Management of Isolated Anal Strictures in Crohn's Disease.

BACKGROUND: Anorectal stricturing is a particularly morbid manifestation of Crohn's disease resulting in a diminished quality of life related to pain, incontinence, and recurrent operative interventions. OBJECTIVE: To determine the role of medical therapy, endoscopic dilation, and surgical intervention for the treatment of isolated anorectal stricturing. DATA SOURCES: An organized search of MEDLINE, PubMed, EMBASE, Scopus, and the Cochrane Database of Collected Reviews was performed from January 1, 1990 through May 1, 2020. STUDY SELECTION: Full text papers which included management of isolated anorectal strictures in the setting of Crohn's disease. INTERVENTION(S): Medical and surgical management. MAIN OUTCOME MEASURES: Symptomatic relief, need for proctocolectomy. RESULTS: Our search identified a total of 553 papers; after exclusion based on title (n = 430) and abstract (n = 47), 76 underwent full text review with 65 relevant to the management of anorectal strictures. A summary of the retrospective reports suggests that medical therapy can help control luminal inflammation, but fibrosis may ultimately set in resulting in a need for endoscopic or surgical intervention. Surgical options are limited in the anal canal due to inflammation and ulceration and concomitant perianal fistulizing disease. While fecal diversion can provide symptomatic relief, successful restoration of intestinal continuity remains uncommon and most patients ultimately undergo a total proctocolectomy with end ileostomy. LIMITATIONS: Limited literature published, all retrospective in nature. CONCLUSIONS: Despite significant advances in medical and surgical therapy in Crohn's disease over the last decades, there is clearly an unmet need in the management of anorectal strictures in Crohn's disease.

Naloxone prolongs abdominal constriction writhing-like behavior in a zebrafish-based pain model.

The ability to detect noxious stimuli is essential to survival. However, pathological pain is maladaptive and severely debilitating. Endogenous and exogenous opioids modulate pain responses via opioid receptors, reducing pain sensibility. Due to the high genetic and physiological similarities to rodents and humans, the zebrafish is a valuable tool to assess pain responses and the underlying mechanisms involved in nociception. Although morphine attenuates pain-like responses of zebrafish, there are no data showing if the antagonism of opioid receptors prolongs pain duration in the absence of an exogenous opioid. Here, we investigated whether a common opioid antagonist naloxone affects the abdominal constriction writhing-like response, recently characterized as a zebrafish-based pain behavior. Animals were injected intraperitoneally with acetic acid (5.0%), naloxone (1.25 mg/kg; 2.5 mg/kg; 5.0 mg/kg) or acetic acid with naloxone to investigate the changes in their body curvature for 1 h. Acetic acid elicited a robust pain-like response in zebrafish, as assessed by aberrant abdominal body curvature, while no effects were observed following PBS injection. Although naloxone alone did not alter the frequency and duration of this behavior, it dose-dependently prolonged acetic acid-induced abdominal curvature response. Besides reinforcing the use of the abdominal writhing-like phenotype as a behavioral endpoint to measure acute pain responses in zebrafish models, our novel data suggest a putative role of endogenous opioids in modulating the recovery from pain stimulation in zebrafish.

Carotid atherosclerotic plaque instability and cognition determined by ultrasound-measured plaque strain in asymptomatic patients with significant stenosis.

OBJECTIVE This article describes the use of ultrasound measurements of physical strain within carotid atherosclerotic plaques as a measure of instability and the potential for vascular cognitive decline, microemboli, and white matter changes. METHODS Asymptomatic patients with significant (> 60%) carotid artery stenosis were studied for dynamic measures of plaque instability, presence of microemboli, white matter changes, and vascular cognitive decline in comparison with normative controls and premorbid state. RESULTS Although classically asymptomatic, these patients showed vascular cognitive decline. The degree of strain instability measured within the atherosclerotic plaque directly predicted vascular cognitive decline in these patients thought previously to be asymptomatic according to classic criteria. Furthermore, 26% of patients showed microemboli, and patients had twice as much white matter hyperintensity as controls. CONCLUSIONS These data show that physical measures of plaque instability are possible through interpretation of ultrasound strain data during pulsation, which may be more clinically relevant than solely measuring degree of stenosis. The data also highlight the importance of understanding that the definition of symptoms should not be limited to motor, speech, and vision function but underscore the role of vascular cognitive decline in the pathophysiology of carotid atherosclerotic disease. Clinical trial registration no.: NCT02476396 (clinicaltrials.gov).

Transperineal reanastomosis for treatment of highly recurrent anastomotic strictures after radical retropubic prostatectomy: extended follow-up.

OBJECTIVES: To re-evaluate safety and efficacy of transperineal reanastomosis (TPRA) as a viable therapeutic option in highly recurrent anastomotic strictures (AS) after radical retropubic prostatectomy (RRP). METHODS: Retrospective analysis by standardized questionnaire inquiring for recurrence of stricture, stress urinary incontinence (SUI), sexual function, satisfaction, and changes in quality of life (QoL) was performed in all patients undergoing TPRA. Validated questionnaires (ICIQ-UI, EQ-5D, and IPSS) were included. RESULTS: Median follow-up was 45.0 months. The average number of prior operations was 4.69. Success rate was 87% (20/23). Three recurrences were successfully treated via endoscopic means. All patients had urinary incontinence pre-op and post-op. Implantation of an artificial urinary sphincter (AUS) has been performed successfully in 73.9% (17/23). Rate of complications was low (8.7%; 2/23, Clavien-Dindo Grade II + III). EQ-5D-VAS showed a good general state of health (73.5/100). An improvement in QoL was noted in 63.6% (14/22) and patient satisfaction was high (72.7%; 16/22). CONCLUSIONS: This extended follow-up confirms the initial results for TPRA as an excellent treatment option for highly recurrent AS after RRP. Postoperative SUI as a consequence of transsphincteric urethral mobilization can be satisfactorily treated by the implantation of AUS. In case of AS recalcitrant to endoscopic treatment, the described procedure-even though technically challenging-represents a valuable treatment option.

Haloperidol Decreases Hyperalgesia and Allodynia Induced by Chronic Constriction Injury.

Neuropathic pain has proven to be a difficult condition to treat, so investigational therapy has been sought that may prove useful, such as the use of sigma-1 antagonists. Haloperidol (HAL) is a compound that shows a high affinity with these receptors, acting as an antagonist. Therefore, the objective of this study was to demonstrate its effect in an experimental model of neuropathic pain and corroborate its antagonistic action of the sigma-1 receptors under these conditions. BD-1063 was used as a sigma-1 antagonist control, and gabapentin (Gbp) was used as a positive control. The antihyperalgesic and anti-allodynic effects of the drugs were determined after single-dose trials. In every case, the effects increased in a dose-dependent manner. HAL had the same efficacy as both BD-1063 and Gbp. In the analysis of pharmacological potency, in which the ED50 were compared, HAL was the most potent drug of all. The effect of HAL on chronic constriction injury (CCI) rats was reversed by the sigma-1 agonist (PRE-084). HAL reversed the hyperalgesic and allodynic effects of PRE-084 in naïve rats. The dopamine antagonist, (-)-sulpiride, showed no effect in CCl rats. These results suggest that HAL presents an antinociceptive effect via sigma-1 receptor antagonism at the spinal level in the CCl model.

Cerebrovascular Reactivity and Intellectual Outcome in Childhood Stroke With Transient Cerebral Arteriopathy.

BACKGROUND: Hypercapnic-challenge blood oxygen level-dependent magnetic resonance imaging cerebrovascular reactivity (CVR), measures the regional perfusion response to altered carbon dioxide. CVR correlates with the tissue-level microvascular dysfunction and ischemic risk. Among children with arterial ischemic stroke, transient cerebral arteriopathy (TCA) is a frequent, nonprogressive unilateral intracranial arteriopathy, which typically results in basal ganglia infarction and chronic cerebral artery stenosis. Therefore TCA provides a model for studying the consequences of chronic nonprogressive stenosis using CVR and intellectual outcome. We hypothesized that children with TCA and chronic nonprogressive intracranial artery stenosis have impaired CVR distal to the stenosis and associated cognitive impairment. METHODS: We studied children with a prior diagnosis of TCA as defined by infarction limited to the basal ganglia, internal capsule, or both; and significant (greater than 50% diameter) residual stenosis of the supraclinoid internal carotid artery, its proximal branches or both. All children had CVR, intellectual function, and infarct volumes quantified. RESULTS: We performed CVR studies in five children at mean 8.96 years (3.33 to 14.58 years) poststroke. Impaired CVR was limited to the infarct zone and adjacent white matter in most children. Intellectual function was broadly average in all but one subject. CONCLUSIONS: In children with typical TCA, ipsilateral cortical CVR and intellectual function seem to be preserved despite persistent arterial stenosis in the majority. These findings suggest that chronic revascularization strategies in these children may not be indicated and require further exploration in a larger cohort of children.

Pediatric Chronic Abdominal Pain and Median Arcuate Ligament Syndrome: A Review and Psychosocial Comparison.

Chronic abdominal pain (CAP) occurs in children and adolescents with a reported prevalence of 4% to 41% with significant direct and indirect costs to the child, family, and society. Median arcuate ligament syndrome (MALS) is a vascular compression syndrome of the celiac artery that may cause symptoms of epigastric pain and weight loss and is a frequently overlooked cause of CAP in the pediatric population. We have observed that the psychosocial presentation of patients with MALS is notable for various psychiatric comorbidities. In this article, we review MALS as well as our study results of the psychosocial profile of 30 MALS patients. Our data suggest that children and adolescents with MALS have similar psychosocial profiles to children with other gastrointestinal disorders resulting in CAP. The overlap of physical and psychosocial symptoms of patients who have MALS with other CAP disorders leads us to recommend that patients with CAP should be evaluated for MALS. [Pediatr Ann. 2016;45(7):e257-e264.].

Parental Neuropathic Pain Influences Emotion-Related Behavior in Offspring Through Maternal Feeding Associated with DNA Methylation of Amygdale in Rats.

Chronic neuropathic pain has currently become a remarkable public health concern, considerably damaging not only the physiological but also the psychological aspects of humans. This study investigated whether neuropathic pain affects maternal care and assessed the effect of parental neuropathic pain on the development of neuropathic pain and emotion among offspring. Our results showed that mother rats suffered from chronic constriction injury (CCI) exhibited defective maternal care. The offspring fed by CCI mother rats (own or cross-fed) showed a significant increase in anxiety and anxiety-related behavior compared with that fed by sham-operated mother rats. The offspring fed by CCI mother rats also displayed decreased oxytocin expression in their supraoptic nucleus than that fed by sham-operated mother rats. Moreover, DNA methyltransferase (DNMT)1 expression in the amygdale was increased, whereas DNMT3a and DNMT3b expressions remained the same in offspring fed by CCI mother rats, as detected with immunohistochemistry and western blot analysis. In addition, the total DNA methylation in amygdale was upregulated in offspring fed by CCI mother rats. Considering the above findings, the data of this study suggest that parental neuropathic pain influences emotion-related behavior in offspring through maternal feeding behavior rather than through genetic factors and pregnancy experience that was associated with DNA methylation of amygdale in offspring.

Intracranial stenosis, cerebrovascular diseases, and cognitive impairment in chinese.

Extracranial carotid artery disease has been shown to be related to cognitive deficits. However, limited data are available on intracranial stenosis (ICS) and cognitive impairment. We investigate the association between ICS and cognitive impairment in Chinese. Subjects (n=278), recruited from the Epidemiology of Dementia in Singapore Study, underwent comprehensive clinical evaluation, neuropsychological testing, and brain magnetic resonance imaging (MRI), including 3-dimensional-time-of-flight magnetic resonance angiography (MRA). Cognitive function was expressed as composite and domain-specific Z-scores. Cognitive impairment no dementia and dementia were diagnosed according to internationally accepted diagnostic criteria. Linear and logistic regression models were adjusted for age, sex, education, vascular risk factors, and other MRI markers. A total of 29 (10.4%) persons had ICS on MRA, which was significantly associated with both composite cognitive Z-scores [mean difference in Z-score, presence vs. absence of ICS: -0.37 (95% confidence interval: -0.63, -0.12)] and specific domains including executive function, language, visuomotor speed, verbal memory, and visual memory. ICS was also related to significant cognitive impairment (odds ratio: 5.10 [1.24 to 21.02]). With respect to other MRI markers, adjusted for the presence of lacunar infarcts, the associations of ICS with both composite and domain-specific Z-scores, and significant cognitive impairment became nonsignificant; however, adjustment for other MRI markers did not alter these associations. In this Chinese population, presence of ICS was associated with cognitive impairment independent of vascular risk factors. These associations may be mediated through the presence of infarcts.

Pediatric median arcuate ligament syndrome: surgical outcomes and quality of life.

BACKGROUND: The existence, etiology, diagnosis, and treatment of median arcuate ligament syndrome (MALS) have long been subjects of debate. To our knowledge, there have not been any studies assessing the effectiveness of surgical treatment in improving physical and psychological quality of life in pediatric patients. SUBJECTS AND METHODS: This is an Institutional Review Board-approved prospective study including all patients undergoing surgical treatment of MALS between 2009 and 2012 at our institution. Demographic information, presenting symptoms, radiological imaging, procedure duration, hospital length of stay, and perioperative complications were gathered for analysis. Patients and their parents were asked to complete the Child Health Questionnaire, a physical and psychological health survey, both within 1 week prior to and at least 3 months following their surgery. RESULTS: Six patients underwent laparoscopic release for MALS. The majority of patients were female (n=5 [83.3%]), with an average age of 15.7±1.5 years. Presenting symptoms lasted on average 16.5±12.7 months prior to treatment. Average pre- and postsurgical ultrasound celiac artery peak velocities with inspiration were 332.0±34.1 cm/second and 224.3±31.2 cm/second, respectively, with a statistically significant decrease of 107.67 cm/second (P=.03). The average follow-up period from time of surgery to time of quality of life survey completion was 13±11.3 months, with a range of 3-29 months. A significant improvement from pre- to postsurgical scores was observed in the physical functioning (P=.03), mental health (P=.03), and self-esteem categories (P=.03) of the child assessment. Similarly, there was a significant postsurgical improvement in all categories pertaining to the parent's quality of life (P=.03). Improvement was also seen in the parents' perception of their child's physical functioning (P=.03), bodily pain/discomfort (P=.03), mental health (P=.03), and general health perceptions (P=.03). No intraoperative or postoperative complications occurred. CONCLUSIONS: Our preliminary results demonstrate that laparoscopic median arcuate ligament release for MALS in the pediatric population is safe and effective and improves overall quality of life for the patients and their parents. In carefully selected patients, laparoscopic release for MALS without additional celiac artery reconstruction normalizes blood flow in the celiac artery and improves physical and psychosocial quality of life for the child and his or her parents.

Median arcuate ligament syndrome in the pediatric population.

OBJECTIVES: Median arcuate ligament syndrome (MALS) is a vascular compression syndrome with symptoms that overlap chronic functional abdominal pain (CFAP). We report our experience treating MALS in a pediatric cohort previously diagnosed with CFAP. PATIENTS AND METHODS: We prospectively evaluated 46 pediatric (<21years of age) patients diagnosed with MALS at a tertiary care referral center from 2008 to 2012. All patients had previously been diagnosed with CFAP. Patients were evaluated for celiac artery compression by duplex ultrasound and diagnosis was confirmed by computed tomography. Quality of life (QOL) was determined by pre- and postsurgical administration of PedsQL™ questionnaire. The patients underwent laparoscopic release of the median arcuate ligament overlying the celiac artery which included surgical neurolysis. We examined the hemodynamic changes in parameters of the celiac artery and perioperative QOL outcomes to determine correlation. RESULTS: All patients had studies suggestive of MALS on duplex and computed tomography; 91% (n=42) positive for MALS were females. All patients underwent a technically satisfactory laparoscopic surgical release resulting in a significant improvement in blood flow through the celiac artery. There were no deaths and a total of 9 complications, 8 requiring a secondary procedure; 33 patients were administered QOL surveys. 18 patients completed the survey with 15 (83%) patients reporting overall improvement in the QOL. Overall, 31/46 patients (67%) reported improvement of symptoms since the time of surgery. CONCLUSIONS: MALS was found to be more common in pediatric females than males. Laparoscopic release of the celiac artery can be performed safely in the pediatric population. Surgical release of the artery and resultant neurolysis resulted in significant improvement in the blood flow, symptoms, and overall QOL in this cohort. The overall improvement in QOL outcome measures after surgery leads us to conclude that MALS might be earlier diagnosed and possibly treated in patients with CFAP. We recommend a multidisciplinary team approach to care for these complex patients.

[Cognitive functions in patients with stenosing lesion of brachio-cephalic arteries].

Authors studied cognitive functions in 97 patients with different clinical presentations, lateralization and number of stenotic brachio-cephalic vessels. The Luria's syndrome neuropsychological analysis distinctly revealed the topics of cognitive deficits. Wechsler verbal memory tests, the Stroop test (words-figures), the Schulte test were administered as well. Disturbances of high psychic functions were found in 97.9% of patients. The bilateral pathology of lobe regions was noted most often. The dysfunction of parietal and temporal lobes was mainly unilateral and was linked with the side of stenosing process. Disturbances of neurodynamic parameters of mental activity were correlated with the age (higher frequency in patients older than 50 years), a side of stenosis and/or lesion of stroke, bilateral lesions of carotid arteries. The results allow a more precise assessment of clinical significance of carotid stenosis in the development of cognitive dysfunction that may assist in recommendation of surgical treatment.

Ambroxol, a Nav1.8-preferring Na(+) channel blocker, effectively suppresses pain symptoms in animal models of chronic, neuropathic and inflammatory pain.

Neuropathic pain affects many patients, and treatment today is far from being perfect. Nav1.8 Na(+) channels, which are expressed by small fibre sensory neurons, are promising targets for novel analgesics. Na(+) channel blockers used today, however, show only limited selectivity for this channel subtype, and can cause dose-limiting side effects. Recently, the secretolytic ambroxol was found to preferentially inhibit Nav1.8 channels. We used this compound as a tool to investigate whether a Nav1.8-preferring blocker can suppress symptoms of chronic, neuropathic and inflammatory pain in animal models. The drug was tested in the formalin paw model, two models of mononeuropathy, and a model of monoarthritis in rats. Ambroxol's effects were compared with those of gabapentin. Ambroxol at a dose of 1g/kg had to be administered to rats to achieve the plasma levels that are reached in clinical use (for the treatment of infant and acute respiratory distress syndrome). Ambroxol (1g/kg) was only weakly effective in models for acute pain, but effectively reduced pain symptoms in all other models; in some cases it completely reversed pain behaviour. In most cases the effects were more pronounced than those of gabapentin (at 100mg/kg). These data show that a Nav1.8-preferring Na(+) channel blocker can effectively suppress pain symptoms in a variety of models for chronic, neuropathic and inflammatory pain at plasma levels, which can be achieved in the clinic.

A behavioral and pharmacological validation of the acetone spray test in gerbils with a chronic constriction injury.

UNLABELLED: Cold and mechanical allodynia are important symptoms in patients with neuropathic pain. The study of cold allodynia in animals can help us to understand the underlying pathophysiological mechanisms of neuropathic pain and to validate drugs. The evaluation of cold allodynia in gerbils with a chronic constriction injury of the sciatic nerve on the cold plate is not always stable. We developed a new application method of acetone using a specific spray technique with an Eppendorf multistepper pipette. The chronic constriction injury of the sciatic nerve in gerbils resulted in a long-lasting mechanical and acetone spray-induced hyperreactivity throughout the testing period, which is clearly different from what was seen in sham-operated animals. The acetone spray test incorporates a multimodal stimulus different from direct cold stimulation. The reactivity to the acetone spray coincides in time and strength with the reactivity observed in mechanical allodynia in gerbils and with mechanical and thermal allodynia in other species. Furthermore, a pharmacological validation of the acetone spray test by different reference compounds was performed. Different compounds effective in neuropathic pain models in rodents influence the hyperreactivity to the acetone spray after acute and chronic administration. This study indicates that the multimodal acetone spray test is a valuable tool in the study of neuropathic pain in rodents. IMPLICATIONS: The acetone spray test is a multimodal and valuable tool in the evaluation of neuropathic pain behavior in gerbils.

The long term outcome of feminizing genital surgery for congenital adrenal hyperplasia: anatomical, functional and cosmetic outcomes, psychosexual development, and satisfaction in adult female patients.

BACKGROUND: There are only a few reports analyzing the long term outcome of feminizing surgery in females with congenital adrenal hyperplasia (CAH). Such analysis is crucial to evaluate the treatment and to make necessary adjustments. STUDY OBJECTIVES: To evaluate the adult outcome after feminizing surgery in adult females with salt wasting CAH. DESIGN: Retrospective observational followup investigation. SETTING: Outpatient clinic of a University Medical Center, in 2002. PARTICIPANTS: Eight patients (born 1973-1983) who underwent feminizing surgery in infancy by the same procedure and the same pediatric surgeon in our center, and 19 healthy female controls (for visual analog scales). METHODS: (a) Study of patients' records (n=8); (b) Systematic evaluation of the current situation (n=6): uroflowmetry, a written questionnaire to screen for psychopathology (Youth Adult Self Report, YASR), structured gynecologic examination and a structured psychosexual interview, including scoring on visual analog scales. RESULTS: (a) The first surgery (age 0.1-3.7 yr) consisted of clitoris reduction and vaginoplasty (single-stage) in 7 patients and clitoris reduction only in one patient. The latter patient had vaginoplasty in puberty. In puberty, 6 of the 7 patients with an initial single-stage procedure required re-vaginoplasty. All 6 patients who participated in this systematic evaluation had undergone (re-) vaginoplasty in puberty; (b) 2 of the 6 patients experienced some urinary incontinence, and in one of them, the uroflowmetry result was abnormal. The YASR showed no psychopathology, except for 1 patient with a slightly elevated externalizing score. Gynecologic examination (n=5) revealed vaginal strictures in 3 patients (1 severe, 2 mild). The 2 patients without vaginal strictures had coitus regularly. In the interview, 2 patients called themselves bisexual, the other 4 heterosexual. None of the patients had homosexual contacts. Sexual developmental milestones (romantic interest, falling in love, kissing and petting, coitus) had been reached by all, except for 1 patient who did not have coitus yet. In the patient group, satisfaction with height, body hair, and external genitalia and sexual fantasies and interest, measured with visual analog scales, was not different compared to the control group, except for satisfaction with total body appearance, which was significantly lower in the patients. CONCLUSION: Despite the poor outcome of the initial single-stage surgery in infancy and the inevitable re-operation in puberty, the adult outcome in our study population seems more positive than the findings in the few previous reports, especially with respect to sexual development and activity.

Comparison between two types of behavioral variables of non-evoked facial pain after chronic constriction injury to the rat infraorbital nerve.

BACKGROUND AND PURPOSE: Chronic constriction injury to the rat infraorbital nerve (IoN-CCI) was reported to induce asymmetric face grooming directed to the territory of the injured nerve, and localized mechanical allodynia. The model has been used for pharmacologic testing; responsiveness to mechanical stimulation has been used as outcome measure, but face grooming behavior was not studied in this context. METHODS: Face grooming data from a series of four experiments using the IoN-CCI model were retrospectively analyzed, and two types of face grooming were identified: on the one hand, isolated face grooming (i.e., face grooming that is neither preceded nor followed by body grooming); and on the other hand, face grooming during body grooming (i.e., face grooming that is part of more general body grooming behavior). RESULTS: In all four experiments, amount of isolated face grooming was found to be significantly increased after IoN-CCI. In contrast, the amount of face grooming during body grooming was not significantly altered after IoN-CCI in any of the four experiments. CONCLUSIONS: The amount of isolated face grooming is a more sensitive outcome measure of neuropathic pain than is the total amount of face grooming, which includes face grooming during body grooming.

Long-term follow-up of female patients with congenital adrenal hyperplasia from 21-hydroxylase deficiency, with special emphasis on the results of vaginoplasty.

OBJECTIVE: To assess, in a long-term follow-up, female patients with congenital adrenal hyperplasia (CAH), with special emphasis on vaginal functional outcome and sexual activity after vaginoplasty. PATIENTS AND METHODS: Twenty-seven patients with CAH (aged 14-33 years; six Prader grade II, 14 grade III, six grade IV and one grade V) underwent surgery between 1972 and 1988. Three of the patients underwent clitoridectomy, 24 clitoroplasty and 25 vaginoplasty (24 with a Fortunoff flap and one a 'pull-through' procedure). In 20 patients the vaginoplasty was a one-stage procedure, undertaken at a mean (range) age of 3.6 (1-9) years, and in five patients a two-stage operation. The analysis was based on the patients' history and examination; the patients also completed a questionnaire, including a psychological profile. RESULTS: Nine of the 25 patients (36%) who underwent vaginoplasty developed intravaginal stenosis; of these nine, six were Prader grade III and three grade IV. All had undergone a single-stage procedure at a mean (range) age of 4.7 (2-9) years. Of the 16 patients who answered the questionnaire, 14 had problems with their overall body image; patients in whom vaginal stenosis was corrected were particularly anxious about sexual intercourse and had problems with orgasm. CONCLUSION: The main problem during the long-term follow-up was intravaginal stenosis; all the affected patients had undergone a single-stage procedure early in life to correct ambiguous genitalia. This high rate of vaginal stenosis suggests that vaginoplasty should be undertaken at the beginning of puberty, because higher oestrogen levels may prevent stenosis and, if necessary, dilatation can be performed by the patient. These data also underscore the importance of psychological support in the treatment of children with CAH.

Neuropsychological deficits in asymptomatic carotid artery stenosis.

A total of 20 subjects with asymptomatic carotid artery stenosis (ACAS) and a group of healthy controls were compared as to their cognitive performance. Subjects with ACAS showed substantial deficits on tasks of mental speed, learning, visuospatial abilities, verbal processing and deductive reasoning. The cognitive profile of ACAS--subjects indicated non-specific impairment, possibly related to widespread cerebral vascular disease, of which the presence of ACAS may be one indicator.