A Case of Incomplete Intestinal A Case of Incomplete Intestinal MalrotationMalrotation in an Adultin an Adult

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

Beth Israel Deaconess Medical CenterBeth Israel Deaconess Medical CenterHarvard Medical SchoolHarvard Medical School

March 20, 2009 March 20, 2009

Talk OutlineTalk Outline

IntroductionIntroduction: clinical case: clinical case

BackgroundBackground: gastrointestinal : gastrointestinal malrotationmalrotation

ReRe--examinationexamination:: secondary radiographic secondary radiographic findingsfindings

Potential diagnosisPotential diagnosis: : malrotationmalrotation--associated associated heterotaxyheterotaxy

Take home pointsTake home points

Clinical Presentation: Clinical Presentation: 2828--yearyear--old female with abdominal painold female with abdominal pain

Several months durationSeveral months duration

Nausea, vague abdominal pain and bloatingNausea, vague abdominal pain and bloating

Symptoms not associated with food intakeSymptoms not associated with food intake

Normal bowel movements; otherwise healthyNormal bowel movements; otherwise healthy

? Acute appendicitis? Acute appendicitis

Imaging studies:Imaging studies:1.1. Abdominal/pelvic CTAbdominal/pelvic CT2.2. Upper GI with barium and small bowel followUpper GI with barium and small bowel follow--

throughthrough

Abdominal CT FindingsAbdominal CT Findings

PACS, BIDMC

Colon

Small bowel

Incomplete Intestinal Malrotation

PACS, BIDMC

UGI with air & SBFT

Decreased duodenal sweep

PACS, BIDMC

PACS, BIDMC

Normal location: ileocecal junction

UGI with air & SBFT

PACS, BIDMC

PACS, BIDMC

Embryonic Embryonic MidgutMidgut RotationRotation

Moore KL & Dalley AF (1999)

Developmental complications: omphalocele, rotational abnormalities, midgut volvulus

Findings for Intestinal Findings for Intestinal MalrotationMalrotation

Burk MS, et al. Am J Surg (2008)Hill, M. UNSW Embryology. http://embryology.med.unsw.edu.au/

Surgical treatment: Ladd’s procedure

Findings for Intestinal Findings for Intestinal MalrotationMalrotationInversion of SMA (a) SMV (b)

Gamblin TC, et al. Current Surgery (2003)

Matzke GM, et al. Surg Endosc (2005)

“Whirlpool sign”

Surgical Treatment of Incomplete Malrotation

• Removal of band between ascending/ descending colon

Appendectomy

PACS, BIDMC

Incidental Finding #1: Incidental Finding #1: PolyspleniaPolysplenia

PACS, BIDMC

PACS, BIDMC

Incidental Finding #2: Duplicated IVCIncidental Finding #2: Duplicated IVCPACS, BIDMC

PACS, BIDMC

PACS, BIDMC

A

B

A

B

PolyspleniaPolysplenia syndromesyndrome

Abdominal painAbdominal pain

PolyspleniaPolysplenia

HeterotaxyHeterotaxy (stomach, liver, heart)(stomach, liver, heart)

Short pancreasShort pancreas

Intestinal Intestinal malrotationmalrotation

IVC abnormalitiesIVC abnormalities

Azygos/hemizygosAzygos/hemizygos continuationcontinuation

PreduodenalPreduodenal portal veinportal vein

SitusSitus ambiguous/ambiguous/inversusinversus

= PatientGayer G, et al. Abdom Imaging (1999)

Findings for Findings for PolyspleniaPolysplenia SyndromeSyndrome

Gayer G, et al. Abdom Imaging (1999)

Polysplenia, dilated azygos veinHeterotaxy

Gayer G, et al. Abdom Imaging (1999)

Molecular Basis of LeftMolecular Basis of Left--Right Body Axis Right Body Axis PatterningPatterning

Nonaka S, et al. Nature (2002)

Take Home PointsTake Home Points

Intestinal Intestinal malrotationmalrotation should be considered in should be considered in adults with vague abdominal symptomsadults with vague abdominal symptoms

Accurate radiographic diagnosis of intestinal Accurate radiographic diagnosis of intestinal malrotationmalrotation can prevent unnecessary can prevent unnecessary complications and/or surgeriescomplications and/or surgeries

PolyspleniaPolysplenia, IVC abnormality, intestinal , IVC abnormality, intestinal malrotationmalrotation, and cardiac abnormalities can be , and cardiac abnormalities can be syndromicsyndromic in asymptomatic patients. These in asymptomatic patients. These findings may have clinical significance in the findings may have clinical significance in the future.future.

AcknowledgementsAcknowledgements

• Gillian Lieberman, M.D.

• Brian Callahan, M.D.

• Robert Lim, M.D.

Thank you for your attention!

ReferencesReferences

1. 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.

2. 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.

3. 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.

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5. 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…

ReferencesReferences6. Taylor HO, Barish M, Soybel D. “Unraveling intestinal malrotation with 3-

imensional computer tomography”. Clin Gastroenterol Hepatol. 2006. 4(8): xxix.

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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.

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