1. Dr. Phillip Leung Queen Elizabeth Hospital

2. Case presentation M / 69 Good past health Admitted for increased right lower quadrant pain for 3 days Associated with diarrhea Mild paraumbilical pain for 3 weeks Poor appetite for recent few months

3. Examination BP / P stable Afebrile Tenderness over RLQ with rebound and guarding PR: brownish stool, no mass WCC 10.4, Hb 11.4 Na 133, K 4.8, Cr 69 Albumin 27, LFT otherwise normal Amylase 18 Clotting profile normal

4. Abdominal XR

6. CT scan

7. Impression: Phlebosclerotic colitis

8. Phlebosclerotic colitis First reported cases by Koyama et al in 1989 in Japan; until 2000 Yao et al proposed the name “Phlebosclerotic colitis” Yao T etal (2000) Phlebosclerotic colitis: value of radiography in diagnosis–report of three cases. Radiology 214(1):188–192 An “Asian” disease The only “non - asian” case report is from Canada which is a gentleman who is Taiwanese by birth but a resident in Canada

9. Phlebosclerotic colitis Subtype of ischemic colitis caused by venous disease; in contrast to the commoner arterial cause, namely atherosclerosis, thrombosis and embolism Symptom is caused by chronic venous insufficiency and venous congestion secondary to phlebosclerosis Ischemic changes lead to hemorrhage and atrophy of mucosa, submucosal fibrosis and hence motility dysfunction and stenosis of the affected colon Right hemicolon is affected initially and gradually extends to distal colon Superior mesenteric territories are predominantly affected with the inferior mesenteric territories largely spared, unlike the usual arterial type of ischemic colitis

10. Etiology Cause of phlebosclerosis is unknown 1. Portal hypertension Phlebosclerosis is an adaptive changes to increased venous pressure caused by portal hypertension Kusanagi and Kang reported cases associated with portal hypertension Kusanagi M et al (2005) Phlebosclerotic colitis: imaging-pathologic correlation. AJR Am J Roentgenol 185(2):441–447 Kang et al (2009) Phlebosclerotic colitis in a cirrhotic patient with portal hypertension: Med Sci. 2009 Dec;24(6):1195-9. 2. Alcohol consumption Ho et al. proposed alcohol consumption may play role which is his case report, cessation was associated with reduction in disease severity Ho TJ et al (2005) Phlebosclerotic colitis: an unusual cause of ischemic colitis in a 65-year-old man. J HK Coll Radiol 8:53–58 3. Toxin ingestion Causing longstanding hypoxic injury leading to necrosis of muscular coat of veins as suggested by Chang et al Chang KM et al (2007). New histologic findings in idiopathic mesenteric phlebosclerosis. J Clin Med Assoc 2007; 70: 227–35. 4. Possible related condition includes Diabetes mellitus, hyperlipidemia, cardiac disease, CREST syndrome, Churg-Strauss syndrome and lymphocytic phlebitis Majority of patients have no associated disease and etiology remains unknown

11. Presentation Relatively long period of subclinical stage which is usually irreversible and gradually deteriorative Non – specific clinical manifestation but characteristic pathological, imaging and endoscopic findings An under diagnosed disease Subclinical cases were not detected mostly; real number of patients are much more than known cases

12. Presentation Recurrent non specific symptom Abdominal pain, diarrhea, constipation, nausea and vomiting, per rectal bleeding and tarry stool Acute presentation with complication Ileus Mechanical obstruction Perforation Massive bleeding

13. Presentation Examination usually showed tenderness without peritoneal sign, unless complication arises Blood test is non specific Increased white cell count and C reactive protein Mild increase in amylase Abdominal XR Multiple tortuous thread – like calcifications Small bowel dilation and free gas

14. Imaging Computed tomography Multiple tortuous thread – like calcifications Colonic wall thickening Bowel dilatation or evidence of perforation Yoshikawa K et al (2009) Idiopathic phlebosclerosis: an atypical presentation of ischemic colitis treated by laparoscopic colectomy Surgery. 2009 Jun;145(6):682-4

15. Ba enema Luminal narrowing Thumbprinting Disappearance of semilunar folds Yao T etal (2000) Phlebosclerotic colitis: value of radiography in diagnosis–report of three cases. Radiology 214(1):188–192

16. Endoscopy Dark purple, edematous mucosa Rigid wall Small round ulcers Right side colon is predominantly affected Yoshikawa K et al (2009) Idiopathic phlebosclerosis: an atypical presentation of ischemic colitis treated by laparoscopic colectomy Surgery. 2009 Jun;145(6):682-4

17. Hu P et al Phlebosclerotic colitis: three cases and literature review Abdom Imaging. 2013 Dec;38(6):1220-4 Jan YT et al Phlebosclerotic colitis. J Am Coll Surg. 2008 Nov;207(5):785.

18. Kang et al (2009) Phlebosclerotic colitis in a cirrhotic patient with portal hypertension: Med Sci. 2009 Dec;24(6):1195-9 CT colonoscopy

19. Angiography Kang et al (2009) Phlebosclerotic colitis in a cirrhotic patient with portal hypertension: Med Sci. 2009 Dec;24(6):1195-9

20. Histology Thickened and tortuous submucosal veins with fibrosis and calcified degeneration Atrophic mucosa with hemorrhage Fibrotic submucosa Chronic active inflammation with wall thickening Yoshikawa K et al (2009) Idiopathic phlebosclerosis: an atypical presentation of ischemic colitis treated by laparoscopic colectomy Surgery. 2009 Jun;145(6):682-4

21. Treatment No uniform standard Conservative management was adopted for mild cases in most case report, even in progressive disease Ho TJ et al (2005) Phlebosclerotic colitis: an unusual cause of ischemic colitis in a 65-year-old man. J HK Coll Radiol 8:53–58 Hoshino Y et al (2008) Gastrointestinal: phlebosclerotic colitis. J Gastroenterol Hepatol 23(4):670. Yu CJ et al (2009) Phlebosclerotic colitis with nonsurgical treatment. Int J Colorectal Dis 24(10):1241–1242. Hoshino followed a patient for 5 years which disease progression was noted on CT and endoscopy; patient remained asymptomatic and with no complication Hoshino Y et al (2008) Education and imaging. Gastrointestinal: Phlebosclerotic colitis J Gastroenterol Hepatol. 2008 Apr;23(4):670.

22. Treatment Surgery was suggested in severe disease and complication, e.g. perforation, intestinal obstruction Bowel resection from terminal ileum to sigmoid colon is usually required to removed all diseased bowel Kato et al (2010) Perforated phlebosclerotic colitis--description of a case and review of this condition Colorectal Dis. 2010 Feb;12(2):149-51 Markos V et al (2005) Phlebosclerotic colitis: imaging findings of a rare entity. AJR Am J Roentgenol. 2005 May;184(5):1584-6. (IO)

23. Take home message Phlebosclerotic colitis is a subtype of ischemic colitis Non – specific clinical manifestation but characteristic pathological, imaging and endoscopic findings, as simple as an abdominal XR could suggest the diagnosis Mild disease could be managed conservatively; severe colitis with complication required surgery

24. Case presentation CT report Long segment mural thickening in ascending colon and transverse colon Calcifications along mesenteric veins, mainly in right sided colon Impression: phlebosclerotic colitis 2.8cm rim enhancing collection with small extraluminal gas pocket in close association with caecum; Small 8mm defect noted in caecum Findings suggestive of probable perforation at the caecum with associated collection

25. Progress Emergency laparotomy was performed Caecal inflammatory mass with perforation and abscess formation Bowel gangrene from terminal ileum extending to proximal sigmoid colon Thrombosed calcified vessels at mesentery of gangrenous bowel Fecal peritonitis with gross contamination

26. Progress Subtotal colectomy with exteriorization of small bowel and sigmoid colon performed Post – op uneventful except wound infection Patient’s condition gradually improved and was discharged 3 weeks after operation

27. Question?