RESUMO
The Mayer-Rokitansky-Kuster-Hauser is a rare congenital anomaly characterized by lack of vaginal and uterine development variable and normal ovaries. It results from agenesis or hypoplasia Müller duct system. Cervicovaginal agenesis as part of the complex syndrome, is even rarer. We report two cases: adolescent patient with primary amenorrhea, cervicovaginal agenesis and chronic pelvic pain, and a 28-year-old patient with primary amenorrhea, congenital absence of uterus and vagina.
Assuntos
Anormalidades Múltiplas/patologia , Amenorreia/etiologia , Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Múltiplas/diagnóstico por imagem , Anormalidades Múltiplas/embriologia , Anormalidades Múltiplas/epidemiologia , Anormalidades Múltiplas/cirurgia , Adolescente , Adulto , Anormalidades Congênitas , Endometriose/etiologia , Feminino , Humanos , Histerectomia , Incidência , Rim/anormalidades , Rim/diagnóstico por imagem , Rim/embriologia , Rim/patologia , Rim/cirurgia , Ductos Paramesonéfricos/anormalidades , Ductos Paramesonéfricos/diagnóstico por imagem , Ductos Paramesonéfricos/embriologia , Ductos Paramesonéfricos/patologia , Ductos Paramesonéfricos/cirurgia , Dor Pélvica/etiologia , Fenótipo , Somitos/anormalidades , Somitos/diagnóstico por imagem , Somitos/embriologia , Somitos/patologia , Somitos/cirurgia , Coluna Vertebral/anormalidades , Coluna Vertebral/diagnóstico por imagem , Coluna Vertebral/embriologia , Coluna Vertebral/patologia , Coluna Vertebral/cirurgia , Estruturas Criadas Cirurgicamente , Ultrassonografia , Útero/anormalidades , Útero/diagnóstico por imagem , Útero/embriologia , Útero/patologia , Útero/cirurgia , Vagina/anormalidades , Vagina/diagnóstico por imagem , Vagina/embriologia , Vagina/patologia , Vagina/cirurgiaRESUMO
OBJECTIVE: To present and evaluate the histological, anatomical and functional results of the McIndoe procedure, as modified by the application of oxidized cellulose (Surgicel™) in women with vaginal agenesis. STUDY DESIGN: Eleven patients with vaginal agenesis underwent vaginoplasty using a mould that had been wrapped with oxidized cellulose. The surgeries were performed between January 2009 and January 2010. Eight of the patients had been diagnosed with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, and the remaining three had been diagnosed with cervicovaginal agenesis (CVA). The mean follow-up time was 14 months (range, 6-24 months), and it included clinical examinations and evaluation of the Female Sexual Function Index (FSFI). Neovaginal biopsies were taken at the time of surgery and 1-12 months after surgery. The histology of the samples was evaluated to determine squamous epithelialization of the neovaginal tissue over time, and the total collagen content of the neovaginas were compared with normal control subjects. For statistical analysis we employed the ANOVA test and the t-test. RESULTS: At 6 months, anatomical success was achieved in 100% of the MRKH syndrome patients (neovaginal length ≥ 6 cm), and functional success was achieved in 100% of the patients who started their sexual life (FSFI score ≥ 30). Biopsy results showed complete epithelialization of the neovagina after 5 months in all samples, and the collagen content was comparable to that of a normal vagina. One major postoperative complication occurred in a patient with CVA, which culminated in death. The uterovaginal canalization procedure was unsuccessful at creating an outflow tract for regular menses in all cases. CONCLUSIONS: The procedure described here offers patients a functional vagina by means of a simple and low-cost procedure that elicits squamous epithelialization of the neovaginal vault, with total collagen content similar to that of normal vaginal tissue. It is a potential alternative therapeutic approach for MRKH syndrome but not applicable to cases of CVA.