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688 IMAGING Radiography Findings n Well-demarcated mass, with round or elliptical, smooth margins. Utility n Abnormality often first detected on the radiograph n Radiographic findings similar to those of neurogenic tumor CT Findings n Continuity between cerebrospinal fluid in thecal sac and meningocele n Kyphoscoliosis and meningocele usually at apex of curvature on its convex side n Enlargement of intervertebral foramen n Associated vertebral and rib anomalies common Utility n CT usually diagnostic MRI Findings n Continuity between cerebrospinal fluid in thecal sac and meningocele Utility n Diagnostic method of choice; provides best visualiza- tion of spinal cord, meninges, and adjacent structures CLINICAL PRESENTATION n Pain and neurologic symptoms DIFFERENTIAL DIAGNOSIS n Nerve sheath tumors (neurilemoma, neurofibroma, and malignant nerve sheath tumor) n Sympathetic ganglia tumors (ganglioneuromas, gan- glioneuroblastomas, and neuroblastomas) PATHOLOGY n Meningocele and meningomyelocele are rare anoma- lies that consist of herniation of leptomeninges through an intervertebral foramen. n Meningocele contains cerebrospinal fluid only, whereas meningomyelocele also contains neural tis- sue. n Abnormalities occur slightly more often on right side than on the left and are situated anywhere between thoracic inlet and diaphragm. INCIDENCE/PREVALENCE AND EPIDEMIOLOGY n Approximately 75% of patients present between ages of 30-60 years. n Sixty percent to 80% of patients have neurofibromatosis. Suggested Readings Cabooter M, Bogaerts Y, Javaheri S, et al: Intrathoracic meningnocele. Eur J Respir Dis 63:347-350, 1982. Glazer HS, Siegel MJ, Sagel SS: Low-attenuation mediastinal masses on CT. AJR Am J Roentgenol 152:1173-1177, 1989. Rainov NG, Heidecke V, Burkert W: Thoracic and lumbar meningo- cele in neurofibromatosis type 1: Report of two cases and review of the literature. Neurosurg Rev 18:127-134, 1995. Strollo DC, Rosado-de-Christenson ML, Jett JR: Primary mediastinal tumors: II. Tumors of the middle and posterior mediastinum. Chest 112:1344-1357, 1997. Meningocele or Meningomyelocele DEFINITION: Meningocele and meningomyelocele are rare anomalies that consist of herniation of the leptomeninges through an intervertebral foramen. DIAGNOSTIC PEARLS n Continuity between the cerebrospinal fluid in the thecal sac and the meningocele n Enlargement of the intervertebral foramen n Meningocele: contains cerebrospinal fluid only n Meningomyelocele: contains neural tissue n Majority of patients have neurofibromatosis WHAT THE REFERRING PHYSICIAN NEEDS TO KNOW n Association with vertebral and rib anomalies is common and should suggest the diagnosis. n Majority of patients have neurofibromatosis. n MRI is the diagnostic method of choice.

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  • Meningocele or Meningomyelocele688

    IMAGINGRadiographyFindingsn Well-demarcated mass, with round or elliptical, smooth margins.

    Utilityn Abnormality often first detected on the radiographn Radiographic findings similar to those of neurogenic tumor

    CTFindingsn Continuity between cerebrospinal fluid in thecal sac and meningocele

    n Kyphoscoliosis and meningocele usually at apex of curvature on its convex side

    n Enlargement of intervertebral foramenn Associated vertebral and rib anomalies commonUtilityn CT usually diagnostic

    MRIFindingsn Continuity between cerebrospinal fluid in thecal sac and meningocele

    Utilityn Diagnostic method of choice; provides best visualiza-tion of spinal cord, meninges, and adjacent structures

    CLINICAL PRESENTATIONn Pain and neurologic symptoms

    DIFFERENTIAL DIAGNOSISn Nerve sheath tumors (neurilemoma, neurofibroma, and malignant nerve sheath tumor)

    n Sympathetic ganglia tumors (ganglioneuromas, gan-glioneuroblastomas, and neuroblastomas)

    PATHOLOGYn Meningocele and meningomyelocele are rare anoma-lies that consist of herniation of leptomeninges through an intervertebral foramen.

    n Meningocele contains cerebrospinal fluid only, whereas meningomyelocele also contains neural tis-sue.

    n Abnormalities occur slightly more often on right side than on the left and are situated anywhere between thoracic inlet and diaphragm.

    INCIDENCE/PREVALENCE AND EPIDEMIOLOGYn Approximately 75% of patients present between ages of 30-60 years.

    n Sixty percent to 80% of patients have neurofibromatosis.

    Suggested ReadingsCabooter M, Bogaerts Y, Javaheri S, et al: Intrathoracic meningnocele.

    Eur J Respir Dis 63:347-350, 1982.Glazer HS, Siegel MJ, Sagel SS: Low-attenuation mediastinal masses

    on CT. AJR Am J Roentgenol 152:1173-1177, 1989.Rainov NG, Heidecke V, Burkert W: Thoracic and lumbar meningo-

    cele in neurofibromatosis type 1: Report of two cases and review of the literature. Neurosurg Rev 18:127-134, 1995.

    Strollo DC, Rosado-de-Christenson ML, Jett JR: Primary mediastinal tumors: II. Tumors of the middle and posterior mediastinum. Chest 112:1344-1357, 1997.

    DEFINITION: Meningocele and meningomyelocele are rare anomalies that consist of herniation of the leptomeninges through an intervertebral foramen.

    DIAGNOSTIC PEARLSn Continuity between the cerebrospinal fluid in the thecal sac and the meningocele

    n Enlargement of the intervertebral foramenn Meningocele: contains cerebrospinal fluid onlyn Meningomyelocele: contains neural tissuen Majority of patients have neurofibromatosis

    WHAT THE REFERRING PHYSICIAN NEEDS TO KNOW n Association with vertebral and rib anomalies is common and should suggest the diagnosis. n Majority of patients have neurofibromatosis. n MRI is the diagnostic method of choice.

  • Meningocele or Meningomyelocele 689PARAVERTEBRAL MASSESFigure 3. Meningocele in a 29-year-old man. CT scan demonstrates characteristic fluid attenuation of meningocele, which communicates with the thecal sac (arrows).

    Figure 1. Meningocele in a 29-year-old man. Posteroanterior chest radiograph shows paraspinal mass (arrows) at level of T10.

    Figure 2. Meningocele in a 29-year-old man. Lateral chest radiograph shows paraspinal mass (arrows) at level of T10.

    Chapter 276 - Meningocele or MeningomyeloceleIMAGINGRadiographyFindingsUtility

    CTFindingsUtility

    MRIFindingsUtility

    CLINICAL PRESENTATIONDIFFERENTIAL DIAGNOSISPATHOLOGYINCIDENCE/PREVALENCE AND EPIDEMIOLOGYSuggested Readings