link
Bookmarks
Periventricular Cyst
Anne G. Osborn, MD, FACR; Susan I. Blaser, MD, FRCPC
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
2
7
0

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 (GC)
              • 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

                                    1. Drenckhahn A et al: Leukodystrophy with multiple beaded periventricular cysts: unusual cranial MRI results in Canavan disease. J Inherit Metab Dis. ePub, 2015
                                    2. Esteban H et al: Prenatal features of isolated subependymal pseudocysts associated with adverse pregnancy outcome. Ultrasound Obstet Gynecol. ePub, 2015
                                    3. Livingston JH et al: Leukoencephalopathy with calcifications and cysts: a purely neurological disorder distinct from coats plus. Neuropediatrics. 45(3):175-82, 2014
                                    4. 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
                                    5. 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
                                    6. 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
                                    7. 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
                                    8. 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
                                    9. 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
                                    10. van Baalen A et al: Anterior choroid plexus cysts: distinction from germinolysis by high-resolution sonography. Pediatr Int. 50(1):57-61, 2008
                                    11. Chuang YC et al: Neurodevelopment in very low birth weight premature infants with postnatal subependymal cysts. J Child Neurol. 22(4):402-5, 2007
                                    12. van Baalen A et al: [Non-haemorrhagic subependymal pseudocysts: ultrasonographic, histological and pathogenetic variability.] Ultraschall Med. 28(3):296-300, 2007
                                    13. 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
                                    14. Finsterer J et al: Adult unilateral periventricular pseudocysts with ipsilateral headache. Clin Neurol Neurosurg. 108(1):73-6, 2005
                                    15. 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
                                    16. 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
                                    17. Cuillier F et al: [Subependymal pseudocysts in the fetal brain revealing Zellweger syndrome.] J Gynecol Obstet Biol Reprod (Paris). 33(4):325-9, 2004
                                    18. Qian JH et al: [Prospective study on prognosis of infants with neonatal subependymal cysts.] Zhonghua Er Ke Za Zhi. 42(12):913-6, 2004
                                    19. Herini E et al: Clinical features of infants with subependymal germinolysis and choroid plexus cysts. Pediatr Int. 45(6):692-6, 2003
                                    20. 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
                                    21. van der Knaap MS et al: D-2-hydroxyglutaric aciduria: further clinical delineation. J Inherit Metab Dis. 22(4):404-13, 1999
                                    22. Rosenfeld DL et al: Coarctation of the lateral ventricles: an alternative explanation for subependymal pseudocysts. Pediatr Radiol. 27(12):895-7, 1997
                                    23. Shackelford GD et al: Cysts of the subependymal germinal matrix: sonographic demonstration with pathologic correlation. Radiology. 149(1):117-21, 1983
                                    24. Takashima S et al: Old subependymal necrosis and hemorrhage in the prematurely born infants. Brain Dev. 1(4):299-304, 1979
                                    25. De León GA et al: Cystic degeneration of the telencephalic subependymal germinal layer in newborn infants. J Neurol Neurosurg Psychiatry. 38(3):265-71, 1975
                                    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 (GC)
                                                • 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

                                                                      1. Drenckhahn A et al: Leukodystrophy with multiple beaded periventricular cysts: unusual cranial MRI results in Canavan disease. J Inherit Metab Dis. ePub, 2015
                                                                      2. Esteban H et al: Prenatal features of isolated subependymal pseudocysts associated with adverse pregnancy outcome. Ultrasound Obstet Gynecol. ePub, 2015
                                                                      3. Livingston JH et al: Leukoencephalopathy with calcifications and cysts: a purely neurological disorder distinct from coats plus. Neuropediatrics. 45(3):175-82, 2014
                                                                      4. 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
                                                                      5. 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
                                                                      6. 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
                                                                      7. 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
                                                                      8. 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
                                                                      9. 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
                                                                      10. van Baalen A et al: Anterior choroid plexus cysts: distinction from germinolysis by high-resolution sonography. Pediatr Int. 50(1):57-61, 2008
                                                                      11. Chuang YC et al: Neurodevelopment in very low birth weight premature infants with postnatal subependymal cysts. J Child Neurol. 22(4):402-5, 2007
                                                                      12. van Baalen A et al: [Non-haemorrhagic subependymal pseudocysts: ultrasonographic, histological and pathogenetic variability.] Ultraschall Med. 28(3):296-300, 2007
                                                                      13. 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
                                                                      14. Finsterer J et al: Adult unilateral periventricular pseudocysts with ipsilateral headache. Clin Neurol Neurosurg. 108(1):73-6, 2005
                                                                      15. 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
                                                                      16. 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
                                                                      17. Cuillier F et al: [Subependymal pseudocysts in the fetal brain revealing Zellweger syndrome.] J Gynecol Obstet Biol Reprod (Paris). 33(4):325-9, 2004
                                                                      18. Qian JH et al: [Prospective study on prognosis of infants with neonatal subependymal cysts.] Zhonghua Er Ke Za Zhi. 42(12):913-6, 2004
                                                                      19. Herini E et al: Clinical features of infants with subependymal germinolysis and choroid plexus cysts. Pediatr Int. 45(6):692-6, 2003
                                                                      20. 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
                                                                      21. van der Knaap MS et al: D-2-hydroxyglutaric aciduria: further clinical delineation. J Inherit Metab Dis. 22(4):404-13, 1999
                                                                      22. Rosenfeld DL et al: Coarctation of the lateral ventricles: an alternative explanation for subependymal pseudocysts. Pediatr Radiol. 27(12):895-7, 1997
                                                                      23. Shackelford GD et al: Cysts of the subependymal germinal matrix: sonographic demonstration with pathologic correlation. Radiology. 149(1):117-21, 1983
                                                                      24. Takashima S et al: Old subependymal necrosis and hemorrhage in the prematurely born infants. Brain Dev. 1(4):299-304, 1979
                                                                      25. De León GA et al: Cystic degeneration of the telencephalic subependymal germinal layer in newborn infants. J Neurol Neurosurg Psychiatry. 38(3):265-71, 1975