Venous Malformation Intestine

Starlike Lesion Colon Near Rectum

Fig. 14.4 Rectum in a patient after neoadjuvant radiation of a rectal carcinoma.

a Angiodysplasias almost completely covering the mucosa.

b Point coagulation (APC) is used to coagulate newly forming vessels. Only a portion of the angiodysplasias should be coagulated to avoid risk of deep ulcerations or stenosis caused by scarring.

Fig. 14.4 Rectum in a patient after neoadjuvant radiation of a rectal carcinoma.

a Angiodysplasias almost completely covering the mucosa.

b Point coagulation (APC) is used to coagulate newly forming vessels. Only a portion of the angiodysplasias should be coagulated to avoid risk of deep ulcerations or stenosis caused by scarring.

Fig. 14.5 Anal canal in a patient with radiation-induced angiodysplasia in the rectum. Newly forming vessels are also visible on the surface of the hemorrhoids.

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Table 14.1 Criteria for clinical diagnosis of hereditary hemorrhagic telangiectasia (HHT)

Findings

► Epistaxis (spontaneous or recurrent)

► Telangiectasias on skin and mucosa: multiple, characteristic localizations (face, lips, oral cavities, and fingers)

► Visceral arteriovenous malformations (lung, brain, liver, spine) or gastrointestinal telangiectasias (with or without bleeding)

► Family history: immediate family member with HHT (according to above criteria)

Probability of diagnosis

► Definitive diagnosis: >3 criteria

► Probable diagnosis: 2 criteria

■ Differential Diagnosis

It is important to distinguish angiodysplasias from artifacts (i. e., mucosal bleeding) caused by the instrument (Fig. 14.6). This is often difficult, particularly with small, clearly bordered lesions. Localization can sometimes provide a clue: a circumscribed red patch in the sigmoid colon, for example, is more likely to be an artifact than a vascular malformation.

Biopsy of smaller lesions is only of further assistance in positive cases. Despite typical colonoscopic appearances, pathologists in two separate studies observed classic histological appearances (Fig. 14.1) in only half of the patients (16, 36). This could be explained either by pinching of the mucosa during biopsy, causing vessel collapse, or by compression of the vessels as a result of bleeding into the surrounding tissue, rendering diagnosis impossible. Endoscopic aspect thus remains the essential diagnostic criterion. Larger vascular malformations should not be biopsied, given the risk of bleeding.

■ Syndromes/Vascular Malformations in Other Diseases

Hereditary hemorrhagic telangiectasias. Angiodysplasias manifest in the colon in association with numerous syndromes, the most well known of which is hereditary hemorrhagic telangiec-tasia, also known as Osler-Weber-Rendu syndrome. HHT is a hereditary, autosomal dominant disease characterized by the formation of abnormal blood vessels. Clinical picture involves attack in various organs, primarily the brain, lungs, skin, nose, liver and gastrointestinal tract. Two clear genetic defects related to HHT have been identified.

Unlike usual angiectasias, HHT affects younger people as well, with involvement of the entire gastrointestinal tract. Diagnosis should be made based on Curaçao criteria (Tab. 14.1). Gastrointestinal bleeding occurs in one-third of patients with HHT (18) and patients over age 60 are especially at risk. The most common localization of HHT telangiectasias is the stomach and proximal small bowel; the colon is less often affected (22).

Lesions can become symptomatic as a result of acute lower gastrointestinal bleeding or due to anemia from chronic bleeding. Endoscopic intervention promises little long-term success (32), perhaps in part because the lesions in the small bowel are difficult to reach endoscopically if at all.

Portal colopathy. Angiodysplasias can also be detected in the colon (analogous to the stomach) in patients with portal hypertension (Figs. 14.7, 14.8). Prevalence reported in the literature ranges between 12-89% (5,9,13,14,19,34,37). Risk of bleeding from these angiodysplasias appears to be low (between 0-9%) (9,13). However, hemorrhaging can become problematic in rare cases, and can only be controlled by means of a transjugular intrahepatic portosystemic shunt (TIPPSs) (4).

Patients with portal hypertension often also have dilated veins (varices), primarily in the rectum (Fig. 14.9). Prevalence related to portal hypertension reported in the literature ranges from 8-89% (8-10, 13-15, 34, 37, 40). Frequent detection of

Phlebectasie Colon
Fig. 14.6 Artifact in sigmoid colon: submucosal bleeding. Red, textured pattern which can be clearly distinguished from an-giodysplasia.

Fig. 14.7 Angiodysplasia in ascending colon in a patient with portal hypertension due to liver cirrhosis in chronic hepatitis. The angiodysplasia looks similar to a spider

Fig. 14.8 Small angiodysplasia in the right hemicolon in a patient with portal hypertension due to liver cirrhosis.

colonic angiodysplasias and varices in patients with portal hypertension has led to use of the term "portal colopathy."

Other diseases. Increased frequency of angiectasias is also postulated in other diseases such as von Willebrand disease, aortic stenosis (Heyde syndrome), and terminal renal insufficiency, although data are sometimes inconsistent.

Hemangiomas ■ Epidemiology

Hemangiomas are a much less common finding in the colon than angiodysplasias. Hemangiomas are benign dysontogenetic vascular neoplasms, with an incidence of 1:1400 according to one study based on autopsy reports (1).

Fig. 14.9 Rectal varices in a patient with portal hypertension due to liver cirrhosis. This was an incidental finding.

Histologically, hemangiomas are comprised of wide, bloodfilled endothelium-lined sinuses, separated by varying amounts of connective tissue. Hemangiomas occur in capillary, cavernous, and mixed forms. Hemangiomas in the gastrointestinal tract are mainly the cavernous type. Histology shows large, bloodfilled, endothelium-lined cavities separated from each other by thin connective tissue septa.

Kaposi sarcoma (KS) occurs as a multicentric vascular proliferation. Histology reveals small, endothelium-lined vessels, between which are spindle cells; erythrocytes can be detected in small tissue cracks (not lined with endothelium). Kaposi sarcoma is primarily found in patients with AIDS.

■ Course and Prognosis

Hemangiomas have a tendency to bleed and patients become symptomatic through hematochezia or chronic anemia. Therapeutic potential for large and infiltrative hemangiomas is limited, though endoscopic removal can be attempted with smaller lesions. Children are very often affected by bleeding. Complications related to Kaposi sarcoma include abdominal pain, gastrointestinal bleeding, and intestinal obstruction.

Diagnosis

Hemangiomas have a predilection for the rectum or sigmoid colon and more often occur as single lesions than multiple. A distinction is made between polypoid and infiltrative types.

► Polypoid cavernous hemangiomas present as single or multiple broad-based, sessile, deep red, or livid lesions with a strawberry appearance (Figs. 14.10, 14.11). They measure 5-10 mm in diameter and are markedly elevated.

► Infiltrative cavernous hemangiomas are found primarily in the rectum, appearing as blue-red or black undefined masses, bulging into the lumen. A radiograph of the pelvis typically reveals phleboliths, which are characteristic.

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Angiomas Colon
Fig. 14.10 Two small hemangiomas in the transverse colon.

Fig. 14.11 Three hemangiomas in the transverse colon near the hepatic flexure.

Fig. 14.12 Vascular ectasia (phlebectasia) in the ascending colon in a patient with Turner syndrome and recurrent hemato-chezia. Incidental finding: multiple small, glassy lymphoid hyperplasias on the colon mucosa.

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Multiple phlebectasia, dilated mucosal vessels are another type of hemangioma (Fig. 14.12). Kaposi sarcoma is found in patients with AIDS with roughly the same prevalence in the upper gastrointestinal tract and colon. KS presents either similarly to an-giodysplasia with red foci with a slightly elevated center and starlike branches or as a small red polyp, often ulcerated in the center.

■ Syndromes/Vascular Malformations in Other Diseases

Hemangiomas of the gastrointestinal tract are often associated with those in other parts of the body.

Blue rubber bleb nevus syndrome. BRBNS is an autosomal dominant hereditary disease.

Small mucosal nodules with a blue-red sac, not unlike a rubber nipple, present in the rectum and left hemicolon. Flat, livid mucosal changes have also been reported (overview in 27). Lesions occur in multiple numbers, affecting the upper gastrointestinal tract, peritoneum, liver, spleen, oropharynx, eyes, lungs, heart, CNS, skeletomuscu-lar system, bones, and urogenital system. Gastrointestinal lesions tend to bleed and gastrointestinal hemorrhage is the leading cause of death among affected individuals.

Maffucci syndrome. In Maffucci syndrome, blue rubber bleb nevus syndrome is associated with cerebral medulloblastomas, chronic lymphoid leukemia, hypernephromas, pulmonary hypertension, thrombocytopenia, and consumption coagulopathy. Joint involvement, bone malformation and pathological fractures have also been reported (overview in 27).

Klippel-Trenaunay-Weber syndrome. Gastrointestinal hemangiomas are also seen in association with Klippel-Trenaunay-Weber syndrome (2, 3, 17, 21, 25, 41). Even among children, it can already be a source of massive gastrointestinal hemorrhage. Klippel-Trenaunay-Weber syndrome is characterized by a triad of symptoms: asymmetric limb hypertrophy, varicose veins, and nevus flammeus of the skin.

Turner syndrome. Turner syndrome is caused by X-chromosome monosomy or mosaicism (X0/XX). The result is gonadal dysgenesis with hypergonadotropic hypogonadism. Patients also exhibit pterygium colli, lymphedema, dwarfism, a flat, "shieldshaped" chest, and facultative abnormalities such as congenital cardiac defects. Hemangiomas (Fig. 14.13) and phlebectasia (Fig. 14.14) of the colon (7, 20, 23, 26, 28, 33, 39) have also been reported in patients with Turner syndrome. There are no standardized recommendations for therapy in the literature. Treatment must be determined on an individual patient basis (Figs. 14.13, 14.14).

Endoscopic therapy

► Therapy depends on the size and extent of the lesions. Resection of the affected bowel segments and endo-scopic intervention using thermocoagulation have been reported.

► After removing the lesions, new ones may develop elsewhere.

Fig. 14.13 Hemangiomas in Turner syndrome.

a Several hemangiomas in the ascending colon mucosa in a patient with Turner syndrome (same patient as in Fig. 14.12). b Obliteration of the hemangioma using APC. Coagulation of the adjacent area first (due to potential risk of bleeding) and then the hemangioma itself.

Fig. 14.13 Hemangiomas in Turner syndrome.

a Several hemangiomas in the ascending colon mucosa in a patient with Turner syndrome (same patient as in Fig. 14.12). b Obliteration of the hemangioma using APC. Coagulation of the adjacent area first (due to potential risk of bleeding) and then the hemangioma itself.

Gastroscopy Coagulation Yag Laser

Fig. 14.14 Vascular ectasia in the ascending colon in a patient with Turner syndrome (same patient as in Figs. 14.12, 14.13).

a Obliteration of the vein adjacent to the vascular ectasia using APC. b In addition to sclerosis with APC, the veins are also closed mechanically using hemoclips (Olympus). This diminished vascular ectasia and caused complete cessation of lower intestinal rebleeding.

Fig. 14.14 Vascular ectasia in the ascending colon in a patient with Turner syndrome (same patient as in Figs. 14.12, 14.13).

a Obliteration of the vein adjacent to the vascular ectasia using APC. b In addition to sclerosis with APC, the veins are also closed mechanically using hemoclips (Olympus). This diminished vascular ectasia and caused complete cessation of lower intestinal rebleeding.

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  • Roxy
    What is vascular ectasia in transverse colon?
    8 years ago

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