Case Report

Diffuse Cavernous Rectal Hemangioma -Sphincter-Sparing Approach to Therapy Report of a Case

JAMES A. CUNNINGHAM, M.D., VICTOR F. GARCIA, M.D., GUILLERMO QUISPE, M . D .

Cunningham JA, Garcia VF, Quispe G. Diffuse cavernous rectal hemangioma--sphincter-sparing approach to therapy: report of a case. Dis Colon Rectum 1989;32:344-347.

Diffuse cavernous rectal hemangioma is described through a case his- tory. Presentation, diagnostic work up, pathophysiology, and therapy for this condition are reviewed. Emphasis is placed on rectal sparing surgical techniques in the management of this disease process that affects young, otherwise healthy individuals. [Key words: Hemangi- oma; Hematochezial

DIFFUSE CAVERNOUS RECTAL hemangioma is a singu- larly rare lesion, with only 100 cases having been reported. The age range of these patients is 5 to 25 years and the principal presenting complaint is painless, mas- sive, rectal bleeding. The treatment of choice for symp- tomatic patients is complete surgical excision, classically with abdominoperineal resection. Use of sphincter- sparing procedures in the treatment of this condition has been described infrequently. With the heightened empha- sis on functional preservation in this young, otherwise healthy, patient population, we recently applied the principles of mucosectomy and coloanal pull-through in the management of a patient with intractable, life- threatening hemorrhage from cavernous rectal hemangio- ma.

Report of a Case

A 27-year-old Puerto Rican man was admitted to the hospital with a three-year history of painless rectal bleeding. His hematochezia had been intermittent, often massive, and exacerbated by bowel move-

From the Walter Reed Army Medical Center, Washington, D.C.

ments. Over 50 units of blood were transfused during the interval preceding definitive surgery. Angiographic embolization of the infe- rior mesenteric artery was unsuccessful in abating the frequency of severity of bleeding episodes.

Physical examination revealed a thin, pale, young man with no evidence of mucocutaneous vascular lesions. Colonoscopy document- ed bluish, submucosal lesions extending from the dentate line proxi- mally for 15 cm. Additionally, areas of mucosal erosion with evidence of recent hemorrhage were noted. The hematocrit was 28 and the coagulation profile was normal.

Plain film of the abdomen was normal and a barium enema pre- viously revealed polypoid submucosal rectal masses (Fig. 1). Delayed film of an inferior mesenteric and internal iliac artery showed evidence of vascular tufts in the rectal area (Fig. 2). The overall vasculature of the inferior mesenteric artery system was markedly decreased compared with similar views taken before embolization.

At operation, the hemangioma was visibly limited to the rectum (Fig. 3). The sigmoid colon was fully mobilized and then proximally divided. The rectum and mesorectum were dissected to the level of the levator ani with suture ligation of the lateral vascular stalks. The hemangioma infiltrated the bowel wall at this level and spread extramurally onto the pelvic side walls. The rectum was divided 2 cm proximal to the levators and the distal free edge was oversewn with a running, silk suture, effectively controlling the bleeding from the incised rectal cuff. Transanally, the submucosa was infiltrated with a 1:200,000 epinephrine solution and the mucosa and submucosa were stripped, using fine-needle electrocautery and sharp dissection, The normal descending colon was brought down through the muscular cuff and anastomosed to the anoderm with interrupted polyglactin sutures. The pelvis was drained with active, soft-suction catheters, brought out laterally through the abdominal wall. A protective trans- verse loop colostomy was formed in the right upper quadrant. Esti-

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Volume 32 Number .t I)IFFUSI! CAVERNOUS REC~I'AL HEMANGIOMA 345

FIG. 1. Barium enema revealing notch- ing in rectosigmoid area due to submu- cosal masses, here representing the heman- gioma.

mated blood loss was 2500 cc and 4 units of packed red blood cells were given. There were no operative complications and postoperatively the patient noted normal sexual function. Four months later an anal stenosis was noted, and was managed successfully with dilatation. The colostomy was closed in the filth postolzx'rative month. He has con- tinued to do well in the ensuing 18 months with no further bleeding and satisfactory bowel habits.

Discussion

In 1839, P h i l l i p s t descr ibed h e m a t o c h e z i a f r o m intes t i -

na l h e m a n g i o m a . Di f fuse c a v e r n o u s recta l h e m a n g i o m a

is the m o s t agg re s s ive f o r m of the b e n i g n in t e s t ina l vascu-

lar m a l f o r m a t i o n s . T h e s e h e m a n g i o m a s g e n e r a l l y are

c o n f i n e d to the r e c t o s i g m o i d , a l t h o u g h s o m e pa t i en t s

h a v e assoc ia ted m u c o c u t a n e o u s va scu l a r m a l f o r m a t i o n s .

T h e r e is n o a p p a r e n t sex p r e d i l e c t i o n a n d pa t i en t s usu-

a l ly are affected before the i r a d u l t years. I n t e r m i t t e n t

b l o o d loss is c o m m o n a n d d e a t h f r o m e x s a n g u i n a t i o n is n o t i n f r e q u e n t . 2

E n d o s c o p y is the c o r n e r s t o n e for e v a l u a t i o n of such

pa t ien ts . T h e typ ica l f i n d i n g s are b l u i s h s u b m u c o s a l

FIG. 2. Arteriographic study of the infe- rior mesenteric artery revealing vascular tufts and pooling in the distal superior hemorrhoidal branches.

Dis. Gol. &" Re~:t. 346 C U N N I N G H A M , E T AI . . April 1989

FIG. 3. View of mobilized rectosigmoid revealing proximal extent of the diffuse rectal hemangioma.

masses consistent with engorged vascular channels. The proximal extent of the lesion can be delineated precisely. Plain film radiographs may show pelvic phleboliths in the area of the rectal wall. 3 Multiple pelvic phleboliths are

FIG. 4. Photomicrograph of a section of involved rectum showing diffuse hemangiomatous involvement of submucosa infiltrating into the muscular wall.

seen in approximately one fourth of these patients. They are in atypical locations, and are more caudal and medial, as well as in clustered formation, and this may character- ize this lesion. 4 Barium contrast studies typically reveal multiple polypoid lesions in the submucosa. Arteri- ography of the pelvic vessels may be normal or show vascular tufts or delayed venous phase films.

The characteristic histologic features of these lesions are large, thin-walled vessels supported by a scant connec- tive tissue stroma of fibrous and smooth-muscle elements (Fig. 4). Often the condition is infiltrative and may extend through the bowel wall and onto adjacent pelvic tissues, including the bladder. This condition is believed to arise from hamartomatous tissue present at birth that enlarges with bodily development. Malignant transformation is exceedingly uncommon. 5 Differential diagnosis usually is not difficult, and should include local conditions such as hemorrhoids, inflammatory bowel disease, and neo- plasms.

Biopsy of these lesions is contraindicated by the mas- sive l i fe - threa tening hemor rhage that may result. Attempts at arterial embolization of feeding vessels have resulted in marked reduction in inferior mesenteric artery flow, al though without consistent improvement in the patient's condition. It remains that surgical extirpation is the only established successful therapy.

Abdominoperineal resection has been the procedure most often recommended and is effective, with a low incidence of recurrent hemorrhage. 4 The published expe- rience with sphincter-saving procedures in the manage- ment of this condition is limited. Parker et al . , 6 in 1976, described six patients in whom a coloanal pull-through technique was used. A long, 10 to 12 cm rectal cuff was left

Volume 32 Number 4 DIFFUSE CAVERNOUS RECTAL HEMANGIOMA 347

and rectal b l eed ing pers is ted in two pat ients . Recent ly , W a n g 7 r epor t ed two pa t ien ts s imi la r ly treated in China , us ing a shor t rectal cuff, w i th acceptable results.

T h e first i l eoana l sleeve anas tomos is after rectal resec- t ion was descr ibed in 1947 by Ravi tch s a n d Sabis ton. Soave, 9 in 1964, ref ined this t echn ique in ch i ld ren wi th H i r s c h s p r u n g ' s disease to inc lude rectal resect ion wi th m u c o s e c t o m y of the r e t a ined m u s c u l a r rectal cuff a n d a co loana l p u l l - t h r o u g h anas tomosis . T h e rectal mucosa is no t essential for p reserva t ion of cont inence . In tegr i ty of the ana l sphinc te rs and the levator an i c o m p l e x is mos t i m p o r t a n t in p reserv ing the sensat ion and m o t o r cont ro l necessary for vo luntary , con t inen t defecation.I~ Accepta- ble results have been real ized wi th this t echn ique in a wide a r ray of anorec ta l condi t ions . In this r epor t we present a typica l case of diffuse cavernous rectal h e m a n - g ioma , successfully treated wi th a r e c t u m - s p a r i n g tech- n ique. T h e absence of pos tope ra t ive b l eed ing indicates that the p u l l e d - t h r o u g h colon isolates the res idual heman- g i o m a in the m u s c u l a r rectal cuff a n d pe lv ic s idewal ls f rom defecatory t rauma.

We bel ieve tha t the shor t rectal cuff is i m p o r t a n t for m i n i m i z a t i o n of the a m o u n t of re ta ined h e m a n g i o m a , and that the necessary d issect ion of the r ec tum low in to

the pelvis can be done wi th acceptab le morb id i ty . We p ropose wider a p p l i c a t i o n of this p rocedure to the unu- sual condi t ion .

References

1. Phillips B. Surgical cases. London Med Gaz 1838-9;23:514-7. 2. Shackelford RT, Zuidema GD. Surgery of the alimentary tract. 2nd

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the treatment of diffuse cavernous hemangioma involving the rectum. Br J Surg 1976;63:678-82.

4. Coppa GF, Eng K, Localio SA. Surgical management of diffuse cavernous hemangioma of the colon, rectum and anus. Surg Gynecol Obstet 1984;159:17-22.

5. Robbins SL, Angell M. Basic pathology. 2rid ed. Philadelphia: WB Saunders, 1976:285-6.

6. Parker GW, Murney JA, Kenoyer WL. Cavernous hemangioma of the rectum and rectosigmoi& a case report and review. Dis Colon Rectum 1960;3:358-63.

7. Wang C-H. Sphincter-saving procedure for treatment of diffuse cavernous hemangioma of the rectum and sigmoid colon. Dis Colon Rectum 1985;28:604-7.

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9. Soave F. Hirschsprung's disease: a new surgical technique. Arch Dis Child 1964;39:116-24.

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