Case Report: Successful treatment of refractory high-flow priapism in a patient with sickle cell disease by selective trans-catheter embolization using an autologous blood clot: A case report

Introduction

Priapism is an abnormal prolonged and persistent penile erection lasting for more than 4 h that is unrelated to sexual desire, stimulation or activity^1–5. Priapism is categorized into three types: low-flow (ischemic, veno-occlusive), high-flow (non-ischemic, arterial), and stuttering (recurrent or intermittent ischemic)⁵. The low-flow or ischemic form is painful and is the commonest type of priapism (95%)⁴. High-flow or non-ischemic priapism is rare, painless and is commonly associated with pelvic, perineal or direct penile trauma due to injury to the cavernous artery^6–9. Stuttering priapism is ischemic in nature associated with multiple recurrent intermittent self-limiting episodes of persistent erection usually lasting less than 3–4 h and its commonest cause is sickle cell disease (SCD)⁵. Patients with SCD have an increased risk of low-flow and stuttering priapism, but high-flow priapism is relatively uncommon in these patients⁵.

We report a case of non-traumatic refractory high-flow priapism evolving from a stuttering low-flow priapism in a SCD patient. The patient was successfully treated by super-selective transcatheter embolization of the penile arteries with an autologous blood clot.

Case report

A 37-year-old patient, who was known to have SCD, glucose-6-phosphate dehydrogenase deficiency and hypertension, presented with priapism. Initially, he developed self-limiting intermittent episodes of sustained erection without sexual excitation for 3 months. Each episode lasted for less than 2 h. Eventually he presented with a sustained erection for 12 days duration in another facility, where he received treatment for low-flow priapism by repeated corporal aspirations and transfusion of 3 units of blood with no detumescence. He was then referred to our hospital with refractory priapism associated with SCD.

The patient’s blood results revealed a hemoglobin level of 8.9 g/dl (normal, 14–18 g/dl), with 94.7% hemoglobin S. The patient was treated with intravenous hydration and alkalization, nasal oxygen and exchange transfusion. Aspiration of the corpora revealed bright red blood. The patient did not have any significant penile pain at any stage. Color Doppler ultrasound imaging demonstrated a marked increase in the flow of the penile arteries. There were no features of arterio-cavernous fistula or psuedoaneurysm.

After discussing the possibility of impotence, the patient agreed for selective embolotherapy. Following the obtainment of written informed consent, pelvic digital subtraction angiography was performed via a right transfemoral artery approach. A 5 French vascular access sheath was placed in the right common femoral artery and a 5 French C2 catheter was engaged in the right internal iliac artery. A 2.4 French microcatheter was advanced coaxially into the ipsilateral internal pudendal artery, which was embolized with an autologous blood clot (Figure 1 and Figure 2). The C2 catheter was then engaged in to the contralateral left internal iliac artery using the cross-over technique and the left internal pudendal artery was embolized with an autologous blood clot after selective catheterization with a microcatheter (Figure 3 and Figure 4).

Figure 1. Right internal pudendal artery angiogram (pre-embolization).

Figure 2. Right internal pudendal artery angiogram (post-embolization).

Figure 3. Pre-embolization arteriogram of the left internal pudendal artery.

Figure 4. Post-embolization arteriogram of the left internal pudendal artery.

There was incomplete detumescence of the penis. Mild tumescence was expected due to considerable cavernous tissue fibrosis. Reduced blood flow was seen in the penile arteries on color Doppler ultrasound after transcatheter embolization. There were no further episodes of priapism and he had adequate self-limiting erections for intercourse.

Discussion

Ischemic priapism is an emergency due to the potential risk of developing permanent erectile dysfunction, whereas non-ischemic priapism can be treated conservatively or less aggressively¹⁰. Patient history, physical examination, aspirated blood gases and penile Doppler ultrasonography help to categorize priapism into ischemic and non-ischemic types for its appropriate management. In the ischemic form of the priapism, fully rigid corpora cavernosa; relative sparing or little involvement of the corpus spongiosum and glans penis; hypoxic and dark aspirated corporal blood; and absent or minimal arterial blood flow are seen¹⁰. In the non-ischemic type, the corpora cavernosa are not fully rigid, the aspirated corporal blood is bright red without hypoxia or acidosis and is associated with increased blood flow, arteriolar–sinusoidal fistula or pseudoaneurysm¹⁰. Doppler ultrasound study of penile arteries can be helpful in cases with equivocal clinical findings, where mean and peak systolic velocities can differentiate between ischemic and non-ischemic forms¹¹.

Low-flow priapism is initially managed with intravenous hydration, alkalization, analgesia and exchange transfusion⁴. However, if priapism persists, further treatment includes corporeal blood aspiration, irrigation with non-heparinized saline and intracorporeal administration of alpha-adrenergic agonists (sympathomimetic agents such as phenylephrine)⁴. The surgical shunt procedure is the last resort when all attempts of other non-surgical treatment options have failed^4,12. The initial management of high-flow priapism is observation and two-thirds of high-flow priapism patients resolve spontaneously during observation^13,14. In the rest of the patients with high-flow priapism, embolotherapy is a frequent treatment option, which can be performed in a number of way, including by use of autologous blood clot, gel foam, platinum microcoils or acrylic glue^10,15–17. Complications of embolization can include permanent erectile dysfunction, penile gangrene, inadvertent migration of the embolization material into other regional arteries, gluteal ischemia and perineal abscesses¹⁸. Autologous blood clot embolotherapy has been reported in post-traumatic high-flow priapism patients¹⁹. In our case, after the failure of initial management by corporeal aspiration, the non-traumatic high-flow state was successfully treated using autologous blood clot embolotherapy. The autologous blood clot and gelatin foam have transient effect as embolization agents²⁰ and their use in high-flow priapism patients has the theoretical advantage of recanalization of penile arteries, as compared to platinum microcoils, to reduce the risk of permanent erectile dysfunction. Embolization can be repeated in cases of recurrence. If embolotherapy fails, surgical management is the last treatment option²¹.

Conclusions

Super selective embolization of unilateral or bilateral penile arteries with autologous blood clot is a relatively safe and effective non-surgical treatment option for high-flow priapism, even in patients with SCD and has a low risk of erectile dysfunction.

Consent

The patient provided written informed consent for the publication of this case report.

Data availability

All data underlying the results are available as part of the article and no additional source data are required.