Introduction

Diffuse cavernous haemangiomas of the rectum (DCHR) are rare. Usually confused with anorectal conditions that present with bright red bleeding, its correct treatment is commonly delayed(1). Up to 80% of patients with the DCHR have undergone a minimum of one inappropri-ate surgical procedure due to misdiagnosis (2). Proper understanding of the condition and careful use of imag-ing modalities is essential in its early treatment (3). With the paucity of resources in the developing world, the di-agnostic challenge is even more daunting. We present a case of DCHR in a 24 year old managed at our hospital. To our knowledge, this is the first reported case of DCHR in the East African literature.

 

Case Report

A 24 year old female presented with a five month history of recurrent fresh and painless rectal bleeding, occasional passage of clots and had a sensation of incomplete rectal emptying. She denied melaena, haematemesis or muco-sal bleeding. There was no history of abdominal pain, altered bowel habits (no constipation or diarrhoea) or weight loss. Her past surgical history was significant for several transfusions for severe anemia, hemorrhoidecto-my for rectal bleeding, left hemicolectomy with primary anastomosis one year earlier for massive lower gastroin-testinal bleeding requiring seven units of blood transfu-sion. She had no family history of malignancy. She was a college student with no history of smoking and only took alcohol occasionally.

 

 

On first encounter at our institution, she was very pale, not dyspnoeic or jaundiced, the patient was well hydrat-ed, had no generalized lymphadenopathy or lower limb oedema. The blood pressure of 100/70 mmHg, a pulse rate of 84 beats per minute and temperature of 36.8oC. She was fully oriented and had a normal respiratory and cardiovascular examination. Abdominal examination revealed a non-tender abdomen, a well-healed laparoto-my scar and no palpable masses. Digital rectal examina-tion revealed a normal anal tone, smooth anorectal mu-cosa, no masses, and soft stools which were non-bloody. Anoscopy was essentially non-revealing.

 

Her haemoglobin level was 3.6mg/dl and she received four units of packed cells at admission. The coagulation screen was normal. She was prepared for colonoscopy and the findings are shown on Fig. 1. Bleeding contin-ued after colonoscopy necessitating tranfusion of one more unit of packed red blood cells. A CT scan abdo-men done revealed the typical thickening of the rectal wall with multiple serpentine vessels and phleboliths (Fig 2a-c).She was counselled for low anterior resec-tion and coloanal anastomosis. After adequate colonic preparation, her pre-operative haemoglobin was 10.8g/ dl and three more units of packed cells were available for surgery. Through a midline abdominal incision, the pelvis was explored, rectum mobilised and an arterio-venous malformation (AVM) extending from the recto-sigmoid junction to the anorectal junction was found. The AVM was carefully excised and hand-sewn coloanal anastomosis done with a diverting loop transverse colostomy fashioned.

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Click to view figure 1

 

 

Total operative time was four hours and total blood loss was 250mls. She had an unevent-ful post-operative period and was discharged on the 9th postoperative day.

 

On follow up in the surgical outpatient clinic, a gastro-graffin enema revealed stenosis at the anastomosis and rectovaginal fistula 1.5cm from the anal verge. The latter has subsequently closed spontaneously and she is due for colostomy closure. The stenosis responded to serial anal dilatations.

 

The surgical specimen (Fig 3) was sent for histology and confirmed the diagnosis of Diffuse Cavernous Haeman-gioma of the Rectum.

 

Discussion

Diffuse Cavernous Haemangioma of the Rectum (DCHR) is a rare benign lesion described in all age groups and may present with severe bleeding per rec-tum. It was first described by Philips in 1839 since when about 100 cases of DCHR have been reported (4). It may mimic internal haemorrhoids, ulcerative colitis, carci-noma, and adenomatous polyp in patients who present ith a history of intermittent painless bright red rectal bleeding. Hence, a misdiagnosis is not uncommon (1). The delay time between initial symptoms and final diag-nosis ranges from 8 to 50 years(5). The mean delay time between initial symptoms and final diagnosis was 17.63 years (range = 0–48 years) in one series of 17 patients where seven patients had been misdiagnosed as hav-ing haemorrhoids with one undergoing at least seven haemorrhoidectomies(6). In the developing world, the challenges are amplified due to the lack of adequate re-sources to properly investigate this condition. Our case illustrates these challenges as the patient had undergone both a haemorrhoidectomy and left hemicolectomy, with the diagnosis being made much later after referral to a center with investigative resources.

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The submucosal vascular plexuses that form the colonic hemangiomas, result from embryonic sequestration of mesodermal tissue. Histologically distinct from telan-giectasias and angiodysplasias, hemangiomas are clas-sified as capillary, cavernous, or mixed(1). Cavernous hemangiomas, which comprise 70% of colonic haeman-giomas, may occur as part of multisystemic or diffuse

 

 

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Click to view figure 3

 

 

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gastrointestinal angiomatosis, with the rectosigmoid as the most common site of occurrence (7).

 

 

Even though proctosigmoidoscopy may confirm the di-agnosis in some cases, a careful biopsy with its potential hazards of causing profuse haemorrhage may remain the only option in most others. Characteristic endoscop-ic findings are of soft, dilated, easily collapsible submu-cosal masses, ranging in color from deep wine to plum (8). Computed tomography (CT) scans can give an accurate diagnosis, showing a thickened rectosig-moid colon and typical pelvic phleboliths, and possibly, the extrarectal lesions (9). In addition, it can provide a pertinent preoperative information including longitudi-nal extent of bowel involvement, degree of bowel wall thickening, involvement of adjacent pelvic viscera, and multiplicity and vascularity of the lesion(3). Abdominal radiography which might at times be the only available modality in resource poor settings may reveal the char-acteristic multiple pelvic phleboliths but the positive rate of abdominal radiography can be as low as 26–50% in adult patients (5, 6).

 

Due to its origin from the dentate line and the involve-ment of the whole layer of the rectal wall and the rectal mesentery, the treatment of choice for DCHR is com-plete resection by pull-through transection and coloanal anastomosis(6). With the development of surgical skills and the application of the double-staple technique, low anterior resection (LAR) was advocated for the treatment of DCHR (8).

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In conclusion, DCHR is a rare condition that may mimic other causes of lower gastrointestinal bleeding. It must be considered in the differential diagnosis of recurrent rectal bleeding and adequate and timely investigations done in order to institute the correct treatment.

 

 

References

 

1. Lyon DT, Manita AG. Large-bowel hemangiomas. Dis Colon Rectum 1984;27:404-14.

2. Jeffery PJ, HawleyPR, Parks AG. Colo-anal sleeve anastomo-sis in the treatment of diffuse cavernous hemangioma in-volving the rectum. Br J Surg 1976;63:678–82.

3. Sharma S, Gandhi D. Role of Computed Tomography in Di-agnosis of diffuse cavernous hemangioma of the rectum. Am J Gastro 1999;94(7):1986-7.

4. Philips B. Surgical cases. Lond Med Gaz 1839;23:514-7.

5. Kandpal H, Sharma R, Srivastava DN. Diffuse cavernous hae-mangioma of colon: magnetic resonance imaging features. Report of two cases. . Australas Radiol 2007;51:B147-B51.

6. Wang HT, Gao XH. Diagnosis and treatment of diffuse cav-ernous hemangioma of the rectum: Report of 17 Cases. World J Surg 2010;DOI 10.:1007/s00268-010-0691-1.

7. Hsu RM, Horton KM, Fishman EK. Diffuse cavernous hem-angiomatosis of the colon: findings on three-dimensional CT colonography. Am J Roentgenol 2002;179:1042–4.

8. Wang AY, Ahmad NA. Diffuse cavernous hemangioma of the colon and rectum. Clin Gastroenterol Hepatol 2007 (8):A25.

9. Yorozuya K, Watanabe M. Diffuse Cavernous Hemangioma of the Rectum: Report of a Case. Surg Today 2003;33:309-11.