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At least 1,333 records · Page 74Linked to original sources
PubMed1994

Results in the treatment of anorectal malformations with special regard to the histology of the rectal pouch.

The results of surgery for anorectal malformation (ARM) in 536 children treated between 1962 and 1993 are presented. Before 1985, patients underwent an abdominoperineal pullthrough for high and intermediate type of ARM, whereas cut-back procedures or perineoproctoplasty were performed in low type. Since 1985, we have changed our policy and performed a modified posterior sagittal anorectoplasty (PSARP) for intermediate type of ARM, eventually combined with an abdominal mobilization for high type. Perineoproctoplasty was reserved for low type of ARM. Data of 143 patients of Group 1 and 85 patients of Group 2 were available for follow-up. Continence was compared using a clinical score. Good or fair continence was found in 82% of patients operated on before 1985 (low type 85% good, 15% fair, intermediate type 17% good, 50% fair, high type 12% good, 37% fair), comparing to 93% in children treated after 1985 according to the new regimen (low type 88% good, 6% fair, intermediate type 59% good, 35% fair, high type 43% good, 47% fair). Liquid stools and inability to retain bowel contents were the main problem in the older series, whereas incontinence could often be attributed to constipation with overflow incontinence in the recently treated group of patients. Analysis of the innervation of fistula and distal rectal pouch in a prospective study comprising 40 of our most recent patients showed normal innervation in only 5% of patients, whereas 66% had neuronal intestinal malformations (NIM) including aganglionosis, NID and hypoganglionosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Value of a score in the electromanometrical diagnosis of neuronal intestinal dysplasia.

Anorectal manometry was performed in a series of 8 children where NID was histochemically confirmed or suspected. The results were transferred to a electromanometric score theoretically worked out previously. Clinical symptoms as well as histological and manometric findings have been compared concerning the meaningfulness of the electromanometric score in the diagnosis of NID. It was impossible to make a statistical statement due to the small number of patients we examined. It seems, however, that certain electromanometric parameters in NID appear regularly which, when combined in a score, make manometric diagnosis of NID sufficiently reliable.

Adolescent↗
PubMed1994

Quantitative investigations of acetylcholinesterase activities in colorectal malformations.

In cases of colorectal innervation disturbance the histological and histochemical findings are well placed to classify the morphological state and to indicate to the pediatric surgeon the therapeutic direction. Any limitation of their value results from technical errors, age dependent localization of morphological structures and the subjectivity of the assessment. For this reason a quantitative biochemical investigation of cholinesterase activity in homogenized investigation of cholinesterase activity in homogenized rectal mucosa specimens and resected bowel segments was performed. The biochemical standard value of the AChE-activity amounts up to 3-6 x 10(-7) mol ACh/min/g in healthy test patients and the ratio of specific/unspecific esterases 1.1 to 1.3. The percentage of AChE to the total enzyme activity was 47.5%. The standard distribution of the different globular and asymmetric cholinesterase structures is described by the proportion of the molecular types G1/G2/G4 and A12. These main types determine the biochemical nature of the human rectum. Hypertrophy of cholinergic nerve fibres in aganglionic bowel segments correlates pathobiochemically with the increase of the cholinesterases caused by the high molecular tetramer G4. However, the Hirschsprung's disease could not be differentiated by the percentage of the AChE-activity to the total ChE-activity because of the continuous increase in the BChE in the aganglionic tissue. Neuronal intestinal dysplasias with their main cholinergic nerve fibre hyperplasias are characterized by disturbances of the dualism and interrelation of different cholinesterases. By means of pathogiochemical investigations the subjective factor in the assessment of histochemical states is avoided and the diagnostic accuracy increased.(ABSTRACT TRUNCATED AT 250 WORDS)

Acetylcholine↗
PubMed1994

The incidence of occult spinal dysraphism and the onset of neurovesical dysfunction in children with anorectal anomalies.

The urological malformations associated with anorectal anomalies (ARA) are not only anatomical, but also functional, the latter being related to congenital neurovesical dysfunction (NVD). The true incidence of spinal dysraphism (SD) in these children is still unclear and is probably underestimated. The concept of caudal regression could explain its association with the anorectal anomalies. Because of awareness of the late onset of neurovesical dysfunction and/or orthopaedic symptoms in some of our patients, in 1991 we started to screen with magnetic resonance imaging (MRI) the spinal cord of all patients with ARA. Eighteen (44%) out of 41 patients without neurological or orthopaedic symptoms and 7 (78%) of 9 children with neurological or orthopaedics symptoms screened by MRI showed pathological findings. The overall incidence of spinal dysraphism in ARA was 50%, without any great difference with respect to the type of the anomaly (high, low, cloacal). The pathological MRI findings encountered were: fibrolipoma (with or without a thickened filum terminale), tethering of the cord and syringomyelia, and sac anomalies. In order to check the onset of NVD in these children, we performed urodynamic studies with external sphincter electromyography in 24. Grouped by age: 14 were between 5 and 18 months and ten were 4 to 13 years old. Ten patients (71%) out of the first group and 3 (30%) out of the second had a normal urodynamic pattern. A total of 11 children had pathological findings; of these, 4 infants had a hyperreflexic bladder (one with detrusor-sphincter dyssynergia) suggesting upper motor neuron (UMN) lesion. Of the 7 older patients, two had UMN and 3 lower motor neuron (LMN) lesion.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1995

Sensation in the anal region and rectum after surgery for anorectal malformations.

Sensation of the anal region and rectum after surgery for anorectal malformations was investigated in 32 patients (17 with high type, 6 with intermediate type and 9 with low type anomaly), aged 5 to 16 years. All patients were examined as to whether or not they could appreciate sensations of touch, pain, heat and cold in the circumanal skin and in the anal canal. Rectal sensation and compliance were also examined by anorectal manometry. All patients had normal sensation in the circumanal skin, but less than half of them had normal sensation in the anal canal. Patients with normal bowel control showed a significantly higher incidence of touch sensation in the anal canal than those with fecal incontinence or constipation. It was suggested that a sensation defect of the anal canal is related to a disturbance of discrimination between flatus and feces. The results of rectal sensation and compliance showed that reduced elasticity of the neorectum and low rectal compliance were important factors of fecal incontinence after surgery for anorectal malformations.

Adolescent↗
PubMed1995

Posterior sagittal anorectoplasty: functional results of primary and secondary operations in comparison to the pull-through method in anorectal malformations.

BACKGROUND: Control of defaecation after surgical correction of high and intermediate types of congenital anorectal malformations is difficult. The posterior sagittal approach with careful reconstruction of the external sphincter is postulated to give a better outcome than the pull-through operation. The functional results of these procedures performed in one centre were evaluated in order to identify prognostic factors. MATERIAL AND METHODS: Between 1979 and 1992 66 patients with high or intermediate congenital anorectal anomalies were treated in our centre. In 16 patients a pull-through operation (Kĩesewetter-Rehbein) was performed. After 1984, the posterior sagittal anorectoplasty (PSARP) (Peña and deVries) was used in 35 patients as the primary operation. In 22 patients a PSARP operation was done as a secondary procedure, in one third of these patients after a previous pull-through operation in our centre. The functional results were analysed in retrospect. RESULTS: Sixty operations in 53 patients could be evaluated. The overall continence rate was 34%. After the pull-through operation six out of 15 patients (40%) were continent, after a primary PSARP 10 out of 25 (40%) and after a secondary PSARP operation five out of 20 patients (25%). Patients with a sacral defect were continent only in 16% as compared to 44% of the patients with a normal sacrum. Sex was also relevant: 67% of the girls were continent, compared to 30% of the boys. Ten out of 11 girls (90%) with a normal sacrum became continent. CONCLUSION: The PSARP for high and intermediate anorectal malformations does not give better functional results than the pull-through operation. The prognosis is determined by other factors than the type of operation, notably sex and the presence or absence of sacral defects.

Anal Canal↗
PubMed1996

Surgical reconstruction of a high cloacal anomaly with two short vaginas.

We treated a patient with a complex cloacal malformation: a high confluence cloaca and 2 separate short vaginas. At the age of 1 year and 2 months the rectocloacal fistula was closed because of frequent urinary infections, and the vaginas were separated from the wide urogenital sinus and anastomosed to the lower colon. At the age of 2 years and 4 months the lower colon, which had been anastomosed with the 2 vaginas, was pulled down to the perineum to serve as a lower vagina, and the sigmoid colon was pulled through to form a neorectum. She is now 7 years old and goes to school without problems of bowel function or urinary function except that catheterization is done twice a day.

Anus, Imperforate↗
PubMed1996

Neurovesical dysfunction due to spinal dysraphism in anorectal anomalies.

Spinal dysraphism (SD) has been found associated with functional abnormalities of anorectal anomalies (ARA). The incidence of SD in these children is probably underestimated and a complete neuroradiological investigation of the lower urinary tract function has not been carried out routinely. In a 2 years time frame we performed urodynamic (UDS) evaluations on 14 patients (8 males and 6 females) with ARA who showed SD at Magnetic Resonance Imaging (MRI) studies. We divided them into 2 groups by age: group A (5 to 18 months) and group B (3 to 12 years). The UDS findings were classified as upper (UMN) and lower motor neuron (LMN) lesion. Out of the 9 children included in group A, 5 showed normal urodynamic pattern, while 4 had pathological UDS findings suggesting UMN lesion. Among the older (group B) children only one had normal and 4 had pathological findings: 2 hyperreflexia suggesting UMN lesion and 2 external sphincter denervation suggesting LMN lesion. These data support the hypothesis that the neurovesical dysfunction found in children with anorectal anomalies results from a possible association with spinal dysraphism. An early morphological evaluation of the spinal cord is mandatory in all children with ARA prior to definitive surgical correction of the malformation. The low incidence of pathological UDS findings in small children if compared to older ones suggest that SD, although present, may be asymptomatic.

Age Factors↗
PubMed1997

Acetylcholinesterase (ACE) staining shows the abnormal innervation of a pulled-through rectum in a case of repaired anorectal malformation.

Abnormal innervation of the anorectum was noted in relation to anal incontinence in a case of repaired high-type anorectal malformation (ARM). A ten-year-old boy presented with anal incontinence after reconstructive surgery of ARM with a recto-urethral fistula. An anorectal manometrical examination revealed both an adequate tonus of the anal sphincter muscles and the absence of rectoanal reflex relaxation. And a barium enema showed a narrow region in the rectosigmoid colon, which was similar to that of Hirschsprung's disease (HD). Furthermore, an acetylcholinesterase (ACE) histochemical study of the rectal suction biopsies revealed an increased number of ACE-positive nerve fibers in the lamina propria mucosae and muscularis mucosae of the pulled-through colon. At the same time, however, some ganglia cells were also observed in the submucosa of the affected rectosigmoid colon and these cells could not be found in HD. Although the mechanism by which the abnormally innervated parasympathetic nerve fibers arose in the pulled-through colon remains unclear, this neuronal abnormality is considered to be the cause of anal incontinence in this case.

Acetylcholinesterase↗
PubMed1998

Neurovesical dysfunction in patients with anorectal malformations.

UNLABELLED: The aim of the study is to investigate the incidence of neurovesical dysfunction (NVD) in patients with operated anorectal malformations (ARM) and its relationship with sacral abnormalities. Between September 1992 and April 1994 cystomictiometric and electromyography investigations were performed for 33 patients operated on for anal atresia. The age of these 14 male and 19 female patients ranged between 1-17 years (mean age: 7.7). The level of ARM was supralevator in 10 patients and infralevator in 23 patients. The x-ray of 14 patients showed lumbosacral abnormalities. In urodynamic investigation, NVD was observed in 15 patients (45.4%). 4 of the patients had signs developed secondary to organic subvesical obstruction. The urodynamic results of 14 patients were in the normal range. NVD rate was found to be 70% in patients with supralevator anal atresia, 34.7% in patients with infralevator anal atresia, 64.2% in patients with sacral abnormalities and 31.5% in patients with no sacral abnormality. CONCLUSION: NVD incidence increased in patients with supralevator ARM and in patients with sacral abnormalities. However, presence of infralevator abnormality or absence of sacral abnormality does not eliminate NVD. We think that, whenever NVD is suspected in a patient with ARM, urodynamic investigations should be performed as a routine investigation of the urinary tract.

Adolescent↗
PubMed1998

A complex, high cloacal malformation: case report.

Cloacal malformations are rare, complex inhibitional anomalies of early embryogenesis. We report a patient with a cloacal malformation in which a septate vagina and a rectal fistula emptied through a common orifice onto an exstrophic bladder plate. Additional anomalies included an omphalocele and malformations of the upper urinary tract and the lower extremities, skeleton, and vertebral column.

Abnormalities, Multiple↗
PubMed2000

Congenital anomalies in mice induced by etretinate.

We report on the experimental induction of anorectal malformations (ARM) and other internal and external malformations in mouse fetuses induced by maternal administration of etretinate, a long-acting vitamin A analogue. The teratogen was administered to pregnant mice between the 7th and 10th gestational days (E7 and E10). The mice of the control group were given pure sesame oil on E9. We examined survival rates, crown-rump length, and the incidence of internal and external malformations, with particular attention to ARM, in each group. All mice in the E8 group exhibited rectovesical fistula, hydronephrosis, and spina bifida. All males and females in the E9 group given 60 mg/kg of etretinate developed rectoprostatic urethral fistula and rectocloacal fistula, respectively. The E10 group, given 60 mg/kg of etretinate displayed cleft palate (63.6%), forelimb malformation (68.2%), and a short club-shaped tail (100%). The fetuses had more severe types of ARM when etretinate had been administered on an earlier gestational day. The E9 group is a useful model for anorectal malformation, whereas the E8 group is a model for hydronephrosis and spina bifida.

Abnormalities, Drug-Induced↗
PubMed1987

Repair of cloacal malformation using combined posterior sagittal and abdominal perineal approaches.

Cloacal malformations in female infants are not very common and their treatment is most challenging because every case differs from the others. In the light of a well-documented case, the authors describe the complexity of the malformation, the difficulties of making a correct detailed diagnosis, and the chronology of repair. They stress the advantage of the posterior sagittal approach for the first part of the operation, changing for the second part to a supine and later to a lithotomy position. In most cases a satisfactory reconstruction is possible, anatomically as well as functionally.

Abdomen↗
PubMed1987

[Morphologic studies in swine with anal atresia].

33 piglets with anorectal malformations were investigated histologically. 24 animals were male, 9 female. 18 piglets had high deformities with recto-urethral, respectively recto-vaginal fistulas. 15 animals had low deformities. In all animals we were able to demonstrate a sphincter internus muscle and a sphincter externus muscle. The sphincter internus muscle was localised around the fistula. These fistulas also had other characteristics of a normal anal opening.

Anal Canal↗