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Laparoscopically assisted anorectoplasty using perineal ultrasonographic guide: a preliminary report.

BACKGROUND/PURPOSE: As minimal invasive surgery, laparoscopically assisted anorectal pull-through has been reported with new devices. However, it is not easy to create an accurate pull-through canal (PTC) because of the narrow space between the urethra and puborectal sling. The authors describe a new method using perineal ultrasonography. METHODS: The rectourethral prostatic fistula was dissected laparoscopically. Externally, electrostimulation identified the center of the muscle contraction, over which a 1.2-cm skin incision was made, and the lower part of PTC was created by hemostat forceps guided by electrostimulation. An ultrasonographic probe applied to the perineum demonstrated the urethra, and the forceps was advanced behind the urethra into the pelvic cavity using the ultrasonographic guide. Anorectal pull-through was performed after dilatation of the PTC with dilators. RESULTS: The authors applied this procedure in 5 cases of male high and intermediate anomalies. Surgical damages to the urethra and the levator and vertical muscles were not encountered. Postoperative fluoroscopic study demonstrated good anterior angulation and intact contraction and relaxation of those muscles. CONCLUSION: The combination of laparoscopic dissection, pinpointing the center of anal sphincter by electrostimulation, and identification of the urethra by ultrasonographic images from the perineum facilitated creation of appropriate PTC in the muscle complex.

Anal Canal↗

An exceptional type of anorectal malformation: anorectal atresia with posterior fistula in a newborn male.

The authors report the case of a newborn male with an exceptional type of anorectal atresia with a fistula that passes posterior to the muscle complex and exits at a site just to the tip of the coccyx. This anorectal malformation was associated with other abnormalities including agenesis of the right kidney, pyelic dilatation of the left kidney, duplicity, and left megaureter. There was no defect in the spine. The anorectal malformation was managed successfully by a posterior sagittal approach according to the Pena procedure.

Abnormalities, Multiple↗

Shake, rattle, and roll.

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Abnormalities, Multiple↗

Reproductive and developmental toxicity screening test of basic rubber accelerator, 1,3-di-o-tolylguanidine, in rats.

Twelve male and female rats per group were exposed to the rubber accelerator 1,3-di-o-tolylguanidine (DTG) by gavage at 0, 8, 20 or 50 mg/kg bw/day. Males were dosed for a total of 49 days beginning 14 days before mating. Females were dosed for a total of 40-49 days beginning 14 days before mating to day 3 of lactation throughout the mating and gestation period. At 50 mg/kg bw/day, deaths were observed in two males and three females. Lowered body weight gain and food consumption were noted in males at 50 mg/kg bw/day and females at 20 and 50 mg/kg bw/day. Mydriasis, decreased locomotor activity, bradypnea, prone position, tremor and/or salivation were observed in males and females at 20 and 50 mg/kg bw/day. No effects of DTG were found on the estrous cyclicity, precoital interval, copulation, fertility and gestational indices, numbers of corpora lutea and implantations, or gestation length. A significant decrease in the number, body weight and viability of offspring and increase in the incidence of fetuses with external malformations were found at 50 mg/kg bw/day. Oligodactyly, anal atresia and tail anomalies were observed. These data suggest that DTG may be teratogenic. The NOAELs of DTG for general and developmental toxicity in rats are 8 and 20 mg/kg bw/day, respectively.

Abnormalities, Drug-Induced↗

Are congenital anorectal malformations more frequent in newborns conceived with assisted reproductive techniques?

One of the major concerns about ART is the risk of birth defects in children born after in vitro fertilization. We report on a cohort of consecutive children affected with anorectal malformation (ARM) requiring surgical correction in which we found a significantly high proportion (Odds ratio 13.31, 95% confidence limits 4.0-39.6) of children born after ART. Our data is in agreement with the result of a recent epidemiological study in Sweden. Further studies are necessary to define the risk and identify the causes, if any. At present, couples undergoing ART should be informed of the general risk of congenital anomalies, of which, ARM can be suggested as an example.

Abnormalities, Multiple↗

Atresia ani with diphallus and separate scrota in a calf: a case report.

Atresia ani, a common genetic defect in animals, is often accompanied by urogenital defects in calves. This paper reports a case of atresia ani with diphallus and separate scrota in a calf. The calf was born with atresia ani; surgery (to open the anus) was performed 3 days after birth. No urogenital abnormalities were noticed until 4 months after birth. At that time, two separate scrota (each containing a testis) and a sac-like structure in the middle of two scrota, were visible. The gait was abnormal, with abduction of the hind limbs while walking. Additionally, the hind legs appeared wider than usual at the hip joints. Two weeks later, two peni (diphallia) was observed, each in a separate preputial sheath. The calf had a normal karyotype on cytogenetic examination. Plasma concentrations of testosterone at 5.5, 6, and 7 months of age were 3.5, 1.9, and 1.7 ng/ml, respectively. At necropsy (7 months of age), the prepuce was thick and the glans of the right penis was adhered to the prepuce. The left penis did not have a urethra or retractor penis muscles. The sac-like structure in the middle of the two scrota contained the urinary bladder and a loop of small intestine. The pubic bone had failed to fuse at the pelvic symphysis. In conclusion, this is the first reported case of atresia ani with diphallus, separate scrota, and pubic bone separation in a calf.

Animals↗

[Hearing loss in Townes-Brocks syndrome].

In nineteen seventy two Towns and Brocks reported on a family in which the father and seven children showed anal atresia, triphalangeal thumb, metatarsal synostosis, extra fingers, neurosensorial deafness and ear abnormalities. The main traits were those related to hand, ear and anus alterations, transmitted under a dominant autosomal pattern. In this paper we present several members of the same family with similar alterations who finally were diagnosed of Townes-Brocks syndrome.

Abnormalities, Multiple↗

Congenital corneal anesthesia in children with the VACTERL association.

PURPOSE: To describe ocular manifestations in patients with the VACTERL (vertebral, anal, cardiovascular, tracheoesophageal, renal, and limb defects) association. METHOD: We reviewed the medical records of patients with the VACTERL association who had undergone treatment for amblyopia at the orthoptic department. RESULTS: A striking similarity was found in three unrelated children, who, in addition to clinical manifestations of VACTERL association, showed total corneal anesthesia of one or both eyes that had resulted in recurrent corneal erosions, keratitis, and permanent corneal opacities. Other ocular manifestations included nonparalytic strabismus, anisometropia, and amblyopia. CONCLUSION: Congenital corneal anesthesia may be part of the VACTERL association.

Abnormalities, Multiple↗

Advances in the management of anorectal malformations.

BACKGROUND: Anorectal malformations represent a continuing challenge for the pediatric surgeon. A significant number of these children suffer from fecal incontinence, urinary incontinence, and sexual inadequacy. However, major advances in the management of these children have occurred during the last 10 years. The posterior sagittal approach has led to a better understanding of the internal anatomy of these defects, and in turn, a more rational way to manage the patients. We present here our experience with the long-term follow-up of a large series of these patients operated on by us. METHODS: Clinical information from our database of patients with anorectal malformations was retrospectively reviewed. A total of 1, 192 patients operated on by the authors and observed for the last 19 years are included in the database. We searched for type of malformation, associated defects, results in terms of voluntary bowel movements, soiling, constipation, and urinary control. RESULTS: Globally, 75% of all patients have voluntary bowel movements. Half of this group still soils their underwear occasionally. Therefore, about 37.5% of all cases are considered totally continent. Constipation was the most common sequelae. Urinary incontinence was relatively common after the repair of cloacas. Male patients rarely suffered from urinary incontinence. Twenty-five percent of all cases suffered from fecal incontinence but they significantly improved their quality of life when subjected to a bowel management program. An operation called "continent appendicostomy" further improved their quality of life. CONCLUSION: All patients born with anorectal malformations can be kept clean of stool and dry of urine, either because they achieve bowel control or because they are subjected to a bowel management program.

Age Factors↗

Case report: the antenatal ultrasound diagnosis of megalourethra.

The antenatal ultrasound findings in a fetus which postnatally had fusiform megalourethra, rectal agenesis, recto-vesical fistula and unilateral renal hypoplasia are described. At 16 weeks gestation a cystic area was seen arising from the perineum. It later became evident that this was the urethra. Initially, an echogenic mass was noted in the pelvis, but by the second trimester, fluid-filled loops of bowel were evident. This combination of developmental abnormalities though rare is well defined. Only one previously recorded case has been suspected antenatally.

Anus, Imperforate↗

Multiple congenital malformation in a Holstein calf.

A 10-day-old male Holstein dairy calf with orthopaedic abnormalities was unable to stand but was alert with a suckle reflex. At necropsy, the calf showed multiple defects, including partial agenesis of the left rib plate, deformed left scapula, shortened left humerus, agenesis of the left kidney, atresia ani and scoliosis. The cause of these anomalies could not be determined. This report is the first to describe partial agenesis of ribs in a calf.

Abnormalities, Multiple↗

Home bowel preparation for elective colonic procedures in children: cost savings with quality assurance and improvement.

BACKGROUND/PURPOSE: The current health care environment pressures providers to lower cost and demands quality care that is measured by outcomes and patient satisfaction. Most insurers will not approve bed days for in-hospital preoperative bowel preparations for elective colorectal procedures. This policy does not take into account that infants and children are unable to tolerate large volumes of enteral preparation, which adversely affects outcome because of an inadequate preparation. This report describes a prospective evaluation of a standard home bowel preparation regimen utilizing local and regional home health care agency support. METHODS: For an elective colorectal procedure, pediatric patients underwent a home bowel preparation using GoLYTELY (100 mL/kg) via a nasogastric tube infused over 4 hours by a pediatric home health nurse trained in this technique. During the bowel preparation, the nurse educated the family members about the service and performed physiological monitoring to insure safety. At the completion of the preparation, any unusual events were transmitted to the staff surgeon for further instructions. Our initial 30 patients were treated by our hospital home health agency personnel to insure safety. Since then, 41 additional bowel preparations have been performed by statewide agencies. RESULTS: Seventy-one patients underwent complete home bowel preparation (45 boys; 26 girls). The age range was 3 months to 9 years (average, 5 months). There was one complication caused by incorrect mixing of GoLYTELY causing gastrointestinal cramping. All 71 home bowel preparations were recorded as good at the time of the colorectal procedure by the staff pediatric surgeon. The average cost for home bowel preparation was $300 in network, and $350 out of network. This compares with an inpatient hospital day cost of greater than $800 ($36,000 savings). CONCLUSIONS: This technique offers the pediatric surgeon an opportunity to maintain a high standard of quality care while using home health agency personnel to minimize cost. This program is safe, effective, and associated with a good outcome and a high degree of family satisfaction.

Anus, Imperforate↗

Rectal manometry, computed tomography, and functional results of anal atresia surgery.

Children with anal atresia often have compromised function. This is especially common with the anatomic "high lesion." Twenty-five patients have been studied using clinical evaluation, computed tomography (CT), and manometry to make an objective evaluation. The patients were an average of 9.6 years old; the male-to-female ratio was 3:2. Thirty-three percent had acceptable bowel function as defined by a continence score of 1 or 2. Only two (11%) had an abnormal sacrum. The mean rectal pressure was 20 cm H2O and 5 patients (23%) had a normal rectal relaxation reflex. Thirty percent had CT scans that demonstrated an intact external sphincter and puborectalis. In comparing the objective criteria and clinical results no technique could predict continence. CT demonstrated no significant group (P = .046 to .659) and manometry demonstrated no significant group (P = .082 to .752). We found no objective criteria that could evaluate the patient's clinical result or dictate therapy.

Adolescent↗