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Midgut Volvulus

KEY FACTS
Gastrointestinal

TERMINOLOGY • US or CT: Whirlpool sign


• Ligament of Treitz: Suspends duodenojejunal junction TOP DIFFERENTIAL DIAGNOSES
(DJJ), defines normal duodenal rotation
• Malrotation with obstructing Ladd band
• Malrotation: Abnormal rotation & fixation of small bowel
• Spectrum of congenital duodenal obstructions
(SB) mesentery that can lead to complications
○ Bowel obstruction by Ladd bands PATHOLOGY
○ Midgut volvulus (MV) due to short mesenteric base, • If bowel malrotated, DJJ-cecal distance (mesenteric base) is
prone to twisting short, predisposing to twisting (volvulus)
• MV: Twisting of SB about superior mesenteric artery →
CLINICAL ISSUES
bowel obstruction, ischemia/necrosis
• Classic presentation: Infant with bilious vomiting
IMAGING ○ > 90% present within first 3 months of life
• Radiographs: Most common appearance is normal ○ Requires emergent upper GI (best imaging tool)
○ Distended stomach & proximal duodenum with ↓ distal • Delayed diagnosis can lead to diffuse bowel necrosis
bowel gas very suggestive • Treatment: Surgical emergency (Ladd procedure)
○ May rarely show diffuse distal bowel distention/ileus ○ Reduce volvulus, resect nonviable bowel, transect Ladd
from ischemia/necrosis bands (if present), place SB in right & colon in left
• Upper GI: Dilated duodenum to D2-D3 segment with abdomen
corkscrew/spiral sign just beyond duodenal "beak"

(Left) Anterior graphic shows a


midgut volvulus (MV) with
dilation of the proximal
duodenum ﬊ that tapers into
a coil of twisted, narrowed
loops ﬇. The cecum st is
malpositioned within the right
upper quadrant medially &
fixed by a Ladd band ﬈. Note
the purple discoloration &
dilation of the remaining small
bowel due to an ischemic ileus.
(Right) AP radiograph shows a
nonobstructive bowel gas
pattern in a patient with
bilious emesis who was
ultimately found to have MV
on a subsequent upper GI
series.

(Left) Lateral upper GI in a 3-


day-old boy with bilious
vomiting shows a dilated
duodenum st up to D3, which
ends in a beak-like
configuration ﬊ with a wisp
of contrast ﬈ extending
distally, highly suggestive of
MV. (Right) Frontal upper GI
image in the same patient (a
few seconds later) shows
proximal duodenal dilation st
with partial obstruction at D3
﬊. The corkscrew/spiral sign
﬉ is diagnostic of MV.
Thickened loops ſt in this
context suggest bowel
ischemia.

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Midgut Volvulus

Gastrointestinal
○ Atresia has double bubble sign: Marked proximal
TERMINOLOGY duodenal dilation with no distal gas
Definitions ○ Stenosis or web usually has transition to normal distal
• Ligament of Treitz: Suspends duodenojejunal junction duodenum & normal DJJ
(DJJ), defines normal duodenal rotation
• Malrotation: Abnormal rotation & fixation of small bowel PATHOLOGY
(SB) mesentery that can lead to complications General Features
○ Bowel obstruction by Ladd (peritoneal) bands
• Etiology
○ Midgut volvulus (MV) due to short mesenteric base
○ With normal rotation, DJJ positioned in left upper
prone to twisting
quadrant & cecum positioned in right lower quadrant
• MV: Abnormal twisting of SB about superior mesenteric
– Results in long fixed mesenteric base between
artery (SMA) that can lead to bowel obstruction &
ligament of Treitz & cecum that keeps mesentery
ischemia/necrosis
from twisting
○ If bowel malrotated, DJJ-cecal distance (mesenteric
IMAGING base) is short, predisposing to twisting (volvulus)
Radiographic Findings
• Distended stomach & proximal duodenum with ↓ distal CLINICAL ISSUES
bowel gas very suggestive Presentation
○ Different from marked longstanding bulbous dilation
• Most common signs/symptoms
without distal gas, as seen in duodenal atresia
○ Bilious emesis in 1st month of life
• May rarely show diffuse distal bowel distention/ileus from
– Green/yellow vomit typically from obstruction of
ischemia/necrosis
duodenum distal to ampulla of Vater
○ Such children often extremely ill
• Other signs/symptoms
• Most common early finding: Normal
○ Acute abdominal pain
Fluoroscopic Findings ○ Patients may be asymptomatic or have atypical or
• Upper GI chronic symptoms
○ Dilated duodenum to D2-D3 Demographics
– Degree of proximal duodenal dilation depends on
• > 90% present within first 3 months of life
chronicity
• 39% present within first 10 days of life
○ Often beaked appearance at level of twist, ± complete
• Can occur at any age
obstruction
○ Usually spiral/corkscrew appearance distal to beak Natural History & Prognosis
○ May see malrotation without MV • Delay in diagnosis can lead to diffuse bowel necrosis
– In patients with bilious emesis, this may reflect
intermittent volvulus Treatment
• Surgical emergency
Ultrasonographic Findings
○ Ladd procedure: Reduce volvulus, resect nonviable
• Proximal duodenum usually dilated bowel, transect Ladd bands (if present), place SB in right
• Whirlpool sign of swirling vessels (SMV) & SB mesentery & colon in left abdomen
around SMA on grayscale & color Doppler
CT Findings SELECTED REFERENCES
• CECT 1. Carroll AG et al: Comparative effectiveness of imaging modalities for the
diagnosis of intestinal obstruction in neonates and infants: a critically
○ Whirlpool sign of swirling vessels (SMV) & SB mesentery appraised topic. Acad Radiol. 23(5):559-68, 2016
around SMA 2. Drewett M et al: The burden of excluding malrotation in term neonates with
bile stained vomiting. Pediatr Surg Int. 32(5):483-6, 2016
○ Potentially ↓ or no enhancement of SB due to
3. Dumitriu DI et al: Ultrasound of the duodenum in children. Pediatr Radiol.
obstruction of SMA (causing ischemia/necrosis) 46(9):1324-31, 2016
○ May have SB distention due to ischemic ileus 4. Horsch S et al: Volvulus in term and preterm infants - clinical presentation
and outcome. Acta Paediatr. 105(6):623-7, 2016
Imaging Recommendations 5. Shrimal PK et al: Midgut volvulus with whirlpool sign. Clin Gastroenterol
• Best imaging tool Hepatol. 14(2):e13, 2016
6. Mitsunaga T et al: Risk factors for intestinal obstruction after Ladd
○ Infant with bilious vomiting → emergent upper GI procedure. Pediatr Rep. 7(2):5795, 2015
7. Marine MB et al: Imaging of malrotation in the neonate. Semin Ultrasound
DIFFERENTIAL DIAGNOSIS CT MR. 35(6):555-70, 2014
8. Nehra D et al: Intestinal malrotation: varied clinical presentation from infancy
Malrotation With Obstructing Ladd Bands through adulthood. Surgery. 149(3):386-93, 2011
• May be completely obstructive with beaking, mimicking MV
Spectrum of Congenital Duodenal Obstruction
• Duodenal atresia, duodenal stenosis, annular pancreas,
duodenal web

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