Arteriovenous Malformation of the U p p e r Rectum in an Unusual Site: Report of a Case and Review of the Literature*
ADIL H . A L - H U M A D I ,
M.D., ALFRED
F. DOMALESKI,
M.D., J.
EDWIN ALFORD, M . D .
Olean, New York; Coudersport, Pennsylvania; Buffalo, New York
ANGIODYSPLASIA ( v a s c u l a r e c t a s i a , t e l a n g i e c t a s i a , hamartoma, hemangioma) primarily involves the right colon. Patients are usually middle aged and older. Methods of detecting intermittent rectal bleedi n g , s u c h as b a r i u m - e n e m a studies and, colonoscopic and mesenteric angiographic examinations, have routinely failed to demonstrate this vascular anomaly. We present a case of a vascular anomaly in the upper rectum, an unusual location not previously reported. Exploratory laparotomy and pathologic studies of the specimen confirmed the diagnosis.
Report of a Case A 32-year-old, mentally retarded man was admitted to the hospital with a 5-month history of intermittent rectal bleeding of bright, fresh blood that was not related to bowel movements. The patient's foster mother mentioned that he had had a severe episode of rectal bleeding associated with shock, which had necessitated blood transfusion in a nearby community hospital one and a half years prior to this admission. The final diagnosis had been necrotic lesion of the upper anterior rectum (about 10 cm from the anal verge) with hemorrhage of undetermined origin. The patient had no other gastrointestinal symptom, and careful history excluded a self-inflicted injury, or homosexuality. His general health was good. Results of physical examination were unremarkable except for sinus bradycardia with a ventricular rate of 43/min. Sigmoidoscopic examination showed an ulcerative, inflammatory lesion extending from the 10-15 cm level on the anterior wall of the upper rectum, and involving half the rectal circumference. The posterior rectal wall at that level was normal. Test results included hemoglobin, 15 g--dl; hematocrit, 44 per cent. Coagulation tests showed no abnormality foi tests of stool culture for parasites, tests for venereal disease, complement-fixation test for amebiasis, PPD skin tests for t u b e r c u l o s i s and coccidioidomycosis. SMA 12 results and serum lysozyme levels, and results of barium-enema studies (Fig. 1), colonoscopic and inferior mesenteric examination (Fig. 2), intravenous pyelogram, and cystogram were all normal. The only positive finding resulted from sigmoidoscopic examination, with biopsy: the biopsy specimen re~ealed chronic ulcerative changes. T h e patient was treated t e m p o r a r i l y with azulfidine a n d therapeutic cortisone-enemas for colitis, without improvement. He was then treated ~ith tetracycline for 10 days to rule out lymphogranuloma, again, with no improvement. T h e patient f n a l y t, nderwent exploratory laparotomy. Using a proctoscope, we tat* Received for publication September 28, 1978. Address reprint requests to Dr. Alford: 50 High Street, Suite 901, Buffalo, New York 14203.
tooed the area of rectal ulceration with methylene blue. To our surprise, the operative field contained an unusual vascular lake that had been stained a clear blue in a subserosal vessel (like a bag of worms), at the upper rectosigmoidaljunction (Fig. 3). A pulsating vessel could be felt in the area. Upper anterior resection was performed, and the vessel was immediately isolated through the injection of methylene blue. This resulted in immediate discoloration of the well-demarcated ulcerated segment (Fig. 4). Pathologic findings confirmed the presence of the vascular hamartoma, which was 6 cm in diameter, with a medium-sized artery in the submucosa (Fig. 5). The arteries showed areas of fibrous proliferation of the intima and reduplication of the internal elastica. The muscularis propria appeared to be slightly hypertrophied, with somewhat tortuous-appearing, large thin-walled blood vessels that resembled abnormal thin-walled veins (angiomatous malformation; (Fig. 6). T h e increased n u m b e r of vascular spaces in the mucosa and submucosa near the ulcer were considered part of this vascular hamartomatous anomaly (Fig. 7). The serosa showed fibrous thickening with leukocytic infiltration. T h e postoperative course was uneventful. The patient has been well for the past two years, and there has been no recurrence of rectal bleeding.
Fto. 1.
Barium-enema study with spot film of normal rectum.
0012-3706/79/0400/0189/$00.80 9 American Society of Colon and Rectal Surgeons
189
190
AL-HUMADI,
ET
AL.
Dis. Col. April& Rect. 1979
Discussion It is s o m e t i m e s m o r e difficult to d e t e r m i n e the p r e s e n c e o f a colonic vascular a n o m a l y t h a n it is to identify the a n o m a l y ' s f r e q u e n t l y obscure etiology. H o w e v e r , the source o f i n t e r m i t t e n t , chronic gastrointestinal b l e e d i n g a n d s o m e t i m e massive h e m o r r h a g i n g is usually successfully d i a g n o s e d t h r o u g h conventiona[ m e t h o d s such as b a r i u m - e n e m a studies, c o l o n o s c o p i c a n d selective a n g i o g r a p h i c e x a m i n a tions, a n d e v e n m a g n i f i c a t i o n a r t e r i o g r a p h y , but rarely through exploratory laparotomy. Vascular ecstasia can n e i t h e r be seen n o r palpated; angiography, 2,a''-'~ usually the m o s t reliable m e t h o d o f d e n > onstrating this a n o m a l y reveals a large cluster o f small arteries along the a n t i m e s e n t e r i c b o r d e r d u r i n g the arterial phase, contrast m e d i u m in the vascular space and opacification o f bowel wall in the capillary-blush
Fro. 2. Inferior mesenteric angiographic study, showing norreal vascular structtlre.
Fro. 3. Operating room view, showing subserosal vascular discoloration at the upper rectum after endoscopic tattooing of the ulcer.
Fro. 4. Methylene blue discoloration of the localized ulceration in the upper rectum after injection into the inferior mesenteric vessel.
Volume 22
N~mbe~ 3
RECTAL ARTERIOVENOUS
MALFORMATION
19 1
Fro. 5. P h o t o m i c r o g r a p h , s h o w i n g the mucosal def`ect at the base of` the vascular anomaly, a n d the increased the n u m b e r of" vascular spaces in m u c o s a a n d s u b m u c o s a (medium-sized artery).
phase, and early opacification o f the venous system in the venous phase. In this case, the nature of the ulcerative segment was d e t e r m i n e d by exclusiort of more c o m m o n lesions. Most of the vascular anomalies are not visible on gross examination. However, the unique ulceration o f the anterior wall and the noninvolvement o f the posterior wall o f the rectosigmoid area hardly constituted p r o o f o f segmental ulcerative colitis or Crohn's disease. A diagnosis of ischemic proctosigmoiditis 9 was excluded because the patient was so young, and because local involvement has never been reported. Although sinus bradycardia may have been
a contributing factor, the specific events triggering the localized ulceration could not be d o c u m e n t e d . T h e r e was no m e m b r a n o u s , patchy, superficial ulceration in the mucosa, n o r were there microthrombi in the s u b m u c o s a l vessels. H e r e d i t a r y h e m o r r h a g i c telangiectasia la ( O s l e r - W e b e r - R e n d u disease) was ruled out, since there was no family history of the disease and the patient did not manifest any o f the characteristic lesions o f the skin, n a s o p h a r y n g e a l mucous m e m b r a n e , or gastrointestinal tract. H e m a n g i o m a t o s i s o f the c o l o n a n d r e c t o s i g moid
ADIL H . A L - H U M A D I ,
M.D., ALFRED
F. DOMALESKI,
M.D., J.
EDWIN ALFORD, M . D .
Olean, New York; Coudersport, Pennsylvania; Buffalo, New York
ANGIODYSPLASIA ( v a s c u l a r e c t a s i a , t e l a n g i e c t a s i a , hamartoma, hemangioma) primarily involves the right colon. Patients are usually middle aged and older. Methods of detecting intermittent rectal bleedi n g , s u c h as b a r i u m - e n e m a studies and, colonoscopic and mesenteric angiographic examinations, have routinely failed to demonstrate this vascular anomaly. We present a case of a vascular anomaly in the upper rectum, an unusual location not previously reported. Exploratory laparotomy and pathologic studies of the specimen confirmed the diagnosis.
Report of a Case A 32-year-old, mentally retarded man was admitted to the hospital with a 5-month history of intermittent rectal bleeding of bright, fresh blood that was not related to bowel movements. The patient's foster mother mentioned that he had had a severe episode of rectal bleeding associated with shock, which had necessitated blood transfusion in a nearby community hospital one and a half years prior to this admission. The final diagnosis had been necrotic lesion of the upper anterior rectum (about 10 cm from the anal verge) with hemorrhage of undetermined origin. The patient had no other gastrointestinal symptom, and careful history excluded a self-inflicted injury, or homosexuality. His general health was good. Results of physical examination were unremarkable except for sinus bradycardia with a ventricular rate of 43/min. Sigmoidoscopic examination showed an ulcerative, inflammatory lesion extending from the 10-15 cm level on the anterior wall of the upper rectum, and involving half the rectal circumference. The posterior rectal wall at that level was normal. Test results included hemoglobin, 15 g--dl; hematocrit, 44 per cent. Coagulation tests showed no abnormality foi tests of stool culture for parasites, tests for venereal disease, complement-fixation test for amebiasis, PPD skin tests for t u b e r c u l o s i s and coccidioidomycosis. SMA 12 results and serum lysozyme levels, and results of barium-enema studies (Fig. 1), colonoscopic and inferior mesenteric examination (Fig. 2), intravenous pyelogram, and cystogram were all normal. The only positive finding resulted from sigmoidoscopic examination, with biopsy: the biopsy specimen re~ealed chronic ulcerative changes. T h e patient was treated t e m p o r a r i l y with azulfidine a n d therapeutic cortisone-enemas for colitis, without improvement. He was then treated ~ith tetracycline for 10 days to rule out lymphogranuloma, again, with no improvement. T h e patient f n a l y t, nderwent exploratory laparotomy. Using a proctoscope, we tat* Received for publication September 28, 1978. Address reprint requests to Dr. Alford: 50 High Street, Suite 901, Buffalo, New York 14203.
tooed the area of rectal ulceration with methylene blue. To our surprise, the operative field contained an unusual vascular lake that had been stained a clear blue in a subserosal vessel (like a bag of worms), at the upper rectosigmoidaljunction (Fig. 3). A pulsating vessel could be felt in the area. Upper anterior resection was performed, and the vessel was immediately isolated through the injection of methylene blue. This resulted in immediate discoloration of the well-demarcated ulcerated segment (Fig. 4). Pathologic findings confirmed the presence of the vascular hamartoma, which was 6 cm in diameter, with a medium-sized artery in the submucosa (Fig. 5). The arteries showed areas of fibrous proliferation of the intima and reduplication of the internal elastica. The muscularis propria appeared to be slightly hypertrophied, with somewhat tortuous-appearing, large thin-walled blood vessels that resembled abnormal thin-walled veins (angiomatous malformation; (Fig. 6). T h e increased n u m b e r of vascular spaces in the mucosa and submucosa near the ulcer were considered part of this vascular hamartomatous anomaly (Fig. 7). The serosa showed fibrous thickening with leukocytic infiltration. T h e postoperative course was uneventful. The patient has been well for the past two years, and there has been no recurrence of rectal bleeding.
Fto. 1.
Barium-enema study with spot film of normal rectum.
0012-3706/79/0400/0189/$00.80 9 American Society of Colon and Rectal Surgeons
189
190
AL-HUMADI,
ET
AL.
Dis. Col. April& Rect. 1979
Discussion It is s o m e t i m e s m o r e difficult to d e t e r m i n e the p r e s e n c e o f a colonic vascular a n o m a l y t h a n it is to identify the a n o m a l y ' s f r e q u e n t l y obscure etiology. H o w e v e r , the source o f i n t e r m i t t e n t , chronic gastrointestinal b l e e d i n g a n d s o m e t i m e massive h e m o r r h a g i n g is usually successfully d i a g n o s e d t h r o u g h conventiona[ m e t h o d s such as b a r i u m - e n e m a studies, c o l o n o s c o p i c a n d selective a n g i o g r a p h i c e x a m i n a tions, a n d e v e n m a g n i f i c a t i o n a r t e r i o g r a p h y , but rarely through exploratory laparotomy. Vascular ecstasia can n e i t h e r be seen n o r palpated; angiography, 2,a''-'~ usually the m o s t reliable m e t h o d o f d e n > onstrating this a n o m a l y reveals a large cluster o f small arteries along the a n t i m e s e n t e r i c b o r d e r d u r i n g the arterial phase, contrast m e d i u m in the vascular space and opacification o f bowel wall in the capillary-blush
Fro. 2. Inferior mesenteric angiographic study, showing norreal vascular structtlre.
Fro. 3. Operating room view, showing subserosal vascular discoloration at the upper rectum after endoscopic tattooing of the ulcer.
Fro. 4. Methylene blue discoloration of the localized ulceration in the upper rectum after injection into the inferior mesenteric vessel.
Volume 22
N~mbe~ 3
RECTAL ARTERIOVENOUS
MALFORMATION
19 1
Fro. 5. P h o t o m i c r o g r a p h , s h o w i n g the mucosal def`ect at the base of` the vascular anomaly, a n d the increased the n u m b e r of" vascular spaces in m u c o s a a n d s u b m u c o s a (medium-sized artery).
phase, and early opacification o f the venous system in the venous phase. In this case, the nature of the ulcerative segment was d e t e r m i n e d by exclusiort of more c o m m o n lesions. Most of the vascular anomalies are not visible on gross examination. However, the unique ulceration o f the anterior wall and the noninvolvement o f the posterior wall o f the rectosigmoid area hardly constituted p r o o f o f segmental ulcerative colitis or Crohn's disease. A diagnosis of ischemic proctosigmoiditis 9 was excluded because the patient was so young, and because local involvement has never been reported. Although sinus bradycardia may have been
a contributing factor, the specific events triggering the localized ulceration could not be d o c u m e n t e d . T h e r e was no m e m b r a n o u s , patchy, superficial ulceration in the mucosa, n o r were there microthrombi in the s u b m u c o s a l vessels. H e r e d i t a r y h e m o r r h a g i c telangiectasia la ( O s l e r - W e b e r - R e n d u disease) was ruled out, since there was no family history of the disease and the patient did not manifest any o f the characteristic lesions o f the skin, n a s o p h a r y n g e a l mucous m e m b r a n e , or gastrointestinal tract. H e m a n g i o m a t o s i s o f the c o l o n a n d r e c t o s i g moid
