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Background: Atresia of the uterine cervix is an uncommon Müllerian anomaly. Total hysterectomy remains the classical treatment of this malformation. The purpose of this study was to evaluate functional results and reproductive performance of women who had conservative surgical procedure.Methods and Results: The medical records of 18 patients admitted to our centre between 1969 and 1998 for the treatment of uterine cervix atresia, were reviewed. Seven women had an associated high vaginal aplasia. Fifteen women had a history of abdominal or pelvic surgery before referral, with an unsuccessful attempt at canalization in five cases. Associated pelvic endometriosis or adhesions were observed in 12 cases. The utero-vaginal anastomosis procedure was performed successfully in all cases. A secondary stenosis of the anastomosis occurred in one case and this required canalization. Median follow-up after surgery was 4.5 years. Sexual intercourse was satisfactory for the 12 patients who began sexual activity. Ten patients had a pregnancy, four of which resulted in a total of six successful spontaneous pregnancies. Of the six remaining women, five had an evident cause of infertility.Conclusions: Utero-vaginal anastomosis should be proposed in women with congenital atresia of the uterine cervix, even when it is associated with vaginal aplasia. Early diagnosis and surgery appear necessary to avoid the development of pelvic associated lesions. [ABSTRACT FROM AUTHOR]

The possibility of using first trimester maternal serum human chorionic gonadotrophin (HCG) profiles to predict fetal growth retardation (FGR) was tested in 236 women with singleton pregnancies obtained after in-vitro fertilization (IVF). Pregnancies were monitored by serial analysis (two or more) of serum HCG at at least 48 h intervals. Serum was obtained between the 13th and the 35th day after conception (i.e. on the day of IVF). Early miscarriage occurred in 23.7% and FGR in 10.9% of pregnancies. Serum HCG profiles were higher than the 90th and lower than the 10th percentile in 12.3% and 19.5% of the cases respectively. FGR was significantly more frequent in women with serum HCG profiles lower than the 10th percentile than in women with normal profiles (45.5% versus 7.2%; P < 0.001), with a relative risk of 6.5 (95% confidence interval 2.7-15.6). FGR rates were similar in women with normal and high profiles of serum HCG. Pre-eclampsia and premature delivery rates were similar in women with normal and abnormal profiles of serum HCG. First trimester serum HCG should be further investigated as a potential marker of FGR. [ABSTRACT FROM AUTHOR]

Our purpose was to analyse the reproductive performance of women with obstructed hemivagina after surgical treatment. After laparoscopic exploration of 42 cases (mean age: 18 years), didelphic and complete septate uterus were found in 78 and 22% of cases respectively. Resection of vaginal septum and hemihysterectomy with ipsilateral hemicolpectomy were performed in 88% and 12% of the cases, respectively, between 1970 and 1997. Long-term results were assessed by a questionnaire and obtained for 38 patients (mean years after treatment and range: 6.5; 1-23). Dysmenorrhoea and abdominal pain were resolved in 87% and 100% of the cases, respectively. Nine patients experienced 20 pregnancies (13 living children, four early spontaneous abortions, two early terminations and one ectopic pregnancy). Nine offspring (69% of live births) were delivered after 37 weeks. Four patients had four pregnancies ipsilateral to blind hemivagina after vaginal septum resection (two living children, one early spontaneous abortion and one ectopic pregnancy). These results suggest that laparoscopic exploration and resection of vaginal septum are the appropriate treatments for obstructed hemivagina. Subsequent reproductive performance was comparable to that reported following treatment of the associated uterine malformation. [ABSTRACT FROM AUTHOR]

Abstract Background Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and the upper part of the vagina in women showing normal development of secondary sexual characteristics and a normal 46, XX karyotype. The uterovaginal aplasia is either isolated (type I) or more frequently associated with other malformations (type II or Müllerian Renal Cervico-thoracic Somite (MURCS) association), some of which belong to the malformation spectrum of DiGeorge phenotype (DGS). Its etiology remains poorly understood. Thus the phenotypic manifestations of MRKH and DGS overlap suggesting a possible genetic link. This would potentially have clinical consequences. Methods We searched DiGeorge critical chromosomal regions for chromosomal anomalies in a cohort of 57 subjects with uterovaginal aplasia (55 women and 2 aborted fetuses). For this candidate locus approach, we used a multiplex ligation-dependent probe amplification (MLPA) assay based on a kit designed for investigation of the chromosomal regions known to be involved in DGS. The deletions detected were validated by Duplex PCR/liquid chromatography (DP/LC) and/or array-CGH analysis. Results We found deletions in four probands within the four chromosomal loci 4q34-qter, 8p23.1, 10p14 and 22q11.2 implicated in almost all cases of DGS syndrome. Conclusion Uterovaginal aplasia appears to be an additional feature of the broad spectrum of the DGS phenotype. The DiGeorge critical chromosomal regions may be candidate loci for a subset of MRKH syndrome (MURCS association) individuals. However, the genes mapping at the sites of these deletions involved in uterovaginal anomalies remain to be determined. These findings have consequences for clinical investigations, the care of patients and their relatives, and genetic counseling.

Cell-mediated immunity directed against human papillomavirus 16 (HPV-16) antigens was studied in six patients affected with grade 3 vulvar intraepithelial neoplasia (VIN3, also known as bowenoid papulosis). Five of the patients presented with a chronic and persistent disease that relapsed after destructive treatments. They showed no detectable anti-HPV blood T-cell responses and no T-cell intraepidermal vulvar infiltrate containing both CD4+ and CD8+ lymphocytes. The last patient had a complete clearance of viral lesions, 8 months after disease onset and 2 months after electrocoagulation of <50% of the VIN3 lesions. She showed high frequency anti-E6 and anti-E7 effector blood T cells by ex vivo ELISpot-IFNgamma assay before clinical regression. Immunohistochemical study of her vulvar biopsy revealed a marked dermal infiltrate containing a majority of CD4+ T lymphocytes and an epidermal infiltrate made up of both CD4(+) and CD8(+) T cells. This seems to be the first evidence of an association between spontaneous regression of VIN3 lesions and HPV-specific T-cell responses detectable in the blood. Hence, an increase of HPV-specific effector T lymphocyte responses by vaccine-based therapeutic strategies might be useful to clear the lesions in bowenoid papulosis disease.