Klippel-Trenaunay syndrome and spinal arteriovenous malformations: an erroneous association.

BACKGROUND AND PURPOSE: KTS is a rare limb overgrowth disorder with slow-flow vascular anomalies. This study examines the presumed association between KTS and spinal AVMs. MATERIALS AND METHODS: We performed a MEDLINE search of articles and reviewed textbooks of spinal diseases to study the association between KTS and spinal AVM. Our goal was to ascertain the basis on which the diagnosis of KTS was established and to evaluate the evidence of its association with spinal AVMs. In addition, the data base of the Vascular Anomalies Center at Children's Hospital Boston was queried for patients with KTS, and the association with spinal AVM was investigated. RESULTS: Twenty-four published reports on spinal AVMs in 31 patients with KTS were reviewed. None of these references provided solid evidence of the diagnosis of KTS in any patient. Clinical data were either incompatible with the diagnosis of KTS or were inadequate to establish the diagnosis. Alternative possible diagnoses (CLOVES syndrome and CM-AVM) were suggested by the first author for 9 of the patients reported in these articles. The medical records of 208 patients with the diagnosis of KTS were analyzed; not a single patient had clinical or radiologic evidence of a spinal AVM. CONCLUSIONS: An association between KTS and spinal AVM, as posited in numerous references, is most likely erroneous. The association has neither been reliably proved in the limited published literature nor encountered in a large cohort.

The role of intracavernosal vasoactive agents to overcome impotence due to spinal cord injury.

Twenty-two spinal cord injured men (mean age 35.2 years) referred because of impotence were treated with intracavernosal vasoactive agents. Papaverine 2-20 mg, papaverine 40 mg plus phentolamine 0.5 mg or prostaglandin E1 (1-20 micrograms) were used. Nineteen responded and were taught to self administer the medication with varying degrees of assistance from their partners. Fourteen men participated in a postal survey; 12 (86%) continued to use the drugs every 1 to 4 weeks and reported satisfaction with the method. Partner responses were positive in half of the group and when not so it influenced the patients to abandon or reduce the frequency of treatment. Counselling was essential to allay anxiety. Clinical management was not compromised and side effects were minimal. Overall we found this treatment effective, safe and worthwhile.

Effects of a sclerosant, ethanolamine, on the venous drainage system of the dog penis.

To explore the possibility of using venous sclerosant therapy to overcome venogenic impotence we studied the effects of ethanolamine on the veins draining the dog penis. Following injection of the sclerosant into the deep dorsal vein radiological and histological evidence of occlusion was obtained but by one month recanalization of thrombi had occurred and collateral venous channels had formed. These findings cast doubt on the effectiveness of this sclerosant as a means of treating impotence.

Effects of acute alcohol on penile tumescence in normal young men and dogs.

To determine the cause of the inhibition of sexual function observed in normal subjects with elevated blood alcohol concentrations (BAC), nocturnal penile tumescence (NPT) was monitored in 11 subjects over three consecutive nights. On the third night alcohol was administered (BAC = 0.154 g/100 mL). We observed no effect of alcohol on the size, duration, or number of erections. These results were confirmed in dogs. The latent period, magnitude, and duration of corpus cavernosal pressure changes produced by pelvic nerve stimulation were not affected by mean blood alcohol levels of 0.327 g/100 mL in three dogs. These results suggest that the inhibition of sexual response caused in conscious subjects by the ingestion of alcohol is not due to a suppression of the underlying spinal reflex but may be the result of its effect on perceptual or cognitive sexual mechanisms.

Prostaglandin E1 therapy for impotence, comparison with papaverine.

The efficacy of prostaglandin E1 as a pharmacological treatment of erectile dysfunction (impotence) was compared with the standard treatment, papaverine, in a single blind, crossover trial. A total of 129 impotent men received intracavernous injections of either prostaglandin E1 (5 micrograms) or papaverine (18 mg.) 1 month apart. Observations by 1 observer recorded 10 to 20 minutes after injection demonstrated that prostaglandin E1 generated a better erection in 72 men (55.8%) compared to papaverine. Papaverine was more effective than prostaglandin E1 in 23 men (17.8%, chi-square 6.26, p less than 0.025). Subjective assessment by the patients who detected a difference showed that 71 (55%) preferred prostaglandin E1 and 35 (27%) preferred papaverine (chi-square 11.56, p less than 0.001). A total of 34 men achieved full erections with prostaglandin E1 compared to 17 who used papaverine.

Complications associated with penile implants used to treat impotence.

The complications associated with a variety of penile prostheses used in the treatment of 302 men with erectile dysfunction are described. Infection of the prostheses resulted in their removal from 11 patients (3.6%), antibiotic therapy and replacement in five men. Mechanical failures required reoperation in 43 men (14.2%); however, recent advances in prosthesis design have reduced this type of malfunction. Adverse psychological reactions prompted removal of the prostheses from two men. Despite these complications, this surgery is remarkably effective.

Treatment of impotence by intrapenile injections. A comparison of papaverine versus papaverine and phentolamine: a double-blind, crossover trial.

The efficacy of papaverine, and a combination of papaverine and phentolamine as a pharmacological treatment of impotence was compared in a double-blind, crossover trial. A total of 40 impotent men received intracavernous injections of papaverine (40 mg.) or a combination of papaverine (20 mg.) and phentolamine (0.5 mg.) at monthly intervals. Observations at 20 minutes after injection demonstrated that papaverine caused full erections in 27 per cent of the men and partial erections in 65 per cent. The combined injection resulted in full erections in 48 per cent of the men and 52 per cent had partial erections. The difference was significant (Z equals 2.29, p less than 0.05).

The role of dynamic corpora cavernosography in the management of impotence.

Sixty eight impotent men underwent corpora cavernosography and corpora cavernosal pressure measurements during saline infusion. Nineteen of the men had internal pudendal arteriograms. Correlation between the cavernosograms and saline infusion induced pressure measurements was "good" in 56/66(84%), "approximate" in 5/66(8%) and there was no correlation in 5/66(8%). Two patients were excluded for technical reasons. The spread of results and the good correlation of the two tests speaks for the accuracy of the method. It was of value in diagnosis and will be of paramount importance if veno occlusive surgery becomes a reliable method of treatment.

Treatment of impotence by intrapenile injections of papaverine and phenoxybenzamine: a double blind, controlled trial.

The efficacy of papaverine and phenoxybenzamine as a pharmacological treatment of impotence was compared in a double blind, crossover, placebo controlled trial. Thirty-four impotent men received intra-cavernosal injections of papaverine (60 mg), phenoxybenzamine (6 mg) or normal saline (10 ml) at monthly intervals. Observations by EJK up to 45 minutes after injection demonstrated that papaverine caused full erections in 35% of men and partial erections in 62%. Phenoxybenzamine injection resulted in full erections in 20% of men and 62% had partial erections. The differences were not significant. Despite 82-97% of patients attaining some degree of erection in the clinical setting only six of the patients decided to use this mode of treatment for more than three months. Sexual function during the month after injection was better with either drug when compared with placebo.

Experience in the management of erectile dysfunction using the intracavernosal self-injection of vasoactive drugs.

We report the results of a survey of 75 patients with erectile dysfunction, all of whom were treated by self-injection of the corpora cavernosa with vasoactive agents. At the time of the survey each patient had been self-injecting at home for at least 3 months. Of the patients 62 were followed for 3 to 21 months. Patients used this form of treatment with minimal assistance from our clinic staff. It was acceptable to them with few complications except for prolonged erections in 11 per cent of the men. Fifteen patients (24 per cent) with psychogenic impotence reported spontaneous improvement in erections during treatment and 4 discontinued treatment for this reason. Another 26 patients (42 per cent) continue to use self-injection as the definitive mode of treatment for erectile dysfunction.

Prolonged erections following intracorporeal injection of medications to overcome impotence.

We describe the management of 17 men who developed prolonged erections following the intracorporeal injection of vasoactive drugs (e.g. papaverine) for the treatment of erectile dysfunction. Twelve men who had psychogenic impotence developed prolonged erections following injection compared with 5 who had an organic aetiology. The aspiration of 20 to 50 ml of blood from the penis together with the intracorporeal injection of 1 to 2 mg metaraminol is an effective treatment of this complication.

Experience with the Nesbit procedure in the treatment of penile curvature.

Thirteen men who had undergone Nesbit's operation for penile curvature were assessed to determine the efficacy of the procedure. Ten patients had Peyronie's disease and three had a congenital curvature. Follow-up ranged from 8 months to 28 months. The degree of penile curvature was improved in twelve patients at the time of follow-up. The three men with a congenital curvature were all satisfied, whereas only seven of the 10 with Peyronie's disease were satisfied with the results of surgery. Two men believed that the operation had reduced their erectile function and a third man felt that the operation had not improved his penile deformity. Patients with a penile curvature associated with deteriorating erectile function should be counselled that a Nesbit's procedure will not improve their potency and that the insertion of a malleable penile prosthesis may be necessary.

Human sexuality: a survey of the knowledge of medical students.

A survey of New Zealand medical students is described in which knowledge of human sexuality is documented to vary with age, sex, marital status, participation in a human sexuality teaching programme and year of course. Whilst no group scored disastrously and all groups scored well in some areas of sexuality, most groups also demonstrated significant gaps in their knowledge. Within any large medical school it is likely that there will be student subgroups with widely different levels of knowledge and therefore different needs requiring to be met in terms of education. Knowledge of sexuality was demonstrated to increase during the Otago medical course and for this process to have been facilitated by a formal human sexuality teaching programme. Support is therefore given to the need for, and effectiveness of, such teaching programmes in medical schools.

Abortion: some observations on the contraceptive practice of women referred for psychiatric assessment in Dunedin.

Factors associated with the contraceptive practice of 31 women referred for psychiatric assessment prior to therapeutic termination of pregnancy are analysed. Poor contraceptive practice is a significant contributing factor to the increasing rate of unwanted pregnancy and abortion. More research at a psychological level into this important sociological phenomenon is needed.