Downloaded from www.ajronline.org by 112.2.0.75 on 10/09/15 from IP address 112.2.0.75. Copyright ARRS. For personal use only; all rights reserved
105
Case
Blue Rubber Bleb Nevus Intussusception Robert
T. Tyrrel,1
Bruce
R. Baumgartner,1
Syndrome:
and Karen
Case Report
CT ofthe tolerated, Multiple
and pelvis was performed
abdominal
symptoms.
but IV contrast dilated
loops
and trunk. These masses were compressible, soft, and occasionally tender to palpation. They measured between 2 mm and 1 0 mm in diameter. Moderate epigastric and right-lower-quadrant tenderness was present, but there was no rebound tenderness or guarding. Laboratory studies revealed that the hematocrit had fallen from a baseline level of 30% to 1 7%. Endoscopic examination showed multiple nevi in the esophagus, stomach, and proximal small bowel. No active bleeding was identified.
radiograph
scattered
punctate
obtained
with the patient supine showed
calcifications
in the abdomen
Received May 22, 1989; accepted after revision July 10, 1989. 1 Department of Radiology, Emory University School of Medicine,
to A. T. Tyrrel. 2 Kaiser-Permanente,
Southeast
Permanente
(Fig. 1 A).
Emory
University
of small
C American
Roentgen
to evaluatethe
contrast
was administered
bowel
with
air/fluid
patient’s
medium
was
without levels
not
incident.
were
noted
throughout the abdomen, consistent with severe ileus or obstruction. Hundreds of small, round, polypoid filling defects were noted projecting from the walls into the lumen of the bowel (Fig. 1 B). Several small hemangiomas in the subcutaneous tissues also were identified. In the right lower quadrant, a high-density soft-tissue mass was noted associated with an irregular gas pattern and bowel wall thickening (Fig. 1C). These findings raised the question of an ileocolic intussusception and pneumatosis intestinalis. was
performed.
clinical condition During
were found to be distended,
surgery,
fluid-filled,
and CT findings, the colon
and covered
and small
a lapabowel
with numerous
hemangiomas. An inflammatory mass in the right lower quadrant was confirmed, and gangrene of a short segment of the distal ileum was discovered. Other locations of hemangiomas included the liver, gallbladder, adrenal gland, and mesentery. The involved portion of bowel was resected. Pathologic examination of the specimen showed a 9cm ileocolic intussusception with a 3-cm area of volvulus and infarction. Both the serosa and submucosa were studded with cavernous hemangiomas. A large clot was present in the cecum.
Discussion Blue rubber bleb nevus syndrome is a rare entity consisting of multiple cutaneous and gastrointestinal hemangiomas. Although first described by Gascoyen in 1 860, Bean coined the term “blue rubber bleb nevus syndrome” in 1 958 [1 ]. He
Hospital,
1 364 Clifton Rd., N.E., Atlanta,
Group, Inc., 3065 Hargrove Ad., Ste. 1 10, Atlanta, GA 30339.
January 1990 0361-803X/90/1541-0105
Oral
medium
In view of the patient’s
A 20-year-old woman was admitted with acute abdominal pain, nausea, and vomiting. The vomitus was guaiac positive. The patient had a 10-year history of recurrent gastrointestinal bleeding producing chronic anemia and requiring multiple transfusions. Family history did not include intestinal bleeding or anemia. Physical examination revealed a pale, ill-appearing woman with multiple subcutaneous blue nevi on the upper and lower extremities
AJR 154:105-106,
abdomen
increasing
rotomy
An abdominal
of
A. Montemayor
Blue rubber bleb nevus syndrome is a rare entity consisting of multiple rubbery blue nevi and hemangiomas in the gastrointestinal tract and cutaneous tissues. The multiple angiomatous lesions commonly cause chronic anemia due to gastrointestinal bleeding. Typically, the diagnosis of this syndrome is made on the basis of the clinical findings, before imaging is performed. However, the various organs affected, their extent of involvement, and serious complications such as intussusception often can be defined easily with CT.
multiple,
CT Diagnosis
Report
Ray Society
GA 30322. Address reprint requests
TYRREL
Downloaded from www.ajronline.org by 112.2.0.75 on 10/09/15 from IP address 112.2.0.75. Copyright ARRS. For personal use only; all rights reserved
106
ET AL.
AJA:154, January 1990
Fig. 1.-A, Abdominal radiograph shows multiple, scattered, punctate calcifications (arrows) in phleboliths within venous portion of hemangiomas. B, CT scan reveals dilated fluid-filled loops of bowel consistent with ileus or obstruction. Note round polypoid filling defects (hemangiomas, solid arrows) in lumen and calcified phleboliths (open arrow). C, CT scan shows high-density mass (arrow) in right lower quadrant, with layers of various densities within it. This is a typical pattem for intussusception.
divided the syndrome into three main forms: type 1 a large disfiguring cavernous angioma that may increase in size and obstruct vital tissue; type 2, “blue rubber nipple” covered with thin skin that is compressible and refills rapidly; and type 3, irregular blue or black macules or papules. Type 2 is most common. To date, fewer than 50 cases have been reported [2]. Although an autosomal-dominant inheritance has been documented in some patients, most, as in our case, have no family history of the syndrome [3]. Histologically, lesions consist of a spectrum of vascular abnormalities ranging from capillary telangiectasias to cavernous hemangiomas to frank arteriovenous communication. The most common lesion is the cavernous hemangioma [4]. Typically, the skin and gastrointestinal tract are most preponderantly involved [5]. Many other locations, including the peritoneal cavity, mesentery, liver, lung, eye, glans penis, and central nervous system, may be involved [2]. The angiomas may range in size from 1 mm to 1 0 cm in diameter; the number of lesions ranges into the hundreds. There is no evidence of malignant change in these lesions [6]. The cutaneous hemangiomas may be painful, but they usually require no treatment. The gastrointestinal lesions commonly bleed, producing anemia. They also may cause complications such as intussusception, volvulus, and infarction [7]. In a patient with blue rubber bleb nevus syndrome and evidence of intestinal obstruction, the possibility of one of these vascular lesions producing localized hemorrhage in a bowel wall or acting as the leading edge of an intussusception ,
should be suspected. Various CT findings have been reported with intussusception, including, as in this case, bandlike areas of high density and adjacent curvilinear regions of low density that produce a layered pattern in an abdominal mass [8]. It is important to recognize the syndrome so that when clinicians see specific skin lesions, they suspect associated gastrointestinal disease. Other diseases similar in this respect to blue rubber bleb nevus syndrome include Maffucci syndrome and hereditary hemorrhagic telangiectasia. In patients with blue rubber bleb nevus syndrome, CT may be useful in defining the extent of the disease and its complications.
REFERENCES 1 . Bean WB. Vascular spiders and related lesions of the skin. Springfield, IL: Thomas, 1958:178-185 2. Sandhre KS, Choen H, Radin A, Buck FS. Blue rubber bleb nevus syndrome presenting with recurrences. Dig Dis Sd 1987;32:214-219 3. Walshe MM, Evans CD, Warn RP. Blue rubber blob nevus. Br Med J 1966;2:931-932
4. Jorizzo JA, Amparo EG. MA imaging of blue rubber J Comput
Assist
Tomogr
bleb nevus syndrome.
1986;10:686-688
5. McCauley
AGK, Leonidas JC, Bartoshesky LE. Blue rubber bleb nevus Radiology 1979;133:375-377 6. Wong 5H, Lau WY. Blue rubber bleb nevus syndrome. Dis Colon Rectum syndrome.
1982;25:371-374
7. Browne AF, Katz 5, Miser J, Boles ET. Blue rubber of intussusception.
J Pediatr
Surg
bleb nevi as a cause
1983;18:7-9
8. Iko BO, Teal JS, Siram SM, Chinwuba CE, Aoux VJ, Scott VF. Computed tomography of adult colonic intussusception: clinical and experimental studies. AJR 1984;143:769-772
105
Case
Blue Rubber Bleb Nevus Intussusception Robert
T. Tyrrel,1
Bruce
R. Baumgartner,1
Syndrome:
and Karen
Case Report
CT ofthe tolerated, Multiple
and pelvis was performed
abdominal
symptoms.
but IV contrast dilated
loops
and trunk. These masses were compressible, soft, and occasionally tender to palpation. They measured between 2 mm and 1 0 mm in diameter. Moderate epigastric and right-lower-quadrant tenderness was present, but there was no rebound tenderness or guarding. Laboratory studies revealed that the hematocrit had fallen from a baseline level of 30% to 1 7%. Endoscopic examination showed multiple nevi in the esophagus, stomach, and proximal small bowel. No active bleeding was identified.
radiograph
scattered
punctate
obtained
with the patient supine showed
calcifications
in the abdomen
Received May 22, 1989; accepted after revision July 10, 1989. 1 Department of Radiology, Emory University School of Medicine,
to A. T. Tyrrel. 2 Kaiser-Permanente,
Southeast
Permanente
(Fig. 1 A).
Emory
University
of small
C American
Roentgen
to evaluatethe
contrast
was administered
bowel
with
air/fluid
patient’s
medium
was
without levels
not
incident.
were
noted
throughout the abdomen, consistent with severe ileus or obstruction. Hundreds of small, round, polypoid filling defects were noted projecting from the walls into the lumen of the bowel (Fig. 1 B). Several small hemangiomas in the subcutaneous tissues also were identified. In the right lower quadrant, a high-density soft-tissue mass was noted associated with an irregular gas pattern and bowel wall thickening (Fig. 1C). These findings raised the question of an ileocolic intussusception and pneumatosis intestinalis. was
performed.
clinical condition During
were found to be distended,
surgery,
fluid-filled,
and CT findings, the colon
and covered
and small
a lapabowel
with numerous
hemangiomas. An inflammatory mass in the right lower quadrant was confirmed, and gangrene of a short segment of the distal ileum was discovered. Other locations of hemangiomas included the liver, gallbladder, adrenal gland, and mesentery. The involved portion of bowel was resected. Pathologic examination of the specimen showed a 9cm ileocolic intussusception with a 3-cm area of volvulus and infarction. Both the serosa and submucosa were studded with cavernous hemangiomas. A large clot was present in the cecum.
Discussion Blue rubber bleb nevus syndrome is a rare entity consisting of multiple cutaneous and gastrointestinal hemangiomas. Although first described by Gascoyen in 1 860, Bean coined the term “blue rubber bleb nevus syndrome” in 1 958 [1 ]. He
Hospital,
1 364 Clifton Rd., N.E., Atlanta,
Group, Inc., 3065 Hargrove Ad., Ste. 1 10, Atlanta, GA 30339.
January 1990 0361-803X/90/1541-0105
Oral
medium
In view of the patient’s
A 20-year-old woman was admitted with acute abdominal pain, nausea, and vomiting. The vomitus was guaiac positive. The patient had a 10-year history of recurrent gastrointestinal bleeding producing chronic anemia and requiring multiple transfusions. Family history did not include intestinal bleeding or anemia. Physical examination revealed a pale, ill-appearing woman with multiple subcutaneous blue nevi on the upper and lower extremities
AJR 154:105-106,
abdomen
increasing
rotomy
An abdominal
of
A. Montemayor
Blue rubber bleb nevus syndrome is a rare entity consisting of multiple rubbery blue nevi and hemangiomas in the gastrointestinal tract and cutaneous tissues. The multiple angiomatous lesions commonly cause chronic anemia due to gastrointestinal bleeding. Typically, the diagnosis of this syndrome is made on the basis of the clinical findings, before imaging is performed. However, the various organs affected, their extent of involvement, and serious complications such as intussusception often can be defined easily with CT.
multiple,
CT Diagnosis
Report
Ray Society
GA 30322. Address reprint requests
TYRREL
Downloaded from www.ajronline.org by 112.2.0.75 on 10/09/15 from IP address 112.2.0.75. Copyright ARRS. For personal use only; all rights reserved
106
ET AL.
AJA:154, January 1990
Fig. 1.-A, Abdominal radiograph shows multiple, scattered, punctate calcifications (arrows) in phleboliths within venous portion of hemangiomas. B, CT scan reveals dilated fluid-filled loops of bowel consistent with ileus or obstruction. Note round polypoid filling defects (hemangiomas, solid arrows) in lumen and calcified phleboliths (open arrow). C, CT scan shows high-density mass (arrow) in right lower quadrant, with layers of various densities within it. This is a typical pattem for intussusception.
divided the syndrome into three main forms: type 1 a large disfiguring cavernous angioma that may increase in size and obstruct vital tissue; type 2, “blue rubber nipple” covered with thin skin that is compressible and refills rapidly; and type 3, irregular blue or black macules or papules. Type 2 is most common. To date, fewer than 50 cases have been reported [2]. Although an autosomal-dominant inheritance has been documented in some patients, most, as in our case, have no family history of the syndrome [3]. Histologically, lesions consist of a spectrum of vascular abnormalities ranging from capillary telangiectasias to cavernous hemangiomas to frank arteriovenous communication. The most common lesion is the cavernous hemangioma [4]. Typically, the skin and gastrointestinal tract are most preponderantly involved [5]. Many other locations, including the peritoneal cavity, mesentery, liver, lung, eye, glans penis, and central nervous system, may be involved [2]. The angiomas may range in size from 1 mm to 1 0 cm in diameter; the number of lesions ranges into the hundreds. There is no evidence of malignant change in these lesions [6]. The cutaneous hemangiomas may be painful, but they usually require no treatment. The gastrointestinal lesions commonly bleed, producing anemia. They also may cause complications such as intussusception, volvulus, and infarction [7]. In a patient with blue rubber bleb nevus syndrome and evidence of intestinal obstruction, the possibility of one of these vascular lesions producing localized hemorrhage in a bowel wall or acting as the leading edge of an intussusception ,
should be suspected. Various CT findings have been reported with intussusception, including, as in this case, bandlike areas of high density and adjacent curvilinear regions of low density that produce a layered pattern in an abdominal mass [8]. It is important to recognize the syndrome so that when clinicians see specific skin lesions, they suspect associated gastrointestinal disease. Other diseases similar in this respect to blue rubber bleb nevus syndrome include Maffucci syndrome and hereditary hemorrhagic telangiectasia. In patients with blue rubber bleb nevus syndrome, CT may be useful in defining the extent of the disease and its complications.
REFERENCES 1 . Bean WB. Vascular spiders and related lesions of the skin. Springfield, IL: Thomas, 1958:178-185 2. Sandhre KS, Choen H, Radin A, Buck FS. Blue rubber bleb nevus syndrome presenting with recurrences. Dig Dis Sd 1987;32:214-219 3. Walshe MM, Evans CD, Warn RP. Blue rubber blob nevus. Br Med J 1966;2:931-932
4. Jorizzo JA, Amparo EG. MA imaging of blue rubber J Comput
Assist
Tomogr
bleb nevus syndrome.
1986;10:686-688
5. McCauley
AGK, Leonidas JC, Bartoshesky LE. Blue rubber bleb nevus Radiology 1979;133:375-377 6. Wong 5H, Lau WY. Blue rubber bleb nevus syndrome. Dis Colon Rectum syndrome.
1982;25:371-374
7. Browne AF, Katz 5, Miser J, Boles ET. Blue rubber of intussusception.
J Pediatr
Surg
bleb nevi as a cause
1983;18:7-9
8. Iko BO, Teal JS, Siram SM, Chinwuba CE, Aoux VJ, Scott VF. Computed tomography of adult colonic intussusception: clinical and experimental studies. AJR 1984;143:769-772
