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.     

Low anterior resection with a long posterior anorectal myectomy and sphincterectomy for Hirschsprung's disease

Nine patients with the severe form of Hirschhsprung's Disease (HD) underwent low anterior resection with posterior anal myectomy and sphincterectomy. Good results were achieved in six patients. Three patients had bouts of enterocolitis following the surgical treatment which was attributed to residual spasm of the aganglionic rectum and the internal anal sphincter. Myectomy had to be redone in two patients. One patient developed constipation which responded to toilet training. One patient died at home of an unknown cause, and one patient was lost to follow-up. The different methods of treatment of HD are discussed with emphasis on the role of the internal anal sphincter. The relatively high complication rate in this small group of patients does not justify its use in the severe form of HD.     

Colocalization of NADPH-diaphorase staining and VIP immunoreactivity in neurons in opossum internal anal sphincter

Nitric oxide and vasoactive intestinal polypeptide (VIP) are important inhibitory neurotransmitters mediating relaxation of the internal anal sphincter. The location and coexistence of these two neurotransmitters in the internal anal sphincter has not been examined. We performed a double-labeling study to examine the coexistence of nitric oxide synthase and VIP in the opossum internal anal sphincter using the NADPH-diaphorase technique which is a histochemical stain for nitric oxide synthase. In perfusion-fixed, frozen-sectioned tissue, VIP-immunoreactive neurons were labeled using immunofluorescence histochemistry. After photographing the VIP-immunoreactive neurons, nitric oxide synthase was labeled using the NADPH-diaphorase technique. Ganglia containing neuronal cell bodies were present in the myenteric plexus for the entire extent of the internal anal sphincter. VIP-immunoreactive and NADPH-diaphorase-positive neurons were present in ganglia in the myenteric as well as the submucosal plexuses. Most of the VIP-immunoreactive neurons were also NADPH-diaphorase positive. VIP and nitric oxide synthase are present and frequently coexist in neurons in the internal anal sphincter of the opossum. These neurons may be an important source of inhibitory innervation mediating the rectoanal reflex-induced relaxation of the sphincter. The demonstration of the coexistence of these two neurotransmitters will be of fundamental importance in unraveling their relationship and interaction in the internal anal sphincter as well as other systems.     

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.     

Total colectomy for Crohn''s disease

BACKGROUND: Hypertrophy of the internal anal sphincter may be apparent in some children, but its significance has not yet been determined. OBJECTIVE: To assess anal endosonographic findings in children with chronic constipation. Materials and methods. We performed anal endosonography in 46 children with chronic constipation and compared the results with values considered normal. RESULTS: We did not find a significant relationship between age and thickness of the internal or external anal sphincters. The clinical response to medical management did not differ between patients with or without sphincter hypertrophy. CONCLUSIONS: Although we did not find a significant correlation between sphincter hypertrophy and constipation or age, further studies may clarify its place amongst other techniques which are used in the investigation of anorectal pathology.     

Clinical effects of childbirth with median episiotomy and anal sphincter injury on fecal incontinence of primiparous women

Obstetric damage of the anorectal continence organ can lead to impaired anal continence. To assess the effect of birth, either with or without direct injury of the anal sphincter, 123 primiparae were studied. 41 patients with a midline episiotomy and 82 patients with an additional injury of the anal sphincter were assessed at a median of 21 weeks postpartum and compared with 18 healthy volunteers. Anorectal manometry as well as a standardized questionnaire were employed. Patients with an additional injury of the anal sphincter reported persistent flatus incontinence significantly more often (p = 0.0069) than patients with a midline episiotomy only. Incontinence of solid or liquid stool occurred only transiently. Compared to nulliparae in all primiparae a significant shortening of anal canal and a decreased squeeze pressure were observed. In addition, a significantly reduced resting pressure was seen in patients with an anal sphincter injury. The rectoanal inhibitory reflex was absent significantly more often following anal sphincter tear (p = 0.0023). Vaginal delivery, both with and without anal sphincter injury, leads to early detectable changes in anorectal sphincter function.     

Glutaraldehyde cross-linked collagen in the treatment of faecal incontinence

BACKGROUND: The treatment of faecal incontinence secondary to internal anal sphincter dysfunction is unsatisfactory. The aim of the study was to evaluate the efficacy of anal glutaraldehyde cross-linked (GAX) collagen injections in patients with a surgically incorrectable disorder. METHODS: Seventeen patients were studied: nine had idiopathic faecal incontinence, three had incontinence following haemorrhoidectomy, two following internal sphincterotomy, two following an internal sphincter defect from obstetric injury and one following treatment for fistula in ano. All patients were refractory to conservative treatment and were unsuitable for surgical repair. All had anorectal physiology and endoanal ultrasonography before and after GAX collagen injections. RESULTS: All patients tolerated the injection without side-effects. All patients had an intact external anal sphincter. Following injection, 11 patients showed marked symptomatic improvement. One patient reported symptomatic improvement but remained in clinical grade 3, and two reported minimal improvement. There was no improvement in three patients, but one of these had a repeat injection and showed significant improvement subsequently. CONCLUSION: Injection of GAX collagen in the anal canal is a simple and well tolerated method of treating faecal incontinence due to internal sphincter dysfunction. Early results suggest it provides an easy and reliable alternative to the currently available methods that are often unsuccessful and at best unpredictable.     

Diagnosing anal sphincter injury with transanal ultrasound and manometry

PURPOSE: This study was undertaken to evaluate how well anorectal manometry and transanal ultrasonography diagnose anal sphincter injury. METHODS: Anorectal manometry and transanal ultrasonography were performed in 20 asymptomatic nulliparous women and 20 asymptomatic parous women, and the results were compared with those obtained in 31 incontinent women who subsequently underwent sphincteroplasty and, thus, had operatively verified anal sphincter injury. By using computerized manometry analysis, mean maximum resting and squeeze pressures, sphincter length, and vector symmetry were determined in all women. All transanal ultrasounds were interpreted blinded as to the patient's history, physical examination, and manometry results. RESULTS: Manometric resting and squeeze pressures were significantly higher in the asymptomatic nulliparous women than in the asymptomatic parous women, and both groups had significantly higher pressures than the incontinent women (P < 0.001). Anal sphincter length and vector symmetry index were significantly decreased in incontinent women compared with asymptomatic women (P < 0.01). Decreased resting and squeeze pressures suggestive of possible sphincter injury were found in 90 percent of incontinent women with known anal sphincter injury. Decreased anal sphincter length and vector symmetry were found in only 42 percent of women with known anal sphincter injury. Transanal ultrasound was able to identify 100 percent of the known sphincter injuries but also falsely diagnosed injury in 10 percent of the asymptomatic nulliparous women with intact anal sphincters. False identification of sphincter injury increased when transanal ultrasound scanning was performed proximal to the distal 1.5 cm of the anal canal. CONCLUSION: Although nonspecific, decreased resting and squeeze pressures were found in 90 percent of patients with anal sphincter injury. Decreased anal sphincter length or vector symmetry index were present in only 42 percent of patients with known sphincter injury. When limited to the distal 1.5 cm of the anal canal, transanal ultrasound identified all known sphincter injuries but falsely identified injury in 10 percent of women with intact anal sphincters. Transanal ultrasound in combination with decreased anal pressures correctly identified all intact sphincters and 90 percent of known anal sphincter injuries.     

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.     

Prospectively evaluating anal sphincter function after ileal pouch-anal canal anastomosis

The decreased anal sphincter pressure that occurs after ileal pouch-anal canal anastomosis (IPAA) has usually been attributed to damage of the internal and sphincter. We hypothesized that the operation damages both the internal and the external anal sphincter. Resting pressure in the anal canal (a function of internal and external sphincters), anal squeeze pressure (a function of external sphincter only), and the rectal-anal inhibitory reflex (involving the internal sphincter) were measured manometrically in 10 patients with ulcerative colitis (4 women and 6 men; mean age, 33 years; range: 20 to 49 years). The patients were studied while awake before IPAA, under general anesthesia with striated muscle blockade just before incision, awake 2 months later before ileostomy takedown, and again under anesthesia with blockade just before takedown. The operation decreased maximum resting anal pressure while awake and during anesthesia with blockade. The decrease was detected in the proximal anal canal but not in the distal anal canal. In addition, the operation impaired anal squeeze pressure and abolished the rectal-anal inhibitory reflex. We conclude that IPAA damages both the internal and the external anal sphincter.     

Topical nitroglycerin in the management of anal fissure: an explosive outcome!

Anal sphincter spasm is a common finding in patients with anal fissure disease. It is postulated that spasm impedes mucosal blood flow and impairs healing. Topical nitroglycerin (NTG), a nitric oxide donor compound, has been shown to cause relaxation of the anal sphincter and may have treatment efficacy in the management of anal fissure. The purpose of this study was to assess the usefulness of NTG for anal fissure. We performed a retrospective review of patients with anal fissure treated with various concentrations of topical NTG ointments over an 18-month period ending July 1997. Of the 81 patients studied, 44 (54%) were male. There were 42 acute and 39 chronic fissures. NTG preparations included 1 per cent isosorbide (n = 37), 0.2 per cent NTG (n = 38), and 0.5 per cent NTG (n = 6). Healing with NTG therapy occurred in 29 acute (69%) and 21 chronic fissure (54%) patients. There was no difference in the incidence of healing of acute or chronic fissure between the various NTG treatment preparation groups. When acute and chronic fissure therapy was subdivided by time of NTG treatment (immediate versus post-conservative therapy failure (PCF)), 14 (74%) of acute PCF and 5 (42%) of chronic PCF patients healed. We conclude that no single formula was superior. When patients were subdivided into a PCF group, NTG therapy demonstrated a significant salvage rate, thus avoiding surgery.     

Computer-generated profiles of the anal canal in patients with anal fissure

PURPOSE: The pathophysiology of anal fissure remains poorly understood. This study examines manometric findings in patients with anal fissure with use of a computer-assisted system, which helps to standardize manometric performance as well as generating longitudinal and cross-sectional profiles of the anal canal. METHODS: Water-perfused, eight-channel, computer-assisted manometry was performed on 12 patients with chronic anal fissure and compared with 12 age-matched and sex-matched controls. RESULTS: Mean maximum average resting pressure (MARP) was 120.5 mmHg in patients and 82.6 mmHg in controls (P = 0.0005). Pain was felt during manometry in six patients. In these patients, MARP was 123.2 mmHg, and, in the other six patients, MARP was 117.8 mmHg. Sphincter length was 4.72 cm, and the high pressure zone or that part of the sphincter with pressure more than 50 percent of MARP) was 2.78 cm in length. The high pressure zone/sphincter length ratio was 58 percent compared with 48 percent in controls. Longitudinal profile was bell shaped. Elevated pressures were not confined to the site of the fissure. Cross-sectional analysis showed higher anterior pressures in the distal sphincter. Ultraslow waves were seen in as many as 91 percent of patients and 73 percent of controls. However, ultraslow wave amplitude was 31 mmHg in patients and 15 mmHg in controls (P = 0.03). The rectoanal reflex was normal; overshoot was not seen. CONCLUSIONS: The primary abnormality in fissure is persistent hypertonia affecting the entire internal sphincter, unrelated to pain. Cross-sectional pressure profiles may explain the predilection of fissures to occur in the posterior midline; other factors must prevent chronic fissures from healing.     

Distribution of the interstitial cells of Cajal in the human anorectum

The interstitial cells of Cajal are proposed to have a role in the control of gut motility. The aim of this study was to establish the distribution of interstitial cells of Cajal in the wall of the normal human anorectum. Interstitial cells of Cajal express the proto-oncogene c-kit. Interstitial cells of Cajal were identified in the colon by immunohistochemical staining, using a rabbit polyclonal anti-c-kit antibody. Anorectal tissue was obtained at surgical resection for carcinoma of the colorectum. Density of interstitial cells of Cajal was graded. Statistical analysis was performed using chi2 tests. In the longitudinal and circular muscle layers of the rectum interstitial cells of Cajal were seen in the bulk of the muscle layer. In the intermuscular plane interstitial cells of Cajal encased the myenteric plexus. Interstitial cells of Cajal were found at the inner margin of the circular muscle and in association with neural elements of the submuscular plexus. Within the internal anal sphincter interstitial cells of Cajal were infrequently scattered among the muscle fibres. The density of interstitial cells of Cajal in the internal anal sphincter was significantly lower than that observed in the circular muscle layer of the rectum (P = 0.014). In conclusion, interstitial cells of Cajal are evenly distributed in the layers of the muscularis propria of the rectum, but have a lower density in the internal anal sphincter.     

Anal sphincter defects. Correlation between endoanal ultrasound and surgery

OBJECTIVE: This study was performed to (1) correlate and sphincter defects, identified by endoanal ultrasound with operative findings, and (2) define the appearance of such sphincter defects as seen at operation. SUMMARY BACKGROUND DATA: Endoanal ultrasonography is a minimally invasive method of imaging the anal sphincter complex and enables identification of anal sphincter defects. Little is known about the accuracy and limitations of endoanal ultrasound in identifying such defects. Furthermore, there are no data about the appearances of these endosonic sphincter defects as seen at operation. METHODS: Forty-four patients (40 women; age range, 26 to 80 years; mean age, 56 years) with fecal incontinence, undergoing pelvic floor repair, were investigated by endoanal ultrasound before operation. Endosonic findings were correlated with the appearances of external anal sphincter, internal anal sphincter, and intersphincteric space, at operation. Diagnosis of the site and type of defect was made by macroscopic appearances. Uncertainty about the type of sphincter defect was resolved by obtaining muscle biopsies for histology. RESULTS: All external sphincter defects seen by endoanal ultrasound (n = 23) were confirmed at operation. Twenty-one of 22 internal sphincter defects identified by endosonography also were confirmed at operation. In ten patients with a neuropathic anal sphincter complex, the morphology was normal on endosonography, and this was confirmed at operation. (Sensitivity and specificity of 100% for external anal sphincter; 100% and 95.5%, respectively, for internal and sphincter) CONCLUSIONS: These data show that endoanal ultrasound is an accurate method of identifying anal sphincter defects.     

Internal and external anal sphincter anatomy as it relates to midline obstetric lacerations

OBJECTIVE: To examine the anatomy of the internal and external anal sphincters in the area of midline obstetric lacerations, to gain insight into sphincter damage and repair. METHODS: The length, craniocaudal extent, and overlap of the internal and external anal sphincters in the perineal body were measured in 17 cadavers. Further anatomic observations were made in four sets of whole pelvis cross-sections taken in the sagittal, coronal, and transverse planes. During the repair of 20 acute fourth-degree lacerations, observations were made to determine the internal sphincter visibility following birth. RESULTS: The external and internal and sphincters overlap by 17.0 mm (standard deviation [SD] 6.9), with the internal sphincter lying between the external sphincter and the anal canal. The internal sphincter extends an additional 12.2 mm (SD 5.9) cranial to the proximal extent of the external sphincter, whereas the caudal margin of the internal sphincter lies 3.7 mm (SD 7.2) cranial to the distal margin of the external sphincter. In pregnant women who sustained a fourth-degree laceration, we found that the internal sphincter can be identified as a rubbery white layer adjacent to the anal submucosa lying between the external sphincter and the anal canal. CONCLUSION: The internal anal sphincter lies between the anal mucosa and the external anal sphincter and extends more than a centimeter above the cranial margin of the external sphincter, a region where it is disrupted when a fourth-degree obstetric laceration has occurred.     

Intracranial pressure in single-suture craniosynostosis

To assess changing patterns of treatment for chronic anal fissure, a retrospective analysis of treatment for chronic anal fissure within one hospital between January 1990 and December 1996 was undertaken. A total of 221 patients received treatment for a chronic anal fissure in this period, of whom 209 had a surgical procedure. Manual dilatation of the anus was performed in 21 patients (10%) and has not been performed since 1995. Lateral internal sphincterotomy was performed in 183 patients (88%) and continues to be the mainstay of treatment. Five female patients (2%) were identified as having a sphincter defect by anal manometry combined with endoanal ultrasound and were treated by an anal advancement flap. From 1996 onwards, 15 patients (7%) were treated by topical glyceryl trinitrate (GTN) paste as the first line of treatment. Of these patients, nine have experienced healing of their fissure, and three have had relief of pain without healing of the fissure. Three have gone on to have a lateral internal sphincterotomy. Lateral internal sphincterotomy remains the primary form of treatment for chronic anal fissure. GTN cream has increasingly been offered as preliminary treatment over the last 12 months. Perioperative use of endoanal ultrasound allowed identification of patients who may be at high risk of postoperative incontinence from a sphincterotomy. An anal advancement flap has been used as an alternative surgical approach for these patients.     

A critical appraisal of pathogenesis and morbidity of surgical treatment of chronic anal fissure

Preoperative and postoperative manometric findings and the results of lateral internal sphincterotomy were analyzed in 44 consecutive patients affected with chronic anal fissure. Preoperatively, resting anal pressure was increased in 32 patients. At one month postoperatively, 23 patients showed normal pressures, whereas 14 were still hypertonic and seven, hypotonic. Only three patients still had a weak sphincter six months postoperatively. The overall morbidity rate was 31.8 percent. Minor complications occurred in 11 patients. Major complications affected three patients. Overall, impaired continence was recorded in eight patients, although only two complained of persistent, albeit lesser, defects of continence not requiring the use of pads. Nonoperative treatment should be reserved for few selected patients with recent, acute fissures. As for chronic and fissures, compared with other operative or nonoperative modalities of treatment, lateral internal sphincterotomy is a highly successful procedure and its minimal morbidity is well accepted by the patient.     

Chronic anal fissure: results of the surgical treatment of 220 patients

Since results from non-surgical procedures designed for treatment of chronic anal fissure are still controversial, sphincterotomy remain as the "state of the art" therapy for this condition. In a retrospective basis, the authors intend to review results from treatment of chronic anal fissure in 220 patients who underwent surgical procedure between 1984 and 1995. Data from chart review included age, sex, location of the lesion at the canal anal, associated anorectal disease, delivered surgical technique and complications. Seventy per cent of the patients were male. Mean age was 37.1 years. Fissure was located at the posterior midline in 86.1%. Associated anorectal conditions occurred in 41.4%. Fissurectomy plus posterior sphincterotomy was the treatment of choice in 84.1%. Complications occurred in 5 (2.3%) cases. There were no incontinent patients. Mean follow-up was 2.6 years. The authors conclude that partial internal anal sphincter section produces excellent results in treatment of chronic anal fissure. Posterior sphincterotomy may persist effective and safe since continence impairment was not identified in the present study.     

Idiopathic constipation in childhood is associated with thickening of the internal anal sphincter

This study was undertaken to search for a rational basis for the use of anal dilatation and internal sphincterotomy as the treatment for chronic intractable constipation in children. Sixteen children, age 5 months to 13 years, who had constipation resistant to conservative treatment were compared with 39 age-matched controls. History and current symptoms were assessed using a standard questionnaire. Internal and external and sphincter morphology was assessed on clinical examination and by anal endosonography, using a 10-MHz rotating endoprobe to provide accurate measurement of the various components of the anal canal. The control group showed a linear correlation between the thickness of the internal anal sphincter and both age and weight, increasing from 0.4 mm in infancy to 0.9 mm in adolescence. Children who had constipation displayed significant thickening of the internal sphincter (range, 0.5 to 1.9 mm, P = .005) which was independent of the length of the history (P = .103). There was no difference in the morphology of the external anal sphincters between the groups. The finding of a hypertrophied internal anal sphincter could provide a rational basis for anal dilatation and internal sphincterotomy as treatment for idiopathic constipation.     

Anorectal pull-through and vector volume manometry

Anorectal manometry has been developed from an object of clinic oriented scientific interest to an established tool within the spectrum of proctologic diagnostics. At present it represents the only objective diagnostic method with regards to continence disorders; it is routinely applied in the evaluation of constipation, of incontinence, of pre- and postoperative conditions in diseases of the anorectum as well as in the indication and therapeutic control investigations during biofeedback training of the anal sphincter. The new technique of determination of the anal sphincter pressure vector volume allows for the most sensitive functional separation of patients with different degrees of continence; this method delivers a 3-dimensional graphic imaging of the sphincter pressure profile, thus allowing for an objective visualisation of the present mechanic defect and an aid in planning a surgical correction.