Accepted ArticleSegmental colitis caused by idiopathic myointimalhyperplasia of mesenteric veins

Mariana N. Costa, Joana Saiote, Maria José Pinheiro, PedroDuarte, Teresa Bentes, Mário Ferraz Oliveira, Jaime Ramos

DOI: 10.17235/reed.2016.4051/2015Link: PDF

Please cite this article as: Costa Mariana N., Saiote Joana,Pinheiro Maria José, Duarte Pedro, Bentes Teresa, FerrazOliveira Mário, Ramos Jaime. Segmental colitis caused byidiopathic myointimal hyperplasia of mesenteric veins. RevEsp Enferm Dig 2016. doi: 10.17235/reed.2016.4051/2015.

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NC 4051 inglés

Segmental colitis caused by idiopathic myointimal hyperplasia of mesenteric veins

Mariana N. Costa1, Joana Saiote1, Maria José Pinheiro2, Pedro Duarte1, Teresa Bentes1,

Mário Ferraz-Oliveira3 and Jaime Ramos1

Departments of 1Gastroenterology, 2Surgery and 3Pathology. Centro Hospitalar Lisboa

Central. Lisbon, Portugal

Received: 24/10/15

Accepted: 28/10/15

Correspondence: Mariana Nuno Costa. Department of Gastroenterology. Alameda de

Sto. António dos Capuchos. 1169-050 Lisbon, Portugal

e-mail: mariananunocosta@gmail.com

ABSTRACT

Diseases causing colonic ischemia may be mistaken with other causes of segmental

colitis such as inflammatory bowel disease, especially in young patients. The authors

present the case of a 47-year-old male with severe proctosigmoiditis. Assessment

excluded infectious causes, thrombophilia and systemic vasculitis. The initial

histological specimen was suggestive of inflammatory bowel disease and therapy was

initiated with intravenous steroids and, at day 5, infliximab, with no response. The

patient was proposed for surgery. Pathological examination of the surgical specimen

revealed an idiopathic myointimal hyperplasia of mesenteric veins, a rare entity

exhibiting necrotizing phlebitis with rapid progression to segmental necrosis in the

rectosigmoid colon. In this paper the authors discuss the differential diagnosis of

proctosigmoiditis in young ages and the approach to this exceptionally rare ischemic

entity.

Key words: Ischemic colitis. Idiopathic myointimal hyperplasia of mesenteric veins.

INTRODUCTION

Diseases causing colonic ischemia may have similar presentation symptoms to other

causes of segmental colitis such as inflammatory bowel disease, especially in young

patients. Idiopathic myointimal hyperplasia of mesenteric veins (IMHMV) is a rare

condition causing segmental colonic ischemia that should be considered in the

differential diagnosis of severe colonic inflammatory bowel disease refractory to

intensive medical treatment. Preoperative diagnosis of IMHMV could be difficult as

intimal thickening venules are in the submucosa and deeper layers. Even when a full-

thickness biopsy is performed the diagnosis may be inconclusive. Standard treatment

is surgical resection and there are no reports of postoperative disease recurrence. The

following case illustrates this rare clinical condition in a young adult male patient.

CASE REPORT

A 47-year-old Caucasian male patient was admitted to our medical department on

February 2012 with bloody diarrhea (more than 20 stools per day), lower abdominal

cramping pain, proctalgia and malaise.

The patient reported a previous 9 month history of hypogastric cramp-like pain, 4-5

small volume stools, without blood, mucus or pus, as well as a compelling urge to

defecate and fecal incontinence. One month before, the patient had been assessed on

the emergency department of another hospital due to clinical deterioration with anal

pain, persistent urge to defecate, tenesmus and straining at stool. There, he had

undergone a colonoscopy with biopsy and a pelvic CT scan. The colonoscopy showed

edema of the rectal mucosa and sigmoid colon. The biopsies did not reveal any

significant alterations. The pelvic CT scan identified parietal thickening of the rectum.

At that time, the patient was treated with an antispasmodic. No improvement was

observed.

Patient’s personal and family histories were irrelevant and epidemiological context

was unremarkable. He had not been undergoing any other course of medication and

denied known allergies, smoking, alcohol and history of injected or inhaled drug use.

Upon physical examination, he was afebrile with lower abdominal tenderness with no

signs of peritoneal irritation.

Routine blood analysis revealed normal hemoglobin (13.8 g/dL), no leukocytosis nor

neutrophilia with a slightly raised level of C-reactive protein (6.3 mg/dL; normal level: <

5 mg/dL). Infectious causes were excluded.

Radiologically, there was a marked diffuse parietal thickening (13 mm) of the rectum

and sigmoid colon, peri-colic fat densification with some small ganglion formations and

mild vascular ectasia. There was no intra peritoneal free fluid (Fig. 1).

The rectosigmoidoscopy showed diffuse edema of the rectal mucosa with sudden

transition to a circumferential and continuous mucosal ulceration and luminal stenosis

of the sigmoid colon. Histologic examination showed mucosal edema and vascular

congestion without significant glandular lesions, with mild inflammatory infiltration

and fragments of base of ulcer without pericriptitis, crypt abscesses or granulomas.

Intravenous methylprednisolone (40 mg daily) was initiated. There was no

improvement in the clinical course after 5 days of intravenous steroids, so treatment

with intravenous 400 mg infliximab (5 mg/kg) was started.

Colonoscopy was repeated to evaluate the disease extent and severity, revealing a

granular edematous mucosa in the proximal rectum, circumferential necrotic

ulceration extending from 10 to 30 cm from the anal verge and a nodular mucosa

followed by a well-defined transition to normal mucosa at this level. Above the

ulcerated area, the colon mucosa was normal (Fig. 2). Biopsy samples were taken.

Histologic evaluation showed edema and hemorrhage of the chorion, focal ischemic

type changes and several small vessels with fibrinoid necrosis and lumen thrombosis,

aspects suggestive of ischemic etiology (Fig. 3).

Autoimmunity, systemic vasculitis and thrombophilia tests were negative.

The patient was proposed for surgery and a Hartmann procedure was performed. The

macroscopic examination of the surgical specimen showed an extensive ulcerated area

13 cm long (Fig. 4). On histological examination of the ulcerated region granulation

tissue was observed, which reached the submucosa with neovascular proliferation and

extensive lesions of fibrinoid necrosis and vasculitis, thrombosis and nerve hyperplasia.

On the adventitia, several vessels with intimal hyperplasia conditioning luminal

stenosis were seen. The lymph nodes showed sinus histiocytosis and ectasia. The

mesorectal adipose tissue had lesions with steatonecrosis. The histological aspects

were consistent with IMHMV (Fig. 5).

DISCUSSION

A significant diagnostic challenge is raised by segmental colitis because of its broad

differential diagnosis. Many diseases such as diverticulitis, radiation colitis, mucosal

prolapsed/solitary ulcer syndrome, infectious diseases, colon carcinoma, inflammatory

bowel disease and colonic ischemia may present a segmental colonic involvement.

Colonic ischemia may result from various etiologies but it can be broadly classified as

non-occlusive and occlusive. Venous occlusion is an uncommon cause of ischemic

bowel disease, and the majority of such cases are caused by venous thrombosis (1,2).

Non-thrombotic occlusion of the mesenteric veins is rare and has previously been

described in vasculitis associated with systemic lupus erythematosus, Behçet’s disease,

enterocolic lymphocytic phlebitis and IMHMV (3,4).

Colonic ischemia is more common in the elderly but younger patients may also be

affected. In these patients, segmental ischemic colitis is a rare differential diagnosis of

inflammatory bowel disease (5) and it results of a hypoxic tissue injury with secondary

necrosis and inflammation (6). It may present a spectrum of severity ranging from

mild, transient mucosal erosion to fibrous scarring with stricture formation or even

transmural infarction (7).

IMHMV is a rare entity exhibiting necrotizing phlebitis with rapid progression to

segmental necrosis in the rectosigmoid colon. The precise incidence of IMHMV is

unknown, as only 21 cases have been reported in the literature to date (Table I). This

disease is known to occur predominantly in young, healthy men, with a sudden onset

of crampy abdominal pain accompanying repeated episodes of diarrhea and bloody

stools (4,8). The pathophysiologic mechanism seems to be an abnormal arteriovenous

shunting with elevated venous pressure and consecutive congestion and ischemia of

the involved bowel segment (3). IMHMV diagnosis can be based on clinical

manifestations causing acute, progressive ulcers in the rectosigmoid colon and

pathohistologic findings on the resected specimen. A biopsy is generally not helpful for

diagnostic purposes since specimens that include concentric intimal thickening venules

in the submucosa and deeper layers are often not obtained (9). Pathohistologic

examination of a resected specimen reveals a striking pattern of scattered

circumferential intimal thickening of vessels, with or without lymphocyte infiltration

around venules and complete obliteration of venules from the submucosa to

subserosa (4). Regarding treatment, complete resection of the necrotic segments is

recommended. The postoperative clinical prognosis is generally good, with no

recurrence (4).

Our patient is a typical case of this extremely rare condition. The results of the initial

biopsy revealed nonspecific changes in the intestinal mucosa. As with other cases of

IMHMV, medical therapies were ineffective. Given that subsequent biopsies suggested

a possible ischemic etiology, our patient underwent a surgical resection. The

histological examination of the resected specimen established the diagnosis of

IMHMV. Overall, this case presents an entity that is difficult to diagnose since both the

endoscopic and biopsy appearance are nonspecific and only the surgical specimen

provides the diagnosis.

REFERENCES

1. Grendell JH, Ockner RK. Mesenteric venous thrombosis. Gastroenterology

1982;82:358-72.

2. Hunter GC, Guernsey JM. Mesenteric ischemia. Med Clin North Am

1988;72:1091-115.

3. Abu-Alfa AK, Ayer U, West AB. Mucosal biopsy findings and venous

abnormalities in idiopathic myointimal hyperplasia of the mesenteric veins. Am J Surg

Pathol 1996;20:1271-8. DOI: 10.1097/00000478-199610000-00014

4. Genta RM, Haggitt RC. Idiopathic myointimal hyperplasia of mesenteric veins.

Gastroenterology 1991;101:533-9.

5. Brandt LJ, Boley SJ. AGA technical review on intestinal ischemia. American

Gastrointestinal Association. Gastroenterology 2000;118:954-68.

6. Feuerstadt P, Brandt LJ. Colon ischemia: Recent insights and advances. Curr

Gastroenterol Rep 2010;12:383-90. DOI: 10.1007/s11894-010-0127-y

7. Hwang S-S, Chung W-C, Lee K-M, et al. Ischemic colitis due to obstruction of

mesenteric and splenic veins: A case report. World J Gastroenterol 2008;14:2272-6.

DOI: 10.3748/wjg.14.2272

8. Kao PC, Vecchio JA, Hyman NH, et al. Idiopathic myointimal hyperplasia of

mesenteric veins: A rare mimic of idiopathic inflammatory bowel disease. J Clin

Gastroenterol 2005;39:704-8. DOI: 10.1097/00004836-200509000-00011

9. Mizoshita T, Tanida S, Joh T. A case of punched-out ulcer occurring in the

rectosigmoid colon with sudden onset of bloody stools. Gastroenterology 2011;141:e9-

10. DOI: 10.1053/j.gastro.2010.05.092

10. Savoie LM, Abrams A V. Refractory proctosigmoiditis caused by myointimal

hyperplasia of mesenteric veins: Report of a case. Dis Colon Rectum 1999;42:1093-6.

DOI: 10.1007/BF02236711

11. Lavu K, Minocha A. Mesenteric inflammatory veno-occlusive disorder: A rare

entity mimicking inflammatory bowel disorder. Gastroenterology 2003;125:236-9. DOI:

10.1016/S0016-5085(03)00663-2

12. García-Castellanos R, López R, De Vega VM, et al. Idiopathic myointimal

hyperplasia of mesenteric veins and pneumatosis intestinalis: A previously unreported

association. J Crohns Colitis 2011;5:239-44. DOI: 10.1016/j.crohns.2010.12.003

13. Chiang C-K, Lee C-L, Huang C-S, et al. A rare cause of ischemic proctosigmoiditis:

Idiopathic myointimal hyperplasia of mesenteric veins. Endoscopy

2012;44(Suppl2):E54-5. DOI: 10.1055/s-0031-1291529

14. Feo L, Cheeyandira A, Schaffzin DM. Idiopathic myointimal hyperplasia of

mesenteric veins in the elderly. Int J Colorectal Dis 2013;28:433-4. DOI:

10.1007/s00384-012-1480-0

15. Korenblit J, Burkart A, Frankel R, et al. Refractory pancolitis: A novel

presentation of idiopathic myointimal hyperplasia of mesenteric veins. Gastroenterol

Hepatol 2012;8:696-700.

16. Thomas BS. Myointimal hyperplasia of the mesenteric veins mimicking

infectious colitis. Int J Colorectal Dis 2013;28:727. DOI: 10.1007/s00384-012-1522-7

17. Lanitis S, Kontovounisios C, Karaliotas C. An extremely rare small bowel lesion

associated with refractory ascites. Idiopathic myointimal hyperplasia of mesenteric

veins of the small bowel associated with appendiceal mucocoele and pseudomyxoma

peritonei. Gastroenterology 2012;142:e5-7. DOI: 10.1053/j.gastro.2011.11.052

18. Wangensteen KJ, Fogt F, Kann BR, et al. Idiopathic myointimal hyperplasia of

the mesenteric veins diagnosed preoperatively. J Clin Gastroenterol 2015;49:491-4.

DOI: 10.1097/MCG.0000000000000290

19. Sahara K, Yamada R, Fujiwara T, et al. Idiopathic myointimal hyperplasia of

mesenteric veins: Rare case of ischemic colitis mimicking inflammatory bowel disease.

Dig Endosc 2015;27:767-70. DOI: 10.1111/den.12470.

20. Abbott S, Hewett P, Cooper J, et al. Idiopathic myointimal hyperplasia of the

mesenteric veins: A rare differential to be considered in idiopathic colitis. ANZ J Surg

2015 Jul 3. [E-pub ahead of print] DOI: 10.1111/ans.13210.

21. Laskaratos F-M, Hamilton M, Novelli M, et al. A rare cause of abdominal pain,

diarrhoea and GI bleeding. Gut 2015;64:214. DOI: 10.1136/gutjnl-2014-308319

22. Zijlstra M, Tjhie-Wensing JW, Van Dijk M, et al. Idiopathic myointimal

hyperplasia of mesenteric veins: An unusual cause of diarrhoea. Ned Tijdschr

Geneeskd 2014;158:A7752.

Table I. Reported cases of IMHMV to date

Age/Ge

nder

Presenting

symptoms

Site Clinical

diagnosis

Surgical

indication

Time to

surgery

Reference

1 30/M Abdominal pain/

hematochezia

Sigmoid Stricture Bowel

obstruction

1 mo (4)

2 38/M Abdominal pain/

hematochezia/diarr

hea/

constipation

Sigmoid Ulcerative

colitis

Toxic

megacolon

2 mo (4)

3 25/M Abdominal pain/

hematochezia/diarr

hea/

constipation

Rectosigmoid Ulcerative

colitis

Perforation > 6 mo (4)

4 67/M Abdominal

pain/diarrhea/

constipation

Sigmoid Crohn’s

disease

Bleeding,

severe pain

3 mo (4)

5 58/M Abdominal pain/

hematochezia/diarr

hea

Sigmoid IBD

ischemia

Bleeding > 1 y (3)

6 22/M Hematochezia/diar

rhea

Rectosigmoid Ulcerative

colitis

Bleeding NA (10)

7 38/M Abdominal pain/

hematochezia/

constipation

Rectosigmoid IBD Perforation 5 mo (8)

8 54/F Abdominal pain/

hematochezia/diarr

hea

Sigmoid IBD Severe pain 15 mo (11)

9 32/F Abdominal pain/

hematochezia/diarr

hea

Sigmoid Primary

pneumatos

is

intestinalis

Severe pain

and

bleeding

3 mo (12)

10 60/M Abdominal pain/

hematochezia/diarr

hea

Rectosigmoid Ulcerative

colitis

Pain and

bleeding

1 mo (13)

11 75/F Abdominal pain/

hematochezia/diarr

hea

Rectum Inflammato

ry

mass

Severe pain

and

bleeding

NA (14)

12 62/M Abdominal

pain/hematochezia

/diarrhea

Whole colon Ulcerative

colitis

Bleeding 18 mo (15)

13 62/M Hematochezia/diar

rhea

Rectosigmoid Infectious

colitis

Severe pain 4 wk (16)

14 81/M Abdominal

pain/ascitis/weight

loss

Terminal

ileum

NA NA 6 mo (17)

15 62/M Abdominal

pain/hematochezia

/diarrhea

Whole colon,

rectum spared

Ulcerative

colitis

Symptoms

refractor to

medical

manageme

nt

10 mo (15)

16 59/M Abdominal

pain/constipation/i

ncontinence

Rectosigmoid Colitis Symptoms

refractor to

medical

manageme

nt

1 mo (15)

17 62/F Abdominal pain/

hematochezia/diarr

hea

Rectosigmoid Idiopathic

myointimal

hyperplasia

Pain and

lack of

healing

2 mo (18)

18 76/M Diarrhea/abdomina

l pain/weight loss

RS IBD,

ischemia

Severe pain 12 mo (19)

19 58/M Abdominal pain/

hematochezia/diarr

hea

Descendent

colon,

rectosigmoid

IBD,

ischemia

Symptoms

refractor to

medical

manageme

nt

NA (20)

20 62/F Abdominal Terminal Ileitis Small NA (21)

pain/diarrhea ileum bowel

perforation

21 62/M Diarrhea Left-sided

colon

Ischemia Acute

abdomen

NA (22)

22 47/M Diarrhea/abdomina

l pain

Rectosigmoid IBD,

ischemia

Symptoms

refractor to

medical

manageme

nt

9 mo Present case

M: Male; F: Female; mo: Months; IBD: Inflammatory bowel disease.

Fig. 1. Diffuse parietal thickening of the sigmoid colon (A) and mild vascular ectasia (B)

were seen on pelvic CT scan.

Fig. 2. Colonoscopy showed circumferential ulceration extending from 10 to 30 cm of

the anal verge (A and B). Upstream there were feces with no blood and an underlying

normal mucosa (C).

Fig. 3. Histologic examination showed hemorrhage of the chorion, focal ischemic type

changes and several small vessels with fibrinoid necrosis and lumen thrombosis.

Fig. 4. Resected colon with a 13 cm long ulcerated area.

Fig. 5. Histologic evaluation showed myointimal hyperplasia of the veins (thin arrows);

arteries were unaffected (large arrows).