Rectal Bleeding and Hypertensive Colopathy in May‐Thurner Syndrome
Makaleler > Tam Makale · 2012
Yazarlar: Masallah Baran, Ali Kanik, Ender Can, Mustafa Parildar, Emel E. Pala, Mehmet Helvaci
Journal of Pediatric Gastroenterology and Nutrition, Uluslararası, Wos, 2012, cilt 56, ss e42-3
DOI: 10.1097/mpg.0b013e318264569d
Özet
May-Thurner syndrome, known as iliac vein compression syndrome (IVCS), is defined as compression of the left iliac vein between the right iliac artery and the vertebrae (1). External pressure on the vein leads to intraluminal alterations and a clinical picture of deep vein thrombosis (DVT) or venous hypertension without venous thrombosis. The syndrome frequently imitates the DVT clinical picture, and symptoms such as pain and swelling may be seen in the lower left extremity in some cases. Early diagnosis and treatment are important (2). Computed tomography (CT) and magnetic resonance angiography are imaging techniques used for diagnosis, venography being the most important diagnostic tool (2). In addition to surgical techniques and compression relaxation for treating the disease, techniques such as interventional radiology and stent placement have also recently entered into use (3). Childhood age group cases have been reported, albeit rarely (2,4). We report a previously undescribed case presenting with rectal bleeding, initially diagnosed as ulcerative colitis and subsequently treated with a diagnosis of IVCS. A 17-year-old girl presented with rectal bleeding. The patient had experienced periodic rectal bleeding during the preceding 4 years. Inflammatory bowel disease had been suspected for 1 year and erythrocyte transfusion had been administered. She was hospitalized when the rectal bleeding persisted despite receiving azathioprine and mesalamine treatment for 1 year. The patient weighed 47 kg (3rd–10th percentile) and was 160 cm (25th–50th percentile) tall. No pathological findings apart from anemia and tachycardia were identified at physical examination. Initial laboratory findings were hemoglobin 7.2 g/dL, hematocrit 25%, mean cell volume 61 fL, white blood cell count 7000, platelet 344,000, sedimentation 11 mm/hour, C-reactive protein 0.8 mg/dL, prothrombin time 15 s, international normalized ratio 1.2, aspartate aminotransferase 17, alanine aminotransferase 7, γ-glutamyl transpeptidase 11 (IU/L), total protein 7.1, albumin 4.2, globulin 2.9 (g/dL), urea 24, creatinine 0.8 mg/dL, and ferritin 6 ng/mL, and erythrocyte transfusion was performed. Perinuclear anti-neutrophil cytoplasmic antibodies and anti-neutrophil antibodies were both negative. Colonoscopy revealed findings of erythema and congestion from the left colon, increasing toward the rectum, with grade 2 varices and venous dilation in the rectal region (Fig. 1). Histopathological examination of the transverse colon, sigmoid colon, and rectum revealed minimal mix inflammation, intense edema, congestion, microhemorrhage, and dilated capillaries in the lamina propria (Fig. 2). Portal Doppler ultrasonography (USG) and upper gastrointestinal endoscopy were normal. Anti-phospholipid antibody, anti-smooth muscle antibodies, liver kidney microsomal antibodies, anti-mitochondrial antibodies, and celiac serology were all negative. Immunoglobulin (Ig)A was 150 mg/dL, IgG was 895 mg/dL, and IgM was 179 mg/dL. No mutation was determined at familial Mediterranean fever mutation analysis. Viral serological examination revealed that hepatitis B surface antigen, Epstein-Barr virus IgM, cytomegalovirus IgM, antihepatitis C virus, and human immunodeficiency virus were negative. Cytomegalovirus IgG, anti-hepatitis B surface antibodies, and Epstein-Barr virus IgG were positive. CT angiography performed for vascular thrombosis revealed a suspicion of thrombosis in the left iliac vein together with a thickening in the rectum wall and collateral venous structures dilated on the left and exhibiting tortuosity disgorging to the vena cava inferior. MTHFR, FV Leiden, and prothrombin mutations investigated in terms of a tendency to thrombosis were negative. Protein C was 71%, protein S was 76%, and antithrombin 3 antigen was 112%, which were within normal limits.FIGURE 1: View of the distal colon at diagnosis showing diffuse erythema, congestion, capillary, and venous dilatation.FIGURE 2: Dilated capillaries, edema, and microhemorrhage in the colonic lamina propria (hematoxylin & eosin, original magnification ×100).Iliac venography was performed with suspicion of iliac vein thrombosis or IVCS, and the findings were compatible with IVCS (Fig. 3). A stent was inserted in the iliac vein in the same session. The patient was started on coumadin for anticoagulant therapy. In a month during colonoscopy, the rectal varices were contracted but mucosal erythema persisted. May-Thurner syndrome was diagnosed on the basis of the existing findings. The azathioprine and mesalamine treatments were discontinued and propranolol was initiated. In 6 months, Doppler USG showed that the iliac vein was open. Control colonoscopy revealed reduced, but persisting erythema in the left colon and decreased rectal varices. Transfusion was not required.FIGURE 3: Left iliac venography of the patient revealed smooth contoured external compression (arrow) of the left main iliac vein and excessive engorgement of the left internal iliac vein (double arrow). The external iliac vein could not be opacified due to the compression.DISCUSSION May and Thurner examined 430 cadavers and reported that 22% had obstructive fibrous spur-like bands within the lumen of the left iliac vein (3). Mean compression in this asymptomatic population was 35.5% (5). Symptomatic IVCS occurs predominantly in young to middle-aged (ages 20–40 years) women (2). Frequent clinical findings are swelling and edema in the lower extremity (often the left leg), a DVT picture, and chronic venous stasis and ulceration (3); however, the literature contains no case reports progressing with rectal bleeding and a colitis picture. The fact that the patient's rectal bleeding had persisted for 4 years is interesting in terms of constituting an early finding. Secondary vascular thrombosis is one frequent complication in inflammatory bowel diseases. Venous and arterial thromboembolisms are both serious extraintestinal manifestations of inflammatory bowel disease (6). Although the findings in this case with rectal bleeding suggested inflammatory bowel disease, laboratory and histopathological examinations did not support this. Colonoscopy revealed an erythematous mucosa in the left colon, with a distinct line in contrast to the other half, and the findings increased distally. These findings suggested a vascular pathology. No increase in findings was observed after cessation of immunosuppressive therapy. Laboratory examinations based on a tendency to thrombosis were within normal limits. Doppler USG and iliac vein examination can assist diagnosis in suspected cases (7). Iliac venography provides the most certain diagnosis in cases with significant clinical findings. No vascular pathology was determined with Doppler USG in our case; however, iliac vein thrombosis was suspected at CT angiography, and the diagnosis was made with iliac venography in our case. Today, endovascular therapy is an important treatment modality in patients with May-Thurner syndrome. It is useful in ensuring long-term luminal flow (2,8). We observed a partial improvement in the hypertensive colitis picture and a significant improvement in rectal varices after vascular catheterization and stent placement. In the light of this case, IVCS should be borne in mind in cases of rectal bleeding with an erythematous appearance in the distal colon and in which congestion and/or rectal varices are determined.