Gardner's syndrome Case Study

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  • Suggested Treatment :Total colon removalThey decided to remove the colon ! The countless polyps in the colon predispose to the development of colon cancer; if the colon is not removed, the chance of colon cancer is considered to be very significant.
  • Confirm cuz( it was dx out of KFSH)it was ordered by oncologic to r/o other associated lesions this is bec the criteria of the FAP says that it may have others like desmoids in 10 – 15 percentMap answering a lot of Qs in surgeons head..like is there vessels encasement..if yes they need to close it b4 surgery so no bleeding may occur..also mapping for asociated tumors to know thier site and whether they’re accessible for biopsy and behavior
  • http://withfriendship.com/images/i/44597/vessels-in-the-mesentery.jpg img ref
  • The image on the rt also shows adrenal gland with green dot on it (annotation)
  • http://hal.inria.fr/docs/00/51/68/89/IMG/anatomy.jpg img ref
  • This is differntialdaig.
  • Can be the mass effect causes these symptoms of vomitting
  • progression : ya3ni better? This is the desmoid
  • Number 3 previously
  • Started to mets
  • Desmoid tumors are difficult to be cut out (non resectable)…so at least palliative treatment.
  • necrosis(low density)
  • The distal duodenum appears sandwiched between the the aorta and the SMA casuing significant narrowing. due to the adesions post chemo
  • Img ref http://www.sngbio.com/v2/images_product/small_prod_6.jpg dud: http://www.sciencephoto.com/image/253454/350wm/M1310482-Duodenal_stent-SPL.jpg
  • 1. A catheter was advanced into the distal duodenum.
  • Two overlapping stents were deployed from the proximal jejunum to the distal duodenum. uncovered enteral stentsNotice the suctioning tube last img.. Say: no immediate complications were noted.
  • They thought it can be due to obstruction by food stuck there or stent migration and usually distally due to peristalsis. Or can be due to proliferation after stent.
  • Adenomatous: (precancerous)
  • Air/cm image: http://www.rushradiology.com/web/Sections/GeneralRadiology/GastrointestinalImaging.aspx endoscopy: wikipedia
  • Image ref: http://www.aradnj.com/3D-Virtual-Colonoscopy.html and the other one: http://www.sprucestreetinternalmedicine.com/Medical/vColon.html
  • Imaging of intra- and extraabdominaldesmoid tumors.Casillas J, Sais GJ, Greve JL, Iparraguirre MC, Morillo G.Pubmed articleCurrent trends in the management of extra-abdominal desmoidtumoursPanayiotis J Papagelopoulos1*, Andreas F Mavrogenis1, Evanthia A Mitsiokapa2, KleoTh Papaparaskeva3, Evanthia C Galanis4 and Panayotis N Soucacos1http://www.dtrf.org/dtrf_aboutdesmoids.htm web
  • Gardner's syndrome Case Study

    1. 1. Gardners SyndromeCase StudyShatha J. Al Mushayt
    2. 2. Patient History Male 32 Y/O UpperGI bleeding Anorexia Outsidepathology report Weight loss
    3. 3. Patient history • Multiple polyps all Suggested over the Treatment: colon (*FAP) Colon Removal Colonoscopy Outside * Familial Adenomatosis Polyposispathology report
    4. 4. C+ CAP CTwas ordered@ KFSH
    5. 5. CAP CT Why? To confirm FAP. To r/o associated tumors (FAP criteria).
    6. 6. C+ CAP CTMany polyps are shownas filling defects FAP is confirmed
    7. 7. ELSE?WHAT
    8. 8. C+ CAP CT shows:Multiple soft tissue mesentericmasses.Ill-defined,Infiltrative Mesentery& heterogeneous >> images
    9. 9. 1. The largest is in the RT mid abdomen
    10. 10. 2. In LT upper abdomen
    11. 11. 3. Upper mass along the proximal SMVs
    12. 12. 4. in LT lower abdomen, lobulated mass
    13. 13. posterior RT abdominal wall lower posterior LT chest wallSheath-like soft tissue enhancement in thesubcutaneous fat.
    14. 14. C+ CAP CTNo small bowel obstruction.Patent SMVs.
    15. 15. Mesenteric & subcutaneous masses Differential diagnosis Lesspossible Lastly Likely Biopsy >>
    16. 16. 1. Biopsy of the mesenteric tumors Benign fibrous proliferation, suggestive offibromatosisi.e. Desmoid tumorsGardner’s syndrome is confirmed.
    17. 17. Then.. ProctoColectomy-ve Pre op CXR
    18. 18. After Proctocolectomy..Abdominal Nausea& flank pain & vomiting Mild distension Abd x-ray
    19. 19. Abdomen X-raywas orderedSTAT
    20. 20. ABDOMEN X-RAY standing
    21. 21. ABDOMEN X-RAY report Other doctorsFew mildly dilated Considered“small” bowel normal(no pathologicsegments with dilatation)air/fluid levels An early obstruction cannot be ruled out. CT
    22. 22. C+ CT of Abd. &pelvisSTATSame day To r/o small bowel obstruction.
    23. 23. C+ CT abd. & pelvisNo bowel obstruction or ischemia.No free air or loculated collections.
    24. 24. Progression of the mesenteric mass
    25. 25. Increase of the soft tissue encasing the SMvein w/ compression & engorgement of thedistal mesenteric veins
    26. 26. Newly developed soft tissue mesentericmass along the LT common iliac vessel.Desmoids havemetastasized. Chemo
    27. 27. Chemotherapy For desmoid tumors palliative care CT
    28. 28. C+ CAP CTTo assess response after chemotherapy.
    29. 29. C+ CAP CT Result:  No response to chemotherapy (desmoids were unchanged in size).
    30. 30. C+ CAP CTResult cont. a very tiny hypodense noduleseen in the LT thyroid lobe.
    31. 31. Significant narrowing of the duodenum(due to the very adjacent desmoid tumor)
    32. 32. dilatation of duodenum proximal partparitial obstruction of distal part Stenting
    33. 33. Gastric StentingTo relieve obstruction Duodenul stent
    34. 34. Stenting 11. A guided catheter was advanced to the area ofthe stenosis at duodenal/jejunal flexure; Stenting 2
    35. 35. Stenting 12. After several attempts, they could not cross the stenotic area. Stenting 2
    36. 36. Stenting 1the procedure was terminated ! Stenting 2
    37. 37. Stenting 2 after14 d1. Injection of contrast revealed very tight stricture in the proximal jejunum.2. the catheter stopped due to recoil in the stomach and could not cross into the jejunum.
    38. 38. Stenting 2The procedure was abandoned foran attempt with endoscopic help. gastro scopy
    39. 39. Gastroscopy after 2 dEndoscopic crossing of the tumor wasattempted and was unsuccessful. gastro stomy
    40. 40. Gastrostomy & stenting same dayCrossing of the Deploying of two overlapping proximal jejunal stents disease
    41. 41. After stentingAbdominal Vomiting pain Abd x-ray
    42. 42. Acute seriesAbdomen X-raySTAT r/o obstruction
    43. 43. Negative acute series Abd. X-ray CT
    44. 44. C+ CT OF Abd.& pelvisSTAT1 day later r/o obstruction
    45. 45. CT Good stenting No obstruction but mild dilatation proximal to the stenting. Otherwise, no change from previous CT.
    46. 46. WHAT’S NEXT?
    47. 47. Patient follow up Stable Well-looking For follow upand palliative care.
    48. 48. To be done.. Gastrostomy tube removal
    49. 49. About The PathologyOutline: Familial Adenomatosis Polyposis (FAP) Desmoids Gardner’s Syndrome
    50. 50. Familial Adenomatosis Polyposis An inherited condition caused by a mutation in a gene. Characterized by the formation of hundreds to thousands of colon polyps.
    51. 51. Desmoids Tendonlike tumors of the connective tissues Associated with FAP in 5-10 % Benign, rarely metastasize; but can be locally aggressive & invasive to surrounding tissues  difficult to be cut out.
    52. 52. Gardners syndrome A subtype of FAP. Characterized by: Multiple colon polyps + tumors outside the colon. The extracolonic tumors may include: • Desmoid tumors • Bone & soft tissue tumors.
    53. 53. Comparative Imaging of FAP Colonoscopy The diagnostic test of choice(quantification & histology). Endoscopic image of sigmoid colon of patient with FAP. Air/contrast Barium Enema  Detect larger colonic polyps but can miss smaller ones. Air/contrast barium enema
    54. 54. Comparative Imaging of FAP Virtual colonoscopy (by CT or MRI)  Detect >80% of large polyps;  Is beginning to be done for screening outside research settings. CT
    55. 55. Desmoids imaging No specific imaging features to distinguish desmoids from other masses. ( Biopsy is always needed). CT & MRI are the most useful modalities for size & extent. US: initially for superficial tumors involving the abdominal wall.
    56. 56. Desmoids imaging CT: variable intensity & margin.  If C+ usually enhanced; but may not. MRI: variable signal intensity on T1 & T2. US: variable echogenesity & margin.
    57. 57. Golden Standard modality for this caseCT: for size and extent of desmoids. Confirming FAP. Colonoscopy.
    58. 58. “I’m a great believer in luck, and I find the harder I work the more I have of it”. Thomas Jefferson THANK YOUPresentation is over !

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