235Archivio Italiano di Urologia e Andrologia 2016; 88, 3 CASE REPORT Priapism and glucose-6-phosphate dehydrogenase deficiency: An underestimated correlation? Aldo Franco De Rose 1, Guglielmo Mantica 1, Mattia Tosi 1, Giulio Bovio 2, Carlo Terrone 1 1 Department of Urology, IRCCS San Martino Hospital, University of Genova, Genova, Italy; 2 Department of Radiology, IRCCS San Martino Hospital, University of Genova, Genova, Italy. Priapism is a rare clinical condition char- acterized by a persistent erection unrelat- ed to sexual excitement. Often the etiology is idiopathic. Three cases of priapism in glucose-6-phosphate dehydroge- nase (G6PD) deficiency patients have been described in lit- erature. We present the case of a 39-year-old man with glu- cose-6-phosphate dehydrogenase deficiency, who reached out to our department for the arising of a non-ischemic pri- apism 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 dehydroge- nase (G6PD) deficiency. Submitted 14 May 2016; Accepted 30 May 2016 Summary No conflict of interest declared. 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 defi- ciency, documented by laboratory tests. He denied having taken any drugs, phosphodiesterase type 5 (PDE5) inhibitors, intracavernosal agents or any other pharmaco- logical agents. He denied any other risk factor for pri- apism. At clinical examination he presented a painless erection. Systemic hemoglobin was 13.6 g/dL. Intracorporal blood resulted as arterial at gas analysis (P02 = 76 mmHg; PC02 = 44.5 mmHg; pH = 7.35). The Color Doppler ultrasonography didn’t point out any arte- rocavernosa 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 SpongostanTM 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. DOI: 10.4081/aiua.2016.3.235 Figure 1. Pelvic arteriography showing a bilateral increase of the arterial level of the corpora cavernosa, higher in the left one, without a fistula. INTRODUCTION There are three types of priapism with different patho- physiological causes: the ischemic priapism (or “low flow”), which is a dolorous and prolonged erection with a tissue ischemia, the non-ischemic (or “high flow”) pri- apism, which is usually due to a perineal injury with the formation of an arteriolacunar fistula, and the stuttering (intermittent) priapism, which is a recurrent form of ischemic priapism in which unwanted painful erections occur repeatedly with intervening periods of detumes- cence. All types of priapisms can lead to an erectile dys- function (1), not only the ischemic one. For that reason, it is important to treat it in time and in correct way, but also to identify the possible risk factors. Three cases of priapism in patients with G6PD deficiency have been described in literature (2). We report an unique case of “high flow” priapism without fistula in a patient with G6PD deficiency. CASE PRESENTATION In October 2015, a 39-year-old man reached to our observation for an intermittent, painless erection, every- day, for about one month. He reported erections lasting about 4-6 hours during the first days of the month. These erections stopped in the following days, then he had another erection lasting more than 17 hours, which Mantica_Stesura Seveso 21/09/16 09:06 Pagina 235 Archivio Italiano di Urologia e Andrologia 2016; 88, 3 A.F. De Rose, G. Mantica, M. Tosi, G. Bovio, C. Terrone 236 This time the erection was higher and mildly painful. He still denied having taken drugs or medications. We per- formed a gas analysis (p02 = 21.3 mmHg; pC02 = 56.4; mmHg; pH = 7.34). We carried out an evacuation of blood from the cavernosa corpora through transglandu- lar cannula and injection of Etilefrine which lead to a detumescence. After an observation time of 24 hours without erections, we decided to discharge the patient from our department. Three months later he affirmed to have preserved a sufficient erectile function (IIEF-5: 18). CONCLUSIONS The number of priapisms, classified as idiopathic, both ischemic and not-ischemic, continues to be high. Among the clinically significant enzymatic defects, G6PD defi- ciency is the most common (3), with hundreds and mil- lions of affected people. With the proliferation of the G6PD deficiency, especially in some countries, we suggest it could be a casual factor or a co-factor, responsible for some cases of priapism classified as idiopathic. Therefore, now we consider the G6PD deficiency as a underestimat- ed risk factor for the emergence of priapism. DISCUSSION Priapism can be associated with some hematologic, coag- ulative and enzymatic disorders (4-5) such as leukemia and sickle cell anemia. Hematologic diseases are usually associated with a “low flow” priapism, but some associa- tions have also been reported between “high flow” pri- apism and sickle cell anemia (6). Both type of priapism are also associated with Fabry disease (7, 8). Nowadays, few cases of priapism, related to a G6PD deficiency, are present in literature, even if the correlation between the two disorders has been discovered (2, 9, 10). The cases, which have emerged until now, showed a link between the G6PD deficiency and the “low flow” priapism. In our case, the patient initially showed a “high flow” priapism, without the presence of other risk factors for the disor- der. The interesting thing was the absence of fistula at the arteriography, but the presence of an increase of the blood flow in the corpora cavernosa. Although the arrangements are unknown, the G6PD deficiency could be responsible for that increase of blood flow and it could lead to a not-ischemic priapism other than an ischemic one, as it’s already documented and assumed for the other enzymatic and hematologic defects (6-8). REFERENCES 1. Zacharakis E, Ralph David J, Walkden M, et al. Distal corpus cavernosum fibrosis and erectile dysfunction secondary to non- ischaemic priapism. Arch It Urol Androl. 2015; 87:258-9. 2. Morrison BF, Thompson EB, Shah SD. Ischaemic priapism and glucose-6-phosphate dehydrogenase deficiency: a mechanism of increased oxidative stress? West Indian Med J. 2014; 63:658-60. 3. WHO Working Group. Glucose-6-phosphate dehydrogenase defi- ciency. Bull World Health Organ. 1989; 67:601-11. 4. Kalathia J, Agrawal S, SudirChipde S, Agrawal R. Homo - cysteinemia: a rare cause of priapism. Urol Ann 2016; 8:118-21. 5. Morrison BF, Burnett AL. Priapism in hematological and coagu- lative disorders: an update. Nat Rev Urol. 2011; 8:223-230. 6. Ramos CE, Park JS, Ritchey ML, Benson GS. High flow priapism associated with sickle cell disease. J Urol. 1995; 153:1619-1621. 7. Foda MM, Mahmood K, Rasuli P, et al. High-flow priapism asso- ciated with Fabry’s disease in a child: a case report and review of the literature. Urology. 1996; 48:949-952. 8. Backenroth R, Landau EH, Goren M, Raas-Rothschild A. Fabry disease and G6PD in three family members with priapism: is the nitric oxide pathway to blame? J Sex Med. 2010; 7:1588-1591. 9. Burnett AL, Bivalacqua TJ. Glucose-6-phosphate dehydrogenase deficiency: an etiology for idiopathic priapism? J Sex Med. 2008; 5:237-240. 10. Finley DS. Glucose-6-phosphate dehydrogenase deficiency asso- ciated stuttering priapism: report of a case. J Sex Med. 2008; 5:2963-2966. Correspondence Aldo Franco De Rose, MD aldofrancoderose@gmail.com Guglielmo Mantica, MD (Corresponding Author) guglielmo.mantica@gmail.com 3383699431 Mattia Tosi, MD matti.grifone@hotmail.it Carlo Terrone, MD Department of Urology, IRCCS San Martino Hospital, University of Genova Largo Rosanna Benzi 10 - 16131, Genova, Italy Giulio Bovio, MD giulio.bovio@hsanmartino.it Department of Radiology, IRCCS San Martino Hospital, University of Genova Largo Rosanna Benzi 10 - 16131, Genova, Italy Mantica_Stesura Seveso 21/09/16 09:06 Pagina 236