Variante incomum da síndrome de Herlyn- -Werner-Wunderlich: relato de caso / Unsual Herlyn-Werner-Wunderlich syndrome: case report

Malformações müllerianas correspondem a variações anatômicas do trato repro- dutor feminino. Comumente assintomáticas, o diagnóstico e a verdadeira incidên- cia são difíceis de determinar. A síndrome de Herlyn-Werner-Wunderlich, clas- sicamente descrita pela tríade útero didelfo, hemivagina cega e agenesia renal ipsilateral também pode ter variações diferentes. Em virtude da mesma origem embrionária dos tratos genital e urinário, anomalias renais devem ser investigadas nesses casos, sendo a mais comum a agenesia renal. Este artigo relata o caso de uma paciente de 18 anos, do sexo feminino, com história de piocolpo por cinco anos. Em propedêutica complementar, foi identificado útero com septação com- pleta associado a hemissepto de terço superior de vagina, formação de piocolpo e agenesia renal à direita. Apesar de não ser a definição clássica, o quadro está incluído nos casos de síndrome de Herlyn-Werner-Wunderlich.

Mullerian malformations correspond to anatomical variations of the female repro- ductive tract. Commonly asymptomatic, the diagnosis and true incidence are difficult to determine. The Herlyn-Werner-Wunderlich syndrome, classically described by the triad: uterus didelphus, blind hemivagina and ipsilateral renal agenesis, can also have different variations. Due to the same embryonic origin of the genital and urinary tracts, renal anomalies must be investigated in these cases, the most common being renal agenesis. This article reports the case of an 18-year-old female patient with a 5-year history of pyocolpus. In complementary exams, a uterus with complete septa- tion was identified, associated with a hemiseptum in the upper third of the vagina, formation of pyocolpus and renal agenesis on the right side. Despite not being the classic definition, it is included in the cases of Herlyn-Werner-Wunderlich syndrome.

Development of personalized molds for neovagina creation by 3D printer

SUMMARY Additive Manufacturing (AM), also known as Rapid Prototyping, is a set of production technologies used in the synthesis of a particular physical object by adding layers to form a part based on data generated by Computer-Aided Design (CAD) systems. These technologies are widely used to quickly create prototypes of products and tools for commercial purposes. Over time, it has also been integrated with other areas, such as healthcare, since these tools have allowed health professionals to assist in diagnoses, surgical planning, and synthesis of orthoses and prostheses for patient rehabilitation. OBJECTIVE: To develop models for the construction of dilators for the treatment of vaginal agenesis. METHODS: Use CAD software and create a physical model using AM to analyze the viability of its production in the elaboration of customized dilators for each patient. RESULTS: The production through AM provides an advantage in the development, facilitating physical alterations just by adjusting the three-dimensional models made by the software in a quick way, thus making the customization process viable. CONCLUSION: The proposed procedure for the manufacture of dilators presented good results and technological feasibility, indicating that it can be a good solution for the production and customization of gynecological devices.

RESUMO A Manufatura Aditiva (MA), também conhecida como Prototipagem Rápida, é um conjunto de tecnologias de produção utilizado na síntese de determinado objeto físico por meio da adição de camadas para formar uma peça com base em dados gerados por sistemas de projeto auxiliado por computador (CAD - Computer Aided Design). Essas tecnologias são muito utilizadas para criação de protótipos de produtos e ferramentas de maneira rápida para fins comerciais. Com o tempo, também se integrou a outras áreas, como a área da saúde, uma vez que essas ferramentas permitiram auxiliar o profissional da saúde em diagnósticos, planejamento cirúrgico e na síntese de órteses e próteses para reabilitação de pacientes. OBJETIVO: Elaborar modelos computadorizados para a construção de dilatadores para tratamento de agenesia vaginal pela impressão 3D. MÉTODO: Utilização de software CAD e criação do modelo físico por meio de MA para análise da viabilidade de sua produção na elaboração de dilatadores customizados para cada paciente. RESULTADOS: A produção por meio de MA atribui uma vantagem ao desenvolvimento, facilitando as alterações físicas apenas ajustando os modelos tridimensionais feitos pelos softwares de maneira rápida, tornando o processo de customização viável. CONCLUSÃO: O procedimento proposto para a fabricação dos dilatadores apresentou bons resultados e viabilidade tecnológica, indicando que pode ser uma boa solução de produção e customização de dispositivos ginecológicos.

Revisión pictográfica de las anomalías de los conductos de Müller por resonancia magnética / Pictographic review of Müllerian duct anomalies by magnetic resonance imaging

Las anomalías útero-vaginales o müllerianas son un grupo de patologías congénitas que se originan por un defecto del desarrollo, fusión o canalización de los conductos de Müller en la etapa embrionaria, excepcionalmente pueden deberse a una alteración adquirida. La mayoría se diagnostica en forma tardía, como resultado de estudios de infertilidad o complicaciones obstétricas tempranas o tardías. La resonancia magnética (RM) es un examen no invasivo, que no utiliza radiación ionizante, con una alta resolución de contraste, que permite la evaluación multiplanar de las malformaciones müllerianas, permitiendo su aproximación diagnóstica y caracterización, complementando otras técnicas de imágenes como la histerosalpingografía y la ecografía ginecológica. El radiólogo debe estar familiarizado con las alteraciones anatómicas de los genitales femeninos y su representación en RM permitiendo un diagnóstico diferencial apropiado, oportuno y en consecuencia, un tratamiento adecuado. Este artículo describe los hallazgos en este grupo de malformaciones según la clasificación de la American Fertility Society, la más utilizada en la literatura actual.

The uterus-vaginal or Müllerian anomalies are a group of congenital diseases that are caused by a developmental defect, merger or channeling of Müllerian ducts in the embryonic stage, exceptionally may be due to an acquired disorder. Most belatedly diagnosed as a result of studies of infertility or early or late obstetric complications. Magnetic resonance imaging (MRI) is a noninvasive test that does not use ionizing radiation, with high contrast resolution, which allows multiplanar evaluation of Müllerian malformations, allowing its diagnostic approach and characterization, complementing other imaging techniques such as hysterosalpingography and gynecological ultrasound. The radiologist should be familiar with the anatomical abnormalities of the female genitalia and its representation in MRI, allowing an appropriate and timely differential diagnosis, and consequently, an appropriate treatment.

Bladder acellular matrix seeding with autologous cultured vaginal smooth cells repaired rabbit vaginal defect / 中国医师杂志

Objective To evaluate the feasibility of bladder extracellular matrix (BACM) seeding with autologous cultured vaginal smooth muscle cells (VSMC) repaired rabbit vaginal defect.Methods This study included 24 female rabbits.BACM and vaginal acellular matrix (VACM) were obtained from 8 rabbits by decellularization process.The other 16 rabbits were randomly divided into experimental and control groups.Vaginal tissue biopsies ( ～ 1 cm2) were harvested from female New Zealand rabbits.VSMC were cultured and stained with a-smooth muscle actin antibodies. Cultured VSMC cells were seeded on BACM ( experimental group) or VACM ( control group) at a cell density of 1 × 107 cells/cm2.The cell-seeded matrixes were cultured for 5days.In experimental group of 8 rabbits,a 2 cm segment of vagina was resected and replaced with BACM seeding with VSMC.Then the regenerative segment was studied with histological technique by hematoxylin-eosin staining after 3,6 and 12 weeks postoperative aud vaginography was performed at 12 weeks postoperative.The 8 rabbits in control group underwent the exact same procedure as above but the vaginal defect was repaired with VACM seeding with VSMC,Results The prepared BACM and VACM were transparent,HE staining and scanning electron microscopy showed the two acellular matrix was both consisted of abundant network of fibers with the regular arrangement,without cellular debris.Primary culture of VSMC was successfully established and passaged,and was uniformly spindle-shaped in the confluent state and showed a characteristic hill and valley'formation.Immunohistochemical staining showed VSMC in culture stained positively with a-smooth muscle actin antibodies.VSMC began to adhere to the BACM 5 hour after implanting and the number of the adhered cells increased with time.Cells gradually expanded and showed the typical morphology of smooth muscle cells.Three weeks after implantation in vivo,the luminal surface of matrix was completely covered by vaginal epithelial tissue,a layer of smooth muscle cells was formed in the outer surface of the matrix.Multilayered vaginal epithelial and improved development of organized muscle bundles was observed after 6 weeks.The regenerative tissue was equivalent to the normal vaginal tissue at 12 weeks postoperatively.Vaginography demonstrated the maintenance of full patency and a wide vaginal caliber without fibrosis and graft rejection.There was no significant difference in all evaluated items between experimental and control groups.Conclusions BACM has the same regenerative process as VACM in the replacement of vaginal defect.Moreover,BACM has wider source and appears to be an suitable material for vaginal replacement.

Neocolpoplastia com alça exclusa de sigmóide / Neocolpoplasty with excluded sigmoid segment

Introdução: O emprego de alças intestinais para tratamento de agenesia de vagina remontaao início do século vinte, porém tem sido pouco popularizado. Método: Os autores apresentamsete casos em que a neocolpoplastia foi realizada com o emprego de alça exclusa desigmóide, transposta através do fundo de saco de Douglas, por incisão peritoneal, e alojadaem um túnel dissecado entre o reto e a bexiga. A extremidade inferior foi suturada ao intróitovaginal com incisão quebrada em V, e a extremidade superior fechada em fundo cego, ousuturada ao redor do colo uterino telescopado através dela, quando o útero estava presente.Resultados: A evolução é de três a dez anos. A técnica foi executada em três casos comausência de vagina associada à agenesia de útero, um caso de síndrome adrenogenital comatresia de vagina associada a útero infantil, e três casos de agenesia de vagina com presençade útero funcional. O útero foi preservado, com seu colo dentro da neovagina, permitindomenstruações normais em dois deles. Discussão: Não foram necessários, nem indicados,dilatações ou uso de moldes no período pós-operatório. Os resultados foram bons e o índicede complicações pequeno. As vaginas permitem penetração fácil. São amplas, profundas eelásticas, com lubrificação e aspecto visual normais. Conclusão: A neocolplastia mostrouseuma técnica viável com baixo índice de complicação, principalmente estenoses.

Introduction: The use of intestinal loops for treatment of vaginal agenesis remounts to thebeginning of XX century; however it has been little popularized. Methods: The authorspresent seven cases wherein the neocolpoplasty has been realized with the use of excludedsigmoid loop, transposed trough the bottom of Douglas sack by peritoneal incision andaccommodated in a dissected tunnel between the rectum and the bladder. The inferiorextremity was sutured to the vaginal introit with incision in V, and the superior extremityclosed in a blind bottom, or sutured around of the telescoped uterine cervix trough her, whenthe uterus was present. Results: The evolution is from three to ten years. The techniquewas executed in three cases with vagina absence associated to the uterus agenesis, onecase of adrenogenital syndrome with the vagina atresia associated to an infantile uterus,and three cases of vaginal agenesis with the presence of functional uterus. The uterus waspreserved with his lap inside of the neovagina, allowing normal menstruation in two ofthem. Discussion: Weren’t necessary, not even indicated, dilations or use of molds in thepostoperative period. The results were good and the index of complications was small.The vaginas allow easy penetration. They are ample, deep and elastic, with lubrication andnormal visual aspect. Conclusion: The neocolpoplasty show it to be a viable techniquewith low complication index, mainly estenoses.

MR Findings of Mayer-Rokitansky-Kuster-Hauser Syndrome: Two Cases Report

Magnetic resonance imaging (MRI) was used to study Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome in two 21-year-old women. In one case MRI revealed that fluid filled an area of the uterus measuring about 4 x 4cm, but that the ovaeies were normal ; in the other case, a rudimentary uterus and normal ovaries were seen. MRI is useful both in the planning of surgical management and in evaluating the details of pelvic anatomy and uterine morphologic characteristics.