Technical considerations and mid-term follow-up after vaginal hysterocolpectomy with colpocleisis for pelvic organ prolapse.

OBJECTIVES: At the time of controversies on surgical treatment of pelvic organ prolapse, our aim was to describe an effective technique of hysterocolpectomy with colpocleisis for elderly patients not wishing to maintain vaginal sexual activity and present mid-term results including pelvic floor symptoms and quality of life, patient satisfaction and surgical complications using validated scores. STUDY DESIGN: We conducted a retrospective study of all patients having undergone this surgery between June 2006 and June 2016. Women were examined using POP-Q classification and completed validated questionnaires concerning symptoms and quality of life before and after the surgery. Patient satisfaction was assessed using the PGI-I. Complications were described according to the Clavien-Dindo classification. RESULTS: During the 10-year period, 37 women underwent the surgery with a mean age at surgery of 81.2 years (range: 61-93 years). One per-operative complication occurred (a rectal wound that was sutured) and five Clavien-Dindo grade 3b postoperative complications. Three repeat operations were necessary within 15 days; one suburethral sling had to be lowered because of urinary retention; one tension-free vaginal tape had to be unilaterally sectioned for acute urinary retention; and one woman presented a pararectal abscess requiring surgical drainage. The mean duration of hospitalization was 5.5 (+/-4.2) days. The mean follow-up time was 44.1 (±30.1) months. All symptoms and quality of life scores decreased significantly after the surgery and patient satisfaction was good (PGI-I score = 1.55 +/-0.8). CONCLUSIONS: Hysterocolpectomy with colpocleisis appears to be an effective treatment with a high level of patient satisfaction among the elderly.

Associated Lichen Sclerosis Increases the Risk of Lymph Node Metastases of Vulvar Cancer.

The most important prognostic factor in vulvar cancer is inguinal lymph node status at the time of diagnosis, even in locally advanced vulvar tumors. The aim of our study was to identify the risk factors of lymph node involvement in these women, especially the impact of lichen sclerosis (LS). We conducted a retrospective population-based cross-sectional study in two French referral gynecologic oncology institutions. We included all women diagnosed with a primary invasive vulvar cancer. Epithelial alteration adjacent to the invasive carcinoma was found in 96.8% (n = 395). The most frequently associated was LS in 27.7% (n = 113). In univariate analysis, LS (p = 0.009); usual type VIN (p = 0.04); tumor size >2 cm and/or local extension to vagina, urethra or anus (p < 0.01), positive margins (p < 0.01), thickness (p < 0.01) and lymphovascular space invasion (LVSI) (p < 0.01) were significantly associated with lymph node involvement. In multivariate analysis, only LS (OR 2.3, 95% CI [1.2-4.3]) and LVSI (OR 5.6, 95% CI [1.7-18.6]) remained significantly associated with positive lymph node. LS was significantly associated with older patients (p = 0.005), anterior localization (p = 0.017) and local extension (tumor size > 2 cm: p = 0.001). LS surrounding vulvar cancer is an independent factor of lymph node involvement, with local extension and LVSI.

Vulvar Arteriovenous Malformation in a 16-Year-Old Girl.

BACKGROUND: Congenital arteriovenous malformations (AVMs) are infrequent but represent a serious medical challenge because of their unpredictable progression and high hemodynamic activity. CASE: We report on the treatment of a voluminous vulvar AVM in a 16-year-old girl. After failure of medical therapy, we performed a radical surgical resection with preliminary embolization and flap reconstruction. SUMMARY AND CONCLUSION: A multidisciplinary approach is required for AVM management. Early intervention and complete surgical resection combined with preliminary embolization represents the ideal therapy.

Sexual and functional results after creation of a neovagina in women with Mayer-Rokitansky-Küster-Hauser syndrome: a comparison of nonsurgical and surgical procedures.

OBJECTIVE: To compare nonsurgical and surgical procedures for creation of a neovagina in women with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome in terms of sexual satisfaction. STUDY DESIGN: We report a cross-sectional study of 91 women with MRKH syndrome undergoing a neovagina creation procedure. They were members of the French National Association of Women with MRKH syndrome. We analyzed all answers to a questionnaire mailed to each woman. The questionnaire solicited short answers concerning the diagnosis and the neovagina procedure, and included the standardized FSFI (Female Sexual Function Index) questionnaire. All analyses were performed using the chi-squared test and Student's t-test. A p-value of <0.05 was considered statistically significant. RESULTS: Forty women answered the questionnaire. Twenty had been treated by Frank's method (non-surgical group) and 20 had undergone a surgical procedure, sigmoid vaginoplasty (12 cases) or Davidov's technique (8 cases) (surgical group). The mean time after neovagina creation was 7 years (range 1-44 years). The population characteristics did not differ significantly between the nonsurgical and surgical groups. The total FSFI score indicated good and similar functional results in the two groups (25.3±7.5 versus 25.3±8.0). CONCLUSIONS: Functional sexual outcomes after nonsurgical and surgical methods were similar. Therefore, the Frank's method should be proposed as first line therapy because it is less invasive than surgical procedures. In the case of failure of this technique or of refusal by the patient, surgical reconstruction may then be offered.

Utero-vaginal aplasia (Mayer-Rokitansky-Küster-Hauser syndrome) associated with deletions in known DiGeorge or DiGeorge-like loci.

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.

Phenotyping and genetic studies of 357 consecutive patients presenting with premature ovarian failure.

OBJECTIVE: Premature ovarian failure (POF) encompasses a heterogeneous spectrum of conditions, with phenotypic variability among patients. The etiology of POF remains unknown in most cases. We performed a global phenotyping of POF women with the aim of better orienting attempts at an etiological diagnosis. DESIGN AND METHODS: We performed a mixed retrospective and prospective study of clinical, biological, histological, morphological, and genetic data relating to 357 consecutive POF patients between 1997 and 2008. The study was conducted at a reproductive endocrinology referral center. RESULTS: Seventy-six percent of the patients presented with normal puberty and secondary amenorrhea. Family history was present in 14% of the patients, clinical and/or biological autoimmunity in 14.3%. Fifty-six women had a fluctuating form of POF. The presence of follicles was suggested at ultrasonography in 50% of the patients, and observed in 29% at histology; the negative predictive value of the presence of follicles at ultrasonography was 77%. Bone mineral density alterations were found in 58% of the women. Eight patients had X chromosomal abnormalities other than Turner's syndrome, eight other patients evidenced FMR1 pre-mutation. Two other patients had autoimmune polyendocrine syndrome type 2 and 1. CONCLUSION: A genetic cause of POF was identified in 25 patients, i.e. 7% of the whole cohort. POF etiology remains most often undiscovered. Novel strategies of POF phenotyping are in such content mandatory to improve the rate of POF patients for whom etiology is identified.

Laparoscopic-assisted uterovaginal anastomosis for uterine cervix atresia with partial vaginal aplasia.

This case report describes the surgical technique of laparoscopic-assisted uterovaginal anastomosis. At a tertiary university hospital, a girl with uterine cervix atresia and vaginal aplasia underwent laparoscopic-assisted uterovaginal anastomosis. We assessed feasibility and anatomic outcome. The operating time was 2 hours and 45 minutes. No perioperative or postoperative complications occurred. The hospital stay was 4 days. The surgical procedure resulted in adequate vaginal length (6 cm) and normal menstruation. Laparoscopic-assisted uterovaginal anastomosis without using a probe introduced by fundal incision is feasible with good anatomic results.

A suggested modification to FIGO stage III vulvar cancer.

OBJECTIVE: FIGO Stage III vulvar cancer includes tumors that invade the lower urethra, vagina, or anus, and/or tumors that have metastasized to the inguino-femoral lymph nodes of one groin. We hypothesized that locally advanced stage III vulvar cancer and regional metastatic stage III vulvar cancer (lymph node involvement) have different prognoses. METHODS: Using the Surveillance, Epidemiology, and End Results (SEER) registry public use data tapes, we identified patients diagnosed with vulvar carcinoma from 1988 through 2004. Overall survival (OS) was measured as the time from diagnosis to the date of death or last follow-up. We used the Kaplan-Meier method to estimate OS and the log-rank test to assess for differences between patient groups. The staging performance was quantified with respect to discrimination. RESULTS: The study cohort included 895 patients. The survival difference between stage III patients with locally advanced vulvar cancer and stage III patients with regional metastatic node(s) disease was highly significant (P<10(-10)). The 5-year and 10-year OS of patients with locally advanced vulvar tumors without metastatic nodes were 62% and 47%, respectively. The 5-year and 10-year OS of patients with regional metastatic node(s) disease were 39% and 27%, respectively. Separating locally advanced stage III and regional metastatic stage III disease would improve discrimination (concordance index: 72% vs 69% with the actual staging system). CONCLUSION: Involvement of the inguinal lymph nodes in FIGO (1988) stage III patients carries a significantly worse prognosis compared with invasion of the lower urethra, vagina or anus alone. This difference in prognosis would favor restaging these two entities.

Sonography of the clitoris.

INTRODUCTION: The prevalence of invasive procedures in diagnosing female sexual dysfunctions and pathologies is high. There is a need for a less invasive evaluation tool and medical imaging of the clitoris may be a solution. The clitoris has already been studied with nuclear magnetic resonance but there are very few sonographic 2D and 3D studies despite the fact that it is a simple, noninvasive, and inexpensive method. AIM: This study aims at determining the feasibility of using ultrasound (US) techniques to image the clitoris in sufficient detail to permit evaluation of anatomy for possible use in study. METHODS: The ultrasounds were performed in five healthy volunteers with the Voluson GE Sonography system (GE Healthcare Ultrasound, Zipf, Austria), using one 12-MHz flat probe. MAIN OUTCOME MEASURES: The clitoral body's diameter, the length of the raphe. RESULTS: The three planes-the cross-section, sagittal section, and coronal section-were revealed making it possible to study the entire organ. CONCLUSION: The sonography is a simple, inexpensive, noninvasive mean which might help for the evaluation of this organ.

Development and validation of a nomogram for predicting outcome of patients with vulvar cancer.

OBJECTIVE: To construct and validate a nomogram to predict relapse-free survival of patients treated for vulvar cancer. METHODS: Data from 244 patients treated for vulvar cancer at a single institution (Creteil, France) were used as a training set to develop and calibrate a nomogram for predicting relapse-free survival and local relapse-free survival. We used bootstrap resampling for the internal validation and we tested the nomogram on an independent validation set of patients (Torino, Italy) for the external validation. RESULTS: The nomograms were based on a Cox proportional hazards regression model. Covariates for the relapse-free survival model included age, T stage, number of metastatic nodes, bilateral lymph node involvement, omission of the lymphadenectomy, margin status, lymphovascular space invasion, and depth of invasion. The concordance indices were 0.85 and 0.83 in the training set before and after bootstrapping, respectively, and 0.83 in the validation set. The predictions of our nomogram discriminated better than did the International Federation of Gynecology and Obstetrics stage (0.83 compared with 0.78, P = .01). The calibration of our nomogram was good. In the validation set, 2-year and 5-year relapse-free survival were well predicted with less than 5% difference between the predicted and observed survivals for each quartile. A nomogram for predicting local relapse was also developed. CONCLUSION: We have developed nomograms for predicting distant and local relapse of vulvar cancer at 2 and 5 years and validated them both internally and externally. These nomograms will be freely available on the International Society for the Study of Vulvovaginal Disease Web site. LEVEL OF EVIDENCE: III.

Unusual presentation of Bartholin's gland duct cysts: anterior expansions.

The medical records of 32 patients who underwent surgery for a vulvar mucinous cyst located between the urethral meatus and the fourchette were reviewed. Nineteen (59%) patients had been referred to our centre because of cyst recurrence after incision or excision. The majority of the cysts were lined by a single layer of tall columnar cells with zones of squamous metaplasia. Subacute or chronic bartholinitis was demonstrated in 94% of the cases demonstrating that these cysts were anterior expansions of Bartholin's gland duct cysts. No ipsilateral recurrence was observed after cyst and gland excision.

Sentinel node localization should be interpreted with caution in midline vulvar cancer.

OBJECTIVE: Carcinomas of the vulva situated on the midline or close to it, are supposed to have a bilateral lymphatic drainage. The aim of this study was to evaluate sentinel node identification in these tumors. METHODS: Between April 2002 and February 2004, 17 patients with operable vulvar cancer situated on, or close to the midline were entered in a prospective study. All patients underwent sentinel node identification with (99m)Tc-labelled nanocolloid (preoperative lymphoscintigraphy and intraoperative use of a handheld probe). Depending on the surgeon, intraoperative blue dye was associated. Radical excision of the tumor and routine bilateral lymphadenectomy were then performed. Sentinel nodes were sent separately for histologic examination. Negative sentinel nodes on hematoxylin/eosine were further examined with immunohistochemistry. RESULTS: One or more sentinel nodes were identified in the 17 patients and in 21 of the 34 groins. In 5 patients, the sentinel nodes were metastatic. There was no false negative (negative sentinel node and metastatic non-sentinel node). In 13 patients, lymphoscintigraphy and then intraoperative identification suggested a unilateral drainage of the tumor with sentinel nodes localized in only one groin. Among these 13 patients, 3 groins with no sentinel node identified contained in fact massively metastatic nodes. CONCLUSION: Unilateral finding of a sentinel node in tumors of the midline does not preclude a metastatic node in the other groin. Lymph node assessment should remain bilateral in these lesions.

Spontaneous regression of grade 3 vulvar intraepithelial neoplasia associated with human papillomavirus-16-specific CD4(+) and CD8(+) T-cell responses.

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.

Maternal and perinatal outcomes during expectant management of 239 severe preeclamptic women between 24 and 33 weeks' gestation.

OBJECTIVE: This study was undertaken to determine maternal and perinatal outcomes after expectant management of severe preeclampsia between 24 and 33 weeks' gestation. STUDY DESIGN: A prospective observational study of 239 women with severe preeclamptic and undelivered after antenatal steroid prophylaxis was performed. Pregnancy prolongation and maternal and perinatal morbidities were analyzed according to the gestational age at time of expectant management: 24 to 28, 29 to 31, and 32 to 33 weeks. Statistical analysis was performed by Student t test and chi(2) test. RESULTS: The days of pregnancy prolongation were significantly higher among those managed at less than 29 weeks (6) compared with the other groups (4). There were 13 perinatal deaths: 12 in those managed at less than 29 weeks and 1 in those managed at 29 to 31 weeks. Neonatal morbidities were significantly higher among those managed at less than 29 weeks compared with the other groups. There were no instances of maternal death or eclampsia. Maternal morbidities were similar among the groups. CONCLUSION: Expectant management of severe preeclampsia at 24 to 33 weeks in a tertiary care center is associated with good perinatal outcome with a minimal risk for the mother.

Frozen section examination of the endocervical margin of cervical conization specimens.

OBJECTIVE: We conducted this retrospective study to determine accuracy of frozen section examination of endocervical margin during cold knife conization. METHODS: Between June 1993 and June 2001, 310 consecutive patients underwent cervical conization for squamous intraepithelial lesion or stage IA1 cervical cancer. Before 1997, the surgical specimens of 149 patients were processed following a standard pathological procedure (historical group). After 1997, a frozen section of the upper endocervical margin was processed during surgery for 161 patients. If the upper endocervical margin was involved with intraepithelial neoplasia, the surgeon performed a second resection if possible. Results of the frozen section examination were compared with the final diagnoses to determine sensitivity, specificity, and positive and negative predictive values. The usefulness of this procedure was evaluated by comparison of positive margin status rate with the one of the historical control group. RESULTS: For the diagnosis of intraepithelial neoplasia involving the endocervical margin, the sensitivity, specificity, and positive and negative predictive values of frozen section were 91%, 100%, 100%, and 98%, respectively. Eleven patients had definitive positive endocervical margin in the frozen section group (three false negatives, six patients without additional resection, and two patients with intraepithelial neoplasia involving the upper margin of the additional resection) and 17 patients in the historical group (P =.16). CONCLUSION: Frozen section examination of the endocervical margin of cervical specimen obtained during cold knife conization is highly accurate. Its clinical relevance has to be demonstrated in a multicenter study.

Correction of uterine prolapse by the vaginal route using the uterosacral ligaments: Shirodkar procedure.

OBJECTIVE: To study long-term results of Shirodkar procedure for treatment of uterine prolapse in women wishing to retain their uterus. STUDY DESIGN: Fifty-one patients with a symptomatic uterine prolapse who desire to preserve their uterus without further childbearing were treated between 1988 and 2000. By the vaginal route, uterosacral ligaments were separated from the uterus, shortened, transposed in front of the uterine isthmus, and fixed bilaterally under the opposite lateral surface of the cervix or of the cervical stump after amputation. RESULTS: Median age of patients was 37 years. Median operating time was 120 min (range 60-190). One patient was re-operated four days after the initial surgery to drain a haematoma. Median follow-up was 81 months (range 11-134). Eight patients were lost of follow-up. Eleven (25.6%) patients underwent iterative surgery. The indication for re-operation was recurrence of prolapse in four patients, introital dyspareunia in three patients, cervix stenosis in two patients, urinary incontinence in one patient and pyosalpinx in one patient. Only one patient (2.3%) experienced a recurrence of the uterine prolapse. CONCLUSION: Shirodkar procedure is safe and effective. Long-term functional results are satisfactory but associated procedures may alter patient satisfaction.

Treatment of vaginal outflow tract obstruction in graft-versus-host reaction.

We describe five patients with vaginal stenosis that was due to chronic graft-versus-host reaction after bone marrow transplantation. All patients underwent surgical procedures followed by local corticosteroids administration. Vaginal function was restored in all cases. This satisfactory result persists in the long term.

Inguinofemoral dissection for carcinoma of the vulva: effect of modifications of extent and technique on morbidity and survival.

BACKGROUND: The aim of this study was to evaluate the impact of modifications of extent (medial inguinal and medial femoral lymphadenectomy, inguinal lymphadenectomy, inguinal and medial femoral lymphadenectomy, and inguinofemoral lymphadenectomy) and surgical technique of lymphadenectomy (including sartorius transposition, preservation of the fascia lata, and preservation of the saphenous vein) on morbidity, groin recurrence, and survival in patients with vulvar carcinoma. STUDY DESIGN: A retrospective review of 194 patients with primary squamous cell cancer of the vulva was conducted. Clinical, surgical, histopathologic, postoperative short- and longterm complications, and followup data were collected from patient records. RESULTS: Inguinal lymphadenectomy and medial inguinal and medial femoral lymphadenectomy produced about half fewer nodes than did other surgical procedures. On the other hand, number of lymph nodes removed did not differ notably between inguinofemoral lymphadenectomy and inguinal and medial femoral lymphadenectomy. Logistic regression showed that obesity was associated with increased risk of cellulitis. Age greater than 70, obesity, and extent of lymphadenectomy increased wound breakdown risk. Factors associated with leg edema persisting for more than 6 months were: extent of lymphadenectomy, sartorius transposition, and adjuvant irradiation of groin area. With a mean followup time of 38 months, neither groin recurrence rate nor disease-specific survival markedly differed according to technique of lymphadenectomy. CONCLUSION: Techniques of lymphadenectomy with preservation of fascia lata and saphenous vein are associated with a decreased risk of postoperative morbidity without jeopardizing outcomes.

Local relapse in patients treated for squamous cell vulvar carcinoma: incidence and prognostic value.

To evaluate the risk factors for local relapse in vulvar cancer patients and its impact on survival as a function of its different patterns, which include local recurrences at the primary tumor site, recurrences remote from the primary tumor, and skin bridge recurrences. Between January 1978 and June 1999, 215 patients were treated for vulvar squamous cell carcinoma. The median follow-up was 38 months. To evaluate the role of local relapse in survival, a Cox regression multivariable analysis was performed using local relapse as a time-dependent covariate. The local relapse-free survival rate was 78.6% (+/- 7.1%) at 5 years. Multivariable analyses showed that the local relapse was increased by the factors margin status and depth of invasion. Pathologic nodal status, tumor size, margin status, and depth of invasion were predictors of cancer-related death in the Cox multivariable model with fixed covariates. The contribution of local relapse for disease-specific survival at the site of the primary tumor (relative risk [RR] 6.35; 95% confidence interval [CI] 2.07, 15.76) or skin bridge recurrence (RR 6.48; 95% CI 2.54, 16.49) was highly significant, whereas the contribution of local relapse at the other sites was not (RR 2.29; 95% CI 0.53, 9.91). In this model, margin status was not significant. The risk of cancer-related death after local relapse was 58.4% (+/- 18.3%) at 1 year and 70.9% (+/- 17.6%) at 3 years.Local relapses at the site of the primary tumor or skin bridge recurrences are strong predictors for cancer-related death, but not local relapse at a distant site.