Restless Genital Syndrome Before and After Clitoridectomy for Spontaneous Orgasms: A Case Report

IntroductionFemales despairing of restless genital syndrome (ReGS) may request clitoridectomy for treatment of unwanted genital sensations.AimThe aim of this study was to report persistence of ReGS despite clitoridectomy.MethodsFollowing a clitoridectomy for spontaneous orgasms, a 77-year-old woman was referred to our clinic for persistent unwanted genital sensations and feelings of imminent orgasm. An in-depth interview, routine and hormonal investigations, electroencephalography (EEG) and magnetic resonance imaging (MRI) of the brain and pelvis were performed. The localizations of genital sensations were investigated by manual examination of the ramus inferior of the pubic bone (RIPB) and by sensory testing of the skin of the genital area with a cotton swab.Main Outcome MeasuresThe main outcome measures included sensitivity of dorsal nerve of the clitoris in RIPB and MRI-pelvis.ResultsGenital dysesthesias, paresthesias, intolerance (allodynia) for tight clothes, aggravation of symptoms during sitting, restless legs, and overactive bladder were diagnosed. Laboratory assessments, and EEG and MRI of the brain were in agreement with aging, but all results were within the normal range. MRI of the pelvis disclosed varices of the uterus and of the left ovarian vein, and a visible scar in the region of the clitoris. Sensory testing of the genital area showed various points of static mechanical hyperesthesia at the left dermatome of the pudendal nerve. Manual examination of the RIPB also elicited the genital sensations at the left side of the vagina at about the 3 o'clock position.ConclusionsThis patient fulfilled all clinical criteria of ReGS that is believed to be caused by neuropathy of the left pudendal nerve. Clitoridectomy abolished spontaneous orgasms for a great part but not completely, and it did not diminish the typical dysesthesias, paresthesias, and feelings of imminent orgasms that typically belong to ReGS. Clitoridectomy is no optional treatment of ReGS. There is a need for publications of ReGS in general medical journals. Waldinger MD, Venema PL, van Gils APG, Schutter EMJ, and Schweitzer DH. Restless genital syndrome before and after clitoridectomy for spontaneous orgasms: A case report.     

Restless Legs Syndrome and Severe Preeclampsia: A Case-Control Study

ObjectiveThis study sought to determine whether there is an association between restless legs syndrome (RLS) and severe preeclampsia using a case-control study design.MethodsA total of 147 patients with severe preeclampsia and 147 patients with normal pregnancies were evaluated for symptoms of RLS. In the first phase, before or immediately after delivery, participants were given a questionnaire on common complaints experienced during pregnancy. Mixed with these complaints were the symptoms that comprised the diagnostic criteria for RLS. If a participant indicated she met the diagnositic criteria, she was informed about RLS. In the second phase, a severity evaluation was performed in this population using the International Restless Legs Syndrome Study Group Rating Scale.ResultsAmong the participants, independent of the presence of preeclampsia, 13.61% met the criteria for a diagnosis of RLS. There was no statistical difference between groups (severe preeclampsia: 12.93% vs. controls: 14.29%; odds ratio [OR] 0.89; 95% CI 0.46–1.74, P = 0.37). After analysis, 65% of patients with RLS had a score on the International Restless Legs Syndrome Study Group Rating Scale compatible with “very severe” or “severe” RLS. There was again no statistical difference between groups for the combination of “severe” and “very severe” scoring criteria (severe preeclampsia: 68.42% vs. controls: 61.90%; OR 1.33; 95% CI 0.36–4.93, P = 0.66) and “very severe” alone (severe preeclampsia: 21.05% vs. controls: 4.76%; OR 5.33; 95% CI 0.54–52.73, P = 0.11).ConclusionThe prevalence of RLS among pregnant women in our study was in accordance with the medical literature and avoided the probable bias caused by the high number of other symptoms experienced during pregnancy. There were no significant differences between normotensive participants and those with severe preeclampsia. In general, symptom severity was high, with a tendency toward greater severity in patients with severe preeclampsia, but this difference did not reach statistical significance.     

Female Genital Hair-Thread Tourniquet Syndrome: A Case Report and Literature Systematic Review

“Hair-thread tourniquet syndrome” (HTTS) describes the condition in which fibers of hair or thread wrap around an appendage (ie, toes, fingers, genital structures, tongue, uvula, and neck), eventually causing ischemia and tissue necrosis. To date, few cases of female genitalia HTTS have been described. We report a case of female genitalia HTTS in a 5-year-old girl and report the state of the art by systematically reviewing all existing evidence about female genital HTTS. A total of 29 studies, describing a total of 34 patients, were identified. The presence of a hair-thread wrapping genitalia should be suspected in prepubertal girls complaining of genital pain associated with vulvar/vaginal swelling, wide-based gait, and voiding symptoms. Genital examination disclosing an extremely tender, swollen, and erythematous lesion on the clitoris or labia minora encircled by a hair confirms the diagnosis. The aim of the management is to remove the hair-thread in the shortest time possible, with the use of forceps, scissors, or scalpels, and this is often performed under sedation/anesthesia because of the patient's pain reaction. When the hair-thread is difficult to find or when the lesion is necrotic, excision of the lesion itself can be the only option. Complications include partial or total amputation because of tissue necrosis and recurrence.     

Endometriosis and infertility: Insights into the causal link and management strategies

When evaluating the correlation between endometriosis and infertility with application of the evidence-based guidelines to establish causality in medicine, it becomes apparent that endometriosis causes infertility. This is supported by a strong and consistent association between the two in various settings (prevalence, natural conception, intrauterine insemination (IUI), and assisted reproductive technologies (ARTs)), evidence for a temporal relation, arguments for a dose-response gradient, and proven effects of the removal of lesions on infertility. Next to surgical treatment of endometriosis lesions, medically assisted reproduction treatments such as IUI and ART such as in vitro fertilization are cornerstones of the management of endometriosis-related infertility. Because the revised American Society for Reproductive Medicine (rASRM) staging system is poorly correlated with pregnancy rates as opposed to the Endometriosis Fertility Index (EFI), the latter should be used as the preferred clinical tool to counsel patients on their postoperative fertility management options.     

Recent Insights into the pathogenesis of pre-eclampsia

Pre-eclampsia is more than pregnancy induced hypertension. The emerging view described in this presentation is that pre-eclampsia is secondary to the interactions of reduced placental perfusion with diverse maternal factors that alter endothelial function. The maternal contribution is from factors that antedate pregnancy and are influenced by the usual metabolic adaptations of pregnancy. The endothelium and other targets for the effects of these interactions are more sensitive to insults during pregnancy because of activation of the inflammatory cascade as a normal part of pregnancy. At least part of the response to reduced placental perfusion may be a fetal adaptive response to attempt to overcome the reduced delivery of nutrients. A reasonable convergence point for the interaction is at the level of oxidative stress. This hypothesis has both encouraging and discouraging corollaries. The diversity of maternal factors argues that there will be no single gene to explain the disorder and no single 'magic bullet' to treat the disorder. However, it is encouraging that the recognition of maternal predisposition to the disorder directs therapy to prevent pre-eclampsia at a specific target in subsets of women. Finally, the suggestion that some of the maternal alterations are due to fetal adaptive responses encourages careful choices of agents and meticulous infant follow up in well planned clinical trials.     

Genital rhabdomyosarcoma: current management and review of the literature

Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and 20 per cent of these cancers involve the pelvis or genitourinary system. Radical pelvic surgery, such as exenteration, was considered at one time to be the standard treatment for this cancer which carried a very poor prognosis. Over the past 15 years, a combined modality approach to treating rhabdomyosarcoma, using chemotherapy, radiotherapy, and less radical surgery, has evolved and survival rates have improved. This paper presents a case of rhabdomyosarcoma involving the perineum which was treated by combination chemotherapy of Vincristine, Actinomycin-D, and Cytoxan, followed by wide local excision, interstitial and external beam radiotherapy, and postoperative chemotherapy. The literature on pelvic rhabdomyosarcoma is reviewed and the current approach to treating this cancer using multimodal therapy is discussed.     

Puerperal Infections of the Genital Tract: A Clinical Review

Puerperal genital tract infections, although less common in the 21^st century, continue to affect maternal mortality and morbidity rates in the United States. Puerperal genital tract infections include endometritis as well as abdominal and perineal wound infections. These infections interrupt postpartum restoration, increase the potential for readmission to a health care facility, and can interfere with maternal‐infant bonding. In addition, unrecognized or improperly treated genital tract infection could extend to other sites via venous circulation or the lymphatic system and increase the risk of severe complications or sepsis. Midwives are leaders in education, low rates of intervention, and prompt recognition of deviation from normal. Because puerperal genital tract infection usually begins after discharge, detailed education for women will encourage preventative health care, prompt recognition, and treatment.     

Insights into the pathogenesis and natural history of fetuses with multicystic dysplastic kidney disease.

To better delineate the natural history of multicystic displastic kidney disease (MCDKD) and provide insights into the pathogenesis of this condition, we report our experience in 102 prenatally detected cases. MCDKD is most commonly an incidental finding on prenatal ultrasound examination. The abnormality may be unilateral (76 per cent) or bilateral (24 per cent). In unilateral cases, abnormality of the contralateral kidney is common (33 per cent). Associated non-renal abnormalities occur frequently with both unilateral (26 per cent) and bilateral (67 per cent) MCDKD, and increase the risk for an abnormal chromosome study. Males are more likely to be affected than females with a ratio of 2.4:1, but females are twice as likely to have bilateral MCDKD and associated non-renal abnormalities, and four times more likely to have an abnormal chromosome study. We suggest that the option of chromosomal analysis should be discussed with all patients diagnosed with MCDKD in their fetus, if there is bilateral renal involvement or if an associated non-renal abnormality is present. Unilateral MCDKD without associated renal or non-renal abnormalities was not associated with an abnormal chromosome study, and resulted in favourable outcomes. While unilateral MCDKD, lack of associated anomalies, normal chromosome study and adequate amniotic fluid are all reassuring findings, a complete neonatal urologic work-up should be performed in all newborns. We believe the evaluation should include voiding cystourethrography to rule out vesicoureteral reflux. Our findings allow more precise counselling of patients regarding prognosis, and subsequent management of the fetus found to have MCDKD.     

Abnormal Uterine Bleeding and Cancer of the Genital Tract

All patients in Tasmania admitted to hospital for investigation of abnormal uterine bleeding in 1987 and 1988 were studied. A total of 4,318 patients were investigated. Four of 539 (0.7%) patients with intermenstrual or postcoital bleeding and 31 of 538 (5.8%) patients with postmenopausal bleeding were found to have endometrial cancer. Of the 3,421 patients investigated for 'heavy' periods (menorrhagia) there was no case of endometrial cancer. The detection rate of endometrial cancer on dilatation and curettage before the menopause is extremely low. Patients presenting with menorrhagia should not be treated surgically in the first instance unless an obvious organic cause is present.     

多囊卵巢综合征病理生理的一些新认识

多囊卵巢综合征（PCOS）是以长期无排卵及高雄激素为特征的内分泌综合征,以不孕、多毛、无排卵、月经不调等为主要临床表现。PCOS超声下卵巢呈明显多囊性改变,并伴促性腺激素分泌异常。雄激素过多合成在PCOS发病中起关键性作用,膜细胞对颗粒细胞反应性增高、垂体分泌促黄体激素增多以及高胰岛素血症可能是卵巢雄激素合成过多的机制。胰岛素抵抗和肥胖的出现则可能使PCOS临床症状加剧。雄激素、胰岛素、促性腺激素和肥胖间的相互作用促成PCOS患者的生殖表型。