AJR 2002; 179:1429-1435
© American Roentgen Ray Society
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.
Received April 8, 2002;
accepted after revision May 23, 2002.
The opinions and assertions contained herein are the private views of the
authors and are not to be constructed as official or as reflecting the views
of the Department of the Navy or the Department of Defense.
Presented at the annual meeting of the American Roentgen Ray Society,
Atlanta, April—May 2002.
Address correspondence to P. J. Pickhardt.
Introduction
Congenital malrotation of the midgut often presents within the first month
of life. Pediatric radiologists are, therefore, consciously attuned to this
malady and its associated imaging features. The overall incidence of
malrotation, however, is unknown because some patients will present years
later or remain asymptomatic for life. Because presentation is nonspecific and
the index of suspicion for malrotation progressively decreases in the older
population, the clinical diagnosis is usually not considered in the initial
evaluation. At least some of the surgical literature, however, seems to favor
surgery for malrotation regardless of patient age. This recommendation further
underscores the importance of recognizing this unsuspected diagnosis on
imaging. We review the imaging features of malrotation in adolescents and
adults in the context of various clinical scenarios in which it may be
encountered. Abdominal CT findings will be emphasized because abdominal CT is
a frequent means of detection in patients with malrotation.
Embryology and Classification
Intestinal malrotation can be broadly defined as any deviation from the
normal 270° counterclockwise rotation of the midgut during embryologic
development. Classification of this spectrum of abnormalities into various
subtypes based on the stage of midgut development is possible
[1,
2]. From a practical
standpoint, however, it is useful to simplify categorization into nonrotation
and incomplete rotation [3].
"Incomplete rotation" refers to the spectrum of partial rotational
anomalies possible with either the duodenum or the right colon. Reversed
rotation can also occur but is rare
[1]. Because
"nonrotation" represents most cases identified in the older
population [2], this term will
be considered essentially synonymous with "malrotation" for the
purposes of this review.
Malrotation results not only in the malposition of the bowel but also in
the malfixation of the mesentery. The normally broad mesenteric attachment is
shortened to a narrow pedicle that predisposes the patient to the complication
of midgut volvulus (Fig.
1A,1B).
Internal hernia related to abnormal peritoneal fibrous bands (of Ladd) that
attach to the right colon is another complication of malrotation seen in
adults (Fig.
1A,1B).
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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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Clinical and Imaging Features
Midgut malrotation has been estimated to occur in approximately one in 500
live births [1,
3]. However, it is difficult to
ascertain the true incidence because this condition will go undetected during
childhood in a substantial subset of patients. Reportedly, 64-80% of patients
present within the first month of life, but these figures generally do not
take into account those who remain undiagnosed after childhood
[1,
3]. In older children and
adults, malrotation is rarely suspected on clinical grounds and is generally
first visible on imaging studies or at laparotomy. The radiologist may
encounter this important diagnosis in several different clinical settings such
as an incidental imaging finding, the cause of acute abdominal symptoms, or a
condition associated with abdominal situs abnormalities.
Malrotation as an Incidental Imaging Finding
Detection of uncomplicated or quiescent malrotation on conventional or
cross-sectional imaging should not be trivialized because no reliable means
exists to predict which patients might experience a future complication.
Furthermore, some patients with ostensibly asymptomatic malrotation may, in
fact, relate a history of episodic abdominal pain or vomiting
[3,
4]. Although the recommendation
is controversial, many authorities advocate surgical correction (Ladd's
procedure) for all operative candidates with malrotation, regardless of age
[3]. This viewpoint might come
as a surprise to radiologists who previously viewed incidental malrotation in
adults as little more than an academic
curiosity.
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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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Conventional radiography is neither sensitive nor specific for malrotation,
although right-sided jejunal markings and the absence of a stool-filled colon
in the right lower quadrant may be suggestive of this finding
[4]
(Fig. 2A). The upper
gastrointestinal barium series remains accurate for detection, and the rules
familiar to pediatric radiology also apply for adults—that is, the
duodenal—jejunal junction fails to cross the midline and lies below the
level of the duodenal bulb (Fig.
2B). An abnormal junction in an adult should not be dismissed as a
normal variant [4]. Contrast
enema examination usually shows malposition of the right colon, but the cecum
may assume a normal location in up to 20% of patients. This normal location
may cause malrotation to be missed on this type of study
[3]
(Fig. 2C). The contrast enema
findings are also nonspecific because cecal location can be variable without
malrotation.
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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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Many cases of quiescent malrotation in adults are currently being detected
on cross-sectional imaging (particularly CT) performed for various unrelated
reasons [2]. CT not only shows
the intestinal malpositioning seen on barium studies but also depicts
associated extraintestinal findings not evident on conventional examinations.
For example, deviation from the normal relationship between the superior
mesenteric artery (SMA) and superior mesenteric vein (SMV) is a useful
indicator of malrotation [5].
In most patients with quiescent malrotation, the SMA and SMV will assume a
vertical relationship (Fig.
3A,3B)
or show left—right inversion
[2] (Fig.
4A,4B).
Analogous findings can be seen on sonography
(Fig. 5). Abnormalities of
SMA—SMV orientation are not entirely diagnostic, however, because some
patients with malrotation will have a normal relationship, and a vertical or
inverted relationship can also be seen in patients without malrotation
[2]. Therefore, isolated
detection of such an abnormality is not sufficient for diagnosis but should
warrant closer examination of the bowel. Finally, inspection of the pancreas
in malrotation will reveal underdevelopment or absence of the uncinate process
(Fig.
4A,4B).
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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. 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. 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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In patients with abdominal abnormalities unrelated to coexisting
malrotation, the altered anatomy may result in an atypical clinical
presentation. A prime example is appendicitis, which may present with symptoms
that are more left-sided (Fig.
6A,6B,6C).
Careful review of the CT images, however, should yield the correct diagnosis,
including the unsuspected malrotation. In practice, malrotation may be
incidentally discovered, superimposed on virtually any abdominal disease (Fig.
7A,7B,7C).
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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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Malrotation Presenting with Acute Symptoms
Midgut volvulus is a complication of malrotation in which clockwise
twisting of the bowel around the SMA axis occurs because of the narrowed
mesenteric attachment (Fig.
8A,8B).
This life-threatening condition is a clear indication for emergent surgery.
The clinical diagnosis of midgut volvulus in adolescents and adults is
difficult because the presentation is usually nonspecific and malrotation is
rarely considered. Recurrent episodes of colicky abdominal pain with vomiting
over a period of months or years are typical and may eventually lead to
imaging [2]. Diarrhea and
malabsorption from chronic venous and lymphatic obstruction may also occur
[4].
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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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Findings on abdominal radiographs in midgut volvulus are usually abnormal
but non-specific (Fig.
9A,9B,9C).
Upper gastrointestinal examination (the study of choice in neonates) shows the
typical corkscrew appearance of the proximal small bowel. However, in older
patients with acute symptoms, CT is generally performed instead of a barium
examination. Fortunately, the CT findings of malrotation with midgut volvulus
are characteristic. The CT whirl or whirlpool sign describes the swirling
appearance of bowel and mesentery twisted around the SMA axis
[6] (Figs.
9A,9B,9C
and
10A,10B,10C).
A similar appearance can be seen on sonography. Additional CT findings include
duodenal obstruction, congestion of the mesenteric vasculature, and evidence
of underlying malrotation (Fig.
9A,9B,9C).
The presence of intestinal ischemia or necrosis is an ominous sign
(Fig. 8B).
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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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Internal hernia caused by abnormal peritoneal bands is an underrecognized
complication of malrotation after childhood
[3]. This condition may also be
life-threatening because of the risk of bowel obstruction and strangulation.
CT findings of malrotation and small-bowel obstruction (without volvulus) may
be seen in patients having this complication (Fig.
11A,11B,11C).
Evidence of ischemic bowel again portends a poor prognosis (Fig.
11A,11B,11C).
Some patients may present with a combination of midgut volvulus and internal
hernia (Fig.
12A,12B,12C).
These life-threatening events in adults who may be otherwise healthy
underscore the importance of earlier detection and treatment.
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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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Malrotation Associated with Heterotaxy
Rotational abnormalities are seen in approximately 70% of patients with
situs ambiguous (heterotaxy), including both asplenia and polysplenia
syndromes [7]. Most patients
with heterotaxy syndromes die in childhood because of their complex congenital
heart disease. Approximately 10% of patients with polysplenia (left-sided
isomerism) may reach adulthood without any complication
[8]. Unless they present for
imaging, these patients will go undiagnosed.
Because polysplenia represents a broad spectrum of anomalies, a variety of
CT findings are seen in adults with this syndrome. In addition to multiple
spleens, other abdominal findings may include a left-sided inferior vena cava,
azygos or hemiazygos continuation of an interrupted inferior vena cava, a
preduodenal portal vein, and a truncated appearance to the pancreas
[8] (Figs.
13A,13B,14A,14B,15).
The multiple spleens are typically adjacent to the stomach, which can be
left-sided or right-sided (Fig.
15). Intestinal malrotation may have the same right—left
orientation seen in situs solitus or have a reversed mirror image
configuration, but both forms cause the patient to be at risk for midgut
volvulus [8,
9]. The SMA—SMV
relationship is also variable (Figs.
13A,13B
and
14A,14B).
Like malrotation itself, polysplenia may represent an unsuspected imaging
diagnosis in an otherwise healthy adult (Fig.
13A,13B).
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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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Conclusion
The clinical diagnosis of malrotation after childhood is usually not
considered; this oversight underscores the importance of recognizing this
unsuspected condition on imaging. Regardless of patient age, surgical
treatment of quiescent malrotation should be considered because surgery
remains the only real safeguard against complications. In older patients who
present with acute symptoms related to unsuspected malrotation, rapid imaging
diagnosis and surgery may be life-saving.
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718 - 728.
[Abstract]
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Introduction
Embryology and Classification
