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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 . Talk Outline. Introduction : clinical case Background : gastrointestinal malrotation

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variant of polysplenia syndrome with intestinal malrotation

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

talk outline
Talk Outline
  • Introduction: clinical case
  • Background: gastrointestinal malrotation
  • Re-examination:secondary radiographic findings
  • Potential diagnosis: variant of polysplenia syndrome with malratotion
  • Take home points
our patient mf clinical presentation
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.

slide4

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.

slide6

Our patient MF: Abdominal CT Findings

Small bowel

Colon

PACS, BIDMC

C+ CT (coronal reconstruction)

slide7

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

slide8

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

stage 1 midgut exits the abdomen
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.

stage 2 counterclock wise midgut rotation
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.

stage 3 cecal descent and colonic tacking
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.

slide13

Any part of the process of midgut rotation can go awry. What are the most common developmental complications?

developmental complications of midgut rotation
Developmental Complications of Midgut Rotation
  • Omphalocele: failure of midgut to return to the abdominal cavity. 1:5000 live births.
  • Rotational abnormalities: most commonly, non-rotation, or arrest of cecal descent and colonic tacking. 1:500 by some estimates.
  • Midgut volvulus: compromise of vascular supply from volvulus around narrow mesenteric pedicle. Surgical emergency. 1:5000 live births.
slide15

Let’s now look at some comparison cases for classic radiographic findings for intestinal malrotation.

comparison case 1 ladd band
Comparison case #1: Ladd Band

Hill, M. UNSW Embryology.

http://embryology.med.unsw.edu.au/

Ladd band

Midgut volvulus

UGI with barium and air + SBFT

Burk MS, et al. Am J Surg (2008)

comparison case 2 inversion of sma smv
Comparison case #2: Inversion of SMA/SMV

SMA (A)

SMV (B)

C+ axial CT

Gamblin TC, et al. Current Surgery (2003)

comparison case 3 mesenteric rotation around narrow pedicle whirlpool sign
Comparison case #3: Mesenteric Rotation Around Narrow Pedicle (“Whirlpool Sign”)

C+ axial CT

Matzke GM, et al. Surg Endosc (2005)

slide19

Let’s 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.

our patient mf surgical treatment
Our patient MF: Surgical Treatment

PACS, BIDMC

Appendectomy

Removal of band between ascending / descending colon

C+ CT (coronal reconstruction)

our patient mf clinical course
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.

slide22

Let’s now return to MF’s abdominal CT findings and point out some interesting incidental findings.

our patient mf incidental ct finding polysplenia
Our patient MF: Incidental CT Finding - Polysplenia

PACS, BIDMC

PACS, BIDMC

C+ axial CT

Multiple splenules

C+ CT (coronal reconstruction)

our patient mf incidental ct finding duplicated inferior vena cava ivc

A

B

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)

slide25

Let’s discuss one possible unifying diagnosis to explain all of patient MF’s radiographic findings.

clinical presentation of polysplenia syndrome
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)

comparison case 4 radiographic findings for polysplenia syndrome
Comparison case #4: Radiographic Findings for Polysplenia Syndrome

Polysplenia

Dilated azygos vein

C- axial CT

Gayer G, et al. Abdom Imaging (1999)

comparison case 5 heterotaxy in polysplenia syndrome
Comparison case #5: Heterotaxy in Polysplenia Syndrome

Liver

Heart

Stomach

C+ axial CT

Gayer G, et al. Abdom Imaging (1999)

our patient mf clinical outcome
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.

take home points
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.
acknowledgements
Acknowledgements
  • Gillian Lieberman, M.D.
  • Maria Levantakis
  • Brian Callahan, M.D.
  • Dan Jones, M.D.
  • Robert Lim, M.D.
references
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 review of the literature”. Abdom Imaging. 1999. 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 of the literature”. Curr Surg. 2003. 60(5): 517-20.
  • 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…

references34
References

6. Taylor HO, Barish M, Soybel D. “Unraveling intestinal malrotation with 3-imensional computer tomography”. Clin Gastroenterol Hepatol. 2006. 4(8): xxix.

7. Lin CJ, Tiu CM, Chou YH, Chen JD, Liang WY, Chang CY. “CT presentation of ruptured appendicitis in an adult with incomplete intestinal malrotation”. Emerg Radiol. 2004. 10(4): 210-2.

8. Tsuda Y, Nishimura K, Kawakami S, Kimura I, Nakano Y, Konishi J. “Preduodenal portal vein and anomalous continuation of inferior vena cava: CT findings”. Journal of Computer Assisted Tomography. 1991. 15(4): 585-588.

9. Pickhardt PJ and Bhalla S. “Intestinal malrotation in adolescents and adults: spectrum of clinical an imaging features.” AJR. 2002. 179: 1429-1435.

9. Moore KL & Dalley AF. Clinical Oriented Anatomy. 4th Edition. 1999.

10. Hill, M. The University of North South Whales. Embryology Project. (http://embryology.med.unsw.edu.au)