Idiopathic Neonatal Subpial Hemorrhage with Underlying Cerebral Infarct: Imaging Features and Clinical Outcome

Abstract

BACKGROUND AND PURPOSE: Neonatal subpial hemorrhage with underlying cerebral infarct is a previously described but poorly understood clinicoradiographic syndrome. We sought to further characterize the cranial ultrasound and MR imaging characteristics and associated outcomes of this condition across the full range of gestational ages, including extreme and very preterm neonates. MATERIALS AND METHODS:This was a single tertiary pediatric center retrospective case series. Brain MR imaging and cranial ultrasound of neonates with subpial hemorrhage with underlying cerebral infarct were identified from a population-based radiology registry (2006)(2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014)(2015)(2016)(2017)(2018)(2019)(2020). Original images were reviewed by 2 neuroradiologists blinded to history and outcome. Clinical presentation, course, and outcome at .12 months were abstracted from medical records. The diagnostic utility of cranial ultrasound was compared with that of MR imaging.RESULTS: Sixteen patients were included (median gestational age, 36.5 weeks; range, 27-41 weeks; 31% premature). MR images were obtained acutely at the time of presentation between days 0 and 9 of life. On T2WI and DWI, a consistent presence of a hypointense subpial bleed and an underlying hyperintense cerebral cortex were recognized, which created a distinct MR imaging pattern resembling the yin-yang symbol. Findings of all the MRAs and MRVs were normal. Cranial ultrasound detected 6 of 7 MR imaging lesions with sonographic features correlating well with MR imaging. The 3 extreme or very preterm neonates did not survive. The remainder survived with relatively mild neurologic deficits.CONCLUSIONS: Subpial hemorrhage with underlying infarction is a recognizable condition with unique MR imaging and sonographic features. Improved recognition may advance understanding of risk factors and outcomes.

Neuroimaging in Perinatal Stroke and Cerebrovascular Disease

“…Some genetic arteriopathies associated with both fetal and neonatal hemorrhagic stroke include collagen IVA and JAM3 mutations, which can appear identical to hemorrhagic venous infarctions (77)(78)(79)(80)(81)(82)(83)(84). In addition to hemorrhages occurring later in life, fetal and neonatal patients may present with subpial hemorrhages (Figure 8), which may be related to local venous thrombosis or birth trauma (85)(86)(87). In these cases, blood is seen between the pia mater and the displaced brain parenchyma, often accompanied by venous infarction and subarachnoid or parenchymal blood.…”

Ultrasound findings of subpial hemorrhage in neonates

“…Subpial hemorrhage (SPH) is a subtype of intracranial hemorrhage that was not widely recognized until recently and that remains not to be fully understood [1][2][3][4][5][6]. SPH is not uncommon in neonates and infants [1][2][3][4][5][6] and is estimated to account for approximately 15% of perinatal intracranial hemorrhages in one postmortem study [1].…”

“…Subpial hemorrhage (SPH) is a subtype of intracranial hemorrhage that was not widely recognized until recently and that remains not to be fully understood [1][2][3][4][5][6]. SPH is not uncommon in neonates and infants [1][2][3][4][5][6] and is estimated to account for approximately 15% of perinatal intracranial hemorrhages in one postmortem study [1]. The subpial space is a potential space between the pia mater and the glia limitans at the outermost layer of the cortex as well as a tight anatomic space composed of collagen [1,5].…”

“…The subpial space is a potential space between the pia mater and the glia limitans at the outermost layer of the cortex as well as a tight anatomic space composed of collagen [1,5]. Therefore, SPH occurs along the cerebral sulci in a manner similar to subarachnoid hemorrhage (SAH), but SPH tends to be thicker and more confined than SAH [2][3][4][5][6].…”

Factor XIII deficiency in a neonate presenting as subpial haemorrhage

“…Few cases of subpial haemorrhage have been described in the literature due to their relative rarity, which is estimated at approximately 4% according to pathological reports. 4 , 5 , 6 , 7 Consequently, there is a relative paucity of knowledge regarding its aetiology, associated clinical manifestations and outcomes. The risk factors and pathophysiology of this entity are unique compared with other forms of extra-axial haemorrhage.…”