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Post-operative magnetic resonance evaluation of children after laparoscopic anorectoplasty for imperforate anus.

BACKGROUND AND AIMS: Laparoscopic anorectoplasty (LAR) is a relatively new procedure in the treatment of imperforate anus. Using magnetic resonance imaging (MRI), we evaluated the anatomical features of the anorectal region of children treated with LAR and compared this with conventional posterior sagittal anorectoplasty (PSARP). The findings were correlated with functional outcome. PATIENT/METHODS: A retrospective review of ten children with the high/intermediate types of imperforate anus underwent LAR between May 2000 and December 2002. MRI of the pelvis was performed post-operatively and a semi-quantitative score was used to assess the degree of sphincter symmetry, peri-rectal fibrosis, and the position of the pull-through rectum. The defecation status of these patients was also recorded. Eight historical patients who had undergone PSARP served as a control group. RESULTS/FINDINGS: When compared with PSARP patients, a significantly lower proportion of LAR patients had sphincter asymmetry (40 vs. 100%, p < 0.05) and peri-rectal fibrosis (40 vs. 87.5%, p < 0.05). The positioning of the rectum was, however, central for both groups (90 vs. 87.5%). No statistical correlation was found between defecation status and the degree of sphincter asymmetry or peri-rectal fibrosis. INTERPRETATION/CONCLUSION: LAR allows more optimal anatomical reconstruction for patients with the high/intermediate types of imperforate anus. However, additional factors that are not correctable by surgery, such as intrinsic innervation deficiency, also influence the clinical outcome.

Anal Canal↗

Findings of pelvic musculature and efficacy of laparoscopic muscle stimulator in laparoscopy-assisted anorectal pull-through for high imperforate anus.

BACKGROUND: Laparoscopic findings of levator muscle and the efficacy of laparoscopic muscle stimulator (LMS) in infants with high imperforate anus have not been reported. METHODS: Twelve patients underwent laparoscopically assisted anorectoplasty for high imperforate anus. Following laparoscopic dissection of the distal rectum and division of the fistula, levator muscles in the pelvic floor were stimulated with a 5-mm-diameter LMS. Dilatation was done by inserting a guidewire and balloon catheter through the center of the levator muscle sling and muscle complex. Rectal pull-through and anastomosis between the rectum and anus were successfully completed. RESULTS: LMS showed good contraction of levator muscles and enhanced accurate midline placement of pull-through rectum. LMS was particularly useful in observing weak muscles in infants with rectovesical fistula. CONCLUSIONS: Laparoscopy and LMS offer excellent visualization of the pelvic musculature and precise tract of rectal pull-through. Fecal continence will be assessed by long-term follow-up.

Anal Canal↗

A new ultimate anus-preserving operation for extremely low rectal cancer and for anal canal cancer.

To avoid permanent colostomy, we perform a new ultimate anus preserving operation for extremely low rectal cancer or for anal canal cancer. According to our pathologic study, two different removal methods of anal canal were theoretically considered. One is internal sphincter resection (ISR method), and the other is both deep-superficial external sphincter and internal sphincter resection (ESR method). Six patients received ISR and ten patients ESR. No severe intraoperative complications occurred and the postoperative course was uneventful. All patients receiving ISR had excellent anal function without soiling. Some patients receiving ESR sometimes complained of night soiling but satisfied the anus preservation. The median follow-up was 15 months, (range, 3-28 months). We had recurrences in two female patients receiving ISR. One had para-aortic and lateral lymph node recurrences without anastomotic recurrence. She underwent lateral and para-aortic lymphadenectomy, but died of lung metastasis, regardless of intensive chemotherapy. Another had pelvic recurrence with abdominal dissemination. She underwent abdominoperineal resection and is alive with pelvic re-recurrence. ISR and ESR are excellent procedures for anus preservation, but ISR needs a strict indication.

Anus Neoplasms↗

Percutaneous transperineal pouch localization in low imperforate anus: a new approach.

Patients who have an imperforate anus with associated infralevator, or low rectal pouch, are candidates for a perineal anoplasty. However, in the absence of a perineal fistula, intraoperative localization and isolation of the rectal pouch can be difficult. We have developed a technique to facilitate isolation and dissection of the rectal pouch for perineal anoplasty in patients with a low imperforate anus who do not have a perineal fistula present. Pouch localization is carried out preoperatively by fluoroscopic percutaneous transperineal placement of a Fogarty embolectomy catheter through the center of the anal wink. We have used this technique successfully in four consecutive patients who had a low imperforate anus, in the absence of a perineal fistula, with the pouches 1.0 to 1.5 cm from the perineum. Results have been excellent, and the dissection of the rectal pouch was facilitated greatly by the presence of the inflated Fogarty balloon.

Anal Canal↗

Association of imperforate anus with short colon: a report of eight cases.

Eight cases of imperforate anus with short colon were seen in our hospital from April 1982 to December 1987. Five were boys, three were girls, and their ages ranged from 2 days to 2 years. The international literature about this kind of disease was reviewed. The disease's name, embryology, diagnoses, differential diagnosis, treatment, and prognosis are discussed herein. We suggest that this case, which was not combined with exstrophy of the bladder and/or intestine, be called association of imperforate anus with short colon (AIASC). Other cases, combined with exstrophy of the bladder and/or intestine could be called, association of imperforate anus with exstrophy splanchnica (AIAES). This distinction is necessary because each group differs in symptoms, signs, diagnosis, treatment, and prognosis.

Anal Canal↗

Occult neurovesical dysfunction in children with imperforate anus and its variants.

Neurovesical dysfunction (NVD) is frequently encountered in children with imperforate anus and its variants. Such functional urologic problems are often compounded by associated anatomic urologic abnormalities that in combination may profoundly alter the course and prognosis of children with imperforate anus. Herein, we report 16 such cases. Management of NVD in children with imperforate anus offers several unique challenges that require important alterations in management. Specific recommendation are presented.

Anus, Imperforate↗

Cryptorchidism in boys with imperforate anus.

In a retrospective study of the case reports of 136 boys who were operated on for an imperforate anus and who survived at least 18 months, cryptorchidism was the most common associated anomaly, involving 26 cases (19%). The higher the level of the anorectal malformations, the higher was the incidence of cryptorchidism. The incidence of renal and ureteric malformations and dysplasias showed a parallel tendency. The incidence of vertebral malformations and dysplasias in the T10-S5 area was low among patients with a covered anus or a perineal fistula. Cryptorchidism was found associated with urological and with T10-S5 vertebral malformations and dysplasias. Recognition of this association is probably new. The histopathological findings of testicular biopsy specimens and the location of the undescended testes in patients with an imperforate anus showed the same pattern as seen in undescended testes from patients with cryptorchidism only. These findings, together with the existing literature on the subject, indicate that further studies on the association of cryptorchidism, urological, and T10-S5 vertebral malformations and dysplasias may be very helpful toward a better understanding of cryptorchidism in general.

Abnormalities, Multiple↗

Male infertility after surgery for imperforate anus.

Congenital abnormalities of the genitourinary tract often coexist, and cryptorchidism is common in patients who have had imperforate anus. Twenty men who had pull-through procedures for imperforate anus in infancy have been evaluated for infertility. Seven had coexisting renal abnormalities, 4 had had recurrent epididymitis, 3 had had bilateral orchidopexies (at age 7 to 12), 2 had spina bifida, and 1 had a pituitary adenoma. Seven had no ejaculate (aspermia), 11 were azoospermic, 1 was severely oligozoospermic, and 1 had a normal sperm concentration in a small volume of ejaculate. Both vasa were blocked in 5 men, and this appeared to be a result of the original operative procedure. One vas was blocked in another 7 patients who had abnormalities on the contralateral side; three had epididymal blocks after epididymitis, and four had congenital malformations associated with an absent or ectopic kidney. After reconstruction (4), insertion of sperm reservoirs (4), microscopic epididymal sperm aspiration (2), or artificial insemination (1), sperm were retrieved from 9 men (ejaculated by 4) 2 pregnancies occurred. Male infertility after treatment of imperforate anus in infancy can be related to a wide variety of cause, some of which are amenable to treatment.

Abnormalities, Multiple↗

Midterm follow-up study of high-type imperforate anus after laparoscopically assisted anorectoplasty.

PURPOSE: The aim of this study was to evaluate postoperative anal function of high-type imperforate anus after laparoscopically assisted anorectoplasty (LAARP). METHODS: Between 2000 and 2002, LAARP was performed in 13 patients with high-type imperforate anus. Clinical data of the LAARP group were compared with the posterior sagittal anorectoplasty (PSARP) group (n = 7) operated before 2000. All patients were treated with initial colostomy in the newborn period. After anorectoplasty, daily glycerin enemas were given for postoperative bowel management. Anorectal function of these patients was evaluated using the Kelly score and manometry at the age of 3 to 5 years (LAARP) and 5 to 6 years (PSARP). RESULTS: Age at evaluation in the LAARP group was younger than that in the PSARP group (51 +/- 10 vs 73 +/- 12 months, P < .01). Kelly score was 3.8 +/- 1.3 vs 3.4 +/- 0.8, respectively. Resting pressure of internal sphincter was 31 +/- 11 vs 33 +/- 10 cm H(2)O, respectively. Relaxation reflex of the internal anal sphincter was observed in 62% (8/13) and 29% (2/7), respectively. CONCLUSIONS: Midterm follow-up study revealed that satisfactory fecal continence can be achieved in patients with high-type imperforate anus after LAARP. Laparoscopically assisted anorectoplasty may be a good alternative in this patient population. However, long-term follow-up is necessary to compare the benefits of LAARP against PSARP.

Anal Canal↗

Neuroradiologic evaluation of sacral abnormalities in imperforate anus complex.

Neurologic impairment and sacral spinal anomalies are a finding in children with imperforate anus. The neurologic deficits previously had been considered static rather than progressive. Recent experience at this institution has documented the frequent association of imperforate anus patients having bony sacral and other spinal deformities with progressive neurologic dysfunction. These deficits are related to underlying spinal cord anomalies, which may be amenable to neurosurgical correction. To investigate the nature and frequency of such lesions, spinal cord imaging was done in these patients. From July 1976 to June 1985, 106 patients with imperforate anus were screened with plain radiographs of the sacral spine; 37 (35%) patients had abnormalities. Of these, 26 have been evaluated with a variety of spinal cord imaging techniques, including magnetic resonance imaging (18), digital metrizamide myelotomography (14), computed tomography (10), metrizamide myelography (5), or ultrasound (4). Four patients were lost to follow-up, and seven have not yet been studied. Fifteen of the 26 patients (53%) had one or more abnormal studies. Thirteen were considered to have progressive dysfunction with operable lesions and have undergone neurosurgical correction. At follow-up, there was improved leg function in five patients and improved bladder function in two patients. No patient had additional neurologic deficits after surgery. Because of the high incidence of progressive but potentially correctable myelodysplasias in patients with coexisting anorectal and sacral anomalies, routine radiographic screening of the spine is recommended, followed by spinal cord imaging and neurosurgical evaluation if warranted.

Abnormalities, Multiple↗

Hirschsprung's disease, imperforate anus, and Down's syndrome: a case report.

Patients with trisomy 21 have a higher incidence of several gastrointestinal anomalies. However, the coexistence of imperforate anus, Hirschsprung's disease, and trisomy 21 had not been reported previously. This report describes the case of an infant girl born with trisomy 21 and imperforate anus, without a fistula, who presented with bowel obstruction 3 months after anoplasty. The obstruction was attributable to Hirschsprung's disease. This was managed by a leveling colostomy in the descending colon, followed by an endorectal pull-through after 4 weeks. She has a normal stooling pattern 11 months after colostomy closure. Hirschsprung's disease should be suspected in infants with trisomy 21 who have constipation after repair of imperforate anus. The authors believe that the endorectal pull-through is the safest technique to use for Hirschsprung's disease after a previous anoplasty.

Abnormalities, Multiple↗

Preexisting neurogenic voiding dysfunction in children with imperforate anus: problems in management.

We evaluated 22 boys and 2 girls 1 month to 8 years old with imperforate anus to determine the relationship between neurogenic voiding dysfunction and bony sacral or spinal cord anomalies. Lower urinary tract function before anorectoplasty was normal in 12 children (group 1), abnormal (detrusor-sphincter dyssynergia) in 9 (group 2) and not evaluated in 3 (group 3). High lesions of imperforate anus were present in 22% of the patients in group 1 and in 100% of those in group 2. Plain radiography revealed partial sacral agenesis in 1 child in group 1 and 4 in group 2. Magnetic resonance imaging detected occult spinal dysraphism in 1 patient in group 1 and 2 in group 2. (Occult spinal dysraphism included sacral lipoma, tethered cord, syringomyelia and thick filum terminale.) Seven children in group 2 had vesicoureteral reflux before anorectoplasty. All children in group 2 were placed on clean intermittent catheterization for the management of neurogenic voiding dysfunction. Normal upper urinary tract function was maintained in all patients in group 1 and 6 in group 2. The remaining 3 children in group 2 had high grade reflux at presentation associated with severe renal damage or noncompliance with clean intermittent catheterization. These findings indicate that in children with imperforate anus lower urinary tract function should be evaluated before anorectoplasty because of the high incidence of associated congenital neurogenic voiding dysfunction and the potential risk for renal deterioration. When possible, neurogenic voiding dysfunction should be managed with clean intermittent catheterization to prevent renal damage.

Anus, Imperforate↗

The management of the neuropathic bladder in adolescents with imperforate anus.

The management of 58 patients born with an imperforate anus who presented to the urology service and who are currently 18 years old or older is discussed. Of the 44 patients with a high imperforate anus 43 were treated by a rectal pull-through and 1 by an anal cut back procedure, while an anal cut back procedure was used in all 14 children with a low imperforate anus. A total of 32 children (55%) had a neuropathic bladder (hyperreflexic in 29 and atonic in 3). A spinal deformity was present in 72% of the hyperreflexic group but not in the atonic group. Vesicoureteral reflux occurred in 41% of the patients, two-thirds of whom had a neuropathic bladder. A total of 30 children had an associated genital anomaly and 27 had an upper tract anomaly. Nineteen children underwent an operation for the neuropathic bladder, with modern reconstructive methods involving substitution or augmentation cystoplasty, supplemented with self-catheterization, being superior to older methods with regard to continence. The incidence of a neuropathic bladder in these children is high in both operative groups but it is usually associated with a spinal deformity and unlikely to be iatrogenic in nature unless proved to be of lower motor neuron origin. Early operative management is advised to achieve continence and minimize renal impairment.

Abnormalities, Multiple↗

Urodynamic evaluation of the patient with an imperforate anus: a prospective study.

Prospective urodynamic evaluation of 14 patients with an imperforate anus revealed detrusor-sphincter dyssynergia in 4. Subsequent contrast voiding cystourethrograms demonstrated radiographic changes in all 4 cases, consisting of bladder trabeculation, new onset of vesicoureteral reflux or hydroureteronephrosis. Of the 4 patients 3 had a high (supralevator) imperforate anus, while 1 had a low (infralevator) lesion. Two patients had bony vertebral abnormalities and 2 had normal plain radiographs and magnetic resonance imaging of the spine. Therefore, early urodynamic study is recommended for all patients with an imperforate anus and it should complement neonatal uroradiographic evaluation. The finding of detrusor-sphincter dyssynergia should alert the urologist to the need for adjunctive management.

Abnormalities, Multiple↗

Hyperchloremic acidosis and imperforate anus.

The predominant electrolyte imbalance associated with enterourinary fistulas is hyperchloremic acidosis. The mechanism is the absorption of urinary electrolytes across the colonic mucosa. One of the genitourinary associated anomalies of a high imperforate anus is a rectourinary fistula. There have been 5 cases of hyperchloremic acidosis as a complication of an imperforate anus with a rectourinary fistula reported in the literature to date. An additional case is presented with a clinical analysis of the previously reported cases. The important factors in the development of hyperchloremic acidosis in patients with an imperforate anus are 1) the presence of a rectourinary fistula, 2) an initial diverting colostomy permitting a long segment of colonic mucosa for the absorption of urinary electrolytes, 3) distal urinary tract obstruction allowing significant volumes of urine to flow into the colonic segment and 4) the presence of urinary tract infection contributing to the urinary obstruction. Management should consist of vigorous electrolyte therapy, decreasing the retrograde flow of urine into the colon by temporary catheterization and early repair of the fistulous tract.

Acidosis↗

One-stage correction of imperforate anus and rectovestibular fistula in girls: Preliminary results.

BACKGROUND/PURPOSE: This prospective study was designed to assess the safety, cost effectiveness, and advantages of performing posterior sagittal anorectoplasty without colostomy on girls with imperforate anus and rectovestibular fistula. METHODS: Four girls with imperforate anus and rectovestibular fistula were entered into the study. Chest x-ray, renal ultrasound scan, and lateral fistulogram were done. Rectal pouches were washed through the fistula with NaCl and aqueous povidone-iodine solutions. Peña's posterior sagittal anorectoplasties were done in the prone positions. Cephalosporin and metronidazole were given as perioperative antibiotics. RESULTS: All patients had intermediate anomalies. There were no other major associated congenital anomalies. Washout through the fistula was easy. There were no particular problems with posterior sagittal anorectoplasty in the prone positions. Two patients had perianal skin excoriations; one had superficial infection of the posterior sagittal wound. Two patients have undergone follow-up for a year. All are having monthly dilatations. All patients pass stool without need of stool softeners or enemas. CONCLUSIONS: This preliminary study shows that it is feasible for girls with imperforate anus and rectovestibular fistula to have safe posterior sagittal anorectoplasty without colostomy. The advantages of one, instead of 3 major operations, are many, especially in developing countries.

Abnormalities, Multiple↗

Cloaca, the most severe degree of imperforate anus: experience with 195 cases.

OBJECTIVE: To provide a follow-up of 195 patients with cloacal malformations seen by the author from 1959 to 1998. SUMMARY BACKGROUND DATA: Cloaca, which occurs in approximately 1 of 50,000 births, is the most complex type of imperforate anus with confluence of the rectum, vagina, and bladder in a urogenital sinus. Functional results for the bowel, the genital tract, and the urinary tract were formerly poor. Cloacal exstrophy, which is an even more complex spectrum of malformations, was uniformly fatal until 1960. In addition to imperforate anus, these babies have an omphalocele, two exstrophic bladders, between which there is an open cecum, and a blindly ending colon hanging down in the pelvis from the cecum. Although both of these diagnoses contain the word "cloaca," which is Latin for sewer, they are really two separate entities in terms of surgical management. Cloaca and cloacal exstrophy in most cases are very different anatomic problems. However, there are variants that are like a hybrid, which is the rationale for reporting together an experience with both entities. METHODS: Records were reviewed of 154 patients with cloaca and 41 patients with cloacal exstrophy to assess anorectal function, urinary continence, and sexual function where available. RESULTS: Follow-up was available in 141 cloaca patients: 82 have spontaneous bowel movements and satisfactory control, 38 use enemas to evacuate, 9 have a colostomy, 7 have fecal soiling, and 5 are too recently operated to evaluate. Regarding urinary control, 83 void spontaneously, 40 catheterize to empty, 4 have urinary diversion, 1 has a continent diversion, 5 patients are wet, and 8 are too recently operated to judge. Twenty-four patients are now adults, 17 of who have experienced coitus and 7 have not. Seven have had babies, all except one by cesarean section. Results of surgery for cloacal exstrophy are not as good, but are encouraging nonetheless for an anomaly that was uniformly fatal before 1960. Of the 41 cloacal exstrophy patients being followed, 7 have not undergone surgery. Fifteen have a colostomy; 19 had pull-through of the colon, but 3 were subsequently reversed for fecal incontinence. Most depend on enemas to evacuate. Urinary dryness was attained in 30 patients, usually by intermittent catheterization of the bladder, which was augmented with small bowel or stomach or both. Only three void voluntarily. Fifteen of the completed long-term patients wear no bag. Only three of the completed patients wear two bags. The rest have one bag. CONCLUSIONS: Imperforate anus and associated malformations in cloaca and cloacal exstrophy are not hopeless problems. A reasonable lifestyle can be achieved for most of these children with comprehensive surgical planning.

Anus, Imperforate↗

Squamous cell carcinoma of the anus and rectum in homosexual men: CT findings.

OBJECTIVE: Homosexual men are at risk for a variety of infectious and neoplastic diseases of the anus and rectum, many of which have been reported in the radiologic literature. The purpose of this study was to describe the CT findings in homosexual men with squamous cell carcinoma of the anus and rectum, a topic that has received little attention. MATERIALS AND METHODS: Computed tomography examinations of the abdomen and pelvis of nine homosexual men with squamous cell carcinoma of the anus and rectum were reviewed retrospectively. Six HIV-positive men were 28-44 years old (average, 37 years). Three HIV-negative men were 47-61 years old (average, 54 years). RESULTS: In six of the nine patients, CT showed a mass involving the anal canal. In five of these six patients, the tumor extended caudad to involve perianal fat and skin (one patient), cephalad to involve the rectum and perirectal fat (two patients), or both (two patients). In three of the nine patients, the anal canal did not appear to be involved by the tumor; CT showed circumferential rectal wall thickening (one patient), an excavated eccentric rectal tumor (one patient), or a cystic extramucosal rectal mass (one patient). Computed tomography evidence of noncontiguous metastasis was limited to inguinal and iliac lymph node enlargement in two of the nine patients. CONCLUSION: Because CT findings are varied and nonspecific, squamous cell carcinoma should be included in the differential diagnosis of anorectal lesions in homosexual men.

Adult↗