地中海貧血合併反覆發作之缺血性陰莖異常勃起:個案報告及文獻回顧

陳冠宇^1,2、陳順郎^1,2、謝佐宜^1,2、陳文榮^1,2、宋文瑋^1,2、楊旻鑫^1,2、何承儒^1,2、王紹全^1,2

¹中山醫學大學附設醫院 泌尿科；²中山醫學大學醫學院

Recurrent ischemic priapism with thalassemia: A Case report and literature review

Kuan-Yu Chen^1,2, Sung-Lang Chen^1,2, Tuzo-Yi Hsieh^1,2, Wen-Jung Chen^1,2, Wen-Wei Sung^1,2, Ming-Hsin Yang^1,2, Cheng-Ju Ho^1,2, Shao-Chuan Wang^1,2

¹ Department of Urology, Chung Shan Medical University Hospital, Taichung, Taiwan;

² School of Medicine, Chung Shan Medical University, Taichung, Taiwan

Introduction: Priapism, a persistent erection of the penis without sexual stimulation or desire, is a relatively rare condition. It can be classified as ischemic or nonischemic. Ischemic priapism is considered a urologic emergency due to the possibility of ischemia, compartmental syndrome, and irreversible changes in the cavernous and endothelial smooth muscle cells ^[1]. The estimated incidence rate was 0.73 per 100,000 men per year in the United States ^[2]. Priapism can be seen in any age, and there is a bimodal peak distribution of 5 to 10 years in children and 20 to 50 years in adults. Though the underlying cause is not identified in most cases, certain associated conditions were identified, such as intracavernosal injections and sickle cell disease. Treatments of ischemic priapism include intracavernosal injection of a sympathomimetic drug (ICI), aspiration, shunt surgery, and placement of a penile prosthesis. Here, we are going to present a case of recurrent ischemic priapism with thalassemia.

Case presentation: A 34-year-old man with a history of thalassemia, asthma, and depression under medication control. He suffered from intermittent painful erection for 1 year. The symptoms have exacerbated in the last 2 months. It occurred up to 5 times in one month. The duration of the erection may last 2-3 hours but spontaneous detumescences. Sometimes, the erection may be prolonged more than 4 hours and accompanied by dramatic pain. He denied using any Phosphodiesterase-5 (PDE5) inhibitors or other recreational drugs. He had visited Chinese Medical University Hospital (CMUH) for help. Under the impression of ischemic priapism, he had several times of aspiration at the emergency department of CMUH. Surgical intervention with a shunt procedure was suggested, but he hesitated about the operation. Another priapism episode was noted in 2022-06, and he visited our emergency department (ED) for help. At ED, his vital signs were a body temperature of 36.8 degrees Celsius, pulse rate of 111, blood pressure of 140/61mmHg, and respiratory rate of 17 times per minute. Physical examination showed swelling and a dark-purple appearance of the penis, suspecting ischemic priapism. Lab data showed hemoglobin of 9.4. Cavernosal blood gas values showed pCO2 of more than 60 mmHg. Aspiration therapy for symptom relief and blood transfusion due to a near-fainting sensation were performed. After a full explanation and discussion with the patient, he had a T-shunt operation and circumcision on 2022-07-15. No more priapism episode was noticed after the procedure during outpatient department follow-up.

Discussion:

Conservative and medical treatments are considered the first step in ischemic priapism. According to a systematic review, Intracavernous injections with sympathomimetic agents (ICIs) were the most frequently utilized treatment with efficacy ranging from 0 to 100% of cases. The combination of ICIs with corporeal aspiration with or without saline irrigation was successful in 70 to 100% of cases ^[3]. In a large database analysis consisting of 6392 men, there were 693 (11%) proceeded to surgical shunt and 144 (2%) underwent initial penile prosthesis (PP) placement ^[4]. In a systemic review of penile prosthesis (PP), there was no superiority of immediate implantation over delayed implantation, but early implantation was favored due to the reduced complication rate and the ease of the procedure ^[5]. Although the exact mechanism is unknown, various antipsychotic drugs (APDs) have been implicated in inducing priapism with a possible hypothesis of α-adrenergic blockade in the corpora cavernosa ^[6]. In a literature review of thalassemia and priapism, there were only 8 reported cases from 1986 to 2020 ^[7]. Postulated mechanisms include increased blood viscosity, thrombotic occlusion of efferent venules in the corpora cavernosa, abnormal nitric oxide activity in the penile tissue, downregulation of phosphodiesterase-5 activity, and the overresponse of the penile smooth muscle ^[8]. Early identification and prompt management of priapism are essential to mitigate the risk of erectile dysfunction.

Conclusion:

In this case, we demonstrated the presentation and management of recurrent ischemic priapism in a patient with thalassemia. Timely identification and appropriate intervention aim to prevent further tissue damage and preserve erectile function. More effective preventive strategies and correlated outcomes rely on further ongoing research to validate.