a case of incomplete intestinal malrotation malrotation in...
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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.
4. 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.
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.
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)