1.       Introduction

Priapism is a urologic emergency and is defined as a prolonged and persistent penile erection that is unrelated to sexual interest or stimulation lasting more than 4 hours [1-3]. Three types have been described ischemic (low flow), non-ischemic (high flow) and stuttering (recurrent) [1,3,4]. It was first described by Tripe in 1845 [3,5]. Its association with SCA was documented by Diggs and Ching in 1934 [5]. In the general population, an incidence rate of up to 1.5/100,000 person-years has been reported [2,4,6]. Ischemic priapism accounts for more than ninety-five percent of all cases of priapism [3-6], with an incidence of 5.34/100,000 men’s per year [1].

Priapism in SCA is of ischemic low flow sub type with an incidence of 3.6% in those younger than 18 years old, 42% in those 18 years and above [2,4], with a prevalence rate of up to 35 to 40 percent [5,6]. It is associated with time dependent devastating complications such as erectile tissue necrosis and fibrosis. Late presentation is the norm in developing countries like ours due to low public awareness and visits to poor health facilities before getting to the experts. In these situations, invasive procedures are adopted. We report the use of Al-Ghorab shunting plus Burnett shunting in prolonged IP to achieve complete detumescence.

2.       Case

A 27-year-old male presented to the accident and emergency with a 9 days’ history of painful, unprovoked penile erection. There was no sexual arousal, no use of aphrodisiacs and recreational drugs. He had a similar episode 2 year back that resolved spontaneously after 6 hours of onset. He did not know his genotype. He had visited a clinic where aspiration with a needle and irrigation with normal saline of the CC were done without achieving detumescence.

He was pale, pyrexic, mildly icteric and in distress. He had a woody hard tender and erect penile shaft pointing cephalad (Figure 1), with Erection Hardness Score (EHS) of 4.

The glans and corpus spongiosus were flaccid. The CC showed maximal tumescence.

The results of investigations are as shown (Table 1).

Patient was adequately hydrated with intravenous fluids and anemia corrected with blood transfusions. Parentral analgesia was given. Intravenous levofloxacin, 500mg daily was given for 5 days followed by oral levofloxacin for another 7 days. The patient was aseptically catheterized to monitor urinary output.

Under general anesthesia, with tourniquet temporally put at the base of the penis to minimize bleeding, Al-Ghorab shunting followed by Burnett shunting were carried out as follows. A 2cm transverse incision was made on the glans, 1cm distal the coronal sulcus. The cavernous tissue of the glans was retracted. We excised circular cone segment of tunica albuginea tissue, 5mm in diameter at the tip of each CC thus establishing a shunt between the CC blood flow and that of the glans. An immediate upward slash of dark deoxygenated blood was observed indicating very high pressure within the cavernosal chambers. Retrogradely, we passed 7/8 Hegars dilator into each CC distal opening dilating both and removing clogged blood to ensure adequate drainage. Manual compression of the penis from proximal to distal was done until bright red blood was seen. Complete detumescence was achieved on the table (Figure 2). The incision on the glans was closed with running vicryl 3/0 suture (Figure 3).

The patient was discharged after 5 days and at 2 weeks post shunt, the penis was still flaccid (Figure 4). He however developed Erectile Dysfunction (ED) 14 days after the shunt. There has been no recurrent IP till date.

3.       Discussion

There was a buildup of deoxygenated blood within the CC in our patient as evidenced by PH of 7.18, PCO₂ of 66, pO₂ of 25 mmHg respectively from analysis of CC blood aspirate (Table 1). This buildup of deoxygenated blood was consequent upon persistent relaxation of the CC smooth muscles compressing the subtunical veins with prevention of outflow of deoxygenated blood via the sinusoids. Thus, there was increase in intracorporal pressure above the mean arterial compressing the cavernosa arteries, preventing arterial inflow [4]. Consequently, there was blood stagnation within the two CC leading to CC rigidity and pain as in the index patient.

This represents a compartment-like syndrome with high pressure within the cavernosa spaces. The index patient was in this state for nine days explaining upward splash of deoxygenated blood during the shunt procedure. Furthermore, there are hypoxic, hyperbaric and acidotic reactions within the cavernosa spaces leading to various histological changes manifesting beyond 24 hours as erectile tissue necrosis and fibrosis with resultant Erectile Dysfunction (ED) which may be as high as 90% [2,3,5-8].

The index patient presented late which is the norm in developing countries like ours. This is due to low awareness and existing poor health facilities. He visited three clinics before getting a urology consult in our centre. In this state, we had to use Al-Ghorab shunting followed by Burnett shunting to remove clogs of deoxygenated blood thereby maintaining adequate corporal blood flow making it possible to achieve 100% detumescence on the table.

 It is not surprising that the patient developed ED post shunt due to at the molecular level, there are decreases in phosphodiesterase type 5 and RhoA/Rho kinase, increased levels of adenosine and the presence of acidocis, glucopenia and hypoxia all contributing to cavernosal smooth muscle dysfunction with resultant irreversible devastating changes within the corporal tissues [8,9].

4.       Conclusion

We have reported on a successful management of ischemic priapism in a SCA patient presenting 9 days after onset, achieving full detumescence after Al-Ghorab and Burnett shunting procedures. Due to late presentation leading to irreversible hypoxic and acidotic changes within the CC tissues, he developed ED 2 weeks post shunt. However, there has been no recurrence of priapism till date. Finally, energetic approach should be adopted in developing countries to increase awareness of this condition in the society. This will go a long way in decreasing to the barest minimum the irreversible damage occurring within the corporal tissues thus preserving erection after achieving detumescence.

Figure 1: Priapism showing cephalad pointing penis.

Figure 2: Detumescence after Al-Ghorab plus Burnett shunting showing bright red blood at the right incision.

Figure 3: Running vicryl suture closing on the glans.

Figure 4: Penis two weeks after Shunt.

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