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Priapism in Children

Ali Ghribi, Manef Gasmi, Mourad Hamzaoui

Department of Pediatric Surgery, Children’s Hospital, Tunis El Manar University, Tunis, Tunisia

 

Correspondence:

Ali Ghribi

Department of Pediatric Surgery, Children’s Hospital, Tunis El Manar University

Place Bab Saadoun 1007 Tunis, Tunisia

Tel: 002167156810 Fax : 0021671562810

Email : This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Introduction: Priapism is persistent penile erection that is unrelated to sexual stimulation. Low-flow type is a urological emergency. The aim of this study is to illustrate our personal experience concerning the diagnostic and therapeutic management of priapism in children.

Patients and methods: A retrospective study was conducted involving 6 patients with priapism managed at our department, over a 20-year period, going from 1992 through 2012. Among them, 5 were because of sickle cell disease and 1 was of idiopathic origin in newborn.

Results: The study involved a 14-days-old neonate and 5 children whose ages ranged from 9 to 13 years. Mean duration of symptoms was 19.6 hours. The priapism was associated with confirmed sickle cell disease (SCD) and with newly diagnosed SCD in two others. It was of idiopathic origin in the newborn. The neonate was kept under observation and priapism disappeared after 18 hours without recurrence. In the older children, initial management was similar to that of other sickle cell crises (hyperhydration, oxygen, and analgesia). Because of the long duration of the priapism, emergency surgery was indicated under general anaesthesia. Successful detumescence was achieved after corporal aspiration in 4 patients. Caverno-glandular shunting, indicated after failure of corporal aspiration in one case. After surgery, spontaneous transient erection was self-reported by 4 patients without recurrence of priapism. One child presented after 6 months recurrence treated conservatively.

Conclusion: The first step of management of priapism is to distinguish ischemic from nonischemic type. Sickle cell disease is the commonest cause of ischemic priapism. Corporeal aspiration and modified Winter’s shunt were successful alternatives for patients with low-flow priapism. Management of idiopathic priapism in the newborn by observation alone seems to be safe. A long-term follow-up is necessary to assess erectile function.

Keywords: priapism, sickle cell disease, children, corporal aspiration

 

Introduction

Priapism is persistent penile erection for longer than 4 hours that is unaccompanied by sexual excitation. Low-flow or ischemic priapism is a urological emergency. Unlike high-flow priapism, it is excruciatingly painful and carries the risk of permanent impotence if treatment is delayed. A vast majority of the cases of priapism in children are due to sickle-cell disease. The aim of this study is to report our experience of management of six children with priapism over the past 20 years.

Patients and methods

A retrospective study was conducted involving 5 children and a neonate with priapism managed at the department of pediatric surgery of Tunis Children’s Hospital, over a 20-year period, going from 1992 through 2012. The priapism was associated with confirmed sickle cell disease (SCD)and with newly diagnosed SCD in two others. It was of idiopathic origin in the newborn. A workup for a secondary cause of priapism other than SCD was done including complete blood count, color Doppler ultrasonography (CDU) of the perineum and penis, and hemoglobin electrophoresis. Low-flow type priapism is characterized by painful rigid erection, a history of hemoglobinopathies or the presence of low-flow pattern in penile Doppler. Nonsurgical management was advocated only for the newborn with prolonged penile erection. The other patients underwent corporal aspiration (5 cases) or percutaneous caverno glanular shunt, Winter's shunt (1 case).

For corporal aspiration, a needle was inserted laterally at the 3 or 9 o’clock positions to avoid damaging the corpus spongiosum, the urethra and the dorsal neurovascular bundles. For the caverno glanular shunt, a needle was thrust into the corpora cavernosum through the glans. Multiple punctures are made in both corporal bodies, lateral to the urethra. After removing the needle, the puncture wound in the glans was closed with a single 6-0-catgut stitch. No patient received oral or intracorporeal alphasympathomimetics.

Results

The study involved 14-day-old neonate and 5 children whose ages ranged from 9 to 13 years (mean, 11.2 years). All of them presented with painful, rigid erection of penis that is characteristic of low-flow priapism. There were no signs of cyanosis in all cases. Mean duration of symptoms was 19.6 hours (range, 12-36 hours). Two children had been followed up, since early childhood, for homozygous sickle cell disease with chronic transfusion therapy programs and one for sickle β+ thalassemia with an enlarged spleen that required splenectomy for hypersplenism 2 years before. The 2 others hadn’t a history of SCD but in one case we found a history of unexplored anemia and jaundice. The other child reported that he had been the victim of a perineal trauma 7 days before the priapism occurred. CDU of the perineum and penis didn’t reveal anyarterio cavernous fistula. Homozygous SCD was confirmed by hemoglobin electrophoresis in these last two children. The newborn had no history of birth canal trauma or blood transfusion. Complete blood count didn’t show polycythemia (the hematocrit was at 39%) and penile CDU findings were normal. So, the patient was kept under observation and priapism disappeared after 18 hours without recurrence. After a follow-up period of 9 months, the penis was normal, and the mother reported periods with normal penile erection. In the older children, initial management was similar to that of other sickle cell crises (hyperhydration, oxygen, and analgesia). Because of the long duration of the priapism, emergency surgery was indicated under general anaesthesia. Successful detumescence was achieved after corporal aspiration in 4 patients (Fig. 1 and 2).

Figure1. Corporal aspiration. Two needles were inserted laterally at the 3 and 9 o’clock positions.

11.2 4 1

 

Figure 2. Complete detumescence was achieved after corporal aspiration.

11.2 4 2

Needle aspiration had to be repeated twice (in 2 cases) because of immediate recurrences. Caverno glandular shunting, indicated after failure of corporal aspiration in one case, produced improvement in cavernosal rigidity. Blood loss ranged from 100 to 150 ml and no patient required blood transfusion. One case of hematoma that developed in the subcutaneous tissue at the puncture site was controlled with direct pressure. After surgery, spontaneoustransient erection was self-reported by 4 patients without recurrence of priapism. One child presented after 6 months recurrence treated conservatively.

Discussion

There are two main kinds of priapism: low-flow ischaemic or veno-occlusive priapism, and highflow or arterial priapism that usually results from penile or perineal trauma injuring the wall of the cavernosal arteries and creating an arteriocavernous fistula [1-3]. The majority of cases of low-flow priapism are associated with SCD. Other predisposing factors including leukemia, lymphoma, Fabry’s disease, medication, and idiopathic causes have been reported. Stuttering priapism, describes recurrent priapism that usually due to SCD, lastes anywhere from minutes to just a few hours and typically resolves spontaneously. Neonatal priapism is also described, 18 cases have been reported [4]. It usually occurs in the first few days of life and persists for 2 to 12 days (average 5 days). Neonatal priapism is mostly idiopathic, like in our study. Identifiable etiologies are polycythemia, blood transfusions, congenital syphilis, cranial birth trauma, respiratory distress syndrome and umbilical artery catheterization.

There are no generalizable data on the prevalence of priapism in all children, which is considered rare. In children with sickle cell anemia, the incidence is reported to range from 6–27% and is higher in patients with HbSS [5]. Initial management aims to assess the type of priapism and to achieve detumescence. Low-flow priapism is typically painful with marked rigidity of the corpora cavernosa with a flaccid glans. Masturbation and nocturnal erections are common precipitators. However, high-flow priapism is characterized by painless, persistent semi-rigid erection. Perineal compression with the thumb resulting in immediate detumescence with relapse after withdrawal of the thumb is known as Piesis clinical sign and indicates high-flow priapism [1,2,6]. If there is difficulty in differentiating low-vs-high flow priapism, imaging studies or blood gas measurement from a cavernosal sample may help.

In ischaemic priapism CDU demonstrates a high resistance and low-flow arterial waveform. It detects nearly 100% of arteriosinusoidal fistulae in case of high-flow priapism. In blood gas analysis, values similar to arterial blood are indicative of high-flow priapism, whereas samples more consistent with venous blood sampling suggest the low-flow etiology [3]. Acute treatment for ischemic priapism should be instituted within hours given the increasing likelihood of cavernosal fibrosis and permanent erectile dysfunction (ED). Management involves corporeal aspiration, and phenylephrine injection to induce smooth muscle contraction and detumescence [3]. The corpora should be aspirated until bright red blood is seen (not exceeding 10% of the circulating blood volume). Simple intracorporeal aspiration will accomplish permanent detumescence in approximately half of patients. It was successful, in our study, in 80%. If conservative measures fail, penile shunt surgery should be performed.

Distal shunts, between corpora cavernosa and glans, are preferred over proximal ones (cavernospongiosal or cavernovenous). Distal shunt may be done either by open surgery (Al-Ghorab shunt) or by percutaneous techniques (Winter’s shunt).Several modifications of Winter's shunt have been introduced to get better results. Raveenthiran reported a modified technique using multiple punctures by a large bore needle in the tip of corpora cavernosa through the glans. The needle tracks functioned as temporary cavernoglandular fistula. This technique was almost always successful, like in our case report, and erectile capacity was retained in a high proportion of children (>71%)[7]. SCD patients may also benefit from hydration, blood transfusion,exchange transfusion, or hyperbaric oxygen. Non-operative management may be successful provided patients present early. Children with sickle cell anemia are at a higher risk of developing ED [7]. The lifetime probability of ED secondary to priapism is over 30%.

Expectant management in high-flow priapism is acceptable and safe. Because venous outflow is not compromised there is no damage to the corporal bodies. Up to 62% of non-ischemic priapism cases have spontaneous resolution [8]. For patients who request treatment or do not improve on observation, there are a few effective non-invasive options, like alpha adrenergic agonist injections, compression methods of the penile artery or compression of the fistula with a duplex ultrasound. Percutaneous transcatheter embolization is the most effective treatment.

Surgical options, involving resection of the fistula and ligation of the internal pudendal artery or cavernous artery are reserved for failed embolization attempts. The incidence of erectile dysfunction after embolization was about 25% [8]. Neonatal priapism may be treated less urgently. Initial evaluation of prolonged erections in newborns should include clinical examination and/or ultrasonography, full blood count, and C-reactive protein. The majorities of reported cases were idiopathic; they were managed conservatively with spontaneous resolution within a period of approximately 5 days. Phlebotomy and exchange transfusion for polycystic newborns were successful options. Priapism persisting for more than 5 days may be treated by ketamine hydrochloride anaesthesia or corporal aspiration. Long-term undesirable consequences, such as erectile dysfunction, have not been reported in the newborn group [9,10,11].

Conclusion

Priapism is a medical emergency commonly associated with significant long-term morbidity. The first step of management is to distinguish ischemic from nonischemic using findings from careful history taking, physical and finding from cavernosal blood gas determination and CDU examination.Sickle cell disease is the commonest cause of ischemic priapism in children and should be usually sought. There are many therapeutic modalities to achieve detumescence, depending on the etiology. Corporeal aspiration and modified Winter’s shunt were successful alternatives for patients with low-flow priapism who did not respond to conservative treatment. Management of priapism in the newborn by observational one seems to be safe. A long-term follow-up is necessary to assess erectile function.

 

 

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