Periventricular cysts of newborn: Encompasses wide variety of periventricular cystic lesions
Anterior choroid plexus cyst (ACPC)
Cyst of anterior portion of choroid plexus in body of lateral ventricles
Subependymal pseudocyst (SEPC)
Subependymal cyst in region of caudate nucleus; may be congenital (germinolytic) or acquired (posthemorrhagic)
Connatal cyst (CS), a.k.a. coarctation of anterior horns
Controversial; some authors consider CS to be germinolytic cysts (GCs)
Cystic periventricular leukomalacia (cPVL), previously known as paraventricular cysts
Considerable overlap and confusion in literature
IMAGING
General Features
CT Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
Apatean D et al: Congenital lactic acidosis, cerebral cysts and pulmonary hypertension in an infant with FOXRED1 related complex 1 deficiency. Mol Genet Metab Rep. 18:32-8, 2019
Gilani A et al: Distinguishing encephaloclastic lesions resulting from primary or secondary pyruvate dehydrogenase deficiency from other neonatal or infantile cavitary brain lesions. Pediatr Dev Pathol. 23(3):189-96, 2019
Wang M et al: Isolated periventricular pseudocysts do not affect white matter microstructure development in neonatal stage: a retrospective case-control diffusion tensor imaging study. Eur J Radiol. 116:152-9, 2019
Weise J et al: Analyses of pathological cranial ultrasound findings in neonates that fall outside recent indication guidelines: results of a population-based birth cohort: survey of neonates in Pommerania (SNiP-study). BMC Pediatr. 19(1):476, 2019
Zagaglia S et al: Neurologic phenotypes associated with COL4A1/2 mutations: expanding the spectrum of disease. Neurology. 91(22):e2078-88, 2018
Shinar S et al: Familial brain periventricular pseudocysts. Fetal Diagn Ther. 42(1):42-7, 2017
Cooper S et al: Prenatal evaluation, imaging features, and neurodevelopmental outcome of prenatally diagnosed periventricular pseudocysts. AJNR Am J Neuroradiol. 37(12):2382-8, 2016
Drenckhahn A et al: Leukodystrophy with multiple beaded periventricular cysts: unusual cranial MRI results in Canavan disease. J Inherit Metab Dis. 38(5):983-4, 2015
Esteban H et al: Prenatal features of isolated subependymal pseudocysts associated with adverse pregnancy outcome. Ultrasound Obstet Gynecol. 46(6):678-87, 2015
Livingston JH et al: Leukoencephalopathy with calcifications and cysts: a purely neurological disorder distinct from coats plus. Neuropediatrics. 45(3):175-82, 2014
Cevey-Macherel M et al: Neurodevelopment outcome of newborns with cerebral subependymal pseudocysts at 18 and 46 months: a prospective study. Arch Dis Child. 98(7):497-502, 2013
Tan ZY et al: Case of the month. Ultrasound and MRI features of connatal cysts: clinicoradiological differentiation from other supratentorial periventricular cystic lesions. Br J Radiol. 83(986):180-3, 2010
Fernandez Alvarez JR et al: Diagnostic value of subependymal pseudocysts and choroid plexus cysts on neonatal cerebral ultrasound: a meta-analysis. Arch Dis Child Fetal Neonatal Ed. 94(6):F443-6, 2009
Leijser LM et al: Brain imaging findings in very preterm infants throughout the neonatal period: part I. Incidences and evolution of lesions, comparison between ultrasound and MRI. Early Hum Dev. 85(2):101-9, 2009
van Baalen A et al: From fossil to fetus: nonhemorrhagic germinal matrix echodensity caused by mineralizing vasculitis--hypothesis of fossilizing germinolysis and gliosis. J Child Neurol. 24(1):36-44, 2009
Soares-Fernandes JP et al: Neonatal pyruvate dehydrogenase deficiency due to a R302H mutation in the PDHA1 gene: MRI findings. Pediatr Radiol. 38(5):559-62, 2008
van Baalen A et al: Anterior choroid plexus cysts: distinction from germinolysis by high-resolution sonography. Pediatr Int. 50(1):57-61, 2008
Chuang YC et al: Neurodevelopment in very low birth weight premature infants with postnatal subependymal cysts. J Child Neurol. 22(4):402-5, 2007
van Baalen A et al: [Non-haemorrhagic subependymal pseudocysts: ultrasonographic, histological and pathogenetic variability.] Ultraschall Med. 28(3):296-300, 2007
Epelman M et al: Differential diagnosis of intracranial cystic lesions at head US: correlation with CT and MR imaging. Radiographics. 26(1):173-96, 2006
Finsterer J et al: Adult unilateral periventricular pseudocysts with ipsilateral headache. Clin Neurol Neurosurg. 108(1):73-6, 2005
Read MH et al: Clinical, biochemical, magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (1H MRS) findings in a fourth case of combined D- and L-2 hydroxyglutaric aciduria. J Inherit Metab Dis. 28(6):1149-50, 2005
Wilson CJ et al: Severe holocarboxylase synthetase deficiency with incomplete biotin responsiveness resulting in antenatal insult in samoan neonates. J Pediatr. 147(1):115-8, 2005
Cuillier F et al: [Subependymal pseudocysts in the fetal brain revealing Zellweger syndrome.] J Gynecol Obstet Biol Reprod (Paris). 33(4):325-9, 2004
Qian JH et al: [Prospective study on prognosis of infants with neonatal subependymal cysts.] Zhonghua Er Ke Za Zhi. 42(12):913-6, 2004
Herini E et al: Clinical features of infants with subependymal germinolysis and choroid plexus cysts. Pediatr Int. 45(6):692-6, 2003
Rott HD et al: Cyst-like cerebral lesions in tuberous sclerosis. Am J Med Genet 111(4):435-9, 2002
Pal BR et al: Frontal horn thin walled cysts in preterm neonates are benign. Arch Dis Child Fetal Neonatal Ed. 85(3):F187-93, 2001
Related Anatomy
Loading...
Related Differential Diagnoses
Loading...
References
Tables
Tables
KEY FACTS
Terminology
Imaging
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
Neonatal periventricular cysts (PCs)
Definitions
Periventricular cysts of newborn: Encompasses wide variety of periventricular cystic lesions
Anterior choroid plexus cyst (ACPC)
Cyst of anterior portion of choroid plexus in body of lateral ventricles
Subependymal pseudocyst (SEPC)
Subependymal cyst in region of caudate nucleus; may be congenital (germinolytic) or acquired (posthemorrhagic)
Connatal cyst (CS), a.k.a. coarctation of anterior horns
Controversial; some authors consider CS to be germinolytic cysts (GCs)
Cystic periventricular leukomalacia (cPVL), previously known as paraventricular cysts
Considerable overlap and confusion in literature
IMAGING
General Features
CT Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
Apatean D et al: Congenital lactic acidosis, cerebral cysts and pulmonary hypertension in an infant with FOXRED1 related complex 1 deficiency. Mol Genet Metab Rep. 18:32-8, 2019
Gilani A et al: Distinguishing encephaloclastic lesions resulting from primary or secondary pyruvate dehydrogenase deficiency from other neonatal or infantile cavitary brain lesions. Pediatr Dev Pathol. 23(3):189-96, 2019
Wang M et al: Isolated periventricular pseudocysts do not affect white matter microstructure development in neonatal stage: a retrospective case-control diffusion tensor imaging study. Eur J Radiol. 116:152-9, 2019
Weise J et al: Analyses of pathological cranial ultrasound findings in neonates that fall outside recent indication guidelines: results of a population-based birth cohort: survey of neonates in Pommerania (SNiP-study). BMC Pediatr. 19(1):476, 2019
Zagaglia S et al: Neurologic phenotypes associated with COL4A1/2 mutations: expanding the spectrum of disease. Neurology. 91(22):e2078-88, 2018
Shinar S et al: Familial brain periventricular pseudocysts. Fetal Diagn Ther. 42(1):42-7, 2017
Cooper S et al: Prenatal evaluation, imaging features, and neurodevelopmental outcome of prenatally diagnosed periventricular pseudocysts. AJNR Am J Neuroradiol. 37(12):2382-8, 2016
Drenckhahn A et al: Leukodystrophy with multiple beaded periventricular cysts: unusual cranial MRI results in Canavan disease. J Inherit Metab Dis. 38(5):983-4, 2015
Esteban H et al: Prenatal features of isolated subependymal pseudocysts associated with adverse pregnancy outcome. Ultrasound Obstet Gynecol. 46(6):678-87, 2015
Livingston JH et al: Leukoencephalopathy with calcifications and cysts: a purely neurological disorder distinct from coats plus. Neuropediatrics. 45(3):175-82, 2014
Cevey-Macherel M et al: Neurodevelopment outcome of newborns with cerebral subependymal pseudocysts at 18 and 46 months: a prospective study. Arch Dis Child. 98(7):497-502, 2013
Tan ZY et al: Case of the month. Ultrasound and MRI features of connatal cysts: clinicoradiological differentiation from other supratentorial periventricular cystic lesions. Br J Radiol. 83(986):180-3, 2010
Fernandez Alvarez JR et al: Diagnostic value of subependymal pseudocysts and choroid plexus cysts on neonatal cerebral ultrasound: a meta-analysis. Arch Dis Child Fetal Neonatal Ed. 94(6):F443-6, 2009
Leijser LM et al: Brain imaging findings in very preterm infants throughout the neonatal period: part I. Incidences and evolution of lesions, comparison between ultrasound and MRI. Early Hum Dev. 85(2):101-9, 2009
van Baalen A et al: From fossil to fetus: nonhemorrhagic germinal matrix echodensity caused by mineralizing vasculitis--hypothesis of fossilizing germinolysis and gliosis. J Child Neurol. 24(1):36-44, 2009
Soares-Fernandes JP et al: Neonatal pyruvate dehydrogenase deficiency due to a R302H mutation in the PDHA1 gene: MRI findings. Pediatr Radiol. 38(5):559-62, 2008
van Baalen A et al: Anterior choroid plexus cysts: distinction from germinolysis by high-resolution sonography. Pediatr Int. 50(1):57-61, 2008
Chuang YC et al: Neurodevelopment in very low birth weight premature infants with postnatal subependymal cysts. J Child Neurol. 22(4):402-5, 2007
van Baalen A et al: [Non-haemorrhagic subependymal pseudocysts: ultrasonographic, histological and pathogenetic variability.] Ultraschall Med. 28(3):296-300, 2007
Epelman M et al: Differential diagnosis of intracranial cystic lesions at head US: correlation with CT and MR imaging. Radiographics. 26(1):173-96, 2006
Finsterer J et al: Adult unilateral periventricular pseudocysts with ipsilateral headache. Clin Neurol Neurosurg. 108(1):73-6, 2005
Read MH et al: Clinical, biochemical, magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (1H MRS) findings in a fourth case of combined D- and L-2 hydroxyglutaric aciduria. J Inherit Metab Dis. 28(6):1149-50, 2005
Wilson CJ et al: Severe holocarboxylase synthetase deficiency with incomplete biotin responsiveness resulting in antenatal insult in samoan neonates. J Pediatr. 147(1):115-8, 2005
Cuillier F et al: [Subependymal pseudocysts in the fetal brain revealing Zellweger syndrome.] J Gynecol Obstet Biol Reprod (Paris). 33(4):325-9, 2004
Qian JH et al: [Prospective study on prognosis of infants with neonatal subependymal cysts.] Zhonghua Er Ke Za Zhi. 42(12):913-6, 2004
Herini E et al: Clinical features of infants with subependymal germinolysis and choroid plexus cysts. Pediatr Int. 45(6):692-6, 2003
Rott HD et al: Cyst-like cerebral lesions in tuberous sclerosis. Am J Med Genet 111(4):435-9, 2002
Pal BR et al: Frontal horn thin walled cysts in preterm neonates are benign. Arch Dis Child Fetal Neonatal Ed. 85(3):F187-93, 2001
STATdx includes over 200,000 searchable images, including x-ray, CT, MR, and ultrasound images. To access all images, please log in or subscribe.