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Thread: Rectal Nevus Pictures - Atlas of Colon and Ileum

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    Default Rectal Nevus Pictures - Atlas of Colon and Ileum

    We present two cases of hemangiomatosis of the gastrointestinal tract. The first case describes a 59-year-old patient with upper gastrointestinal hemorrhage due to the Blue Rubber Bleb Nevus Syndrome. The second case illustrates a 26-year-old patient with recurrent rectal bleeding due to Klippel-Trenaunay Syndrome. These two syndromes are distinct disorders characterized by cavernous hemangiomas of the skin, soft-tissue, bones, and viscera. In addition, we review the available literature on the epidemiology, transmission, clinical features, associated conditions, diagnosis, and treatment of these two disorders.

    Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare disease characterized by multiple "rubbery" blue nevi located both in the skin and internal organs. Although these vascular lesions were first described by Gascoyen in 1860, the term blue rubber bleb nevus was first used by Bean in 1958 to describe patients with vascular skin lesions and hemangiomas of the gastrointestinal tract. A MEDLINE search yielded more than 70 case reports published since 1966. We describe a patient who had acute upper gastrointestinal hemorrhage due to gastric blue rubber nevi.

    A 59-year-old man with a history of coronary artery disease was transferred to our hospital for management of "bleeding gastric lesions." Two months earlier, he had had hematemesis and melena. An esophagogastroduodenoscopy done at another hospital revealed several sclerosed vascular lesions in the stomach. The diagnosis of possible gastric varices was made. He was treated with iron and H2 receptor blockers. Three days before transfer, he had dyspnea on exertion and easy fatigability. His hematocrit value was 36%, and the mean corpuscular volume was 83 fL. He described intermittent melena but denied dyspepsia, abdominal pain, or hematochezia. Family history revealed gastrointestinal bleeding of unknown cause in a sister. Physical examination disclosed two raised, soft, nontender bluish lesions, each measuring approximately 1 cm in diameter, located on his back and the dorsum of his right foot (Fig 1). He recalled his father having similar skin lesions but denied that he ever had gastrointestinal bleeding. Esophagogastroduodenoscopy showed approximately 20 blue lesions ranging from 1 to 2 cm in diameter located in the fundus and body of the stomach. Several of these lesions, which appeared to be oozing blood, were injected with 3% sodium morrhuate, resulting in hemostasis. A diagnosis of BRBNS was made on the basis of the characteristic skin and gastric lesions. Follow-up endoscopy 6 weeks later showed several persistent lesions, and sclerotherapy was again done. Follow-up 3 months later disclosed no recurrent melena and a stable hematocrit level.

    This syndrome appears to be a congenital disorder since skin lesions are present at/or within a few years of birth, though several cases have been described during adulthood. These lesions tend to increase both in number and in size with time, but malignant transformation has not been reported. Lesions may recur after excision or appear "de novo" throughout life.

    The disease appears to be transmitted in an autosomal dominant fashion. Boon et al and Gallione et al studied two families with venous malformation syndrome and found a linkage to the 9p chromosome. Vikkula et al concluded that these families had an activating mutation in the tyrosine kinase (TIE2) receptor that participates in normal angiogenesis. Despite this evidence, however, sporadic cases are well recognized.

    The skin lesions can be found anywhere over the entire surface of the body but are most commonly located on the upper extremities and trunk, and the number of lesions vary widely from a few to several hundred. The size of the skin lesions is also variable, ranging from several millimeters to 5 cm. Three types of cutaneous lesions have been described: Type 1 is a large disfiguring lesion that may compress vital tissues; Type 2 is the typical red to blue-violet nodule or macule covered with skin; and Type 3 is an irregular macule or papule that may punctuate or merge with adjacent nevi. Although the skin lesions may be painful or tender to palpation, they are most often asymptomatic. The lesion can be partially emptied of blood with application of pressure. Also, excessive sweating from the overlying skin can occur from the close association of the nevi with sweat glands. In contrast to lesions of the gastrointestinal tract, skin lesions rarely bleed spontaneously.

    Gastrointestinal tract lesions are also characteristically multiple and can be located anywhere from the mouth to the anus, but the small intestine appears to be the most common location of the lesions. In the colon, they have a left-sided predilection. The typical lesion is a mucosal nodule with a bluish tinge, but it may also appear as a bluish-red spot or polypoid lesion. Although its presentation with acute gastrointestinal bleeding is well recognized, the most common manifestation of gut involvement is iron-deficiency anemia due to chronic blood loss. Intestinal lesions, when large, can also be manifested as intussusception, volvulus, intramural hemorrhage, or infarction.
    Rectal Nevus Pictures Atlas Colon attachment.php?s=a9369dd699f02a649e363a5ee480b9df&attachmentid=2081&d=1442249063

    Many other locations of these nevi have been described, including the nasopharynx and oropharynx, central nervous system, eye, parotid glands, liver, spleen, pleura, lung, trachea, skeletal muscle, penis, vulva, urinary bladder, pericardium, and bone. The BRBNS has also been observed in association with a variety of other disorders, including Maffucci's syndrome (skin and soft tissue hemangiomas and enchondromatosis), chronic lymphocytic leukemia and hypernephroma,cerebellar medulloblastoma, pulmonary hypertension, disseminated intravascular coagulopathy,and diffuse angiokeratoma. Whether these diseases are coincidental or are linked with BRBNS is unknown because of the rarity of these entities. A number of disorders present themselves with skin lesions, resembling those seen in BRBNS, which makes accurate diagnosis essential. Skin lesions in BRBNS most closely resemble multiple glomus tumors, but visceral hemangiomas are absent in the latter disorder. Other diseases have characteristic lesions and patterns of involvement, including: Osler-Weber-Rendu disease (multiple skin and mucosal membrane telangiectasias); Klippel-Trenaunay-Weber syndrome (vascular nevus of the lower limb, varicosities, and hypertrophy); Maffucci's syndrome (by definition should not have visceral hemangiomas); Sturge-Weber syndrome (facial angioma, seizures, and mental deficiency); von Hippel-Lindau disease (retinal angiomas, cerebellar hemangioblastoma, renal cysts, and hypernephroma); and ataxia-telangiectasia (cerebellar ataxia, oculocutaneous telangiectasias, chronic sinopulmonary disease, and immunodeficiency).

    Histologically, the blue rubber bleb is a cavernous hemangioma with tortuous, dilated, blood-filled spaces composed of ectatic vessels lined by a single layer of flattened or cuboidal endothelium, with surrounding connective tissue. Smooth muscle, fibrous tissue, and sweat glands can be present. Skin lesions tend to be subcutaneous. Visceral involvement is characterized by mucosal and submucosal lesions, though transmural lesions have been reported.

    The diagnosis of BRBNS is purely clinical. Radiologic studies such as plain films may detect calcification from lesions that are thrombosed and have undergone organization. Computed tomography and magnetic resonance imaging may aid in the localization of lesions. Barium studies (barium enema, enteroclysis) may show filling defects resembling sessile polyps. Angiography has also been used both for localization and embolization.The most sensitive method of detecting the gastrointestinal tract lesions is endoscopy.

    When symptomatic or for cosmetic reasons, skin lesions can be excised or ablated with laser therapy. Gastrointestinal tract lesions have been treated expectantly with the use of iron supplementation and blood transfusions for patients with anemia due to chronic blood loss. Successful treatment has been described with angiographic embolization, endoscopic ligation electrocautery, and laser photocoagulation and sclerotherapy. Comparative trials have not been done, and therefore no therapy can be considered the treatment of choice. Enterotomies with excision or combined with photocoagulation have also been done. Bowel resection should be reserved for significant bleeding that cannot be controlled endoscopically and for complications of intussusception and ischemia. As mentioned, these lesions can recur, and thus periodic endoscopic examination may be warranted.

    Parenteral steroids have been used in an attempt to induce regression of the hemangiomas but without success, though one case report suggested a decreased incidence of rebleeding with the use of oral prednisone. Also, interferon alfa-2a has been used with success for patients with the Kasabach-Merritt syndrome and with pulmonary hemangiomatosis. In one case report, intravenous gamma globulin was used with success in a patient with disseminated intravascular coagulation due to BRBNS.

    Blue Rubber Bleb Nevus Syndrome is a rare disease, most commonly manifested as skin and gastrointestinal lesions that may cause occult or overt gastrointestinal blood loss and iron-deficiency anemia. There appears to be an autosomal dominant pattern of inheritance, but sporadic cases may occur. The choice of therapy will depend on the location of the lesions, severity of bleeding, and local endoscopic, radiographic, and surgical expertise. Because BRBNS is so uncommon, surveillance protocols have not been formulated. With further advances into the genetics of this disorder, a better appreciation of the natural history and benefit of screening family members will be realized.

    Klippel-Trenaunay Syndrome (KTS) is a rare congenital disorder characterized by these distinct findings: capillary malformations, soft tissue and/or bony hypertrophy, and venous malformations or varicosities. Currently, the diagnosis of KTS is established by the presence of at least two of these clinical features. The French physicians Klippel and Trenaunay first described the syndrome in 1900. Since then, many case reports and case series have been published, the majority of which have described the typical. In the following report, we describe a patient who had recurrent rectal bleeding and hematuria due to extensive pelvic hemangiomas.
    Rectal Nevus Pictures Atlas Colon attachment.php?s=a9369dd699f02a649e363a5ee480b9df&attachmentid=2082&d=1442249073

    A 26-year-old man was transferred to our institution for evaluation and management of recurrent rectal bleeding. Rectal bleeding had first occurred after birth, and at the age of 2 months he had a diverting colostomy for "congenital hemangioma" of the rectosigmoid. At the age of 7 months, the colostomy was revised because of recurrent bleeding. Since then, he had had infrequent episodes of rectal bleeding. Approximately 2 months before the present admission, he had noticed an increase in the frequency and severity of these episodes, requiring multiple blood transfusions. In addition, he had noticed intermittent painless hematuria. Physical examination disclosed a functioning diverting colostomy located in the left upper quadrant, with guaiac-negative stool. Anal inspection did not reveal any abnormalities, but on digital examination, bright red blood was noticed. A large left scrotal mass consistent with varicocele was palpated. No hemihypertrophy or varicosities were noted on examination of the extremities.

    The hematocrit value was 25%, with a mean corpuscular volume of 79 fL. Flexible sigmoidoscopy showed extensive submucosal vascular lesions resembling varicosities that extended from the rectum to the proximal sigmoid colon. The mucosa was covered with fresh blood, but no definite bleeding site was identified. Computed tomography of the abdomen and pelvis showed low-density masses with multiple phleboliths involving the entire sigmoid and rectum, consistent with hemangiomas. Hemangiomas and varicosities were identified in the anterior abdominal wall, suprapubic area, urinary bladder, and scrotum. A large calcified cystic-appearing mass in the abdominal cavity measuring 8 x 6 x 5 cm was also identified (Fig 2).

    Medscape: Medscape Access

    Last edited by Medical Photos; 09-14-2015 at 04:44 PM.

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