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PubMed1984

[Upper anal imperforation, treatment and results].

As shown by Stephens the only element of the sphincteric mechanism which persists in case of high imperforate anus is the pubo rectalis sling of the levator. It is essential that the néo rectum be brought down to the perineum throught the sling which must be meticulously dissected and preserved. An operative technique with definition of the sling by an anterior perineal approach was described by the author. Results of the procedure in 43 patients are reported: early post operative complications (1 death, 4 occlusions, 2 ischemia of the neo rectum), anal complications (stricture, prolapses and ectropion) and urinary complications. Concerning the continence the author studied 19 patients: 16 children operated over 10 years ago and 3 adults. Of these 19: 2 are failures with stool incontinence, 10 are excellent and 7 fair. A "perfect" result was never obtained but many are compatible with a normal social life. Age is of great importance (our 3 adults became continent within several months), morning evacuations, diet, cleanliners, some drugs such a imodium are helpful adjuncts. Then the paper discusses 16 patients with desastrous results after previous surgery in another hospital. In 10 patients the new ano-rectum has been placed some that it missed a portion or all of the pubo-rectalis muscule. A further operation with re-routing resulted in marked improvement in 7 patients. A poor result with the neo-rectum in a good position means that the incontinence is related to a hypoplastic or denervated sling. A free autogenous muscle transplantation (Hakelius and Grotte) gave 4 excellent, 2 fair and 2 poor results.

Abnormalities, Multiple↗
PubMed1980

[Congenital recto-urethral fistulas in adults. Two cases (author's transl)].

Presentation of two cases of imperforate anus managed in infancy. In first case, at age of 19, surgical closure of recto-urethral fistula associated to excision of a rectal pouch complicated with infected lithiasis and renal damage provides definitive therapy. In second case, recto-urethral fistula was well tolerated for more than 50 years without renal damage. Simple removal of a prostatic lithiasis partially obstructing fistula, provided fair good result. Etiology of these fistulae, well known in pediatric patients, is then discussed. Management must be adapted to complaints and risks.

Adult↗
PubMedDate not supplied

The VATER association: analysis of forty six cases without karyotyping.

Forty-six patients with two or more features of the VATER association admitted to the Mackay Memorial Hospital from May, 1983 to Mar, 1992 were retrospectively enrolled in this study. We compared the incidence of major features with that reported in the literature. Imperforate anus, congenital heart disease, and renal anomalies were the three most common major features in our study. Thirteen patients died. Heart failure was the major cause of mortality. We noted many patients associated minor features, and among them there was a relatively high ratio of cleft lip, cleft palate, and hypospadius. The overall outcome and development were good among the survivals. We suggest that children who have any congenital anomaly included in the VATER association should get a careful examination and evaluation of their heart, genitourinary tract, limbs and vertebrae. Early correction if possible is indicated in such patients.

Abnormalities, Multiple↗
PubMed1994

Exclusion therapy for terminal reservoir syndrome in children with repaired anorectal malformations.

Constipation, dyschezia and encopresis in association with fecal impaction in a hugely dilated ectatic terminal bowel were noted in 12 cases of imperforate anus after creation of an anorectal canal. The original anorectal malformations were of low anomalies in four cases, intermediate in one, and high in seven. Associated anomaly in the pelvis or sacrospinal area was present in four of the 12 cases, and dilated rectal pouch at birth was found in four of the eight patients examined. The symptom of constipation started invariably within a few months after anoplasty or closure of colostomy. Suction biopsy of the rectum excluded the possibility of aganglionosis. As elaborate conservative measures, including revision of the anteriorly displaced anus, failed to relieve the symptoms, exclusion of the ectatic terminal bowel by either excision or endorectal pull-through was carried out in nine of the 12 cases. The results were satisfactory: eight of the nine patients developed regular bowel movements and were totally continent within six months of surgery. It is suggested that the terminal reservoir is caused by primary rectal ectasia in the low anomalies and by primary or secondary ectasia, or a combination of both, in the high anomalies. Early exclusion of the ectatic terminal bowel is recommended for patients suffering from this syndrome after repair of anorectal malformations.

Anal Canal↗
PubMed1993

Anorectal anomaly in Pfeiffer syndrome.

A Japanese girl with Pfeiffer syndrome is described. She has clinical and radiological manifestations typical of the syndrome. In addition, she had an imperforate anus not described previously in the syndrome. This seems to expand the phenotype associated with Pfeiffer syndrome or represent a new acrocephalosyndactyly syndrome.

Acrocephalosyndactylia↗
PubMedDate not supplied

[OEIS complex. Description of a case associated with cardiopathy].

We describe a newborn affected by OEIS complex (Omphalocele--Exstrophy of bladder--Imperforate anus--Spinal defect) associated with atrial septal defect. Terminal ileostomy, closure of the abdominal wall defect and suture of the cecal and vesical plates were performed. The patient died at 5 months and four days.

Abnormalities, Multiple↗
PubMed1977

Cloacal dysgenesis.

Cloacal dysgenesis is a rare anomaly. Fifty cases have been reported in the literature. This condition differs from the usual imperforate anus. The diagnosis, evaluation, and therapeutic management require an understanding of the embryogenesis of the cloaca. The presence at birth of a single interlabial orifice or an abnormality sited anus is a surgical emergency. Early salvage of the kidneys and relieving intestinal obstruction are emphasized. There is a high incidence of multiple system congenital abnormalities.

Anus, Imperforate↗
PubMed1996

Caudal developmental field defect with female pseudohermaphroditism and VACTERL anomalies.

We describe an infant who died shortly after birth, with Potter sequence and prune belly anomaly, omphalocele, single umbilical artery, imperforate anus and micropenis with empty scrotum. Fetopathological examination revealed multiple vertebral anomalies with rudimentary sacrum, hypoplasia o2 the first metacarpus on right hand, complex cardiovascular anomalies, malrotation of intestines, a dilated and blind-ending cloaca to which both ureters and a bicornuate uterus were connected, normal ovaries, hypoplastic kidneys with cystic renal dysplasia. The descending colon ended blindly and showed a fistulous communication with the cloaca. Chromosome studies on peripheral blood lymphocytes and fibroblasts of a skin biopsy demonstrated a normal 46,XX karyotype. The possible mechanisms underlying the concurrence of a caudal developmental field defect with female pseudo-hermaphroditism and additional features of the VACTERL association are discussed.

Abnormalities, Multiple↗
PubMed1996

Continence and rectoanal sphincteric inhibitory reflex after posterior sagittal anorectoplasty.

Rectoanal sphincteric inhibitory reflex and continence were evaluated in 23 patients with high and intermediate imperforate anus who underwent posterior sagittal anorectoplasty from 1985 to 1993. The postoperative anorectal function was followed both manometrically and clinically at regular intervals. The presence of the reflex was positively related to postoperative continence in patients older than 4 years but not in younger patients. Two patients with a rectoprostate fistula after an internal sphincter saving procedure had unsatisfactory results. Seven of nine patients with a blind rectal pouch, whose distal rectal end (where the internal sphincter lies) was partially excised or trimmed during reconstruction, had fair postoperative defecation. The internal sphincter saving procedure is helpful but it does not seem essential for the development of continence. The fact that patients with a negative reflex turned out to have a positive reflex 7 to 33 months after the initial test suggested that the rectum or perirectal tissue had recovered from surgical trauma. Time and age are important factors in clinical evaluation and interpretation of manometric results.

Anal Canal↗
PubMed1997

[Ureteral ectopia in pediatric age: treatment and evolution in 55 cases].

In the last twenty years 55 children with ureteral ectopia were diagnosed and treated in our Hospital. Thirty seven of them were female (67.3%) and they presented the urinary tract infection like the most frequent symptomatology. Seven children were diagnosed during the first days after being born because the detection of hydronephrosis in the antenatal ultrasound; 36 were associated with complete ureteric duplication (65.4%) and the other 19 with single system (34.6%). Congenital abnormalities associated were seen in 14 patients with more frequency in those which presented single-system ectopic ureter (12/14), being the imperforate anus and the kidney agenesia those the most incidence. The most frequent points of opening were the bladder neck and posterior uretra. Diagnostic work-up included some radiological studies (abdominal ultrasound, intravenous urography, sequence mictional cystourethrography and vaginogram), isotopical tests, meticulous genital exploration, cystoscopy and vaginoscopy. 49.1% underwent conservative surgical treatment (7 ipsilateral ureteroureterostomy and 20 ureteral reimplantation) and 50.9% underwent total or partial nephroureterectomy, being the type of treatment depended on functionality of renal units. After treatment, 74.5% had not any incident; among all postsurgical complications, reflux reappearance was the most frequent (3 cases).

Anus, Imperforate↗
PubMed1998

Tracheal agenesis with multiple congenital anomalies: a case report.

Tracheal agenesis (TA) is a rare congenital anomaly that is incompatible with prolonged life. It may occur alone or with other associated anomalies. A term infant presented with cyanosis, hypotonia, absence of crying and respiratory distress at birth. Intubation was difficult. Esophageal intubation was performed under laryngoscopy. As TA was suspected, a bronchoscopy was performed and the infant was found to have a normal epiglottis and vocal cords; however, there was no trachea. Cardiorespiratory deterioration developed and the patient died on the night of the second day at the postnatal age of 41 hours. Tracheal agenesis was confirmed at autopsy. Associated anomalies included bronchoesophageal fistula, double outlet of the right ventricle with ventricular septal defect, bicuspid pulmonary valve, single lobe of the right lung, imperforate anus and a rectourethral fistula. According to development theory, tracheal agenesis and VACTERL (vertebral defects, anal atresia, cardiovascular defects, tracheoesophageal fistula, radial dysplasia or renal defects and limb defects association may result from a mesodermal deficiency caused by abnormal blastogenesis.

Abnormalities, Multiple↗
PubMedDate not supplied

Colostomy--the Port Harcourt experience.

Over the 7 years period from January 1986 to December 1992, 36 colostomies were performed at the University of Port Harcourt Teaching Hospital (UPTH). The indications for the procedure were classified under congenital, traumatic, neoplastic and miscellaneous groups. Imperforate anus and Hirschsprungs disease, under the congenital group, formed the commonest indication (19 cases); traumatic conditions followed with 9 cases, carcinoma of the rectum comprised 5 cases while there were 3 cases in the miscellaneous group--made up of 2 sigmoid volvulus and one of stercoral perforation of the sigmoid colon. 27 of the colostomies were loop transverse, 6 absolute defunctioning transverse and 3 terminal Permanent). The peculiar problems encountered with this procedure are also highlighted.

Adolescent↗
PubMed1998

Cryptorchidism as a caudal developmental field defect. A new description of cryptorchidism associated with malformations and dysplasias of the kidneys, the ureters and the spine from T10 to S5.

Cryptorchidism is a feature of abnormalities in the hypothalamo-pituitary-testicular axis, and almost all disorders of sexual differentiation in which a testis is present. We found cryptorchidism to be associated with malformations and dysplasias of the kidneys, the ureters and the spine from T10 to S5. The description of this association is new. The association was seen in 18% of cryptorchid boys younger than 3 years of age in a department of paediatric surgery, in 34% of cryptorchid foetuses who died in the third trimester, in 65% of cryptorchid patients with imperforate anus, and in all individuals with tritonmelia, the male variant of sirenomelia. Sirenomelia/tritonmelia is an extreme degree of abnormal differentiation of the caudal developmental field, also called caudal dysplasia, the caudal regression syndrome and the caudal regression malformation sequence. Caudal developmental field defects were also the predominant abnormalities in the other groups of patients. Thus, cryptorchidism may be a feature of abnormal differentiation of the caudal developmental field. Position and histology of the undescended testes of the patients included in the association were similar to in cryptorchidism in general. In the literature the association was reported in 5-10% of boys considered to suffer from cryptorchidism only. Furthermore, our observations are concordant with recent theories about cryptorchidism. Consequently, we propose that cryptorchidism in general may be a caudal developmental field defect. Study of cryptorchid patients exhibiting malformations or dysplasias of the kidneys, the ureters or the spine from T10 to S5 is essential in order to isolate new genetic disorders and to spot environmental factors causing cryptorchidism.

Animals↗
PubMed1989

Anorectal malformations and Down syndrome.

During 1980 to 1986, 89 children with Down syndrome and 42 with imperforate anus were diagnosed among 64,870 liveborn infants in the Jewish population of Jerusalem. Two of the children had both Down syndrome and imperforate anus. This indicates a high incidence of imperforate anus among children with Down syndrome (2.2%).

Abnormalities, Multiple↗
PubMed2001

Exstrophy of the cloaca and exstrophy of the bladder: two different expressions of a primary developmental field defect.

Exstrophy of the bladder (EB) and exstrophy of the cloaca (EC) are generally recognizable as distinct clinical entities. In patients with EB, the posterior bladder wall is exposed through a midline defect of the abdomen. The umbilicus is inferiorly displaced and located close to the superior margin of the exstrophic bladder. Genital abnormalities are common in boys and girls who may present epispadias and a small, split phallus or a split clitoris, a bifid uterus, and a duplicate or exstrophic vagina. In contrast to classic EB, EC is commonly associated with omphalocele, spinal defects, and incompletely formed external genitalia and is always associated with imperforate anus. Some authors state that EC and EB constitute two distinct disorders, but others consider them part of a "continuum," representing different levels of severity within the same spectrum. The use of the acronym OEIS to refer to the combination of omphalocele, exstrophy, imperforate anus, and spinal defects, in our opinion, has not helped to clarify the clinical definition, pathogenesis, or cause of this multiple congenital anomaly (MCA) pattern, mostly because the term makes no distinction between EC or EB. Here we present the epidemiological analysis of a group of characteristics in infants with EC and infants with EB to determine if they constitute two different entities. We also analyze if the different combinations of omphalocele, imperforate anus, and spinal defects are more frequent in infants with EC than in infants with MCA patterns other than EC and EB. The prevalence in our data for EC was 1:200,233 live births and 1:35,597 for EB. The clinical analysis indicated that the study defects (omphalocele, spine defects, spina bifida, and imperforate anus) tend to occur together in the same child with a higher frequency if the child has the EC defect than in infants with MCA patterns that did not include EC or EB. Our findings of low birth weight, twinning, single umbilical artery, and preferentially associated malformations suggest that EC is the result of damage occurring very early in development and that EC and EB are two different expressions of a primary polytopic developmental field defect.

Abnormalities, Multiple↗
PubMed1998

Anorectal malformations and Down's syndrome.

BACKGROUND/PURPOSE: Down's syndrome is a common association in patients with anorectal malformations. The purpose of this study was to determine whether the anorectal defect in patients with Down's syndrome had specific characteristics and whether the presence of Down's syndrome represented a serious detriment to the patient's functional prognosis. METHODS: Nine hundred eighty-seven patients with anorectal malformations were studied retrospectively. Twenty patients (2%) had Down's syndrome. Nineteen of these (95%) had the same specific type of anorectal defect: imperforate anus with no fistula. This defect has a good prognosis, the rectum is located about 2 cm above the perineal skin, the sacrum is normal, and the sphincter mechanism is good. For comparison, a group of 34 patients with the same defect but without Down's syndrome was also studied. All patients were operated on via posterior sagittal approach by the same surgeon. RESULTS: Imperforate anus without fistula occurs in 5% of all patients with anorectal malformations and in 95% of those patients who also suffer from Down's syndrome. The characteristics of the defect were the same in both groups of patients, and surprisingly, the prognosis was good in both groups (80% to 96% of patients had voluntary bowel movement, 100% had urinary continence). CONCLUSIONS: The association of Down's syndrome with imperforate anus without fistula is not coincidental. This particular benign defect can be predicted to occur in most patients with Down's syndrome. The presence of Down's syndrome in cases of anorectal malformations should not be a contraindication to repairing the imperforate anus and to closing the colostomy.

Anus, Imperforate↗
PubMed2004

Neurovesical dysfunction in children after surgery for high or intermediate anorectal malformations.

AIM: To evaluate problems with bladder dysfunction in patients operated on for imperforate anus. METHODS: All patients (11M, 25F) with high or intermediate imperforate anus seen in this institution between 1987 and 1997 were subjected to the investigation. They had all undergone a posterior sagittal anorectoplasty procedure with the aim of preserving as much as possible of the fistula. Hospital charts were reviewed. A detailed and structured interview regarding micturition habits was performed. If the results of this were abnormal the patients were also subjected to flow registration, measurements of residual urinary volume and in some cases also cystometry. RESULTS: Micturition habits were normal in 30 out of 36 children. The remaining six, considered to have neurovesical dysfunction (NVD), had difficulties in emptying their bladder and exhibited pathological residual volumes. Two were boys with rectourethral fistulae, two were girls with cloacal malformations and two were girls with rectovestibular fistulae. Four out of six children with NVD had sacral anomalies. CONCLUSION: NVD may occur even in the absence of sacral anomalies. A 4 h micturition observation is recommended in all newborns with intermediate or high anorectal anomalies to recognize the occurrence of NVD at an early stage.

Anus, Imperforate↗