International Society for the Study of Women’s Sexual Health (ISSWSH) Review of Epidemiology and Pathophysiology,and a Consensus Nomenclature and Process of Care for the Management of Persistent Genital Arousal Disorder/Genito-Pelvic Dysesthesia (PGAD/GPD)

BackgroundPersistent genital arousal disorder (PGAD), a condition of unwanted, unremitting sensations of genital arousal, is associated with a significant, negative psychosocial impact that may include emotional lability, catastrophization, and suicidal ideation. Despite being first reported in 2001, PGAD remains poorly understood.AimTo characterize this complex condition more accurately, review the epidemiology and pathophysiology, and provide new nomenclature and guidance for evidence-based management.MethodsA panel of experts reviewed pertinent literature, discussed research and clinical experience, and used a modified Delphi method to reach consensus concerning nomenclature, etiology, and associated factors. Levels of evidence and grades of recommendation were assigned for diagnosis and treatment.OutcomesThe nomenclature of PGAD was broadened to include genito-pelvic dysesthesia (GPD), and a new biopsychosocial diagnostic and treatment algorithm for PGAD/GPD was developed.ResultsThe panel recognized that the term PGAD does not fully characterize the constellation of GPD symptoms experienced by patients. Therefore, the more inclusive term PGAD/GPD was adopted, which maintains the primacy of the distressing arousal symptoms and acknowledges associated bothersome GPD. While there are diverse biopsychosocial contributors, there is a common underlying neurologic basis attributable to spontaneous intense activity of the genito-pelvic region represented in the somatosensory cortex and its projections. A process of care diagnostic and treatment strategy was developed to guide the clinician, whenever possible, by localizing the symptoms as originating in any of five regions: (i) end organ, (ii) pelvis/perineum, (iii) cauda equina, (iv) spinal cord, and (v) brain. Psychological treatment strategies were considered critical and should be performed in conjunction with medical strategies. Pharmaceutical interventions may be used based on their site and mechanism of action to reduce patients’ symptoms and the associated bother and distress.Clinical ImplicationsThe process of care for PGAD/GPD uses a personalized, biopsychosocial approach for diagnosis and treatment.Strengths and LimitationsStrengths and Limitations: Strengths include characterization of the condition by consensus, analysis, and recommendation of a new nomenclature and a rational basis for diagnosis and treatment. Future investigations into etiology and treatment outcomes are recommended. The main limitations are the dearth of knowledge concerning this condition and that the current literature consists primarily of case reports and expert opinion.ConclusionWe provide, for the first time, an expert consensus review of the epidemiology and pathophysiology and the development of a new nomenclature and rational algorithm for management of this extremely distressing sexual health condition that may be more prevalent than previously recognized.Goldstein I, Komisaruk BR, Pukall CF, et al. International Society for the Study of Women’s Sexual Health (ISSWSH) Review of Epidemiology and Pathophysiology, and a Consensus Nomenclature and Process of Care for the Management of Persistent Genital Arousal Disorder/Genito-Pelvic Dysesthesia (PGAD/GPD). J Sex Med 2021;18:665–697.     

Choosing between bad,worse and worst: what is the preferred mode of delivery for failure of the second stage of labor?*

Objective: To determine the preferred mode of delivery (vacuum, forceps or cesarean delivery) for second-stage dystocia.

Methods: Retrospective cohort study of women delivered by forceps, vacuum or cesarean delivery due to abnormalities of the second stage of labor. Primary outcome included neonatal and maternal composite adverse effects.

Results: A total of 547 women were included: 150 (27.4%) had forceps delivery, 200 (36.5%) had vacuum extraction, and 197 (36.1%) had cesarean section. The rate of neonatal composite outcome was significantly increased in vacuum extraction (27%) compared to forceps delivery (14.7%) or cesarean section (9.7%) (p?p?=?0.004).

Conclusion: Operative vaginal delivery was associated with reduced postpartum infection compared to cesarean section. Forceps delivery was associated with reduced risk for adverse neonatal outcome compared to vacuum extraction, with no increase in the risk of composite maternal complications.     

The Missing Uterus,the Missed Diagnosis,and the Missing Care. Mayer-Rokitansky-Küster-Hauser Syndrome in the Lives of Women in Malaysia

Study ObjectiveTo explore the effect of the diagnosis of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome on affected Malaysian women.DesignQualitative study with a quantitative component.SettingPediatric and adolescent gynecology unit at Universiti Kebangsaan Malaysia Medical Centre, Malaysia.ParticipantsTwelve women with MRKH.InterventionsFace-to-face interview and short questionnaire.Main Outcome MeasuresThematic analysis was used to understand participants’ experiences.ResultsThere were 7 themes identified: (1) delayed diagnoses; (2) doctors' roles and attitudes; (3) gender identity; (4) family and society's response; (5) reaction toward infertility; (6) managing sexual intimacy; and (7) coping mechanisms. Several participants consulted their physicians regarding their primary amenorrhea at an opportunistic setting. When they were referred to the gynecologists, they were dismayed at the lack of information given. The term, “MRKH” plays an important role to ease information-seeking. Participants felt that the doctors were insensitive toward them. Mental illness is a significant complication of MRKH. All participants acknowledged that infertility was the hardest part of the condition. The importance of blood lineage affects their outlook on childbearing options. Some were afraid of sexual intimacy and worried that they would not be able to satisfy their partners. Participants gained support and bonded with their counterparts in the MRKH support group.ConclusionA multidisciplinary approach including medical, psychological, and social support is essential for the management of MRKH. Adequate information and sexual education plays the utmost importance in preventing social-related complications of MRKH.     

Corticosteroids for the syndrome of hemolysis, elevated liver enzymes, and low platelets (HELLP): what evidence?

The fundamental premise that has governed the proposal relative to the use of corticosteroids for the purpose of disease modification in Hemolysis, Elevated Liver Enzymes, and Low Platelets (HELLP) syndrome was that pre-eclampsia is a condition characterized by an inappropriate maternal systemic inflammatory response and possibly immune-mediated impairment in maternal-fetal communication, while corticosteroids have the capacity to exercise anti-inflammatory and immunosuppressive effects. The present article reviews the evidence behind this proposal, concluding that corticosteroids administration, either antepartum or postpartum, does not improve the outcome of pregnancies affected by HELLP syndrome. The risks associated with such an approach, especially in fetuses manifesting growth restriction and absent end-diastolic flow, are also discussed. The literature published in English between 1990 and 2006 was searched for papers dealing with corticosteroids treatment for disease modification in pre-eclampsia and HELLP syndrome, using a combination of keywords including 'HELLP syndrome ', 'pre-eclampsia', 'corticosteroids', and 'maternal and fetal outcomes'. The MEDLINE bibliographic database yielded 9 studies relevant to this topic, including one retrospective analysis, 7 randomized trials, and one meta-analysis. Until more convincing data become available, corticosteroids for disease modification in women with HELLP syndrome should not be used outside the setting of an approved investigational protocol.     

Hormone profiling,including anti-Müllerian hormone (AMH), for the diagnosis of polycystic ovary syndrome (PCOS) and characterization of PCOS phenotypes

Objective was to evaluate serum anti-Müllerian hormone (AMH) levels in polycystic ovary syndrome (PCOS) and in its different phenotypes in relation to clinical, endocrine and metabolic parameters using a new automated VIDAS^® method and to compare it with the Gen II method. Study design was multi-center study including 319 PCOS women and 109 healthy controls. Serum AMH levels measured using VIDAS^® were significantly higher in PCOS women than controls (p?<?.001), and they correlated with those measured using the AMH Gen II method. An AMH cutoff value of 42.1?pmol/L distinguished PCOS women from controls with 67% sensitivity and 83% specificity. The PCOS women with three Rotterdam criteria or hyperandrogenism displayed significantly higher AMH levels compared with those with two Rotterdam criteria or normoandrogenism. In PCOS, AMH levels correlated positively with luteinizing hormone (LH), androgen and sex hormone-binding globulin (SHBG) levels and negatively with BMI, abdominal obesity, follicle-stimulating hormone (FSH), fasting glucose and insulin, and insulin resistance. In conclusion, AMH evaluated using the VIDAS^® method distinguished PCOS patients from healthy controls relatively well, especially in those with more severe phenotypes. Further studies are needed to establish whether AMH measurements can distinguish PCOS patients with different metabolic risk factors.     

Validation of the Hebrew Birth Satisfaction Scale – Revised (BSS-R) and its relationship to perceived traumatic labour

ABSTRACT

Objective and background: The Birth Satisfaction Scale-Revised (BSS-R) is a multi-dimensional measure which is recommended by international clinical guidelines for global use as the birth satisfaction outcome measure of choice. The current investigation sought to develop a Hebrew version of the BSS-R for use in the Jewish–Israeli context and examine the relationship between BSS-R domains and the perception of the experience of labour as traumatic.

Methods: Following review, translation, and back-translation for linguistic equivalence, a Hebrew version of the BSS-R (H-BSS-R) was prepared and psychometrically evaluated using key indices of validity and reliability. Complete multivariate normal data from 288 first-time Jewish Israeli mothers within two years after childbirth was entered into the analysis.

Results: The H-BSS-R was found to have a good fit to the BSS-R tridimensional measurement model, excellent internal consistency, divergent and known-group discriminant validity. Moreover, women who experienced their labour as traumatic had significantly lower H-BSS-R subscale scores than women who reported that their birth experience was non-traumatic.

Conclusion: The H-BSS-R is a robust and valid measure suitable for use with Jewish–Israeli women, as well as for investigating the relationship between traumatic labour and birth satisfaction.     

The Labour Progression Study (LaPS): Duration of labour following Zhang's guideline and the WHO partograph – A cluster randomised trial

ObjectiveTo investigate labour duration in different phases of labour when adhering to Zhang's guideline for labour progression compared with the WHO partograph.DesignA secondary analysis of a cluster randomised controlled trial.SettingFourteen Norwegian birth care units, each with more than 500 deliveries per year constituted the clusters.ParticipantsA total of 7277 nulliparous women with singleton foetus in a cephalic presentation and spontaneous onset of labour at term were included.InterventionSeven clusters were randomised to the intervention group that adhered to Zhang's guideline (n = 3972) and seven to the control group that adhered to the WHO partograph (n = 3305) for labour progression.MeasurementsThe duration of labour from the first registration of cervical dilatation (≥ 4 cm) to the delivery of the baby and the duration of the first and second stages of labour; the time-to-event analysis was used to compare the duration of labour between the two groups after adjusting for baseline covariates.FindingsThe adjusted median duration of labour was 7.0 h in the Zhang group, compared with 6.2 h in the WHO group; the median difference was 0.84 h with 95% confidence interval [CI] (0.2–1.5). The adjusted median duration of the first stage was 5.6 h in the Zhang group compared with 4.9 h in the WHO group; the median difference was 0.66 h with 95% CI (0.1–1.2). The corresponding adjusted median duration of the second stage was 88 and 77 min; the median difference was 0.18 h with 95% CI (0.1–0.3).Key Conclusions: The women who adhered to Zhang's guideline had longer overall duration and duration of the first and second stages of labour than women who adhered to the WHO partograph.Implications for practice: Understanding the variations in the duration of labour is of great importance, and the results offer useful insights into the different labour progression guidelines, which can inform clinical practice.     

Laparoscopic supracervical hysterectomy for the larger uterus (&gt;500 g): a case series and literature review

Background

In clinical practice, the current trend for surgical hysterectomy for the large uterus is by laparotomy, where vaginal hysterectomy is not feasible. The benefits of a laparoscopic approach to hysterectomy are well documented, but limited literature is available on laparoscopic hysterectomy in the larger uterus. A supracervical hysterectomy should be considered for women, where there is no contraindication to a residual cervix.

Objective

To compare surgical outcomes for laparoscopic supracervical hysterectomy (LSH) in the large uterus (equal or greater than 500 g) compared with normal (<500 g), results were compared to the limited literature on this topic.

Methods and study design

A retrospective review was undertaken of 207 women who underwent an LSH between 2005 and 2015 at Whipps Cross University hospital. The operative outcome of patients undergoing surgery, for uteri both greater than 500 g and less than 500 g, was analysed. Data included patient demographics, intra-operative details, and complications. A literature search was performed using keywords ‘laparoscopic supracervical hysterectomy’ and ‘laparoscopic sub-total hysterectomy’ which yielded six informative articles.

Results

In the study group of 207 patients, 67 had a large uterus (56 patients 500–1000 g, 11 patients >1000 g). The operation time and intra-operative blood loss were both greater in the larger uterus cohort (P < 0.0001 and P = 0.0021, respectively). The hospital stay and intra-operative complication rate were similar for the two study cohorts (six for the study, n = 207). Review of the literature revealed six relevant studies evaluating patients with uteri greater than 500 g. There was an agreement between all the studies that a larger uterus resulted in an increase in both intra-operative time and blood loss, but this did not translate to increase in hospital stay or intra-operative complications.

Conclusion

The use of LSH for the larger uterus is feasible and safe due to the low level of operative complications as demonstrated in our study and following a review of the literature.

     

Pelvic radiotherapy in patients with hydronephrosis in stage IIIB cancer of the cervix: renal effects and the optimal timing for urinary diversion?

BACKGROUND AND PURPOSE: Many patients with Stage IIIB cervix cancer (Ca) and hydronephrosis will require ureteral stenting. The timing is important as delays or prolonged overall treatment times adversely affect outcome. Our aim was to measure the effect of pelvic radiotherapy (R/T) on renal function and identify a subset of patients at high risk of acute urinary obstruction during R/T. PATIENTS AND METHODS: From 1/1/2000 to 1/1/2002, all patients with Stage IIIB cervix Ca and hydronephrosis were analysed retrospectively. To quantify the impact of pelvic R/T, all eligible patients from 1/7/2002-1/7/2004 had prospectively recorded baseline biochemistry, creatinine clearance and renal ultrasounds; these were repeated weekly to detect any change in renal function or degree of hydronephrosis. RESULTS: 13 eligible patients were analysed retrospectively, 5 with unilateral hydronephrosis with 40% requiring urinary diversion (UD). 8 had bilateral hydronephrosis, with 75% requiring UD; 50% before R/T and 35% during R/T. Average creatinine clearance (CrCl) was 74 mL/min (1.24 mL/s) in unilateral hydronephrosis , bilateral = 52 mL/min (0.87 mL/s), in those stented during R/T it was < 40 mL/min (0.67 mL/s). The resulting break in R/T was 6 and 19 days. In the prospective study, 5 patients were eligible and 4 consented. 75% had unilateral hydronephrosis and did not require UD with an average CrCl = 71 mL/min (1.19 mL/s). 1 patient with bilateral hydronephrosis had a CrCl of < 20 mL/min (0.33 mL/s) with bilateral stents placed before R/T. CONCLUSIONS: Patients with bilateral hydronephrosis and a low CrCl < 50 mL/min (0.84 mL/s) should be considered for elective UD prior to R/T. Pelvic R/T did not induce any deterioration in renal function or degree of hydronephrosis.     

Occupation and risk of cesarean section: study based on the perinatal survey of Baden-Württemberg,Germany

OBJECTIVES: The aim of this study was to assess the current influence of educational attainment and occupation on maternal obstetrical risk associated with cesarean section under the conditions of an industrialized country and provision of universal coverage. MATERIALS AND METHODS: The perinatal survey data 1998-2001 of the German state of Baden-Württemberg were studied comparing method of delivery for the different occupational categories raised in the survey and the influence of antenatal care utilization. For statistical analysis chi square test, Fisher's exact test and chi square test for trends were used. Relative risks describe the risk for the exposed. RESULTS: Cesarean section rate increased for all groups over the studied four years period. High rates of surgical deliveries were found for the categories "high salaried position" and "unskilled workers". Housewives, the largest group of pregnant women in Baden-Württemberg, delivered significantly less often by cesarean section (75% vaginal deliveries) than women of all other groups (66% vaginal deliveries on the average). In case of low utilization of antenatal care (<5 checks) risk of cesarean section and rate of complications (as blood loss >1,000 ml, hysterectomy) associated with cesarean section are significantly higher. CONCLUSIONS: Differences in an individual woman's risk of having a cesarean section is, besides many other factors, associated with occupation. Antenatal care promotion should target the collective of unskilled workers, at special risk for insufficient use of prenatal care and high rate of cesarean delivery.     

Genetic counseling and prenatal decision for hemoglobin H disease caused by the rare α2 codon 30 (-GAG) (HBA2: c.91_93delGAG) mutation and the SEA deletion: Case series study

ObjectiveWe report a rare mutation on the α2-globin gene, HBA2: c.91_93delGAG and its potential functions.Case ReportWe mainly described four patients with hemoglobin (Hb) H disease caused by the rare mutation and the SEA deletion but diversity in clinical presentation. Two had survived to adulthood with normal physical and mental development, except for mild anemia. However, two were children, who had more severe clinical manifestations. One child had developmental disorders of speech and language and mild growth retardation, and the other child suffered from severe hemolytic crises precipitated by infection and received blood transfusion.ConclusionThis study is of great significance for clinicians to provide genetic counseling to couples at-risk of having offspring with Hb H disease and let them make the pregnancy decision, particularly reduce the occurrence of severe Hb H disease.