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Single stage proctocolectomy for severe ulcerative colitis
Single Stage Proctocolectomy Severe Ulcerative Comparison
for
Colitis
with Less Extensive
Surgical Procedures H WILLIAM
SCOTT, Jr, MD, Nashville,
JOHN E WIMBERLY, HARRISON J SHULL,
MD, Pensacola, MD, Nashville,
DAVID H LAW, IV, MD, Albuquerque,
Idiopathic ulcerative colitis appears to be encountered less frequently in the south central part of the United States than in the large metropolitan centers of the northern and eastern parts of the country. However, during the last decade the disease has been recognized with much greater frequency in the Gastroenterology Clinic of Vanderbilt University Hospital than was the case twenty years ago. During the past fifteen years at Vanderbilt University Hospital and the affiliated Nashville Veterans Administration Hospital, fifty patients have undergone major colonic resection for ulcerative colitis. This surgically treated group represents about 20 per cent of the patients in whom the disease was diagnosed in these two institutions during this period of time. This report compares the results achieved by single stage proctocolectomy in thirty-six patients with results obtained by the use of less extensive resections of the large bowel in fourteen patients. Selection of Patients
The fifty patients in this series ranged from twelve to sixty-three years of age at the time of colon resection; the average age at the time of operation was 38.5 years. Thirty-one were male and nineteen were female. Duration of symptoms ranged from one month to twenty-one years with an average symptomatic period of 6.9 years prior to operation. All patients in the group were initially treated vigorously with various medical regimens to which they failed to show adequate response. Thirty-one had been treated with adrenal corticosteroids prior to operation without a lasting satisfactory response. Complications of ulcerative colitis were observed in many patients durFrom the Department of Surgery and the Division of Gastroenterology, Department of Medicine. Vanderbilt University Medical Center, Nashville. Tennessee. Presented at the Tenth Annual Meeting of the Society for Surgery of the Alimentary Tract, New York, New York, July 12 and 13, 1969.
Vol. 119, January
1970
Tennessee Florida Tennessee
New Mexico
ing the course of the disease. These included development of pseudopolyps in twenty-five patients, “toxic megacolon” in six, and colonic or rectal stricture in three. Two patients with toxic dilatation of the colon had colonic perforations at the time of resection. Carcinoma of the colon was found in two other patients. One patient had severe hemorrhage from the colon which required massive blood replacement and emergency colectomy, whereas five other patients with colonic bleeding required multiple transfusions during the course of medical treatment prior to operation. A rectovaginal fistula was present or had been repaired in four patients. Three patients had perianal abscesses and problems with fistula or fissure. Severe hemorrhoids were significant in five patients. (Table I. ) Concurrent lesions possibly related to ulcerative colitis observed in the group prior to colectomy were thrombophlebitis in nine patients, arthritis in seven, renal stones in one, thyroiditis in one, and iritis in one. Table II also lists a variety of other concurrent problems in these patients. Prior to colectomy, eight patients had had ileostomy, one had had cecostomy, and one other had undergone colostomy in an effort to control symptoms of colitis. Another had undergone drainage of an abdominal abscess. Indications for Operation
Although each patient usually had more than one indication for operation, the most common indication in these patients with ulcerative colitis was severe, progressive disease unresponsive to medical therapy for which thirty-seven resections were carried out. (Table III.) So-called toxic megacolon was the reason for emergency resection in six cases. Three patients were operated on for acute fulminating exacerbation of disease with other manifestations. Two patients had carcinoma of the colon suspected prior to operation. Massive a7
Scott et al TABLE
I
Complications
of
Ulcerative
Colitis
in
Fifty
Number Complication
Patients
Anal
fissure,
Toxic
flstulo,
dilatation
Severe
hemorrhoids
of colon
hemorrhage
Rectovoginal Stricture
or rectum
Carcinoma
dilatation
8
Acute
fulminating
6
Carcinoma
of
Colon
in
Fifty
Patients
Number
disease
of Patients
37
of colon
6
disease
of colon
and
3 chronic
ulceration
colitis
4
Massive
3
Severe
2 hemorrhage
1
1
pseudopolyposis
2
of colon
Pathologic Aspects
Pathologic confirmation of the clinical diagnosis of ulcerative colitis in this study was based on criteria similar to those suggested by the late John Van Prohaska [1] in identifying and classifying inflammatory diseases of the large and small bowel. In brief, Prohaska believes that there are “great differences between true ulcerative colitis on the one hand and granulomatous (Crohn’s) disease on the other hand. These differences not only express themselves histologically but also reflect themselves clearly in the gross specimen, in the course of the disease, in the nature of the complications, in roentgenographic examinations, and in the response to seemingly curative surgical procedures. It is, therefore, possible to identify clinical events, anatomic and histologic criteria which in their totality separate the inflammatory diseases into: ( 1) ulcerative colitis; or (2) granulomatous disease of the large and small bowel, with or without histologically recognizable granulomas.” We further agree with Prohaska that “there are instances where the assemblage of all clinical, anatomic and histologic signs produces a confusing picture short of positive identification.” The fifty patients in the present study with the diagnosis of ulcerative colitis have been accepted only after careful scrutiny using Prohaska’s clinical and pathologic criteria. A secondary review of pathologic mateConcurrent
lesions
lesion
Prior
to
Colrctomy
Number
of Patients
Thrombophlabitis
9
Arthritis
7 stones
1
lritis
1 artery
rial has been made by our surgical pathologist, Barton McSwain, in each instance in which the primary clinicopathologic diagnosis was not absolutely classical for ulcerative colitis. Table IV summarizes the current clinical and pathologic classification of ulcerative colitis in the fifty patients of this study. Patients with granulomatous colitis have been excluded. Forty-seven patients had classical ulcerative colitis in this series with diffuse mucosal ulcerations limited to the colon and rectum. (Figure 1.) One patient had segmental ulcerative colitis with disease limited to the descending colon. (Figure 2.) Another patient with classical ulcerative colitis involving the colon and rectum had associated ulcerative ileitis (“backwash ileitis”) which could be differentiated from Crohn’s disease. (Figure 3.) The remaining patient had the classical findings of ulcerative colitis involving the colon and rectum in association with the equally classical manifestations of Crohn’s disease which ultimately involved the entire small bowel. (Figure 4.) Operative Procedures and Early Postoperative Results
Table V lists the definitive operations for ulcerative colitis in this group of patients. Total proctocolectomy with ileostomy in one stage was carried out in thirty-six patients. In the last decade, the operative technic of total proctocolectomy for patients with ulcerative colitis has become fairly well standardized in this clinic. Preoperative preparation of the bowel has included restriction of diet to clear liquids for four to five days with appropriate use of oral nonabsorbable sulfonamides or kanamytin. Cathartics and enemas are avoided. Anesthesia has usually been halothane with succinylcholine as a muscle relaxant. The patient is placed in a lithotomy position
1
Thyroiditis
Iliac
progressive
Toxic
hemorrhage and severe pseudopolyposis were individual indications for resection in two other patients.
Renal
Resection
2
Perforation
II
for
Indication
25
6
flstula
of colon
Indications
of Chronic
Pseudopolyposis
occlusion
Pathologic
TABLE IV
and
1
Neuroflbromatosis
1
Ulcerative
Mitral
stenosis
I
Segmental
ulcerative
colitis
Aortic
stenosis
1
Ulcerative
colitis
with
“backwash
1
Ulcerative
colitis
with
regional
enteritis
1
Number
Disease
1
Regional
Cllnlcal Classifkatlon
of Disease
In Fifty Patients
Cholelithiasis
Diabetes
St3
Ill
Patients
---
TABLE
TABLE
(Crohn’s
of Patients _ 47
colitis
1 1
ileitis” enteritis
1
disease)
The American
Journal
of Surgery
Proctocolectomy
for Ulcerative
Colitis
Gross specimen of reFigure 1. sected colon and rectum with classical changes of diffuse ulcerative colitis typical of majority of patients in this study. Figure 2. Gross specimen of resected descending colon from a patient with segmental ulcerative colitis confined to descending colon. No clinical or histologic features have suggested Crohn’s disease.
and a two team synchronous combined abdominoperineal approach has usually been employed. Great care is taken during proctocolectomy to avoid injury to diseased bowel with fecal contamination, and the entire colon and rectum are removed in continuity. The ileum has been divided as close to the ileocecal valve as technically feasible with preservation of the ifeocolic artery when it exists. A matured ileostomy of the Brooke [2] type is constructed in the right lower quadrant, and the mesentery of the terminal ileum is carefully sutured to the anterior abdominal peritoneum and falciform ligament to close the right lumbar gutter. A closely fitting ileostomy bag is cemented in place in the operating room. In recent years the perineal wound has been closed in layers and two suction drainage catheters are left in the pelvis and exteriorized lateral to the suture line. In the thirty-six patients with single stage total proctocolectomy, there was one early postoperative death (an operative mortality of 2.8 per cent). This occurred one month after operation in a patient with fulminating disease who had been treated with adrenocorticosteroids before, during, and for one week after operation. At operation there was toxic dilatation of the colon with multiple colonic perforations. He died with generalized sepsis and renal failure. Another patient who also had carcinoma of the colon died of hepatitis three months after operation. (Table V.) Among the thirty-four surviving patients, twenty-two patients had an uneventful and uncomplicated recovery from single stage proctocolectomy. However, significant nonfatal complications occurred during the early postoperative period in twelve persons. (Table VI.) As seen in the table, a few of these patients had more than one complication. It is of considerable interest that these complications occurred only in patients who had been treated with adrenocorticosteroids for prolonged periods prior to operation. As might be expected, infections in the form of a wound abscess or intra-abdominal abscess were the most frequent complication. Each of these abscesses was successfully treated hy incision and drainage; however, Vol. 119. January
1970
during one such drainage procedure in a patient with iatrogenic Cushing’s syndrome, the jejunum was injured and a transitory jejunal fistula resulted which required prolonged parenteral alimentation until spontaneous closure occurred. Acute mechanical small bowel obstruction was the next most frequent postoperative problem and required operative release of adhesions in four patients. Ileostomy dysfunction required early postoperative revision in one patient. Although convalescence was delayed, each of these twelve patients with early postoperative complications progressed to full recovery. Major colonic resections of lesser extent than single stage total proctocolectomy have been carried out in fourteen patients. Of these, the initial resective procedure was total abdominal colectomy and ileostomy with the rectum left in place in twelve patients. Another patient had total abdominal colectomy with ileoproctostomy and one had transverse colostomy and resection of the descending colon for segmental ulcerative colitis. (Table VII. ) In this group of fourteen patients, one patient who had ileostomy and colectomy with the rectum left in place died from peritonitis and sepsis one week after operation. The patient who had colectomy with ileoproctostomy died after an anastomotic leak developed, despite subsequent ileostomy, drainage, and intensive supportive therapy. One patient, who also had carcinoma of the colon, died with metastases three months after colectomy and ileostomy with the rectum left in place. Another patient who had staged proctocolectomy also had regional enteritis and finally died after multiple small bowel resections for bleeding. (Table VIII. ) Among the ten surviving patients, eight had an uneventful and uncomplicated early recovery from the initial operation. However, persistent manifestations of colitis in the remaining colon and rectum in two patients necessitated removal of the remaining distal sigmoid and rectum from two weeks to four months later for complete recovery. In one of these patients an abdominal abscess developed after the initial operation 89
Scott et al 3
Gross specimen of terFigure 3. minal ileum with “backwash ileitis” resected secondarily after single stage ileostomy and proctocolectomy for classical ulcerative colitis. Figure 4. Gross specimen of resected colon and rectum in patient with pathologic changes of classical ulcerative colitis; regional enteritis coexisted
and a wound abscess after the removal of the rectum. (Table IX. ) Follow-Up Results
All surviving patients have been followed up by ourselves in collaboration with the patient’s family physician for periods ranging from a few months to fifteen years. Except in four patients who were operated on in recent months, we have obtained follow-up data from one to fifteen years since colectomy in the entire group who have survived the initial operation. Satisfactory rehabilitation has been achieved in the thirty-four survivors after single stage total proctocolectomy. In no instance has there been evidence of persistent or recurrent inflammatory bowel disease in the ileostomy or remaining small bowel. Every patient in this group has made a considerable gain in weight and a satisfactory adjustment to the ileostomy. However, as summarized in Table X, crcatricial stenosis of the ileal stoma has occurred in five patients and satisfactory relief of this problem has required a total of eleven revisionary operative procedures. Three of these patients who required multiple revisions were among the nine patients who had nonmature ileostomies performed originally. One of four patients with a Turnbull [3] ileostomy required two revisions, and one of twentyone patients with a Brooke ileostomy also required two subsequent revisionary procedures. Acute mechanical small bowel obstruction has required laparotomy for relief in two patients. Failure of primary healing of the perineal site of removal of the rectum with a chronic perineal sinus has occurred in three patients, all of TABLE
V
Single
Stage
Total Proetocolectomy
in Thirty-Six
Patients Number
of
Patients
Single Stage Total Proctocolectomy
TABLE VI
Survivors): Early Postoperative Complications
None Nonfatal
abscess
Intestinal
2
Early
1
Urinary
Late
1
Ileostomy
Survivors Survival
34 rate
Jejunal
abscess
embolism tract
Number
of Patients
22
(13*)
4*
obstruction
Pulmonary
(Thirty-pour
Complications
12*
complications
Intra-abdominal Deaths
bowel.
whom were on prolonged steroid therapy before proctocolectomy. In two of these, spontaneous healing occurred after several months whereas in one patient a perineal sinus has persisted for six years. Among the ten patients who survived a definitive colon resection of lesser extent than single stage total proctocolectomy, two patients required early (two weeks to four months) removal of the rectum for persistent disease as previously mentioned. In five others recurrent disease has developed in the rectum during the period of follow-up study which has required abdominoperineal resection one to eight years after initial colectomy. Despite these late complications, rehabilitation of each person with recurrence has been accomplished by resection of all bowel involved by persistent or recurrent colitis. The other less significant late complications in this group are summarized in Table X. These include three patients with ileostomy dysfunction. These three were among nine patients who had nonmatured ileostomies carried out initially. One of these had “backwash ileitis” which led to a total of four revisionary procedures during a fifteen year period. In three other patients initial colectomy for ulcerative colitis was performed without resection of the rectum. These men have been followed up for one, twelve, and fifteen years since colectomy without evident recurrence of disease and each has maintained good health.
Wound
Results
in small
infection
dysfunction flstula
4* 3* 1* 1* 1* 1*
94Yo * Prolonged
preoperative
medical
therapy
The
with
American
steroids.
Journal
of Surgery
Proctocolectomy Deflnitive
TABLE VII
Operations
Performed
in Fifty Patients
TABLE
IX
Colon
Resection
coloctomy Operation Total
proctocolectomy,
Staged
total
Colectomy, Resection Colectomy,
Number
ileostomy, of
rectum
descending
colon,
left
in place
colostomy
4
None
1
Nonfatal
Total
Procto-
Postoperative
Number
complications
Persistent
1
ileoproctostomy
Than Early
Complications
a
ileostomy
Extensive
Survivors):
Colitis
Complications
36
ileostomy
proctocolectomy,
of Patents
Less
(Ten
for Ulcerative
disease
Abdominal Wound
in rectum
abscess abscess
2
(l*)
1
(I*)
1
(I*)
of Patients
a
(4*)
2
(l*)
Comments * Prolonged
As mentioned earlier, we have made a concerted effort in this study to apply the clinical, radiologic, anatomic, histologic, and pathologic criteria originally suggested by Morson and Lockhart-Mummery [4] and more recently enunciated by Prohaska [I] to differentiate true ulcerative colitis from granulomatous colitis (Crohn’s disease). Since the cause of the two major varieties of inflammatory bowel disease remains obscure, there are no absolute criteria for differentiation. However, the differences, as previously outlined by Morson, Prohaska, and others, are sufficiently great in our opinion to recognize ulcerative colitis as a separate disease entity which in most cases can be accurately differentiated from granulomatous colitis and the other variants of Crohn’s disease. Our views concerning the characteristics of ulcerative colitis are summarized subsequently herein. Ulcerative colitis is typically an inflammatory disease of the mucosa of the bowel, usually diffusely involving the colon and rectum and limited to the large bowel. Its clinical course is characterized by cyclical exacerbations and remissions of bloody diarrhea with fulminant compiications in some patients and prolonged periods of quiescence in others. In patients with ulcerative colitis pseudopolyposis frequently develops, less fre-, quently the lethal problem of toxic dilatation of the colon, and with chronicity there is clearly an increased incidence of carcinoma of the colon and rectum. Spon-, taneous perforations of the colon in ulcerative colitis are very rare except when toxic dilatation occurs. Fistulization is almost exclusively confined to the perineum and vagina where fistulas are usually secondary to perianal abscesses. Total resection of the colon and rectum with ileostomy is curative in ulcerative colitis, and the disease does not tend to recur in the small bowel except in the infrequent cases when there is an accompanying “backwash ileitis.” TABLE VIII
Colon
Resection
Proctocolectomy Results
Less
Extensive
in Fourteen
Than
Total
Patients Number
TABLE X
Late
Early
2
Late
2
Survivors Survival
Vol.
119,
January
1970
rate
with
steroids.
of
All Colon
Colitis (Forty-Four
Resections
for
Survivors) Number
Number of Procedure Single
stage
No
late
Late
and total
Complications proctocolectomy
obstruction
Peristent
perineal
sinus
less than
11
2
2
3
0
3
6
4 6
complications
Persistent
5
IO
complications
Intestinal
Operative Procedures
total
proctocolectomy late
with
34
dysfunction
resection
Number
Complications
10
complications
Intestinal
Colon
Patients
of
Secondary
24
complications
lleostomy
Late
4
therapy
Complications
Ulcerative
lleostomy Deaths
medical
Pathologic examination of the colon in ulcerative colitis shows it to have multiple diffuse ulcerations of the mucosa and in most cases a relatively thin colonic wall without extensive submucosal fibrosis. The colonic mesentery, which usually does not contain enlarged lymph nodes, is apt to be normally thin or only slightly thickened and certainly not massively fibrotic. Microscopic examination of the wall of the inflamed colon shows diffuse macro- and microulcerations with adjacent edema, polymorphonuclear and eosinophilic cellular invasion of the mucosa, and destruction of the villi. Microscopic abscesses form in the crypts of Lieberkuhn and penetrate into the submucosa with the production of wider areas of ulceration of the overlying mucosa. Granulomas of the type seen in Crohn’s disease are conspicuously absent. On the other hand, there is usually definite increase in the number of Paneth cells in the colonic crypts. These cells are present in infrequent numbers in normal colons; however, Paterson and Watson [5] find them to be greatly increased in patients with ulcerative colitis. Instead of one or two Paneth cells per crypt in the normal colon, there may be 100 to 300 Paneth cells per crypt in colons with ulcerative colitis. The etiologic significance of this Paneth
No
of Patiantc
preoperative
dysfunction obstruction perineal
sinus
Thrombophlebitis 10
Abdominal
wall
71Yo
Recurrent
disease
abscess in rectum
1
1
1
2
1
0
1
1
5
5
_
91
Scott et al cell metaplasia sponse
is not known,
to ulcerative
but it is most likely a re-
colitis rather
than a factor
in etiol-
ogy. Since the cause and pathogenesis of ulcerative colitis remain unknown, the development of medical and surgical treatment of the disease has been totally empiric. Ileostomy alone has been given extensive trial in the past and has proved to be unsatisfactory as a definitive form of surgical treatment. However, as early as 1931 Rankin [6] demonstrated that ileostomy combined with total removal of the colon and rectum in stages could be curative in this disease. In 1940 Macguire [7] suggested and in 1949 Miller, Gardner, and Ripstein [8] reported that colectomy carried out at the time of ileostomy might be a more effective form of surgical management of the disease. In 1951 Ravitch and Handelsman [9] described a technic of single stage total proctocolectomy and used it successfully in six patients with chronic ulcerative colitis and in four with congenital polyposis of the colon. Since their report, the concept of performing ileostomy and total proctocolectomy simultaneously has become widely accepted as the procedure of choice when surgical intervention is indicated in the management of patients with ulcerative colitis. Despite the magnitude of the operation, this single stage procedure offers the cardinal advantage to the patient of immediate ablation of all inflammatory bowel disease, thus eliminating the serious complications of persistent or recurrent ulcerative colitis with the attendant morbidity and mortality. In 1958 in a series of forty patients, Bacon [IO] demonstrated that single stage ileostomy and proctocolectomy could be performed with an operative mortality of 2.5 per cent. More recent reports of larger series by Goligher [II], Prohaska [I], and others substantiate this commendable performance. Our experience with single stage proctocolectomy in thirty-six patients with an operative mortality of 2.8 per cent is further evidence of the validity of this approach. Klein et al [Z2] have raised the question as to whether a lesser procedure than single stage proctocolectomy should be performed in the critically ill patient with toxic dilatation of the colon. In our small surgical experience in six patients with toxic dilatation of the colon, five were submitted to the single stage procedure and in one patient, who was in shock during the conduct of emergency ileostomy with abdominal colectomy, removal of the distal sigmoid and rectum was deferred until two weeks later. The single death in this group occurred in a man who had multiple sealed off colonic perforations with unavoidable gross fecal contamination of the peritoneal cavity during emergency single stage proctocolectomy; he died with sepsis one month later. Both Goligher [II] and Prohaska [I] have advocated the use of the single stage procedure in both elec92
tive and ulcerative cessity for nitude can of patients
emergency management of patients with colitis. Our experience suggests that the neconsidering a primary procedure of less magbe diminished materially by earlier selection for surgical intervention. In this series of patients with ulcerative colitis treated by single stage proctocolcctomy. early postoperative complications occurred only in those patients who had received massive or prolonged preoperative steroid treatment. Accordingly, it is reasonable to raise the question as to the advisability of the use of prolonged or massive steroid therapy in ulcerative colitis. Goligher [II] has gone on record as opposing their use in this regard. We do not have controlled data available to help in answering this highly controversial question, but the incidence of postoperative complications in our patients who received steroids makes us very reluctant to use high dose or continuous long-term adrenal steroid therapy in patients with ulcerative colitis. The most important complication among the survivors of the multiple stage method of operative treatment in our small series was persistence or recurrence of ulcerative colitis in the rectum. Seven of ten survivors in this group have required secondary abdominoperineal resection of the rectum for this reason. Although no sweeping statistically significant conclusions may be drawn from this retrospective clinical study, our experience with single stage proctocolectomy for severe ulcerative colitis adds further support to the validity of the concept behind the procedure and prompts us to advocate its continued use. Summary Fifty patients with severe ulcerative colitis were submitted to major colonic resections during a fifteen year period. Thirty-six patients had single stage ileostomy and proctocolectomy with one early death (operative mortality 2.8 per cent). Fourteen patients had multiple stage procedures: usually ileostomy, abdominal colectomy with rectum left in place as the first stage. There were two early deaths in this group (operative mortality 14 per cent) and two later deaths. Second stage abdominoperineal resection of rectum because of recurrent ulcerative colitis has been necessary in seven of ten survivors of this group. This retrospective study validates the continued advocacy of single stage proctocolectomy in both elective and emergency management of patients with severe ulcerative colitis. An impressive degree of rehabilitation has been obtained in every patient after successful proctocolectomy. References 1. Prohaska JV: The inflammatory diseases of the large and small bowel. In: Current Problems in Surgery. Chicago, 1969. Year Book Medical Publishers, Inc. of ileostomy. Lancet 2: 102, 2. Brooke BN: Management 1952. The American
Journal
of Surgery
Proctocolectomy RB Jr: Colectomy with simultaneous ileostomy 3. Turnbull as the surgical treatment of diffuse ulcerative colitis. Surgery 4: 843, 1957. AE: Crohn’s disease 4. Morson BC and Lockhart-Mummery occurring primarily in colon. Gastroenterofogia 92: 168, 1959. in 5. Paterson JC and Watson SH: Paneth cell metaplasia ulcerative colitis. Amer J Path 38: 243, 1961. of colon and rectum: six 6. Rankin Fw: Total extirpation consecutive successful cases. Proc Staff Meet Mayo Clin 6: 436, 1931. 7. Macguire DP: Aseptic total colectomy. New York J Med 40: 1515, 1940. 8. Miller CG, Gardner CM, and Ripstein CB: Primary resecy;igof colon in ulcerative colitis. J Canad M A 60: 584, 9.
10. 11. 12.
Ravitch MM and Handelsman JC: One stage resection of entire colon and rectum for ulcerative colitis and polypoid adenomatosis. Bull Johns Hopkins Hosp 88: 59, 1951. Bacon HE: Ulcerative Colitis, p 322. Philadelphia, 1958. J B Lippincott Co. Goligher JC: Treatment of ulcerative colitis. In: Current Problems in Surgery. Chicago, 1965. Year Book Medical Publishers, Inc. Klein SH, Edelman S, Kirschner PA, Lyons AS, and Baronofsky ID: Emergency cecostomy in ulcerative colitis with acute toxic dilatation. Surgery 47: 399, 1960.
Discussion WILEY F BARKER: I certainly agree with the general premises and conclusions of this paper. We have some differences, however. As we consider our cases more seriously, the more we are finding that some of them we thought were not are indeed the granulomatous form. In the past fifteen years we have had about thirty emergency colectomies. Either of the types of colitis may appear in the first of these three classifications: fulminant, perforating, or bleeding. Toxic megacolon is largely restricted to the mucosal form. A total one stage colectomy should be performed for hemorrhage, perforation, and the fulminant form, unless the rectum is not involved in the granulomatous disease; otherwise, I think one has the series of complications Dr. Scott has shown. Our primary treatment of toxic megacolon has been early resection. We have not had a very good batting average until recently, but we now have a cumulative mortality of about 30 per cent. Difficulty has arisen in cases of perforation of the bowel or free contamination, or when we have inadvertently put a hole in the bowel. I would urge all to consider the lesser staged procedures of cecostomy that Dr Lyons has suggested and the “blow-hole” colostomy that Dr Turnbull has suggested, as an alternative means of handling the toxic megacolon. RICHARD T MYERS (Winston-Salem, NC) : I would like to compliment Dr Scott on his skill and cunning in keeping mortalities quite low in this desperate type of situation. Our experience has essentially been in agreement with his. My colleagues and I have treated 105 cases since 1949. In all we have attempted to complete a single stage proctocolectomy or to stage it, depending on the condition of the patient. Perhaps we have not been able to select our patients as well as we should have, that we Vol.
119, January
1970
for Ulcerative
Colitis
should have been placing some of the patients, on whom we have performed a single stage proctocolectomy, into a staged group. The mortality for patients with a single stage proctocolectomy was 8 per cent and for those with multiple stage or incomplete stages at this time the mortality was 13.1 per cent. If we average out the mortalities of each of these series, they will fall grossly, for the total cases, between 6 and IO per cent. It is possible that the single stage procedure is the answer; however, the answer may lie in other factors such as earlier operation or avoidance of the routine use of steroids, as alluded to by Dr Scott. We have found that our fatalities have usually been due to infection. LOUIS T PALUMBO: I would like to add our experience in support of the program and policies discussed, because we find that our results, both in operative mortality and long-range follow-up study, are very similar to those in their series. In 1950 we started performing one stage total colectomy and ileostomy at the same time. In 1956 we changed our approach so that in the last eighteen patients since 1956 we removed the entire colon via the abdominal route, taking out the rectum and anal canal, dilating the anal outlet, and using it for a drainage site for the presacral space. This decreases the operating time considerably as well as blood loss and chemical losses, and results in lesser morbidity. In one patient we performed total one stage procedure using the abdominal approach solely without using the lithotomy or perineal approach. In addition to extensive ulcerative colitis this man also had congenital mucoceles of the colon; probably 150 or 200 of these were present. These were removed in 1956 and part of the specimen was sent to the Armed Forces Institute of Pathology. They reported this was the first case on record in the Institute at that time. CLARENCE DENNIS (Brooklyn, NY) : At the time of my departure from the University of Minnesota in 19.51, after sixteen years, the experience of a group of four of us with idiopathic ulcerative colitis was reviewed and reported in Surgery in 1952. There were 116 colectomies, of which fifty-five were primary, as published by WangenSteen and Toon in 1948, with one fatality. Of these, thirteen were primary total proctocolectomies with ileostomies, with no mortality. Since 1951, in Brooklyn, all proctocolectomies for idiopathic ulcerative colitis on the University Surgical Services have been primary, one stage procedures, and the mortality has remained at about 5 per cent. With regard to carcinomatous deterioration, which Dr Scott mentioned, cancer occurred in twenty-three patients in our own experience; twenty patients are dead and one is terminal. In a prospective study, cancer in the large intestine occurred in about 4 per cent per annum in those patients who had had the disease longer than ten years and who still retained their colon. The figure on the rectum alone was about 1 per cent per annum. We have found it very important to have rapid frozen section study of the terminal ileum before making an ileostomy. One can look grossly at the serosal and mucosal 93
Scott et al surfaces in this disease without recognizing so-called “backwash ileitis.” Our own figures would suggest that about 30 per cent of these patients have involvement of the ileum, and the ileostomies have not worked satisfactorily if made in diseased intestine. There have been many discussions on what to do with the perineum. I have never tried to remove all of the rectum from within the abdomen, but put the patient up in the lithotomy position. I repeatedly tried closing the perineum and concluded that there are sufficient complications in about one third of patients so closed which renders their convalescence difficult and prolonged; therefore, they have been packed open routinely. Periodically other surgeons tell me this course is overly cautious. I would like to know what other people do. OLIVER H BEAHRS (Rochester, Minn) : I cannot resist making a few comments regarding closure of the perineal wound, as brought up by Dr Dennis in his discussion. Over-all, in our experience, fifty per cent of perineal wounds, when packed open, heal satisfactorily whereas in 50 per cent there are complications. When the wound is closed primarily, 75 per cent heal and only twenty-five per cent have complications. Since adding suction drainage to the hollow of the sacrum five or six years ago, it is rare for any complications to develop when the perineal wound is closed primarily. We began to use primary closure in 1951 and, in addition to drainage, continue occasionally to use streptokinase and streptodornase as an irrigating agent in selected cases when it is thought that hematoma, necrotic tissue or exudate is present. DAVID H LAW (closing) : In answering the questions I will speak as a gastroenterologist. Doctor Barker, we are specifically concerned with chronic ulcerative colitis. I want to stress this point since you were referring in part to Crohn’s disease of the colon, in which quite probably the problem is different. In our series we took great pains to differentiate these two, both at initial pathologic and clinical review and at subsequent pathologic and radiologic review prior to reporting this series. Certainly toxic dilatation occurs primarily with ulcerative colitis. It is worth while to remember, however, that it has been reported also in Crohn’s disease of the colon, with amebiasis and one or two other disorders. In regard to Dr Myers’ question and comment, our low mortality may perhaps be related in part to earlier sur-
94
gery. I would like to comment mart on that later. I do rlc)t want to take all the credit away. hut I do think earlier surgery plays a role in the mortality. I would like to thank Drs Pa~umbo and Dennis fe.)r their confirmatory comments, and 1 WOLII~like to re:er their question on closing the pet-ineum to Dr Dunphy I know where it ib but I cannot close it. It is important to stress that the onus of operative martality lies not only with the operating surgeon but also with the referring physician. This has been accepted for a good period of time. Our studies suggest that the responsibility of the referring physician should also be extended to the associated operative morbidity since all of our early complications occurred in patients who had received either long-term or massive adrenocortical steroid therapy. Adrenocortical steroids do have a place in the treatment of ulcerative colitis. However, I would like to stress two points: (1) total proctocolectomy does remove ulcerative colitis, and (2) there is increasing practicality, as shown in the previous studies as well as in ours, of the well functioning ileostomy. Remembering these two things, we can and should begin to prepare the patient for operation at a somewhat earlier date, once operation is considered for ulcerative colitis. PRESIDENT DUNPHY: I cannot resist a comment, so I will say that we do close the posterior wound, at least I do and the residents who work with me do, leaving a drain, and then irrigating it. I do not think it makes much difference what you use for four or five days, but I am certain there are more complications in healing of the posterior wound in patients with ulcerative colitis than there are in patients with cancer. Some may recall the paper Dr Thomas Hunt in our department presented at the American Surgical Association on the use of topical vitamin A to reverse the deleterious effects of high-dose cortisone in the treatment of the healing wound. We are using this routinely in patients in this group if their wounds break down. I am convinced it is of great value; in fact, I recently repaired a total anal fistula. going above the levator ani in a woman with Crohn’s disease who was on high-dose steroids at the time. The disease was in moderate remission, and with topical vitamin A the wound healed as if she did not have either Crohn’s disease or steroids. If you have occasion to try it, we would like to hear from you.
The American Journal of Surgery
for
Colitis
with Less Extensive
Surgical Procedures H WILLIAM
SCOTT, Jr, MD, Nashville,
JOHN E WIMBERLY, HARRISON J SHULL,
MD, Pensacola, MD, Nashville,
DAVID H LAW, IV, MD, Albuquerque,
Idiopathic ulcerative colitis appears to be encountered less frequently in the south central part of the United States than in the large metropolitan centers of the northern and eastern parts of the country. However, during the last decade the disease has been recognized with much greater frequency in the Gastroenterology Clinic of Vanderbilt University Hospital than was the case twenty years ago. During the past fifteen years at Vanderbilt University Hospital and the affiliated Nashville Veterans Administration Hospital, fifty patients have undergone major colonic resection for ulcerative colitis. This surgically treated group represents about 20 per cent of the patients in whom the disease was diagnosed in these two institutions during this period of time. This report compares the results achieved by single stage proctocolectomy in thirty-six patients with results obtained by the use of less extensive resections of the large bowel in fourteen patients. Selection of Patients
The fifty patients in this series ranged from twelve to sixty-three years of age at the time of colon resection; the average age at the time of operation was 38.5 years. Thirty-one were male and nineteen were female. Duration of symptoms ranged from one month to twenty-one years with an average symptomatic period of 6.9 years prior to operation. All patients in the group were initially treated vigorously with various medical regimens to which they failed to show adequate response. Thirty-one had been treated with adrenal corticosteroids prior to operation without a lasting satisfactory response. Complications of ulcerative colitis were observed in many patients durFrom the Department of Surgery and the Division of Gastroenterology, Department of Medicine. Vanderbilt University Medical Center, Nashville. Tennessee. Presented at the Tenth Annual Meeting of the Society for Surgery of the Alimentary Tract, New York, New York, July 12 and 13, 1969.
Vol. 119, January
1970
Tennessee Florida Tennessee
New Mexico
ing the course of the disease. These included development of pseudopolyps in twenty-five patients, “toxic megacolon” in six, and colonic or rectal stricture in three. Two patients with toxic dilatation of the colon had colonic perforations at the time of resection. Carcinoma of the colon was found in two other patients. One patient had severe hemorrhage from the colon which required massive blood replacement and emergency colectomy, whereas five other patients with colonic bleeding required multiple transfusions during the course of medical treatment prior to operation. A rectovaginal fistula was present or had been repaired in four patients. Three patients had perianal abscesses and problems with fistula or fissure. Severe hemorrhoids were significant in five patients. (Table I. ) Concurrent lesions possibly related to ulcerative colitis observed in the group prior to colectomy were thrombophlebitis in nine patients, arthritis in seven, renal stones in one, thyroiditis in one, and iritis in one. Table II also lists a variety of other concurrent problems in these patients. Prior to colectomy, eight patients had had ileostomy, one had had cecostomy, and one other had undergone colostomy in an effort to control symptoms of colitis. Another had undergone drainage of an abdominal abscess. Indications for Operation
Although each patient usually had more than one indication for operation, the most common indication in these patients with ulcerative colitis was severe, progressive disease unresponsive to medical therapy for which thirty-seven resections were carried out. (Table III.) So-called toxic megacolon was the reason for emergency resection in six cases. Three patients were operated on for acute fulminating exacerbation of disease with other manifestations. Two patients had carcinoma of the colon suspected prior to operation. Massive a7
Scott et al TABLE
I
Complications
of
Ulcerative
Colitis
in
Fifty
Number Complication
Patients
Anal
fissure,
Toxic
flstulo,
dilatation
Severe
hemorrhoids
of colon
hemorrhage
Rectovoginal Stricture
or rectum
Carcinoma
dilatation
8
Acute
fulminating
6
Carcinoma
of
Colon
in
Fifty
Patients
Number
disease
of Patients
37
of colon
6
disease
of colon
and
3 chronic
ulceration
colitis
4
Massive
3
Severe
2 hemorrhage
1
1
pseudopolyposis
2
of colon
Pathologic Aspects
Pathologic confirmation of the clinical diagnosis of ulcerative colitis in this study was based on criteria similar to those suggested by the late John Van Prohaska [1] in identifying and classifying inflammatory diseases of the large and small bowel. In brief, Prohaska believes that there are “great differences between true ulcerative colitis on the one hand and granulomatous (Crohn’s) disease on the other hand. These differences not only express themselves histologically but also reflect themselves clearly in the gross specimen, in the course of the disease, in the nature of the complications, in roentgenographic examinations, and in the response to seemingly curative surgical procedures. It is, therefore, possible to identify clinical events, anatomic and histologic criteria which in their totality separate the inflammatory diseases into: ( 1) ulcerative colitis; or (2) granulomatous disease of the large and small bowel, with or without histologically recognizable granulomas.” We further agree with Prohaska that “there are instances where the assemblage of all clinical, anatomic and histologic signs produces a confusing picture short of positive identification.” The fifty patients in the present study with the diagnosis of ulcerative colitis have been accepted only after careful scrutiny using Prohaska’s clinical and pathologic criteria. A secondary review of pathologic mateConcurrent
lesions
lesion
Prior
to
Colrctomy
Number
of Patients
Thrombophlabitis
9
Arthritis
7 stones
1
lritis
1 artery
rial has been made by our surgical pathologist, Barton McSwain, in each instance in which the primary clinicopathologic diagnosis was not absolutely classical for ulcerative colitis. Table IV summarizes the current clinical and pathologic classification of ulcerative colitis in the fifty patients of this study. Patients with granulomatous colitis have been excluded. Forty-seven patients had classical ulcerative colitis in this series with diffuse mucosal ulcerations limited to the colon and rectum. (Figure 1.) One patient had segmental ulcerative colitis with disease limited to the descending colon. (Figure 2.) Another patient with classical ulcerative colitis involving the colon and rectum had associated ulcerative ileitis (“backwash ileitis”) which could be differentiated from Crohn’s disease. (Figure 3.) The remaining patient had the classical findings of ulcerative colitis involving the colon and rectum in association with the equally classical manifestations of Crohn’s disease which ultimately involved the entire small bowel. (Figure 4.) Operative Procedures and Early Postoperative Results
Table V lists the definitive operations for ulcerative colitis in this group of patients. Total proctocolectomy with ileostomy in one stage was carried out in thirty-six patients. In the last decade, the operative technic of total proctocolectomy for patients with ulcerative colitis has become fairly well standardized in this clinic. Preoperative preparation of the bowel has included restriction of diet to clear liquids for four to five days with appropriate use of oral nonabsorbable sulfonamides or kanamytin. Cathartics and enemas are avoided. Anesthesia has usually been halothane with succinylcholine as a muscle relaxant. The patient is placed in a lithotomy position
1
Thyroiditis
Iliac
progressive
Toxic
hemorrhage and severe pseudopolyposis were individual indications for resection in two other patients.
Renal
Resection
2
Perforation
II
for
Indication
25
6
flstula
of colon
Indications
of Chronic
Pseudopolyposis
occlusion
Pathologic
TABLE IV
and
1
Neuroflbromatosis
1
Ulcerative
Mitral
stenosis
I
Segmental
ulcerative
colitis
Aortic
stenosis
1
Ulcerative
colitis
with
“backwash
1
Ulcerative
colitis
with
regional
enteritis
1
Number
Disease
1
Regional
Cllnlcal Classifkatlon
of Disease
In Fifty Patients
Cholelithiasis
Diabetes
St3
Ill
Patients
---
TABLE
TABLE
(Crohn’s
of Patients _ 47
colitis
1 1
ileitis” enteritis
1
disease)
The American
Journal
of Surgery
Proctocolectomy
for Ulcerative
Colitis
Gross specimen of reFigure 1. sected colon and rectum with classical changes of diffuse ulcerative colitis typical of majority of patients in this study. Figure 2. Gross specimen of resected descending colon from a patient with segmental ulcerative colitis confined to descending colon. No clinical or histologic features have suggested Crohn’s disease.
and a two team synchronous combined abdominoperineal approach has usually been employed. Great care is taken during proctocolectomy to avoid injury to diseased bowel with fecal contamination, and the entire colon and rectum are removed in continuity. The ileum has been divided as close to the ileocecal valve as technically feasible with preservation of the ifeocolic artery when it exists. A matured ileostomy of the Brooke [2] type is constructed in the right lower quadrant, and the mesentery of the terminal ileum is carefully sutured to the anterior abdominal peritoneum and falciform ligament to close the right lumbar gutter. A closely fitting ileostomy bag is cemented in place in the operating room. In recent years the perineal wound has been closed in layers and two suction drainage catheters are left in the pelvis and exteriorized lateral to the suture line. In the thirty-six patients with single stage total proctocolectomy, there was one early postoperative death (an operative mortality of 2.8 per cent). This occurred one month after operation in a patient with fulminating disease who had been treated with adrenocorticosteroids before, during, and for one week after operation. At operation there was toxic dilatation of the colon with multiple colonic perforations. He died with generalized sepsis and renal failure. Another patient who also had carcinoma of the colon died of hepatitis three months after operation. (Table V.) Among the thirty-four surviving patients, twenty-two patients had an uneventful and uncomplicated recovery from single stage proctocolectomy. However, significant nonfatal complications occurred during the early postoperative period in twelve persons. (Table VI.) As seen in the table, a few of these patients had more than one complication. It is of considerable interest that these complications occurred only in patients who had been treated with adrenocorticosteroids for prolonged periods prior to operation. As might be expected, infections in the form of a wound abscess or intra-abdominal abscess were the most frequent complication. Each of these abscesses was successfully treated hy incision and drainage; however, Vol. 119. January
1970
during one such drainage procedure in a patient with iatrogenic Cushing’s syndrome, the jejunum was injured and a transitory jejunal fistula resulted which required prolonged parenteral alimentation until spontaneous closure occurred. Acute mechanical small bowel obstruction was the next most frequent postoperative problem and required operative release of adhesions in four patients. Ileostomy dysfunction required early postoperative revision in one patient. Although convalescence was delayed, each of these twelve patients with early postoperative complications progressed to full recovery. Major colonic resections of lesser extent than single stage total proctocolectomy have been carried out in fourteen patients. Of these, the initial resective procedure was total abdominal colectomy and ileostomy with the rectum left in place in twelve patients. Another patient had total abdominal colectomy with ileoproctostomy and one had transverse colostomy and resection of the descending colon for segmental ulcerative colitis. (Table VII. ) In this group of fourteen patients, one patient who had ileostomy and colectomy with the rectum left in place died from peritonitis and sepsis one week after operation. The patient who had colectomy with ileoproctostomy died after an anastomotic leak developed, despite subsequent ileostomy, drainage, and intensive supportive therapy. One patient, who also had carcinoma of the colon, died with metastases three months after colectomy and ileostomy with the rectum left in place. Another patient who had staged proctocolectomy also had regional enteritis and finally died after multiple small bowel resections for bleeding. (Table VIII. ) Among the ten surviving patients, eight had an uneventful and uncomplicated early recovery from the initial operation. However, persistent manifestations of colitis in the remaining colon and rectum in two patients necessitated removal of the remaining distal sigmoid and rectum from two weeks to four months later for complete recovery. In one of these patients an abdominal abscess developed after the initial operation 89
Scott et al 3
Gross specimen of terFigure 3. minal ileum with “backwash ileitis” resected secondarily after single stage ileostomy and proctocolectomy for classical ulcerative colitis. Figure 4. Gross specimen of resected colon and rectum in patient with pathologic changes of classical ulcerative colitis; regional enteritis coexisted
and a wound abscess after the removal of the rectum. (Table IX. ) Follow-Up Results
All surviving patients have been followed up by ourselves in collaboration with the patient’s family physician for periods ranging from a few months to fifteen years. Except in four patients who were operated on in recent months, we have obtained follow-up data from one to fifteen years since colectomy in the entire group who have survived the initial operation. Satisfactory rehabilitation has been achieved in the thirty-four survivors after single stage total proctocolectomy. In no instance has there been evidence of persistent or recurrent inflammatory bowel disease in the ileostomy or remaining small bowel. Every patient in this group has made a considerable gain in weight and a satisfactory adjustment to the ileostomy. However, as summarized in Table X, crcatricial stenosis of the ileal stoma has occurred in five patients and satisfactory relief of this problem has required a total of eleven revisionary operative procedures. Three of these patients who required multiple revisions were among the nine patients who had nonmature ileostomies performed originally. One of four patients with a Turnbull [3] ileostomy required two revisions, and one of twentyone patients with a Brooke ileostomy also required two subsequent revisionary procedures. Acute mechanical small bowel obstruction has required laparotomy for relief in two patients. Failure of primary healing of the perineal site of removal of the rectum with a chronic perineal sinus has occurred in three patients, all of TABLE
V
Single
Stage
Total Proetocolectomy
in Thirty-Six
Patients Number
of
Patients
Single Stage Total Proctocolectomy
TABLE VI
Survivors): Early Postoperative Complications
None Nonfatal
abscess
Intestinal
2
Early
1
Urinary
Late
1
Ileostomy
Survivors Survival
34 rate
Jejunal
abscess
embolism tract
Number
of Patients
22
(13*)
4*
obstruction
Pulmonary
(Thirty-pour
Complications
12*
complications
Intra-abdominal Deaths
bowel.
whom were on prolonged steroid therapy before proctocolectomy. In two of these, spontaneous healing occurred after several months whereas in one patient a perineal sinus has persisted for six years. Among the ten patients who survived a definitive colon resection of lesser extent than single stage total proctocolectomy, two patients required early (two weeks to four months) removal of the rectum for persistent disease as previously mentioned. In five others recurrent disease has developed in the rectum during the period of follow-up study which has required abdominoperineal resection one to eight years after initial colectomy. Despite these late complications, rehabilitation of each person with recurrence has been accomplished by resection of all bowel involved by persistent or recurrent colitis. The other less significant late complications in this group are summarized in Table X. These include three patients with ileostomy dysfunction. These three were among nine patients who had nonmatured ileostomies carried out initially. One of these had “backwash ileitis” which led to a total of four revisionary procedures during a fifteen year period. In three other patients initial colectomy for ulcerative colitis was performed without resection of the rectum. These men have been followed up for one, twelve, and fifteen years since colectomy without evident recurrence of disease and each has maintained good health.
Wound
Results
in small
infection
dysfunction flstula
4* 3* 1* 1* 1* 1*
94Yo * Prolonged
preoperative
medical
therapy
The
with
American
steroids.
Journal
of Surgery
Proctocolectomy Deflnitive
TABLE VII
Operations
Performed
in Fifty Patients
TABLE
IX
Colon
Resection
coloctomy Operation Total
proctocolectomy,
Staged
total
Colectomy, Resection Colectomy,
Number
ileostomy, of
rectum
descending
colon,
left
in place
colostomy
4
None
1
Nonfatal
Total
Procto-
Postoperative
Number
complications
Persistent
1
ileoproctostomy
Than Early
Complications
a
ileostomy
Extensive
Survivors):
Colitis
Complications
36
ileostomy
proctocolectomy,
of Patents
Less
(Ten
for Ulcerative
disease
Abdominal Wound
in rectum
abscess abscess
2
(l*)
1
(I*)
1
(I*)
of Patients
a
(4*)
2
(l*)
Comments * Prolonged
As mentioned earlier, we have made a concerted effort in this study to apply the clinical, radiologic, anatomic, histologic, and pathologic criteria originally suggested by Morson and Lockhart-Mummery [4] and more recently enunciated by Prohaska [I] to differentiate true ulcerative colitis from granulomatous colitis (Crohn’s disease). Since the cause of the two major varieties of inflammatory bowel disease remains obscure, there are no absolute criteria for differentiation. However, the differences, as previously outlined by Morson, Prohaska, and others, are sufficiently great in our opinion to recognize ulcerative colitis as a separate disease entity which in most cases can be accurately differentiated from granulomatous colitis and the other variants of Crohn’s disease. Our views concerning the characteristics of ulcerative colitis are summarized subsequently herein. Ulcerative colitis is typically an inflammatory disease of the mucosa of the bowel, usually diffusely involving the colon and rectum and limited to the large bowel. Its clinical course is characterized by cyclical exacerbations and remissions of bloody diarrhea with fulminant compiications in some patients and prolonged periods of quiescence in others. In patients with ulcerative colitis pseudopolyposis frequently develops, less fre-, quently the lethal problem of toxic dilatation of the colon, and with chronicity there is clearly an increased incidence of carcinoma of the colon and rectum. Spon-, taneous perforations of the colon in ulcerative colitis are very rare except when toxic dilatation occurs. Fistulization is almost exclusively confined to the perineum and vagina where fistulas are usually secondary to perianal abscesses. Total resection of the colon and rectum with ileostomy is curative in ulcerative colitis, and the disease does not tend to recur in the small bowel except in the infrequent cases when there is an accompanying “backwash ileitis.” TABLE VIII
Colon
Resection
Proctocolectomy Results
Less
Extensive
in Fourteen
Than
Total
Patients Number
TABLE X
Late
Early
2
Late
2
Survivors Survival
Vol.
119,
January
1970
rate
with
steroids.
of
All Colon
Colitis (Forty-Four
Resections
for
Survivors) Number
Number of Procedure Single
stage
No
late
Late
and total
Complications proctocolectomy
obstruction
Peristent
perineal
sinus
less than
11
2
2
3
0
3
6
4 6
complications
Persistent
5
IO
complications
Intestinal
Operative Procedures
total
proctocolectomy late
with
34
dysfunction
resection
Number
Complications
10
complications
Intestinal
Colon
Patients
of
Secondary
24
complications
lleostomy
Late
4
therapy
Complications
Ulcerative
lleostomy Deaths
medical
Pathologic examination of the colon in ulcerative colitis shows it to have multiple diffuse ulcerations of the mucosa and in most cases a relatively thin colonic wall without extensive submucosal fibrosis. The colonic mesentery, which usually does not contain enlarged lymph nodes, is apt to be normally thin or only slightly thickened and certainly not massively fibrotic. Microscopic examination of the wall of the inflamed colon shows diffuse macro- and microulcerations with adjacent edema, polymorphonuclear and eosinophilic cellular invasion of the mucosa, and destruction of the villi. Microscopic abscesses form in the crypts of Lieberkuhn and penetrate into the submucosa with the production of wider areas of ulceration of the overlying mucosa. Granulomas of the type seen in Crohn’s disease are conspicuously absent. On the other hand, there is usually definite increase in the number of Paneth cells in the colonic crypts. These cells are present in infrequent numbers in normal colons; however, Paterson and Watson [5] find them to be greatly increased in patients with ulcerative colitis. Instead of one or two Paneth cells per crypt in the normal colon, there may be 100 to 300 Paneth cells per crypt in colons with ulcerative colitis. The etiologic significance of this Paneth
No
of Patiantc
preoperative
dysfunction obstruction perineal
sinus
Thrombophlebitis 10
Abdominal
wall
71Yo
Recurrent
disease
abscess in rectum
1
1
1
2
1
0
1
1
5
5
_
91
Scott et al cell metaplasia sponse
is not known,
to ulcerative
but it is most likely a re-
colitis rather
than a factor
in etiol-
ogy. Since the cause and pathogenesis of ulcerative colitis remain unknown, the development of medical and surgical treatment of the disease has been totally empiric. Ileostomy alone has been given extensive trial in the past and has proved to be unsatisfactory as a definitive form of surgical treatment. However, as early as 1931 Rankin [6] demonstrated that ileostomy combined with total removal of the colon and rectum in stages could be curative in this disease. In 1940 Macguire [7] suggested and in 1949 Miller, Gardner, and Ripstein [8] reported that colectomy carried out at the time of ileostomy might be a more effective form of surgical management of the disease. In 1951 Ravitch and Handelsman [9] described a technic of single stage total proctocolectomy and used it successfully in six patients with chronic ulcerative colitis and in four with congenital polyposis of the colon. Since their report, the concept of performing ileostomy and total proctocolectomy simultaneously has become widely accepted as the procedure of choice when surgical intervention is indicated in the management of patients with ulcerative colitis. Despite the magnitude of the operation, this single stage procedure offers the cardinal advantage to the patient of immediate ablation of all inflammatory bowel disease, thus eliminating the serious complications of persistent or recurrent ulcerative colitis with the attendant morbidity and mortality. In 1958 in a series of forty patients, Bacon [IO] demonstrated that single stage ileostomy and proctocolectomy could be performed with an operative mortality of 2.5 per cent. More recent reports of larger series by Goligher [II], Prohaska [I], and others substantiate this commendable performance. Our experience with single stage proctocolectomy in thirty-six patients with an operative mortality of 2.8 per cent is further evidence of the validity of this approach. Klein et al [Z2] have raised the question as to whether a lesser procedure than single stage proctocolectomy should be performed in the critically ill patient with toxic dilatation of the colon. In our small surgical experience in six patients with toxic dilatation of the colon, five were submitted to the single stage procedure and in one patient, who was in shock during the conduct of emergency ileostomy with abdominal colectomy, removal of the distal sigmoid and rectum was deferred until two weeks later. The single death in this group occurred in a man who had multiple sealed off colonic perforations with unavoidable gross fecal contamination of the peritoneal cavity during emergency single stage proctocolectomy; he died with sepsis one month later. Both Goligher [II] and Prohaska [I] have advocated the use of the single stage procedure in both elec92
tive and ulcerative cessity for nitude can of patients
emergency management of patients with colitis. Our experience suggests that the neconsidering a primary procedure of less magbe diminished materially by earlier selection for surgical intervention. In this series of patients with ulcerative colitis treated by single stage proctocolcctomy. early postoperative complications occurred only in those patients who had received massive or prolonged preoperative steroid treatment. Accordingly, it is reasonable to raise the question as to the advisability of the use of prolonged or massive steroid therapy in ulcerative colitis. Goligher [II] has gone on record as opposing their use in this regard. We do not have controlled data available to help in answering this highly controversial question, but the incidence of postoperative complications in our patients who received steroids makes us very reluctant to use high dose or continuous long-term adrenal steroid therapy in patients with ulcerative colitis. The most important complication among the survivors of the multiple stage method of operative treatment in our small series was persistence or recurrence of ulcerative colitis in the rectum. Seven of ten survivors in this group have required secondary abdominoperineal resection of the rectum for this reason. Although no sweeping statistically significant conclusions may be drawn from this retrospective clinical study, our experience with single stage proctocolectomy for severe ulcerative colitis adds further support to the validity of the concept behind the procedure and prompts us to advocate its continued use. Summary Fifty patients with severe ulcerative colitis were submitted to major colonic resections during a fifteen year period. Thirty-six patients had single stage ileostomy and proctocolectomy with one early death (operative mortality 2.8 per cent). Fourteen patients had multiple stage procedures: usually ileostomy, abdominal colectomy with rectum left in place as the first stage. There were two early deaths in this group (operative mortality 14 per cent) and two later deaths. Second stage abdominoperineal resection of rectum because of recurrent ulcerative colitis has been necessary in seven of ten survivors of this group. This retrospective study validates the continued advocacy of single stage proctocolectomy in both elective and emergency management of patients with severe ulcerative colitis. An impressive degree of rehabilitation has been obtained in every patient after successful proctocolectomy. References 1. Prohaska JV: The inflammatory diseases of the large and small bowel. In: Current Problems in Surgery. Chicago, 1969. Year Book Medical Publishers, Inc. of ileostomy. Lancet 2: 102, 2. Brooke BN: Management 1952. The American
Journal
of Surgery
Proctocolectomy RB Jr: Colectomy with simultaneous ileostomy 3. Turnbull as the surgical treatment of diffuse ulcerative colitis. Surgery 4: 843, 1957. AE: Crohn’s disease 4. Morson BC and Lockhart-Mummery occurring primarily in colon. Gastroenterofogia 92: 168, 1959. in 5. Paterson JC and Watson SH: Paneth cell metaplasia ulcerative colitis. Amer J Path 38: 243, 1961. of colon and rectum: six 6. Rankin Fw: Total extirpation consecutive successful cases. Proc Staff Meet Mayo Clin 6: 436, 1931. 7. Macguire DP: Aseptic total colectomy. New York J Med 40: 1515, 1940. 8. Miller CG, Gardner CM, and Ripstein CB: Primary resecy;igof colon in ulcerative colitis. J Canad M A 60: 584, 9.
10. 11. 12.
Ravitch MM and Handelsman JC: One stage resection of entire colon and rectum for ulcerative colitis and polypoid adenomatosis. Bull Johns Hopkins Hosp 88: 59, 1951. Bacon HE: Ulcerative Colitis, p 322. Philadelphia, 1958. J B Lippincott Co. Goligher JC: Treatment of ulcerative colitis. In: Current Problems in Surgery. Chicago, 1965. Year Book Medical Publishers, Inc. Klein SH, Edelman S, Kirschner PA, Lyons AS, and Baronofsky ID: Emergency cecostomy in ulcerative colitis with acute toxic dilatation. Surgery 47: 399, 1960.
Discussion WILEY F BARKER: I certainly agree with the general premises and conclusions of this paper. We have some differences, however. As we consider our cases more seriously, the more we are finding that some of them we thought were not are indeed the granulomatous form. In the past fifteen years we have had about thirty emergency colectomies. Either of the types of colitis may appear in the first of these three classifications: fulminant, perforating, or bleeding. Toxic megacolon is largely restricted to the mucosal form. A total one stage colectomy should be performed for hemorrhage, perforation, and the fulminant form, unless the rectum is not involved in the granulomatous disease; otherwise, I think one has the series of complications Dr. Scott has shown. Our primary treatment of toxic megacolon has been early resection. We have not had a very good batting average until recently, but we now have a cumulative mortality of about 30 per cent. Difficulty has arisen in cases of perforation of the bowel or free contamination, or when we have inadvertently put a hole in the bowel. I would urge all to consider the lesser staged procedures of cecostomy that Dr Lyons has suggested and the “blow-hole” colostomy that Dr Turnbull has suggested, as an alternative means of handling the toxic megacolon. RICHARD T MYERS (Winston-Salem, NC) : I would like to compliment Dr Scott on his skill and cunning in keeping mortalities quite low in this desperate type of situation. Our experience has essentially been in agreement with his. My colleagues and I have treated 105 cases since 1949. In all we have attempted to complete a single stage proctocolectomy or to stage it, depending on the condition of the patient. Perhaps we have not been able to select our patients as well as we should have, that we Vol.
119, January
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should have been placing some of the patients, on whom we have performed a single stage proctocolectomy, into a staged group. The mortality for patients with a single stage proctocolectomy was 8 per cent and for those with multiple stage or incomplete stages at this time the mortality was 13.1 per cent. If we average out the mortalities of each of these series, they will fall grossly, for the total cases, between 6 and IO per cent. It is possible that the single stage procedure is the answer; however, the answer may lie in other factors such as earlier operation or avoidance of the routine use of steroids, as alluded to by Dr Scott. We have found that our fatalities have usually been due to infection. LOUIS T PALUMBO: I would like to add our experience in support of the program and policies discussed, because we find that our results, both in operative mortality and long-range follow-up study, are very similar to those in their series. In 1950 we started performing one stage total colectomy and ileostomy at the same time. In 1956 we changed our approach so that in the last eighteen patients since 1956 we removed the entire colon via the abdominal route, taking out the rectum and anal canal, dilating the anal outlet, and using it for a drainage site for the presacral space. This decreases the operating time considerably as well as blood loss and chemical losses, and results in lesser morbidity. In one patient we performed total one stage procedure using the abdominal approach solely without using the lithotomy or perineal approach. In addition to extensive ulcerative colitis this man also had congenital mucoceles of the colon; probably 150 or 200 of these were present. These were removed in 1956 and part of the specimen was sent to the Armed Forces Institute of Pathology. They reported this was the first case on record in the Institute at that time. CLARENCE DENNIS (Brooklyn, NY) : At the time of my departure from the University of Minnesota in 19.51, after sixteen years, the experience of a group of four of us with idiopathic ulcerative colitis was reviewed and reported in Surgery in 1952. There were 116 colectomies, of which fifty-five were primary, as published by WangenSteen and Toon in 1948, with one fatality. Of these, thirteen were primary total proctocolectomies with ileostomies, with no mortality. Since 1951, in Brooklyn, all proctocolectomies for idiopathic ulcerative colitis on the University Surgical Services have been primary, one stage procedures, and the mortality has remained at about 5 per cent. With regard to carcinomatous deterioration, which Dr Scott mentioned, cancer occurred in twenty-three patients in our own experience; twenty patients are dead and one is terminal. In a prospective study, cancer in the large intestine occurred in about 4 per cent per annum in those patients who had had the disease longer than ten years and who still retained their colon. The figure on the rectum alone was about 1 per cent per annum. We have found it very important to have rapid frozen section study of the terminal ileum before making an ileostomy. One can look grossly at the serosal and mucosal 93
Scott et al surfaces in this disease without recognizing so-called “backwash ileitis.” Our own figures would suggest that about 30 per cent of these patients have involvement of the ileum, and the ileostomies have not worked satisfactorily if made in diseased intestine. There have been many discussions on what to do with the perineum. I have never tried to remove all of the rectum from within the abdomen, but put the patient up in the lithotomy position. I repeatedly tried closing the perineum and concluded that there are sufficient complications in about one third of patients so closed which renders their convalescence difficult and prolonged; therefore, they have been packed open routinely. Periodically other surgeons tell me this course is overly cautious. I would like to know what other people do. OLIVER H BEAHRS (Rochester, Minn) : I cannot resist making a few comments regarding closure of the perineal wound, as brought up by Dr Dennis in his discussion. Over-all, in our experience, fifty per cent of perineal wounds, when packed open, heal satisfactorily whereas in 50 per cent there are complications. When the wound is closed primarily, 75 per cent heal and only twenty-five per cent have complications. Since adding suction drainage to the hollow of the sacrum five or six years ago, it is rare for any complications to develop when the perineal wound is closed primarily. We began to use primary closure in 1951 and, in addition to drainage, continue occasionally to use streptokinase and streptodornase as an irrigating agent in selected cases when it is thought that hematoma, necrotic tissue or exudate is present. DAVID H LAW (closing) : In answering the questions I will speak as a gastroenterologist. Doctor Barker, we are specifically concerned with chronic ulcerative colitis. I want to stress this point since you were referring in part to Crohn’s disease of the colon, in which quite probably the problem is different. In our series we took great pains to differentiate these two, both at initial pathologic and clinical review and at subsequent pathologic and radiologic review prior to reporting this series. Certainly toxic dilatation occurs primarily with ulcerative colitis. It is worth while to remember, however, that it has been reported also in Crohn’s disease of the colon, with amebiasis and one or two other disorders. In regard to Dr Myers’ question and comment, our low mortality may perhaps be related in part to earlier sur-
94
gery. I would like to comment mart on that later. I do rlc)t want to take all the credit away. hut I do think earlier surgery plays a role in the mortality. I would like to thank Drs Pa~umbo and Dennis fe.)r their confirmatory comments, and 1 WOLII~like to re:er their question on closing the pet-ineum to Dr Dunphy I know where it ib but I cannot close it. It is important to stress that the onus of operative martality lies not only with the operating surgeon but also with the referring physician. This has been accepted for a good period of time. Our studies suggest that the responsibility of the referring physician should also be extended to the associated operative morbidity since all of our early complications occurred in patients who had received either long-term or massive adrenocortical steroid therapy. Adrenocortical steroids do have a place in the treatment of ulcerative colitis. However, I would like to stress two points: (1) total proctocolectomy does remove ulcerative colitis, and (2) there is increasing practicality, as shown in the previous studies as well as in ours, of the well functioning ileostomy. Remembering these two things, we can and should begin to prepare the patient for operation at a somewhat earlier date, once operation is considered for ulcerative colitis. PRESIDENT DUNPHY: I cannot resist a comment, so I will say that we do close the posterior wound, at least I do and the residents who work with me do, leaving a drain, and then irrigating it. I do not think it makes much difference what you use for four or five days, but I am certain there are more complications in healing of the posterior wound in patients with ulcerative colitis than there are in patients with cancer. Some may recall the paper Dr Thomas Hunt in our department presented at the American Surgical Association on the use of topical vitamin A to reverse the deleterious effects of high-dose cortisone in the treatment of the healing wound. We are using this routinely in patients in this group if their wounds break down. I am convinced it is of great value; in fact, I recently repaired a total anal fistula. going above the levator ani in a woman with Crohn’s disease who was on high-dose steroids at the time. The disease was in moderate remission, and with topical vitamin A the wound healed as if she did not have either Crohn’s disease or steroids. If you have occasion to try it, we would like to hear from you.
The American Journal of Surgery