotation and volvulus 4
