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Results for “ANUS, IMPERFORATE”

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At least 595 records · Page 33Linked to original sources
PubMed2001

Johanson-Blizzard syndrome. a new case with autopsy findings.

Here we present a new case of Johanson-Blizzard Syndrome. Clinical features consistent with the diagnosis of Johanson-Blizzard Syndrome in a term neonate are described: intra-uterine growth retardation, aplasia of the nasal alae, midline scalp defect, total situs inversus, imperforate anus, malrotation of the small intestine, pancreatic insufficiency, deafness, and lethal congenital heart defects with dextrocardia. These features were confirmed by findings at autopsy. Parents were consanguineous. We compare these clinical features and findings at autopsy with previous cases reported in the literature.

Abnormalities, Multiple↗
PubMedDate not supplied

Sacrococcygeal teratoma with anorectal malformation.

A 7-month-old child presented with imperforate anus, penoscrotal hypospadias and transposition, and a midline mucosa-lined perineal mass. At surgery the mass was found to be supplied by the median sacral artery. It was excised and the anorectal malformation was repaired by posterior sagittal anorectoplasty. Histologically the mass revealed well-differentiated colonic tissue. The final diagnosis was well-differentiated sacrococcygeal teratoma in association with anorectal malformation.

Abnormalities, Multiple↗
PubMed1958

Emergency operations in the newborn.

With the present-day development and understanding of anesthetic methods, fluid and electrolyte therapy, antibiotic medications and pediatric care, many congenital anomalies once uniformly fatal are now being successfully treated by emergency operations in the neonatal period. The eight most common of these which demand emergency operation in the immediate postnatal period are esophageal atresia and tracheoesophageal fistula, diaphragmatic hernia with dislocation of the abdominal viscera into the chest, malrotation of the intestine with obstruction, intestinal atresia, meconium ileus, imperforate anus, omphalocele and myelomeningocele. Although infants born with any of these serious problems often are born prematurely and often have more than one congenital anomaly, survival rates in the surgical treatment of these conditions are steadily improving. Early diagnosis and prompt treatment are the most important factors in the continued improvement of these survival rates.

Anus, Imperforate↗
PubMed1992

Experimentally induced axial dysraphism and anorectal malformation in male rat fetuses by intragastric administration of ethylenethiourea.

Pregnant Sprague Dawley rats were separated into two groups. In the experimental group, single intragastric doses of ethylenethiourea (ETU) were given on the 11th day of gestation. In the control group, a single intragastric dose of distilled water was given on the same day of gestation. Fetuses were recovered on day 20 of gestation, and were prepared for dissection and light microscopy. A high incidence of axial dysraphic disorders (omphalocele and rachischisis) and imperforate anus with recto-urethral fistula were noted in experimental male rat fetuses obtained from dams subjected to varying doses of ETU.

Abnormalities, Drug-Induced↗
PubMed1992

[Intestinal implantation of an artificial sphincter].

In order to restore anal sphincteric function, artificial AMS sphincter was used with his 3 components: the peri-intestinal cuff giving anal sphincter tone, the pump used for active opening and the pressure regulating balloon. In the same fashion a continent valve may be created on an intestinal segment as a part of a reconstructed bladder. In this case the pump may be replace by a subcutaneous port allowing fluid injection to adjust volume and pressure in the cuff. Two patients with fecal incontinence related to sequela of a high imperforate anus syndrome were implanted. Follow-up is greater than 1 year with normal continence in the day for the first case (degree 2) and continence day and night in the second case. Artificial valve has been implanted in seven cases of neobladder pouch (or related situations), with in 4 cases very good results and in 3 cases a failure (Follow-up 3-36 months).

Adult↗
PubMedDate not supplied

[A rare variant of neuroenteric cyst: split notochord syndrome].

OBJECTIVE: We present a case of split notochord syndrome, an extremely rare form of spinal dysraphism. DESCRIPTION: We treated a 2 month-old boy presenting with an extensive lumbosacral deformity, hydrocephalus and apparent enteric segments in the dorsal midline, accompanied by an enteric fistula and imperforated anus. The malformation was diagnosed as split notochord syndrome. The baby died as a result of sepsis before surgical treatment could be attempted. COMMENTS: Split notochord syndrome is the rarest form of neuroenteric cyst described until this moment (< 25 cases in the literature). It is frequently associated with anorectal malformation, intestinal fistulae and hydrocephalus. Prognosis is not necessarily poor and survival is possible if digestive malformations, hydrocephalus and the dysraphism itself are treated simultaneously.

Abnormalities, Multiple↗
PubMed1992

Transvesical cesarean following bowel and urinary tract reconstructive surgery.

Intentional transvesical cesarean was done for delivery in a woman born with an imperforate anus, ectopic intravaginal urethra, and vaginal and urethral strictures. She had undergone multiple reconstructive procedures that left her bladder completely covering the anterior uterine surface. The rest of the uterus, including the fundus and the broad ligaments, was obscured by multiple bowel adhesions. Cesarean delivery was necessary because of pelvic bone and soft-tissue deformity. Anterior and posterior vertical cystotomies resulted in exposure of the anterior uterine wall, and the fetus was delivered through a vertical uterine incision. The woman recovered and 6 months later has no genitourinary complaints.

Abnormalities, Multiple↗
PubMed1991

[Vesico-intestinal fissure: a multidisciplinary problem].

Vesico-intestinal fissure or exstrophy of the cloaca is a rare but serious birth defect of the urogenital tract and distal part of the digestive tract. The most important hallmarks are: bladder exstrophy, fusion between bladder and the exstrophied iliocoecal region, short blind-ending colon and imperforate anus. In addition the lower part of the body shows defects of the abdominal wall, symphysis, upper urinary tract, the internal and external genitals, lumbosacral spine, spinal cord and the lower extremities. It is just after 1960 that corrective surgery started to become successful. Because of the complexity of the anomaly it seems that only management by a team of specialists in a centre for pediatric surgery guarantees optimal treatment. The quality of live of these children can still be improved by application of the newest surgical techniques and excellent cooperation between the different involved specialists. The defects, surgical treatment and results in eight patients with vesico-intestinal fissure, seen at the Universitair Centrum voor Chirurgie bij Kinderen Nijmegen (UCCKN) from 1974 till 1989 inclusive, are presented.

Abnormalities, Multiple↗
PubMed1990

[A new clinical evaluation scale for incontinence: its use in anorectal atresias].

We present a clinical scoring system to obtain a quantitative and accurate assessment of anorectal incontinence. We evaluate ten components, each of one is pointed between 0 and two, then overall incontinence punctuation (I.P.) is between 0 and 20. Variables define clinical data relative to patient's intestinal habits and hygienic methods, voluntary control degree of defecation and repercussion of patient's incontinence in social and physical capabilities. Punctuation lower than nine concerns to a poor continence; It is fair between nine and 14 points and 15 or higher punctuation indicates a good continence mechanism. This incontinence punctuation method (I.P.) has been applied to 23 patients, aged between two and 17 years, operated because high imperforate anus by PSARP procedure in 15 cases and by abdomino-perineal pull-through according to Romualdi-Soave technique in eight cases. We found statistic significative difference between the group of patients aged less than 5 years and the group aged between 5 and 10 years. There is not statistic significative differences between the two procedures used in surgical treatment, although I.P. is slightly higher in cases operated by PSARP procedure than in cases treated with Romualdi-Soave technique, when we compare the two groups with similar mean age.

Adolescent↗
PubMed1990

Male pseudohermaphroditism with persistent müllerian structures, mental retardation and Borjeson-Forssman-Lehmann-like features: a new syndrome?

We report two sibs with an undescribed MCA/MR syndrome. Both had a 46,XY chromosome constitution. The first patient is profoundly mentally retarded. Clinical features include short stature, coarse face, deep set eyes, microphthalmia, large ears, gynecoid obesity, imperforate anus, sacral spina bifida, pseudovaginal perineoscrotal hypospadias, persistence of Müllerian structures, and low gonadotrophin levels. His XY sib was raised as a girl. She was slightly mentally impaired. She had microphthalmia and large ears, and was short. A complete uterus with tubae and a single intraabdominal gonad with testicular organization were removed during infancy. Those anomalies do not fit any previously reported syndrome, although the general aspect of the propositus clearly resembles Borjeson-Forssman-Lehmann syndrome. Inheritance could be either autosomal recessive or X-linked.

Abnormalities, Multiple↗
PubMed1989

Neonatal intestinal obstruction.

Our experience in the management of 138 infants with various causes of intestinal obstruction has provided us with important principles of diagnosis and treatments that we adhere to in the course of our practice. 1. The overall mortality of neonates suffering from intestinal obstruction should be fewer than 5 per cent. The high survival rate is accounted for by improved care these patients receive in specialized units. 2. Prematurity did not appear to play a significant factor in the outcome of these infants with intestinal obstruction. Our experience shows that premature infants tolerate operative procedures well, even in those instances in which an associated surgically correctable lesion is simultaneously repaired. 3. The major risk factor in any neonate with intestinal obstruction is the delay in diagnosis and operative intervention, especially in infants diagnosed to have midgut volvulus. The additional second risk factor is the association of chromosome abnormality. 4. Traditional diagnostic studies such as plain films of the abdomen supplemented by either an upper GI or lower GI contrast study for specific indications have been very effective in obtaining an accurate diagnosis of intestinal bowel obstruction. 5. Hirschsprung's disease can be diagnosed in the neonatal period if the index of suspicion for this is high. 6. Special surgical techniques as described should be used whenever indicated to minimize morbidity. 7. The traditional Wangensteen-Rice evaluation of a patient with imperforate anus is accurate, and specialized studies should be deferred for the postoperative period. Collaborative care provided by the neonatologist, pediatric anesthesiologist, and pediatric surgeon for these patients is the key to a favorable outcome.

Abnormalities, Multiple↗
PubMedDate not supplied

Computed tomography of ano-rectal anomalies. Correlation between radiologic findings and clinical evaluation of faecal incontinence.

Computed tomography (CT) was performed in 9 patients with faecal incontinence more than 8 years after rectoplasty for high imperforate anus. The radiologic findings were correlated with the clinical evaluation of faecal incontinence. No statistically significant correlation was found between function and anatomic findings as demonstrated by CT. Thus, although CT demonstrates the post-operative anatomy in a detailed way, the information does not seem to be directly applicable when selecting the type of reoperation for faecal incontinence.

Anal Canal↗
PubMedDate not supplied

The value of physiotherapy for faecal continence after correction of high anal atresia. A clinical and electromyographic study.

A follow-up study of 12 patients who had undergone Stephens rectoplasty for high imperforate anus is presented. Clinical assessment and anal electromyography were performed more than 7 years after surgery, both before and after a year's physiotherapeutic training of the perianal musculature. Electromyography showed correlation between faecal continence and tonus of the anal sphincter, the anocutaneous reflex and maximal voluntary activity in the perianal muscles. After the physiotherapeutic training there was significant improvement of faecal continence. This effect was electromyographically corroborated. The results confirmed clinical observations that postoperative physiotherapy and training of the voluntary muscles in the perianal region is of value for the achievement of faecal continence.

Adolescent↗
PubMed1986

[Sonographic diagnosis of the gastrointestinal tract].

Ultrasound examination is an established screening procedure for illdefined abdominal complaints and is complementary to the abdominal plain film. The bull's eye phenomenon due to bowel wall thickening is the characteristic feature of bowel pathology. Ultrasound is the method of proof for hypertrophic pyloric stenosis, imperforate anus or ascites. Findings of edematous (intussusception), inflammatory (Crohn disease) and neoplastic stomach or bowel disease require confirmation by radiology or endoscopy. Ultrasound of the gastrointestinal tract allows the selection of further diagnostic procedures and thus accelerates the diagnostic work-up.

Anus, Imperforate↗
PubMed1986

Sacral hemangiomas and multiple congenital abnormalities.

Five infants with sacral hemangiomas and a particular constellation of congenital abnormalities are described. Three of the five infants had an imperforate anus associated with a fistula. Three of the five had renal anomalies; four had bony abnormalities of the sacrum, and three of these also had a lipomeningomyelocele. Four had skin tags, three of which were in the genital and sacral areas. Two of the five also had abnormalities of the external genitalia. This constellation of defects associated with a sacral hemangioma has not been, to our knowledge, elaborated previously.

Abnormalities, Multiple↗
PubMed1986

Cutis marmorata telangiectatica congenita with multiple congenital anomalies.

Cutis marmorata telangiectatica congenita (CMTC) is a rare cutaneous vascular anomaly. Congenital persistent livedo reticularis, telangiectases, and superficial ulceration are the hallmarks of this syndrome. There is an association of CMTC with other congenital abnormalities in at least 50% of the patients. A newborn girl is described with a diagnosis of CMTC combined with multiple congenital abnormalities including imperforate anus, rectovaginal and urethrovaginal fistulas, and absent clitoris. These abnormalities have not been previously reported with CMTC. Congenital abnormalities that have been recognized with this syndrome are reviewed.

Abnormalities, Multiple↗
PubMed1977

Secondary effects of aganglionosis in the piebald-lethal mouse model of Hirschsprung's disease.

An attempt was made to identify the factors secondary to fecal stasis and megacolon that are related to overall debilitation and eventual death of the piebald mouse. The piebald mouse showed periodic leukocytosis and bacteremia that were associated with lesions in the mucosal surface of the megacolon. Defective growth, loss of weight, and retardation of development with megacolon were documented by systematic study of body weight and of the relationship between body weight and organ weights. Alterations in body weight and in organ weight to body weight ratios were parallel in the piebald mouse, in mice with imperforate anus, and in mice with surgically-induced megacolon. Histologic studies confirmed a pattern of hypertrophy of the muscularis externa in the piebald mouse. The body temperature was lower than for normal siblings, but the sodium, potassium, and water content of the feces, the general blood chemistry profiles, and the intestinal microflora did not differ from those of the normal mouse. There was no evidence of bacterial overgrowth in the small intestine of the piebald mouse with fecal stasis and megacolon.

Animals↗