Drug eruptions and the vulva.

Drug reactions affecting the vulva are understudied and underreported, with some having the potential to cause serious morbidity through long-term sequelae. We conducted a literature review to investigate the current evidence about vulval drug eruptions. We aimed to establish the extent of drug reactions affecting the vulva, identify the common culprit drugs, and review current evidence and guidelines regarding their management. The vulval involvement seen in Steven-Johnson syndrome, toxic epidermal necrolysis and fixed drug eruption forms the focus of this review, but we also summarize the current evidence regarding less common reactions.

Nocturnal Vulval Pain in Girls-A Subset of Vulvodynia?

OBJECTIVES: The aims of the authors' case series were to outline the clinical features of prepubertal nocturnal vulval pain syndrome and to look at management and outcomes. METHODS: Clinical details of prepubertal girls experiencing episodes of nocturnal vulval pain with no identifiable cause were recorded and analyzed. Parents completed a questionnaire to look at outcomes. RESULTS: Eight girls with age at onset of symptoms between 3.5 and 8 years (mean 4.4 years) were included. Each patient described intermittent episodes of vulval pain lasting between 20 minutes and 5 hours, starting 1-4 hours after falling asleep. They were crying and rubbing or holding the vulva with no obvious cause seen. Many were not fully awake and 75% had no recollection of the events. Management focused on reassurance alone. The questionnaire showed that 83% had full resolution of symptoms with a mean duration of 5.7 years. CONCLUSIONS: Prepubertal nocturnal vulval pain syndrome may be a subset of vulvodynia (generalized, spontaneous, intermittent) to be included in the clinical spectrum of night terrors. Recognizing the clinical key features should aid prompt diagnosis and reassurance of the parents.

Are virtual consultations suitable for patients with vulval disease? A multicentre audit of outcomes in the COVID-19 pandemic.

Background: During the COVID-19 pandemic, virtual consultation (VC) was used to replace in-person consultations. This raises specific questions when dealing with vulval conditions. Objectives: To assess the feasibility and the efficiency of VC with and without supplementary imaging, in patients with vulval conditions, and to evaluate the images provided as an aid to diagnosis. Methods: This prospective multicentre audit took place in three specialized vulval clinics in London and Paris. Anonymized data on patients' clinical characteristics, consultation characteristics (including the number and quality of any supplementary images provided) and consultation outcomes (diagnostic certainty and physician satisfaction) were collected. Characteristics and outcomes in those with or without supplementary imaging were compared amongst both new and follow-up consultations. Results: A total of 316 VCs were included. In total, 18.7% (n = 59) were new patient consultations and 81.3% (n = 257) were follow-up. Supplementary imaging (photographs and/or video recordings) were provided by 28.5% (n = 90) of the total cohort. Median photographic quality was significantly higher on a five-point Likert-type scale when photographs were taken by a third party as opposed to the patient themselves (4 vs. 3, Mann-Whitney U-test, p < 0.0001). There was no association between the provision of supplementary imaging and diagnostic certainty amongst new patient consultations. However, a higher proportion of follow-up patients who provided supplementary imaging received definitive management decisions (χ 2 test, p < 0.001) and physician satisfaction with these consultations, as measured on a five-point Likert-type scale, was significantly higher (Mann-Whitney U-test, p < 0.0001). Furthermore, median physician satisfaction scores ≥4 were observed in follow-up consultations for candidiasis, lichen simplex/eczema and vulvodynia. Conclusions: Although in-person consultation remains the gold standard of care, VC may have a role in the management of selected patients with vulval disease. It is possible to provide good-quality photographs for clinical assessment, particularly with the help of a third party and follow-up patients with an established, cancer-unrelated diagnosis may be best suited for this consultation modality.

Early adolescent language development following intrathecal chemotherapy for acute lymphoblastic leukaemia.

PURPOSE: Central nervous system (CNS) prophylaxis in the treatment of childhood acute lymphoblastic leukaemia (ALL) is routinely achieved through intrathecal chemotherapy (ITC). The presence of high level language deficits in older children who received CNS-directed ITC for ALL in early childhood is yet to be elucidated, with previous research suggesting that high level language deficits may appear later in ALL survivors' development at an age when these skills typically emerge. METHOD: A test battery covering foundational language skills and higher-order language skills was administered to five participants (aged 10-15 years) with a history of ITC for ALL. Conversion of each child's language performance scores to z scores allowed for clinical interpretation of data across the language areas tested. RESULT: Foundational language skills were, in general, of no clinical concern. Three of the five children presented with clinically impaired language skills in areas including resolving ambiguity, making inferences and composing novel sentences. Performance variation between the participants and within the individual participants was noted. CONCLUSION: Given the importance of early adolescent language abilities to academic and social development in late primary and secondary schooling, these preliminary findings suggest further research into emerging adolescent language abilities following ITC for ALL is warranted.

"Trying to Get a Grip": Language Competence and Self-Reported Satisfaction With Social Relationships Three Decades Post-Childhood Traumatic Brain Injury.

OBJECTIVE: (1) To investigate outcomes in language competence and self-reported satisfaction with social relationships in long-term survivors of childhood traumatic brain injury (TBI); and (2) to establish whether language competence contributes to self-reported satisfaction with social relationships decades after sustaining childhood TBI. PARTICIPANTS: Twelve females and 8 males aged 30 to 55 (mean = 39.80, standard deviation = 7.54) years who sustained a TBI during childhood and were on average 31 years postinjury (standard deviation = 9.69). An additional 20 participants matched for age, sex, handedness, years of education, and socioeconomic status constituted a control group. MAIN MEASURES: Test of Language Competence-Expanded Edition and the Quality of Life in Brain Injury questionnaire. RESULTS: Individuals with a history of childhood TBI performed significantly poorer than their non-injured peers on 2 (Ambiguous Sentences and Oral Expression: Recreating Sentences) out of the 4 Test of Language Competence-Expanded Edition subtests used and on the Quality of Life in Brain Injury subscale assessing satisfaction with social relationships. In the TBI group, scores obtained on the Ambiguous Sentences subtest were found to be a significant predictor of satisfaction with social relationships, explaining 25% of the variance observed. CONCLUSIONS: The implication of high-level language skills to self-reported satisfaction with social relationships many decades post-childhood TBI suggests that ongoing monitoring of emerging language skills and support throughout the school years and into adulthood may be warranted if adult survivors of childhood TBI are to experience satisfying social relationships.

Vulval symptoms after the menopause - Not all atrophy!

Vulval and vaginal symptoms are common after the menopause and are frequently assumed to be due to the normal physiological changes that occur at this time. However, there are several important dermatoses that can occur in this patient group which need accurate diagnosis and appropriate management. This review discusses the clinical features and basic management of some of the common vulval problems occurring after the menopause.

Quality of pre-school children's pretend play and subsequent development of semantic organization and narrative re-telling skills.

PURPOSE: This study investigated if the quality of pre-school children's pretend play predicted their semantic organization and narrative re-telling ability when they were in early primary school. It was hypothesized that the elaborateness of a child's play and the child's use of symbols in play were predictors of their semantic organization and narrative re-tell scores of the School Age Oral Language Assessment. METHOD: Forty-eight children were assessed using the Child-Initiated Pretend Play Assessment when they were aged 4-5 years. Three-to-five years after this assessment their semantic organization and narrative re-telling skills were assessed. RESULT: Results indicate that the elaborateness of a child's play and their ability to use symbols was predictive of semantic organization skills. Use of symbols in play was the strongest play predictor of narrative re-telling skills. The quality of a pre-school child's ability to elaborate complex sequences in pretend play and use symbols predicted up to 20% of a child's semantic organization and narrative re-telling skills up to 5 years later. CONCLUSION: The study provides evidence that the quality of pretend play in 4-5 year olds is important for semantic organization and narrative re-telling abilities in the school-aged child.

Unilateral vulval swelling in cyclists: a report of 8 cases.

OBJECTIVE: To define the clinical features, imaging results, histopathologic patterns, and clinical outcomes seen in patients who develop vulval swelling as a result of intensive cycling. METHODS: The case notes of 8 female cyclists were retrospectively reviewed. RESULTS: The mean age of the patients was 45 years, and all were cycling long distances each week (range, 125-450 km; median, 210 km). All patients had a unilateral swelling of the labium majus. Five patients had magnetic resonance imaging showing asymmetry of the vulva with no enhanced signaling associated with inflammation. Histologic examination in 3 cases revealed dermal fibrosis and dermal edema associated with dilatation of lymphatic vessels. In 1 case, a perifollicular inflammatory infiltrate containing epithelioid granulomas was seen but Crohn's disease was excluded. In another case, fibromuscular hyperplasia was seen. Six patients had surgical excision of the swollen area, and 4 patients were able to resume cycling without problems up to 5 years of follow-up. The cosmetic appearance was initially considered satisfactory by 5 patients. However, 2 patients required a second surgical procedure to further improve the cosmetic appearance. In 1 case, there was a postoperative hematoma followed by an immediate recurrence of the swelling, which persisted. CONCLUSIONS: Unilateral swelling of the labium majus occurring in cyclists can be related to dermal fibroedema associated with lymphatic dilatation or to an increase in adipose tissue. Surgical results were satisfactory in 5 of the 6 patients who underwent excision.

Pre-linguistic communication skill development in an infant with a diagnosis of galactosaemia.

BACKGROUND: Neonatal screening for galactosaemia (GAL) identifies the condition early, but subsequent biomedical and genetic testing fails to identify which subgroup of infants with GAL are at most risk of the language disorders associated with the condition. This study aims to present preliminary data on an infant with GAL based on assessment of pre-linguistic communication behaviours known to underpin language development. METHODS: This single case-control study profiles the pre-linguistic skills of a 13-month-old infant with GAL. The Index Infant's performance was descriptively compared to that of a typically developing, suitably matched control infant. RESULTS: The Index Infant was identified as presenting with clinically significant delays on 9 of the 11 pre-linguistic skills assessed. DISCUSSION AND CONCLUSION: The early identification of risk of developmental language difficulties in the Index Infant allows for the implementation of early intervention using the infant's parents as facilitators of language stimulation. Monitoring of the infant's progress is warranted.

Development of figurative language skills following central nervous system-directed chemotherapy delivered in early childhood.

Central nervous system (CNS)-directed chemotherapy is delivered for the treatment of childhood acute lymphoblastic leukaemia (ALL). Figurative language deficits have been described in children following CNS-directed chemotherapy; however, comprehensive analysis of figurative interpretation errors, potentially providing clinical utility to assist with intervention planning, has never been performed. The present study aimed to compare the figurative language skills of seven children treated with CNS-directed chemotherapy for ALL before the age of 6 years (mean age at diagnosis 3 years 10 months) and a matched control group of children, using the Test of Language Competence-Expanded Edition (TLC-E) Figurative Language sub-test. It was hypothesised that the children treated with CNS-directed chemotherapy would demonstrate a decreased performance in and an alternative method of interpreting figurative language. The results suggest no negative effects of CNS-directed chemotherapy on figurative language. There were no statistically significant differences between groups for TLC-E Figurative Language sub-test composite scores and picture component errors, nor were there clinically significant differences observed from descriptive comparisons of individual case data and error analysis. As these skills continue to emerge beyond childhood, the need to monitor skill development in ALL survivors beyond childhood is highlighted.

Charting a seven-year trajectory of language outcomes for a child with galactosemia.

Cross-sectional methodologies have revealed age-related deterioration in cognitive performance, reflecting progressive neurodegenerative change in a minority of children and adolescents with classic galactosemia (GAL). The application of longitudinal methodologies sensitive to age-related changes at the individual level is needed to determine the extent of any possible decline in function in children with GAL. The authors report on the developmental language outcomes of a 9-year-old female with GAL through an examination of her language development over a 7-year period using a performance tracking system based on the use of raw performance scores required for attainment at the 50th percentile for age. Raw scores typically increase systematically over time and are thus more sensitive to developmental changes. Results suggest that there was no decline in the child's language skills over the course of the investigation. For the case presented, the use of raw scores offered a means of examining the child's patterns of individual change, which revealed stable language skills over the period of monitoring, perhaps indicating a stable disease process for this particular child. The authors propose this descriptive application of raw performance scores that offers a means to determine neurodevelopmental outcomes in the disorder.

The spectrum of histopathologic patterns secondary to the topical application of EMLA® on vulvar epithelium: clinicopathological correlation in three cases.

EMLA(®) (eutectic mixture of local anesthetics, 2.5% each of lidocaine and prilocaine in an oil and water emulsion) is used as a topical anesthetic. We report three cases of EMLA(®) -induced histopathologic changes on the vulvar epithelium. While there are some similar histopathologic features to those reported in extragenital skin, we describe additional findings on vulvar epithelium, which, to our knowledge, have not been reported previously. The patients presented with clinical signs suggestive of lichen sclerosus or erosive lichen planus (LP), but were all confirmed histopathologically as LP. The biopsy was taken after 15 min of EMLA(®) application and intradermal injection of 1% lidocaine. Blistering prior to intradermal lidocaine and the biopsy procedure was observed in two patients. The histopathologic changes observed in the epithelium included pallor of the upper epidermis, mild spongiosis, intraepidermal subcorneal and suprabasal acantholysis, congestion of the papillary dermal capillaries and extravasated erythrocytes. Basophilic granules were present, but rare, while the necrosis with multifocal clefting was more marked than in extragenital skin. It is important to be aware of these changes occurring on genital mucosa; these may occur in the absence of clinical signs and may obscure the primary underlying pathology, thus representing a diagnostic pitfall.

Differential phonological awareness skills in children with classic galactosemia: a descriptive study of four cases.

Educational achievement, which for individuals with the metabolic disorder classic galactosemia (GAL) is significantly lower than in the wider population, correlates with self-reported quality of life. Phonological awareness skills underpin the development of literacy, and although literacy is a key contributor to successful academic outcomes, no study to date has investigated phonological awareness skills in children with GAL. This study investigated phonological awareness (PA) in four school-aged children with the disorder, two of whom were siblings. Age range for the children was 7 years 7 months to 9 years 2 months. Each child was assessed with the Phonological Awareness criterion-referenced subtest from the Clinical Evaluation of Language Fundamentals-Fourth Edition. Included in the data for analysis was each child's performance measures obtained from their most recent assessment of cognitive and lexical development. A number of descriptive analyses were undertaken on the data. One child, who met her age criterion for PA, had cognitive and lexical development skills in the average range. The remaining three children failed to meet their age criteria. Although these three children presented with clinically similar cognitive and lexical development skills, disparate PA skills were identified. The PA skills of one of the sibling pair were notably more advanced than his older sibling. The limitations of relying on behavioural test results in children with GAL to predict those most at risk of reduced skill development are discussed in terms future research directions.

Differential language trajectories following treatment for pediatric posterior fossa tumor: an investigation of four cases.

Up to 85% of children treated for brain tumor survive beyond five years; hence optimizing quality of life in survivorship has become a priority. As multiple factors contribute to the heterogeneity of neurocognitive and language outcomes for individual children following treatment, a means of monitoring subsequent development is needed for the individual child, particularly when pre-morbid performance indices are not available. The current study investigated the use of developmental language trajectories as a means of monitoring language development subsequent to treatment for tumors located within the posterior fossa. The language skills of four children treated for posterior fossa tumor (PFT) were monitored over time (range of monitoring: 2-6 years) and the resultant trajectories were plotted against the trajectories based on tests' normative data as well as the trajectories of control children drawn from each child's local community. Each child's trajectory was considered in terms of age-appropriate developmental gains and discussed regarding the need for ongoing clinical monitoring of emerging, developing or established language skills. The study's findings highlight the heterogeneity of language outcomes following PFT. The utility of the application of developmental trajectories for the provision of individualized post-treatment support is discussed.

Longitudinal language outcomes following intrathecal chemotherapy for acute lymphoblastic leukaemia.

Intrathecal chemotherapy (ITC) is the treatment option for acute lymphoblastic leukaemia (ALL). Neurocognitive deficits have been described following ITC, but language status post-treatment is yet to be clarified. This study examined the language skills of nine children following ITC for ALL (mean age 7;8 years and 3;2 years post-diagnosis at baseline measurement) and nine age- and sex-matched controls, at baseline then 2 years later, using a battery of tests assessing general language skills. An assessment of cognitively-demanding high level language skills was undertaken on a sub-group of the children (n =12). Statistical analysis revealed no significant difference between children treated with ITC and controls when comparing change in performance scores from baseline measurement to 2 years post-baseline measurement. Descriptive analysis of three of the ALL participants in the Intermediate Stage survivorship at language re-assessment indicated no clinically-significant change in performance over 2 years for all measures except receptive language skills, which improved over the time for two of the children. As language skills continue to develop into late adolescence, the need for the monitoring of language abilities of children treated at a young age with ITC as they enter the Intermediate and Late Stages of survivorship is discussed.

Lacrimal canalicular duct scarring in patients with lichen planus.

BACKGROUND: Lichen planus (LP) is a mucocutaneous inflammatory disease that affects multiple sites, including the skin, oral cavity, vulva, and vagina and can result in scarring and stricture formation. It has also been shown to cause lacrimal canalicular blockage in a series of patients attending an ophthalmology clinic. We describe a cohort of women with vulvovaginal LP who also had signs of lacrimal canalicular scarring on examination. OBSERVATIONS: We report 9 cases of LP with scarring of the conjunctiva around the lacrimal ducts. Seven of 9 women had symptoms of epiphora, and in 2 women lacrimal canalicular scarring was an incidental finding. Seven of 9 cases were diagnosed by an ophthalmologist. All women had biopsy-proven LP at 1 mucocutaneous site each. Seven of 9 women had vulvovaginalgingival syndrome, which is a subgroup of severe erosive LP. CONCLUSIONS: Given the strong association between erosive mucocutaneous LP and multisite scarring sequelae, it is not unexpected that ocular inflammation may lead to lacrimal duct stenosis. We believe that this complication has been underreported among patients with LP and that an ophthalmological history and examination of the punctum of the lacrimal duct should be sought, especially in patients with the erosive subtype of LP.

Indices of language outcome 11 years after intrathecal chemotherapy for acute lymphoblastic leukemia: a sibling case-control study.

Studies are emerging that suggest that major language indices do not differentiate children treated for acute lymphoblastic leukemia (ALL) with risk-adapted intrathecal chemotherapy (ITC) from control children matched on age, gender, and educational level. No study to date has controlled for cognitive environment, an important variable influencing language achievement and outcome. This case-control study applies the deconfounding principle by using a sibling as a control to investigate language outcomes in a male child 11 years after administration of ITC for ALL at the age of 2 years 3 months. A comprehensive behavioral language test battery failed to differentiate the siblings on current language performance when descriptively compared, but neurophysiological assessment revealed that the ITC-treated child required more time and elicited a smaller N400 component compared to his sibling during picture-word matching. The findings suggest that in the absence of pretreatment performance indices, comparison with sibling achievement may supplement what is known on posttreatment language skill development drawn from comparative studies using children matched on age, sex, and educational level drawn from the community. The study's findings offer pilot data of language outcomes following ITC beyond the early stage of survivorship. The benefits and limitations of using siblings in research where the cognitive environment is known to make an important contribution to skill development are discussed.