A simplified approach to diagnostic rectal biopsy in infants and children

Ninety-four patients underwent rectal biopsy with a suction capsule during a two year period. This painless procedure was performed in seventy-one patients for diagnosis of aganglionosis (Hirschsprung's disease). In nine patients, no ganglion cells were encountered, and subsequent surgery confirmed the diagnosis of Hirschsprung's disease in each of these patients. In twenty-three patients, biopsies were taken to search for neuronal storage disease. Six specimens contained abnormal material in the cytoplasm of the ganglion cells.     

Megacolon and megarectum in adults. Abdominal proctectomy with colo-anal anastomosis

Severe constipation in adults with megarectum may be a manifestation of Hirschsprung's disease or idiopathic megarectum. The differential diagnosis is discussed in the light of two case histories. Total abdominal proctectomy with coloanal anastomosis eliminated the aganglionic rectal segment in Hirschsprung's disease and the adynamic distal colorectum of idiopathic megarectum respectively. The surgical and functional results of a hand-sewn transanal end-to-end-anastomosis were excellent in both patients.     

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.     

Physiological and functional outcome following ultra-low anterior resection with colon pouch-anal anastomosis

BACKGROUND: Low rectal cancer is usually managed by ultra-low anterior resection (ULAR) with total mesorectal excision and straight coloanal anastomosis. However, following this procedure patients often suffer from frequency, urgency of bowel action and, occasionally, faecal incontinence. To overcome such problems, a colon pouch may be fashioned and a subsequent colon pouch-anal anastomosis performed. The physiological and functional outcome following the use of a colon pouch are appraised. METHODS: All relevant papers identified from a Medline search and papers from cross-referencing were reviewed. RESULTS AND CONCLUSION: Creation of a colon pouch following ULAR results in reduced bowel frequency, and a lower incidence of urgency and faecal incontinence. Although there is a slightly increased incidence of evacuatory disorder and need for enemas or suppositories, this appears to be a minor problem which may possibly be overcome by using a smaller colon pouch. Compared with straight coloanal anastomosis following ULAR, the creation of a colon pouch produced a superior functional outcome.     

Zonal adult Hirschsprung's disease

BACKGROUND: Hirschsprung's disease is a congenital disorder which is rare in adulthood. In typical cases the aganglionosis involves mainly the rectum or rectosigmoid colon and the lesion starts from the anal valve. Zonal segmental aganglionosis is a very rare type even in children. PATIENT: A 54 year old women with zonal segmental aganglionosis had an aganglionic segment 18 cm in length located in the rectosigmoid colon with an 8 cm long normal appearing rectum and dilated proximal colon. Resection of the stenotic segment with end to end anastomosis was performed. CONCLUSION: The functional result was excellent five years after the operation.     

Surgery for constipation: a review

PURPOSE: Constipation is related to intestinal motility disorders (colonic inertia (CI)), pelvic floor disturbances (pelvic outlet obstruction), or a combination of both problems. This review summarizes the physiologic and pathophysiologic changes in patients with intractable constipation and gives an overview of surgical treatment options. RESULTS: Although subtotal colectomy with ileorectal anastomosis is the best surgery for CI, there are still approximately 10 percent of patients who will complain of pain and constipation. A completion proctectomy and an ileoanal pouch procedure may be a viable option in a highly select group of patients. In patients with megabowel, reported results are mixed. Subtotal colectomy, partial colectomy for megacolon, and the Duhamel procedure for megarectum have all been reported with variable results. In patients with an isolated distended sigmoid colon, sigmoid colectomy has achieved good results. Anorectal myectomy has not been proven to be successful in the long term. However, in patients with adult short segment Hirschsprung's disease, myectomy can be successful. Patients with pelvic outlet obstruction can be successfully treated with biofeedback. In a small group of patients with a rectocele or a third degree sigmoidocele, surgical intervention yields a high success rate. Division or resection of the puborectalis muscle is not recommended. In patients with a mixed pattern of CI and pelvic outlet obstruction, surgical intervention alone is often not successful. These patients achieve better results by conservative treatment of pelvic outlet obstruction, followed by a colectomy. CONCLUSION: Surgical intervention for patients with intractable constipation is rarely necessary. However, thorough preoperative physiologic testing is mandatory for a successful outcome.     

Physiopathology and therapy of vertigo or dizziness due to cerebrovascular disorders

PURPOSE: The aim of this study is to demonstrate the feasibility and usefulness of mechanical suturing in children for low rectal anastomosis. METHODS: The study group includes 31 children operated on from January 1993 to July 1996 by the same senior surgeon, performing the modified Duhamel procedure for Hirschsprung's disease in 17 children, intestinal neuronal dysplasia in seven, and the Knight-Griffen procedure in seven pediatric patients with chronic ulcerative colitis. RESULTS: In all the cases the technique of "viscero-synthesis" was performed using the mechanical stapler. A circular stapler has been used for the end-to-end and the end-to-side anastomosis between the anal canal or the back wall of the rectum with the pulled viscus, while a linear endoscopic stapler (GIA) has been used for the consolidation of the rectocolic wall in the modified Duhamel technique. CONCLUSIONS: The results obtained demonstrate that the mechanical staplers in children are safe and effective in low rectal anastomosis, sparing operative time and reducing the risk of anastomotic dehiscence; however, the size of circular instruments limits its use in neonates and small infants.     

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.     

Anterior luxtasphincteric stapled rectumresection

BACKGROUND/AIMS: Sphincter saving deep anterior rectumresection improved the live quality of the rectal cancer patients. With the technical progress the resection level could be moved significant distally. The initial experiences are referred with the use of the stapler with flexible shaft in six patients operated due to very deep rectal cancer. METHODOLOGY: After the usual rectum mobilization the Proximate access AX55 mm (Ethicon) is positioned onto the stretched rectum. The flexible shaft has to be curved to the front and the jaw has to be angled parallel to the handle. The partially closed jaw of the stapler is then pushed towards the anus and then fired. The rectum is the resected. The operation is completed with EEA stapled anastomosis. RESULTS: Excellent results in all six patients could be noticed 3-18 months after the procedure. No cancer recurrence occurred. Functional sphincter investigation was performed in two patients with result of physiologic sphincter function. Stolen frequency has been stabilised between 2-8 a day. CONCLUSION: Improved transabdominal stapled method of rectum resection guarantees comfortable and oncologically safe resection of the distal rectum with maintenance of the sphincter function.     

Late clinical outcome in a randomized prospective comparison of colonic J pouch and straight coloanal anastomosis

BACKGROUND: Functional outcome after rectal excision with coloanal anastomosis is improved by construction of a colonic J pouch. Present prospective randomized studies lack follow-up beyond 1 year. The aim of this study was to assess the clinical outcome at both short- and long-term follow-up. METHODS: Forty patients with low rectal cancer were randomized prospectively to either J colonic pouch-anal anastomosis or a straight coloanal anastomosis. Clinical assessments were performed 3, 12 and 24 months after colostomy closure using a standard questionnaire and physical examination. RESULTS: There was no significant difference in the complication rate between the two groups. There was a significant (P < 0.01) improvement in frequency of defaecation at 3, 12 and 24 months for patients with a reservoir. Similarly, fragmentation (clustering of stools) was significantly less at 3 and 12 months (P < 0.01) in the reservoir group, and incontinence occurred less frequently in the first year (P = 0.09). By 24 months no patient in either group suffered from major or minor incontinence. CONCLUSION: The functional improvement gained from a colonic reservoir in coloanal anastomosis continues to benefit the patient for at least 2 years.     

Surgical treatment of adenocarcinoma of the rectum

OBJECTIVE: The authors' aim was to determine survival and recurrence rates in patients undergoing resection of rectal cancer achieved by abdominoperineal resection (APR), coloanal anastomosis (CAA), and anterior resection (AR) without adjuvant therapy. SUMMARY BACKGROUND DATA: The surgery of rectal cancer is controversial; so, too, is its adjuvant management. Questions such as preoperative versus postoperative radiation versus no radiation are key. An approach in which the entire mesorectum is excised has been proposed as yielding low recurrence rates. METHODS: Of 1423 patients with resected rectal cancers, 491 patients were excluded, leaving 932 with a primary adenocarcinoma of the rectum treated at Mayo. Eighty-six percent were resected for cure. Surgery plus adjuvant treatment was performed in 418, surgery alone in 514. These 514 patients are the subject of this review. Among the 514 patients who underwent surgery alone, APR was performed in 169, CAA in 19, AR in 272, and other procedures in 54. Eighty-seven percent of patients were operated on with curative intent. The mean follow-up was 5.6 years; follow-up was complete in 92%. APR and CAA were performed excising the envelope of rectal mesentery posteriorly and the supporting tissues laterally from the sacral promontory to the pelvic floor. AR was performed using an appropriately wide rectal mesentery resection technique if the tumor was high; if the tumor was in the middle or low rectum, all mesentery was resected. The mean distal margin achieved by AR was 3 +/- 2 cm. RESULTS: Mortality was 2% (12 of 514). Anastomotic leaks after AR occurred in 5% (16 of 291) and overall transient urinary retention in 15%. Eleven percent of patients had a wound infection (abdominal and perineal wound, 30-day, purulence, or cellulitis). The local recurrence and 5-year disease-free survival rates were 7% and 78%, respectively, after AR; 6% and 83%, respectively, after CAA; and 4% and 80%, respectively, after APR. Patients with stage III disease, had a 60% disease-free survival rate. CONCLUSIONS: Complete resection of the envelope of supporting tissues about the rectum during APR, CAA, and AR when tumors were low in the rectum is associated with low mortality, low morbidity, low local recurrence, and good 5-year survival rates. Appropriate "tumor-specific" mesorectal excision during AR when the tumor is high in the rectum is likewise consistent with a low rate of local recurrence and good long-term survival. However, the overall failure rate of 40% in stage III disease (which is independent of surgical technique) means that surgical approaches alone are not sufficient to achieve better long-term survival rates.     

Current status in the diagnosis and therapy of meconium ileus

Meconium ileus is in most cases difficult to diagnose preoperatively; however, aganglionosis should be excluded in every case (rectal double-suction biopsy, manometry). In cases of uncomplicated meconium ileus (no perforation, no further intestinal obstructions) lysis of the meconium by a Gastrografin enema is tried first. In case of no relief, an intraoperative puncture of the bowel and Gastrografin instillation is performed. If there is no relief a small bowel resection is performed and a Bishop-Koop anastomosis or a chimney anastomosis established. A distinction must be made between meconium ileus, meconium disease, meconium plug syndrome, and curd obstruction. Results show a survival rate of 80%.     

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.     

Surgical treatment of lower rectal cancer with sphincter preservation using handsewn coloanal anastomosis

The present study was designed to evaluate the technical feasibility and oncologic results of performing handsewn coloanal anastomosis (CAA). A total of 46 patients treated for lower rectal cancer using CAA were retrospectively studied, and the oncologic results were compared with those of 105 patients treated with abdominoperineal resection (APR). CAA was performed in patients who had both good mobility of the tumor and a distal clearance margin of more than 1.0 cm. No significant difference was noted in the mortality rates following the two operations (CAA 2.2% vs APR 1.9%). Pelvic recurrence was detected in two patients (4.5%) after CAA and in six patients (7.2%) after APR. The 5-year survival rate after CAA was 79.2% and that after APR was 72.6%. No significant difference was noted in the incidence of pelvic recurrence or the survival rates between the two operations. These results show that CAA could be an excellent reconstructive option in the treatment of lower rectal carcinoma for selected patients.     

Coordinated activity of the new "rectum" and anal sphincter after sphincter-saving resection of the rectum for colitis or carcinoma

PURPOSE: The aim of this study was to determine whether coordinated activity exists across a stapled enteroanal anastomosis. METHODS: Twenty-nine patients were studied for a median of one year after complete excision of the rectum and stapled enteroanal anastomosis; 12 patients underwent low anterior resection with coloanal anastomosis for carcinoma, and 17 patients underwent restorative proctocolectomy with ileoanal anastomosis. RESULTS: Maximum anal resting pressures were slightly lower after coloanal anastomosis than after ileoanal anastomosis [median range, 56 (11-60) cm H2O, cf 69 (40-107) cm H2O, P = NS]. During distention of the neorectum, anal sphincter pressures at 2.5, 1.5, and 0.5 cm from the anal verge were significantly lower after coloanal anastomosis compared with after ileoanal anastomosis (P < 0.01 at each station). The volume of neorectal distention required to produce maximal inhibition of the anal sphincter was significantly less after coloanal anastomosis at 50 (range, 20-60) ml of air than after ileoanal anastomosis at 240 (range, 100-420) ml of air (P < 0.01). Minor fecal leakage and urgency of bowel action were significantly more common after coloanal anastomosis (P < 0.01). CONCLUSION: Alterations in the dynamic response of the anal sphincter to distention of the neorectum may explain why the clinical results were better after ileal pouch-anal anastomosis than after coloanal anastomosis.     

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.     

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.     

Preliminary experience with intrasphincteric botulinum toxin for persistent constipation after pull-through for Hirschsprung's disease

Although most children who have Hirschsprung's disease have an excellent result after pull-through surgery, some experience persistent constipation caused by "internal sphincter achalasia." Anal myectomy has been advocated for this problem, but it results in permanent injury to the sphincter and is not universally effective. Botulinum toxin has been safely used to selectively and reversibly weaken a variety of voluntary muscles and sphincters in both adults and children. Injection of botulinum toxin into the internal anal sphincter (IAS) should theoretically produce the same functional result as anal myectomy without permanent sphincter injury. Four children aged 4 to 8 years presented with persistent constipation after a pull-through procedure for Hirschsprung's disease. Two had associated encopresis, both of whom had previous myectomies. The authors performed four-quadrant intrasphincteric botulinum toxin injection (total dose, 15 U). Resting IAS pressure decreased in all children 4 to 8 weeks after injection. Patients have been followed up for 7 to 9 months. One child (with Down's syndrome) remained symptomatically unchanged. The other three families reported significant improvement in bowel function in their children. In two of these, there was a return of symptoms 6 months after injection; one child underwent reinjection with good results. Postinjection incontinence occurred in three children, but resolved after several weeks in the one who did not have encopresis before botulinum toxin injection. These preliminary results suggest that botulinum toxin may represent a less invasive alternative to anal myectomy for children who have severe constipation after surgery for Hirschsprung's disease. If myectomy is contemplated, botulinum toxin may also be useful as a means of predicting which children may benefit.     

Role of preoperative sonourethrography in bulbar urethral reconstruction

PURPOSE: The accuracy of sonourethrography for the evaluation of bulbar urethral strictures has been well documented. Thus, we sought to define the role of preoperative sonourethrography in establishing objective criteria for procedure selection during bulbar urethral reconstruction. MATERIALS AND METHODS: Sonourethrography was performed preoperatively, just before incision, in 67 men selected for bulbar urethroplasty. All patients had strictures 25 mm. or less in length on preoperative radiographic retrograde urethrography, thus potentially amenable to resection and end-to-end anastomosis. Ultrasonic measurements were prospectively recorded, compared with those on preoperative retrograde urethrography, and used to guide the selection of urethroplasty technique. RESULTS: Overall, a significant trend for retrograde urethrography to underestimate stricture length was demonstrated (r = 0.678, p < 0.0001). Indeed, sonographic measurements were frequently twice those of retrograde urethrography, occasionally more. All 26 patients with short strictures on retrograde urethrography (10 mm. or less) were successfully treated by resection and end-to-end anastomosis, and sonographic assessment did not alter management. However, ultrasonic measurement changed the reconstructive procedure selected in 15 of 41 patients (37%, 3 penile flaps, 12 graft procedures) with bulbar strictures of intermediate length on retrograde urethrography (11 to 25 mm.). CONCLUSIONS: Sonourethrography has a major influence on selection of therapy in patients with bulbar strictures of intermediate length. By prospectively identifying strictures too long for resection and end-to-end anastomosis, sonourethrography enables quantitative criteria for selection of patients who may be more appropriately treated by flap or graft procedures. We advocate excisional therapy for strictures appearing sonographically to be 25 mm. or less, and substitution urethroplasty for longer strictures.     

Endoscopic Nd:YAG laser treatment of inoperable lower gastrointestinal cancer

Many patients with colorectal cancer are not amenable to curative resection at the time of presentation. Nevertheless, palliative resection still remains as the treatment of choice in the majority of patients. A small group of patients that are poor candidates for surgical resection may benefit from some non-surgical palliative procedures to relieve their symptoms. Electrocoagulation, cryosurgery and radiotherapy are some of the non-surgical procedure used and they are associated with high morbidity and mortality. The use of Neodymium: Yttrium-Aluminium-Garnet (Nd:YAG) laser photoablation to palliate patients with advanced colorectal carcinoma is well documented. It is associated with relatively low morbidity and perioperative mortality. It requires no anaesthesia and is the only non-surgical procedure that can be safely carried out above the peritoneal reflection. Nd:YAG laser had been used in some centres as a preresectional procedure in patient presenting with high grade obstruction. It allows proper bowel preparation followed by primary excision and anastomosis. As a palliative procedure, most patients showed rapid improvement in obstructive symptoms, bleeding and rectal discharge. The size of the lesion and circumferential extent of the tumour base correlate well with the response rate. Most patients remained asymptomatic before they succumb to the advanced disease. In our series, good palliation of obstructive symptoms was achieved in all obstructive cases with one laser treatment, bleeding tumours required an average of two sessions for complete haemostasis. In conclusion, Nd:YAG laser therapy is a safe and efficacious means for palliation of obstructive symptoms and bleeding in advanced rectal carcinoma.