Abstract
Context: Recurrent priapism is characterized by repetitive episodes of erections which are unrelated to sexual interest or stimulation. It is extremely rare in patients with spinal cord injury (SCI). There are a number of conservative agents used in the treatment.
Findings: We describe the case of a 20-year-old male with cervical-5 American Spinal Injury Association (ASIA) Impairment Scale (AIS) grade A tetraplegia as a result of a diving accident 2 years ago. He declared that the recurrent penile erections occurred up to 15–20 times day and night every day and lasted up to 20 min each time unrelated to sexual interest or stimulation. He was prescribed baclofen 10 mg, twice daily. The frequency and duration of erections decreased to 3–5 times/day lasting for about 5 min each and the patient reported a high treatment satisfaction.
Conclusion: The presentation of this case is to remind clinicians this rare but distressing condition of recurrent priapism seen in men with complete spinal cord lesions and to summarize the use of medications, most commonly baclofen, to alleviate the condition.
Keywords: Priapism, Spinal cord injury, Sexual dysfunction, Rehabilitation, Baclofen
Introduction
Priapism is a pathologic condition representing a true disorder of penile erection that persists beyond or is unrelated to sexual interest or stimulation. It can be classified into ischemic, arterial, or recurrent (intermittent/stuttering). Recurrent priapism is a distinct condition characterized by repetitive, painful episodes of prolonged erections. Erections are self-limited with intervening periods of detumescence. The duration of the erectile episodes in recurrent priapism is generally shorter than in the ischemic type.1 Recurrent priapism after SCI is an extremely rare entity which is usually seen in acute complete and cervical spinal cord lesions.2–4 The frequency of priapism in SCI or why priapism occurs only in some SCI patients are not known.3 There are a number of conservative agents used in the treatment of recurrent priapism.1,5,6
We aimed to discuss the recurrent priapism and its treatment in a patient with chronic SCI.
Case report
We describe the case of a 20-year-old male with cervical-5 American Spinal Injury Association (ASIA) Impairment Scale (AIS)7 grade A tetraplegia as a result of a diving accident 2 years ago. He was consulted to the sexual rehabilitation unit of our rehabilitation hospital because of his recurrent penile erections unrelated to sexual interest or stimulation. He declared that the recurrent penile erections occurred up to 15–20 times day and night every day and lasted up to 20 min each time unrelated to sexual interest or stimulation. He first realized his erections after a 17 days period of hospitalization in the intensive care unit. The penile erections were not only triggered during clean intermittent catheterization and exercise but also sometimes occurred spontaneously unrelated to a triggering factor. These episodes became distressing for the patient and his caregivers especially during rehabilitation sessions. There was no history of trauma to pelvis or perineum, chronic conditions like sickle cell disease, hemoglobinopathies and hypercoagulable states. The laboratory tests (Complete blood count, liver, renal, and thyroid function tests, serum testosterone, and serum electrolyte levels) were in normal ranges. The abdominopelvic ultrasonography and color duplex ultrasonography (CDU) examination of penis showed no abnormalities. Bilateral corpora cavernosa were normal on gray-scale US examination. In the examination performed after papaverine injection, the erection was grade 3. In the examination performed in color mode and spectral analysis, the peak systolic velocities of the cavernosal artery were measured at 40 cm/s on the right, 45 cm/s on the left, and 0 cm/s on both sides of the end systolic velocities.
The patient was consulted with the urology department and got a diagnosis of recurrent priapism related to SCI. He was taking trospium chloride 45 mg/day and oxybutynin chloride 15 mg/day for the neurogenic detrusor overactivity. He was also taking 50 mg/day sertraline for about 1 month for depression. Although sertraline was a potential causal factor for ischemic priapism,1 it was not thought to be associated with priapism in our case as the patient's complaint was started 17 days after the spinal cord injury (about 2 years ago).
He was prescribed baclofen 10 mg, twice daily. The frequency and duration of erections decreased to 3–5 times/day lasting for about 5 min each and the patient reported a high treatment satisfaction.
Discussion
Chronic priapism represents a challenging therapeutic dilemma. Inadequate or deferred treatment can result in impaired quality of life, and permanent erectile dysfunction.8 Recurrent priapism's episodes usually start after the spinal shock is over in patients with SCI. Although the erections are self-limited, the frequency and duration usually increase in time leaving the patient in a very difficult situation.2–4 The loss of sympathetic outflow to the penile vasculature leads to increased parasympathetic effect resulting in uncontrolled arterial inflow into the penile sinusoidal spaces.3 In the literature, priapism is reported only in complete SCI and most of the patients had cervical lesions. The frequency of priapism in SCI or why priapism occurs only in some SCI patients are not known.3 There are a number of conservative agents used in the treatment of recurrent priapism. The most commonly used one in SCI is oral baclofen. Intrathecal baclofen can also be used when the oral form fails. Baclofen is a Gamma Aminobutyric Acid (GABA) agonist which can inhibit erection and ejaculation through GABA activity.1,5 It is presumed that baclofen relaxes the ischiocavernosus and bulbospongiosus muscles, which are involved in penile erection.9
In the literature review, there are few recurrent priapism patients treated with oral baclofen. Vaidyanathan et al.2 treated a 46-year-old male sustained C-4 complete tetraplegia who suffered from recurrent priapism. The authors prescribed oral baclofen 10 mg three times daily. The frequency of erection and erection duration decreased 24 h after treatment. Rourke et al.8 treated a 41-year-old male with a 3-year history of recurrent idiopathic nocturnal priapism episodes occurred several times a night and caused severe sleep deprivation. The authors initiated treatment at a dose of 10 mg given at bedtime, with dose escalation in 10-mg increments. Complete alleviation of symptoms was achieved with a dose of 40 mg.
Low dose phosphodiesterase type 5 (PDE5) inhibitors (Sildenafil 25 mg/day or tadalafil 5 mg/day) can also be used in the treatment of recurrent priapism. Although these agents are routinely used in erectile dysfunction, in low doses they have a paradoxical effect preventing priapism.1 It is understood that PDE5 enzyme dysregulation plays a role in the pathophysiology of priapism. As a result of PDE5 dysregulation, cGMP increases and causes overt vasorelaxation in erectile tissues leading to priapism. Low doses of long-lasting PDE5 inhibitors allows normal active secretion of PDE5 and cGMP breaks down effectively. As a result, priapism is prevented. PDE5 inhibitors should be started when penis is not erected.10 Other agents used in the treatment of recurrent priapism are; hormonal therapies (gonadotropin-receptor hormone agonists and antagonists), antiandrogens (flutamide, bicalutamide), oestrogens, 5-alpha reductase inhibitors, ketoconazole, digoxin, alpha adrenergic agonists (pseudo-ephedrine, etilefrine), gabapentine, terbutaline and hydroxyurea. But except the oral and intrathecal forms of baclofen, the above mentioned agents are not demonstrated to be efficacious in SCI.1,6 They may be used in unique cases where oral/intrathecal baclofen fails. Moreover, antiandrogen agents may not be suitable in chronic SCI because of the high prevelance of androgen deficiency and hypogonadism in chronic SCI males.11–14
Conclusion
Recurrent priapism after SCI is an extremely rare entity and there are a number of conservative agents used in the treatment. The presentation of this case is to remind clinicians this rare but distressing condition of recurrent priapism seen in men with complete spinal cord lesions and to summarize the use of medications, most commonly baclofen, to alleviate the condition.
Disclaimer statements
Contributors None.
Funding None.
Conflicts of interest We acknowledge that there are no conflicts of interest related to this case report.
Ethics approval None.
References
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