The pediatric central skull base
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The Pediatric Central Skull Base. Gary L. Hedlund, D.O. Primary Children ’ s Medical Center Salt Lake City, Utah. The Chondrocranium. Portion of the neocranium formed by endochondral ossification 25 centers of ossification 18 sutures and/or synchondroses

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The Pediatric Central Skull Base

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The pediatric central skull base

The Pediatric Central Skull Base

Gary L. Hedlund, D.O.

Primary Children’s Medical Center

Salt Lake City, Utah


The chondrocranium

The Chondrocranium

  • Portion of the neocranium formed by endochondral ossification

  • 25 centers of ossification

  • 18 sutures and/or synchondroses

  • Anatomic variants and developmental anomalies abound


Central skull base

Central Skull Base

Jinkins JR . Atlas of Neuroradiologic Embryology, Anatomy, and Variants. 2000: LW&W


Newborn central skull base

Newborn

Newborn Central Skull Base


Sphenooccipital synchondrosis

Sphenooccipital Synchondrosis

NB

3 yrs

5 yrs


Sphenooccipital synchondrosis1

10 yrs

14 yrs

Sphenooccipital Synchondrosis


18 year old female with closed head trauma

18-year-old female with closed head trauma


Neonatal central skull base

Neonatal Central Skull Base

6 Mo

Newborn


Craniopharyngeal canal

Craniopharyngeal Canal

5 yrs


Craniopharyngeal canal1

Craniopharyngeal Canal

Larsen WJ. Human Embryology, 2ND ed. Saunders; 1997


Craniopharyngeal canal2

Craniopharyngeal Canal

Courtesy Bronwyn E. Hamilton, MD


Infant central skull base

Infant Central Skull Base


Age related t1wi marrow changes

Age Related T1WI Marrow Changes

Newborn

3 yrs

7 yrs


Sphenoid bone pneumatization

Sphenoid Bone Pneumatization

  • Follows marrow conversion

  • Begins at about 1 – 2 years

  • Reaches the sella by about 7 years

  • Mature by 15 years

  • Asymmetric pneumatization is common

  • Lateral recess pneumatization

    • Splays distance between f. rotundum & vidian canal


11 year old boy with headache and lethargy

11-year-old boy with headache and lethargy

Leukemic marrow infiltration - ALL


Three year old with nasal congestion

Three-year-old with nasal congestion


Diagnostic considerations

Diagnostic Considerations?

  • Metastasis

  • Rhabdomyosarcoma

  • LCH

  • Chordoma

  • Chondrosarcoma


Langerhans cell histiocytosis

Langerhans’ Cell Histiocytosis

  • Denditic cell proliferation

  • Skull (calvarium>orbit>skullbase)

    • Mandible> ribs> femur> pelvis> spine

  • Imaging

    • Punched-out, beveled, lack of sclerosis

    • Sequestration +/-

    • T1 hyper - isointensity ~ lipid laiden histiocytes

    • T2 signal variable


Metastatic disease

Metastatic Disease

  • Leukemia

  • Neuroblastoma

  • Sarcomas

    • Rhabdomyosarcoma

    • Soft part sarcoma

    • Clear cell sarcoma


Seven year old female with headache and nasal congestion

Seven-year-old female with headache and nasal congestion


Rhabdomyosarcoma

Rhabdomyosarcoma

  • Most common childhood soft tissue sarcoma

  • More common in African American children

  • H&N involvement in 50%

    • Orbit

    • Parameningeal

      • Nasal cavity, NP, sinuses, parapharyngeal, masticator, pterygopalatine fossa, middle ear

    • Other

      • Cervical nonparameningeal


Imaging of rhabdomyosarcoma

Imaging of Rhabdomyosarcoma

  • CT

    • Bony lysis

    • ST attenuation

  • MR

    T1 hypo to isointense

    T2 hyperintense

    Variable enhancement


Nonrhabdomyosarcoma soft tissue sarcomas nrstss

NonrhabdomyosarcomaSoft Tissue Sarcomas (NRSTSs)

  • Fibrosarcoma

  • Primitive neuroectodermal tumor (PNET)

  • Malignant peripheral nerve sheath tumor

  • Ewing sarcoma

  • Synovial sarcoma


Six year old male with chronic headaches

Six-year-old male with chronic headaches


Clival chordoma

Clival Chordoma

  • Primitive notocord remnant

  • Location

    • 35% skull base

    • 50% sacrococcygeal

    • 15% vertebral body


Clival chordoma1

Clival Chordoma

  • T1WI

    • Intermediate to low signal

    • Focal hemorrhage

  • T2WI

    • High signal intensity

    • Heterogeneous

  • T1 C+

    • Honeycomb enhancement

Hemorrhage ~ 30%


Chordoma and tsc

Chordoma and TSC

  • TSC is a harmatoneoplastic syndrome

  • Autosomal dominant trait

  • Nonsense mutations in TSC1 (9q34)

    • Found in:

      • Symptomatic father

      • Clival chordoma of the son

Borgel J et al. Eur J Pediatr (2001) 160:138


Fourteen year old female with frontal headaches

Fourteen-year-old female with frontal headaches


The pediatric central skull base

Neurenteric Cyst

Kapoor V et al AJNR 23: 476-479, March 2002


Paraclival neurenteric cyst

Paraclival Neurenteric Cyst

  • Dysgenesis of notocord & neurenteric canal. Similar to Rathke cleft and colloid cysts

  • Most involve

    • Craniovertebral junction and posterior fossa

  • Histopathiology

    • Type A, resemble respiratory or GI epithelium

    • Type B, smooth muscle, glandular, and lymphoid

    • Type C, like Type B + glial elements


Summary

Summary

  • Review age related ossification and maturation

  • Identify anatomic variants

  • Review anomalies of development

  • Highlight pseudolesions and tumefactions of the central skull base


Thank you

Thank you

[email protected]


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