A 4 year old boy with delayed development

 

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Doctor's Information

Name : Morteza
Family : Sanei Taheri
Affiliation :Radiology Department,Shohada Tajrish Hospital,SBMU
Academic Degree : Associate Professor of Radiology
Email : This email address is being protected from spambots. You need JavaScript enabled to view it.
Resident : Mohsen Zakavati Avval

 

Case Section

Neuroradiology

 

Patient's Information

Gender : Male
Age : 4

 

Clinical Summary

A 4 year old boy with delayed development, particularly a delay in motor skills such as crawling, walking, and coordinating movements.

 

Imaging Findings

A large cystic area occupies the posterior aspect of the posterior fossa communicating directly with the fourth ventricle. No normal vermis can be identified, with a small vermian remnant appearing displaced and rotated superiorly.

 

Differential Diagnosis


Common • Dandy-Walker Continuum
• Herniation Syndromes, Intracranial
• Chiari 1
• Chiari 2
Less Common
• Arachnoid Cyst
• Ependymoma
• Meningioma
• Metastasis
• Intracranial Hypotension
Rare but Important
• Subependymoma
• Epidermoid Cyst
• Dermoid Cyst
• Hemangioblastoma
• Neurenteric Cyst

 

Final Diagnosis

Dandy–Walker syndrome (DWS)

 

Discussion (Related Text)

Dandy–Walker syndrome (DWS), or Dandy–Walker complex, is a congenital brain malformation involving the cerebellum and the fluid filled spaces around it. A key feature of this syndrome is the partial or even complete absence of the part of the brain located between the two cerebellar hemispheres (cerebellar vermis).[1] The Dandy–Walker complex is a genetically sporadic disorder that occurs one in every 30,000 live births.[2] Prenatal diagnosis and prognosis of outcomes associated with Dandy-Walker can be difficult.[3] It is named for Walter Dandy and Arthur Earl Walker.[4] Key Differential Diagnosis Issues:
• Dandy-Walker Continuum (DWC)
» DWC a broad spectrum of cystic posterior fossa (PF) malformations.
» DW malformation: Large posterior fossa and large CSF cyst, normal 4th ventricle absent, lambdoid-torcular inversion
» DW variant: Failure of "closure" of 4th Ventricle, vermian hypoplasia
» Mega cisterna magna: Communicates freely with 4th ventricle, basal subarachnoid spaces
» 2/3 have associated C Sand/or extracranial anomalies
• Cisterna magna (CM) between medulla (anterior), occiput (posterior) (a.k.a., cerebellomedullary cistern)
» Below/behind inferior vermis
» Large medullary cistern masses may extend laterally, posteriorly into CM
• Most common adult lesions are tonsillar-associated
» Indirect (secondary effect on tonsil) > direct (lesion in tonsil)
• MR and clinical information helps DDx Helpful Clues for Common Diagnoses
• Herniation Syndromes, Intracranial
» Most often 2° to posterior fossa (PF) mass effect
» Tonsils pushed down into CM
» "Peg-like" configuration of tonsils
» Tonsil folia usually oriented horizontally ~ become vertically oriented when herniated
» 4th ventricle may obstruct, cause obstructive hydrocephalus
• Chiari 1
» Pointed cerebellar tonsils "- 5 mm below foramen magnum
» Posterior fossa (PF) usually normal size
» Age-related tonsil descent below "opisthion-basion line" common
» Treatment aim = restore normal CSF flow at foramen magnum (FM)
• Chiari 2
» Small PF ~ contents shift j.
» "Cascade" of tissue (vermis, not tonsil) herniates j. through FM
» - 100% associated myelomeningocele[5]

 

References

1.National Institute of Neurological Disorders and Stroke, "NINDS Dandy–Walker Syndrome Information Page," http://www.ninds.nih.gov/disorders/dandywalker/dandywalker.htm. Last updated September 16, 2008. Last accessed July 6, 2009.
2.Osenbach RK, Menezes AH (1992). "Diagnosis and management of the Dandy-Walker malformation: 30 years of experience.". Pediatr Neurosurg 18 (4): 179–89. PMID 1472430
3.Guibaud L, Larroque A, Ville D, Sanlaville D, Till M, Gaucherand P et al. (2012). "Prenatal diagnosis of 'isolated' Dandy-Walker malformation: imaging findings and prenatal counselling.". Prenat Diagn 32 (2): 185–93. doi:10.1002/pd.3828. PMID 22418964
4.http://www.whonamedit.com/doctor.cfm/418.html
5.Osborn, Ross,Salzman; 2009, "Expert ddx Brain and Spine"

 

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