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Ten boys with a fistula between the rectum and the urinary tract are presented. Six resulted from complications of imperforate anus surgery, three were various congenital abnormalities, and one was associated with an inflammatory pelvic mass. The management of these patients is described and the problems are discussed.
Bladder duplication is a rare anomaly. Less than 100 cases of all types have been described in the literature. When duplication of the bladder occurs, it has previously been described as a mirror image or as a septate bladder. A newborn female presented with what appeared to be a complete bladder exstrophy, a large ruptured omphalocele with perforated large bowel, an imperforate anus, and a bifid clitoris. Urine was seen emanating from various sites: the bladder mucosa, the mucous fistula constructed at the time of the colostomy, and the left hemiclitoris. The anatomic puzzle was resolved when multiple studies disclosed the problem to be a bladder duplication. The bladder on the anterior abdominal wall was exstrophied, the intraabdominal bladder was a cloaca, and the left hemiclitoris contained a phallic urethra, which drained the intraabdominal bladder. Total correction with normal function was obtained. This patient demonstrates that bladder duplication may present in various ways.
A complete staged reconstruction has been performed on a 4-year-old girl with imperforate anus, rectovestibular fistula, and colonic atresia. The rectal remnant was a small 10 cm long segment, widely separated from the right colon. An end colostomy had been performed in the neonatal period elsewhere. Associated urinary anomalies were treated first. At the age of 4 years, distal colon remnant dilatations were started using a balloon catheter through the vestibular orifice. Four months later, end-to-end ascending colon-sigmoid anastomosis was done, after colonic mobilization and tapering. A temporary loop ileostomy was left at the left upper quadrant. Three months later a posterior sagittal anorectoplasty was successfully performed. At the age of 5 years she has achieved fecal continence, after the ileostomy closure.
A retrospective clinical study was conducted to determine the success of a strict regimen employing the prolonged use of daily enemas in 203 children with chronic constipation. This study confirmed that the use of long-term daily enemas did eliminate constipation as well as the primary complaint of fecal soiling. The majority of children treated had an excellent to good result (85.8%) over an extended period of time. Patients with a past medical history of imperforate anus or Hirschsprung's disease required longer treatment periods (32.6 and 20.1 months, respectively) than children with other medical problems (rectal prolapse, rectal stricture, malrotation, spina bifida, mental retardation, psychological; 13 months) or patients with functional constipation (5.9 months).
Poor postoperative continence in patients operated for high imperforate anus may result from an inadequate bowel pull-through behind the puborectal muscle. Between 1979 and 1983, we used the anterior perineal approach for reoperations in five such cases (3 males, 2 females). In all of them, preoperative continence was socially unacceptable. Each time it was possible to identify a partially or totally missed puborectal muscle. Although some complications occurred postoperatively, all patients acquired a socially acceptable level of continence with a mean follow-up of 4.5 years. Preoperative and postoperative rectal manometries are presented. The anterior perineal approach not only provides adequate exposure of the urethra and puborectal muscle, but also has the advantage of operating through an area that has not been previously dissected.
The early recognition and treatment of correctable lesions of the terminal spinal cord in patients with anorectal malformations may preserve important neurologic function. Tethered cord and intraspinal masses are detectable with the use of high-resolution ultrasonography in the neonate. Fourteen infants and children with anorectal malformations and associated spinal dysraphism have been identified in our institution over the past 7 years. Six patients had cloacal exstrophy, and eight had imperforate anus (four high and four low lesions). The spinal lesions caused symptoms in only seven children; progressive neurologic deficit in five, and urinary incontinence or retention in two others. Five asymptomatic patients with cutaneous abnormalities on the back were studied and two were discovered during scoliosis evaluation. Imaging techniques included high-resolution ultrasonography, computed tomography with and without metrizamide myelography, and magnetic resonance imaging. Spinal sonography was highly accurate in the neonatal period. The application of ultrasonography can be of great advantage in early screening of patients with anorectal malformations and, in some cases, may eliminate the need for invasive imaging techniques.
Eighteen girls with major uterovaginal malformations were admitted to the Pediatric Surgical Service over a 17-year period. The diagnosis was not suspected or delayed in more than one half of the patients. The encountered anomalies were divided into four groups: I, isolated uterovaginal malformations (UVM) (4); II, UVM with anorectal anomalies (8); III, UVM with cloacal (urogenital sinus) abnormalities (5); and IV, caudal twinning (1). Imperforate hymen, vaginal web, low vaginal obstruction, or disorders of gonadal or chromosomal development were excluded. Patients presented with an abdominal mass or distension (5), abdominal pain (4), "sciatic"-like pain (1), purulent vaginal discharge with perineal pain (1), amenorrhea (2), and a pelvic and prerectal mass (1). The introitus was reported as normal in 11 patients with vaginal atresia or agenesis by the primary physician. Diagnostic studies, in addition to clinical and endoscopic examination, included routine radiological workup, genitourinary contrast studies, pelvic and perineal sonography, computerized tomography (CT) scanning, and more recently, magnetic resonance imaging. In complicated UVM, especially vaginal duplications with unilateral atresia, the CT scan was the most helpful diagnostic tool. Laparotomy was necessary, not only for therapeutic, but diagnostic reasons; even so, complex anomalies, such as vaginal duplication with unilateral atresia and a septate uterus, could not be suspected. Treatment was directed toward the restoration of a functional uterovaginal tract and the frequently associated anorectal anomalies. A review indicated that contrary to our expectations, the major UVM occurred in children with a low imperforate anus rather than the high variety.
From 1985 to 1994, 27 patients with high- or intermediate type imperforate anus who underwent anorectoplasty were studied for postoperative function, particularly with respect to the rectoanal relaxation reflex and continence. Fourteen of the patients had a rectourogenital fistula and were treated with posterior sagittal anorectoplasty using the fistular end as the neoanus (internal sphincter-saving). Nine patients had a blind rectal pouch and received posterior sagittal anorectoplasty using the trimmed bowel end for reconstruction of the neoanus (incomplete internal sphincter-saving). The other four had Rehbein's mucosa-stripping endorectal pull-through combined with anterior sagittal anorectoplasty (none internal sphincter-saving). A positive rectoanal relaxation reflex was found in 8 of 14 (57.1%), 7 of 9 (77.8%), and 3 of 4 (75%), respectively. It appears that the internal sphincter-saving procedure is not essential for the development of the rectoanal relaxation reflex. Compensation or adaptation most likely contributes to the presence of the rectoanal relaxation reflex, and perhaps to postoperative continence.
Currarino et al, in 1981, described an association of a congenital anorectal stenosis, or another type of low anorectal malformation, an anterior sacral defect, and presacral mass. Eleven patients with this anomaly were treated at the Department of Pediatric Surgery, Seoul National University Children's Hospital from 1984 to 1995. Among these patients, low-type imperforate anus (IA) was seen in three cases and anorectal stenosis was present in eight cases. Presacral masses included seven teratomas, two meningoceles, one dermoid cyst, and one enteric cyst with dermoid cyst. All had a deformed sacrum. Among the eight with anorectal stenoses, posterior sagittal anorectoplasty (PSARP) with diverting colostomy was performed in seven cases, and repeated rectal dilatation was performed in one case. Among the three low-type IA, anoplasty was performed in two cases and PSARP was performed in one case. Although PSARP is a safe and satisfactory method facilitating the excision of the presacral mass, in meningoceles the repair should be performed before correction of anorectal malformation because of the risk of meningitis that can occur when surgeries are done simultaneously. In two cases, untethering of a tethered spinal cord was performed. All patients are continent. Because the incidence of Currarino triad is high when there is an anorectal stenosis (38% in the present series), the Currarino triad should be suspected in anorectal stenosis. Magnetic resonance imaging is a preferred diagnostic method because the incidence of association of tethered cord in this triad is high (18% in the present series).
The authors present a case of a boy newborn who had bladder pseudoexstrophy and multiple congenital anomalies. In addition to this unusual variant of the exstrophy-epispadias complex, the patient was found to have a posterior cleft palate, an omphalocele, and an imperforate anus.
PURPOSE: The authors report the postoperative follow-up results of patients who underwent posterior rectal flap anorectoplasty (PRFA), with emphasis in fecal continence. Variables considered on each patient were sex, presence of fistula, location of the fistula, severity of the defect during the correction, sacral condition, dysraphism, and age at surgery. METHODS: Continence was rated using the Peña method and defecogram. The authors studied 20 patients (17 boys and three girls). RESULTS: Postoperatively, normal continence was found in 18 patients. Of the two remaining (boys), one had grade II spotting (sacrum agenesia and uretrobulbar fistula) and the other had grade II staining; this patient had the most severe malformation requiring an abdominoperineal approach. All of the defecograms showed complete emptying of the rectum. No recurrent fistulas occurred. CONCLUSIONS: The posterior rectal flap anorectoplasty is a new technique that takes into consideration all known factors that contribute to continence. This initial report confirms that this technique results in excellent continence for patients with imperforate anus.
BACKGROUND/PURPOSE: Fecal incontinence is common in patients operated on for anorectal malformations. Treatment with enemas, laxatives, and medications are often given by clinicians in an indiscriminate manner and without a demonstrated benefit. A systematic diagnostic approach and bowel management program was developed for patients suffering from fecal incontinence, and a retrospective analysis of the results is presented. METHODS: Three hundred forty-eight patients were seen in consultation for fecal incontinence after repair of imperforate anus at other institutions. Clinical and radiological evaluation helped determine different types of patients. Group I consisted of 147 patients who were considered candidates for reoperation and forms the basis of a future report. Group II included 172 patients who had no potential for bowel control and were therefore candidates for bowel management. These patients fell into two categories; group IIA included 44 patients with incontinence and constipation. The bowel management involved the use of daily large enemas only. Group IIB included 128 patients with incontinence and a tendency to diarrhea. Group III consisted of 29 patients who had pseudoincontinence. They had an original defect with good prognosis, good sphincters, good sacrum, and a well-located rectum. They suffered from severe constipation, megasigmoid, chronic fecal impaction, and overflow pseudoincontinence and were treated with laxatives or sigmoid resection. RESULTS: Bowel management was successful in 93% of patients in the constipation group (IIA) and 88% in the diarrhea group (IIB). Ninety-seven percent of patients in group III became fecally continent. CONCLUSION: Bowel management consisting of enemas, laxatives, and medications is successful when administered in an organized manner. It is vital to determine the type of fecal incontinence from which the patients suffer and to target their treatment accordingly.
BACKGROUND/PURPOSE: Children with anorectal malformations often have less than optimal results after repair. The authors report on five patients (ages 3 to 17 years) born with imperforate anus and treated with anoplasty as a newborn. At presentation, all patients were completely incontinent of stool. None had ever experienced voluntary bowel movements, and all wore diapers continuously. METHODS: Perineal examination with the nerve stimulator showed the muscle complex was largely intact with good contraction, but the neoanus was outside of the muscle complex. Contrast enema showed massive dilation of the rectosigmoid colon and fecal impaction. A tethered spinal cord was excluded by magnetic resonance imaging (MRI). We treated these patients with a combined reoperative anoplasty via the posterior sagittal approach and sigmoid resection. RESULTS: Within 6 months after the procedure, all patients had achieved complete continence. They had from one to three voluntary bowel movements per day without soiling. CONCLUSIONS: Children with severe constipation and fecal incontinence after anoplasty should undergo evaluation by a surgeon. If examination shows a reasonably intact muscle complex and correctable anatomic defects, an excellent result can be achieved with appropriate reoperation.
BACKGROUND/PURPOSE: Spinal dysraphism and neurovesical dysfunction (NVD) frequently are associated in children with anorectal malformations (ARM). This study compares the urodynamic data from a selected group of patients with the results of their spinal and urologic imaging studies. METHODS: Twenty-six children (20 with isolated imperforate anus and six with persistent cloacal malformations) were investigated. All patients were evaluated with leak point pressures (LPP), renal ultrasound scan, and voiding cystourethrography. Eight children had urodynamics performed before and after posterior sagittal anorectoplasty (PSARP). The spinal cord was assessed using ultrasonography or magnetic resonance imaging. Current urologic status was obtained to provide long-term follow-up. RESULTS: Twenty-one of 26 children demonstrated elevated LPPs above the established normal value of 40 cm H2O, and 15 of these children had normal spinal imaging study findings. Uroradiographic studies findings showed that 12 of 21 children with elevated LPPs had hydronephrosis or vesicoureteral reflux with seven of these patients having normal spinal cords. LPPs in the eight patients with pre- and postoperative studies were 74 +/- 14.7 cm H2O and 68 +/- 31.8 cm H2O (mean +/- SD), respectively. CONCLUSIONS: These urodynamic and radiographic data confirm that NVD (elevated LPP) is common in patients with anorectal malformations despite normal spinal cords. Bladder dysfunction does not appear to be a sequelae of a properly performed PSARP. Patients with ARM and any uroradiographic or clinical urologic abnormality should undergo urodynamic testing even though the spinal studies are normal.
We describe a 9-year-old boy and his 34-year-old father with the Pallister-Hall syndrome. The proband had precocious puberty, imperforate anus, postaxial polydactyly, hypospadias, a hypothalamic mass, and a displaced pituitary gland. The father had polydactyly, a hypothalamic mass, and a flattened pituitary gland. We conclude that the most likely cause of the Pallister-Hall syndrome is a mutation in a gene inherited in an autosomal dominant manner.
Fifty children and adolescents who had severe fecal incontinence associated with either imperforate anus surgery in infancy or longstanding functional constipation were given biofeedback training for the purpose of achieving anal sphincter control. Feedback was in the form of oscilloscope tracings which the children learned to produce by contracting small air-filled balloons positioned at the internal and external anal sphincters. Forty-seven of these patients learned to have voluntary bowel movements, and 30 eliminated soiling accidents completely during follow-up periods ranging from six months to three years.
Etretinate, a synthetic retinoid, is a potent teratogen. It has previously been shown that acute exposure of gestational day 8 (equivalent to human week 4 post-fertilization) C57BL/6J mouse embryos to this retinoid results in a spectrum of abnormalities that are recognized as constituting caudal regression (dysgenesis). These defects, which include spina bifida, imperforate anus, genitourinary anomalies, omphalocele and limb anomalies, result from a major insult to the primitive streak, that is the gastrulation process. Developmental stages present early on gestational day 9 in mice represent the final stages during which the primitive streak contributes to the trunk of the embryo and, therefore, the last opportunity for abnormalities within the realm of caudal regression to be induced. In fact, acute etretinate exposure on gestational day 9 resulted in anal and urethral atresia, bladder and ureteral dilatation, and tail deficiencies as observed in 251 near-term fetuses in this study. To examine in further detail the gestational day 9 etretinate induced urogenital and anal abnormalities and their pathogenetic basis, analyses were conducted using scanning electron microscopy, light microscopy, antegrade cystourethrograms and a vital staining technique as early as 6 hours following maternal drug administration. It appears that diminution of the caudal cell populations, including those of and those surrounding the cloaca, at this critical stage of embryogenesis accounts for the observed phenotype. We propose that anal and urethral atresia temporally represents the end of the caudal regression (dysgenesis) syndrome.
Appendicovesicostomy is a useful adjunct to continent reconstruction of the bladder. During the last 3 years we have used this procedure in 14 children with difficult incontinence due to spina bifida (7), exstrophy/epispadias (5), imperforate anus (1) and urethral agenesis (1). In 4 patients in whom previous operations had failed to achieve continence the bladder neck was closed. In the remaining 10 children, all of whom had difficulty or discomfort catheterizing the native urethra, the bladder neck/urethra was preserved or reconstructed to serve as a vent for elevated bladder pressures. Continence was achieved in all 14 children with no complications related to the appendix. All children in whom the bladder neck/urethra was salvaged preferentially empty via the appendix and remain dry, except when the bladder is overly full.