The first case of neonatal priapism during hypothermia for hypoxic-ischemic encephalopathy and a literature review

Abstract

Neonatal priapism is a rare condition with only 26 described cases in literature since 1879. It is defined as a persistent penile erection occurring in the first 28 days of life, lasting at least 4 h that usually happens in the first days (from 2 to 12 days). It is a very different condition compared to the adult one because in newborns it is a relatively benign phenomenon. As a result of this paucity of described cases, classification and management are not well known by most of neonatologists and currently there are no established guidelines for its management. Most cases are idiopathic but other aetiologies are possible (polycythemia, blood transfusion and drugs). We describe our only case, which occurred during hypothermia therapy and review the literature to clarify the best choice in management of this rare entity.

Neonatal priapism: first reported case in South East Asia and literature review

“…Newborn males commonly have erections which were triggered by a distended urinary bladder, tactile stimulation, diaper changing, bathing and urethral catheterization. 1 These erections quickly subsided following withdrawal of the precipitating factor. These physiological erections also occured spontaneously and demonstrated that the nerves to penis were normal.…”

“…7 Available literature on this pathology was limited to less than 30 case reports. 1 These reports were spread across the various continents including one in Asia. Sood et al reported a case of neonatal priapism in India in 2006.…”

“…First line investigations included blood counts and C-reactive protein. 1,4 Neonatal priapism was thought to be a form of non ischaemic priapism. Colour Doppler ultrasonography (CDU) and cavernous blood gas can be used to distinguish ischaemic from non ischaemic priapism.…”

“…Majority of the reported cases were managed conservatively and achieved spontaneous detumescence within a period of 5 days. 1,10 Medical or surgical intervention should be reserved for scenarios where the priapism lasted more than 5 days. 6 Intravenous ketamine had been described as an option with the child achieving spontaneous detumescence.…”

“…10,11 Fannie et al cautions against the use of ketamine citing its possible role in neuroapoptosis in newborns. 1 Venesection and partial exchange transfusion were reported to be successful in polycythaemia associated priapism. 12,13 Alternatively, a conservative approach had also been successful in two cases associated with polycythaemia.…”