1/27. Aphallia as part of urorectal septum malformation sequence in an infant of a diabetic mother.A male patient with aphallia, anal stenosis, tetralogy of fallot, multiple vertebral anomalies including sacral agenesis and central nervous system (CNS) malformations was born after a pregnancy complicated by poorly controlled maternal diabetes. Aphallia is an extremely rare abnormality and can be part of the urorectal septum malformation sequence (URSMS). While aphallia has not been reported in infants of diabetic mothers, urogenital malformations are known to occur with increased frequency. Two female products of pregnancies complicated by diabetes presented with multiple malformations including anal atresia and recto-vaginal fistula consistent with the diagnosis of URSMS. The three patients share CNS, cardiac, and vertebral anomalies, abnormalities secondary to abnormal blastogenesis and characteristic of diabetic embryopathy. URSMS is also caused by abnormal blastogenesis. Therefore, this particular malformation should be viewed in the context of the multiple blastogenetic abnormalities in the cases reported here. The overlap of findings of URSMS in our cases with other abnormalities of blastogenesis, such as VATER association or sacral agenesis is not surprising, as these associations are known to lack clear diagnostic boundaries.- - - - - - - - - - ranking = 1keywords = vagina (Clic here for more details about this article) |
2/27. A case of association of duplication of the urogenital and intestinal tracts.Report of the case of a Caucasian girl, presently 3 years old, who has been followed at the Pediatric Service of the Walter Cantidio Hospital (Federal University of Ceara; Fortaleza, brazil) since she was 15 days old. At birth she presented a duplication of the bladder, urethra, external genitalia, vagina, colon, and rectum. No cardiac anomalies, bone deformities, or other congenital malformations were detected. When she was 16 months old the child underwent corrective surgery, with rectal vault reconstruction, placing a single and continent anal sphincter in its usual position and resection of the longitudinal vaginal septum, forming a single vaginal cavity. The surgery also resulted in a perineum of normal appearance. Presently, the patient is doing well.- - - - - - - - - - ranking = 3keywords = vagina (Clic here for more details about this article) |
3/27. A case report of caudal regression syndrome associated with an intraspinal arachnoid cyst.We report here a rare case of caudal regression syndrome associated with an intraspinal arachnoid cyst. The patient was a 6-month-old baby girl with multicomplex congenital abnormalities: sacrococcygeal dysgenesis and ventral curvature, large terminal cyst (myelocystocele), spinal arachnoid cyst, cerebellar hypertrophy (suspected), high imperforate anus, partial dysgenesis of the large intestine, omphalocele, atresia of the vagina, bilateral incomplete ureter duplication, incomplete pseudoduplicated bladder and bilateral talipes equinovarus. We performed plastic repair of the myelocystocele and perineal lesion for caudal regression syndrome and partial removal of the cyst wall for the intraspinal arachnoid cyst. She has been well for 3 years postoperatively, and her mental development is normal.- - - - - - - - - - ranking = 1keywords = vagina (Clic here for more details about this article) |
4/27. Long-term results of treatment of single-system ectopic ureters.Single-system ureteral ectopia (UE) encompasses a spectrum of malformations involving the bladder trigone, ureter, and kidney. The clinical presentation is variable, and both diagnostic and therapeutic problems are common. Reduced renal function in these patients may result from primary dysplasia, obstruction, vesicoureteral reflux, or recurrent infection. Based on our experience of seven patients, suggestions for diagnostic procedures and criteria for renal saving versus nephrectomy are offered. The relationship between ostium localization, renal function, and long-term results was investigated. From 1972 to 1990, five female and two male patients were studied. During the same period, 31 patients with UE and duplex kidneys were seen. Ages ranged from 1 day to 7 years. A ureteric opening into the bladder neck was associated with dilatation of the ureter and renal pelvis. Two patients had vaginal ectopia and severe renal dysplasia. In one, a cyst of the vaginal wall (Gardner's cyst) was detected at birth. A male newborn had multicystic renal dysplasia on the left and ureteric ectopia to the ductus deferens on the right side. To our knowledge, he is the first patient reported with renal function totally dependent on a kidney with severe UE. Follow-up ranged from 4 to 9 years. One patient died in the postoperative period because of renal failure and sepsis. All the others are well and have normal creatinine values. Improvement of renal function was noted after ureteral reimplantation (URI) in patients with bladder-neck ectopia. The numbers of infections were also drastically reduced. Our observations suggest that the combination of ultrasound, cyst urethrography, and cystoscopy will be diagnostic in most patients. A suspicion of UE should be raised in symptomatic patients with apparently solitary kidneys, enuresis ureterica, or atypical obstructive uropathy. Reduced renal function in some patients with ectopia to the bladder neck will improve after URI. This may be of importance in patients with bilateral anomalies and marginal renal function.- - - - - - - - - - ranking = 2keywords = vagina (Clic here for more details about this article) |
5/27. Two cases of the caudal duplication anomaly including a discordant monozygotic twin.We present two unrelated patients with various duplications in the caudal region. One patient presented with a duplication of the distal spine from L4, left double ureter, duplication of the vagina and cervix, and duplication of the distal colon. The second patient was diagnosed with a duplication of the colon, bladder, vagina and uterus. The first patient had an unaffected monozygotic twin sister. Dominguez et al. [1993: Am J Dis child 147:1048-1052] presented six similar cases, and introduced the name "caudal duplication syndrome." The pathogenesis of the caudal duplication anomaly is unclear. The possibility of a polytopic primary developmental field defect or a disruptive sequence are discussed. On the other hand, somatic or germline mutations in certain developmental genes could be involved, as illustrated by the mouse mutations disorganisation and fused. dna-analysis of the AXIN1 gene, the human homologue of the gene responsible for fused, performed in our first patient, did not show any apparent pathogenic mutation.- - - - - - - - - - ranking = 2keywords = vagina (Clic here for more details about this article) |
6/27. A rare association of urogenital duplication and anorectal malformation.An unusual case of urogenital duplication in association with anorectal malformation is presented. A 3-year-old girl was referred to the authors' hospital with double vagina, double urethra, double sacrum, double ureters on the right side, multiple vertebral anomalies, together with anorectal malformation. Successful surgical reconstruction was performed.- - - - - - - - - - ranking = 1keywords = vagina (Clic here for more details about this article) |
7/27. hematocolpos in double vagina associated with uterus didelphus: US and MR findings.The malformative syndrome of double vagina in association with uterus didelphus and kidney agenesis is a rare condition, often asymptomatic: if this condition is accompanied by imperforated obstructed hemivagina, the clinical manifestations depend on the presence of hematocolpos. MRI plays an important role for diagnosis because it allows to characterize the nature of the lesion and to evaluate the anatomical details of the uterine malformation. The authors describe this complex genital malformation and discuss the main US and MRI features.- - - - - - - - - - ranking = 6keywords = vagina (Clic here for more details about this article) |
8/27. Retroperitoneoscopic nephrectomy of a horseshoe kidney with renal-cell carcinoma.A 55-year-old man had a solid tumor in the right part of a horseshoe kidney. He had previously undergone open cholecystectomy and appendectomy with panperitonitis. Retroperitoneoscopic nephrectomy was performed, with rapid recovery and good short-term results. This appears to be the first case of endoscopic surgery on a horseshoe kidney using ultrasonic shears for cancer.- - - - - - - - - - ranking = 0.0014517404933667keywords = cancer (Clic here for more details about this article) |
9/27. Complex genital malformation: ectopic ureter ending in a supposed mesonephric duct in a woman with renal agenesis and ipsilateral blind hemivagina.We present a clinical case of a patient with left renal agenesis and ipsilateral blind hemivagina who also had one or more atretic ectopic ureters opening into supposed mesonephric duct, which in turn opened into, or joined onto, the ipsilateral hemicervix, continuing with the blind hemivagina. The diagram of this complex genitourinary malformation would strongly support our hypothesis of the embryology of the human vagina as deriving from the wolffian ducts and the Mullerian tubercle.- - - - - - - - - - ranking = 7keywords = vagina (Clic here for more details about this article) |
10/27. Surgical management of vaginal vault prolapse in a woman with a neovagina and pelvic kidneys.BACKGROUND: women with Mayer-Rokitansky-Kuster-Hauser syndrome have congenital absence of the uterus and upper two-thirds of the vagina, which is frequently accompanied by skeletal and renal anomalies. Mechanical dilation or surgical creation of a vagina allows for function but does not provide endopelvic fascial support of the vagina. Vaginal prolapse may occur. CASE: A 32-year-old woman presented with pelvic kidneys and a 5-year history of prolapse of her mechanically created neovagina. She underwent a sacrospinous ligament suspension with a cadaveric fascia lata bridge. The apex of the neovagina was 5 cm above the hymen 30 months postoperatively. CONCLUSION: An allograft colpopexy to the sacrospinous ligament is an effective method of surgical treatment of women with a prolapsed shortened vagina and an inaccessible presacral space.- - - - - - - - - - ranking = 14keywords = vagina (Clic here for more details about this article) |
| | Next -> |