University of Groningen Pathologic erections Vreugdenhil, Sanne

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Pathologic erections

Vreugdenhil, Sanne

DOI:

10.33612/diss.95437816

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Publication date: 2019

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Vreugdenhil, S. (2019). Pathologic erections: historical, pathophysiological and clinical aspects. Rijksuniversiteit Groningen. https://doi.org/10.33612/diss.95437816

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CHAPTER 6

Sanne Vreugdenhil MD1_{,Igle Jan de Jong MD PhD}1_{, Mels Frank van Driel MD PhD}1 1_{Department of Urology, University Medical Center Groningen,}

University of Groningen, Groningen, the Netherlands

A summarized version of this chapter was published Urology. 2018 Aug;118:21-24. https://doi.org/10.1016/j.urology.2018.04.029

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Abstract

Background Prolonged and painful penile erection is a rare condition, which has fascinated mankind for centuries. Insights concerning its etiology and treatment evolved considerably throughout the ages.

Aim To highlight medico-historical and sexual medicine-related aspects of priapism and sleep-related painful erections throughout the ages.

Methods Review of old German, Dutch, French and English medical books on male sexual functioning and review of old articles on priapism and painful sleep-related erections in the scientific literature.

Results The first recorded evidence of priapism was found in the Egyptian Ebers papyrus originating from 1550 BC. In 1552 the French physician Thierry de Hery was the first who described priapism in modern scientific literature, with clear references to Galen. The etiology, diagnosis and treatment options greatly evolved throughout the ages. Until the nineteenth century, treatments were without real efficacy, except sometimes a temporary analgesic effect. The list of the used ‘medication’ is long, varying from rhubarb, chamomile, flowers of the elderberry and opium. In the nineteenth century the treatment strategies were threefold; localized medical and surgical therapies, systemic treatments such as emetics and bloodletting and those specifically intended to sedate or temper sexual desire. In 1824 Thomas Callaway was the first to perform a surgical treatment. However, without antibiotics the infection rate was high and the results with regard of erectile function were disappointing, just as those of several types of shunt surgery in the second half of the 20th century. The first report on traumatic high-flow priapism in modern scientific literature appeared in France in 1938 and focused on injury to the perineum while bicycling. In 1984 Brindley reported his first successful experiments with the intracavernous injection of the adrenergic drug metaraminol and after 2009 T-shunt surgery with ‘snake tunneling’ became more or less the procedure of first choice after failed intracavernous treatment.

Conclusions The etiology, diagnosis and treatments of priapism greatly evolved throughout the past human history. A better understanding of the pathophysiology and the different types of long lasting erections significantly changed the diagnostic pathway. However, the overall treatment results with regard of erectile function are up till now disappointing. A major problem remains that a lot of patients with priapism present too late, in many cases due to ignorance, guilt or shame. Urologists should explain them the very high risk of erectile dysfunction, despite active intervention or no treatment at all and the documentation of this should be meticulous.

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89

Introduction

Priapism is relatively rare and many patients come too late out of ignorance, guilt or shame. The condition has also been reported in animals as the dog, cat, rat and the sea lion.(1) For horse breeders, it is a signifi cant disease in economic perspective while unresolved priapism leads to impotence of the stallions and thus the loss of income.(2) In urological terms, long lasting penile erections are closely associated with the god Priapus. In Greek mythology he was the god of the city of Lampsacus, which now is the Turkish town Lampsaki on the Dardanelles.(3) Originally, however, Priapus came from Asia. In ancient Greece he was regarded as the son of Dionysus, god of the vine, and Aphrodite, goddess of beauty. Due to a curse, imposed by her jealous mother Hera, Aphrodite gave birth to a misshapen dwarf with a large erect penis.(4) As a result this dwarf was subjected to ridicule and assumed to lack intelligence.

One version of the rest of the story tells that Aphrodite sent her son away to Lampsacus, where the citizens loved him and embraced and deifi ed him. After his arrival, they believed that Lampsacus had become more dynamic with increased productivity, which they attributed to his enormous phallus. As a result, the inhabitants regarded him as the god who protected their vineyards and gardens. However, at the end of the story he was banished, because of his advances to male citizens’ spouses. Another version tells that Priapus became a god of fertility, since he was brought up by shepherds who noticed that wherever they took him fl owers bloomed abundantly and animals copulated furiously.

In the Roman empire the Priapus cult came to expression relatively late. The specifi c sexual function of his erect, red painted penis was conceived more diff use and magical.(5) Priapus also became the protector of livestock, shipping and fi shing. His statues along roads and fi elds served as protection against thieves and robbers and in the Roman gardens Priapus’ representation threatened intruders with rape.(6) The main purpose of the Priapus idolatry, however, was to defuse the ‘evil eye’. For that reason, one worn little images of Priapus around the neck as an amulet in order to provide magical protection against sickness, to ensure a victory for militaries and obviously also to prevent erectile dysfunction. Sometimes off erings of fruits and vegetables were placed nearby his statue. Priapus also starred more than eighty surviving Latin poems. Later on, these verses were named Priapea. Many of these are, even today, remarkable for their extreme obscenity. Most appeared in the Augustan age (c. 43 BC–AD 18). Originally, they either may have been the leisure products of aristocratic voluptuaries or genuine inscriptions on shrines of Priapus. Since antiquity, long-lasting erections have been the object of scientifi c interest. In this article we present the historical developments leading up to the current state of knowledge about this potentially dangerous medical condition. We have reviewed original primary sources written in Latin, German, English, Dutch and French. We have placed our fi nding in a historical context and summarized the contributions of not just physicians but also historians and philosophers so that we can refl ect on the observations that have led to the current insights.

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90

Antiquity and the Middle Ages

The first recorded evidence of priapism is found in the Egyptian Ebers papyrus originating from 1550 BC.(7) It was purchased in 1872 by the German novelist Georg Ebers, after whom it was named. Today, the papyrus can be found in the library of the University of Leipzig, Germany. The twenty meters long document is a hundred-and- ten-page scroll and one of the oldest preserved medical treatises.

Figure 1. Photograph of the original Egyptian Ebers papyrus from 1550 BC. [Permission was obtained at the Library of the University of Leipzig, where the original papyrus can be visited.

According to this document, a persistent erection can be treated with watermelon, flax, pine and hyoscyamus (a small genus of flowering plants in the nightshade family).

Centuries later, the Greek scientist Demetrius of Apamea (fl. late-third to early-first century BC) systematically performed dissections of human cadavers. Demetrius gave one of the first descriptions of a persistent erection and distinguished priapism from satyriasis (hypersexuality).(8) Without any doubt Galen of Pergamon (129-200/216 AD) was the most famous of the ancient physicians after Hippocrates. His treatises were mainly based on the dissection of animals.(9,10) Seven of his case reports concerned priapism. In his view priapism could be due to dilated orifices of the arteries and by the formation of pneuma in the cavernous nerve. According to the philosopher Michel Foucault (1926-1984), Galen also blamed the presence of too much sperm: ‘This kind of disease was found in those who had too much sperm and who, contrary to their usual habits, abstained from sexual intercourse (unless they found a means of dissipating in numerous occupations the surplus quantities of their blood), or in those who, while practicing self-control, imaged sexual pleasures after seeing certain spectacles or, as a result of recurring memories.’ (11)

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91 Like Galen, the Medieval physician and surgeon Guy de Chauliac (c. 1300-1368) thought that priapism was due to dilatation of the arteries.(12) This French doctor wrote a lengthy and infl uential treatise on surgery in Medieval Latin, entitled Chirurgia Magna. From 1363 onwards, his treatise circulated in manuscript form before its fi rst printing in 1478. It was translated into English, French, Dutch, Italian and widely read by physicians in late medieval Europe.

The sixteenth century up till the end of the eighteenth century

According to Coscione et al. the fi rst modern description of priapism is often ascribed to Petraens in his article entitled Gonorrhoea, satyriasis et priapisme, which was published in 1616.(13,14) However, in his book La méthode curatoire de la maladie vénérienne, published in 1552, the French physician Thierry de Héry (1505-1599) had already given an outstanding overview of priapism and cited Galen in describing ‘un unwanted erection unrelated to sexual desire.’ (15) In those days the medical treatments of priapism had no signifi cant eff ect, but only an occasional temporary analgesic eff ect. The list of used medication is long, varying from rhubarb, chamomile, fl owers of the elderberry and opium. Ambroise Paré (1510-1590), for example, recommended a poultice of nightshade, rhubarb, salad and water lily.(16) Today, he is still considered as one of the big fathers of surgery and pathology and a pioneer in surgical techniques on the battlefi eld. Paré described the case of a travelling abbot who gained the good graces of a widow in a village where he stopped. She had put cantharides in some comfi ts that she had made for him in order to stimulate his love. On the following day, however, the poor abbot had not only passed bloody urine, but also experienced violent priapism and after a few days he died of penile gangrene. In his book on the seats and causes of diseases investigated through anatomy, which was published in 1761, Giovanni Battista Morgagni (1682-1771) discussed a peculiar form of priapism, namely the postmortem variant in hanged men.(17) However, The Ephemerides from the 1st millennium BC Babylonian astronomy had already discussed this phenomenon. It was the fi rst observation to indicate that priapism could also be of neurogenic origin. In that era, the exact mechanism of priapism due to hanging neck injury was not clear. However, now it can be assumed that the abrupt loss of sympathetic input to the pelvic vasculature as a result of hanging leads to uncontrolled arterial infl ow directly into the sinusoidal spaces of the cavernous bodies. (18) The sympathetic outfl ow arises from approximately T2 to the conus (L1-2) of the spinal cord, which is where the sympathetic nerves to the penis arise from. This means that a lesion at any level in the spinal cord can induce priapism. It usually begins within a few hours after the injury, occasionally up to 30 hours, but rarely requires medical treatment.

In 1772 Maurel, a French surgeon from Bain de Bretagne described an observation of a young boy, suff ering from painful priapism. Herbal tea emulsifi ed with lettuce seed, poultices with breadcrumbs and milk on the penis, soothing embrocation on the bladder region and cold baths did not succeed to resolve the priapism.(19) On examination Maurel found that the erectile muscles were in severe spasm, which he assumed was a consequence of a putrid fever. Videlicet, the patient was exposed to cold air that caused constriction of the skin pores. Maurel hypothesized that the morbid putrid blood had collected in the penis instead, consequently caused irritation of the erectile muscles and the initiation of a vigorous spasm that resulted in priapism.

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92

The nineteenth century

In 2003, Hodgson reviewed solely British case reports on priapism from the nineteenth century, a time when physicians started to benefit from scientific methods and the formal recording of empirical experiences in books and scientific journals.(20) He argued that the treatment strategies described in the British literature were threefold; localized medical- (e.g. cooling, leeches on the perineum, mercury) and surgical therapies, systemic treatments such as emetics, bromide of camphor and bloodletting and special ones intended to sedate or suppress sexual desire.(21) In 1824, Thomas Callaway was the first to perform an operation on a patient with a priapism that had lasted 16 days. Modern pain control through anaesthesia was only discovered in the mid-19th century, so in 1824 surgery had to be done as swift as possible. Callaway incised the left crus of the corpora cavernosa (CC) and after squeezing out a large quantity of dark blood with numerous small coagula the penis became flaccid. Despite this good immediate result, his treatment did not prevent ED. Another early account of priapism in the English medical literature was recorded in 1845 in the Lancet.(22) Mr. John W. Tripe described a `stout’ seaman having `unusually fierce desire’ and an erection that persisted despite of the fact that `congress was frequently resorted to.’ In the Lancet of 1888 Mr. Hulke described a patient that was `drunk on cider’ when he had intercourse before priapism developed, although `neither he nor his wife was aware of the occurrence of anything unusual during the sexual act.’(23)

Until the end of the 19th century, journals in the English language were relatively few and textbooks probably had more influence on physicians. In one of his books, the British gynecologist and anti-masturbation activist William Acton (1813-1875) described how an erection ‘instead of being absent or imperfect may be only too persistent and too perfect.’(24) It is a `terrible and humiliating condition’ and younger clergy who have ‘never given themselves up to self-abuse are not infrequently affected by very distressing instances’, he wrote.

As early as 1842 a French information book for laymen mentions that men can be raided at night by prolonged painful erections.(25) ‘Life can be witnessed through it’, claimed the author, after which he recommended so-called Salmonson pills and emphasized that the condition concerned a ‘nerve issue’ rather than ‘inflammation’. In addition, he advised a ‘cooling’ diet in order to protect the reproductive mechanism.

In 1845 the Frenchman Jadioux reported the first case of priapism in leukemia.(26) In Germany, many scientists also focused on this symptom.(27,28) In 1879, Salzer from Berlin discussed the possible pathophysiological mechanisms of priapism in leukemia, namely an impeded circulation in the smaller vessels and the formation of thrombi due to the altered circulation in the CC or an irritation of the nervi erigentes. He decided in favor of the last, in some cases at least.(29) In his review from 1882 Neumann from Vienna added two case reports.(30)

In 1907 two Parisian surgeons provided an overview of 48 case reports on priapism described in the 19th century.(31) They classified the origin of priapism into five categories: nervous, leukemic, traumatic, inflammatory and idiopathic. We analyzed their report and specified their findings in Figure 2.

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Figure 2. Schematic representation of Terrier & Dujarier’s classifi cation and review of all priapism cases (n = 48) in literature

In 34 out of the 48 cases the priapism ceased spontaneously, but only 3 of these 34 showed no ED. 10 out of the 48 underwent surgical treatment incising the CC with crural (21) proximal, (32-34) middle, (31,35,36) or distal shaft incisions (37) and even transversal punctures of both corpora using a trocar.(38)

In most cases the incision site was left open and compressed with wet hot gauzes, although Terrier and Dujarier themselves advised to suture the tunica albuginea to ‘prevent inner adhesions’. Three out of the 10 patients who underwent surgery remained potent compared to only 3 out of the 38 in whom no surgery was performed. Unfortunately, surgery was often complicated by hematoma and, in an era without antibiotics, the infection rate was high.

By the end of the 19th century, Kast was the fi rst to describe the late histological consequences of long lasting priapism on the erectile tissue. (39) During the autopsy of a leukemic patient who suff ered from prolonged (2 weeks) priapism he found the CC to be transformed into a homogeneous block of dense connective tissue with no trace of spongy tissue left and a thickened tunica albuginea. In response to this, Terrier and Dujarier stated that this fi brotic transformation could be avoided by an early operative intervention.

The fi rst part of the 20th_century

At the start of the 20th_{century the famous American urologist, Frank Hinman Sr. (1880-1961)}

published a long article on the pathophysiology of priapism.(40) His work was carried on by his son who postulated that venous stasis, combined with increased blood viscosity and ischemia, played an important part in the development of priapism.(41) In 1914, Hinman, Sr. found 184 cases reported in the whole of medical literature. Since he mastered diff erent languages, Hinman was in a privileged position. He could analyze case reports of colleagues from non-English speaking countries. In the course of the 20th century, English became the language of science. The common use of the English language opened new worlds, but concomitantly closed the doors to ‘old’ knowledge. A Dutch professor qualifi ed this as ‘organized memory loss’.(42)

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94

In 97 of the 184 formally reported cases found by Hinman, the priapism had occurred between the ages of 20 and 50 in most of the patients. He proposed the following classification (Figure 3). He agreed that it was not always possible to differentiate sharply between nervous and local mechanical causes.

Hinman also analyzed the 33 surgically treated cases. He emphasized that an incision of sufficient length, extending well into the spongy tissue, had to be made slightly back to the mid part of the corpus at the dorsolateral side to prevent the dorsal vessels and nerves at the mid dorsum and the ventrally located urethra from being damaged. A small wick or rubber drain was usually placed before suturing the skin and facial layers. He stated that asepsis during and after the operation was of great importance, since in a lot of the 33 cases infection or even urinary fistulae developed after the surgery.

Figure 3. Schematic representation of Hinman’s classification and review of all priapism cases (n = 170) in literature from 1772-1913

On page 704 of his article Hinman also described painful nocturnal erections and distinguished them from real priapism. He emphasized that these usually subsided quickly if the awakened patient urinated, had a walk around or did something to distract his mind. He already recognized that these erections could disturb the normal sleep pattern. In 1972, Jovanovic from Germany rediscovered the phenomenon of painful sleep-related erections and called these erectio nocturna dolorosa. (43) He treated his patients successfully with sleeping pills and tranquillizers.

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95 In the interwar period, McKay and Colston had already introduced a less invasive method of treatment.(44) They performed aspiration of clots and washing of the CC with saline using a No. 8 Luer needle on a 20cc syringe. They described three patients in whom this procedure was succesfully performed with regard to the achievement of detumescence and pain relief. The authors stated that, besides the fact that this procedure could eff ortlessly be repeated, ‘the method of aspiration causes much less trauma to the neurovascular mechanism producing erection than the more radical surgical procedures.’ However, erectile function was not known or described in two and the other patient experience complete ED.

A rediscovery of surgical, medical and mechanical treatments after World War II

The most extensive review after World War II was published in 1950 by clinicians from Baltimore. (45) They noticed that the fi rst case of priapism associated with sickle cell disease was reported by Diggs and Ching in 1934.(46) Later on, all case series out of the United States concerning priapism showed relatively higher percentages of patients with sickle cell disease. In the same year, Kemper from Rio de Janeiro published a booklet about functional sexual disorders, which included his psychoanalytic treatment of idiopathic recurrent priapism.(47) However, it took an average of 10 hours on the sofa to cure patients.

In 1957, after the unsuccessful application of ethyl chloride spray, spinal anesthesia and sedation, Brody et al. utilized a `new’ technique, which consisted out of heparinization for 4 days, followed by forcible massage of the penis under general anesthesia during 10 minutes. At follow up, however, their patients reported ED, so the technique was abandoned.(48) In 1964 Grayhack et al. described a procedure in which an incision of the CC was followed by the creation of an anastomosis between the distal end of the divided saphenous vein and the right cavernous body to enhance drainage.(49) They choose the saphenous vein because it was readily available, expendable and autologous. Near the base of the penis, the right CC was opened on its dorsolateral aspect and thick old blood and clots were evacuated by irrigation with a heparin solution. The saphenous vein was exposed through a vertical incision, mobilized and divided about 8 cm from its junction with the femoral vein. The free end of the obliquely cut vein was led through a subcutaneous tunnel and anastomosed to an elliptical defect in the tunica albuginea. [Figure 4a] Postoperatively, a blood pressure cuff on the distal end of the penis was infl ated intermittently in order to maintain fl ow through the shunt. Subsequently, the CC gradually became softer. Five months after surgery the anastomosis appeared to have remained patent, since the patient reported satisfactory sexual function.

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96

Figure 4a. Shunting technique: according to Grayhack

Ten years later Cosgrove & Larocque reported that in fifty patients who underwent this operation 62% had remained potent when unilateral shunts had been performed.(50) Later on, however, several studies reported much lower success rates as well as severe complications including pulmonary embolisms and penile gangrene.

Additionally, in 1964 Raymond Quackels (1914-2013) wrote about proximal corpus spongiosum shunting, which is surgically less difficult than the previously described approach.(51) Genuinely, he was inspired by Bolliger who had suggested this approach a few years earlier.(52) With the patient in lithotomy position, Quackels performed a vertical incision over the bulbar urethral area followed by a longitudinal incision in the tunica of one of the CC and the corpus spongiosum. After irrigation, he carefully sutured the anterior and posterior walls of each opposing incision. [Figure 4b]

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97

Figure 4b. Shunting technique according to Quackels

Although such a procedure could be performed anywhere along the shaft, Quackels obviously preferred the bulbar area because of the low risk of inadvertent entry to the urethral lumen, since the corpus spongiosum has a relatively large diameter in this region. In accordance with this, Sacher and coworkers described a series of 12 priapism patients who were successfully treated with a cavernospongiosal shunt using the abovementioned perineal approach. It caused a satisfactory reduction in all 12 of them and 9 also reported partial or complete return of erectile function.(53) Afterwards, there appeared very little information about the long-term results, except for some reports about complications as the formation of urethral strictures, cavernositis, penile gangrene and late development of urethrocavernous fi stula. (54,55)

In 1981, Mohammed Al-Ghorab published his technique that involved a 2 cm long transverse incision on the dorsal surface of the glans, about 1 cm from the coronal ridge. [Figure 4c] He excised a 5 to 5 millimeter segment of both CC, including a part of the septum, creating a relatively large shunt.(56,57) In more than ninety percent of the patients the priapism disappeared, but most of them ended up with ED. (58)

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Figure 4c. Shunting technique according to Al Ghorab

Figure 4d. Percutaneous hunting techniques according to Winter (1), Ebbehøj (2) and Brant & Lue’s T-shunt (3)

In the early seventies Nelson & Winter reverted to the aforementioned surgical old treatments when they stated that the initial therapy should consist out of aspiration and irrigation, and intermittent pneumatic cuff compression for a trial period of 12 to 36 hours, repeating the aspiration 2 or 3 times if necessary.(59) However, in 1976, the second author (Winter) had described a new minimally invasive surgical approach which could be performed under local anesthesia. He created a fistula between the glans and the CC using a true-cut biopsy needle followed by aspiration and irrigation.(60) [Figure 4d-1] He thought that this minor procedure eventually could replace open shunt surgery. However, the long-term results were poor. According to Nixon et al. the recurrence rate and the necessity for reoperation was as high as 85.7%.(58)

In retrospect, the Scandinavian urologist Ebbehøj was the first who published about a percutaneous technique to create a caverno-glandular shunt [Figure 4d-2].(61) Long term follow up of 18 consecutive patients showed that 11 of them had kept normal erectile function.(62) Brant and Lue introduced a corporoglandular T-shunt with intracavernous tunneling performed under local anesthesia.(63) The success of a T-shunt with ‘snake- tunneling’ appeared to be strongly dependent on the duration of priapism.(64) In patients with priapism >48 hours the T-shunt procedure failed to prevent ED.

Intracavernous medical treatments

The first report about the results of intracavernous injection of papaverine in 1982 by Ronald Virag became a milestone in the progress of understanding penile erection.(65) Giles Brindley, a British physiologist, who at the same time had experimented with the alpha- blocker phenoxybenzamine, elicited wide surprise in the spring of 1983 during the annual AUA meeting in Las Vegas.(66) One of the organizers of the meeting challenged him to prove his intracavernous drug therapy’s

4d.

1

2

3 4c

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99 eff ectiveness beyond tables and statistics.(67,68) The answer was unexpected: wearing sweat pants Brindley presented his phenoxybenzamine induced erection to his audience and even invited them to palpate his rigid penis. One year later he reported his fi rst successful experiments with the adrenergic drug metaraminol to treat iatrogenic priapism that had arisen after the intracavernous injection of phenoxybenzamine.(69) For the second time he had followed the great physiological investigators of the past who used themselves as the subject of their own experiments. Without any doubt Virag’s and Brindley’s serendipitous discoveries paved the way for further research and fund raising. Before their experiments there was as a sort of taboo against studying erectile functioning including priapism. `If you studied the heart everyone applauded. But the penis? People thought you were a pervert’, that was what one of the founders of modern sexual medicine, urologist Irwin Goldstein, said in September 1996 in an interview with journalist David Friedman.(70) This allegation was reminiscent of the Dutch scientist Reinier de Graaf (1641-1673) who was already aware of the fact that in science the penis could be a tricky subject. (71) In 2003, Montague et al. reported that, for all patients with ischemic priapism, resolution occurred in 81% treated with epinephrine, 70% with metaraminol, 43% with norepinephrine and in 65% with phenylephrine.(72) However, phenylephrine defi nitely became the drug of choice because of the lowest cardiovascular risks.

Current insights

Today one distinguishes low fl ow, high fl ow and stuttering priapism from sleep- related painful erections. The determination of the acid-base status in the cavernous blood is essential in diagnosing priapism. Penile blood gas studies were fi rst performed by Hashmat in 1977 and reported at the annual AUA meeting in Boston in 1981.(12)

High fl ow priapism

The fi rst report on traumatic high-fl ow priapism in modern scientifi c literature appeared in France in 1938 and focused on injury to the perineum while bicycling.(73) However, priapism as result of handlebar injury is rare. We found only a few other case reports.(74-76) As this type of priapism is painless, patients usually present late. The high arterial infl ow and non- ischemic nature of this type of priapism was scientifi cally conceptualized in 1983 by Hauri et al., based on their fi ndings of penile arteriography and cavernosography.(77) In a review of 201 cases regarding the etiology 70.5% had experienced blunt trauma or iatrogenic laceration of the penile vasculature (perineal trauma 40.4%, straddle injury 24.4%, direct penile trauma 3.8%, complication of cavernosography 0.6% and penile re-vascularisation surgery 1.3%).(78) The fi rst detailed report about the surgical treatment of high-fl ow priapism was published in 1960. Surgery consisted of the ligation of the right internal pudendal artery and was not accompanied by loss of sexual function at follow up in this case.(79) However, with regard of preserving erectile function the results of this surgical treatment performed by others were very disappointing, especially if the high-fl ow priapism was complicated by the formation of a false aneurysm at the place where the cavernous artery had been lacerated. Therefore, endovascular embolization was introduced in 1977 by Wear et al.(80) Today, selective embolization has a success rate up to 90% and leads to signifi cant lower rates of ED compared to open surgery.(81) Unfortunately, none of the recent guidelines gives specifi c advice with regard of the timing of embolization.

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Low flow priapism

Aspiration of ischemic blood followed by intracavernous injection of phenylephrine is the standard initial treatment. However, in general this treatment is not effective in priapism lasting longer than 48 hours, because acidosis and very low glucose levels impair the cavernous smooth muscle response to phenylephrine.(82,83) The objective of shunt surgery – regarded as the second step in the treatment of priapism, especially in the US - is to facilitate blood drainage from the CC bypassing the veno-occlusive mechanism. According to the most recent European guideline no clear recommendation of one type of shunt over another can be given. It is recommended to begin with distal shunting and then escalate to proximal shunting, although the evidence for the benefits of this strategy is limited. Particularly in the US reasonable results have been reported of new shunt surgery: the surgical opening of the distal ends of both CC to the glans, followed by retrograde insertion of a 7/8 Hegar dilator to release old congested viscous blood.(84) Obviously, the success of a T-shunt with ‘snake tunneling’ is dependent on the duration of the priapism.(85) If performed <24 hours after its onset the results will be favorable, although ED will still be present in 50%. In those with a priapism >48 hours the T-shunt procedure fails to resolve the priapism and all patients end up with ED due to smooth muscle necrosis. The European guideline states that in patients experiencing ischemic priapism >36 hours, any shunt procedure may only serve to limit pain sensations without preserving erectile functioning.(81) ED after unsuccessful treatment of priapism is a challenging problem because of the fibrosis within the CC. Patients can be treated with the implantation of a prosthesis in an early or in a later phase. However, after surgery in a later phase nearly all of them will be dissatisfied with the result of the prosthesis with regard of its length, since unsuccessfully treated priapism leads to shortening of the penis. Sometimes, an inflatable prosthesis cannot even overcome the rigidity of the severe fibrosis. This means that occasionally only a semirigid malleable model will be appropriate. For these reasons experts advise to discuss the possibility of prosthesis implantation within two weeks after the onset of the priapism.(86) In that phase it is possible to keep the original length of the CC. Obviously, the potential disadvantages of early implantation should also be discussed, while compared to `virgin’ cases there is a six times higher risk of infection due to necrosis and the preceding intracavernous injections of phenylephrine. In case of preceding distal shunt surgery there is a six percent risk of erosion and urethral injury during dilatation of the corpora.(87) Another serious disadvantage is the overtreatment of patients with either no or only scattered intracavernous necrosis. In practice this means that one should not perform prosthetic surgery within 24 hours after the onset of the priapism. If there is any doubt, one can make a gadolinium-enhanced high definition magnetic resonance imaging (MRI) scan of the penis. Used for the detection of smooth muscle necrosis, it has a sensitivity of nearly hundred percent.(88)

Stuttering priapism and sleep- related painful erections

Today, the existence of sleep-related painful erections (SRPEs) is well recognized. Patients with SRPEs experience episodes of penile pain during nocturnal erections, frequently waking up the person concerned. The SRPEs typically start during a rapid eye movement sleep period. The erections related to sexual activities are not painful and normal in terms of duration and rigidity and generally no penile anatomic abnormalities are found at physical examination. The

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101 underlying pathophysiological mechanism and predisposing factors with regard to SRPEs are not yet known and treatment is still in an expert-based opinion phase.(89) There are some similarities in SRPE patients and in those with so-called stuttering priapism (also mentioned intermittent or recurrent priapism). The term ‘stuttering priapism’ comes from Emond et al. who studied patients with sickle cell disease in a Jamaican clinic.(90) The European guideline says that the causes of stuttering priapism are similar to that of low- fl ow priapism, sickle cell disease being the most common one. (81)

There are whatsoever signifi cant diff erences between SRPEs and stuttering priapism: SRPE patients do not suff er from hematological diseases and the duration of the last nocturnal erection in patients with stuttering priapism often persists during three to four hours and ends in ischemic priapism requiring emergent intervention in one third of them.(91) In contrary to stuttering priapism, the duration of SRPEs after awakening is often less than fi fteen minutes and almost all patients with SRPE do not need intervention other than cooling, micturition, walking around or simply ‘thinking the erection away’.

Conclusive remarks

Historically, the term ‘priapism’ originates from the appellation given to the ancient mythological god named Priapus and a qualifying criterion of the disorder is that it persists beyond 4-6 hours. However, erections of shorter duration as well as those lasting up to months encompass the spectrum of its clinical representations. The fi rst recorded evidence of priapism was found in the Egyptian Ebers papyrus originating from 1550 BC. The etiology, diagnosis and treatments have evolved considerably since then. A better understanding of the pathophysiology and the diff erent types of long-lasting erections signifi cantly changed the diagnostic pathway and treatment options. However, the overall results with regard to erectile function in patients after long-lasting ischemic priapism are disappointing to date. With the current availability of diff erent types of antibiotics with think, based on our review, that a re-exploration of old aggressive surgical approaches may be worthwhile, especially in patients who experience priapism for more than 48 hours.

The last fi fty years the increasing use of psychiatric medication, the non-medical use of psychotropic drugs as cocaine as well as the intracavernous injection programs for patients suff ering from ED increased the numbers of patients with priapism. In the eighties of the last century drugs were responsible for at least thirty percent of cases of priapism.(92) Today, this percentage is probably higher. A major problem remains that a lot patient present too late, according to our experience in many cases out of ignorance, shame or sense of guilt. Urologists should explain these latecomers the very high risk of developing ED, despite active intervention or no treatment at all and the documentation of this should be meticulous.

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102

References

Rochat MC. Priapism: a review. Theriogenology 2001;56(5):713-22.

van Driel MF, Hesselink JW. Priapism in the stallion and in man. Tijdschr Diergeneeskd 2003;128(8):255. Okten AI. Mythology and neurosurgery. World Neurosurg 2016;90:315-321.

Mattelaer JJ. The phallus in art & culture. Arnhem: Historical Committee European Association of Urology; 2003.(26-31).

Van Emde BC. Geschiedenis van de seksuele normen. 1985: Dekker & Van de Vegt; 1985.(26-7). McLaren A. Impotence - a cultural histrory. 4th ed. Chicago: The University of Chicago press; 2007. Shokeir AA, Hussein MI. The urology of Pharaonic Egypt. BJU Int 1999;84(7):755-61.

Von Staden HH. Demetrius of Apamea. In: The art of medicine in early Alexandria Cambridge. Cambridge University Press;1989.(506-7).

van Driel MF. Physiology of penile erection-A brief history of the scientific understanding up till the eighties of the 20th Century. Sex Med 2015;3(4):349-57.

Galen. De usus partium corporis humani.1562;15:221-2.

Foucault M. The history of sexuality. (Part 4 The Body - Are they good? Are they bad?). New York: Random house; 1986;3(114-5).

Hashmat AI RJ. Priapism. In: Hashmat AI DS, editor. The penis. Philadelphia and London: Lea & Febiger;1993. (219-43).

Coscione A, Varnavas M, Katmawi-Sabbagh S. Priapism – a historical perspective from antiquity to the more recent past. J Urol 2016;195(4, Supplement):527-8.

Petraens H. De: gonorrhoea, satyriasis et priapisme. Ref Becker, Nosol harmonica dogmat et Hermat, 4 Morparige Cattorum 1616;2:361.

De Héry T. La méthode curatoire de la maladie venerienne vulgairement appelée grosse vérole & de la diversité de ses symptômes. Paris: Saint et Ican;1552:211-2.

Paré A. Les œuvres d’Ambroise Paré en 28 livres. 4th ed. Paris;1585.(15-6).

Morgagni GB. De sedibus, et causis morborum per anatomen indagatis libri quinque. Venice: Remondini; 1761. (177).

Todd NV. Priapism in acute spinal cord injury. Spinal Cord 2011;49(10):1033-5.

Maurel M. Sur un priapisme suivi de retention d’urine. J de Méd Chir Pharm;1772:150- 3.

Hodgson D. Of gods and leeches: treatment of priapism in the nineteenth century. J R Soc Med; 2003;96(11):562-5.

Callaway T. Unusual case of priapism. London Medical Repository 1824:286-7. Tripe JW. Case of continued priapism. 1845(2):8.

Hulke. A case of long-continued priapism after coitus; remarks. Lancet 1888(131):321. Acton W. Functions and disorders of the reproductive organs London: Churchill; 1857: 87.

Bousquet JB. Natuurkundige beschouwing van het huwelijk, in betrekking tot de maatschappelijke zedelijkheid, de bevolking en de gezondheid. ‘s Gravenhage: Fuhri; 1842.

Jadioux J. Hypertrophie ancienne du foie et de la rate: priapisme probablement dû à l’administration des cantharides, et suivi de mort; observation recueille par M. Foureau- Beauregard. Gaz Hôp 1845;7:626. Scholz F. Über Infiltration der Schewellkörper des männlichen Gliedes. Allgem Med Zeitung 1858;8:837-9. Neidhart. Über Priapismus bei Leukaemie. Allgem Med Zeitung 1876;55:681-2.

Salzer F. Ein Fall von langdauerndem Priapismus nebst Bemerkungen über die Beziehungen desselben zu Leukämie. Berlin Klin Woch 1879:152-5.

Neumann I. Priapismus und Cavernitis. Wien: Braumüller 1882.(143-58). Terrier F DC. Du priapisme prolongé. Rev Chir 1907;35(713):740. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31

(18)

103 Dawson JL. Priapism, its causes and treatment, including the history of a remarkable case. Med New Phila 1887:63.

Sothoron. A unique case of priapism. Virg Med 1903;3(7):437-8.

Goebel C. Un cas de priapisme idiopathique. Egypt Med Alexandria 1902;2:200-2.

Birkett J. A case in which persistent priapism was caused by extravasation of blood into the corpora cavernosa of the penis. Lancet 1867;89:207-8.

Haillot MM, Viardin E. Priapisme et grosse rate. Bulletin Med 1904;72:803-4. Mackie J. Notes of a case of persistent priapism. Edinb Med J 1872:418-23.

Velpeau M. Priapisme traité avec succés par le ponction du corps caverneux. Bull Gen Thérapeut 1852;43:324-5.

Kast. Ueber den leukämischen Priapismus. Zeitsch F Klin Med 1895(t.XXVIII):79.

Hinman F. Priapism: report of cases and a clinical study of the literature with reference to its pathogenesis and surgical Treatment. Ann Surg 1914;60(6):689-716.

Hinman F Jr. Priapism; reasons for failure of therapy. J Urol1960;83:420-8.

Draaisma D. Het verdriet van de kosmopoliet. Oratie bij de aanvaarding van het bijzonder hoogleraarschap. Archief en Documentatiecentrum Nederlandse Gedragswetenschappen 2005.

Jovanovic U. Sexuelle Reaktionen und Schläfperiodik bei Menschen. Stuttgart: Enke verlag; 1972:157-93. McKay RW CJ. Priapism; a new method of treatment. J Urol 1927;19:121-9.

Abeshouse BS TL. True priapism; report of 4 cases and review of literature. Urol Cutaneous Rev 1950;54:449-65.

Diggs LW CR. Pathology of sickle cell anemia. South Med J 1934;27:839-45.

Kemper W. Die Functionellen Sexualstörungen. Stuttgart: Georg Thieme Verlag; 1950. Brody HS, Lahr PA, Carrol WA. Primary priapism: a new treatment. J Urol 1957 ;78(2):153- 4.

Grayhack JT, McCullough W, O’Conor VJ Jr, Trippel O. Venous bypass to control priapism. Invest Urol 1964;1:509-13.

Cosgrove MD, LaRocque MA. Shunt surgery for priapism: review of results. Urology 1974;4(1):1-4. Quackels R. Treatment of a case of priapism by cavernospongious anastomosis. Acta Urol Belg 1964;32:5-13. Bolliger G. Priapism. Z Urol 1961;54:551-68.

Sacher EC, Sayegh E, Frensilli F, Crum P, Akers R. Cavernospongiosum shunt in the treatment of priapism. J Urol 1972;108(1):97-100.

Fortuno RF, Carrillo R. Gangrene of the penis following cavernospongiosum shunt in a case of priapism. J Urol 1972;108(5):752-3.

Robbins DM, Crawford ED, Lackner HL. Late development of a urethrocavernous fi stula after cavernospongiosum shunt for priapism. J Urol 1984;132(1):126-7.

Hanafy HM, Saad SM, El-Rifaie M, Al-Ghorab MM. Early arabian medicine: contribution to urology. Urology 1976;8(1):63-7.

Ercole CJ, Pontes JE, Pierce JM,Jr. Changing surgical concepts in the treatment of priapism. J Urol 1981;125(2):210-1.

Nixon RG, O’Connor JL, Milam DF. Effi cacy of shunt surgery for refractory low fl ow priapism: a report on the incidence of failed detumescence and erectile dysfunction. J Urol 2003;170(3):883-6.

Nelson JH,3rd, Winter CC. Priapism: evolution of management in 48 patients in a 22- year series. J Urol 1977;117(4):455-8.

Winter CC. Cure of idiopathic priapism: new procedure for creating fi stula between glans penis and corpora cavernosa. Urology 1976;8(4):389-91.

Ebbehoj J. A new operation for priapism. Scand J Plast Reconstr Surg 1974;8(3):241-2. 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61

(19)

104

Lund K, Ebbehoj J. Results of glando-cavernous anastomosis in 18 cases of priapism. Scand J Plast Reconstr Surg 1980;14(3):269-72.

Brant WO, Garcia MM, Bella AJ, Chi T, Lue TF. T-shaped shunt and intracavernous tunneling for prolonged ischemic priapism. J Urol 2009;181(4):1699-705.

Zacharakis E, Raheem AA, Freeman A, Skolarikos A, Garaffa G, Christopher AN, et al. The efficacy of the T-shunt procedure and intracavernous tunneling (snake maneuver) for refractory ischemic priapism. J Urol 2014;191(1):164-8.

Virag R. Intracavernous injection of papaverine for erectile failure. Lancet 1982;23;2(8304):938.

Brindley GS. Pilot experiments on the actions of drugs injected into the human corpus cavernosum penis. Br J Pharmacol 1986;87(3):495-500.

Goldstein I. The hour lecture that changed sexual medicine--the Giles Brindley injection story. J Sex Med 2012;9(2):337-42.

Glina S, Shindel A, Eardley I, Ghanem H. Cavernosal alpha-blockade: a new technique for investigating and treating erectile impotence by GS Brindley. J Sex Med 2008;5(8):1791- 4.

Brindley GS. New treatment for priapism. Lancet 1984.28;2(8396):220-1.

Friedman DM. A mind of its own - a cultural history of the penis. New York. The Free Press 2001:280. Schlote N, Wefer J, Stief C. Regnier de Graaf: Vascular physiology of erection. In: Schultheiss D, Musitelli S, Stief CG, Jonas U, editor. Classical writings on erectile dysfunction. An annotated collection of original texts from three millennia. Berlin: ABW Wissenschafstverslag; 2005:99-104

Montague DK, Jarow J, Broderick GA, Dmochowski RR, Heaton JP, Lue TF, et al. American Urological Association guideline on the management of priapism. J Urol 2003;170(4 Pt 1):1318-24.

Chauvin E. Some observations on the pathogenesis of priapism. J Urol 1938;46:204.

Golash A, Gray R, Ruttley MS, Jenkins BJ. Traumatic priapism: an unusual cycling injury. Br J Sports Med 2000;34(4):310-1.

De Rose AF, Giglio M, De Caro G, Corbu C, Traverso P, Carmignani G. Arterial priapism and cycling: a new worrisome reality? Urology 2001;58(3):462.

Ul Islam J, Browne R, Thornhill J. Mountain bikers priapism: a rare phenomenon. Ir Med J 2014;107(1):21-2. Hauri D, Spycher M, Bruhlmann W. Erection and priapism: a new physiopathological concept. Urol Int 1983;38(3):138-45.

Kuefer R, Bartsch G,Jr, Herkommer K, Kramer SC, Kleinschmidt K, Volkmer BG. Changing diagnostic and therapeutic concepts in high-flow priapism. Int J Impot Res 2005;17(2):109- 13.

Burt FB, Schirmer HK, Scott WW. A new concept in the management of priapism. J Urol 1960;83:60-1. Wear JB,Jr, Crummy AB, Munson BO. A new approach to the treatment of priapism. J Urol 1977;117(2):252-4. Salonia A, Eardley I, Giuliano F, Hatzichristou D, Moncada I, Vardi Y, et al. European Association of Urology guidelines on priapism. Eur Urol 2014;65(2):480-9.

Saenz de Tejada I, Ware JC, Blanco R, Pittard JT, Nadig PW, Azadzoi KM, et al. Pathophysiology of prolonged penile erection associated with trazodone use. J Urol 1991;145(1):60-4.

Muneer A, Minhas S, Freeman A, Kumar P, Ralph DJ. Investigating the effects of high- dose phenylephrine in the management of prolonged ischaemic priapism. J Sex Med 2008;5(9):2152-9.

Burnett AL, Pierorazio PM. Corporal “snake” maneuver: corporoglanular shunt surgical modification for ischemic priapism. J Sex Med 2009;6(4):1171-6.

Zacharakis E, Raheem AA, Freeman A, Skolarikos A, Garaffa G, Christopher AN, et al. The efficacy of the T-shunt procedure and intracavernous tunneling (snake maneuver) for refractory ischemic priapism. J Urol 2014;191(1):164-8.

Garaffa G, Ralph DJ. Penile prosthesis implantation in acute and chronic priapism. Sex Med Rev 2013;1(2):76-82. 62 63 64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 79 80 81 82 83 84 85 86

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105 Ralph DJ, Garaff a G, Muneer A, Freeman A, Rees R, Christopher AN, et al. The immediate insertion of a penile prosthesis for acute ischaemic priapism. Eur Urol 2009;56(6):1033-8.

Ralph DJ, Borley NC, Allen C, Kirkham A, Freeman A, Minhas S, et al. The use of high- resolution magnetic resonance imaging in the management of patients presenting with priapism. BJU Int 2010;106(11):1714-8. Vreugdenhil S, Weidenaar AC, de Jong IJ, van Driel MF. Sleep-related painful erections- a case series of 24 patients regarding diagnostics and treatment options. Sex Med 2017 21.;5(4):e237-43

Emond AM, Holman R, Hayes RJ, Serjeant GR. Priapism and impotence in homozygous sickle cell disease. Arch Intern Med 1980;140(11):1434-7.

Hoeh MP, Levine LA. Management of recurrent ischemic priapism 2014: a complex condition with devastating consequences. Sex Med Rev 2015(3):24-35.

Banos JE, Bosch F, Farre M. Drug-induced priapism. Its aetiology, incidence and treatment. Med Toxicol Adverse Drug Exp 1989;4(1):46-58.

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