一例成人不完全性肠旋转不良影像学表现

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 Center Harvard 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 CT 2.2. 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 MalrotationMalrotation Inversion 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 IVC PACS, 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 = Patient Gayer G, et al. Abdom Imaging (1999) Findings for Findings for PolyspleniaPolysplenia SyndromeSyndrome Gayer G, et al. Abdom Imaging (1999) Polysplenia, dilated azygos vein Heterotaxy 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… ReferencesReferences 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) A Case of Incomplete Intestinal Malrotation in an Adult Talk Outline Clinical Presentation: �28-year-old female with abdominal pain Slide Number 4 Slide Number 5 Slide Number 6 Slide Number 7 Embryonic Midgut Rotation Findings for Intestinal Malrotation Findings for Intestinal Malrotation Slide Number 11 Incidental Finding #1: Polysplenia Incidental Finding #2: Duplicated IVC Polysplenia syndrome Findings for Polysplenia Syndrome Molecular Basis of Left-Right Body Axis Patterning Take Home Points Acknowledgements References References