AJR 2002; 179:1042-1044
© American Roentgen Ray Society
Diffuse Cavernous Hemangiomatosis of the Colon: Findings on Three-Dimensional CT Colonography
Raymond M. Hsu1,
Karen M. Horton and
Elliot K. Fishman
1 All authors: Department of Radiology, Johns Hopkins Hospital, 601 N. Caroline
St., Rm. 3254, Baltimore, MD 21287-0801.
Received December 28, 2001;
accepted after revision February 20, 2002.
Address correspondence to E. K. Fishman.
Introduction
Cavernous hemangiomas of the colon, although uncommon, are important for
radiologists to recognize. Accurate radiologic diagnosis is crucial if a
biopsy—which may cause catastrophic hemorrhaging—is to be avoided.
Patients with cavernous hemangiomas usually present with rectal bleeding and
often undergo a variety of diagnostic tests; yet up to 80% of patients are
subjected to an unnecessary surgical procedure before the correct diagnosis is
made [1]. In fact, the average
elapsed time between presentation and diagnosis has been reported to be 19
years [2].
CT colonography, or "virtual colonoscopy," is an accurate
noninvasive screening test for colorectal polyps and cancer
[3,
4]. CT colonography is also
useful for cancer staging and problem solving, especially when colonoscopy is
unsuccessful. In addition, this technique may be used to evaluate nonmalignant
disease of the colon. In the case of cavernous hemangiomas, CT colonography
offers key diagnostic information that cannot be provided by conventional
radiography, barium enema, angiography, or endoscopy. Our patient represents a
case of colonic cavernous hemangiomatosis that was confirmed on CT
colonography. We discuss pathognomonic findings and diagnostic information not
readily available through other modalities. To our knowledge, the findings of
colonic hemangiomatosis on three-dimensional (3D) CT colonography have not
been previously reported.
Case Report
A 44-year-old man presented with a 25-year history of intermittent episodes
of rectal discharge of bright red blood and cramplike abdominal pain.
Conventional colonoscopy showed extensive hypervascular submucosal lesions
that were suspected to represent cavernous hemangiomatosis, although no biopsy
was performed because of the risk of hemorrhage (Fig.
1A,1B,1C,1D,1E,1F).
Arteriographic results were also suggestive of cavernous hemangiomatosis,
revealing colonic hypervascularity with delayed venous pooling. The patient
was referred for 3D CT colonography to further characterize these lesions and
to evaluate the extent of disease.
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Fig. 1A. —44-year-old man with 25-year history of intermittent episodes
of rectal discharge of bright red blood. Conventional colonoscopic image shows
submucosal vascular masses protruding into rectal lumen and intraluminal
hemorrhage.
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Fig. 1B. —44-year-old man with 25-year history of intermittent episodes
of rectal discharge of bright red blood. Axial CT scan obtained during virtual
colonoscopy of air-insufflated colon at level of upper sacrum shows marked
thickening of rectosigmoid wall with intramural phleboliths. Multiple small
mesenteric soft-tissue masses are also seen.
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Fig. 1C. —44-year-old man with 25-year history of intermittent episodes
of rectal discharge of bright red blood. Axial CT scan obtained during virtual
colonoscopy of air-insufflated colon at level of lower lumbar spine shows
thickening of ascending colon with few intramural phleboliths. One area is
closely opposed and possibly adherent to anterior abdominal wall. As in
B, innumerable small mesenteric masses are visible.
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Fig. 1D. —44-year-old man with 25-year history of intermittent episodes
of rectal discharge of bright red blood. Volume-rendered image derived from
axial CT examination presented in coronal oblique projection shows multiple
abdominal phleboliths outside pelvic venous plexus.
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Fig. 1E. —44-year-old man with 25-year history of intermittent episodes
of rectal discharge of bright red blood. Three-dimensional CT colonogram
simulates single-contrast barium enema, showing multiple submucosal masses,
with most prominent masses visible in rectosigmoid.
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Fig. 1F. —44-year-old man with 25-year history of intermittent episodes
of rectal discharge of bright red blood. Three-dimensional CT colonogram
simulates double-contrast barium enema, revealing additional mucosal detail,
with multiple irregular sessile and polypoid masses.
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Overnight preparation of the patient's bowel was performed. We insufflated
the colon with approximately 1 L of room air and administered 120 mL of
nonionic contrast material at a rate of 3 mL/sec through an 18-gauge
antecubital venous catheter. We waited 25 sec after starting the injection
before we began to scan the supine patient with a multidetector CT scanner
(Volume Zoom; Siemens Medical Systems, Iselin, NJ). The 4 x 1 mm
collimator setting was used to obtain 1.25-mm slices at 1-mm intervals. After
the completion of the supine position scanning, the patient was placed in the
prone position and the acquisition protocol, repeated. The data was
transferred to a workstation running 3D Virtuoso software (Siemens Medical
Systems).
Axial CT scans showed marked colonic wall thickening caused by confluent
submucosal polypoid lesions with heterogeneous enhancement; the lesions were
most prominent in the rectosigmoid and ascending colon. Multiple round
calcifications were noted in the areas of wall thickening, a finding
compatible with phleboliths in cavernous hemangiomas. No direct infiltration
of adjacent soft-tissue structures was seen. However, innumerable abnormal
mesenteric soft-tissue masses were believed most likely to represent
involvement by additional hemangiomas and to be a component of dilated feeding
and draining vessels.
Volume-rendered imaging depicted the extent of colonic involvement from a
3D perspective. Coronal volume rendering showed multiple abdominal phleboliths
outside their usual location in the pelvic venous plexus. A simulated
single-contrast barium enema allowed the visualization of submucosal lesions
throughout the colon. A simulated double-contrast barium enema revealed
additional mucosal detail.
Discussion
Colonic hemangiomas are rare benign lesions arising from the submucosal
vascular plexus that are attributable to embryonic sequestration of mesodermal
tissue [5]. Hemangiomas are
histologically distinct from telangiectasias and angiodysplasias.
Approximately 80% of colonic hemangiomas are of the cavernous type. Cavernous
hemangiomas can be distinguished from the capillary type of hemangiomas, which
are usually solitary and cause no symptoms. Cavernous hemangiomas are composed
of large thin-walled vascular channels and have no capsule. The characteristic
phleboliths arise because of thrombosis from inflammation or stasis
[2]. Similar lesions may also
coexist in the skin, central nervous system, and elsewhere in the
gastrointestinal tract and accessory organs of digestion. Full-thickness mural
involvement is typical, often with infiltration into the surrounding
connective tissue and occasionally into adjacent organs. Lesions may be
characterized as discrete or diffuse, and extensive cases of the latter have
been described as "hemangiomatosis."
Cavernous hemangiomas of the colon show a tendency to run in families and
are also associated with Klippel-Trénaunay-Weber syndrome; blue rubber
bleb nevus syndrome; and vertebral defects, imperforate anus,
tracheoesophageal fistula, and radial and renal dysplasia (VATER) complex
[6]. The skin is most commonly
affected, and the clinical presentation of skin lesions and rectal bleeding is
highly suggestive of colonic involvement. The colonic lesions are most often
distal, involving the rectum in up to 70% of cases. The average age of the
patient at presentation is 12 years. The disease seems as likely to afflict
males as females [5].
These lesions are a significant cause of rectal bleeding in children and
young adults. Presenting symptoms include frank bleeding (60-90%), anemia
(43%), obstruction (17%), and, rarely, platelet sequestration
[5], although approximately 10%
of patients remain asymptomatic. Up to 80% of patients undergo an unnecessary
surgical procedure before an accurate diagnosis is made
[1], with an average delay of
19 years between appearance of the initial symptoms and diagnosis
[2]. Common mimicking lesions
include internal hemorrhoids, adenomatous polyps, carcinoma, inflammatory
bowel disease, and proctitis
[1,
7].
Radiographic diagnosis relies on the detection of clustered phleboliths,
particularly in unusual locations or in young patients. This radiographic
finding is only 50% sensitive
[1,
7], but when present, it offers
reasonable specificity, particularly in young patients. In one series, only
four of 12,000 healthy 12-year-old children had pelvic phleboliths on
radiographs [3]. Barium enema
reveals nonspecific polypoid or multilobular annular masses that may collapse
with air insufflation. Classically, angiography of cavernous hemangiomas shows
mural hypervascularity with delayed venous pooling; however, this finding is
complicated by hypovascularity in regions of thrombosis.
On endoscopy, cavernous hemangiomas characteristically present as nodular,
compressible lesions that are deep blue to dull red and are associated with
mucosal congestion and edema. Unfortunately, chronic inflammatory changes
often mask findings that could lead to proper diagnosis. Biopsy may cause
profuse hemorrhaging, partially because of decreased smooth muscle in the
abnormal vascular channels.
CT colonography offers key diagnostic information that is not provided by
conventional radiography, barium enema, angiography, or endoscopy. With
adequate bowel cleansing, barium enema, and air distention, mucosal lesions
and intraluminal characteristics of submucosal lesions can more easily be
evaluated with noninvasive CT colonography than with axial CT alone. CT
colonography allows one to make a confident diagnosis of colonic
hemangiomatosis and to gain a more complete knowledge of the characteristics
and distribution of lesions throughout the colon from a single 3D image.
Pathognomonic findings consist of inhomogeneously enhancing transmural
bowel-wall thickenings containing phleboliths. CT is more sensitive for
phleboliths than are radiographs or barium enemas and can be used to
accurately confirm whether phleboliths are within areas of the thickened bowel
wall, rather than in the pelvic veins. A primary advantage of choosing CT over
endoscopy is that wall thickening and extramural extension can be accurately
evaluated. In fact, many mimicking lesions on endoscopy are mucosal
inflammatory processes, most of which would be excluded by the visualization
of transmural wall thickening on CT colonography.
Surgical resection is the definitive therapy for symptomatic colonic
hemangiomas. Other measures that have been used in selected patients include
sclerotherapy, polypectomy, electrocautery, embolization, and irradiation. Our
patient has elected to continue receiving conservative follow-up rather than
to undergo a specific therapy at this time. He has symptoms only
intermittently, and he would require a total colonectomy for cure because of
the diffuse distribution of his lesions.
In summary, the radiologist has an important role in detecting cavernous
hemangiomas early, thereby obviating further testing and unnecessary
procedures that may carry with them a high risk of morbidity.
References
- Perez C, Andreu J, Llauger J, Valls J. Hemangioma of the rectum: CT
appearance. Gastrointest Radiol
1987;12:347
-349[Medline]
- Djouhri H, Arrive L, Bouras T, Martin B, Monnier-Cholley L, Tubiana
JM. Diffuse cavernous hemangioma of the rectosigmoid colon: imaging findings.
J Comput Assist Tomogr
1998;22:851
-855[Medline]
- Yeoman LJ, Shaw D. Computerized tomography appearances of pelvic
haemangioma involving the large bowel in childhood. Pediatr
Radiol 1989;19:414
-416[Medline]
- Yee J, Akerkar GA, Hung RK, Steinauer-Gebauer AM, Wall SD, McQuaid
KR. Colorectal neoplasia: performance characteristics of CT colonography for
detection in 300 patients. Radiology
2001;219:685
-692[Abstract/Free Full Text]
- Lyon DT, Mantia AG. Large bowel hemangiomas. Dis Colon
Rectum 1984;27:404
-414[Medline]
- Fenlon HM, Nunes DP, Schroy PC 3rd, Barish MA, Clarke PD, Ferrucci
JT. A comparison of virtual and conventional colonoscopy for the detection of
colorectal polyps. N Engl J Med
1999;341:1496
-1503[Abstract/Free Full Text]
- Bortz JH. Diffuse cavernous hemangioma of the rectum and sigmoid.
Abdom Imaging
1994;19:18
-20[Medline]
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Introduction
Case Report
