Colitis Cystica Profunda: Imaging Diagnosis and Conservative Treatment Report of Two Cases

Manuel Valenzuela, Ph.D.,* Jos~ L. Martin-Ruiz, Ph.D.,* Enrique Alvarez-Cienfuegos, M.D.,]-Antonio M. Caballero, Ph.D.,* Francisco Gallego, M.D.,~: Isabel Carmona, M.D.,:~ Manuel Rodriguez-T~llez, M.D.~

From the *Department of Medicine, School of Medicine, University of Granada, tRadiology Service, University of Granada Hospital, }Gastroenterology Service, University of Granada Hospital, Granada, Spain

Rectally localized colitis cystica profunda can simulate mu- cosecretory carcinoma. PURPOSE AND METHODS: Because endoscopic examination and barium enema do not clarify the diagnosis, other diagnostic imaging methods such as transrectal ultrasonography, computerized tomography, or magnetic resonance imaging are needed. RESULTS: Trans- rectal ttltrasonography identifies multiple cysts in the rectal submucosa, with areas of echorefringent fibrosis between cysts, and confirms the absence of lymph node involvement or invasion of the muscular layer. Findings with computer- ized tomography and magnetic resonance imaging have not previously been described for colitis cystica proftmda. With computerized tomography, the lesion appears as a noninfil- trating entity in the submucosa, with loss of perirectal layers of fatty tissue and thickening of the levator ani muscle. With nuclear magnetic imaging, nodulations produce intense sig- nals that increase in T2, illustrating the mucoprotein con- tent of the cysts. The presence in surgical biopsy material of large, whole cysts confinns the diagnosis. CONCLUSION: Reeducation of bowel habits aimed at avoiding straining and a high-fiber diet together with bulk laxatives can lead to complete remission of lesions in 6 to 18 months. [Key words: Colitis cystica profunda; Imaging techniques; Con- servative treatment]

Valenzuela M, Martin-Ruiz JL, Mvarez-Cienfuegos E, Cabal- lero AM, Gallego F, Carmona I, Rodrlguez-Tdllez M. Colitis cystica profunda: imaging diagnosis and conservative treat- ment. Report of two cases. Dis Colon Rectum 1996;39: 587- 59O.

C olitis cystica profunda, a rare disease character- ized by the presence of mucous cysts in the

colonic submucosa, 1 can be diffuse 2-4 or localized. 5-s

Localized disease usually appears in the distalmost segments of the colon and rectum, 9 and its etiopatho-

genesis has been related with solitary rectal ulcer syndrome and rectal prolapse. 10 Macroscopically, co-

litis cystica profunda can easily be confused with mucosecretory rectal carcinoma. ~1, 12

Preoperative diagnosis is important to avoid unnec- essarily mutilating surgical treatment for a benign en- tity. Endoscopy and barium enema do not provide

Address reprint requests to Dr. Valenzuela: C/O Recogidas 39, E-18005 Granada, Spain.

characteristic signs that distinguish this type of colitis from rectal carcinoma; moreover, routine biopsy fails to find indications of malignancy. Transrectal ultra- sonography, 13 computerized tomography (CT), and

magnetic resonance imaging (MRI) can aid in diag- nosing a benign lesion. Intraoperative transanal mac-

robiopsy 14 provides a definitive diagnosis. Conserva- tive treatment aimed at avoiding straining and

learning regular bowel habits, as is recommended for patients with solitary rectal ulcer and rectal pro- lapse, 15 can lead to complete remission of lesions.

We describe two patients in whom a preoperative diagnosis of colitis cystica profunda was made with the help of imaging techniques. Long-term conserva-

tive treatment led to complete remission of lesions in both patients.

R E P O R T O F T W O CASES

Case 1

A 30-year-old woman (multipara) was seen for anal expulsion of blood and mucus of 15 months duration. Rectal palpation revealed rosary-like cords that were not fixed to deep planes on the anterior right wall of

the rectum. Colonoscopy showed ulcerated, nodular cords and abundant mucus secretion extending along the anterior wall of the rectum from the anal margin to

7 cm from the pectinate line (Fig. la). A large, solitary rectal ulcer was also found. No lesions were seen in higher segments of the colon. Culture of a rectal

lesion swab was negative. Pathologic examination detected proliferative ischemic lesions similar to those of solitary rectal ulcer and rectal prolapse, but no signs of malignancy. A barium enema revealed mul- tiple nodular rectal lesions, which were suggestive of rectal carcinoma (Fig. 2).

Transrectal ultrasonography with a linear 5-MHz probe showed multiple hypoechoic and anechoic

587

588 VALENZUELA ETAL Dis Colon Rectum, May 1996

Figure 1. Colonoscopy: nodular lesions in the submuco- sal layer of the rectal wall, suggesting malignancy. After conservative treatment, only cicatricial fibrosis is ob- served.

Figure 3. Transrectal ultrasonography: multiple hypo- echoic or anechoic cysts limited to the submucosa.

Figure 2. Barium enema: nodular rectal lesions simulat- ing rectal carcinoma.

cysts with posterior acoustic enhancement, ranging in

size from 2 to 3 mm and from 1 to 2 cm. Cysts did not affect the muscularis propria. Hyperechogenic bands corresponding to zones of fibrosis were seen between cysts. No lymph node involvement or invasion of

deep layers of the rectum was noted (Fig. 3). Computerized tomography showed submucosal in-

filtration of the rectal wall, which caused asymmetry and thickening of the levator ani muscle on the side of the lesion. No local adenopathies were evident

(Fig. 4). Findings with magnetic resonance imaging were

similar to those with CT and confirmed that the lesion

Figure 4. Computerized tomography: submucosal lesion of the rectal wall and thickening of the levator ani muscle on the side of the lesion. No local lymph node infiltration.

was localized submucosally and did not affect the muscular layer. An intraoperative macrobiopsy yielded whole cysts of up to 1 cm in diameter.

The patient was treated with lubricants, bulk laxa- tive, and a high-fiber diet and was advised to abstain from straining during defecation. Surgery was ruled out because location of the lesion low in the rectum would have led to permanent damage of the anal

sphincter. Clinical improvement was first observed after six

months of conservative treatment, with no change in

Vol. 39, No. 5

appearance of the lesions. After 12 months of treat- ment, endoscopic lesions disappeared, although mu- cus secretion continued. After 18 months, clinical re- mission was complete, and only cicatricial fibrosis remained at the site of the lesion (Fig. lb).

Case 2

A 45-year-old man previously in good health was seen for pain, rectal tenesmus, and anal expulsion of blood and mucus of one months duration. Rectal palpation revealed an indurated, polypoid, proliferat- ing mass that occupied the posterior left wall of the rectum. Colonoscopy detected a prominent nodular, apparently submucosal lesion that extended 2 and 7 cm from the anal margin. The ulcerated lesion se- creted abundant mucus and was suggestive of rectal carcinoma. Pathologic analysis was negative.

Transrectal ultrasonographic examination with a linear 5-MHz probe revealed multiple submucosal, hypoechogenic cysts. Computerized tomography showed submucosal lesions that protruded into the rectal lumen, loss of perirectal layers of fatty tissue, and thickening of the levator ani muscle on the side of the lesion, with no evidence of invasion or local adenopathies.

Magnetic resonance imaging (Fig. 5) with a medi- um-power device showed loss of the perirectal fatty layer at T1, which was especially evident in the coro- nal plane, with thickening of the levator ani muscle, asymmetry of the rectal lumen and paraluminal nod- ulation anterior and left with respect to the serous plane. In the T2 sequence, the signal of the nodular lesion was slightly stronger, and changes were sug-

Figure 5. Magnetic resonance imaging 0-1): submucosal noninfiltrating lesion of the rectal wall, thickening of the levator ani muscle on the side of the lesion, and loss of perirectal fatty layer. No evidence of local adenopathies.

COLITIS CYSTICA PROFUNDA 589

gestive of a submucosal, noninfiltrating lesion, with

complex signal related to the lesion contents (blood

and mucus). Intraoperative biopsy revealed mucus cysts in the

rectal submucosa. Conservative treatment was started with fiber and bulk laxatives. One month later, there was notable clinical improvement and partial remis- sion of endoscopic lesions. Six months later, endo- scopic lesions had remitted completely, and the pa- tient was free of symptoms. Twelve months after treatment was started, the patient enjoys complete clinical and endoscopic remission.

DISCUSSION

Mucus cysts can appear in any segment of the digestive tract submucosa, including the stomach 16-19 and small intestine, 19' z0 although they arise most fre- quently in the colon, where they are designated colitis cystica profunda. 1 They can be progressive and dif- fuse or localized and frequently recur after surgical ablation of the affected zone] ' 9 Rectal localization is most frequent in patients with defecation disorders during the third and fourth decades of life. From this site, cysts can extend to the anal margin, l~

where they manifest as expulsion of blood and mucus from the anus. These symptoms make differential diagnosis from mucosecretory rectal carcinoma diffi- cult. Noninvasive imaging techniques are very useful in reaching a correct preoperative diagnosis, which avoids mutilating surgical treatment for a benign entity.

Transrectal ultrasonography is the simplest, most innocuous, and most accurate aid to diagnosis. 13 Mul- tiple images of submucosal cysts with hyperecho- genic bands of fibrosis that do not reach the muscle layer and absence of local or regional lymph node infiltration confirm the diagnosis of colitis cystica pro- funda.

Although CT has been used to diagnose this entity in gastric localizations, 18 images of colitis cystica pro- funda with CT and MRI have not previously been reported. In our patients, we identified a submucosal mass that did not invade deep layers, loss of perirectal layers of fatty tissue, thickening of the levator ani muscle on the same side as the lesion, and confirmed the absence of local or regional lymph node involve- ment. Straining on defecation is the likely cause of levator ani muscle thickening. Intraoperative deep biopsies obtained transanally with anesthesia yield

590

large, whole cysts, TM which can confirm the diagnosis

when endoscopic images are inconclusive.

In both patients reported here, prolonged conser-

vative treatment brought about a complete remission

of lesions after 6 to 18 months, obviating the need for

aggressive surgical intervention. Our r ecommended

treatment is similar to that used for patients with other

defecation disorders and consists of a fiber-rich diet,

lubricants and bulk laxatives, together with reeduca-

tion of bowel habits a imed at avoiding straining or aggressive maneuvers to remove feces. 15' 24

In summary, we believe transrectal ultrasonogra-

phy, CT, and MRI can be used to accurately diagnose

colitis cystica profunda, although diagnosis should be

confirmed with deep transanal intraoperative biopsy.

Once malignant disease is ruled out, conservative

treatment and reeducation of bowel habits can lead to

complete remission of this rare disease.

A C K N O W L E D G M E N T S

The authors thank Ms. Karen Shashok for translat-

ing the original manuscript into English.

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