1/8. Separation of Siamese twins in Bratislava.At the pediatric surgical clinic in Bratislava, in the year 2000, we successfully carried out the separation of Siamese twins, which we classified as "ischiopagus tetrapus" . The pelvic ring, the gastrointestinal tract and the urogenital system were all malformed. There was a common terminal ileum and only a short segment of the large intestine with a blind end. This was a case of non-developed anal segment and rectum. Each of the twins had two kidneys and two commonly shared urinary bladders. There were two uteruses, which were both bicornuate in nature with a fallopian tube arising from each of the horns as well as an ovary. The orifices of both urinary bladders and those of the two uteruses joined into a common urogenital sinus. The external ostia of this sinus represented a bizarre fissure with a single skin fold similar to the labium majus, located dorsally at the point of fusion of the pelvic structures. The act of separation was performed in two stages. Separation of the gastrointestinal tract was urgent due to the severe ileus caused by aplasia of the anus and the rectum. This first stage procedure was performed on the third day of admission (24.2.2000) after the babies had spent a month in other health institutions. The definitive separation was carried out a month after the first procedure (28.3.2000) following the optimal stabilization of the functions of the gastrointestinal tract. The uropoietic and genital systems were separated. A new pelvic ring and a pelvic floor were formed. The anterior abdominal wall was reconstructed and plastic correction of the skin in the gluteal and perineal regions was performed. The colostomies are functioning well and the twins pass well-formed stools. Lucia has long dry intervals between mictions. Andrea has shorter dry intervals. The girls have recently celebrated their third birthday. Their mental development is excellent; they speak Slovak and Hungarian languages. They have a well-developed locomotive apparatus, without any movement impairments. (Tab. 1, Fig. 16, Ref. 10.)- - - - - - - - - - ranking = 1keywords = fallopian tube, tube (Clic here for more details about this article) |
2/8. 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 = 0.0077915395072936keywords = tube (Clic here for more details about this article) |
3/8. Mullerian agenesis and ovarian torsion. A case report and review of literature.A case report of a premenarcheal patient with an ovarian torsion and mullerian agenesis is presented. An 11-year-old prepubertal girl presented with severe left lower quadrant abdominal pain and mild rebound. A computed tomography showed a normal appendix; an ultrasound showed a left ovary measuring 3 x 2 cm with multiple 0.5-mm simple cysts. A diagnostic laparoscopy showed the left ovary tube to be twisted, but the uterus was absent, and the right ovary and tube were not visualized in the appropriate location; instead, a right adnexal structure was buried in the right sidewall. The ovary was untwisted and fixed to the pelvic sidewall. chromosomes were 46,XX, and her hormonal evaluation was normal. Ovarian torsion and mullerian agenesis are rarely reported. An association between the lax attachment of the adnexa and torsion may be a contributing factor in this condition.- - - - - - - - - - ranking = 0.015583079014587keywords = tube (Clic here for more details about this article) |
4/8. uterus-like ovarian mass presenting near menarche.An eleven year-old girl presented with right lower quadrant abdominal pain near menarche that coincided with the onset of her most recent menstrual period. A smooth-lined cystic structure with a thick wall was discovered in the right adnexa at surgery that was adherent to a multicystic ovary and a dilated fallopian tube. Histologic examination determined the lesion to be a uterus-like ovarian mass. The patient had a history of a resected right pelvic kidney at four months of age. This case is believed to represent a congenital malformation of the urogenital system rather than smooth muscle metaplasia of ovarian stromal cells.- - - - - - - - - - ranking = 1keywords = fallopian tube, tube (Clic here for more details about this article) |
5/8. Apparent Ruvalcaba syndrome with genitourinary abnormalities.The Ruvalcaba syndrome is a rare malformation syndrome characterized by skeletal dysplasia, facial anomalies, and mental retardation. We report on a 22-year-old woman with severe growth and mental retardation and numerous manifestations characteristic of the Ruvalcaba syndrome. In addition, she has several anomalies not previously described in the Ruvalcaba syndrome, including upslanting palpebral fissures, torus palatinus, hiatal hernia with gastroesophageal reflux, recurrent respiratory infections, pectus excavatum, equinovarous deformity, hypotonia, unilateral renal hypoplasia, an accessory ovary, and atretic fallopian tube. review of published reports of Ruvalcaba syndrome confirms variability of the clinical and radiographic changes. Findings present in at least 50% of reported patients include mental retardation, short stature, pubertal delay, an abnormal nose (usually beaked) with hypoplastic nasal alae, microstomia with narrow maxilla, thin upper lip vermilion, broad hips, small hands, joint limitation, short fingers and toes, and vertebral abnormalities. Because 5 of the reported patients had renal abnormalities, a renal ultrasound or contrast study is indicated in the evaluation of these patients. Additional reports, particular from multiplex families, will be important to better characterize this syndrome.- - - - - - - - - - ranking = 1keywords = fallopian tube, tube (Clic here for more details about this article) |
6/8. Diphallus with duplication of cloacal derivatives: report of a rare case.We report on a 3-month-old infant who had diphallus and a mass on the penis. On the undersurface of the mass there were 2 prolapsed mucosal lined tubes and catheterization revealed 2 blind tracts. The rest of the mass was covered by skin. On top of the mass was a pseudophallus with rugae on either side, suggesting formation of a scrotum. Duplication of the urethra and epispadias also were diagnosed. The bladder was single with good sphincteric control. We believe that the mass represents a duplication of the derivatives of primitive ectodermal and endodermal cloaca.- - - - - - - - - - ranking = 0.0077915395072936keywords = tube (Clic here for more details about this article) |
7/8. A rare association of myelomeningocele with cloacal duplication malformation.A Jordanian baby girl, born prematurely at 36 weeks gestation, was found to have myelomeningocele associated with duplication of the rectum, urinary bladder, ureters and vagina. This is most likely to have resulted from duplication of the cloaca during early development. The case demonstrates that duplication of the cloaca, albeit rate, may occur in combination with major malformations of the neural tube. Also, the question is raised as to whether or not this is a modification of the VACTERL syndrome since the child also had a cardiac defect.- - - - - - - - - - ranking = 0.0077915395072936keywords = tube (Clic here for more details about this article) |
8/8. Cloacal anomalies: role of vesicostomy.The purpose of this report is to discuss the use of vesicostomy in the treatment of cloacal anomalies. In 4 years, the authors have performed primary reconstruction in four children who had cloacal anomalies. Three children had hydrocolpos, which in two cases failed to decompress with tube vaginostomy or clean intermittent catheterization. In both cases, cutaneous vesicostomy effectively prevented urinary sepsis, which allowed the children to thrive before definitive reconstruction was performed. Vesicostomy is technically simple to perform and is easily reversed without loss of bladder volume. Further, it lends itself well to definitive reconstruction of cloacal anomalies.- - - - - - - - - - ranking = 0.0077915395072936keywords = tube (Clic here for more details about this article) |