Anal sphincter dysfunction in Parkinson's disease.

Striated anal sphincter function was studied electrophysiologically and radiologically in six patients with Parkinson's disease and chronic constipation. In five cases, there was paradoxic anal sphincter muscle contraction during simulated defecation straining resembling anismus-type pelvic outlet obstruction. Radiologic studies showed functional improvement of the defecatory mechanism following the administration of the dopamine receptor agonist apomorphine in four patients. Dysfunction of the striated anal sphincter musculature may be a significant cause of constipation in some parkinsonian patients, occurring as part of the generalized extrapyramidal motor disorder.

Paediatric sedation for CT scanning: the safety and efficacy of quinalbarbitone in a district general hospital setting.

The aim of this study was to review retrospectively the safety and efficacy of a paediatric sedation protocol in a district general hospital radiology department. 256 children attended for CT scanning over a 40-month period. 40 children required sedation and were given quinalbarbitone. 34 (85%) of this group were adequately sedated. Of the children who received quinalbarbitone, 35 were under 5 years of age. 32 of this group (91.4%) were adequately sedated. Failures in children under 5 years were all caused by problems with administration whilst failures in the older children were due to paradoxical excitement. No problems with respiratory depression were encountered. Sedation can be safely performed in a district general hospital radiology department if a structured protocol is adhered to. Quinalbarbitone is a safe, effective oral agent in children under the age of 5 years.

Clavicular overgrowth in association with cystic hygroma.

Cystic hygroma is a rare congenital abnormality of the lymphatic system of the neck which is now considered to form part of a spectrum of lymphangiomatous malformations. Lytic bone lesions are described in systemic lymphangiomatosis and bony overgrowth (hemihypertrophy) in association with angiomatous malformations of the limbs. Two infants with ipsilateral clavicular overgrowth adjacent to cystic hygromas are reported. This association has not been recorded previously; the possible etiology is discussed.

Anal endosonography: technique and normal anatomy.

Anal endosonography using a specially designed hard cone attachment to a radial 7-MHz probe has been performed in 26 normal patients -3 patients following lateral anal sphincterotomy, 1 patient undergoing electromyophysiological mapping of the external and sphincter, and in 2 resected specimens. The examinations were rapid, simple, and well tolerated, and they provided high-resolution images of the five layers of the anal canal: mucosa, submucosa, internal and sphincter, intersphincteric plane, and external anal sphincter. Views of the ischiorectal fossa were limited. The configuration of the anterior part of the external anal sphincter differed between males and females.

Anal endosonography in the investigation of faecal incontinence.

Forty-four consecutive patients with incontinence of solid stool of traumatic or idiopathic aetiology were examined by anal endosonography and standard anorectal physiology tests. Anal endosonography showed an external anal sphincter defect in four out of 11 (36 per cent) patients with idiopathic (neurogenic) incontinence. In the remaining seven patients both parts of the sphincter were intact and a linear relationship was found between the resting anal canal pressure and the endosonographic thickness of the internal anal sphincter. Twenty-eight out of 33 (85 per cent) patients with incontinence of traumatic origin had external sphincter defects, confirmed by concentric needle electromyogram mapping in the 19 patients in whom this was performed. Eleven of these 28 (39 per cent) patients also had disruption of the internal sphincter. Anal endosonography has revealed significant abnormalities in patients with faecal incontinence and has a complementary role to anorectal physiology in the routine investigation of these patients.

A comparison between electromyography and anal endosonography in mapping external anal sphincter defects.

Mapping of the external anal sphincter by anal endosonography was compared with the electromyographic findings in 15 patients with fecal incontinence after perineal trauma. Both examinations showed no defect in three patients. In the 12 patients with muscle defects, there was agreement on the quadrant involved in all patients. In seven patients, there was total agreement in the hours of the defect, in four there was a one hour discrepancy, and in one there was a two-hour difference in the measured defect. Correlation between the two techniques was high (r = 0.96; P less than 0.01). Anal endosonography is better tolerated by patients than electromyographic mapping and is a useful technique for assessing posttraumatic defects of the external anal sphincter.

Anal endosonography in the evaluation of perianal sepsis and fistula in ano.

Anal endosonography has been performed in 22 patients with fistula in ano and perianal sepsis and compared with the operative findings. Using a special hard cone attachment to a radial 7 MHz probe the examination was well tolerated, rapid and generally accurate, detecting two unsuspected foreign bodies and all seven complicated fistula in ano preoperatively.

Hereditary internal anal sphincter myopathy causing proctalgia fugax and constipation. A newly identified condition.

A newly identified myopathy of the internal anal sphincter is described. In the affected family, at least one member from each of five generations had severe proctalgia fugax; onset was usually in the third to fifth decades of life. Three members of the family have been studied in detail. Each had severe pain intermittently during the day and hourly during the night. Constipation was an associated symptom, in particular difficulty with rectal evacuation. Clinically the internal anal sphincter was thickened and of decreased compliance. The maximum anal canal pressure was usually increased with marked ultraslow wave activity. Anal endosonography confirmed a grossly thickened internal anal sphincter. Two patients were treated by internal anal sphincter strip myectomy; one showed marked improvement and one was relieved of the constipation but had only slight improvement of the pain. The hypertrophied muscle in two of the patients showed unique myopathic changes, consisting of vacuolar changes with periodic acid-Schiff-positive polyglycosan bodies in the smooth muscle fibers and increased endomysial fibrosis. In vitro organ-bath studies showed insensitivity of the muscle to noradrenaline, isoprenaline, carbachol, dimethylpiperazinium, and electrical-field stimulation. Immunohistochemical studies for substance P, calcitonin gene-related peptide, galanin, neuropeptide Y, and vasoactive intestinal peptide showed staining in a similar distribution to that in control tissue. A specific autosomal-dominant inherited myopathy of the internal anal sphincter that causes anal pain and constipation has been identified and characterized.