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Surgical management of ulcerative colitis in childhood
Surgical Management of Ulcerative Colitis in Childhood Robert Foglia, MD, Los Angeles,
California
Marvin E. Ament, MD,* Los Angeles, California David Fleisher, MD,* Los Angeles, California Eric W. Fonkalsrud, MD, Los Angeles, California
Ulcerative colitis (UC) is a common inflammatory disease of the bowel, which in children is character-
ized by recurrent bouts of bloody diarrhea, abdominal discomfort, decreased protein synthesis, weight loss, and failure of growth. Although children less than eighteen years old have been reported to account for only 15 to 20 per cent of all diagnosed cases of UC, several authors believe that the disease is more active in this age group than in adults [I] in whom the incidence is between 4 and 7.3 per 100,000 [2]. Today there is an apparent increase in the incidence of UC
in children, perhaps owing in part to greater cognizance among pediatricians concerning this disease, so that patients are now being specifically diagnosed. Nonetheless, there have been remarkably few reports summarizing experiences with the surgical management of UC in the pediatric age group [3,4]. The present report reviews our experience with fortyseven children who have undergone colectomy for UC at the UCLA Hospital during the past twenty years, with particular emphasis on the indications for operation and the long-term results. Various constitutional, psychosomatic, and immunologic theories have been proposed to explain the cause of UC. Each is based on the premise that the individual patients have some common antecedent factor which may have played a role in initiating their illness. However, it is impossible to indicate clearly whether any of these factors are etiologic rather than secondary to the disease. At present, there is no known definite etiology for UC. UC commonly begins in the rectum and sigmoid colon and may extend to involve the remainder of the colon. There may be radiologic evidence of inflammation of the distal 10 cm of ileum in one third of the cases [I]. The histologic abnormalities are generally confined to the mucosa and submucosa and are From the Departments of Surgery and Pediatrics’, UCLA School of Medicine, Los Angeles, California. Reprint requests should be addressed to Eric W. Fonkalsrud. MD, Department of Surgery, UCLA School of Medicine, Los Angeles, California 90024. Presented at the Forty-EighthAnnual Meeting of the Pacific Coast Surgical Association, Palm Springs, California, February 20-23, 1977.
5s
characterized by edema and inflammation. After repeated exacerbations, the mucosa loses its ability to effectively absorb water and electrolytes [2]. As long as the pathologic changes are localized to the mucosa and submucosa, peritoneal signs are rare. As the inflammatory process progresses, it usually penetrates deeper into the muscularis, ultimately causing fibrosis in addition to narrowing and shortening of the colon, with the resultant “pipe stem” appearance seen radiographically. If the inflammation extends to the serosa, peritoneal signs may develop and perforation can occur. This pathologic sequence can lead to loss of motor tone in an area of diseased colon and result in the development of toxic megacolon. Clinical Material Forty-seven children eighteen years of age and younger underwent operative management of UC at the UCLA Hospital during the twenty-year period from 1956 to 1976. Excluded from this review were patients whose initial operation was not performed at the UCLA Hospital or whose pathologic report was not specifically diagnostic of UC. There were thirty males and seventeen females, ranging in age from seven months to eighteen years (mean, 15.9 years) at the time of operation, and six patients were younger than ten years when colectomy was performed. The most frequent symptoms occurring at some time during the course of the patient’s illness were diarrhea (45 patients, 95.7 per cent), rectal bleeding (36 patients, 76.6 per cent), weight loss of more than 5 pounds (36 patients, 76.6 per cent), abdominal pain (31 patients, 66 per cent), fever (17 patients, 36.2 per cent), and nausea and vomiting (10 patients, 21.3 per cent). Frequently noted ‘extracolonic manifestations included secondary amenorrhea, which developed during acute episodes of colitis in three of the eleven females (27.3 per cent) who were more than fourteen years of age. Other extracolonic symptoms reported were arthralgias (9 patients, 19.1 per cent), skin lesions (8 patients, 17 per cent) (primary erythema nodosum in 4 patients and pyoderma gangrenosum in 3 patients), anal fissures (4 patients, 8.5 per cent), convulsions (2 patients, 4.2 per cent), and duodenal ulcers (2 patients, 4.2 per cent).
The American Journal 01 Surgery
Childhood
Conditions that were prominent on physical examination prior to operation were generalized debilitation, muscle weakness, and pallor. Abdominal discomfort in nearly all of the patients was diffuse, mild to moderate in severity, and rarely localized. None of the children experienced colonic perforation, although seven exhibited symptoms and signs suggesting peritonitis. Three children experienced sudden onset of abdominal distention, pain, tachycardia, fever, and ileus and were diagnosed as having toxic megacolon. In one of these patients the acute symptoms may have been precipitated by recent barium enema. Among the abnormalities frequently found in preoperative laboratory studies were bloody stools (35 patients, 74.5 per cent), anemia (hematocrit <30) (31 patients, 66.0 .per cent), hypoalbuminemia (<3 gm/lOO ml) (18 patients, 38.3 per cent), and leukocytosis (>lO,OOO)(10 patients, 21.3 per cent). Eighteen patients received one or more blood transfusions before undergoing colectomy. Barium roentgenograms of the colon showed abnormal features as evidenced by mucosal ulceration, decreased length and caliber of the colon, decreased distensibility, and absence of haustra and were diagnostic of UC in each of the fortyseven patients studied. The severity of the colitis, however, often did not correlate directly with the degree of the radiographic abnormalities. Sigmoidoscopy showed findings consistent with UC in each of forty-four children, as indicated by mucosal friability, erythema, and easy bleeding, but rarely revealed ulceration. Mucosal biopsies showed characteristic crypt and submucosal inflammation without granulomas in each of fourteen patients on whom biopsies were performed. Twenty-one children underwent small bowel roentgenographic examination; six had terminal ileal involvement and two had a duodenal ulcer. Treatment
Each of the children underwent an intensive course of medical therapy before colectomy was considered. For patients treated before 1966, Azulfadinee was usually administered as the initial therapy. When steroids were used, there was usually considerable variation in the dosage and duration of therapy. Currently, children with active UC are initially treated with oral prednisone, which should lead to a remission within fourteen days in approximately 75 per cent of these patients. If the response to steroids is good, the dosage is halved within two weeks and then gradually tapered over the next two months. Azulfadine treatment is then started after remission has occurred. Children who do not respond within two weeks are admitted to the hospital for intravenous steroids, which may be administered up to a maximum daily dosage of 60 mg. If this therapy fails, the patient is usually referred for operation. In the present study, twenty-three patients received both steroids and Azulfadine, fifteen received only steroids, and two received Azulfadine alone at some
Volume 134,July1977
fi
Ulcerative
Colitis
EM:,tdCY
---Intractablediarrhea Figure 1. Indications for operative Intervention.
point during their illness. Rectal steroids provided little benefit in inducing a remission. Patients were rarely maintained on steroids for longer than two months, but many underwent multiple courses of steroid therapy for repeated exacerbations. Although a number of these children experienced a weight gain with steroid therapy, it is believed that in the majority of cases this was due to water retention and change in body habitus rather than to remission of their disease. One boy who received prednisone for ten months developed moderately severe osteoporosis. The duration of symptoms before operation ranged from four months to five and a half years (mean, 21 months). Thirty-four children underwent elective operation, indications for which included failure of medical therapy to cause or sustain a prolonged remission, failure to grow for one or more years, and intractability of symptoms such as diarrhea, tenesmus, and bloody mucoid discharge. (Figure 1.) The remaining thirteen patients underwent emergency operation-seven for peritonitis with suspected colonic perforation, four for significant colonic bleeding, one for toxic megacolon, and one for trolyte disorders. Total colectomy with proctectomy and ileostomy were performed as the initial operation on twentythree children. Subtotal colectomy and ileostomy were performed on twenty-four patients. Colectomy was accomplished through a left paramedian skin incision which was slanted medially in the subcutaneous tissue to the midline to keep the incision distant from the ileostomy stoma. The distal ileum was divided as close to the cecum as feasible and rarely more than 5 cm proximal to the ileocecal valve to retain maximum water-absorbing surface. In our experience, the terminal ileum is rarely inflamed enough to prevent good healing of the ileostomy stoma after colectomy. Proctectomy is performed cautiously by sharp dissection, with ligation of vessels
59
Foglia et al TABLE I
Postoperative Complications
Colectomy Emergency Elective Total
l
Patients with Complications/Total No. Operated 1956 to 1965 1966 to 1976 Total 7’18 9115 16’123 (69.6%)
315 8119 11124 (45.8%)
10’113 (76.9%) 17134 (50%) 27’147 (57.4%)
3 deaths.
TABLE II
Complications versus Type of Operation
atively. Although intravenous hyperalimentation does not lead to remission of UC, as it may in Crohn’s disease, it appears to assist substantially in improving the rate of wound healing and weight gain. Morbidity and Mortality
Three of the forty-seven children (6.3 per cent) died subsequent to emergency subtotal colectomy. Two of them died during the immediate nostoDerative period as a result of sepsis. The third patient at first progressed well after colectomy but experienced progressive rectal inflammation; she underwent proctectomy within eight months and died two months later due to sepsis. Each of these three patients received steroid therapy for more than three months preoperatively, and in each death was caused by wound disruption, bowel perforation, and peritonitis. No deaths have occurred after operation in the last thirty-six patients who underwent colectomy during the past fifteen years. Postoperative complications were more common after emergency colectomy than after elective operations. (Table I.) Ten of the thirteen children who had emergency colectomies developed complications, compared with only seventeen of thirty-four patients who were operated on electively. Sixteen of the twenty-three patients (69.6 per cent) who underwent operation before 1966 developed complications; however, only eleven of twenty-four patients since 1966 (45.8 per cent) suffered complications. Since 1970, the overall morbidity has decreased to 25 per cent. The incidence of complications was 47.8 per cent in patients who had total colectomy and ileostomy, whereas 66.7 per cent of those patients who underwent subtotal colectomy and ileostomy developed complications. (Table II.) Steroid administration made no significant difference in the incidence of complications, which d&eloped in 57.7 per cent of those who received steroids and 57.1 per cent of those who did not. Indeed, poor results correlated more closely with the severity of the disease at the time of operation than with any other factors, The most common complications that occurred during the first postoperative month were wound infection (6 patients) and ileostomy dysfunction (5). Small bowel obstruction (4) and perforation (3) were frequently observed in children receiving long-term steroid treatment during the early years of the study. One patient each developed seizure, subphrenic abscess, pneumonia, and neurogenic bladder. Complications that developed late in the postoperative period included ileostomy stricture (5), ileostomy prolapse (l), wound infection (5), small bowel obA
Colectomy Total Subtotal
Patients with Complications/Total No. Operated 1956 to 1965 1966 to 1976 Total 518 1 l/l5
6115 519
1 l/23 (47.8%) 16124 (66.7%)
close to the muscularis to avoid injuring the neurogenie supply to the bladder and genitalia and to reduce blood loss. Few blood transfusions were used for total colectomy in this group of children. The tissues below the pelvic floor are approximated to obliterate dead space, and the perineal wound is left open with a drain and catheter. The ileostomy stoma is brought through the right lower abdomen below the belt line and matured after excising a disc of skin and underlying muscle. Patients who are scheduled for elective Cole&my are put on a clear liquid diet for 48 hours prior to operation, and if severely debilitated, they are given intravenous hyperalimentation for a period ranging from several days to weeks preoperatively. An enterostomal nurse specialist visits the patients before operation to help prepare them for ileostomy. Parenteral antibiotics are started preoperatively in all patients. Various combinations of antibiotics have been used over the past twenty years, although we now prefer the combination of gentamicin and clindamycin. Enemas, opiates, and anticholinergic drugs are avoided to decrease the likelihood of development of acute toxic megacolon. Children who have received steroids within the past year are administered steroids both pre- and postoperatively. Both the child and his parents are carefully instructed regarding the management of the stoma, and in most instances even a child as young as six years will take charge effectively and will experience only minimal interference with a full, active life. Patients who required emergency colectomy were administered appropriate volumes of crystalloid, protein solutions, and blood when necessary to correct fluid deficits preoperatively. A central venous catheter has been utilized in most patients during the past six years to monitor the fluid replacement and to deliver intravenous hyperalimentation postoper-
60
Lo
The American Journal of Surgery
Childhood Ulcerative Colitis struction (4), peristomal hernia (2), and flank (2), pelvic (l), and peripancreatic abscess (1). Nineteen patients required operative management of their complications. Five of the thirty-six children who underwent proctectomy during the course of their management experienced delayed healing (more than 6 months) of their perineal wounds. This was probably related to the extent of inflammation in the perirectal tissues, since this was .characteristic of children with severe disease. Results of Surgical Treatment
Seventeen of the twenty-three patients who underwent total colectomy and ileostomy have been followed for at least one year. Each has experienced complete remission of symptoms, significant weight gain, resumption of full activities, and their medications were discontinued within three months of operation. Thirteen of the twenty-four children who initially underwent subtotal colectomy have subsequently undergone removal of the rectal segment owing to worsened residual disease; this procedure was performed an average of twenty-eight months (2 months to 6.5 years) after the initial operation. Two of the remaining eleven patients with retained rectal segments died early in the postoperative period and two were followed for less than one year. In seven children rectal segments have now been retained for one to thirteen years after subtotal colectomy. None have had permanent remission or reestablishment of successful bowel continuity. A striking objective change in these children was their weight gain. Twenty patients preoperatively weighed less than the sixteenth percentile for their age, and had a mean of 70.7 per cent ideal weight. Sixteen of these patients followed longer than one year increased their mean weight to 86.7 per cent of ideal weight for age. Comments
UC in children is often more severe than in adults and must be suspected in any child with intractable diarrhea in whom pathogenic organisms are not cultured, regardless of age except for the neonatal period. Initial management after a diagnosis has been made should consist of judicious medical therapy in an attempt to induce long-term remission. Children who have not responded well to steroid therapy in a hospital setting should be considered for colectomy. This should not be done as a last resort after several years of exacerbations, marked growth retardation, multiple blood transfusions, and the development of the side effects of long-term steroid therapy including
Vohme134,July
1977
peptic ulceration, infection, and osteoporosis. Another factor that may influence the decision regarding operative intervention is the knowledge that in young adolescents, because of epiphyseal fusion, growth retardation may be permanent. Furthermore, Devroede et al [5] have indicated the increased risk of developing carcinoma associated with the childhood onset of colitis. Emergency operation should be performed for peritonitis and suspected bowel perforation, significant intestinal bleeding, or toxic megacolon. The number of emergency operations in our hospital has declined over the last ten years, probably due to earlier referral of patients for elective colectomy before significant and often life-threatening complications arise. Inasmuch as UC usually involves the rectum and then, progressively, the remainder of the colon, total coloproctectomy and ileostomy has become the most commonly performed operation, since this completely eliminates the disease. Lesser operations are almost invariably followed by recurrence or persistence of active inflammation in the remaining colon or rectum. Subtotal colectomy was often done during the first ten years of the present study. At that time many children were referred for operation late in the course of the disease, when they were considered too ill to undergo simultaneous removal of the colon and rectum. The decision to leave the rectum has also been based on the hope of eventually reestablishing intestinal continuity, as championed by Aylett [6]. However, only seven of the twenty-four patients who had subtotal colectomies still have not undergone proctectomy, and no patient has had successful bowel continuity established. The overall incidence of complications was 19 per cent lower in the patients who underwent one-stage total colectomy compared with those who underwent subtotal colectomy, although the latter were performed on sicker patients during the early years of our experience. Moreover, for the past six years, the complication rate with total coloproctectomy has decreased to 22 per cent. Finally, the increased risk of developing carcinoma is obviated if the rectum is also removed. Nevertheless, in children with mild rectal disease, there is evidence to suggest that subtotal colectomy and removal of the rectal mucosa followed by an endorectal pullthrough procedure similar to that used for Hirschsprung’s disease may be feasible [7]. Although the Koch ileal reservoir has not been used on any child in the present study, this might be considered as a second step, preferably not until late adolescence, for a child suffering complications with his ileostomy stoma. A modified Koch pouch is cur-
61
Foglia et al
rently under evaluation for use in combination with the endorectal pullthrough operation. Although the overall morbidity for colectomy in the present series was 57.4 per cent, this figure has been reduced to 25 per cent during the past six years. Also, the mean postoperative period of hospitalization before 1966 was 25.4 days. Since 1966, however, this period has decreased to 11.5 days. We believe that these two statistics reflect earlier referrals for operation and better pre- and postoperative management. Our review shows that total colectomy leads to complete cure of the disease as well as to significant reversal of growth retardation in many of these patients. Summary
Forty-seven children with ulcerative colitis (UC) have been treated by colectomy at the UCLA Hospital in the two decades since 1956. An intensive course of medical therapy including a short course of steroid treatment in an attempt to obtain clinical remission is recommended before proceeding with colectomy. Thirty-four children underwent elective colectomy, indicated by intractability of symptoms and a year of growth failure. Postoperative complication rates were higher after emergency colectomy (77 per cent) than after elective operation (50 per cent). The only deaths (3) occurred subsequent to emergency colectomy. Judicious use of preoperative steroids did not increase the incidence of postoperative complications. Thirteen of twenty-four children who underwent initial subtotal colectomy had the remaining rectum removed because of progression of disease within an average period of twenty-eight months. No child underwent successful ileorectal anastomosis after initial subtotal colectomy. Inasmuch as UC usually begins in the rectum, most children with this condition during the past ten years have been treated by total colectomy. Postoperative weight gain and improvement in quality of life were strikingly evident in almost all children who underwent total coloproctectomy. Colectomy is recommended particularly for children who do not experience remission from acute disease after steroid therapy and for those who have chronic disease during the growth period of adolescent years. References 1. Berger M, Gribetz D, Korelitz BI: Growth retardation in children with ulcerative colitis: the effect of medical and surgical therapy. Pediatrics 55: 459, 1975. 2. Ament ME: Inflammatory disease of the colon: ulcerative colitis and Crohn’s colitis. J Pediatr 86: 322, 1975. 3. Levin P, Fonkalsrud FW, Barker WF: Surgical treatment for pe-
62
diatric ulcerative colitis. Surgery 60: 201, 1966. 4. Frey CF. Weaver DK: Colectomy in children with ulcerative and granulomatous colitis. Arch Surg 104: 416, 1972. 5. Devroede GJ, Taylor WF, Saver WG, et al: Cancer risk and life expectancy of children with ulcerative colitis. N Engl J A&d 285: 17, 1971. 6. Aylett SO: Three hundred cases of diffuse ulcerative colitis treated by total colectomy and ileo-rectal anastomosis. Br Med J 1: 1001, 1966. 7. Levin P, Fonkalsrud EW: Fecal continence following colectomy and ileoanal anastomosis through extramucosal rectal tube. J Surg Res 8: 234, 1968.
Discussion Morton M. Woolley (Los Angeles, CA): Any surgeon who has dealt with even one child with chronic ulcerative colitis (UC) recognizes the complex nature of patients whose problems are emotional, nutritional, anatomic, physiologic, and potentially malignant. It takes a great deal of time, patience, and expertise to decide whether to operate, when to operate, and how much to operate. At our present state of knowledge, or perhaps more accurately our current state of ignorance, surgical removal of the colon does alleviate the symptoms of chronic UC and does prevent the development of malignancy. The best operation is removal of the entire colon at one stage, if feasible. This obviates the continuation of the disease process and extracolonic manifestations and bleeding and fever which so often plague the patient who has the rectal stump left in place. One is, and always should be, reticent to commit a child to a lifelong ileostomy. If the illness progresses far enough to convince the patient and his family that ileostomy is better than the existing disease, they accept and adjust to it better than if they believe they have been pushed into an operation somewhat against their will. This does not excuse postponement of a desirable operation beyond the time it can be accomplished safely. The increased complication rate in the emergency group of patients in this series speaks for itself in this respect. Since I deal only with the childhood age group, I find that parents frequently have deep feelings of guilt when their infant or child is sick, particularly mothers who think if they could just try a little harder, they could overcome the problem from which their child is suffering. Recently I performed an urgent colectomy on a thirteen year old boy for exsanguinating hemorrhage from chronic UC. He developed postoperative complications including pelvic abscess and intestinal obstruction. His mother was in constant attendance day and night and reported to me each day regarding any minor ineptness on the part of the nurses, as well as any problem the house officers had in starting the many intravenous units day and night. After the patient recovered adequately to be discharged, his mother attempted suicide and required confined psychiatric care. The psychiatrist who had seen her and the patient in the hospital stated that he was not surprised of this event because of the extreme anxiety and guilt that she turned outward on the nurses and house officers in the hospital and inward on herself later. On the brighter side, after recovering from colectomy one of my
The American Journal of Surgery
Childhood Ulcerative Colitis
young patients said to me, “Dr. Woolley, do I have to go back and see that psychiatrist? You know I am cured now.” I should like to close with three questions to the authors. One, your youngest patient was taken care of between the ages of two and seven months. Did he actually have chronic UC, or was this some variant of necrotizing enterocolitis? (Slide) This is a patient who was perfectly well for five years after total colectomy for chronic UC, at which time she developed necrotizing ileitis and eventually died of invasive sepsis. Have you had any late sequelae such as this? (Slide) This is the x-ray appearance of a patient who obviously has chronic UC. He had required a portacaval shunt for portal hypertension secondary to cirrhosis and subsequently had colectomy for chronic UC. Have you had a patient in the childhood age group who has required both colectomy and portacaval shunt as a part of the complex disease process? Eric W. Fonkalsrud (closing): The fact that so many children with UC have been recognized within the past ten years perhaps reflects the advances in the new field of pediatric gastroenterology. Although it is possible that the condition is occurring more frequently, it is more likely that we have just missed diagnosing it in many children in the past. It has only been during the past decade that we have clearly separated patients with UC from those with Crohn’s disease.
Volume 134, July 1977
As Doctor Woolley has indicated, UC may occur in young children and is more likely to do so than is Crohn’s disease. Six patients less than ten years of age in our hospital underwent colectomy for UC; however, no patient less than ten years of age has undergone bowel resection for Crohn’s disease. Although it is unusual for an infant to require colectomy for UC, we have had one child who experienced severe diarrhea and blood loss since the second month of life. Colectomy was performed at the age of seven months and the pathology specimen was diagnostic of UC. Fortunately, the complications Doctor Woolley presented are uncommon, and we have not had experience with a patient developing necrotizing ileitis and would suspect the diagnosis might be Crohn’s disease. Although mild liver disease in patients with UC is not uncommon, the association with portal hypertension is rare. Our studies suggest that children who experience moderate to severe symptoms from UC are unlikely to obtain remission despite therapy, and colectomy should seriously be considered before the child develops both retardation and systemic complications of the disease. Children are able to accept and use an ileostomy remarkably well provided they are given adequate instructions by the physician and his enterostomal nurse. Parents are likely to exhibit many guilt feelings about the disease and the surgeon must have considerable -patience and empathy. The parents, pediatrician, and even the patient will be pleasantly surprised in most instances with regard to the rapid gain in both weight and height which adolescent children are likely to experience after colectomy.
63
California
Marvin E. Ament, MD,* Los Angeles, California David Fleisher, MD,* Los Angeles, California Eric W. Fonkalsrud, MD, Los Angeles, California
Ulcerative colitis (UC) is a common inflammatory disease of the bowel, which in children is character-
ized by recurrent bouts of bloody diarrhea, abdominal discomfort, decreased protein synthesis, weight loss, and failure of growth. Although children less than eighteen years old have been reported to account for only 15 to 20 per cent of all diagnosed cases of UC, several authors believe that the disease is more active in this age group than in adults [I] in whom the incidence is between 4 and 7.3 per 100,000 [2]. Today there is an apparent increase in the incidence of UC
in children, perhaps owing in part to greater cognizance among pediatricians concerning this disease, so that patients are now being specifically diagnosed. Nonetheless, there have been remarkably few reports summarizing experiences with the surgical management of UC in the pediatric age group [3,4]. The present report reviews our experience with fortyseven children who have undergone colectomy for UC at the UCLA Hospital during the past twenty years, with particular emphasis on the indications for operation and the long-term results. Various constitutional, psychosomatic, and immunologic theories have been proposed to explain the cause of UC. Each is based on the premise that the individual patients have some common antecedent factor which may have played a role in initiating their illness. However, it is impossible to indicate clearly whether any of these factors are etiologic rather than secondary to the disease. At present, there is no known definite etiology for UC. UC commonly begins in the rectum and sigmoid colon and may extend to involve the remainder of the colon. There may be radiologic evidence of inflammation of the distal 10 cm of ileum in one third of the cases [I]. The histologic abnormalities are generally confined to the mucosa and submucosa and are From the Departments of Surgery and Pediatrics’, UCLA School of Medicine, Los Angeles, California. Reprint requests should be addressed to Eric W. Fonkalsrud. MD, Department of Surgery, UCLA School of Medicine, Los Angeles, California 90024. Presented at the Forty-EighthAnnual Meeting of the Pacific Coast Surgical Association, Palm Springs, California, February 20-23, 1977.
5s
characterized by edema and inflammation. After repeated exacerbations, the mucosa loses its ability to effectively absorb water and electrolytes [2]. As long as the pathologic changes are localized to the mucosa and submucosa, peritoneal signs are rare. As the inflammatory process progresses, it usually penetrates deeper into the muscularis, ultimately causing fibrosis in addition to narrowing and shortening of the colon, with the resultant “pipe stem” appearance seen radiographically. If the inflammation extends to the serosa, peritoneal signs may develop and perforation can occur. This pathologic sequence can lead to loss of motor tone in an area of diseased colon and result in the development of toxic megacolon. Clinical Material Forty-seven children eighteen years of age and younger underwent operative management of UC at the UCLA Hospital during the twenty-year period from 1956 to 1976. Excluded from this review were patients whose initial operation was not performed at the UCLA Hospital or whose pathologic report was not specifically diagnostic of UC. There were thirty males and seventeen females, ranging in age from seven months to eighteen years (mean, 15.9 years) at the time of operation, and six patients were younger than ten years when colectomy was performed. The most frequent symptoms occurring at some time during the course of the patient’s illness were diarrhea (45 patients, 95.7 per cent), rectal bleeding (36 patients, 76.6 per cent), weight loss of more than 5 pounds (36 patients, 76.6 per cent), abdominal pain (31 patients, 66 per cent), fever (17 patients, 36.2 per cent), and nausea and vomiting (10 patients, 21.3 per cent). Frequently noted ‘extracolonic manifestations included secondary amenorrhea, which developed during acute episodes of colitis in three of the eleven females (27.3 per cent) who were more than fourteen years of age. Other extracolonic symptoms reported were arthralgias (9 patients, 19.1 per cent), skin lesions (8 patients, 17 per cent) (primary erythema nodosum in 4 patients and pyoderma gangrenosum in 3 patients), anal fissures (4 patients, 8.5 per cent), convulsions (2 patients, 4.2 per cent), and duodenal ulcers (2 patients, 4.2 per cent).
The American Journal 01 Surgery
Childhood
Conditions that were prominent on physical examination prior to operation were generalized debilitation, muscle weakness, and pallor. Abdominal discomfort in nearly all of the patients was diffuse, mild to moderate in severity, and rarely localized. None of the children experienced colonic perforation, although seven exhibited symptoms and signs suggesting peritonitis. Three children experienced sudden onset of abdominal distention, pain, tachycardia, fever, and ileus and were diagnosed as having toxic megacolon. In one of these patients the acute symptoms may have been precipitated by recent barium enema. Among the abnormalities frequently found in preoperative laboratory studies were bloody stools (35 patients, 74.5 per cent), anemia (hematocrit <30) (31 patients, 66.0 .per cent), hypoalbuminemia (<3 gm/lOO ml) (18 patients, 38.3 per cent), and leukocytosis (>lO,OOO)(10 patients, 21.3 per cent). Eighteen patients received one or more blood transfusions before undergoing colectomy. Barium roentgenograms of the colon showed abnormal features as evidenced by mucosal ulceration, decreased length and caliber of the colon, decreased distensibility, and absence of haustra and were diagnostic of UC in each of the fortyseven patients studied. The severity of the colitis, however, often did not correlate directly with the degree of the radiographic abnormalities. Sigmoidoscopy showed findings consistent with UC in each of forty-four children, as indicated by mucosal friability, erythema, and easy bleeding, but rarely revealed ulceration. Mucosal biopsies showed characteristic crypt and submucosal inflammation without granulomas in each of fourteen patients on whom biopsies were performed. Twenty-one children underwent small bowel roentgenographic examination; six had terminal ileal involvement and two had a duodenal ulcer. Treatment
Each of the children underwent an intensive course of medical therapy before colectomy was considered. For patients treated before 1966, Azulfadinee was usually administered as the initial therapy. When steroids were used, there was usually considerable variation in the dosage and duration of therapy. Currently, children with active UC are initially treated with oral prednisone, which should lead to a remission within fourteen days in approximately 75 per cent of these patients. If the response to steroids is good, the dosage is halved within two weeks and then gradually tapered over the next two months. Azulfadine treatment is then started after remission has occurred. Children who do not respond within two weeks are admitted to the hospital for intravenous steroids, which may be administered up to a maximum daily dosage of 60 mg. If this therapy fails, the patient is usually referred for operation. In the present study, twenty-three patients received both steroids and Azulfadine, fifteen received only steroids, and two received Azulfadine alone at some
Volume 134,July1977
fi
Ulcerative
Colitis
EM:,tdCY
---Intractablediarrhea Figure 1. Indications for operative Intervention.
point during their illness. Rectal steroids provided little benefit in inducing a remission. Patients were rarely maintained on steroids for longer than two months, but many underwent multiple courses of steroid therapy for repeated exacerbations. Although a number of these children experienced a weight gain with steroid therapy, it is believed that in the majority of cases this was due to water retention and change in body habitus rather than to remission of their disease. One boy who received prednisone for ten months developed moderately severe osteoporosis. The duration of symptoms before operation ranged from four months to five and a half years (mean, 21 months). Thirty-four children underwent elective operation, indications for which included failure of medical therapy to cause or sustain a prolonged remission, failure to grow for one or more years, and intractability of symptoms such as diarrhea, tenesmus, and bloody mucoid discharge. (Figure 1.) The remaining thirteen patients underwent emergency operation-seven for peritonitis with suspected colonic perforation, four for significant colonic bleeding, one for toxic megacolon, and one for trolyte disorders. Total colectomy with proctectomy and ileostomy were performed as the initial operation on twentythree children. Subtotal colectomy and ileostomy were performed on twenty-four patients. Colectomy was accomplished through a left paramedian skin incision which was slanted medially in the subcutaneous tissue to the midline to keep the incision distant from the ileostomy stoma. The distal ileum was divided as close to the cecum as feasible and rarely more than 5 cm proximal to the ileocecal valve to retain maximum water-absorbing surface. In our experience, the terminal ileum is rarely inflamed enough to prevent good healing of the ileostomy stoma after colectomy. Proctectomy is performed cautiously by sharp dissection, with ligation of vessels
59
Foglia et al TABLE I
Postoperative Complications
Colectomy Emergency Elective Total
l
Patients with Complications/Total No. Operated 1956 to 1965 1966 to 1976 Total 7’18 9115 16’123 (69.6%)
315 8119 11124 (45.8%)
10’113 (76.9%) 17134 (50%) 27’147 (57.4%)
3 deaths.
TABLE II
Complications versus Type of Operation
atively. Although intravenous hyperalimentation does not lead to remission of UC, as it may in Crohn’s disease, it appears to assist substantially in improving the rate of wound healing and weight gain. Morbidity and Mortality
Three of the forty-seven children (6.3 per cent) died subsequent to emergency subtotal colectomy. Two of them died during the immediate nostoDerative period as a result of sepsis. The third patient at first progressed well after colectomy but experienced progressive rectal inflammation; she underwent proctectomy within eight months and died two months later due to sepsis. Each of these three patients received steroid therapy for more than three months preoperatively, and in each death was caused by wound disruption, bowel perforation, and peritonitis. No deaths have occurred after operation in the last thirty-six patients who underwent colectomy during the past fifteen years. Postoperative complications were more common after emergency colectomy than after elective operations. (Table I.) Ten of the thirteen children who had emergency colectomies developed complications, compared with only seventeen of thirty-four patients who were operated on electively. Sixteen of the twenty-three patients (69.6 per cent) who underwent operation before 1966 developed complications; however, only eleven of twenty-four patients since 1966 (45.8 per cent) suffered complications. Since 1970, the overall morbidity has decreased to 25 per cent. The incidence of complications was 47.8 per cent in patients who had total colectomy and ileostomy, whereas 66.7 per cent of those patients who underwent subtotal colectomy and ileostomy developed complications. (Table II.) Steroid administration made no significant difference in the incidence of complications, which d&eloped in 57.7 per cent of those who received steroids and 57.1 per cent of those who did not. Indeed, poor results correlated more closely with the severity of the disease at the time of operation than with any other factors, The most common complications that occurred during the first postoperative month were wound infection (6 patients) and ileostomy dysfunction (5). Small bowel obstruction (4) and perforation (3) were frequently observed in children receiving long-term steroid treatment during the early years of the study. One patient each developed seizure, subphrenic abscess, pneumonia, and neurogenic bladder. Complications that developed late in the postoperative period included ileostomy stricture (5), ileostomy prolapse (l), wound infection (5), small bowel obA
Colectomy Total Subtotal
Patients with Complications/Total No. Operated 1956 to 1965 1966 to 1976 Total 518 1 l/l5
6115 519
1 l/23 (47.8%) 16124 (66.7%)
close to the muscularis to avoid injuring the neurogenie supply to the bladder and genitalia and to reduce blood loss. Few blood transfusions were used for total colectomy in this group of children. The tissues below the pelvic floor are approximated to obliterate dead space, and the perineal wound is left open with a drain and catheter. The ileostomy stoma is brought through the right lower abdomen below the belt line and matured after excising a disc of skin and underlying muscle. Patients who are scheduled for elective Cole&my are put on a clear liquid diet for 48 hours prior to operation, and if severely debilitated, they are given intravenous hyperalimentation for a period ranging from several days to weeks preoperatively. An enterostomal nurse specialist visits the patients before operation to help prepare them for ileostomy. Parenteral antibiotics are started preoperatively in all patients. Various combinations of antibiotics have been used over the past twenty years, although we now prefer the combination of gentamicin and clindamycin. Enemas, opiates, and anticholinergic drugs are avoided to decrease the likelihood of development of acute toxic megacolon. Children who have received steroids within the past year are administered steroids both pre- and postoperatively. Both the child and his parents are carefully instructed regarding the management of the stoma, and in most instances even a child as young as six years will take charge effectively and will experience only minimal interference with a full, active life. Patients who required emergency colectomy were administered appropriate volumes of crystalloid, protein solutions, and blood when necessary to correct fluid deficits preoperatively. A central venous catheter has been utilized in most patients during the past six years to monitor the fluid replacement and to deliver intravenous hyperalimentation postoper-
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Lo
The American Journal of Surgery
Childhood Ulcerative Colitis struction (4), peristomal hernia (2), and flank (2), pelvic (l), and peripancreatic abscess (1). Nineteen patients required operative management of their complications. Five of the thirty-six children who underwent proctectomy during the course of their management experienced delayed healing (more than 6 months) of their perineal wounds. This was probably related to the extent of inflammation in the perirectal tissues, since this was .characteristic of children with severe disease. Results of Surgical Treatment
Seventeen of the twenty-three patients who underwent total colectomy and ileostomy have been followed for at least one year. Each has experienced complete remission of symptoms, significant weight gain, resumption of full activities, and their medications were discontinued within three months of operation. Thirteen of the twenty-four children who initially underwent subtotal colectomy have subsequently undergone removal of the rectal segment owing to worsened residual disease; this procedure was performed an average of twenty-eight months (2 months to 6.5 years) after the initial operation. Two of the remaining eleven patients with retained rectal segments died early in the postoperative period and two were followed for less than one year. In seven children rectal segments have now been retained for one to thirteen years after subtotal colectomy. None have had permanent remission or reestablishment of successful bowel continuity. A striking objective change in these children was their weight gain. Twenty patients preoperatively weighed less than the sixteenth percentile for their age, and had a mean of 70.7 per cent ideal weight. Sixteen of these patients followed longer than one year increased their mean weight to 86.7 per cent of ideal weight for age. Comments
UC in children is often more severe than in adults and must be suspected in any child with intractable diarrhea in whom pathogenic organisms are not cultured, regardless of age except for the neonatal period. Initial management after a diagnosis has been made should consist of judicious medical therapy in an attempt to induce long-term remission. Children who have not responded well to steroid therapy in a hospital setting should be considered for colectomy. This should not be done as a last resort after several years of exacerbations, marked growth retardation, multiple blood transfusions, and the development of the side effects of long-term steroid therapy including
Vohme134,July
1977
peptic ulceration, infection, and osteoporosis. Another factor that may influence the decision regarding operative intervention is the knowledge that in young adolescents, because of epiphyseal fusion, growth retardation may be permanent. Furthermore, Devroede et al [5] have indicated the increased risk of developing carcinoma associated with the childhood onset of colitis. Emergency operation should be performed for peritonitis and suspected bowel perforation, significant intestinal bleeding, or toxic megacolon. The number of emergency operations in our hospital has declined over the last ten years, probably due to earlier referral of patients for elective colectomy before significant and often life-threatening complications arise. Inasmuch as UC usually involves the rectum and then, progressively, the remainder of the colon, total coloproctectomy and ileostomy has become the most commonly performed operation, since this completely eliminates the disease. Lesser operations are almost invariably followed by recurrence or persistence of active inflammation in the remaining colon or rectum. Subtotal colectomy was often done during the first ten years of the present study. At that time many children were referred for operation late in the course of the disease, when they were considered too ill to undergo simultaneous removal of the colon and rectum. The decision to leave the rectum has also been based on the hope of eventually reestablishing intestinal continuity, as championed by Aylett [6]. However, only seven of the twenty-four patients who had subtotal colectomies still have not undergone proctectomy, and no patient has had successful bowel continuity established. The overall incidence of complications was 19 per cent lower in the patients who underwent one-stage total colectomy compared with those who underwent subtotal colectomy, although the latter were performed on sicker patients during the early years of our experience. Moreover, for the past six years, the complication rate with total coloproctectomy has decreased to 22 per cent. Finally, the increased risk of developing carcinoma is obviated if the rectum is also removed. Nevertheless, in children with mild rectal disease, there is evidence to suggest that subtotal colectomy and removal of the rectal mucosa followed by an endorectal pullthrough procedure similar to that used for Hirschsprung’s disease may be feasible [7]. Although the Koch ileal reservoir has not been used on any child in the present study, this might be considered as a second step, preferably not until late adolescence, for a child suffering complications with his ileostomy stoma. A modified Koch pouch is cur-
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Foglia et al
rently under evaluation for use in combination with the endorectal pullthrough operation. Although the overall morbidity for colectomy in the present series was 57.4 per cent, this figure has been reduced to 25 per cent during the past six years. Also, the mean postoperative period of hospitalization before 1966 was 25.4 days. Since 1966, however, this period has decreased to 11.5 days. We believe that these two statistics reflect earlier referrals for operation and better pre- and postoperative management. Our review shows that total colectomy leads to complete cure of the disease as well as to significant reversal of growth retardation in many of these patients. Summary
Forty-seven children with ulcerative colitis (UC) have been treated by colectomy at the UCLA Hospital in the two decades since 1956. An intensive course of medical therapy including a short course of steroid treatment in an attempt to obtain clinical remission is recommended before proceeding with colectomy. Thirty-four children underwent elective colectomy, indicated by intractability of symptoms and a year of growth failure. Postoperative complication rates were higher after emergency colectomy (77 per cent) than after elective operation (50 per cent). The only deaths (3) occurred subsequent to emergency colectomy. Judicious use of preoperative steroids did not increase the incidence of postoperative complications. Thirteen of twenty-four children who underwent initial subtotal colectomy had the remaining rectum removed because of progression of disease within an average period of twenty-eight months. No child underwent successful ileorectal anastomosis after initial subtotal colectomy. Inasmuch as UC usually begins in the rectum, most children with this condition during the past ten years have been treated by total colectomy. Postoperative weight gain and improvement in quality of life were strikingly evident in almost all children who underwent total coloproctectomy. Colectomy is recommended particularly for children who do not experience remission from acute disease after steroid therapy and for those who have chronic disease during the growth period of adolescent years. References 1. Berger M, Gribetz D, Korelitz BI: Growth retardation in children with ulcerative colitis: the effect of medical and surgical therapy. Pediatrics 55: 459, 1975. 2. Ament ME: Inflammatory disease of the colon: ulcerative colitis and Crohn’s colitis. J Pediatr 86: 322, 1975. 3. Levin P, Fonkalsrud FW, Barker WF: Surgical treatment for pe-
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diatric ulcerative colitis. Surgery 60: 201, 1966. 4. Frey CF. Weaver DK: Colectomy in children with ulcerative and granulomatous colitis. Arch Surg 104: 416, 1972. 5. Devroede GJ, Taylor WF, Saver WG, et al: Cancer risk and life expectancy of children with ulcerative colitis. N Engl J A&d 285: 17, 1971. 6. Aylett SO: Three hundred cases of diffuse ulcerative colitis treated by total colectomy and ileo-rectal anastomosis. Br Med J 1: 1001, 1966. 7. Levin P, Fonkalsrud EW: Fecal continence following colectomy and ileoanal anastomosis through extramucosal rectal tube. J Surg Res 8: 234, 1968.
Discussion Morton M. Woolley (Los Angeles, CA): Any surgeon who has dealt with even one child with chronic ulcerative colitis (UC) recognizes the complex nature of patients whose problems are emotional, nutritional, anatomic, physiologic, and potentially malignant. It takes a great deal of time, patience, and expertise to decide whether to operate, when to operate, and how much to operate. At our present state of knowledge, or perhaps more accurately our current state of ignorance, surgical removal of the colon does alleviate the symptoms of chronic UC and does prevent the development of malignancy. The best operation is removal of the entire colon at one stage, if feasible. This obviates the continuation of the disease process and extracolonic manifestations and bleeding and fever which so often plague the patient who has the rectal stump left in place. One is, and always should be, reticent to commit a child to a lifelong ileostomy. If the illness progresses far enough to convince the patient and his family that ileostomy is better than the existing disease, they accept and adjust to it better than if they believe they have been pushed into an operation somewhat against their will. This does not excuse postponement of a desirable operation beyond the time it can be accomplished safely. The increased complication rate in the emergency group of patients in this series speaks for itself in this respect. Since I deal only with the childhood age group, I find that parents frequently have deep feelings of guilt when their infant or child is sick, particularly mothers who think if they could just try a little harder, they could overcome the problem from which their child is suffering. Recently I performed an urgent colectomy on a thirteen year old boy for exsanguinating hemorrhage from chronic UC. He developed postoperative complications including pelvic abscess and intestinal obstruction. His mother was in constant attendance day and night and reported to me each day regarding any minor ineptness on the part of the nurses, as well as any problem the house officers had in starting the many intravenous units day and night. After the patient recovered adequately to be discharged, his mother attempted suicide and required confined psychiatric care. The psychiatrist who had seen her and the patient in the hospital stated that he was not surprised of this event because of the extreme anxiety and guilt that she turned outward on the nurses and house officers in the hospital and inward on herself later. On the brighter side, after recovering from colectomy one of my
The American Journal of Surgery
Childhood Ulcerative Colitis
young patients said to me, “Dr. Woolley, do I have to go back and see that psychiatrist? You know I am cured now.” I should like to close with three questions to the authors. One, your youngest patient was taken care of between the ages of two and seven months. Did he actually have chronic UC, or was this some variant of necrotizing enterocolitis? (Slide) This is a patient who was perfectly well for five years after total colectomy for chronic UC, at which time she developed necrotizing ileitis and eventually died of invasive sepsis. Have you had any late sequelae such as this? (Slide) This is the x-ray appearance of a patient who obviously has chronic UC. He had required a portacaval shunt for portal hypertension secondary to cirrhosis and subsequently had colectomy for chronic UC. Have you had a patient in the childhood age group who has required both colectomy and portacaval shunt as a part of the complex disease process? Eric W. Fonkalsrud (closing): The fact that so many children with UC have been recognized within the past ten years perhaps reflects the advances in the new field of pediatric gastroenterology. Although it is possible that the condition is occurring more frequently, it is more likely that we have just missed diagnosing it in many children in the past. It has only been during the past decade that we have clearly separated patients with UC from those with Crohn’s disease.
Volume 134, July 1977
As Doctor Woolley has indicated, UC may occur in young children and is more likely to do so than is Crohn’s disease. Six patients less than ten years of age in our hospital underwent colectomy for UC; however, no patient less than ten years of age has undergone bowel resection for Crohn’s disease. Although it is unusual for an infant to require colectomy for UC, we have had one child who experienced severe diarrhea and blood loss since the second month of life. Colectomy was performed at the age of seven months and the pathology specimen was diagnostic of UC. Fortunately, the complications Doctor Woolley presented are uncommon, and we have not had experience with a patient developing necrotizing ileitis and would suspect the diagnosis might be Crohn’s disease. Although mild liver disease in patients with UC is not uncommon, the association with portal hypertension is rare. Our studies suggest that children who experience moderate to severe symptoms from UC are unlikely to obtain remission despite therapy, and colectomy should seriously be considered before the child develops both retardation and systemic complications of the disease. Children are able to accept and use an ileostomy remarkably well provided they are given adequate instructions by the physician and his enterostomal nurse. Parents are likely to exhibit many guilt feelings about the disease and the surgeon must have considerable -patience and empathy. The parents, pediatrician, and even the patient will be pleasantly surprised in most instances with regard to the rapid gain in both weight and height which adolescent children are likely to experience after colectomy.
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