Priapism and glucose-6-phosphate dehydrogenase deficiency: An underestimated correlation?

Abstract

Priapism is a rare clinical condition characterized by a persistent erection unrelated to sexual excitement. Often the etiology is idiopathic. Three cases of priapism in glucose-6-phosphate dehydrogenase (G6PD) deficiency patients have been described in literature. We present the case of a 39-year-old man with glucose-6-phosphate dehydrogenase deficiency, who reached out to our department for the arising of a non-ischemic priapism without arteriolacunar fistula. We suggest that the glucose-6-phosphate dehydrogenase deficiency could be an underestimated risk factor for priapism.KEY WORDS: Non-ischemic; Glucose-6-phosphate dehydrogenase (G6PD) deficiency. obliged him to go to the emergency department. He reported a normal and spontaneous erectile function before the arising of the disease. He denied having any gender of trauma and he admitted to have a G6PD deficiency, documented by laboratory tests. He denied having taken any drugs, phosphodiesterase type 5 (PDE5) inhibitors, intracavernosal agents or any other pharmacological agents. He denied any other risk factor for priapism. At clinical examination he presented a painless erection. Systemic hemoglobin was 13.6 g/dL. Intracorporal blood resulted as arterial at gas analysis (P0 2 = 76 mmHg; PC0 2 = 44.5 mmHg; pH = 7.35). The Color Doppler ultrasonography didn't point out any arterocavernosa fistula. After a period of observation in which there wasn't the resolution of the priapism, we decided to submit him to a pelvic arteriography (Figure 1). Panoramic arteriography of the aorto-iliac and selective of internal pudendal artery showed a bilateral increase of the arterial level of the corpora cavernosa, higher in the left one, without a fistula. We performed a temporary embolization with Spongostan TM of the distal left internal pudendal artery. A complete detumescence was obtained and the next arteriography confirmed the procedure was a success. The patient was released. After 2 weeks of a state of good health, he presented a new episode of prolonged erection, after which he returned to the emergency department of our Institute.