Intestinal Malrotation in Adolescents and Adults: Spectrum of Clinical and Imaging Features
Perry J. Pickhardt1,2 and
Sanjeev Bhalla3
1 Department of Radiology, National Naval Medical Center, 8901 Wisconsin Ave.,
Bethesda, MD 20889-5600.
2 Department of Radiology, F. Edward Hébert School of Medicine, Uniformed
Services University of the Health Sciences, 4301 Jones Bridge Rd., Bethesda,
MD 20814.
3 Mallinckrodt Institute of Radiology, Washington University School of Medicine,
510 S. Kingshighway Blvd., St. Louis, MO 63110.
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Fig. 1A. —Schematic drawings of normal rotation and malrotation.
Drawing shows that normal 270° rotation and fixation of midgut results in
familiar positioning of bowel with broad mesenteric attachment (dotted
line).
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Fig. 1B. —Schematic drawings of normal rotation and malrotation.
Drawing shows that malrotation results in malpositioned bowel and narrow base
of mesenteric fixation (dotted line), which is prone to midgut
volvulus. Abnormal fibrous peritoneal bands of Ladd (curved lines)
that attach to right colon predispose to internal hernia in older
patients.
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Fig. 2D. —29-year-old woman with chronic intermittent abdominal pain.
Axial contrast-enhanced CT scan shows cecum (C) and ascending colon
predominately on left, adjacent to sigmoid colon (arrow). Small bowel
occupies right side of abdomen.
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Fig. 2A. —29-year-old woman with chronic intermittent abdominal pain.
Supine frontal abdominal radiograph shows small bowel with jejunal markings on
right (arrowheads) and colon predominately on left. Note absence of
colon in right lower quadrant (arrow).
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Fig. 2B. —29-year-old woman with chronic intermittent abdominal pain.
Spot radiograph from barium upper gastrointestinal series shows contrast
agent—filled duodenum and jejunal loops that remain right-sided without
crossing spine to left.
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Fig. 2C. —29-year-old woman with chronic intermittent abdominal pain.
Supine frontal radiograph from barium enema examination shows near-normal
location of cecum (C), possibly due to air distention or related to chance
positioning on lax mesentery of cecum.
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Fig. 3A. —22-year-old man with episodic colicky abdominal pain. Axial
contrast-enhanced CT scan shows vertical orientation of superior mesenteric
artery (arrowhead) and superior mesenteric vein (v).
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Fig. 3B. —22-year-old man with episodic colicky abdominal pain. Coronal
reformatted image shows contrast agent—filled small bowel on right and
colon on left. Note cecal position (arrow).
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Fig. 4A. —32-year-old man with left flank pain. Axial contrast-enhanced
CT scan obtained through upper abdomen shows inverted relationship between
superior mesenteric artery (arrowhead) and superior mesenteric vein
(v). Note absence of pancreatic uncinate process.
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Fig. 4B. —32-year-old man with left flank pain. Axial contrast-enhanced
CT scan obtained through mid abdomen shows characteristic appearance of small
bowel on right and colon on left.
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Fig. 5. —Transverse sonogram obtained through upper abdomen in
11-year-old girl with malrotation shows vertical or slightly inverted
orientation between superior mesenteric artery (arrowhead) and
superior mesenteric vein (v).
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Fig. 6A. —Appendicitis in two patients with malrotation. Axial
contrast-enhanced CT scans in 56-year-old woman with left lower quadrant
abdominal pain, vomiting, and leukocytosis show abnormal dilated appendix
(arrow, B) with marked periappendiceal stranding extending
from left-sided cecum. Note also superior mesenteric artery—superior
mesenteric vein inversion (arrowhead, A).
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Fig. 6B. —Appendicitis in two patients with malrotation. Axial
contrast-enhanced CT scans in 56-year-old woman with left lower quadrant
abdominal pain, vomiting, and leukocytosis show abnormal dilated appendix
(arrow, B) with marked periappendiceal stranding extending
from left-sided cecum. Note also superior mesenteric artery—superior
mesenteric vein inversion (arrowhead, A).
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Fig. 6C. —Appendicitis in two patients with malrotation. Axial
contrast-enhanced CT scan in 68-year-old woman with left-sided abdominal pain
and clinical diagnosis of diverticulitis shows enlarged appendix (A) with
periappendiceal inflammation on left. Note terminal ileum (asterisks)
crossing to left-sided cecum.
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Fig. 7A. —49-year-old woman with pseudomyxoma peritonei from mucinous
adenocarcinoma of appendix. Axial contrast-enhanced CT scan shows typical
findings of pseudomyxoma peritonei with mass effect and scalloping from
mucinous intraperitoneal loculi. Note also findings of superimposed
malposition with superior mesenteric artery—superior mesenteric vein
inversion (arrowhead).
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Fig. 7B. —49-year-old woman with pseudomyxoma peritonei from mucinous
adenocarcinoma of appendix. Axial CT scan obtained caudad to A shows
intestinal malpositioning with small bowel on left and colon on right.
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Fig. 7C. —49-year-old woman with pseudomyxoma peritonei from mucinous
adenocarcinoma of appendix. Spot radiograph from upper gastrointestinal study
obtained for evaluation of early satiety shows marked antral narrowing
(arrowheads) from peritoneal disease, resulting in retained gastric
contents. Note that duodenum (arrow) fails to cross midline.
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Fig. 8A. —Two patients with malrotation complicated by midgut volvulus.
Intraoperative photograph shows clockwise twisting of proximal small bowel
(arrowheads) around superior mesenteric artery axis. On gross
examination, bowel appears viable in this patient.
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Fig. 8B. —Two patients with malrotation complicated by midgut volvulus.
Intraoperative photograph shows midgut volvulus with ischemic and necrotic
bowel. Note multiple dilated bowel loops with dusky-gray appearance.
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Fig. 9A. —29-year-old man with acute abdominal pain and vomiting from
malrotation with midgut volvulus. His history was significant for similar
prior episodes without diagnosis. (Courtesy of Fleishman MJ, Denver, CO) Scout
image of contrast-enhanced CT scan shows abnormal but nonobstructive bowel-gas
pattern, with air-filled colonic-appearing loops on left. Note absence of
colon in right lower quadrant (arrow).
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Fig. 9B. —29-year-old man with acute abdominal pain and vomiting from
malrotation with midgut volvulus. His history was significant for similar
prior episodes without diagnosis. (Courtesy of Fleishman MJ, Denver, CO) Axial
contrast-enhanced CT scans show characteristic whirllike appearance of bowel
and mesentery wrapping around superior mesenteric artery (arrowheads,
B). Note dilated duodenum (D, B), engorged mesenteric vessels
(arrows, C), and underlying malposition of bowel.
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Fig. 9C. —29-year-old man with acute abdominal pain and vomiting from
malrotation with midgut volvulus. His history was significant for similar
prior episodes without diagnosis. (Courtesy of Fleishman MJ, Denver, CO) Axial
contrast-enhanced CT scans show characteristic whirllike appearance of bowel
and mesentery wrapping around superior mesenteric artery (arrowheads,
B). Note dilated duodenum (D, B), engorged mesenteric vessels
(arrows, C), and underlying malposition of bowel.
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Fig. 10A. —12-year-old girl with acute abdominal pain from malrotation
with midgut volvulus. Axial contrast-enhanced CT scans show characteristic
clockwise twisting of bowel, mesentery, and superior mesenteric vein
(arrowheads) around axis of superior mesenteric artery. No bowel
resection was necessary at surgery that promptly followed imaging.
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Fig. 10B. —12-year-old girl with acute abdominal pain from malrotation
with midgut volvulus. Axial contrast-enhanced CT scans show characteristic
clockwise twisting of bowel, mesentery, and superior mesenteric vein
(arrowheads) around axis of superior mesenteric artery. No bowel
resection was necessary at surgery that promptly followed imaging.
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Fig. 10C. —12-year-old girl with acute abdominal pain from malrotation
with midgut volvulus. Axial contrast-enhanced CT scans show characteristic
clockwise twisting of bowel, mesentery, and superior mesenteric vein
(arrowheads) around axis of superior mesenteric artery. No bowel
resection was necessary at surgery that promptly followed imaging.
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Fig. 11A. —55-year-old man with history of right ureteral transitional
cell carcinoma, now presenting with acute abdominal pain related to
unsuspected malrotation with strangulated internal hernia. Axial unenhanced CT
scan shows dilated bowel loops on right with pneumatosis (arrowheads)
and gas (arrow) in superior mesenteric vein, strongly suggesting
ischemic or necrotic bowel. Extensive small-bowel resection was required at
surgery.
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Fig. 11B. —55-year-old man with history of right ureteral transitional
cell carcinoma, now presenting with acute abdominal pain related to
unsuspected malrotation with strangulated internal hernia. Axial
contrast-enhanced CT scans obtained 1 year before A show findings of
malrotation that were missed, including superior mesenteric
artery—superior mesenteric vein inversion (arrowhead, B)
and malpositioning of bowel with right-sided cecum (C, C). Note also
right hydronephrosis from obstructing ureteral tumor (arrow,
B).
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Fig. 11C. —55-year-old man with history of right ureteral transitional
cell carcinoma, now presenting with acute abdominal pain related to
unsuspected malrotation with strangulated internal hernia. Axial
contrast-enhanced CT scans obtained 1 year before A show findings of
malrotation that were missed, including superior mesenteric
artery—superior mesenteric vein inversion (arrowhead, B)
and malpositioning of bowel with right-sided cecum (C, C). Note also
right hydronephrosis from obstructing ureteral tumor (arrow,
B).
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Fig. 12A. —23-year-old man with acute abdominal pain from malrotation
with internal hernia and partial midgut volvulus. Axial contrast-enhanced CT
scans show dilated duodenum (D, A), small whirl sign involving more
distal superior mesenteric artery (arrowheads, B), and
malpositioning of bowel. Localized cluster of unopacified bowel or fluid is
present inferiorly (arrows, C). Internal hernia with
encapsulated appearance was found at surgery.
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Fig. 12B. —23-year-old man with acute abdominal pain from malrotation
with internal hernia and partial midgut volvulus. Axial contrast-enhanced CT
scans show dilated duodenum (D, A), small whirl sign involving more
distal superior mesenteric artery (arrowheads, B), and
malpositioning of bowel. Localized cluster of unopacified bowel or fluid is
present inferiorly (arrows, C). Internal hernia with
encapsulated appearance was found at surgery.
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Fig. 12C. —23-year-old man with acute abdominal pain from malrotation
with internal hernia and partial midgut volvulus. Axial contrast-enhanced CT
scans show dilated duodenum (D, A), small whirl sign involving more
distal superior mesenteric artery (arrowheads, B), and
malpositioning of bowel. Localized cluster of unopacified bowel or fluid is
present inferiorly (arrows, C). Internal hernia with
encapsulated appearance was found at surgery.
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Fig. 13A. —27-year-old woman with unsuspected polysplenia variant and
malrotation who presented with abdominal pain, fever, and leukocytosis. Axial
contrast-enhanced CT scans show multiple spleens in left upper quadrant
(arrows, A), superior mesenteric artery—superior
mesenteric vein inversion (arrowhead, B), and intestinal
malpositioning. Inflamed appendix was seen on more caudal images (not
shown).
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Fig. 13B. —27-year-old woman with unsuspected polysplenia variant and
malrotation who presented with abdominal pain, fever, and leukocytosis. Axial
contrast-enhanced CT scans show multiple spleens in left upper quadrant
(arrows, A), superior mesenteric artery—superior
mesenteric vein inversion (arrowhead, B), and intestinal
malpositioning. Inflamed appendix was seen on more caudal images (not
shown).
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Fig. 14A. —26-year-old woman with malrotation and complex congenital
heart disease associated with polysplenia syndrome. Axial contrast-enhanced CT
scans show findings of polysplenia, including left inferior vena cava with
hemiazygos continuation (V, A) and short pancreas (P, A). One
spleen is seen in A (S), but multiple additional spleens were present
on more cephalad images (not shown). Note that feeding tube extending into
proximal small bowel (arrowheads) never crosses midline, consistent
with malrotation. Superior mesenteric artery—superior mesenteric vein
relationship is normal in this patient. Ascites is due to congestive heart
failure.
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Fig. 14B. —26-year-old woman with malrotation and complex congenital
heart disease associated with polysplenia syndrome. Axial contrast-enhanced CT
scans show findings of polysplenia, including left inferior vena cava with
hemiazygos continuation (V, A) and short pancreas (P, A). One
spleen is seen in A (S), but multiple additional spleens were present
on more cephalad images (not shown). Note that feeding tube extending into
proximal small bowel (arrowheads) never crosses midline, consistent
with malrotation. Superior mesenteric artery—superior mesenteric vein
relationship is normal in this patient. Ascites is due to congestive heart
failure.
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Fig. 15. —Axial contrast-enhanced CT scan shows 16-year-old girl with
reversed (mirror image) malrotation related to polysplenia syndrome. Note
right-sided stomach (St), multiple spleens (arrowheads), and
interruption of inferior vena cava with azygos continuation
(arrow).
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Copyright © 2002 by the American Roentgen Ray Society.
