Changing diagnostic and therapeutic concepts in high-flow priapism

Abstract

High-flow priapism (HFP) is defined as pathological increased arterial influx into the cavernosal bodies. Since 1960, 202 cases have been published in the literature. This study evaluates the effect of the changing diagnostic and therapeutic concepts. The data of 202 cases of HFP was evaluated regarding diagnostic and therapeutic procedures and long-term results. Success was defined as restored erectile function without recurrent priapism. The major etiology of HFP is trauma, especially in children or young adults; in older men, HFP is a rare event mainly caused by malignoma. Cavernosal blood-gas analysis, color-Doppler ultrasound and angiography were the most effective diagnostic tools to distinguish high- from low-flow priapism. The success rate was 20% for shunt operations and 89% for arterial embolization. In conclusion, embolization was effective in the majority of cases of traumatic HFP, while shunt surgery remained disappointing. For HFP caused by inherited diseases and malignoma conservative therapy is mandatory.

Introduction

Priapism is defined as a persistent erection without sexual stimulus. Until 1960, diminished blood efflux from the cavernous bodies with stasis was known as the only pathophysiological mechanism to cause priapism. In 1960, FB Burt (Baltimore)1 described the first case of high-flow priapism (HFP) postulating a pathologically increased arterial influx into the cavernous bodies as being causative. The clinical feature of this entity is a painless erection of the cavernous bodies while the corpus spongiosum stays flaccid. Blood samples drawn from the cavernous bodies of patients with HFP reveal oxygen saturation as observed in arterial blood. Furthermore, color-Doppler ultrasound and arterial angiography visualize a pathologically increased arterial flow into the cavernous bodies. Without treatment, HFP may lead to an impaired erectile function. In contrast to HFP, the incidence of low-flow priapism is high, as it can be seen as a side effect due to intracavernous pharmacotherapy of erectile dysfunction. HFP remains a rare feature. In the last 42 y, only 202 cases have been published, most of them as case reports. The largest series contains 11 patients.

The aim of this study is to analyze the development of diagnostic tools and the changes of therapeutic approaches and final results in HFP by concluding the cases presented in the world literature.

Materials and methods

Information about HFP in the world literature was acquired using several internet-based data sources such as pubmed, medline, ovid and silver platter.

The published data of a total of 202 cases of HFP was evaluated regarding diagnostic and therapeutic procedures and long-term results. The following information was stored in a database for further analysis: age of the patient, history of previous priapism, etiology, clinical feature, aspiration of blood from the cavernous bodies (including data on blood-gas analysis), findings in B-mode and color-Doppler ultrasound, findings in arterial angiography, precise description of the localization of each arteriocavernosal fistula, and findings in cavernosography, intracavernosal application of drugs and the effects, surgical approaches such as different types of shunt surgeries and their results, arterial transcatheter embolization (including embolization material, localization of the embolization, complications, repeated embolizations and results), conservative approaches, color-Doppler ultrasound or angiography during follow-up, duration of the maximum follow-up, duration until restored erectile function and long-term erectile function.

Treatment success was defined as a completely restored erectile function without recurrent priapism. Failure was defined as recurrent priapism or lost or impaired erectile function, respectively.

Results and discussion

Since the first case of HFP was reported in 1960 by Burt et al1 it took another 23 y until Hauri et al,2 presented a generally accepted hypothesis explaining the etiology and pathomechanism of HFP. Only 18 cases were described in 12 papers in these 23 y. Until now, 202 cases of HFP have been reported in the literature, the majority of them as case reports.

A pathologically increased arterial influx to the cavernous bodies can be caused by different reasons: 70.5% of all reported patients suffered from a traumatic or iatrogenic laceration of a penile artery (blunt penile trauma: 3.8%, blunt perineal trauma 40.4%, straddle trauma: 24.4%, cavernosography: 0.6%, penile revascularization: 1.3%), another 4.5% were caused by a malignant arrosion of the penile vessels. A total of 5.8% of the cases with HFP were described in patients with inherited diseases (sickle cell anemia, Fabry's disease), although the pathomechanism could not exactly be defined: a disturbed autonomic regulation of the penile blood flow, vasculopathies or iatrogenic damages from blood aspirations or shunting have been discussed.3 Drug abuse and intracavernous injection of Prostaglandin E1 known to be associated with low-flow priapism contribute to only 2.5% of all cases of HFP. In 16.7%, the etiology remained unclear. In six cases, aspiration of blood from the cavernous bodies changed from venous to arterial blood in repeated examinations. In these cases, the etiology may be an initial low-flow priapism with secondary iatrogenic arterial damage from aspirations or shunting.

Comparing the age of onset of disease in patients with HFP, the review of the literature showed that traumatic HFP mainly occurs in children and adults under 55 y. HFP caused by inherited diseases affects only children or younger adults. In older men it remains a rare event. In these cases, the main reason for HFP is the malignant arrosion (Figure 1).

Figure 1
figure1

Age distribution and etiology of HFP.

Since the O2-saturation in the cavernous bodies is elevated to arterial levels, there is no risk of ischemic damage of the tissue, but long-term changes like an intracavernous pseudoaneurysm with secondary fibrosis may lead to an impaired erectile function. The longest reported time from trauma to therapy was 36 y in a patient who suffered from erectile impotence after 31 y of HFP and was successfully treated by arterial embolization 5 y later.4 In all, 10 cases with HFP lasting longer than 1 y have been published.4, 5, 6, 7, 8 At least two patients with a HFP for 3 and 10 y had a persistently impaired erectile function following successful arterial embolization. These limited number of cases may indicate long-term damage of untreated HFP leading to impaired success of delayed therapy.

Table 1 shows a timesheet covering the last 42 y looking at published diagnostic and therapeutic approaches to HFP. The main achievements for diagnosis of HFP have been the blood-gas analysis in samples taken from the cavernous bodies showing a high O2-saturation, the color-Doppler ultrasound proving an increased arterial influx to one or both cavernous bodies and the arterial angiography. Using these diagnostic tools, the localization of the arteriocavernous fistula was possible for the first time. The first arteriography examination in a patient with HFP was reported by Wheeler and Simmons10 in 1973, showing a fistula deriving from branches of the internal pudendal artery. The growing experience with this technique allowed a more and more precise localization of the fistula (Table 2): 99% of all fistulas originated from the internal pudendal artery or its branches. Only one case with a fistula from the obturator artery is known.2 Relevant arterial malformations like an accessory pudendal artery remain a rare event. Recently, the arterial angiography and the color-Doppler ultrasound were combined in an interventional approach to reduce radiation exposure, especially in children.30 Attempts like cavernosography had only minor value. Changes of the cavernosal tissue shown by this examination proved to add only limited information. Nuclear medicine proved to differentiate a stagnant from a not stagnant type of priapism.4 The aspiration of blood from the cavernous bodies, the intracavernosal injection of metaraminol or phenylephrine, the caudal anesthesia or shunt operations such as the techniques described by Winter, Ebbehoj, Grayhack or Al Ghorab are accepted therapy options in patients with low-flow priapism. All of these methods were tested for HFP—mainly when the pathomechanism of the priapism was unclear. The cavernosal blood aspiration or intracavernosal injection of drugs failed in all cases of HFP. Shunt operations were of limited value: a total of 35 patients were treated by different shunt operations. Only one patient was cured by the first shunt surgery, another six were cured by a second or third shunt operation resulting in an overall success rate of 20%. Of these 35 patients, 18 were finally cured by a subsequent embolization or ligation of the lacerated artery. The overall failure rate was 29%. Open surgical ligation of the affected artery had a high morbidity and often caused permanent erectile dysfunction. The breakthrough in the therapy of HFP was the first transcatheter embolization of an internal pudendal artery by autologous clots performed by Wear et al12 in 1977. Since then, 100 cases of embolization therapy have been published. With a maximum of three sessions, every patient with HFP was successfully treated by embolization alone with a rate of impaired erectile function of 11%. Since the first embolization of the internal pudendal artery, different techniques and materials have been developed to perform supraselective embolizations. As shown in Figure 2 arteriocavernous fistulas originating from the cavernous artery were treated by more and more selective embolizations of the penile arteries to avoid a severe disturbance of the penile blood flow. The embolization of bulbo-cavernosal arteries became only possible by the use of microcoils. Recent approaches are less-invasive techniques to achieve a closure of the fistula like an ultrasound-guided compression of the damaged artery. For patients with sickle cell anemia, sickle-β-thalassemia or Fabry disease, transfusions and hydratization have been proposed as the therapy of choice with excellent success rates, when an arterial damage could be excluded. In patients with malignant arrosion of penile arteries, the local or systemic therapy of the malignant tumor is predominant, since painless HFP plays a minor role in these patients.

Table 1 Timetable of diagnostic and therapeutic approaches to high-flow priapism
Table 2 Angiographic localization in published data on 84 cases with high-flow priapism
Figure 2
figure2

Arteriocavernous fistula originating from the cavernous artery: site of the embolization.

Conclusion

The diagnostic and therapeutic concepts of HFP have been developed within the last 42 y. Blood-gas analysis of cavernosal blood, color-Doppler ultrasound and angiography are the most effective diagnostic tools to distinguish a high- from a low-flow priapism. Results of patients with a traumatic arterial laceration treated with the supraselective arterial embolization demonstrated that this technique is highly efficient. The rate of impaired erectile function was lower than in other surgical approaches. Only in patients with inherited diseases or malignoma the conservative systemic therapy has to be considered as the state-of-the-art treatment.

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Correspondence to B G Volkmer.

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Kuefer, R., Bartsch, G., Herkommer, K. et al. Changing diagnostic and therapeutic concepts in high-flow priapism. Int J Impot Res 17, 109–113 (2005). https://doi.org/10.1038/sj.ijir.3901257

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Keywords

  • priapism
  • erectile dysfunction
  • embolization

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