Variant of Polysplenia Syndrome with Intestinal Malrotation

1
Variant of Polysplenia Syndrome with Intestinal
Malrotation

• Hannah Chang, Ph.D., HMS III
• Gillian Lieberman, M.D.

Beth Israel Deaconess Medical Center Harvard
Medical School March 20, 2009
2
Talk Outline

• Introduction clinical case
• Background gastrointestinal malrotation
• Re-examination secondary radiographic findings
• Potential diagnosis variant of polysplenia
  syndrome with malratotion
• Take home points

3
Our patient MF Clinical Presentation
MF is a 28-year-old woman with several month
history of vague intermittent abdominal pain,
with nausea and bloating. Her symptoms were not
associated with food intake. She has had normal
bowel movements and it otherwise healthy. After
multiple trips to her primary care physician
without resolution of symptoms, she presented to
our hospital for rule out of appendicitis. As
part of her workup, an Abdominal/Pelvic CT scan,
and an Upper GI study with barium and small bowel
follow-through (UGI with SBFT) were obtained.
4
Our patient MF had an Abdominal/Pelvic CT scan
and an Upper GI (UGI) study with barium and small
bowel follow-through (SBFT) as part of her
workup.
5
Our patient MF Abdominal CT Scan
PACS, BIDMC
6
Our patient MF Abdominal CT Findings
Small bowel Colon
PACS, BIDMC
C CT (coronal reconstruction)
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Our patient MF UGI SBFT Duodenal Finding
PACS, BIDMC
UGI with barium and air SBFT
Incomplete sweep of the 4th part of the duodenal
PACS, BIDMC
UGI with barium and air SBFT
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Our patient MF UGI SBFT Ileocecal Finding
PACS, BIDMC
UGI with barium and air SBFT
PACS, BIDMC
Normal location ileocecal junction
UGI with barium and air SBFT
9
Lets spend a moment to review the process of
embyronic midgut rotation.
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Stage 1 Midgut Exits the Abdomen
Moore KL Dalley AF (1999)
At 6 weeks gestation, the midgut loop is forced
to exit the abdominal cavity due to the large
size of the incipient liver and kidneys. Around
10 weeks, the midgut begins to return. But first,
a series of rotations around the superior
mesenteric artery takes place.
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Stage 2 Counterclock-wise Midgut Rotation
Moore KL Dalley AF (1999)
With the superior mesenteric artery (SMA) as an
axis, the cranial and caudal limbs of the midgut
loop rotate counterclock-wise while returning to
the abdominal cavity at the same time.
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Stage 3 Cecal Descent and Colonic Tacking
Moore KL Dalley AF (1999)
After a total of 270 degrees of
counterclock-rotation, the duodenum, small
bowels, and descending colons are in place. Next,
the cecum descends, bringing with it the
ascending colon. Finally, the mesentary of the
ascending and descending colons fuse with the
peritoneum of the abdominal walls.
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Any part of the process of midgut rotation can go
awry. What are the most common developmental
complications?
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Developmental Complications of Midgut Rotation

• Omphalocele failure of midgut to return to the
  abdominal cavity. 15000 live births.
• Rotational abnormalities most commonly,
  non-rotation, or arrest of cecal descent and
  colonic tacking. 1500 by some estimates.
• Midgut volvulus compromise of vascular supply
  from volvulus around narrow mesenteric pedicle.
  Surgical emergency. 15000 live births.

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Lets now look at some comparison cases for
classic radiographic findings for intestinal
malrotation.
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Comparison case 1 Ladd Band
Hill, M. UNSW Embryology. http//embryology.med.u
nsw.edu.au/
Ladd band Midgut volvulus
UGI with barium and air SBFT
Burk MS, et al. Am J Surg (2008)
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Comparison case 2 Inversion of SMA/SMV
SMA (A) SMV (B)
Gamblin TC, et al. Current Surgery (2003)
C axial CT
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Comparison case 3 Mesenteric Rotation Around
Narrow Pedicle (Whirlpool Sign)
C axial CT
Matzke GM, et al. Surg Endosc (2005)
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Lets now return to our patient MF. Her abdominal
findings suggested it was not a classic
malrotation with RUQ cecum and Ladd band. In
fact, her right-sided colon and left-sided small
bowels were exactly opposite to that expected for
malrotation from Stage 3 arrest. To make a
final diagnosis and possibly provide treatment,
she was taken to the OR for laparoscopic
exploration of her abdomen.
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Our patient MF Surgical Treatment
PACS, BIDMC
Appendectomy Removal of band between ascending /
descending colon
C CT (coronal reconstruction)
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Our patient MF Clinical Course
Patient MF tolerated the surgery well and had
minimal bleeding intra-operatively. She had a
smooth post-operative course and was discharged 1
day after surgery.
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Lets now return to MFs abdominal CT findings
and point out some interesting incidental
findings.
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Our patient MF Incidental CT Finding -
Polysplenia
PACS, BIDMC
PACS, BIDMC
C axial CT
Multiple splenules
C CT (coronal reconstruction)
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Our patient MF Incidental CT Finding
Duplicated Inferior Vena Cava (IVC)
PACS, BIDMC
A
PACS, BIDMC
C axial CT
PACS, BIDMC
B
C axial CT
C CT (coronal reconstruction)
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Lets discuss one possible unifying diagnosis to
explain all of patient MFs radiographic
findings.
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Clinical Presentation of Polysplenia Syndrome

• Abdominal pain

• Polysplenia

• Heterotaxy (stomach, liver, heart)

• Short pancreas

• Intestinal malrotation

• IVC abnormalities

• Azygos/hemizygos continuation

• Preduodenal portal vein

• Situs ambiguous/inversus

Patient MF
Gayer G, et al. Abdom Imaging (1999)
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Comparison case 4 Radiographic Findings for
Polysplenia Syndrome
Polysplenia Dilated azygos vein
C- axial CT
Gayer G, et al. Abdom Imaging (1999)
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Comparison case 5 Heterotaxy in Polysplenia
Syndrome
Liver
Heart Stomach
C axial CT
Gayer G, et al. Abdom Imaging (1999)
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Our patient MF Clinical Outcome
Since discharge, patient MF has presented to our
hospital two more times for vague abdominal pain.
Urinary tract infection and gynecologic
etiologies were ruled out. It remains to be
proven whether her unusual abdominal anatomy may
be causing reversible, transient mesenteric
vascular compromise, which in turn, leads to her
abdominal pain.
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Finally, lets discuss a few take-home points
gained from our patient MF.
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Take Home Points

• Intestinal malrotation should be considered in
  adults with vague abdominal symptoms
• Accurate radiographic diagnosis of intestinal
  malrotation can prevent unnecessary complications
  and/or surgeries
• Polysplenia, IVC abnormality, intestinal
  malrotation, and cardiac abnormalities can be
  syndromic in asymptomatic patients. These
  findings may have clinical significance in the
  future.

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Acknowledgements

• Gillian Lieberman, M.D.
• Maria Levantakis
• Brian Callahan, M.D.
• Dan Jones, M.D.
• Robert Lim, M.D.

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References

• Gayer G, Apter S, Jonas T, Amitai M, Zissin R,
  Sella T, Weiss P, Hertz M. Polysplenia syndrome
  detected in adulthood report of eight cases and
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  24(2) 178-84.
• Zissin R, Rathaus V, Oscadchy A, Kots E, Gayer G,
  Shapiro-Feinberg M. Intestinal malroataion as an
  incidental finding on CT in adults. Abdom
  Imaging. 1999. 24(6) 550-5.
• Matzke GM, Dozois EJ, Larson DW, Moir CR.
  Surgical management of intestinal malrotation in
  adults comparative results for open and
  laparoscopic Ladd procedures. Surg Endosc. 2005.
  19(10)1416-9.
• Gamblin TC, Stephens RE Jr, Johnson RK, Rothwell
  M. Adult malrotation a case report and review
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• Nonaka S, Shiratori H, Saijoh Y, Hamada H.
  Determination of left-right patterning of the
  mouse embryo by artificial nodal flow. Nature.
  2002. 418 (6893) 96-99.

Continued
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