Colitis

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JOCYLINE

Cystica

LEDESMA-MEDINA,’

BARBARA

Profunda S. REID,

revealed

Colitis cystica profunda is characterized by the presence of mucous cysts in the musculanis mucosa of the colon. Although it may diffusely involve the entire colon, this disease primarily affects the pelvic colon and rectum. The exact etiology is not known. Stark [1] first described these lesions as mucous cysts in the colon of two autopsied cases of chronic dysentery. Vinchow [2] introduced the term ‘colitis cystica polyposa.” Since the report of Goodall and Sinclair [3] in 1956, 41 cases have been recorded in the English literatune. Epstein et al. [4] reported a 15-year-old girl and three other adults with colitis cystica profunda. Wayte and Helwig [5] reported 19 adults with localized colitis cystica pnofunda. It is apparent from this review that the disease is extremely rare in the pediatric age group. We report the second case of localized colitis cystica profunda in a child. Case

The

anemia

prolapse

pital

of Pittsburgh

for evaluation

of anemia

and growth

Proctosigmoidoscopy

revealed

no ulcers

in the

was found

Cystic

Hos-

the

microcytic-hypochmomic

type

and

was

at surgery.

Grossly,

the rectal

white-tan present spaces

were cells.

stump

speci-

mucosa. Several in the submucosa in the

lined

musculanis

by flattened

cells

dilatation

of the

types-colitis

profunda.

mucous

cystica The

former

glands

of the

superficialis is almost

and always

colon

are

colitis

cys-

associated

with pellagna. There are a few reports of the disease being associated with tropical sprue and leukemia. In colitis cystica superficialis, cysts are minute and are diffusely distributed throughout the entire colon. In coli-

netarda-

rectum.

was

inflamma-

examination showed large constant (fig. 1A). The retromectal space was gastrointestinal examination was

Discussion

tion. She had a 4 year history of rectal prolapse with bleeding. Rectal prolapse occurred during defecation and was reduced spontaneously. On physical examination hem height and weight were at the fiftieth percentile for a normal 7-year-old girl. Hen chest and abdomen were normal. Rectal examination revealed loose anal sphincter and fissures at the hypempigmented mucocutaneous junction. edematous granular mucosa was visualized line to 10 cm of proximal rectum. A biopsy

and at 12 cm, mild chronic

showed a wrinkled, folded of gelatinous material were (fig. 2A). Microscopically, the cystic mucosa were filled with mucin and (fig. 2B). Theme were no neoplastic

tica

to the Children’s

mucosa

men pools

Report

girl was admitted

normal

R. GIRDANY

believed to be due to chronic blood loss from rectal bleeding. The patient was treated with iron and was readmitted 3 months later for surgical repair of the rectal prolapse. A type III rectal

of two white

BERTRAM

tory change. Barium enema filling defects in the rectum thickened (fig. 1B). Upper normal.

‘

A 13-year-old

AND

tis cystica profunda, in the pelvic colon

submucosal and rectum

cysts are primarily and may be as large

seen as 2

cm in diameter. Wayte and Helwig [5] described a diffuse variety of the disease in five patients whose ages ranged from 4 to 68 years. The 4-year-old child with diffuse submucosal cysts died after a chronic infectious dysentery. Of the 41 cases described in the English literature,

Red

from the pectinate specimen at 10 cm

Fig. 1 . - Barium enema. A, Preevacuation frontal view showing intraluminal filling defects in rectum. B, Lateral view showing thickened retrorectalspace.

Received March 3, 1978; accepted April 28, 1978. All authors: Department of Radiology, Children’s 15213. Address reprint requests to J. Ledesma-Medina. ‘

Am J Roentgenol © 1978 American

131 :529-530, September Roentgen Ray Society

1978

Hospital

of Pittsburgh,

529

University

of Pittsburgh

School

of Medicine,

Pittsburgh,

0361

-803X/78/0900-0529

Pennsylvania

$00.00

530

CASE

REPORTS

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through

mucosal

ulcerations.

No

neoplastic

cells

are

seen in these mucous cysts. Allen [6] reported three adults who had rectal intramural polypoid masses which he called hamartomatous inverted polyps; he believed these were congenital ectopia. The appearance of the submucosal cysts may be preceded by superficial mucosal ulcerations and chronic nonspecific inflammation. The two patients described by Goodal and Sinclair [3] had antecedent ulcerative colitis. Epstein

cosal They

et al.

[4]

abscesses postulated

formation

reported

of submucosal

The radiographic to the

Fig.

2.-A,

Rectal

stump

removed

at surgery

showing

folded

rectal

mucosa

(white arrow) and large submucosal cysts filled with gelatinous material (black arrows). B, Photomicrograph of rectal section showing large cystic spaces filled with mucin and lined by flattened cells. Mucosa exhibits

signs

of regeneration.

neepithelialization

in two patients that this may

rectum.

cysts.

findings Barium

of submu-

with ulcerative colitis. be one mechanism of

in our patient

enema

examination

were

localized

showed

mul-

tiple, irregular, large filling defects in the rectum. The netnonectal space was thickened. There was no pneumatosis intestinalis. Of those reported cases with radiographic examinations, intraluminal filling defects were the most constant findings. Radiographically, colitis cystica pnofunda (localized) should be differentiated from polyps, ulcerative colitis, pneumatosis intestinalis, endometniosis, and carcinoma. It is important to distinguish colitis cystica profunda from these other lesions for therapeutic and prognostic reasons. Local resection of the

diseased

colon

is followed

by relief

of symptoms.

ACKNOWLEDGMENT

36 were localized colitis cystica profunda. Our case is the localized type and our discussion will be limited to localized colitis cystica profunda. Clinically, patients with localized colitis cystica profunda may have rectal bleeding, rectal prolapse, diarrhea, abdominal pain, and anemia. Two adult patients reported by Goodal and Sinclair [3] had diarrhea, abdominal pain, and anemia. Four cases reported by Epstein et al. had rectal prolapse, bleeding, diarrhea, and rectal polyps.” The pathogenesis of these lesions is obscure. The consensus is that the submucosal location of the cysts is a result of herniation on extension of regenerating sunface epithelium into the submucosa tracting down ‘ ‘

We thank

Odessa

Banks

for help

in preparing

the manuscript.

REFERENCES 1 . Stark W: Specimen septem histonias et dissectiones dysentenicorum exhibens (thesis). Leiden, the Netherlands, 1766 2. Virchow A: Die Krankhaften Geschwulste, vol 1 . Berlin, Hirschwald, 1863 3. Goodal HB, Sinclair ISA: Coltis cystica profunda. J Pathol Bacteriol 73 : 33-42, 1957 4. Epstein SE, Ascami WQ, Ablow AC, Seaman WB, Lattes A: Colitis cystica profunda. Am J CIin Pathol 45 : 1 86-201 , 1966 5. Wayte DM, Helwig EB: Colitis cystica profunda. Am J Clin Pathol 48:159-169, 1967 6. Allen MS Jr: Hamartomatous inverted polyps of the rectum. Cancer 19:257-265, 1966