Case Report
Priapism in Neonates: Case Reviews and Management Recommendations
Terah Hennick1*, Hubert S. Swana1,2, Diana Cardona-Grau1,2, and Mark ARich1,2
1Department of Medicine, University of Central Florida College of Medicine, USA
2Arnold Palmer Hospital for Children, Urology Center, USA
*Corresponding author: Terah Hennick, Department of Medicine, University
of Central Florida College of Medicine, 8340 Prestbury Drive, Orlando, FL, USA 32832, Tel: 18-177396265; Email:
thennick@Knights.ucf.edu
Submitted: 11 January 2019; Accepted: 17 April 2019; Published: 19 April 2019
Cite this article: Hennick T, Swana HS, Cardona-Grau D, Rich MA (2019) Priapism
in Neonates: Case Reviews and Management Recommendations. JSM Urol Res 3: 3.
CDU: Color Doppler Ultrasound
Priapism is defined as a prolonged, persistent penile erection
not associated with sexual stimulation or desire [1]. Multiple
types of priapism in children have been described and include
ischemic (veno-occlusive, painful), stuttering, non-ischemic
(arterial, usually painless), and neonatal [2]. Neonatal priapism
is defined as a prolonged and persistent erection lasting greater
than or equal to four hours. It is relatively rare, with only 19 cases
reported in the literature since 1876 [3,4]. In most cases, the
exact cause is unclear. Idiopathic neonatal priapism is the most
common etiology, occurring spontaneously and unassociated
with stimulation [1]. In premature neonates, polycythemia is also
a relatively common cause [2]. The condition usually resolves
spontaneously, but there are currently no guidelines regarding
the management of neonatal priapism.
Case 1
A20 day-old infant presented to the emergency room with
a persistent erection. The patient was the product of a 32-week
gestation. The parents did not recall when the erection started
but the penis was erect for “several days.” It was noted during every diaper change that the infant had an erection. The boy
was feeding and urinating regularly and without difficulty and
was in no apparent pain or discomfort. Urologic consultation
was obtained. On examination, the patient was uncircumcised,
without any penile cyanosis or swelling, and with descended
testes, bilaterally. The penis was tumescent. Both corpora were
firm and the glans was nontender, soft, and non-cyanotic. Workup
included a complete blood count, penile and scrotal duplex
ultrasounds to assess arterial and venous blood flow (Figure 1),
pelvic ultrasound to rule out masses, and a sickle cell preparation.
Complete blood count revealed an elevated hematocrit of 57 g/L
with a normal sickle cell panel.
Case 2
Urologic consultation was obtained for a day-old male in the
neonatal intensive care unit. The child was the product of a 28-
week gestation and had an erection that lasted over 12 hours
prior to consultation. The infant was urinating regularly and
without difficulty and was in no apparent pain or discomfort. On
examination, he was uncircumcised, without any penile cyanosis
or edema. The penis was tumescent, both corpora were erect,
and the glans was soft. Workup also included a complete blood
count, penile and scrotal duplex ultrasound (Figure 2), pelvic
ultrasound, and a sickle cell preparation. In this case the complete
blood count revealed a normal hematocrit of 41.9 g/L. The sickle
cell panel was negative.
Both patient’s erections resolved after approximately two
days with careful observation and without any treatment or
intervention. They were seen in follow-up approximately three
weeks later without any history of recurrent prolonged erection
since discharge. On examination, the penis was normal and
uncircumcised without an erection in both cases.
Priapism is considered a urological emergency with
management directed at prevention of penile disfigurement and
preservation of future erectile function. Classification of priapism
includes both ischemic and non-ischemic. Priapism in childhood
is usually ischemic and a result of multiple etiologies as shown in
Table 1 [2].
Non-ischemic priapism is rare in children and is usually
idiopathic, with subclinical perineal birth canal trauma
hypothesized to be the cause resulting in a benign, non-ischemic
erection [2]. Other causes of primary neonatal priapism include
polycythemia, infection, respiratory distress syndrome, and
umbilical artery catheterization [2,3]. Although sickle cell disease
in the most common cause of childhood priapism, the high levels
of fetal hemoglobin in the newborn are protective against vasoocclusion
and resultant priapism in newborns with sickle cell
disease [4].
Since it is common for healthy newborns to have an erection,
neonatal priapism must be differentiated from prolonged
physiological erections, which are benign and last less than four
hours. Neonatal priapism is a clinical diagnosis, with a persistent
erection lasting greater than or equal to four hours without any
scrotal or penile discoloration, e.g. cyanosis, and no apparent
pain. The duration has reportedly ranged from two to twelve days and does not seem to be associated with any long-term
sexual dysfunction [1].
The process of tumescence includes a latent phase, with
relaxation of arteries in the corpus cavernosum and sinusoidal
smooth muscle leading to an increase in arterial influx and
capacitance, respectively. The subsequent tumescent phase
results from sinusoidal blood trapping. The full erection phase
is caused by sub-tunica venous plexus compression leading to
stretching of the tunica albuginea and resultant occlusion of
emissary veins [5]. Lastly, the rigid erection phase results from
contraction of the ischiocavernous muscles, which increases
pressure in the corpus cavernosum to above systolic blood
pressure for short periods [6]. Although the corpus spongiosum
and glans experience increased arterial influx, they lack a tunica
albuginea and therefore act mainly as an arteriovenous shunt
[2]. An erection can be caused by stimulation including genital/
reflexogenic and central/psychogenic or central origination/
nocturnal, i.e. androgen release during rapid eye movement
sleep in adolescence [6]. Reflexogenic erections are physiologic
in neonates and children and can occur during bathing, diaper
changing, urethral catheterization, or with a full bladder; however,
detumescence should occur once the stimulus is removed.
The postulated pathophysiology of priapism varies depending
on the subtype. Ischemic priapism, according to Hinman’s classic
theory, is a result of reduced blood flow and congestion leading to
increased blood viscosity and the resultant dark, deoxygenated
blood observed during corporal aspirations [7]. The ischemia
leads to necrosis followed by fibrosis of the corpus cavernosum
smooth muscle which causes erectile dysfunction and penile
distortion [2]. The exact pathophysiology of stuttering priapism,
or recurrent prolonged erections, is poorly understood. It is
believed to have a similar etiology as ischemic priapism and
is most commonly caused by sickle cell disease in children.
Proposed mechanisms of stuttering priapism include overresponsiveness
to androgenic or sexual stimulation resulting
from corpus cavernosum endothelial nitrous oxide deficiency
leading to reduced tonic corpus cavernosum smooth muscle
tone, adrenoceptor impairment, transforming growth factor-beta
upregulation, intracavernous venule scarring, and an abnormal
central nervous system control mechanism [2,7]. Non-ischemic
priapism is most commonly caused by an arterio-sinusoidal
fistula. Penile, perineal, or pelvis trauma, usually from straddle
or coital injury, leads to laceration of an artery or arteries, most
commonly the cavernous arterioles in the crura or corporal
bodies. This leads to formation of the arteriolar-sinusoidal fistula.
The resultant high-flow state and sinusoidal blood pooling causes mechanical stimulation of the endothelial nitrous oxide synthase
and smooth muscle relaxation. Although non-ischemic priapism
without a high-flow hemodynamic state, e.g. fistula, may occur,
the etiology is unknown [2].
All published cases of neonatal priapism have reported
full functional recovery, independent of etiology, duration,
or management. Seventy-five percent of cases managed by
observation resolved spontaneously [3]. Although reported
cases that have undergone a workup were found to be nonischemic,
the risk of untreated ischemic priapism is cavernous
body fibrosis and resultant erectile dysfunction. For this reason,
initial testing with color Doppler ultrasound (CDU) in neonatal
priapism is recommended to confirm that it is the non-ischemic
subtype before deciding on conservative management. CDU
is a noninvasive, relatively inexpensive, and safe method of
detecting scrotal and penile blood flow as well as rule out pelvic
masses. Corporal aspiration and blood gas analysis should be
reserved for cases without demonstrable arterial blood flow
via CDU or in which ischemic priapism is otherwise suspected.
A complete blood count, sickle cell panel, and C-reactive protein
have been suggested in the literature and could be considered
in the initial work-up, especially when there is concern for
infection, polycythemia, sickle cell disease, or leukemia [1,3].
In clinical situations associated with polycythemia, such as
this case, studies have suggested performing a red cell volume
reduction (venesection) or phlebotomy and partial transfusion
[1,4]. We managed our cases with close observation and noted
spontaneous resolution without sequalae suggesting that not
all cases of neonatal priapism with concomitant polycythemia
require intervention. Furthermore, there may be a threshold at
which an elevated hematocrit associated with neonatal priapism
required intervention. Future work might aim to identify this
threshold.
In this study we present two cases of non-ischemic neonatal
priapism, one case associated with polycythemia, that both
resolved within two days with minimal workout and without
intervention. All published cases of neonatal priapism have
reported full functional recovery, independent of etiology,
duration, or management, suggesting conservative management
of neonatal priapism is recommended. However, because the risk
of untreated ischemic priapism is cavernous body fibrosis and
resultant erectile dysfunction, we recommend initial testing with
color CDU in neonatal priapism to confirm non-ischemic subtype
before deciding on conservative management. Blood gas analysis
of aspirated corporal blood is reserved for select cases.