Colostomy complications in children. An analysis of 146 cases

This report analyzes the course of 146 pediatric patients with colostomies in reference to problems with colostomy formation, management, and subsequent closure. Colostomy was performed predominantly for Hirschsprung's disease (70 cases) and imperforate anus (46 cases). A transverse colostomy was done in 120 patients (82%), and a sigmoid colostomy in the remaining patients. Loop colostomies were five times more frequent than divided colostomies. Early major complications occurred in 24 patients (16%). Stomal complications occurred in 69 patients and were more frequent after loop colostomies. Colostomy revision was required in 24 cases. Sigmoid colostomy had a significantly lower complication rate (P less than .01). One hundred nine patients underwent colostomy closure. Major complications occurred in 16 cases (15%). There were no deaths related to colostomy closure. The use of a sigmoid colostomy when possible and close attention to technical details, principles of stomal care, and proper parental instruction should minimize morbidity.     

Posterior sagittal resection for rectal aganglionosis: preliminary results of a new approach

BACKGROUND: There is no general agreement about how patients who have short-segment Hirschsprung's disease should be treated. METHODS: Ten patients with Hirschsprung's disease, seven with rectal and three with rectosigmoidal aganglionosis, were operated on through a posterior sagittal incision. In nine patients, a primary rectal resection and coloanal anastomosis was performed. In one patient, a longitudinal posterior myectomy of the rectum was performed as a primary procedure, but the procedure was eventually converted to a rectal resection and coloanal anastomosis through the same incision. RESULTS: One early and one late anastomotic complication occurred. Both were successfully treated with a temporary fecal diversion (left-sided colostomy for 6 to 8 weeks). The functional results as evaluated with anorectal manometry were similar to a group of Hirschsprung's patients treated with transabdominal pull-through resection and coloanal anastomosis. CONCLUSION: This approach might prove to be a useful alternative both to the transabdominal resection and the posterior longitudinal rectal myectomy in Hirschsprung's disease with rectal aganglionosis.     

Surgical correction of Hirschprung's disease: 7 years' experience with the Rehbein technique

Twenty-seven children with Hirschsprung's disease underwent surgery using a modified Rehbein procedure. All the patients were admitted under one unit at the Jos University Teaching Hospital, Nigeria, over a 7-year period from April 1985 to March 1992. There was a male:female ratio of 3:1 with an average age at presentation of 21 months. Skin excoriation (19.0%) following initial colostomy was the commonest postoperative complication, while wound infection (18.5%) featured most often after definitIve surgery. There were no deaths following this procedure. The modified Rehbein technique is a safe and efficacious definitive method of surgical correction of Hirschsprung's disease.     

Repeat pull-through for Hirschsprung's disease

BACKGROUND: A repeat pull-through for Hirschsprung's disease is undertaken when the original procedure has failed. The reasons for failure include a retained aganglionic segment, stricture, fistula, or persisting incontinence. METHODS: All patients who underwent a redo pull-through from 1982 through 1996 were included. Twenty-two patients were underwent surgery; 20 notes were reviewed. The mean age at operation was 6 years (range, 1 to 13). Mean time between operations was 5.1 years (range, 0.5 to 13). RESULTS: Postoperative complications occurred in five patients. These included wound infection, recurrent rectal septum, division of a vas, compartment syndrome, and an anastomotic stricture. Follow-up was for 6.5 years (range, 0.2 to 12). The mean number of bowel movements was 2.2 per day (range, 1 to 6). Fourteen (of 19) patients were continent or soiling less than once per week. Soiling occurred more than once a week in four, but two of these were incontinent after the first operation. One has a colostomy. CONCLUSION: A repeat pull-through is a worthwhile procedure when dealing with an anatomic problem such as retained Hirschsprung's disease, stenosis, or fistula.     

Surgical strategy for abdominal aortic aneurysm with concurrent symptomatic malignancy

In an attempt to improve the guidelines for concurrent management of concomitant abdominal aortic aneurysm (AAA) and symptomatic malignancy, a retrospective study was undertaken. A total of 186 AAA repairs were performed electively, and 25 patients (13.4%) had concurrent symptomatic malignancy from April 1986 to March 1997. Fourteen patients underwent a one-stage operation, including five abdominoperineal rectal resections, four subtotal gastrectomies, three total gastrectomies, and two right hemicolectomies. Eleven others underwent a two-stage operation, including four with total gastrectomy and left hemicolectomy followed by AAA repair, as well as two with right hemicolectomy and one with left hemicolectomy prior to AAA repair. There were no operative deaths or severe postoperative complications. Of the 25 patients, 22 (88.0%) are still alive during follow-up ranging from 8 months to 11 years. Our surgical approach to both lesions is as follows: (1) Using the transperitoneal approach alone, subtotal gastrectomy and abdominoperineal rectal resection can be safely done simultaneously. (2) Although total gastrectomy can also be performed concurrently, the approach used for each lesion is separate. (3) Colorectal resection is generally done separately. However, a one-stage operation can be performed using the thromboexclusion procedure for AAA repair in patients with right-sided colonic cancer or a temporary transverse colostomy for left-sided colorectal cancer.     

Staged pull-through for rectosigmoid Hirschsprung's disease is not safer than primary pull-through

Conventional treatment of Hirschsprung's disease consists of initial colostomy followed by pull-through (staged PT). During the past decade immediate definitive operation (primary PT) has been reported by several investigators with a complication rate ranging from 0% to 20%. In the authors' institution primary PT has been performed since 1992 in patients with rectosigmoid aganglionosis responsive to rectal irrigations. The authors reviewed the records of all patients (n = 124) with Hirschsprung's disease diagnosed and treated between 1989 and 1995. One hundred sixteen patients underwent a definitive operation. This study analysed the complications observed in 87 of these patients with aganglionosis limited to the rectosigmoid colon. Patients were divided into groups according to the type of surgical treatment (staged versus primary), the year of operation (1989 through 1992 versus 1992 through 1995), and the age at primary PT (15 infants age < or = 4 months versus 10 children age > 4 months). There were no deaths. There was no significant difference in complication rates between staged PT and primary PT. Similar complication rates were encountered in infants and children who underwent primary PT. The rate of postoperative complications did not change according to the year of the operation. Major complications were observed in all 3 patients who had a primary Swenson PT compared with 20% who had a primary Duhamel PT. Staged Swenson and Duhamel PT had similar complication rates. The authors conclude that (1) staged PT for rectosigmoid Hirschsprung's disease is not any safer than primary PT; (2) primary PT can be performed safely in young infants; and (3) primary Swenson PT is less satisfactory than primary Duhamel PT.     

The neurobiological bases of aggression: the pharmacotherapeutic implications

The pathophysiology and aetiology of Hirschsprung's disease are still uncertain. The presentation varies with age. Various methods are used to make the diagnosis. Differing views of management are held both for emergency and definitive surgery, e.g. emergency colostomy or not, a covering colostomy or not for the definitive surgery, the type of operation used. The problems of diagnosis and treating diseases which mimic Hirschsprung's disease are highlighted.     

Definitive treatment of Hirschsprung's disease in the newborn with a one-stage procedure

BACKGROUND: The purpose of this study was to review our experience and early follow-up with 15 one-stage pull-through procedures performed at the time of diagnosis in neonates and infants with Hirschsprung's disease. METHODS: Historic chart review of all patients with the new diagnosis of Hirschsprung's disease seen at the C.S. Mott Children's Hospital at the University of Michigan, Ann Arbor, between June 1989 and June 1992 was performed. Progress notes, operative and anesthetic records, pathology reports, and outpatient follow-up notes were used for data collection. RESULTS: Operative technique included a modified endorectal pull-through after determining the presence of ganglion cells by frozen section. No colostomies were performed before or after operation. Three patients had affected family members. All infants were born at term. Operation was performed within 24 hours of diagnosis and as early as 48 hours of age. Twelve patients had standard rectosigmoid disease, two had total colonic disease, and another had long-segment disease. Bowel function returned within 4 days of operation, and feeding was instituted by postoperative day 6 in all patients. All of the patients with rectosigmoid disease are doing well. Patients with long-segment or total colonic disease had more problems but are currently at home and doing well. Morbidity included two postoperative bowel obstructions and three patients with postoperative enterocolitis. CONCLUSIONS: A single definitive operation may be used to treat Hirschsprung's disease diagnosed in the newborn. Long-term follow-up in these patients is required.     

Antegrade colonic enemas

Thirteen children aged 6-14 (mean 8) years in whom an antegrade colonic enema procedure was performed were reviewed retrospectively. All presented with refractory constipation or faecal soiling over a 3-year period. Nine of the children had previously undergone pull-through procedures for Hirschsprung's disease or high anorectal malformations. Two were suffering from spina bifida and two from idiopathic functional constipation. The operation was performed through a right iliac fossa incision. A catheterizable conduit was created. The appendix was brought out to the wound edge and made continent by intussuscepting the appendix base into the caecum. When the appendix was absent or unusable, a caecal tube was formed. Five patients suffered minor morbidity, six required a further operative procedure and two eventually required a sigmoid colostomy. However, the eventual outcome of a continent stoma was attained in 11 of the 13 children, all of whom would have been considered for sigmoid colostomy before introduction of the antegrade colonic enema procedure.     

Oncological implications of RET gene mutations in Hirschsprung's disease

BACKGROUND: Germline mutations of the RET proto-oncogene identical to those found in the tumour predisposition syndrome multiple endocrine neoplasia type 2A (MEN2A), were detected in 2.5-5% of sporadic and familial cases of Hirschsprung's disease. Some patients with Hirschsprung's disease may therefore be exposed to a highly increased risk of tumours. AIMS: To define clinical use of RET gene testing in Hirschsprung's disease and related patient management from an oncological point of view. METHODS: Sixty patients with Hirschsprung's disease were screened for RET mutations. In three, MEN2A type RET mutations were detected. Case reports for these three patients are presented. RESULTS AND CONCLUSIONS: Only 22 families or sporadic patients with Hirschsprung's disease and MEN2A type RET mutations have been reported. Therefore, it is difficult to predict tumour risk for patients with familial or sporadic Hirschsprung's disease, and their relatives, who carry these mutations. For these mutation carriers, periodic screening for tumours as in MEN2A is advised, but prophylactic thyroidectomy is offered hesitantly. RET gene testing in familial or sporadic Hirschsprung's disease is not recommended at present outside a complete clinical research setting. In combined MEN2A/Hirschsprung's disease families RET gene testing, tumour screening, and prophylactic thyroidectomy are indicated as in MEN2A.     

Management of left-sided colonic obstruction by subtotal colectomy and ileocolic anastomosis

OBJECTIVE: To assess complications and functional results of emergency subtotal colectomy with ileocolic anastomosis for acute left-sided colonic obstruction. DESIGN: Retrospective study. SETTING: University hospital, Netherlands. SUBJECTS: 37 patients with acute left-sided colonic obstruction. INTERVENTIONS: Emergency subtotal colectomy with immediate anastomosis (n = 20), Hartmann's procedure (n = 13) or double-loop transverse colostomy (n = 4). MAIN OUTCOME MEASURES: Mortality, morbidity, duration of hospital stay, frequency of defecation, and continence. RESULTS: Morbidity after subtotal colectomy was 10% (n = 2) and mortality 0. There was one anastomotic dehiscence that required a temporary ileostomy. Mean hospital stay was 15 days (range 10-31). All had adequate continence. After 6 weeks mean frequency of defecation was 3/24 hrs (range 2-6). 9 patients died within 2 years of metastatic disease. CONCLUSIONS: Subtotal colectomy with ileocolic anastomosis is a suitable procedure for treating left-sided colonic obstruction provided that pelvic floor function is adequate and a skilled surgeon is available.     

Results of the double stapling procedure in colorectal surgery

In this report we review our results with the double stapling technique (DST) in 162 patients with colorectal diseases in an attempt to identify some of the potential pitfalls of this new technique. Among these 162 patients, there were 125 patients with colorectal cancer, 25 with chronic ulcerative colitis (UC), 9 with familial adenomatous polyposis (FAP), 2 with adult Hirschsprung's disease, and 1 with sigmoid colon fistula. A total of 46 anastomoses (28 for rectal cancer, 13 for UC, 3 for FAP, and 2 for adult Hirschsprung's disease) were performed at or near the dentate line. Of these, 10 had protective diverting colostomy or ileostomy. The results showed that 6 patients with rectal cancer had anastomotic leakage (3.7%); however, 4 of the 6 patients had also received preoperative irradiation. All the leaks healed after the patients had undergone diverting colostomy, but 7 patients with rectal cancer suffered from neurogenic bladder postoperatively (4.3%). Wound infection occurred in 4 patients (2.5%), anastomotic bleeding in 3 (1.9%), and anal pain in 1 (0.6%), respectively. One patient with rectal cancer and multiple liver metastases died of disseminated intravascular coagulation (DIC). These results thus suggest that the double stapling technique provides a safe anastomosis at or near the dentate line not only for rectal cancer but also for UC, FAP, and adult Hirschsprung's disease.     

Primary anastomosis in the treatment of acute disease of the unprepared left colon

Between June 1, 1990 and December 31, 1996, 58 consecutive patients with unprepared colons were urgently explored for nontraumatic disease with intent to proceed with primary left-sided colonic anastomosis. Unprotected anastomoses were not attempted in 15 patients. The causes of exclusion included preoperative and intraoperative shock in three patients, and three patients were on long-term high-dose steroids, four had gross fecal contamination of the peritoneal cavity, four had large pelvic abscesses, and one had ischemic colitis. All 43 patients undergoing anastomosis without protective colostomy had stapled anastomoses. Indications included complicated diverticular disease in 32 cases. There were nine cases of obstruction from colorectal carcinoma and one obstruction due to sigmoid volvulus. There was one case of perforation from pseudomembranous enterocolitis. The most common complications were: atelectasis in nine cases, wound infection in two cases, and prolonged ileus in two cases. Pelvic abscess occurred in one case. There was one wound dehiscence. There was one anastomotic dehiscence, and there was no mortality. Operative time averaged 85 minutes and hospital length of stay 9.7 days. Primary anastomosis of the unprepared left colon is safe in most urgent and emergent situations, thus avoiding the significant morbidity and cost of colostomy closure.     

Long-term clinical outcome of surgery for solitary rectal ulcer syndrome

BACKGROUND: When severe symptoms of solitary rectal ulcer syndrome persist despite medical management, surgery may be necessary. METHODS: A retrospective review was carried out of 81 patients undergoing surgery for solitary rectal ulcer syndrome in the 10-year period from 1984 to 1993 to determine the long-term outcome at a minimum follow-up of 12 months. Of the 81 patients, 15 were excluded from further analysis (11 were followed up for less than 12 months, two died and two were lost to follow-up). Sixty-six patients were studied (median age 38 (range 15-77) years; 53 female). Of these, 49 underwent rectopexy, nine Delorme's operation, two anterior resection and four creation of a stoma as the initial operation. RESULTS: At a median follow-up of 90 (range 12-177) months, the rectopexy had failed in 22 of 49 patients; 19 of these patients underwent further surgery, including rectal resection with coloanal anastomosis (four with three failures), colostomy (11) and other procedures (four). Ultimately, 14 required a stoma. Constipation was the indication for a stoma in nine of the 11 patients who had colostomy as the first procedure after failure of rectopexy. Nine patients had Delorme's operation as the first procedure. At median follow-up of 38 (range 19-107) months, there were four failures. Two of these ultimately required a stoma. Of the seven patients who underwent anterior resection as the initial or subsequent procedure, a stoma was finally necessary in four. Anterior resection used as a salvage procedure was not successful. The overall stoma rate was 30 per cent (20 patients). Of 11 symptoms assessed before operation only incontinence and incomplete evacuation were related to a poor outcome following surgery. CONCLUSION: Antiprolapse operations result in a satisfactory long-term outcome in about 55-60 per cent of patients having surgery for solitary rectal ulcer syndrome. Results of anterior resection are disappointing.     

Preliminary clinical results of surgery for type A acute aortic dissections using gelatin-resorcin-formaldehyde glue

We used gelatin-resorcin-form-aldehyde (GRF) glue to fuse the false lumen of type A acute aortic dissection in four patients. All were operated on within 3-24 hours after onset, and gluing of the two cylinders of the dissecting aorta could be done safely in a short time. Initial intimal tears were located in the transverse aorta in three patients and in the proximal descending aorta in one. Simple transection and end-to-end anastomosis of the ascending aorta was done for the first two cases. But in the last two patients, we resected the intimal tear in the transverse aorta and applied GRF glue to the stump of the aortic arch and to that of the aortic root, followed by graft replacement of the ascending aorta. There were no hospital deaths. But we had to reoperate on one patient five months after the first operation due to potentially residual dissection in the aortic root. GRF glue is a very useful adhesive for acute aortic dissection operations, but further refinement of the operative technique using it is necessary.     

Arginine transferase activity in homogenates from guinea-pig hair follicles

A retrospective 10-year experience with the traditional three-stage plan (diverting colostomy, resection, colostomy closure) for perforated diverticulitis of the colon in four urban hospitals was reviewed to accurately assess the mortality rate. Only patients who were admitted in a non-elective manner with signs of an acute abdomen or who were already hospitalized with another illness and developed an acute abdomen were considered. Fecal or generalized purulent peritonitis, or pelvic peritonitis with abscess were observed at laparotomy in all instances. Two hundred and eight patients representing 211 episodes met the above stated criteria for inclusion in the study. A transverse colostomy was performed in 203 instances associated with 16 deaths, and 8 sigmoid colostomies were associated with two deaths. The overall mortality after the first stage was 8.5%. A loop colostomy was constructed most frequently and a completely divided colostomy performed in only 31 of 211 (15%) instances. Of 147 instances in which the diseased sigmoid colon was resected, 44 (30%) had the colostomy ablated at the same operation, resulting in only one death (0.7% mortality). Colostomy closure as a separate procedure in 103 instances resulted in 4 deaths (3.9% mortality). The highest mortality rate occurred in patients in the in the eighth decade. Staged procedures for perforated colonic diverticula can be carried out with a mortality rate of 11%.     

Abdominoperineal resection and perineal colostomy for low rectal cancer. The Lazaro da Silva technique

PURPOSE: We sought to evaluate a new technique for creation of a continent perineal colostomy following abdominoperineal resection (APR) of the rectum for low rectal cancer. METHODS: Nine selected patients with low rectal cancer (two males; median age, 55.6 years; classified as Dukes A, 6 patients and as Dukes B, 3 patients) underwent APR. Following this, the original Lazaro da Silva technique was used as follows: 1) for performance of three circular myotomies in the distal sigmoid with a distance between each couple of no more than 8 cm; 2) repair of the myotomies, thus creating three circular colonic valves, the most distal of which remained extraperitoneally; 3) for construction of a perineal colostomy lying flush with the perineal skin; 4) after the patient starts consuming a regular diet, enemas through the perineal stoma are done, usually twice per week, to achieve defecation. Functional outcome was assessed by evaluation of bowel movements and neoanal continence. RESULTS: There were no deaths. From January 1994 until October 1995, no tumor recurrence has occurred, and fecal continence has been good. Four of the patients were able to defecate without enemas (2-4 times per week), and in five patients the self-administration of enemas (2-4 times a week) were necessary to accomplish defecation. CONCLUSION: Initial results with the Lazaro da Silva technique have been encouraging.     

Immunohistochemical evaluations of ultrashort-segment Hirschsprung's disease. Report of three cases

PURPOSE: Unlike classic Hirschsprung's disease, short-segment and ultrashort-segment varieties are usually found to be latent and milder. Ultrashort-segment Hirschsprung's disease may present as intractable chronic constipation in children over one year of age, adolescents, and adults. Anorectal myectomy has been shown in many instances to provide effective long-term treatment for certain patients with ultrashort-segment Hirschsprung's disease. Histologically, the affected segment in Hirschsprung's disease has been shown to have increased cholinergic nerves, lack of nitric oxide synthase-containing neuronal elements, and show moderate to severe loss of myenteric neurons. METHODS: Here, we report three cases that showed clinical and manometric evidence of ultrashort-segment Hirschsprung's disease. Two of the three patients responded well to myectomy. RESULTS: Detailed histologic and immunohistochemical evaluation of the internal anal sphincter and a comparison with three normal controls revealed absence of nitric oxide synthase-containing neurons in both cases that responded well to surgery and continued presence of these neurons in the patient who did not respond. A review of the current literature on various treatment modalities is included. CONCLUSIONS: Anorectal myectomy provides long-term relief of this chronic problem in a subgroup of patients with ultrashort-segment Hirschsprung's disease who lack nitrinergic neurons at the internal anal sphincter.     

Hirschsprung-associated congenital anomalies

Between 1976-1993, 302 patients were diagnosed to have Hirschsprung's disease (HD) in Hacettepe University Children's Hospital. All patients have been searched for the occurrence of associated anomalies and other parameters including 1) sex, 2) gestational age, 3) birth weight, 4) length of aganglionic segment, 5) familial occurrence, 6) consanguinity. Forty-nine of 302 patients were proved to have 83 associated congenital anomalies together with HD. Distribution of anomalies was as follows; central nervous system and special senses 28.92%, genitourinary system 26.51%, skeletal 24.09%, gastrointestinal 12.05%, cardiovascular 8.43%. In eleven patients more than one anomaly involving different systems were detected; only one of these patients was female and six of them have shown short segment disease. Down's syndrome was present in 5 patients. Female: male ratio was 1:4.2 for the whole series and 1:6 for the patients with Hirschsprung associated congenital anomalies (HACA). In the HACA group the percentage increased in favor of long-segment and extensive aganglionosis without statistical significance (p>0.05). The majority of patients were term babies and their birth weights were between 2500g < 3500g. Consanguinity was present in 36 families. No affected parents were detected but two sisters and four brothers were proved to have HD. Three pairs of twins, one of which was monozygotic, and all showing discordance were detected. There was no difference between HACA group and HD without HACA in terms of sex, birth weight, length of aganglionic segment and consanguinity. Although HD seemed to be genetically heterogeneous HACA did not have a genetically different origin from that of HD.     

Extra-anal mucosectomy: laparascopic-assisted endorectal pull-through using a prolapsing technique

BACKGROUND/PURPOSE: The mucosectomy of the aganglionic rectal mucosa has been performed transabdominally in the Soave procedure. Recently, mucosectomy with transanal approach was reported both in the laparoscopic and the open surgical procedure. However, the operative view and working field are restricted because the dissection has to be done in the anal canal. The authors report an innovative approach, an extra-anal mucosectomy, to perform mucosectomy in the Soave procedure for Hirschspung's disease in combination with the laparoscopic-assisted prolapsing technique. METHODS: Four patients underwent laparoscopic surgery for Hirschsprung's disease with extra-anal mucosectomy between 1995 and 1997. One 10-mm and 5-mm ports were used throughout the operation. The rectum was pulled out through the anal canal to create a rectal prolapse and was divided outside the anal canal. The mucosectomy was performed in the everted rectal mucosa outside the anal canal to the level of dentate line. The colon was sutured to the anal mucosa 2 mm above the dentate line. Mean operative time was 3 hours. RESULTS: The present technique made the whole mucosectomy possible under direct observation. CONCLUSION: The extra-anal mucosectomy in conjunction with a laparoscopic-assisted prolapsing technique seems to be a safe and reliable modality in the surgical treatment of Hirschsprung's disease.