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.     

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.     

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.     

Treatment of colo-vesical fistulas in one-stage operation

BACKGROUND: Botulinum toxin A is a potent inhibitor of the release of acetylcholine from nerve endings. Local injection of botulinum toxin has recently been suggested to be helpful in sphincter of Oddi dyskinesia by decreasing sphincter of Oddi pressure. AIMS: To explore the mechanism of action of botulinum toxin A on sphincter of Oddi (SO) muscle. METHODS: Four piglets underwent duodenoscopy and SO manometry was performed. After obtaining a baseline pressure, the SO was injected with normal saline and the experiment repeated after one week. The SO was then injected endoscopically with botulinum toxin (40 U) with follow up manometry one week later. The sphincter of Oddi was removed from 10 pigs, cut into three rings, and placed in an organ bath. The force of contraction was measured and registered on a polygraph. Rings were stimulated by 70 V (10 Hz, 0.5 ms) electrical field stimulation for 20 seconds, exogenous acetylcholine (100 microM), and KCl (125 mM). Botulinum toxin (0.1 U/ml) or atropine (1 microM) was added to the incubation medium and the stimulation was repeated. RESULTS: Mean basal SO pressure in the pigs remained unchanged after saline injection but decreased to about 50% of baseline value following botulinum toxin injection (p = 0.04). The contractions induced by direct stimulation of SO smooth muscle with KCl were not significantly affected by either atropine or botulinum toxin. In all rings exogenous acetylcholine induced contractions, which were totally blocked by atropine, but not by botulinum toxin. Electrical field stimulation induced contractions that were inhibited by both atropine and botulinum toxin. CONCLUSION: Botulinum toxin inhibits pig sphincter of Oddi smooth muscle contractions by a presynaptic cholinergic mechanism, similar to that described in skeletal muscle.     

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.     

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.     

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.     

Botulinum toxin in the management of the lower limb in cerebral palsy

The role of intramuscular botulinum toxin A in the treatment of 26 children with cerebral palsy was evaluated. The indication for injection was the presence of a dynamic contracture of lower-limb muscles interfering with positioning or walking. Spastic target muscles were identified by clinical examination and, in ambulant children, by gait analysis. Between 50 and 320 units of botulinum toxin were injected into each muscle group to a total dose of 100 to 400 units per child. The effects of injection were monitored by repeated clinical examination and gait analysis. There were no clinically detectable systemic side-effects, and all but one patient had a reduction in tone, which occurred within three days and persisted for two to four months. There were significant improvements in ambulatory status and in sagittal-plane kinematics. In some cases these gains persisted after the tone-reducing effects of the toxin had worn off.     

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.     

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.     

Orthognathic surgery: the patients'' perspective

We have evaluated our practice of tube caecostomies in 21 children operated on from January 1982 to December 1987 at the Royal Hospital for sick children, Bristol, and 18 children operated on at the Paediatric Surgery Unit of the Korle-Bu Teaching Hospital, Accra, Ghana from January 1989 to December 1996. The indications for surgery were, Hirschsprung's disease (36) and idiopathic constipation (3). The definitive procedures involved were Duhamel's procedure in 36, Soave's procedure in 2 and colo-anal anastomosis in 1 case. This method reduces the total number of surgical operations required by the child from 3 to 2, thereby reducing the total period of hospitalisation for the child. A sample technique of tube caecostomy is described in 39 children undergoing corrective surgery.     

Gluteus maximus augmentation for the treatment of fecal incontinence

Fecal incontinence is a devastating problem for school-aged children and adults. Medical and biofeedback therapies are unsuccessful in most patients who have severely defective internal and external sphincters. Continued fecal incontinence frequently leads to social isolation and withdrawal. Gluteus maximus augmentation of the sphincter mechanism is one surgical method for treating fecal incontinence. The authors present their results with gluteus maximus augmentation of the anal sphincter and describe patient selection criteria. From 1992 through 1996, seven patients underwent gluteus maximus augmentation of the anal sphincter for fecal incontinence. Six of these patients were children 5 to 6 years of age who had major deficiencies of their anorectal sphincter demonstrated by manometry. One patient was a 56-year-old adult woman who had acquired idiopathic fecal incontinence. Four of the six children (67%) had imperforate anus and two had cloacal anomalies (33%). The augmentation was performed in three stages. A sigmoid-end colostomy with a Hartman's pouch was followed 1 month later by rotation of a portion of the gluteus maximus for anorectal sphincter augmentation. A colostomy take down was performed 2 to 4 months later. All patients underwent dilatation after sphincter augmentation and were taught muscle exercises for using their neosphincter during the period before colostomy take down. Four of six children and the adult are continent postoperatively (71%). Both patients who remain incontinent are unable to sense rectal distention clinically or on anal manometric analysis but have excellent voluntary sphincter tone. Fecal incontinence can be successfully treated with gluteus maximus augmentation in carefully selected patients. Patients unable to sense rectal distension are unlikely to benefit from this procedure. The presence of a rectal reservoir and a skin-lined anal canal also appear to be important in attaining fecal continence.     

The effect of peritoneal air exposure on postoperative tumor growth

Botulinum toxin A has been used to treat wrist and finger spasticity mainly through injection of the forearm flexor muscles. This case study describes its first reported use in managing spastic lumbricals of the hand. A 19-year-old male had significant flexion deformity and hypertonicity of the left wrist and hand, particularly the second through fifth metacarpophalangeal joints, after traumatic brain injury. By using the 0-4 Ashworth scale, spasticity of the lumbricals across the second to fourth metacarpophalangeal joints was rated 2, with persistent clonus of the finger flexors as confirmed by electromyography to the middle and ring fingers, even after botulinum toxin A injection of the flexor digitorum sublimis and profundus muscles. By using the electromyography-guided technique, botulinum toxin A was injected into the first lumbrical of the index finger (12 units), second and third lumbricals of the middle and ring fingers, respectively (15 units each), and fourth lumbrical of the little finger (10 units). At follow-up, clinical and electromyographic examination revealed a significant reduction in tone and clonus of the injected lumbricals. Ashworth scores of the lumbricals from the index to little finger improved to 1. Botulinum toxin A injection of the lumbricals can be beneficial in managing spasticity of these muscles. It is well tolerated and effective at doses of 10 to 15 units. Lumbrical injection of botulinum toxin A is a useful adjunct in our percutaneous armamentarium for managing the spastic hand.     

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.     

Ethics in clinical research: searching for absolutes

Recently botulinum toxin has been used with increasing frequency as a safe and effective treatment for many previously refractory conditions associated with excessive muscle activity. The indications for use of botulinum toxin injection continue to expand. This report describes the case of an 83-year-old woman with a history of diabetes mellitus and lumbar spinal stenosis who developed a severe focal dystonia of the left great toe, such that the toe maintained the extended position. Functionally, the resultant deformity prevented the patient from wearing shoes. In addition, the patient had significant pain in the left great toe. Under needle electromyographic localization, 50 units of botulinum toxin were injected into the left extensor hallucis longus muscle. Two weeks after the injection the patient was symptom free and could place her left foot into a shoe. Seven months later, she remained symptom free. This case illustrates that localized injection of botulinum toxin to a specific lower limb muscle can effectively result in decreased muscle activity and functional improvement.     

One hundred cases of anal fissure treated with botulin toxin: early and long-term results

PURPOSE: Sphincterotomy still is considered the therapy of choice to eliminate sphincter spasm in the treatment of uncomplicated chronic anal fissure. The surgery is weighted with the possible surgical risk and the risk of subsequent fecal incontinence. This study reports the effect of botulin toxin injections within the first six months. PATIENTS AND METHODS: One hundred patients were treated (43 females; average age, 34.7 years). The injection of botulin toxin (2.5-5 units of Botox each) was done bilaterally to the fissure, thereby causing paresis of the sphincters for approximately three months. Patients were re-examined after one week and three and six months. RESULTS: Within the first week, 78 percent of patients were free of pain. In 82 percent of patients, complete healing of the fissure occurred within the first three months. Eight patients experienced relapses within the first six months of therapy, three of whom needed surgical intervention. The healing rate after six months was 79 percent. No healing occurred in 21 patients, and they had to undergo surgery. Transitory fecal incontinence resulted in seven cases. CONCLUSIONS: Injection of botulin toxin enables us to treat chronic, uncomplicated anal fissures with increased sphincter tone. It is well tolerated, can be administered on an outpatient basis, does not cause any lesion of the continence organ, and subsequently, does not lead to any permanent latent or apparent fecal incontinence.     

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.     

Anteriorly located anus: is constipation caused by abnormal location of the anus?

Anteriorly located anus (ALA) is frequently associated with severe constipation accompanied by defecation pain. Between 1988 and 1994, the authors treated 27 children (26 girls, 1 boy; age range, 0 to 11 years) to surgically correct ALA. The operation was performed according to a uniform protocol to longitudinally divide the internal sphincter muscle from the anal skin level to 2 cm above the dentate line on the posterior wall of the anorectum. For anal reconstruction, any of the conventional procedures was employed. Twenty-two of the 27 patients have had follow-up in our clinic for 12 months to 6 years (mean, 2.75 years). Eighteen are completely free of constipation and defecation pain and have regular spontaneous bowel movements. The other four require occasional use of enemas or laxatives. Anal incontinence did not occur in any patient. The results of this study suggest that abnormal function of the internal sphincter is the most likely cause of constipation or defecation pain in patients with ALA and that internal sphincterotomy is the cornerstone of surgical treatment.     

A water-soluble antitumor glycoprotein from Chlorella vulgaris

The great majority of children referred to Pediatric Gastroenterologic Units for chronic constipation have a functional disorder and do not require investigative techniques, since functional constipation is easily recognized an anamnestic and clinical basis. Severe chronic constipation indicates a chronic condition believed to be of functional type but unresponsive to the traditional pharmacologic treatment. However, several clinical features might suggest an organic type of constipation during the initial diagnostic approach: growth failure, distension, episodes of diarrhea intermingled with constipation, subocclusive events, dilatation of areas of the gut (megaduodenum, megajejunum) at x-ray examination of the entire gastrointestinal tract. Hirschsprung's disease is the best known organic type of constipation; however, there are other neurogenic and myogenic abnormalities of the colonic (and/or ileal) tract that mimick Hirschsprung's disease and represent development abnormalities of the enteric nervous plexus. Other organic types of constipation are due to systemic diseases, endocrine and metabolic disorders, central nervous system disorders. Organic constipation usually requires extensive investigative approach, including pathologic examination of enteric nervous plexus on full thickness biopsies.     

Botulinum toxin management of childhood intermittent exotropia

OBJECTIVE: Intermittent exotropia is a common form of childhood strabismus that has a late onset and presents a difficult and frustrating management dilemma. Surgical treatments have a high recurrence rate, and multiple surgeries often are required to achieve a desirable motor outcome. This study presents long-term observations on the use of botulinum toxin for the treatment of intermittent exotropia in children. DESIGN: This study is a nonrandomized, case-controlled study of consecutive pediatric patients who had intermittent exotropia. PARTICIPANTS: Thirty-two neurologically normal children ranging from 3 to 144 months in age were diagnosed with intermittent exotropia with a minimum distance deviation of 15 prism diopters (PD). INTERVENTION: Simultaneous bilateral injections of 2.5 units botulinum toxin type A were made into the lateral rectus muscles with the patient receiving nitrous oxide-ethrane inhalation anesthesia. Patients were observed for 12 to 44 months after the initial injection. MAIN OUTCOME MEASURES: A satisfactory outcome was considered to be stable binocular alignment of the eyes to an orthophoric range of +/-10 PD. RESULTS: Bilateral lateral rectus muscle injections of botulinum toxin were effective in reducing the mean preinjection deviation of -29 PD to an average exotropic angle of -6 PD. Stable orthophoria (+/-10 PD) was achieved in 22 patients (69%). Overall, male patients required significantly fewer injections than did female patients. All patients between 24 and 56 months of age, irrespective of gender, required only a single bilateral injection to achieve a favorable motor outcome. CONCLUSIONS: Botulinum toxin is at least as effective as surgical outcomes reported previously for the treatment of intermittent exotropia in children. This treatment method is particularly effective in children between 2 and 4.5 years of age irrespective of the initial strabismic angle and is not associated with any secondary abnormalities.