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Persistent Hyperplastic Primary Vitreous
H. Christian Davidson, MD; Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Persistent hyperplastic primary vitreous (PHPV)
              • Definitions

                • Congenital lesion due to incomplete regression of embryonic ocular blood supply
                • Persistent fetal vasculature = new term in recent literature

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Burns NS et al: Diagnostic imaging of fetal and pediatric orbital abnormalities. AJR Am J Roentgenol. 201(6):W797-808, 2013
                                            2. Rizvi SW et al: Bilateral persistent hyperplastic primary vitreous: a close mimic of retinoblastoma. Semin Ophthalmol. 28(1):25-7, 2013
                                            3. Brennan RC et al: US and MRI of pediatric ocular masses with histopathological correlation. Pediatr Radiol. 42(6):738-49, 2012
                                            4. Khokhar S et al: Approach to cataract with persistent hyperplastic primary vitreous. J Cataract Refract Surg. 37(8):1382-5, 2011
                                            5. Cerón O et al: The vitreo-retinal manifestations of persistent hyperplasic primary vitreous (PHPV) and their management. Int Ophthalmol Clin. 48(2):53-62, 2008
                                            6. Katorza E et al: Prenatal ultrasonographic diagnosis of persistent hyperplastic primary vitreous. Ultrasound Obstet Gynecol. 32(2):226-8, 2008
                                            7. Robinson AJ et al: MRI of the fetal eyes: morphologic and biometric assessment for abnormal development with ultrasonographic and clinicopathologic correlation. Pediatr Radiol. 38(9):971-81, 2008
                                            8. Balmer A et al: Differential diagnosis of leukocoria and strabismus, first presenting signs of retinoblastoma. Clin Ophthalmol. 1(4):431-9, 2007
                                            9. Schulz E et al: Long-term visual function and relative amblyopia in posterior persistent hyperplastic primary vitreous (PHPV). Strabismus. 14(2):121-5, 2006
                                            10. Mackeen LD et al: High-frequency ultrasonography findings in persistent hyperplastic primary vitreous. J AAPOS. 4(4):217-24, 2000
                                            11. Dass AB et al: Surgical results of persistent hyperplastic primary vitreous. Ophthalmology. 106(2):280-4, 1999
                                            12. Castillo M et al: Persistent hyperplastic primary vitreous involving the anterior eye. AJNR Am J Neuroradiol. 18(8):1526-8, 1997
                                            13. Pollard ZF: Persistent hyperplastic primary vitreous: diagnosis, treatment and results. Trans Am Ophthalmol Soc. 95(1):487-549, 1997
                                            14. Kaste SC et al: Persistent hyperplastic primary vitreous of the eye: imaging findings with pathologic correlation. AJR Am J Roentgenol. 162(2):437-40, 1994
                                            15. Mafee MF et al: Persistent hyperplastic primary vitreous (PHPV): role of computed tomography and magnetic resonance. Radiol Clin North Am. 25(4):683-92, 1987
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Pathology

                                                    • Clinical Issues

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Abbreviations

                                                          • Persistent hyperplastic primary vitreous (PHPV)
                                                        • Definitions

                                                          • Congenital lesion due to incomplete regression of embryonic ocular blood supply
                                                          • Persistent fetal vasculature = new term in recent literature

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Gross Pathologic & Surgical Features

                                                                        • Microscopic Features

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Demographics

                                                                              • Natural History & Prognosis

                                                                                • Treatment

                                                                                  DIAGNOSTIC CHECKLIST

                                                                                  • Consider

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Burns NS et al: Diagnostic imaging of fetal and pediatric orbital abnormalities. AJR Am J Roentgenol. 201(6):W797-808, 2013
                                                                                      2. Rizvi SW et al: Bilateral persistent hyperplastic primary vitreous: a close mimic of retinoblastoma. Semin Ophthalmol. 28(1):25-7, 2013
                                                                                      3. Brennan RC et al: US and MRI of pediatric ocular masses with histopathological correlation. Pediatr Radiol. 42(6):738-49, 2012
                                                                                      4. Khokhar S et al: Approach to cataract with persistent hyperplastic primary vitreous. J Cataract Refract Surg. 37(8):1382-5, 2011
                                                                                      5. Cerón O et al: The vitreo-retinal manifestations of persistent hyperplasic primary vitreous (PHPV) and their management. Int Ophthalmol Clin. 48(2):53-62, 2008
                                                                                      6. Katorza E et al: Prenatal ultrasonographic diagnosis of persistent hyperplastic primary vitreous. Ultrasound Obstet Gynecol. 32(2):226-8, 2008
                                                                                      7. Robinson AJ et al: MRI of the fetal eyes: morphologic and biometric assessment for abnormal development with ultrasonographic and clinicopathologic correlation. Pediatr Radiol. 38(9):971-81, 2008
                                                                                      8. Balmer A et al: Differential diagnosis of leukocoria and strabismus, first presenting signs of retinoblastoma. Clin Ophthalmol. 1(4):431-9, 2007
                                                                                      9. Schulz E et al: Long-term visual function and relative amblyopia in posterior persistent hyperplastic primary vitreous (PHPV). Strabismus. 14(2):121-5, 2006
                                                                                      10. Mackeen LD et al: High-frequency ultrasonography findings in persistent hyperplastic primary vitreous. J AAPOS. 4(4):217-24, 2000
                                                                                      11. Dass AB et al: Surgical results of persistent hyperplastic primary vitreous. Ophthalmology. 106(2):280-4, 1999
                                                                                      12. Castillo M et al: Persistent hyperplastic primary vitreous involving the anterior eye. AJNR Am J Neuroradiol. 18(8):1526-8, 1997
                                                                                      13. Pollard ZF: Persistent hyperplastic primary vitreous: diagnosis, treatment and results. Trans Am Ophthalmol Soc. 95(1):487-549, 1997
                                                                                      14. Kaste SC et al: Persistent hyperplastic primary vitreous of the eye: imaging findings with pathologic correlation. AJR Am J Roentgenol. 162(2):437-40, 1994
                                                                                      15. Mafee MF et al: Persistent hyperplastic primary vitreous (PHPV): role of computed tomography and magnetic resonance. Radiol Clin North Am. 25(4):683-92, 1987