A case of unique communication between blind-ending ectopic ureter and ipsilateral hemi-hematocolpometra in uterus didelphys

Uterus didelphys with double vagina and hemi-vaginal atresia is a rare syndrome of congenital anomalies. A 17-year-old girl had a right blind-ending ectopic ureter, the proximal end of which communicated with the ipsilateral uterine cervix of uterus didelphys. The patient presented with vaginal urinary incontinence after incision of the vaginal wall for right hemi-hematocolpometra. Following various examinations, the ipsilateral kidney was found to be absent. The ectopic ureter and communicating duct were resected, and the fistula was closed. The genesis of malformation of the female genitalia and urinary tract resulting in such a unique communication is discussed. The importance of preoperative meticulous examinations, including cysto-genitography, pelvic magnetic resonance imaging and panendoscopy with the patient under anesthesia, is emphasized.     

Cochlear implants

We report on a fetus with cranio-facial anomalies, a narrow thorax, imperforate anus with cloacal cyst, and a genitourinary malformation with absent uterus, vagina, and external genitalia. Major thoracic defects were seen on roentgenographic examination, including absent vertebrae and ribs, a supernumerary vertebra, a hemivertebra, and rib fusion. These findings are compatible with Casamassima-Morton-Nance syndrome. The patient was the carrier of a translocation t(6;9)(p12;q12), inherited from the mother. Although the occurrence of this rearrangement may be coincidental, it may also indicate a possible locus for this autosomal recessive thoracic dysplasia.     

Gonadotropin triggers the generation of proteinaceous factor in vitellogenic perch (Anabas testudineus) oocyte which stimulates ovarian aromatase activity

OBJECTIVE: Evaluation of the feasibility of bilateral sacropinous ligament suspension with a stapler. Morbidity study and short term results. STUDY DESIGN: Prospective study from July 1994 to August 1996. RESULTS: Bilateral sacrospinous ligament suspension with a stapler was possible in 100% of cases and surgical technique is described. Our indications are stage III Bp and stage IV genital prolapses (according to the American Urogynecologic Society classification, 1996), with or without uterus, and when a Bologna's procedure is performed, in order to prevent enterocele. In 24 patients, the uterus was present. 20 vaginal hysterectomies and 4 conservative bilateral uterine suspensions were performed. The sacrospinous ligament suspension was associated to anterior colporrhaphy (in 74% of patients), repair of rectocele (82%), repair of enterocele (26%), posterior colpoperineorrhaphy (79%), bladder neck suspension (71%). No vascular injury nor post operative constipation was noted. In 2 patients, a small rectal laceration occurred, and in one patient one branch of the staple transfixed the rectal mucosa. Removal of the staple was easily performed without any post-operative complication. First results after an average 19 months follow-up (range 9 to 32) shows a perfect anatomic result in 77% of cases. We noted one recurrence of a vaginal vault prolapse; the patient underwent a second sacrospinous ligament fixation with good result. One patient had a stage II Aa cystocele post-operatively and three patients had a short vagina (< 6 cm). Patients who were continent before the sacrocolpopexy did not develop further urinary stress-incontinence. CONCLUSION: Bilateral transvaginal sacrospinous ligament suspension with a stapler facilitates the procedure. No post-operative constipation was noted with this method. Our first results are good. The cost of the stappler may limit its extensive use.     

Uterine arteriovenous malformation necessitating hysterectomy with bilateral salpingo-oophorectomy in a young pregnant patient

This case report describes a 31-year-old woman at 8 weeks' gestation with large arteriovenous malformation of the uterus involving bilateral uterine and ovarian arteries. She had a history of multiple pregnancy losses, as well as spontaneous copious vaginal hemorrhage. The patient underwent an embolization procedure followed by total abdominal hysterectomy and bilateral salpingo-oophorectomy. The uterus was very small (30 g) despite its gravid status, and the overall microscopic findings indicated Müllerian system hypoplasia in addition to vascular malformation.     

Posterior sagittal anorectoplasty in the treatment of anorectal malformations

Posterior sagittal anorectoplasty is a marked change in treatment of anorectal malformations and differs fundamentally from previous techniques. In 1991-1996 86 patients (51 boys and 35 girls) aged 1 day to 22 years were operated. A primary operation was performed in 65 patients, a secondary one in 21 patients. In primary operations posterior sagittal anorectoplasty was used in 50 patients, in 3 cloacal malformations it was extended by reconstruction of the vagina and urethra. Fifteen patients were operated by other techniques. Posterior sagittal anorecto (vaginourethro) plasty was used in all 21 reoperated patients. Of 65 patients with primary operations one patient died from aspiration bronchopneumonia four years after operation. The patient had also an operation on account of oesophageal atresia. Three patients were reoperated. The continence of patients after primary operations is satisfactory, 5-6 points on Kelly's scale. In 19/21 reoperated patients the continence is 3-5 points according to Kelly. In two patients no substantial changes were recorded, one was reoperated twice. As compared with pull-through techniques the posterior surgical method of anorecto (vaginourethro) plasty is excellent. It cannot be used however without adequate experience.     

High-dose-rate intracavitary brachytherapy in the management of cervical and vaginal intraepithelial neoplasia

PURPOSE: To assess the effectiveness of high-dose rate intracavitary brachytherapy (HDR-ICR) in patients with grade 3 cervical intraepithelial neoplasia (CIN-3) and grade 3 vaginal intraepithelial neoplasia (VAIN-3). METHODS AND MATERIALS: This was a retrospective analysis in 20 patients with CIN-3 (n = 14) or VAIN-3 (n = 6), average age 61.9 years, managed with HDR-ICR at Kanagawa Cancer Center. Two patients with CIN-3 with microinvasive foci and 11 other patients with CIN-3 were treated with HDR-ICR for cervical lesions. Six patients with CIN-3 after hysterectomy received HDR-ICR for recurrent or residual VAIN-3 lesions. One patient received radiation therapy for both CIN-3 and VAIN-3 lesions. All these patients but one were postmenopausal. RESULTS: Seventeen patients were treated with HDR-ICR alone, and three with combined external radiation therapy. The dose was calculated at Point A located 2 cm superior to the external os and 2 cm lateral to the axis of the intrauterine tube for intact uterus. For lesions of the vaginal stump, the dose was calculated at a point 1 cm superior to the vaginal apex or 1 cm beyond vaginal mucosa. In the 14 patients treated for CIN-3 lesions, the mean total dose of HDR-ICR was 26.1 Gy (range 20-30). Six patients received HDR-ICR for VAIN-3 lesions with mean dose of 23.3 Gy (range 15-30). At follow-up (mean 90.5 months; range 13-153), 14 patients were alive and 6 had died owing to nonmalignant intercurrent disease. No patient developed recurrent disease. Rectal bleeding occurred in three patients, but this symptom subsided spontaneously. Moderate and severe vaginal reactions were noted in two patients, in whom the treatment had included the entire vagina. CONCLUSIONS: HDR-ICR can be employed as the primary management strategy for postmenopausal women with CIN-3. In intraepithelial neoplasia involving the vaginal wall after hysterectomy, HDR-ICR should be considered as an alternative to total vaginectomy.     

Genital fistulas caused by diverticular disease of the sigmoid colon

OBJECTIVE: Our purpose was to bring to the attention of gynecologists a subject not mentioned in a single textbook of gynecology, namely, genital fistulas resulting from diverticular disease of the sigmoid colon. STUDY DESIGN: We report our experience with 13 genital fistulas caused by sigmoid diverticulitis. RESULTS: Ten fistulas involved the vagina, one the vagina and bladder, one the tube, and one the uterus. Average age of the patients was 68.6 years (range 54 to 89 years). Presenting symptom in 12 patients was a malodorous vaginal discharge. All with vaginal lesions had previously undergone total hysterectomy. A barium enema failed to demonstrate a fistula in 8 of 11 patients. Colonoscopy failed in 8 of 8 patients. All fistulas were demonstrated by retrograde dye studies. Ten patients operated on were cured. Three patients refused surgery; of these, 1 had intestinal obstruction, 1 may have had spontaneous closure of the fistula, and 1 is being observed. Surgery involved staged procedures in 2 patients, fistulectomy in 4, and bowel resection and anastomosis in 4. CONCLUSIONS: Sigmoidovaginal fistulas are the most prevalent variety of cologenital fistula caused by sigmoid diverticulitis. The diagnosis should be considered in a patient > 50 years old who complains of a foul vaginal discharge and has a history of total hysterectomy. Its presence is best demonstrated by vaginogram. Surgical therapy is advised, the extent of which will rest on the surgeon's judgment of the severity of the inflammatory process found at exploration.     

Perineal reconstruction in girls with high vaginal atresia

PURPOSE: We present a novel surgical treatment for secondary hematocolpos in adolescent girls with high vaginal atresia. MATERIALS AND METHODS: Vaginoplasty was performed in 5 girls 12 to 14 years old with high vaginal atresia. The operation included the perineal approach, mobilization of hematocolpos and plastic surgery of the distal portion of the vagina using a U-shaped mucous-submucous vaginal flap, and introital and labial flaps. RESULTS: There were no complications 4 to 6 months after the operation. There were no instances of cosmetic defects and stenosis. CONCLUSIONS: The proposed technique reduces the difficulty and trauma of the operation for high vaginal atresia with hematocolpos and an absent distal portion of the vagina. An increase in plastic reserve provided by the original mobilization of hematocolpos and U-shaped vaginal flap makes intestinal and skin colpopoiesis unnecessary.     

Adenocarcinoma and adenosis of the vagina. On the histogenesis, diagnosis and therapy of a rare genital neoplasms

In the United States, vaginal adenosis and clear-cell carcinoma of the vagina were frequently observed in young women, who had been exposed to the synthetic estrogen diethyl-stilbestrol (DES) during their embryonic development. In Germany, obviously, no such exposure occurred. Clearly, such diseases also develop without the context of intrauterine exposure to certain substances. Our own case of such a partly exophytic, partly endophytic adeno-carcinoma of the vagina with multifocal vaginal adenosis, demonstrates the histogenesis, symptoms, diagnostic procedures and therapy of this rare disease. Since young women during their reproductive years are mostly affected, the possibility of fertility-conserving surgery is discussed despite the current practice of radical cancer surgery (with and without radiation). In the 25-year-old patient, we conducted a colpectomy whilst leaving the uterus and ovaries, and replaced the vaginal defect by a sigmoid transplant anastomosed to the cervix. The patient has regular menstrual cycles, should, however, not become pregnant for 1 to 2 years for oncological reasons.     

The role of rectosigmoid neocolporrhaphy

BACKGROUND: Vaginoplasty for congenital vaginal atresia, a component of the Mayer-Rokitansky-Kuster syndrome, or for gender confirmation, may be achieved by several techniques. This report focuses on the efficacy of rectosigmoid neocolporrhaphy (RSNC) performed either primarily or secondarily after failure of another procedure. METHODS: Sixty patients underwent isoperistaltic RSNC, three primarily and 57 secondarily. The indication was vaginal atresia in 1 patient and gender dysphoria in 59 patients. RESULTS: All 60 patients survived and have a functional neovagina. One major complication, an anastomotic leak with colovaginal fistula, was treated by a temporary colostomy and later reconstruction through a combined anterior and posterior approach. Late complications were reversible stomal stenosis (six patients), reversible conduit narrowing (five patients), transient rhabdomyoblastosis (one patient), and temporary mucosal bleeding from hyperplasia (three patients). Thirty patients have regular intercourse, 12 patients have occasional intercourse, and the others feel "whole," with their intact desired sexual anatomy awaiting a suitable partner. CONCLUSIONS: The number of patients seeking vaginoplasty is increasing. Primary or secondary RSNC is a safe and effective method.     

Characterization of mutations at the mouse phenylalanine hydroxylase locus

By using a three-dimensional computed tomography (CT) scanner, we compared the anatomic features of the pelvis of three fetuses of same gestational age, one with a normal pelvis representing the reference model, one with classic bladder exstrophy, and one with cloacal exstrophy. The tomography slices were selected at the same levels for each case. Three angles expressing external opening of the pelvis were defined. Comparing normal and abnormal pelvises allowed definition of three criteria for the correction of the malformation: (a) the sum of the differential angles gives the amplitude of the correction needed; (b) a supraacetabular osteotomy appears to allow best closure of the pelvic ring; (c) only three slices of a CT scan are needed, which cannot be harmful, especially for neonates. Therefore, we believe that a CT scan of the pelvis should be performed whenever an osteotomy is planned in the surgical reconstruction of bladder and cloacal exstrophy.     

Axial osteomalacia: report of a new case with selective increase in axial bone mineral density

We report a case of a rare congenital nasal malformation. A young male patient was seen with a unilateral duplication of the left nostril. According to the morphogenetic classification, described by van der Meulen, this nasal malformation could be classified as a type IV nasal dysplasia. The other observed abnormalities that were seen were part of the CHARGE association, which is defined as coloboma of the iris, heart deformities, choanal atresia, retarded growth, genital and ear deformities. The patient underwent a surgical correction of the nose using an L-approach, yielding a satisfactory result 1 year postoperatively. Details of the case and a review of the scarce literature are presented.     

Cloacal duplication

Cloacal duplication including the rectum, anus, and genitourinary organs is one of supernumerary development. Only two cases have been reported in the literature. A case with cloacal duplication is presented. The patient was an 8-year-old girl who has had fecal incontinence since birth. Successful surgical treatment was achieved by removing one rectum and exteriorizing the other through perineal raphe.     

Vaginal replacement in children and young adults

PURPOSE: Absence of the vagina in the pediatric population most commonly results from congenital abnormalities, such as the Mayer-Rokitansky syndrome but it may also be seen after treatment for pelvic tumors, such as rhabdomyosarcoma, and in patients who have had previous gender reassignment. We review our experience using bowel for vaginal replacement in a group of children and young adults to assess outcome and satisfaction. MATERIALS AND METHODS: From 1980 to 1996 we evaluated 31 patients 1 to 20 years old who required vaginal replacement. Presenting diagnoses included müllerian failure (the Mayer-Rokitansky syndrome) in 20 patients, androgen insensitivity syndrome in 5, rhabdomyosarcoma in 3, penile agenesis in 1, cloacal exstrophy in 1 and 1 previously separated conjoint twin. A questionnaire was given to 26 of the 31 patients to assess postoperative sexual function and satisfaction. RESULTS: A total of 33 bowel segments in 31 patients were used for vaginal reconstruction, including sigmoid colon in 20, ileum in 8 and cecum in 5. Of the 31 patients 20 were sexually active, 8 were married and 3 had been previously married and divorced. Only 1 patient described chronic dyspareunia. Three patients were on chronic home dilation, while 4 required sanitary pads for vaginal secretions. There were 8 complications in the 31 patients, including stenosis of the bowel segment in 6. Three patients required a second procedure after total stenosis of the small bowel vagina (2) and prolapse of the neovagina (1), which required retroperitoneal fixation. CONCLUSIONS: Experience with this group of patients leads us to believe that isolated bowel segments provide excellent tissue for vaginal replacement. Furthermore, we believe that colon segments, particularly sigmoid, are preferable to small bowel for creation of the neovagina. In many instances the small bowel mesentery may be too short to provide an adequate, tension-free anastomosis in the perineum, particularly in obese patients. Our results would also suggest that sexual activity is more compatible with isolated bowel segments for vaginal replacement than with any of the more traditional methods, such as passive dilations and split thickness skin graft vaginoplasty.     

Pelvic exenteration for malignant melanomas of the vagina or urethra with over 3 mm of invasion

Pelvic exenteration has usually been employed as salvage treatment for gynecologic malignancies which have failed primary radiotherapy. The therapeutic mainstay for vulvar melanomas has become wide local excision with or without concurrent regional node dissection. Patients with primary melanoma of the vagina who undergo exenteration as primary therapy may experience 50% 5-year survival if the pelvic nodes are free of metastases. However, the overall 5-year survival for vaginal melanoma is 15%. In our patient population, there have been four patients with vaginal or urethral melanomas treated primarily with pelvic exenteration. The purpose of this study was to report that patients with vaginal or urethral melanomas over 3 mm in thickness may benefit from primary pelvic exenteration. Four patients underwent pelvic exenteration at Indiana University Medical Center for malignant melanoma of the vagina or urethra between 1986 and 1992. The pathologic specimens of all patients were analyzed for thickness, growth pattern, and nodal metastases. Patient age ranged from 50 to 71. Thickness of the melanomas ranged from > 3 to 12 mm. All four patients underwent exenterations, three total and one anterior. All patients had negative pelvic and inguinal nodes at the time of surgery. None of the patients has experienced a recurrence. Three of four patients are alive without evidence of disease at 31 to 97 months following their exenteration. One patient died postoperatively of cardiopulmonary complications. Patients with melanomas of the vagina and female urethra, greater than 3 mm in thickness, may benefit from primary pelvic exenteration.     

Intestinal atresia and stenosis: a 25-year experience with 277 cases

OBJECTIVE: To evaluate the causes, clinical presentation, diagnosis, operative management, postoperative care, and outcome in infants with intestinal atresia. DESIGN: Retrospective case series. SETTING: Pediatric tertiary care teaching hospital. PATIENTS: A population-based sample of 277 neonates with intestinal atresia and stenosis treated from July 1, 1972, through April 30, 1997. The level of obstruction was duodenal in 138 infants, jejunoileal in 128, and colonic in 21. Of the 277 neonates, 10 had obstruction in more than 1 site. Duodenal atresia was associated with prematurity (46%), maternal polyhydramnios (33%), Down syndrome (24%), annular pancreas (33%), and malrotation (28%). Jejunoileal atresia was associated with intrauterine volvulus, (27%), gastroschisis (16%), and meconium ileus (11.7%). INTERVENTIONS: Patients with duodenal obstruction were treated by duodenoduodenostomy in 119 (86%), of 138 patients duodenotomy with web excision in 9 (7%), and duodenojejunostomy in 7 (5%) A duodenostomy tube was placed in 3 critically ill neonates. Patients with jejunoileal atresia were treated with resection in 97 (76%) of 128 patients (anastomosis, 45 [46%]; tapering enteroplasty, 23 [24%]; or temporary ostomy, 29 [30%]), ostomy alone in 25 (20%), web excision in 5 (4%), and the Bianchi procedure in 1 (0.8%). Patients with colon atresia were managed with initial ostomy and delayed anastomosis in 18 (86%) of 21 patients and resection with primary anastomosis in 3 (14%). Short-bowel syndrome was noted in 32 neonates. MAIN OUTCOME MEASURES: Morbidity and early and late mortality. RESULTS: Operative mortality for neonates with duodenal atresia was 4%, with jejunoileal atresia, 0.8%, and with colonic atresia, 0%. The long-term survival rate for children with duodenal atresia was 86%; with jejunoileal atresia, 84%; and with colon atresia, 100%. The Bianchi procedure (1 patient, 0.8%) and growth hormone, glutamine, and modified diet (4 patients, 1%) reduced total parenteral nutrition dependence. CONCLUSIONS: Cardiac anomalies (with duodenal atresia) and ultrashort-bowel syndrome (<40 cm) requiring long-term total parenteral nutrition, which can be complicated by liver disease (with jejunoileal atresia), are the major causes of morbidity and mortality in these patients. Use of growth factors to enhance adaptation and advances in small bowel transplantation may improve long-term outcomes.     

Screening for intracranial aneurysms in patients with isolated polycystic liver disease

BACKGROUND/PURPOSE: Cloacal exstrophy can now be managed with excellent survival rates and reasonable long-term outcomes with many of these patients living into their late teens and early adulthood. In this report, the authors describe for the first time the association of large ovarian cysts with cloacal exstrophy. METHODS: From 1974 to 1996, 12 patients with cloacal exstrophy have been treated at C.S. Mott Children's Hospital. Massive ovarian cysts developed in four of these. These patients represent the subjects of this study. RESULTS: All four patients have been followed up beyond puberty and massive ovarian cysts have developed, which have caused significant morbidity. Three patients have required surgical intervention. All the patients had reached menarche before the development of the cysts. In all cases, the presentation was severe pelvic pain. Urinary tract obstruction from the large pelvic cysts developed in three of the four. The cysts were bilateral in three of four patients and measured 8 to 10 cm in diameter on ultrasound scan or computed tomography (CT). Cyst aspiration was attempted in two cases and was unsuccessful. Three of the four patients have required bilateral salpingo-oophorectomy. The indications for surgery were uncontrollable pelvic pain in one and urinary obstruction and uncontrollable pelvic pain in two. Surgical findings demonstrated massive thin-walled cysts with essentially no normal ovarian tissue in association with duplicated mullerian structures. The pathology findings were corpus luteal cyst in two and mucinous cystadenoma in one. The fourth patient with an 8- x 10-cm unilateral cyst is being followed up. CONCLUSIONS: The authors have described, for the first time, the association of massive ovarian cysts with cloacal exstrophy. These cysts can lead to severe pelvic pain and urinary tract obstruction. Bilateral oophorectomy has been required in most of these patients.     

Changes in microflora of the cloaca and oviduct of hens after intracloacal or intravaginal inoculation with Salmonella enteritidis

Quantitative and qualitative microbiological examination was carried out on cloacal and oviductal contents pre- and postinfection with Salmonella enteritidis (SE) intracloacally or intravaginally. Before inoculation with SE, the means +/- standard deviation (SD) of total bacterial counts, anaerobic bacterial counts, and aerobic bacterial counts in the cloaca were log10 7.7 +/- 0.7, 7.4 +/- 0.2, and 6.9 +/- 0.8 colony-forming units (CFU)/g, respectively. The predominant bacteria were Bacteroidaceae, Lactobacillus, and Escherichia coli. Before inoculation with SE, the means +/- SD of total bacterial counts, anaerobic bacterial counts, and aerobic bacterial counts in the vagina were log10 5.7 +/- 1.4, 5.5 +/- 1.3, and 3.6 +/- 2.7 CFU/g, respectively. Bacteroidaceae and Lactobacillus were predominant. Following inoculation with SE, only the cloacal population of Lactobacillus in hens inoculated intracloacally was significantly increased compared to that before the inoculation. Other indigenous microflora were stable even after the inoculation. In the uterus, very few bacteria, Lactobacillus and Staphylococcus, were isolated. Five of 20 eggs (25%) from hens inoculated with SE intravaginally were positive for SE, whereas no SE was recovered from 22 eggs in hens inoculated with SE intracloacally. SE was recovered from the uterus after intravaginal inoculation with SE and from the vagina after intracloacal inoculation with SE. Contamination may ascend from the cloaca into the lower parts of the oviduct and subsequently contaminated eggs may occur.     

Immunohistochemical studies on the expression and estrogen dependency of EGF and its receptor and C-fos proto-oncogene in the uterus and vagina of normal and neonatally estrogen-treated mice

BACKGROUND: The final target cell response to estrogen is dependent not only on the estrogen receptor, but also on autocrine/paracrine interactions with growth factors (e.g., EGF) and proto-oncogenes (e.g., c-fos). Because neonatal estrogen treatment results in permanent changes in the female mouse genital tract (permanent vaginal cornification, cervical adenosis and tumors, changed growth control mechanisms in uterus), it was of interest to study possible acute and permanent effects of such treatment on distribution and levels of EGF, its receptor (EGF-r), and c-fos and to relate such changes to morphological development and appearance of epithelial abnormalities. METHODS: Immunohistochemical techniques using frozen sections from the uterus and vagina of neonatal and adult (ovariectomized, estradiol-treated) females, treated with olive oil or diethylstilbestrol in neonatal life. RESULTS: A difference in stromal-epithelial distribution of EGF was demonstrated with respect to region studied (uterus, vagina) and age (neonatal, adult). EGF was localized mainly in the uterine stroma but in both vaginal epithelium and stroma (with a different pattern compared to uterus). In neonatal females, EGF occurred in both tissue components in both regions, and the distribution pattern was quite different from that in adult females. The EGF level was increased by estrogen in adult but not in neonatal females. EGF-r and c-fos occurred in both uterine epithelium and stroma and in the vaginal epithelium; levels and distribution pattern were affected by estrogen. Neonatal estrogen treatment increased the levels of uterine EGF and c-fos in adult life. CONCLUSIONS: There are distinct developmental changes in the distribution and estrogen sensitivity of EGF. Only further studies can prove or disprove the association between the earlier reported disturbed growth control mechanisms in the uterus of adult but neonatally estrogen-treated females and the increased levels of uterine EGF and c-fos. The present results do not seem to explain mechanisms involved in the origin of neonatally estrogen-induced cervicovaginal epithelial abnormalities, nor do they explain the earlier described difference in estrogen-induced proliferative response between the uterine cervix and uterus proper.     

Dysraphic myelodysplasias associated with urogenital and anorectal anomalies: prevalence and types seen with MR imaging

OBJECTIVE: The purpose of this study was to determine by MR imaging the prevalence and types of dysraphic abnormalities of the spinal cord (i.e., myelodysplasias) associated with urogenital and anorectal malformations of childhood. MATERIALS AND METHODS: Since 1987, 92 patients with imperforate anus complex, cloacal malformation, and cloacal exstrophy have had MR imaging as a screening examination for occult dysraphic myelodysplasia. The prevalence and types of myelodysplasia were determined for each group. RESULTS: The prevalence of dysraphic myelodysplasia in each group of children was 17% (1/6) for low imperforate anus (ectopic anus), 34% (11/32) for high imperforate anus (with fistulization), 46% (19/41) for cloacal malformation, and 100% (13/13) for cloacal exstrophy. The most common abnormalities in each group were tethered cord with intradural or filar lipoma in imperforate anus; low-placed or dysplastic conus medullaris and tethered cord with lipoma or myelolipoma in cloacal malformation; and lipomyelocele, lipomyelomeningocele, or lipomyelocystocele in cloacal exstrophy. CONCLUSION: Our results show that the prevalence of myelodysplasia as seen on MR imaging is high in patients with urogenital and anorectal anomalies.