Idiopathic stuttering priapism treated with salbutamol orally: a case report F. Migliorini, A. B. Porcaro, R. Baldassarre & W. Artibani Department of Urology, University Hospital, Azienda Ospedaliera Universitaria Integrata di Verona, Verona, Italy
Keywords Baclofen—cyproterone acetate—recurrent ischaemic priapism—salbutamol Correspondence Filippo Migliorini, MD, Department of Urology, University Hospital, Azienda Ospedaliera Universitaria Integrata di Verona, Piazzale Ludovido Scuro n. 10, CAP 37134, Verona, Italy. Tel.: +39 045 812 3313; Fax: +39 045 812 3471; E-mail: [email protected] Accepted: March 24, 2015
Summary Recurrent ischaemic priapism also known as stuttering priapism is an uncommon form of ischaemic priapism, and its treatment is not yet clearly defined. If left untreated, it may evolve into classic form of acute ischaemic priapism and lead to erectile dysfunction due to fibrosis of corpora cavernosa. Several drugs have been proposed with variable results and only supported with level three or four of evidence. Hormonal therapy such as cyproterone acetate, oestrogen, bicalutamide or Lh-Rh agonist are often effective but can cause side effects such as hypogonadal state and infertility. Other medical options are 5-alphareductase and phosphodiesterase-5 inhibitors, ketoconazole, baclofen, digoxin, gabapentin and beta-2-agonist terbutaline. We report the first case of stuttering priapism treated with beta-2-agonist salbutamol.
Traditionally, priapism has been classified into three main categories: ischaemic, nonischaemic and recurrent or stuttering (Levey et al., 2014). Recurrent ischaemic priapism (RIP) is an uncommon condition whereby patients develop prolonged, self-limiting, episodic, unwanted, painful, sleeprelated erections (SREs) that typically last less than 3–4 h (Muneer et al., 2008). If these episodes are not treated, it may evolve into a classic ischaemic priapism and eventually leads to irreversible corporal fibrosis with erectile dysfunction (Morrison & Burnett, 2012). The goal of the management of RIP is the prevention of future episodes (Hoeh & Levine, 2014). Current medical options include anti-androgens (Dahm et al., 2002), LH-RH agonist (Levine & Guss, 1993), 5-alpha-reductase inhibitors (Rachid-Filho et al., 2009), oestrogen (Serjeant et al., 1985), ketoconazole (KTZ) (Hoeh & Levine, 2014), phosphodiesterase-5 inhibitors (PDE5i) (Burnett et al., 2006), digoxin (Gupta et al., 1998), gabapentin (Perimenis et al., 2004), baclofen (Rourke et al., 2002) and terbutaline (Muneer et al., 2008; Kheirandish et al., 2011; Levey et al., 2012). Herein we report a case of RIP successfully treated with oral salbutamol. According to the best of our knowledge, this is the first case reported in the English literature which has been treated with such medication.
A 22-year-old Caucasian man was referred to our outpatient andrology clinic for prolonged morning erections lasting from 2 to 4 h. The symptom began 3 months before, after an episode of acute ischaemic priapism treated with cavernosal blood aspiration and intracavernosal etilefrine. At that time, diazepam 10 mg tid was started. The patient had no medical comorbidities, in particular no sickle-cell disease or any other haematological pathology and no prior history of genital or pelvic trauma. The penis colour Doppler ultrasound did not show arteriovenous fistula and sexual hormonal levels, and the central nervous system MRI was normal. Diazepam was stopped, and cyproterone acetate 100 mg daily was given with good control of the symptomatology. After 4 months, cyproterone was gradually reduced to 25 mg daily within a month. Two weeks after the reduction of cyproterone to 25 mg per day, there were two ischaemic priapic events in 1 week which required cavernosal puncture and blood aspiration. Cyproterone acetate 100 mg daily was resumed. Because of the patient’s young age, a seminal analysis was performed and showed azoospermia. Baclofen 30 mg daily was started, and cyproterone was gradually reduced until stopped within a month. Two weeks after cyproterone suspension, episodes of ischaemic
smooth muscle relaxation (Tong et al., 2008), enhanced erectile function and priapism-like outcomes. According to the European Association of Urology and American Urological Association Guidelines, the goal of the management of stuttering priapism is the prevention of future episodes which can lead to fibrotic transformation of corpora cavernosa and erectile disfunction. While many management options have been proposed for RIP, no treatment of choice has been established due to the limited information available in the literature supported with level three or four of evidence with small case series and case–control studies (Morrison & Burnett, 2012). Various human and animal studies have shown that androgens play a key role in the regulation of SRE (Montorsi & Oettel, 2005), and it has been reported that libido and the frequency of nocturnal erections can be suppressed by reducing testosterone levels to 10% of normal physiological levels (Bain, 2007). LHRH agonist (Levine & Guss, 1993) and anti-androgenic drugs (Dahm et al., 2002) constitute the classical treatment for stuttering priapism but are often associated with side effects such as hypogonadal state and infertility. Other hormonal therapy include KTZ, an oral active antifungal drug known to inhibit androgen production in both the testicle and adrenal gland (Hoeh & Levine, 2014), oestrogen (Serjeant et al., 1985) and 5-alphareductase inhibitors (Rachid-Filho et al., 2009). Other drugs proposed for stuttering priapism treatment are digoxin (Gupta et al., 1998), baclofen (Rourke et al., 2002), gabapentin (Perimenis et al., 2004), PDE5i (Burnett et al., 2006) and terbutaline. The latter is the only beta-2 agonist used for RIP (Molina Escudero et al., 2010; Priyadarshi, 2014). In our case, salbutamol has been successfully employed. Salbutamol, like terbutaline, is a beta-2 agonist with minor beta-1 effects and some alpha agonistic activity too. The mechanism of action causing penile detumescence has not been clearly elucidated. Experimentally, it has been shown that electrical stimulation to the sympathetic nerve trunk abolished penile erection induced by intracavernosal papaverine injection in canine models (Diederichs et al., 1991). Salbutamol as terbutaline probably acts by relaxation of the stretched corporeal smooth muscles, relaxation of polsters in the penile veins and widening of the diameter of the corpora cavernosa draining veins, thus facilitating penile flaccidity (Priyadarshi, 2014). In the literature, we found the description of two cases in which salbutamol was used, respectively, intravenously and per aerosol for the management of intra-operatory priapism (Vaidyanathan et al., 1996; Prakash et al., 2012). Until now, according to our best knowledge, this is the first case reported in which salbutamol has successfully been given for RIP and should be considered another medical option for the treatment of this uncommon disease. 239
RIP treated with salbutamol
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