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 Table of Contents  
ORIGINAL ARTICLE
Year : 2016  |  Volume : 6  |  Issue : 1  |  Page : 12-16

Surgical treatment of priapism in sickle cell patients: A noble therapy in preserving erection


Department of Surgery, Division of Urology, Ahmadu Bello University, Teaching Hospital, Zaria, Nigeria

Date of Web Publication28-Jul-2016

Correspondence Address:
Dr. Ahmad Bello
Department of Surgery, Ahmadu Bello University, Teaching Hospital, Zaria
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-9596.187200

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  Abstract 

Background: Priapism is a pathological condition of erectile tissue tumescence characterized by prolonged erection without any sexual stimulation and excitement. A classification commonly used to differentiate clinical presentation of priapism divides priapism into ischemic (low-flow) and nonischemic (high-flow) priapism. They have different pathological consequences and also differ in the overall management. The present study reviewed the modes of presentation and management and results of treatment of priapism in sickle cell patients.
Patients and Methods: All consecutive sickle cell patients with priapism who were managed by surgical intervention at our unit between January 2000 and December 2011 were included in this study. The demographic characteristics, predisposing factors, and mode of treatment were reviewed. The outcomes of the treatment were analyzed using statistical software Statistical Package for the Social Sciences (SPSS) version 16.
Results: Thirty-three patients with priapism due sickle cell disease were seen over 12 years; two patients were lost to follow-up and were excluded. Their age range was 2-35 years with a mean of 22.5 years. The patients who had an El-Ghorab cavernospongiosus shunt (ECS) had better results than those who had a conventional perineal cavernospongiosus shunt (PCS). Eleven patients with recurrent previous episodes of priapism had reduced or absent erection. Erection was better in patients with shorter duration of priapism and not more than one previous episode. The mean duration of symptoms from the time of presentation to treatment was 3 days. Of the twenty-one patients who presented in ≤2 days, 18 had normal erections and 3 had reduced erections at 12 weeks. Of the 12 patients who presented in ≥3 days, 5 had normal erections, 2 had reduced erections, and 5 had no erection. Ten patients had treatment with sildenafil during the follow-up period with some improvement. One patient who had PCS developed perineal urethrocutaneous fistula.
Conclusion: Early surgical intervention is recommended in patients with ischemic priapism; regardless of the delay in presentation, and immediate unilateral distal venous shunt should be considered the best way to preserve erection. More prospective randomized studies are required to confirm these assertions.

Keywords: Cavernospongiosus shunt, priapism, sickle cell disease, urethrocutaneous fistula


How to cite this article:
Bello A, Maitama HY, Ahmed M, Sudi A. Surgical treatment of priapism in sickle cell patients: A noble therapy in preserving erection. Arch Int Surg 2016;6:12-6

How to cite this URL:
Bello A, Maitama HY, Ahmed M, Sudi A. Surgical treatment of priapism in sickle cell patients: A noble therapy in preserving erection. Arch Int Surg [serial online] 2016 [cited 2024 Mar 28];6:12-6. Available from: https://www.archintsurg.org/text.asp?2016/6/1/12/187200


  Introduction Top


Priapism is a pathological condition of erectile tissue tumescence characterized by prolonged erection without any sexual stimulation and excitement.[1],[2] The penis is recognized as being the affected body organ, although priapism of the clitoris has also been reported in the medical literature.[3] A time interval of 4 h has frequently been cited as a qualifying criterion since, on a practical basis, pathological consequences are associated with priapism extending beyond this time limit;[4] however, presentation of shorter durations, and conversely those lasting days or months that may not overtly result in erectile tissue damage, are identifiable as representing priapism. A classification commonly used to differentiate clinical presentation of priapism divides priapism into ischemic (low-flow) and nonischemic (high-flow) priapism. They have different pathological consequences.[5] The division also has practical consequences with respect to the overall clinical management. In 1914, Hinman [6] first recognized that some recurrent forms of priapism are suffered for limited durations compared with isolated episodes. He called those recurrent forms acute transitory attacks. The more familiar terminology called stuttering priapism was devised by Emond [7] in reference to the frequent recurrent episodes of priapism observed in patients with sickle cell disease. Such priapism, which progresses in severity, may be considered to represent a subtype of ischemic priapism.[2]

In most patients, priapism is idiopathic, although it is a well-recognized complication of sickle cell anemia, and intracavernosal drug therapy for erectile dysfunction (ED). In Nigeria, sickle cell anemia is complicated by priapism in 2-5% of cases.[5] Various treatments have been described, ranging from corporal aspiration, administration phenylephrine, and shunt, with varying results. Surgical management of ischemic priapism is indicated after repeated penile aspirations and injections of sympathomimetics have failed or if such an attempt has resulted in a significant cardiovascular side effect. At present, there is a paucity of data regarding the timing of surgical intervention following initiation of medical treatment, although the 2004 International Consultation on Sexual Medicine in Paris recommended corporal aspiration and α-adrenergic agonists for at least 1 h before the consideration of surgery.[8] Failure of treatment leads to corporeal fibrosis and ED. Because of ignorance and poverty, the patients with priapism generally present late to the urologist. Other factors responsible for ED include failure to maintain complete detumescence after management and marked penile fibrosis.[9] This present study reviewed the modes of presentation and management, and results of treatment of priapism in sickle cell patients.


  Patients and Methods Top


All consecutive sickle cell patients with priapism who were managed by surgical intervention in our unit between January 2000 and December 2011 were included in this study. All patients with priapism due to other conditions or those who were managed nonoperatively were excluded from the study. At presentation, the following were determined: The age of the patient, time of onset of the priapism, probable predisposing factors, treatment received so far, previous episodes of priapism, reasons for late presentation, and history of any medical or psychiatric illness and any drugs taken. After a full clinical examination, urine was cultured and blood taken for a full count and genotype. The patients were adequately resuscitated with intravenous fluids, broad-spectrum antibiotic, and analgesics and were prepared for the surgery. All patients underwent immediate surgery in the form of an El-Ghorab cavernospongiosus shunt (ECS) or a conventional perineal cavernospongiosus shunt (PCS). The patients underwent PCS if there were blisters on the glans penis otherwise an ECS. All patients had an indwelling urethral catheter after the procedure. In the ECS, a 2.5-cm transverse incision was made on the dorsum of the glans penis <1 cm from the corona. The tissues of the corpus spongiosum were dissected sharply until the distal end of the corpora cavernosa were identified. Circular scalpel incisions were made in the tunica albuginea of both or one of the cavernosa, removing <5 mm of tissue, thus gaining access to the corpora cavernosa. Gentle pressure was applied to the penis to extrude the dark viscid blood, and the corpora irrigated with normal saline until bright blood was seen to exit from the corporotomy. The incision in the glans was closed by a running suture of 4/0 polyglactin. In the PCS, a midline incision was made in the anterior perineum and the bulbocavernosus muscle was split in the midline. The corpora cavernosa and spongiosum were dissected free for 3 cm and a circular fenestration of <5 mm in diameter was made on the medial wall of the corpora cavernosa adjacent to the corpora spongiosum. Gentle pressure on the penis evacuated the blood through the fenestration. The corpora cavernosa were irrigated with normal saline until bright blood was seen to exit. Circular fenestrations of similar size to those in the corpora cavernosa were made on the lateral walls of the corpora spongiosum adjacent to those made on the medial side of the corpora cavernosa, and a 5/0 continuous polypropylene suture was used to effect cavernospongiosus anastomosis on each side. The wound was drained, the bulbocavernosal muscle and colles fascia approximated with interrupted 3/0 polyglactin and the skin sutured with interrupted 3/0 polypropylene. After the surgery, the patients were advised to squeeze the penis intermittently. The wound drain and catheter were removed after 24 h. After the discharge from the hospital, the patients were reviewed at 2 weeks, 6 weeks, and 12 weeks; they were questioned about their erectile ability and orgasm, and the penis palpated for fibrosis. A normal erection and orgasm were recorded if it was the same as the pre-morbid state. A reduced erection was recorded if the patient was still able to have penetrative sexual intercourse. No erection occurred if the patient is unable to have penetrative sexual intercourse or no nocturnal tumescence 6 weeks after the surgery.


  Results Top


Thirty-three patients with priapism due to sickle cell disease were seen during a study period of 12 years; two patients absconded after the surgery and were lost to the follow-up, and were excluded from the study. Their age range is 2-35 years with a mean age of 22.5 years. At the time of presentation, three patients had an infected, blistered penis, out of whom two absconded before the surgery. The duration of priapism before presentation is shown in [Figure 1]. The commonest reason for delay was conservative treatment by physicians at private hospitals (11 patients). Conservative treatment included hydration, analgesics, ice packs, and aspiration of the corpora. Five patients received traditional medical treatment; only when there was no improvement did they report to hospital. Twelve patients thought that the erection was normal and it would subside with time, and usually had previous episodes that had subsided spontaneously. All patients had sickle cell disease and 11 patients had previous episodes of priapism that were managed conservatively. Another predisposing factor was identified in five men that is the use of a local aphrodisiac. The relationship between the etiology and the outcome of the surgery is shown in [Table 1].
Figure 1: Bar chart showing the duration of priapism at presentation and erection status 12 week after shunt procedure

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Table 1: The relationship between etiology and outcome of the surgery, and the results at 12 weeks after the surgery

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Thirty patients had undergone ECS and one had PCS. Detumescence was obtained in all patients at surgery and the penis was maintained in completely flaccid state in 25 cases. In 11 patients, the penis remained semiturgid for some days before gradually resolving. [Table 1] also shows the results of surgery after the final assessment at 12 weeks. The patients who had an ECS had better results than the patient who had a PCS. Eleven patients with recurrent previous episodes of priapism had reduced or absent erection. Erection was better in the patient with shorter duration of priapism and not more than one previous episode. The mean duration of symptoms from presentation to treatment in these patients was 3 days. Of the 21 patients who presented in ≤2 days, 18 had normal erections and 3 had reduced erections at 12 weeks. Of the 12 patients who presented after 3 days, 5 had normal erections, 2 had reduced erections, and the remaining 5 had no erection. Ten patients received treatment with sildenafil during the follow-up period with some improvement. The one patient who had PCS developed perineal urethrocutaneous fistula. One patient had an intact prepuce and had ECS and circumcision.


  Discussion Top


During the last 25 years, the treatment of priapism has changed from a conservative approach to an active surgical procedure with the creation of venous shunts. The results of the more active treatment have been promising in our experience in this study and other reports.[2],[10],[11] In this study, of the 21 patients who presented within 2 days of the onset and had immediate shunt, 85% had their erection preserved. Seventy four percent of the 18 patients that had unilateral distal shunt (ECS), had normal erection. The following two factors seem to be especially important for a good result: Minimum delay from the onset of priapism until operation and quick detumescence following the operative procedure. Also the unilateral shunt may be a contributing factor because of the possibility of obliteration of the fenestration. Since priapism can recur after successful treatment, we recommend unilateral shunting, which makes it possible to use the same technique on the opposite side if necessary. In our study, one patient had recurrence and had repeat ECS. There are, however, other surgeons that advocate bilateral procedure.[11],[12],[13] Erection ability often returns some weeks after a successful operation.

Fibrosis of the corpora cavernosa was seen in patients whose treatment was delayed. This change can be clinically evaluated by palpation of a tough induration of the corpora cavernosa. These patients had poor erectile function after surgery and only 41% had their erection preserved at the time of final assessment in this study. Among the 31 patients, 5 use traditional aphrodisiacs treatment that is an additional risk factor as reported by others.[5],[10] This may also be another reason for the delayed presentation as they feel shy to report the incident to family members or health-care personnel. All our patients had sickle cell disease as the cause of priapism. Priapism due to other causes were excluded from the study. Other causes of hematological disorders and trauma were reported in the literature.[14],[15],[16]

Our results following shunt procedures have been satisfactory, except for the patients with delayed presentation, the patients with penile fibrosis, and the patient who had perineal shunt and developed urethrocutaneous fistula. All other methods for venous shunting seem suitable, but some of them carry certain risks. A fistula into the urethra has been described after a cavernospongiosus shunt,[17] this was also seen in one of our patients. The cavernoglandular shunt sometimes leaves a permanent venous opening between corpora cavernosa and glans, leading to reduced or absent erection; this is commonly seen in patients with the bilateral shunt. Operative closure of the shunt may be required.[18] In this study, five patients with delayed presentation more than 6 days, two had normal erection. Similar result was obtained by Aghaji (2000)[5] in which 14 (40%) patients who had immediate distal shunt had normal erection and 11 (31.4%) patients had reduced erection despite delayed presentation of 6-10 days. Forsberg et al. (1981) show that 5 out of 6 patients treated with surgery and 3 out of 9 patients treated conservatively have their erection preserved. Priapism is a condition that demands surgical treatment in the form of an urgent shunt operation.[19] Although the erection may be satisfactory, these patients may still have fertility issues as a result of the abnormalities in their seminal parameters.[20] We recommend early shunt in all sickle cell patients irrespective of the time of onset of the priapism once other conservative measures failed to result in complete detumescence.


  Conclusion Top


Thus we concluded that, regardless of the delay in presentation immediate distal venous shunt should be considered as may be the best way to preserve erection. More prospective randomized studies are required to confirm these assertions. All patients should be adequately resuscitated in order to avert other complications associated with sickle cell disease. Unilateral distal shunt gives satisfactory detumescence and good erectile outcome postoperatively.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Falandry L, Berlizot P, Fournier R, Mechali P, Thuret R, Palascak R, et al. Surgical treatment of priapism: Experience of 56 cases in an African setting. Med Trop (Mars) 2000;60:70-4.  Back to cited text no. 1
    
2.
Adetayo FO. Outcome of management of acute prolonged priapism in patients with homozygous sickle cell disease. West Afr J Med 2009;28:234-9.  Back to cited text no. 2
    
3.
Monllor J, Taño F, Arteaga PR, Galbis F. Priapism of the clitoris. Eur Urol 1996;30:521-2.  Back to cited text no. 3
    
4.
Adeyoju AB, Olujohungbe AB, Morris J, Yardumian A, Bareford D, Akenova A, et al. Priapism in sickle-cell disease; incidence, risk factors and complications-an international multicentre study. BJU Int 2002;90:898-902.  Back to cited text no. 4
    
5.
Aghaji AE. Priapism in adult Nigerians. BJU Int 2000;85:493-5.  Back to cited text no. 5
    
6.
Hinman F Jr. Priapism: Reasons for failure and therapy. J Urol 1960;83:420-8.  Back to cited text no. 6
    
7.
Emond AM, Holman R, Hyes RJ, Serjeant GR. Priapism and impotence in homologous sickle cell disease. Arch Intern Med 1980:140;1434.  Back to cited text no. 7
    
8.
Bennett N, Mulhall J. Sickle cell disease status and outcomes of African-American men presenting with priapism. J Sex Med 2008;5:1244-50.  Back to cited text no. 8
    
9.
El-Bahnasawy MS, Dawood A, Farouk A. Low-flow priapism: Risk factors for erectile dysfunction. BJU Int 2002;89:285-90.   Back to cited text no. 9
    
10.
Klein LA, Hall RI, Smith RB. Surgical treatment of priapism: With a note on heparin-induced priapism. J Urol 1972;108:104-6.  Back to cited text no. 10
    
11.
Nair VR. Venous shunt in priapism: A report of 4 cases. J R Coll Surg Edinb 1976;21:366-7.  Back to cited text no. 11
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12.
Medeiros A deS, de Carvalho RM. Bilateral caverno-saphenous shunt for priapism: 4 case report. Br J Urol 1973;45:545-7.  Back to cited text no. 12
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13.
Kihl B, Bratt CG, Knutsson U, Seeman T. Sapheno-Cavernos Shunt vid idiopatisk priapism. Acta Societae Medicorum Suecanae 1978;87:607.  Back to cited text no. 13
    
14.
Rosenberg RF. Priapism and sickle-cell disease. Radiology 1971;98:135-6.  Back to cited text no. 14
[PUBMED]    
15.
Goulding FY. Priapism caused by glucose phosphate isomerase deficiency. J Urol 1976;116:819-20.  Back to cited text no. 15
    
16.
Oppenheimer R. Priapism in an 8-year-old boy treated by spongiocavernosum shunt. J Urol 1976:116:818.  Back to cited text no. 16
    
17.
Sacher EC, Sayegh E, Frensilli F, Crum P, Akers R. Cavernospongiosum shunt in the treatment of priapism. J Urol 1972;108:97-100.  Back to cited text no. 17
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18.
Ebbehoj J. A new operation for priapism. Scand J Plast Reconstr Surg 1975;8:241-2.  Back to cited text no. 18
    
19.
Forsberg L, Mattiasson A, Olsson AM. Priapism-conservative treatment versus surgical procedures. Br J Urol 1981;53:374-7.  Back to cited text no. 19
[PUBMED]    
20.
Osegbe DN, Akinyanju O, Amaku EO. Fertility in males with sickle cell disease. Lancet 1981;2:275-6.  Back to cited text no. 20
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