20-28 owm0807 matthew-kabeer. kabeer is a research registrar, department of histopathology; dr....

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20 Ostomy Wound Management FEATURE S tomal bleeding is usually caused by trauma, 1,2 skin excoriation, and stomal ulceration 2 and typically controlled by local measures that include local pressure, silver nitrate application, injection sclerother- apy, and suture ligation of the bleeding point. 2-6 Hemorrhage from stomal varices is a rare but potential- ly fatal cause of stomal bleeds. To increase clinician awareness about this complication, three recent cases involving patients with recurrent stomal bleeding sec- ondary to stomal varices are described and manage- ment strategies are discussed. Literature Review Physiology. Variceal development at sites of por- tosystemic anastomoses is usually associated with por- tal hypertension. 5,7 Varices typically develop at the gas- tro-esophageal junction. Varices elsewhere (ectopic varices) can develop anywhere along the entire gastro- intestinal tract 8 ; they have been reported at unusual sites such as the gallbladder, urinary bladder, uterus, 9 vagina, umbilicus, and retroperitoneum. 10 Ileostomies, colostomies, and ileal conduits 11-14 provide additional sites for the formation of what are called stomal varices. 8 Stomal Varices: A Rare Cause of Stomal Hemorrhage. A Report of Three Cases Muhammad A. Kabeer, MRCS; Lucina Jackson, MB, MRCPI, PhD; Adam L. Widdison, DM, FRCS, PGCE; Giles Maskell, MA, MRCP, FRCR; and Joseph Mathew, FMCPath, FRCPath Stomal varices secondary to portal hypertension are a rare but potentially fatal cause of hemorrhage. Management, determined by the site of the bleeding, centers on preventing additional bleeds and may include providing local pressure, applying silver nitrate, injection sclerotherapy, suture ligation of the bleeding point, and/or the placement of transjugular intrahepatic portosystemic shunts and refashioning the stoma. Two patients (60- and 69-year-old women) had panproctocolectomy for inflammatory bowel disease and presented at the authors’ hospital with bleeding from the ileostomy 1 and 19 years, respectively, following the creation of their stomas. A third patient (a 72-year-old man) bled from an end colostomy following an abdominoperineal resection for Duke’s C rectal adeno- carcinoma performed 3 years previous. All three patients had recurrent admissions for stomal bleeding and stomal varices second- ary to portal hypertension and were initially treated with local measures (pressure, silver nitrate, and suture ligation). Two had under- gone revision of their stomas prior to current treatment. One patient responded to local treatment but later died due to liver failure, one stopped bleeding after transjugular portosystemic shunt placement, and one died from metastatic cancer. Clinicians should main- tain a high index of suspicion of stomal varices in patients with underlying liver disease who present with recurrent stomal bleeds and provide appropriate treatment to stop active bleeding and reduce portal venous pressure. KEYWORDS: stomas, case study, portosystemic shunts, gastrointestinal hemorrhage, portal hypertension Ostomy Wound Management 2007;53(8):20–28 Mr. Kabeer is a Research Registrar, Department of Histopathology; Dr. Jackson is a Consultant Gastroenterologist, Department of Medicine; Mr. Widdison is Consultant, Department of Surgery; Dr Maskell is a Consultant, Department of Clinical Imaging; and Dr. Mathew is Consultant, Department of Histopathology, Royal Cornwall Hospital, Truro, UK. Please address correspondence to: Dr. Joseph Mathew, Department of Histopathology, Royal Cornwall Hospital, Treliske, Truro, Cornwall, TR1 3LJ, UK; email: [email protected]. DO NOT DUPLICATE

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Page 1: 20-28 OWM0807 Matthew-Kabeer. Kabeer is a Research Registrar, Department of Histopathology; Dr. Jackson is a Consultant Gastroenterologist, Department of Medicine; Mr.Widdison is Consultant,

20 OstomyWound Management

FEATURE

Stomal bleeding is usually caused by trauma,1,2 skin

excoriation, and stomal ulceration2 and typically

controlled by local measures that include local

pressure, silver nitrate application, injection sclerother-

apy, and suture ligation of the bleeding point.2-6

Hemorrhage from stomal varices is a rare but potential-

ly fatal cause of stomal bleeds. To increase clinician

awareness about this complication, three recent cases

involving patients with recurrent stomal bleeding sec-

ondary to stomal varices are described and manage-

ment strategies are discussed.

Literature ReviewPhysiology. Variceal development at sites of por-

tosystemic anastomoses is usually associated with por-

tal hypertension.5,7 Varices typically develop at the gas-

tro-esophageal junction. Varices elsewhere (ectopic

varices) can develop anywhere along the entire gastro-

intestinal tract8; they have been reported at unusual

sites such as the gallbladder, urinary bladder, uterus,9

vagina, umbilicus, and retroperitoneum.10 Ileostomies,

colostomies, and ileal conduits11-14 provide additional

sites for the formation of what are called stomal varices.8

Stomal Varices: A Rare Cause ofStomal Hemorrhage.A Report of Three Cases Muhammad A. Kabeer, MRCS; Lucina Jackson, MB, MRCPI, PhD; Adam L. Widdison, DM, FRCS, PGCE; GilesMaskell, MA, MRCP, FRCR; and Joseph Mathew, FMCPath, FRCPath

Stomal varices secondary to portal hypertension are a rare but potentially fatal cause of hemorrhage. Management, determined bythe site of the bleeding, centers on preventing additional bleeds and may include providing local pressure, applying silver nitrate,injection sclerotherapy, suture ligation of the bleeding point, and/or the placement of transjugular intrahepatic portosystemic shuntsand refashioning the stoma.Two patients (60- and 69-year-old women) had panproctocolectomy for inflammatory bowel disease andpresented at the authors’ hospital with bleeding from the ileostomy 1 and 19 years, respectively, following the creation of their stomas.A third patient (a 72-year-old man) bled from an end colostomy following an abdominoperineal resection for Duke’s C rectal adeno-carcinoma performed 3 years previous. All three patients had recurrent admissions for stomal bleeding and stomal varices second-ary to portal hypertension and were initially treated with local measures (pressure, silver nitrate, and suture ligation). Two had under-gone revision of their stomas prior to current treatment. One patient responded to local treatment but later died due to liver failure,one stopped bleeding after transjugular portosystemic shunt placement, and one died from metastatic cancer. Clinicians should main-tain a high index of suspicion of stomal varices in patients with underlying liver disease who present with recurrent stomal bleeds andprovide appropriate treatment to stop active bleeding and reduce portal venous pressure.

KEYWORDS: stomas, case study, portosystemic shunts, gastrointestinal hemorrhage, portal hypertension

Ostomy Wound Management 2007;53(8):20–28

Mr. Kabeer is a Research Registrar, Department of Histopathology; Dr. Jackson is a Consultant Gastroenterologist, Department ofMedicine; Mr. Widdison is Consultant, Department of Surgery; Dr Maskell is a Consultant, Department of Clinical Imaging; and Dr.Mathew is Consultant, Department of Histopathology, Royal Cornwall Hospital, Truro, UK. Please address correspondence to: Dr.Joseph Mathew, Department of Histopathology, Royal Cornwall Hospital, Treliske, Truro, Cornwall, TR1 3LJ, UK; email:[email protected].

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Stomal varices were first described in 1968.5,15,16

All variceal tissue is friable and bleeds easily if trau-

matized.1-3,17 Studies have shown that bleeding can

occur in up to 27% of patients with stomal varices

and is potentially fatal.18 Case series data1,5 suggest

that the interval between fashioning the stoma and

the first bleed ranges from 5 months to 29 years

(average 48 months). The reported mortality rate

from a stomal variceal bleed is 3% to 4%5,15,18 — a

relatively low rate compared with the 20% mortali-

ty associated with esophageal variceal bleeds.19

Diagnosis. Diagnosing stomal varices is chal-

lenging. Clinicians should be highly suspicious of

stomal varices in patients with recurrent stomal

bleeding.20 Assessment of patients’ coagulation sta-

tus, liver function, and stage of liver disease are

important. Liver disease leads to impaired vitamin

K production and prothrombin activity (ie, clot-

ting) is impaired.21 Portal hypertension, a result of

liver disease (eg, cirrhosis, extrahepatic portal vein

occlusion, intrahepatic veno-occlusive disease, and

occlusion of the main hepatic veins) leads to the

formation of varices at sites of portosystemic anas-

tomosis.22 The site of the bleeding (eg, the lumen,

the mucosa, or the muco-cutaneous junction) is a

factor in determining immediate and future man-

agement strategies. The stoma may look normal or

have characteristic bluish surrounding skin with

excoriation and streaks of vessels possibly visible

around the stoma2, 20 (see Figure 1). The mucosa may

appear red and engorged in the presence of hemor-

rhaging and narrowed by annular scarring due to

repeated bleeds.2 An endoscopy is needed to

confirm that other conditions such as arteri-

ovenous malformations, polyps, or ulcers are

not cause for concern. Selective mesenteric

angiography,2,23 computed tomographic

angiography, endoscopic ultrasonography,9 and

magnetic resonance angiography18 help diag-

nose portal hypertension.16,24

Treatment. Treatment is directed at pre-

venting further bleeding and ranges from local

measures to portosystemic shunt placements

and liver transplantation. Local measures are

usually temporary; the underlying cause of the

bleed is not treated.10,15,25

Local measures include adjusting the face plate

to reduce local trauma, applying firm pressure to

the bleeding point with or without epinephrine-

soaked gauze (1:100,000),2,4 gel foam, chemical

cautery (eg, silver-nitrate),15 injection sclerothera-

py2,3,5,15,26 (using 0.5% to 1% polydocanol, 5% phe-

nol in almond oil, sodium tetradecyl sulphate,

absolute ethanol, and strong saline solution),2,27

correcting coagulation abnormalities, and suture

ligation of the bleeding point. All of these strategies

have been tried (and may be repeated) with varying

rates of success.2,4,6,24 Each carries a variable risk of

peristomal ulceration, infection, stricturing, and

necrosis depending on patient factors such as

nutrition, disease state, and technique used.5,15 The

patients should be referred to a wound and ostomy

care nurse for education regarding preventive

measures and immediate treatment.

August 2007 Vol. 53 Issue 8 21

KEY POINTS• Stomal bleeding, especially if recurrent and in patients with liver

disease, may be caused by stomal varices and may be life-threatening.

• Diagnostic studies to determine the presence of portal hyperten-sion are needed to help guide optimal treatment.

• As occurred with the patients described, most patients are man-aged conservatively (eg, by applying pressure) or surgically withplacement of a transjugular portosystemic shunt.

Ostomy Wound Management 2007;53(8):20–28

Figure 1. Stoma showing characteristic visible peristomal ves-sels and narrowing due to fibrosis from repeated bleeds.

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Although mucocutaneous disconnection1 and

stoma re-siting have been suggested,2,4,15 these

approaches did not stop the bleeding in the patients

described at the author’s facility.

Beta-blockers also are used as an attempt to lower

the portal pressure but their efficacy in preventing

subsequent bleeds has not been evaluated.15,24

Percutaneous transhepatic embolization of

stomal varices also has been used successfully,

although the long-term efficacy of this procedure is

unknown. Complications of embolization include

bleeding, bile leak, liver trauma, portal vein throm-

bosis, and bowel infarction.5,12,16,24 28,29

Surgical portosystemic shunts present a definitive

treatment option to reduce the portal pressure4,23,30,31

but are associated with significant morbidity and

mortality2 and may compromise potential liver trans-

plant at a later stage.15,25 Hence, they should be per-

formed only in carefully selected patients.5

A transjugular intrahepatic portosystemic shunt

(TIPSS) is currently the treatment of choice for

portal hypertension.10,32-37 First used in 1989, TIPSS

involves the creation of a portosystemic shunt

(bypass) between the hepatic vein and the intrahep-

atic portal vein using a transjugular approach;

thereby, reducing portal venous pressure. Under

direct x-ray vision using a C-arm, a stent is placed

at the site and dilated until a gradient between the

systemic and portal pressures falls to <12 mm Hg.10

The procedure can be performed even in patients

who are Child-Pugh class C (see Table 1).21 A suc-

cess rate of up to 90% for TIPSS placement has

been reported8; TIPSS was successful in treating and

preventing re-bleeding in one of the patients pre-

sented herein. Most authors presenting on the sub-

ject consider a post-procedure reduction of porto-

caval pressure of ≤12 mm Hg or a 20% reduction

to be sufficient to prevent variceal rebleeding.8,10,32

The incidence of early life-threatening complica-

tions after TIPSS is 1% to 2% and hepatic

encephalopathy procedures are less commonly per-

formed than surgical shunts.10

Although the occlusion rate following this proce-

dure is approximately 50% at 1 year, it can be

reduced by the use of polytetrafluoroethylene

(PTFE)-covered stents.10,26 Regular surveillance with

portography or Doppler ultrasound is indicated.32,38

Case Reports Patient 1. A 60-year-old woman presented with

recurrent, occasionally heavy ileostomy bleeding

that appeared to be of venous and mucosal origin.

She had undergone total proctocolectomy and

ileostomy in 1997 for ulcerative colitis. Between

1998 and 2002, she was admitted to the hospital six

times with stomal hemorrhage. She was managed

initially with under-running sutures and silver

nitrate application to the tissue around the stoma

but later underwent excision and revision of her

ileostomy. The histology report of her stomal stump

showed dilated vessels.

When examined on presentation in 2002, 5 years

after the initial procedure, her stoma was stenosed

due to scarring, appeared cyanosed, and the sur-

rounding skin was bluish and surrounded by

streaks of tiny blood vessels. Further investigation

and an ultrasound scan revealed an enlarged liver

with some nodularity. A mesenteric angiogram

showed an occluded superior mesenteric vein and

lower esophageal and stomal varices. A portogram

(portal venogram) showed signs of portal hyper-

tension and a transjugular liver biopsy confirmed

micronodular cirrhosis; she was in Child-Pugh

Class A (see Table 1) for liver disease. Contrast-

enhanced CT of her abdomen revealed large stomal

22 OstomyWound Management

TABLE 1CHILD-PUGH CLASSIFICATION

ParameterAlbumin (g/dL)Bilirubin (Ìmol/L)Prothrombin (sec >normal)Prothrombin (%)AscitesEncephalopathy

A>3.5<25<4

>64NoneNone

B3.0–3.525–40

4–640–65

ControlledMinimal

C<3.0>40>6

<40RefractoryAdvanced

This classification is used to define the severity of liver diseaseand the following parameters are taken into consideration.Each positive parameter is given 1 point in category A, 2 pointsin category B, and 3 points in category C. Child-Pugh Classesare based on total points scored:A= 5≤7 points; B= 8≤10 points; C=≥11

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varices anastomosing with cutaneous vessels (see

Figure 2), a dilated splenic vein with splenomegaly,

and a possible portal vein stenosis at the hilum of

the liver. The patient underwent a transjugular

intrahepatic portosystemic shunt (TIPSS) place-

ment in 2002 and her bleeding stopped.

Patient 2. A 72-year-old man had an

abdominoperineal resection for a Duke’s C rectal

adenocarcinoma in 2002. He presented through the

emergency department in September and December

2005 with massive episodes of bleeding at the

muco-cutaneous junction of the stoma. The bleed-

ing stopped with pressure to the stoma with epi-

nephrine-soaked gauze and he was managed con-

servatively. He also was found to have abnormal

liver function tests — a CT scan showed extensive

hepatic and pulmonary metastasis and prominent

stomal varices. A colonoscopy revealed only mild

mucosal inflammation. He was terminally ill and

the bleeding stopped with pressure on each occa-

sion. He died later from his metastatic disease.

Patient 3. A 69-year-old woman presented with a

massive bleed from the lumen of her ileostomy in

April 2006, 19 years after the initial procedure. She

had a total proctocolectomy and ileostomy in 1987

for ulcerative colitis. An earlier CT had shown

stomal varices with signs of portal hypertension

and a subsequent liver biopsy showed sclerosing

cholangitis with micronodular cirrhosis; she was in

Child-Pugh Class B (see Table 1) for liver disease.

An ileoscopy confirmed peristomal varices. The

bleeding stopped with pressure to the stoma with

epinephrine-soaked gauze and she was discharged.

She had no further bleeds from her stoma but died

in July 2006 due to liver failure.

Discussion These three patients had typical presentation of

liver disease and stomal hemorrhage. All had been

managed initially with local measures and respond-

ed well. One of these patients had multiple episodes

of stomal hemorrhage and each time responded to

local measures of pressure to the stoma site, under-

running sutures, and silver nitrate application;

however, she had revision of her stoma and later

had TIPSS placement. Two other patients respond-

ed to local measures to stop stomal bleed but were

terminally ill and did not undergo any further inter-

ventions. The procedures used are well recognized

in the literature but whether one would have been

better than the other is a matter of debate as evi-

dence in this regard is lacking. Furthermore, per-

forming TIPSS earlier in the management of the

first patient would have prevented subsequent read-

missions with bleeding.

ConclusionStomal varices are a rare cause of stomal hemor-

rhage. The bleeding is recurrent and occasionally

hemodynamically significant. Patients with under-

lying liver disease presenting with recurrent stomal

bleeds should be considered at risk. Treatment

includes local measures to stop active bleeding and

interventions to reduce portal venous pressure such

as TIPSS. Guidelines for the optimal management

of these patients are not available. Further research

to evaluate each of the measures suggested to weigh

their risks and benefits is warranted.

24 OstomyWound Management

Figure 2. Transverse sections of CT angiogram showing dilatedcutaneous vessels communicating with the mesenteric vesselsaround the stoma (arrow shows the ileum traversing subcuta-neous tissue to ileostomy site; arrowhead defines the tortuousvessels of the peristomal varices).

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AcknowledgmentThe authors acknowledge the consent of one

patient to discuss the case. The other two patients

are deceased. - OWM

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