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We report a case of priapism in a newborn and the results of a review of the publications on neonatal priapism. A preterm newborn developed idiopathic priapism on the first day after birth. The priapism disappeared after 4 days of observation. Periods with normal penile erection were reported at follow-up after 20 months. The results of the literature review also advocate management by observation alone.
We report a case of priapism in an 11-year-old boy known to have Fabry's disease. High flow was confirmed by color flow Doppler ultrasound and intracorporal blood gas measurements. Successful treatment was achieved by unilateral percutaneous Gelfoam embolization of the left internal pudendal artery. Previously reported cases of priapism associated with Fabry's disease were identified through a MEDLINE search of the English literature and review of the publications. Five reported cases of priapism associated with Fabry's disease were found, including those of 3 children. A report of a child with high-flow priapism that did not respond to conventional treatment including cavernovenous shunting was noted. Priapsim associated with Fabry's disease may be caused by unregulated high arterial inflow. Early recognition of the underlying pathophysiology may identify those cases that would be amenable to percutaneous embolization therapy and may obviate the need to pursue other ineffective conventional treatments.
There are two sort of priapism: the venous or low flow priapism, that is usually painful, and the arterial or high flow one, painless and rare, especially in childhood. We present a case of a boy 5 years old, who 6 days after to suffer a perineal closed traumatism, had a painless and arterial priapism, with aneurism and fistula of the dorsal penile arteria, that was resolute spontaneously through self-embolization, 11 days after. We review the bibliography of 14 cases of arterial posttraumatic priapism in childhood, without find any case resolute by spontaneous self-embolization.
Priapism associated with solid tumours is usually produced by penile metastasis or direct tumour infiltration of the corpora cavernosa. Neoplasias are responsible for between 3-8% of all cases of priapism, and in 80% of cases the primary tumour has a genito-urinary origin. Leukemoid reaction is an haematological disorder characterised by a leukocytosis of over 50000/microl, which develops reactively with different pathological processes such as severe inflammation, disseminated infections, intoxications and tumours. The pathophysiologic mechanism that explains priapism following leukemoid reaction is an intravascular obstruction due to blood hyperviscosity, leading to a blood stasis in the cavernosa veins, and impeding the drainage of the corpora cavernosa. We present a case of priapism in a patient with locally advanced bladder cancer, which was probably the result of blood hyperviscosity produced by a paraneoplastic leukemoid reaction.
Priapism has been defined by AFUD as a pathological condition which consists in a penile erection that persists moreover or is not related to sexual stimulation. Priapism pathophysiology has remained unknown until differents groups of clinical investigators began to research about this entity in patients complaining of erectile dysfunction, who where receiving treatment with intracavernosal vasoactive molecules. Priapism can be clasified into ischaemic (venocclusive): the most prevalent type, or Arterial (non-ischaemic). The purpose of this revision is to update the pathophysiology of the two types of priapism and to create an algorithm of therapeutical and diagnostic approach.
PURPOSE: To report cases of non traumatic high flow priapism treated by arterial embolization. MATERIAL AND METHODS: Six men presented with non traumatic high flow priapism, the diagnosis was based on colour Doppler ultrasound, cavernous blood gas analysis with arterial blood saturation levels and failed medical or surgical therapy. Four patients had sickle cell disease. The embolization was performed with Gelfoam and was unilateral in one case, bilateral in the other cases. RESULTS: Detumescence occurred in a few hours in all cases. One patient had recurrent priapism two years after and was treated by embolization. Transient erectile dysfunction was observed in five cases, permanent in one case. CONCLUSION: Arterial embolization is the treatment of choice in high flow priapism with low rate of permanent erectile dysfunction.
Priapism is a prolonged penile erection unrelated to sexual stimulation. High-flow arteriogenic priapism is uncommon and usually occurs after genitoperineal trauma, which may damage a feeding cavernosal artery, leading to an arteriovenous fistula and, occasionally, to an associated pseudoaneurysm. The defects rarely occur bilaterally. Herein, we report successful treatment of high-flow priapism secondary to a traumatic pseudoaneurysm fed from the bilateral cavernosal artery. Diagnosis was made after cavernosal blood gas analysis, color Doppler ultrasonography, and superselective angiography. Treatment consisted of superselective arterial embolization using metallic microcoils and resulted in simultaneous detumescence of the penis with no complications. The patient regained morning erection on the second postoperative day and erectile function remained normal 8 months after treatment. This case shows that bilateral arteriocavernosal fistulae can be successfully treated with superselective arterial embolization without affecting potency and highlights the importance of warning men about the possibility of developing high-flow priapism following a perineal trauma.
Priapism is a prolonged penile erection that is unrelated to sexual stimulation. Low-flow priapism has been associated with sickle cell disease and other hemoglobinopathies, neoplastic syndrome, anticoagulant therapy, psychotropic medication, and idiopathic causes. We report the successful treatment of idiopathic low-flow priapism using the Winter procedure. Initial treatment consisted of aspiration and intracavernous irrigation with iced saline and a vasoconstrictive agent, but in vain. We then performed the Winter procedure, in which fistulas between the corpora cavernosa and the glans penis were created. This resulted in the simultaneous detumescence of the penis, without complication. The erectile function of the penis was normal 1 year after the procedure. This case shows that idiopathic low-flow priapism can be successfully treated using the Winter procedure when conservative treatment fails.
OBJECTIVE: Priapism is a recognized complication of sickle cell anemia (SCA). When initial conventional treatments fail, simple or exchange blood transfusion has been advocated as a secondary intervention. However, recent literature suggests this may not be an effective therapy and may have significant neurologic sequelae. This paper reviews and summarizes the effectiveness and risks of blood transfusion compared with conventional priapism therapy. METHODS: All relevant papers identified from a MEDLINE search were systematically examined for data related to the use of blood transfusion in the setting of priapism due to SCA. The effectiveness of conventional therapy was compared with transfusion therapy using the outcome of "time to detumescence" (TTD). In addition, papers documenting adverse neurologic sequela were reviewed and summarized. RESULTS: Forty-two case reports were identified containing complete information with regard to patient age and TTD. The mean TTD was 8.0 days with conventional therapy (n = 16) and 10.8 days with blood transfusion therapy (n = 26). Adverse neurologic sequelae from blood transfusion therapy was described in 9 cases, with long term outcomes ranging from complete resolution to severe residual deficits. CONCLUSION: The current literature does not support the contention that blood transfusion is an effective therapy in the treatment of priapism due to SCA, as defined by an acceleration of TTD. In fact, numerous reports suggest that serious neurologic sequelae may result from this treatment. We feel the routine use of this therapy cannot be recommended.
OBJECTIVE: The authors observed priapism as a side effect occuring during the intracavernous treatment of erectile dysfunction. Earlier priapism had been treated with an intracavernous injection of sympatomimetics; unfortunately several complications and contraindications were found. PATIENTS AND METHODS: Methylene blue was applied in the treatment of five patients. First a corpus cavernosum punction was performed and some blood was aspirated from the penis. Finally 100 mg of Methylthionin Chlorati was injected into the corpus cavernosum. RESULTS: A sufficient detumescence was observed in all of these cases. There were no complications. The method was applied effectively in two cases after an unsuccessful punction. CONCLUSION: The autors recommend intravenous methylene blue for the treatment of priapism. According to their experience this method is free of complications and as effective as a sympathomimetics treatment. As they think, it can be recommended in any manifestations of priapism because its force of action appears to be both chemically and biologically clear.
The aim of this work is to assess the value of penile duplex in the prediction of intracavernous drug-induced ischemic priapism. A total of 400 patients with erectile dysfunction were evaluated before and after diagnostic intracavernous injection of a trimix solution (papaverine+phentolamine+PGE1) using color Doppler sonography. In all, 29 patients experienced sustained rigid erections for more than an hour. Patients were further divided into two groups. Group A included patients with spontaneous resolution of their rigid erection within 3 h (10/29) and group B included patients with priapism (19/29) that did not resolve within 3 h. In group A, patients had minimal cavernous artery blood flow within the first hour postinjection, that increased with relief of their erection. Group B patients had no blood flow in their cavernous artery an hour after intracavernous injection and for 6 h later. The disappearance of blood flow in the cavernous artery after an hour of sustained rigid erection predicted priapism with 100% specificity and sensitivity. The persistent absence of cavernous artery blood flow for more than an hour, as detected by color Doppler ultrasound, is an objective predictor of priapism. This may guide early intervention to resolve the prolonged erection.
Recurrent idiopathic priapism is a difficult problem to treat and a true emergency for the physicians, and often even invasive therapeutic interventions fail. We recently managed three men with refractory idiopathic priapism with oral gabapentin. They responded to treatment within 48 h. Two men continue not to experience prolonged erections while treated with lower doses of gabapentin for 16 and 24 months, respectively. The third, after a successful treatment for 6 months, stopped gabapentin and priapism recurred. He responded to treatment again and continues to be free of episodes for 9 months. Gabapentin may be a safe alternative for the management of refractory idiopathic priapism.
This is the first report of priapism in a child with Henoch-Schönlein purpura. Priapism is a condition rarely seen in childhood. When seen in the paediatric population, it is most often associated with haematological disorders. On a world-wide basis, sickle cell anaemia accounts for the majority of childhood priapism. Failure to treat priapism can lead to serious sequelae, including fibrosis and impotence.
Post-traumatic high flow priapism is a rare disease. A review of English published reports revealed 63 cases. Enhanced computed tomography (CT) of the penis has not previously been used as a diagnostic method for post-traumatic high flow priapism. We present a case of post-traumatic high flow priapism diagnosed with enhanced CT of the penis. Additionally, diagnostic modalities for post-traumatic high flow priapism are discussed with review of published work.
OBJECTIVE: To investigate the role of allopurinol in the attenuation of ischaemia- and reperfusion-induced corporeal injury in a rat model of veno-occlusive priapism. MATERIALS AND METHODS: Placebo or allopurinol were given to eight groups of rats before priapism (ischaemia) was induced using a vacuum-constriction device for a duration of 6 or 12 h. Half of the groups of rats undergoing the same duration of priapism had 1 h of detumescence after the constriction band was removed (reperfusion). A ninth group was not treated and received no drug, serving as controls. Corporeal homogenates were then examined for malondialdehyde (MDA) accumulation, derived from lipid peroxidation, using a thiobarbituric acid assay. RESULTS: The accumulation of MDA was significantly higher in the groups treated with placebo and undergoing ischaemia/reperfusion compared with the control group (P < 0.001), but was not significantly different between the placebo-treated ischaemic and reperfused groups (P > 0.05). Rats undergoing 6 and 12 h of ischaemia and reperfusion, and receiving allopurinol had significantly less accumulation of MDA compared with those receiving placebo (P < 0.005). CONCLUSIONS: Lipid peroxidation, an indicator of injury induced by reactive oxygen metabolites, occurred in corporeal tissue during and after veno-occlusive priapism in this rat model; when assessed by lipid peroxidation, allopurinol appeared to protect rat corporeal tissue against this injury.
OBJECTIVE: To evaluate the outcome of patients undergoing the immediate insertion of a penile prosthesis as a treatment for acute low-flow priapism. PATIENTS AND METHODS: Eight patients presenting with low-flow priapism with a mean duration of 91 h (range 32-192) were prospectively evaluated. All had failed conservative management with the instillation of alpha-adrenergic agents, and four had already undergone shunt procedures elsewhere. Immediate management consisted of the insertion of a malleable prosthesis in six patients and an inflatable prosthesis in two. One of the malleable prostheses was subsequently changed to an inflatable device. RESULTS: There were no early complications, with all patients being satisfied with the end result, and seven having sexual intercourse. One patient developed a penile deformity after surgery, cause by fibrosis around one inflatable cylinder. All patients have maintained their penile length. CONCLUSIONS: Prolonged low-flow priapism results in a variable degree of cavernosal fibrosis and a subsequent loss of penile length. The delayed insertion of a penile prosthesis can be difficult, with high complication rates. The immediate insertion of a penile prosthesis in patients with prolonged low-flow priapism is simple and maintains penile length. This should always be offered to the patient at initial presentation, as the complication rate is low and the subsequent outcome excellent.
The molecular mechanism for priapism is not well characterized. Although the nitric oxide (NO) pathway is known to mediate penile erection under normal conditions, we hypothesized that the mechanism of priapism rests in aberrant downstream signaling of this pathway based on our previous findings that mice lacking the gene for endothelial nitric oxide synthase (eNOS-/-) and mice lacking both neuronal NOS (nNOS) and eNOS (nNOS-/-, eNOS-/-) have a tendency for priapic activity. We investigated the role of downstream guanylate cyclase and phosphodiesterase type 5 (PDE5A) expression and function in mediating these responses in eNOS-/- and nNOS-/-, eNOS-/- mice. Erectile responses to both cavernous nerve stimulation and intracavernosal injection of the NO donor diethylamine-NONOate were augmented in eNOS-/- and nNOS-/-, eNOS-/- mice but not in WT or nNOS-/- mice. PDE5A protein expression and activity and cGMP levels were significantly lower in eNOS-/- and nNOS-/-, eNOS-/- mice, and this effect was reproduced in WT corpus cavernosum exposed to NOS inhibitors. Moreover, cavernous nerve stimulation was associated with a marked augmentation of cavernosal cGMP levels, suggesting that, although lower at baseline, the production of cGMP is unchecked in eNOS-/- and nNOS-/-, eNOS-/- mice upon neurostimulation. Transfection of eNOS-/- mice with an adenovirus encoding eNOS resulted in a normalization of PDE5A protein and activity as well as a correction of priapic activity. Coupled with the observation that sickle cell disease mice (which show a priapism phenotype) evince dysregulated PDE5A expression/activity, these data suggest that PDE5A dysregulation is a fundamental mechanism for priapism.
Priapism and increased intracranial pressure are both rare, but recognized, manifestations of leukemia. However, they have never been reported in the same patient. We report a 15-year-old male with acute lymphoblastic leukemia who presented with hyperleukocytosis, priapism, and increased intracranial pressure. Central nervous system leukostasis and cerebral edema may have been detected earlier, had his history of priapism been known. Management of hyperleukocytosis complicated by priapism and increased intracranial pressure is discussed.