http://informahealthcare.com/jmf ISSN: 1476-7058 (print), 1476-4954 (electronic) J Matern Fetal Neonatal Med, Early Online: 1–3 ! 2015 Informa UK Ltd. DOI: 10.3109/14767058.2014.1003538

SHORT REPORT

A rare entity: idiopathic priapism in a newborn and review of the literature Ali Erdal Karakaya1, Esad Koklu2, and S¸ enol Ozturk1 J Matern Fetal Neonatal Med Downloaded from informahealthcare.com by Nyu Medical Center on 05/07/15 For personal use only.

1

Department of Pediatric Surgery, Medical Faculty, Kahramanmaras Sutcuimam University, Kahramanmaras, Turkey and 2Division of Neonatology, Megapark Hospital, Kahramanmara¸s, Turkey Abstract

Keywords

Priapism is a pathological condition of a penile erection that persists beyond or is unrelated to sexual stimulation. Priapism is an important medical condition, which requires evaluation and may require emergency management. This condition occurs very infrequently in paediatrics outside of the sickle-cell population and is exceedingly rare in newborns. The evaluation and management of neonatal priapism can be challenging for paediatricians, neonatologists and paediatric urologists alike given the lack of experience with this condition, its poorly understood pathophysiology and the absence of well-established guidelines. We present a case of idiopathic neonatal priapism because of its rarity and review of the literature.

Idiopathic, newborn, priapism History Received 18 February 2014 Revised 5 December 2014 Accepted 29 December 2014 Published online 23 January 2015

Introduction

Case report

Neonatal priapism is a rare phenomenon, with only 17 cases reported in the literature since 1876 [1–3]. Priapism is a pathological condition of a penile erection that persists beyond or is unrelated to sexual stimulation. Priapism is an important medical condition, which requires evaluation and may require emergency management [1–3]. Priapism is broadly classified into two types: (a) ischaemic priapism (veno-occlusive) (low-flow), (b) non-ischaemic priapism (arterial) (high-flow) [1–3]. Ischaemic priapism beyond 4 h is a compartment syndrome requiring emergent medical intervention. (b) Non-ischaemic (arterial) (high-flow) priapism is a less common form of priapism caused by unregulated cavernous inflow. The erection is usually painless and not fully rigid. Preservation of normal erectile function is a major goal in the management of priapism [1–3]. This condition occurs very infrequently in paediatrics outside of the sickle-cell population [2] and is exceedingly rare in newborns. The evaluation and management of neonatal priapism can be challenging for paediatricians, neonatologists and paediatric urologists alike given the lack of experience with this condition, its poorly understood pathophysiology and the absence of well-established guidelines [1–3]. We present a case of idiopathic neonatal priapism because of its rarity.

The patient was born to a healthy mother. She received routine prenatal care and the pregnancy was uncomplicated before spontaneous rupture of membranes and vaginal delivery at 39 weeks 5 d. Apgar scores were eight and nine at 1 and 5 min, respectively. The infant did not require any resuscitation. The birth weight was 3400 g (50th percentile). The infant was noted to have a persistent penile erection on the seventh day of life (Figure 1). There was no discolouration of the scrotum or penis, and the testicles were palpable bilaterally. There was no tenderness of the penis with exam. The glans was itself tense on exam. Physical examination was otherwise normal. A complete blood count, serum platelet count, white blood cell count, glucose, electrolyte and bilirubin were within normal limits. at seventh day of life. Doppler ultrasound of the penis at seventh day of life showed normal arterial flow in the dorsal and central arteries of the penis, as well as normal venous flow. Three normal tests were present on scrotal ultrasound. The newborn voided frequently with a good stream and was in no apparent distress. The priapism disappeared after 3 d of observation. The patient continued to have intermittent erections with minimal stimulation until discharge on the 10th day of life. On follow-up at 2 months of age, the patient was found to have a normal examination and the mother reported normal erections.

Discussion Address for correspondence: Esad Koklu, MD, Department of Pediatrics, Division of Neonatology, Megapark Hospital, Kahramanmara¸s, Turkey. Tel: +90 5327649118. Fax: +90 344 2211010. E-mail: dresad@ megaparkhastanesi.com

Neonatal priapism is a rare phenomenon, with only 17 cases reported in the literature since 1876 [1–15]. The true incidence of neonatal priapism is unknown but, in a small surveillance study, Merlob and Livne found that the incidence

J Matern Fetal Neonatal Med Downloaded from informahealthcare.com by Nyu Medical Center on 05/07/15 For personal use only.

2

A. E. Karakaya et al.

Figure 1. Priapism in a newborn.

in their centre was 0.15 per 1000 live births between 1974 and 1988 [9]. Newborn males will frequently have erections with the slightest tactile stimulation. Erections are usually triggered by a full bladder. Typically, this physiological erection lasts a couple of minutes and disappears quickly after the withdrawal of the stimulus [1]. Erections demonstrate that the nerves to the penis are normal. Merlob and Livne discussed that the term ‘‘neonatal priapism’’ was not appropriate and they recommended to call this condition as ‘‘prolonged penile erection in the newborn’’ [9]. Most of the newborns with persistent penile erection are likely to have an idiopathic aetiology. The aetiology of priapism varies significantly between patient populations, but common identifiable causes include blood dyscrasias, pharmacotherapy, neurological conditions, malignancy and trauma [2]. Sickle-cell disease accounts for nearly 70% of paediatric priapism [2], but does not present in the newborn period due to the predominance of foetal haemoglobin. However, when we consider the identifiable aetiologies, polycythemia may be the most common. Polycythemia can result in hyperviscosity and subsequent sludging in the microvasculature. It may be this sludging that results in decreased penile venous outflow and persistence of a penile erection. In older children, reported associations include sickle-cell disease, birth trauma, respiratory distress syndrome, umbilical artery catherisation, metabolic hypoxia, parenteral nutrition and drugs [15]. Of the 17 neonatal priapism cases reported so far, four were attributed to polycythemia [2,4,11], and one was believed to be related to repeated blood transfusions and hypoxia [5]. No details are provided for two cases reported a century ago implicating

J Matern Fetal Neonatal Med, Early Online: 1–3

congenital syphilis and central nervous system trauma from forceps delivery [6]. Sood et al. [13] recently reported a case of neonatal priapism associated with bilateral spontaneous pyocavernositis. There was no discernible cause identified in the nine other reported cases, making idiopathic priapism the most common aetiology in this age group. We did not identify a secondary cause in our patient despite multiple investigations. Our case was noted to have a persistent penile erection on the seventh day of life. Onset of priapism is usually on the first or second day of life, although one premature infant had onset as late as 37 d of life [6]. Duration, although quite variable, averages 4–5 d (range 2–12 d). The duration of priapism was 3 d in our case. There was detumescence with observation alone in 75% of cases, including two cases for which polycythemia was believed to be the cause [4,5]. Medical management with phlebotomy [4] and partial exchange transfusion [11] was effective in the two other cases associated with polycythemia. One case of idiopathic priapism was treated with intravenous infusion of ketamine, resulting in immediate detumescence [10]. Regardless of possible cause, duration or treatment, reported outcomes have been positive with full functional recovery in all published cases of neonatal priapism. Unfortunately, follow-up has been limited to early infancy in most cases, with the oldest age at last follow-up being 8 years [9]. The patient was 2 months old at the time of this writing, and the patient was found to have a normal examination and the mother reported normal erections. While erectile dysfunction is a significant concern in older children and adults with priapism, we did not find any reports of erectile impairment resulting from priapism in the newborn period. In fact, the term ‘‘persistent penile erection of the newborn’’ has been proposed as more accurately reflecting what appears to be a relatively benign condition [10,11]. However, it is difficult to make this statement based on a limited number of cases with relatively short follow-up. The case presented by Sood et al. [13] suggests that extremely prolonged neonatal priapism may result in serious complications such as pyocavernositis. Initial evaluation of newborns with persistent penile erections should include careful assessment to rule out secondary causes such as polycythemia, neurological conditions or urinary obstruction. Doppler ultrasound of the penis is essential to document normal arterial flow, but cavernous blood gas analysis should be reserved for exceptional circumstances in consultation with a paediatric urologist. Conservative management with close observation is appropriate in most cases of idiopathic neonatal priapism because the majority will resolve spontaneously with no serious sequelae. Burgu et al. [14] presented a case of idiopathic neonatal priapism with normal arterial and venous flow on Doppler ultrasound of the penis. We corroborated these findings by documenting normal arterial and venous flow on Doppler ultrasound. These findings support the theory that priapism in the neonatal age group is more likely to represent a relatively benign form of non-ischaemic priapism and provide more justification for observation alone rather than aggressive intervention in these cases [2,14]. In conclusion, we present a case of idiopathic neonatal priapism because of its rarity and management of priapism in the newborn by observation alone might be the best approach.

DOI: 10.3109/14767058.2014.1003538

Declaration of interest The authors declare no conflicts of interests. The authors alone are responsible for the content and writing of this article.

J Matern Fetal Neonatal Med Downloaded from informahealthcare.com by Nyu Medical Center on 05/07/15 For personal use only.

References 1. Aktoz T, Tepeler A, Gu¨ndog˘du EO, et al. Priapism in the newborn: management and review of literature. Andrologia 2011;43:65–7. 2. Dust N, Daboval T, Guerra L. Evaluation and management of priapism in a newborn: a case report and review of the literature. Paediatr Child Health 2011;16:e6–8. 3. Song PH, Moon KH. Priapism: current updates in clinical management. Korean J Urol 2013;54:816–23. 4. Humbert JR, Abelson H, Hathawa WE, et al. Polycythemia in small for gestational age infants. J Pediatr 1969;78:812–9. 5. Larocque MA, Cosgrove MD. Priapism: a review of 46 cases. J Urol 1974;112:770–3. 6. Amlie RN, Bourgeois B, Huxtable RF. Priapism in preterm infant. Urology 1977;9:558–9.

Priapism in a newborn

3

7. Leal J, Walker D, Egan EA. Idiopathic priapism in the newborn. J Urol 1978;120:376. 8. Shapiro SR. Idiopathic priapism in the newborn. J Urol 1979;121: 838. 9. Merlob P, Livne PM. Incidence, possible causes and followup of idiopathic prolonged penile erection in the newborn. J Urol 1989; 141:1410–12. 10. Stothers L, Ritchie B. Priapism in the newborn. Can J Surg 1992; 35:325–6. 11. Walker JR, Casale AJ. Prolonged penile erection in the newborn. Urology 1997;50:796–9. 12. Meijer B, Bakker HH. Management of priapism in the newborn. Urology 2003;61:224. 13. Sood R, Wadhwa SN, Jain V. Neonatal priapism associated with spontaneous bilateral pyocavernositis. Ann Acad Med Singapore 2006;35:425–7. 14. Burgu B, Talas H, Erdeve O, et al. Approach to newborn priapism: a rare entity. J Ped Urol 2007;3:509–11. 15. Walker JR, Casale AJ. Prolonged penile erection in the newborn. Urology 1997;50:796–99.

A rare entity: idiopathic priapism in a newborn and review of the literature.

Priapism is a pathological condition of a penile erection that persists beyond or is unrelated to sexual stimulation. Priapism is an important medical...
206KB Sizes 3 Downloads 5 Views