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Occipital, Parietal Cephalocele
Paula J. Woodward, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Definitions

                • Cephalocele: Defect in skull and dura with protrusion of intracranial structures
                • Further described by contents
                  • Encephalocele (meningoencephalocele): Cerebrospinal fluid (CSF), brain tissue, and meninges
                  • Meningocele: Meninges and CSF
                  • Atretic cephalocele: Forme fruste of cephalocele with protrusion of dura, fibrous tissue, and ectopic neuroglial rests
                  • Gliocele: Glial-lined cyst containing CSF
                • May also be categorized by location of bony defect
                • Other related terms
                  • Cranioschisis: Congenital failure of skull to close, typically accompanied by defective brain development
                  • Craniorachischisis: Congenital fissure of cranium and vertebral column

              IMAGING

              • General Features

                • Ultrasonographic Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Image Interpretation Pearls

                                      Selected References

                                      1. Da Silva SL et al: Risk factors for hydrocephalus and neurological deficit in children born with an encephalocele. J Neurosurg Pediatr. 15(4):392-8, 2015
                                      2. Kasprian GJ et al: Prenatal imaging of occipital encephaloceles. Fetal Diagn Ther. 37(3):241-8, 2015
                                      3. Jones D et al: First-trimester diagnosis of Meckel-Gruber syndrome by fetal ultrasound with molecular identification of CC2D2A mutations by next-generation sequencing. Ultrasound Obstet Gynecol. 44(6):719-21, 2014
                                      4. Sepulveda W et al: Fetal cephalocele: first-trimester sonographic spectrum in a review of 35 cases. Ultrasound Obstet Gynecol. 46(1):29-33, 2014
                                      5. Gudu W et al: Encephalocele following a periconceptional exposure to efavirenz: a case report. J Perinatol. 33(12):987-8, 2013
                                      6. Bağci S et al: Intestinal atresia, encephalocele, and cardiac malformations in infants with 47,XXX: Expansion of the phenotypic spectrum and a review of the literature. Fetal Diagn Ther. 27(2):113-7, 2010
                                      7. Kiymaz N et al: Prognostic factors in patients with occipital encephalocele. Pediatr Neurosurg. 46(1):6-11, 2010
                                      8. Baradaran N et al: Cephalocele: report of 55 cases over 8 years. Pediatr Neurosurg. 45(6):461-6, 2009
                                      9. Jalali A et al: Linkage to chromosome 2q36.1 in autosomal dominant Dandy-Walker malformation with occipital cephalocele and evidence for genetic heterogeneity. Hum Genet. 123(3):237-45, 2008
                                      10. Mittermayer C et al: Prenatal diagnosis of the Meckel-Gruber syndrome from 11th to 20th gestational week. Ultraschall Med. 25(4):275-9, 2004
                                      11. Kojima K et al: Antenatal evaluation of an encephalocele in a dizygotic twin pregnancy using fast magnetic resonance imaging. Fetal Diagn Ther. 18(5):338-41, 2003
                                      12. Haberle J et al: Cervical encephalocele in a newborn--Chiari III malformation. Case report and review of the literature. Childs Nerv Syst. 17(6):373-5, 2001
                                      13. Bannister CM et al: Can prognostic indicators be identified in a fetus with an encephalocele? Eur J Pediatr Surg. 10 Suppl 1:20-3, 2000
                                      14. Budorick NE et al: Cephalocele detection in utero: sonographic and clinical features. Ultrasound Obstet Gynecol. 5(2):77-85, 1995
                                      15. Wininger SJ et al: Syndromes identified in fetuses with prenatally diagnosed cephaloceles. Prenat Diagn. 14(9):839-43, 1994
                                      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

                                                  • Definitions

                                                    • Cephalocele: Defect in skull and dura with protrusion of intracranial structures
                                                    • Further described by contents
                                                      • Encephalocele (meningoencephalocele): Cerebrospinal fluid (CSF), brain tissue, and meninges
                                                      • Meningocele: Meninges and CSF
                                                      • Atretic cephalocele: Forme fruste of cephalocele with protrusion of dura, fibrous tissue, and ectopic neuroglial rests
                                                      • Gliocele: Glial-lined cyst containing CSF
                                                    • May also be categorized by location of bony defect
                                                    • Other related terms
                                                      • Cranioschisis: Congenital failure of skull to close, typically accompanied by defective brain development
                                                      • Craniorachischisis: Congenital fissure of cranium and vertebral column

                                                  IMAGING

                                                  • General Features

                                                    • Ultrasonographic Findings

                                                      • MR Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross Pathologic & Surgical Features

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      • Treatment

                                                                        DIAGNOSTIC CHECKLIST

                                                                        • Image Interpretation Pearls

                                                                          Selected References

                                                                          1. Da Silva SL et al: Risk factors for hydrocephalus and neurological deficit in children born with an encephalocele. J Neurosurg Pediatr. 15(4):392-8, 2015
                                                                          2. Kasprian GJ et al: Prenatal imaging of occipital encephaloceles. Fetal Diagn Ther. 37(3):241-8, 2015
                                                                          3. Jones D et al: First-trimester diagnosis of Meckel-Gruber syndrome by fetal ultrasound with molecular identification of CC2D2A mutations by next-generation sequencing. Ultrasound Obstet Gynecol. 44(6):719-21, 2014
                                                                          4. Sepulveda W et al: Fetal cephalocele: first-trimester sonographic spectrum in a review of 35 cases. Ultrasound Obstet Gynecol. 46(1):29-33, 2014
                                                                          5. Gudu W et al: Encephalocele following a periconceptional exposure to efavirenz: a case report. J Perinatol. 33(12):987-8, 2013
                                                                          6. Bağci S et al: Intestinal atresia, encephalocele, and cardiac malformations in infants with 47,XXX: Expansion of the phenotypic spectrum and a review of the literature. Fetal Diagn Ther. 27(2):113-7, 2010
                                                                          7. Kiymaz N et al: Prognostic factors in patients with occipital encephalocele. Pediatr Neurosurg. 46(1):6-11, 2010
                                                                          8. Baradaran N et al: Cephalocele: report of 55 cases over 8 years. Pediatr Neurosurg. 45(6):461-6, 2009
                                                                          9. Jalali A et al: Linkage to chromosome 2q36.1 in autosomal dominant Dandy-Walker malformation with occipital cephalocele and evidence for genetic heterogeneity. Hum Genet. 123(3):237-45, 2008
                                                                          10. Mittermayer C et al: Prenatal diagnosis of the Meckel-Gruber syndrome from 11th to 20th gestational week. Ultraschall Med. 25(4):275-9, 2004
                                                                          11. Kojima K et al: Antenatal evaluation of an encephalocele in a dizygotic twin pregnancy using fast magnetic resonance imaging. Fetal Diagn Ther. 18(5):338-41, 2003
                                                                          12. Haberle J et al: Cervical encephalocele in a newborn--Chiari III malformation. Case report and review of the literature. Childs Nerv Syst. 17(6):373-5, 2001
                                                                          13. Bannister CM et al: Can prognostic indicators be identified in a fetus with an encephalocele? Eur J Pediatr Surg. 10 Suppl 1:20-3, 2000
                                                                          14. Budorick NE et al: Cephalocele detection in utero: sonographic and clinical features. Ultrasound Obstet Gynecol. 5(2):77-85, 1995
                                                                          15. Wininger SJ et al: Syndromes identified in fetuses with prenatally diagnosed cephaloceles. Prenat Diagn. 14(9):839-43, 1994