Baby developed Respiratory Distress

Saturday, March 20, 2010
This is  a non contrast(T1 Weighted) sagital MRI of a 3days old male baby who developed apnoea and respiratory distress in the baby care unit who had prominent occiput .This condition was prediagnosed during the antenatal period.


What are the Abnormalities that can be visualized in this MRI?
What is the Diagnosis?


ANSWER

ANSWER - Dandy-Walker Syndrome




Dandy-Walker Syndrome is a rare congenital abnormality in which there is usually an absence of the foramina of Magendie and Luschka.




Occurs more frequently in females than in males.




Dandy-Walker malformation is characterized by
  1. Agenesis or hypoplasia of the cerebellar vermis
  2. Cystic dilatation of the fourth ventricle
  3. Dilatation of the third and lateral ventricles
  4. Enlargement of the posterior fossa with a high tentorium cerebelli and transverse sinuses
  5. Brainstem compression and hypoplasia
  6. Thinning and bulging of the occipital bones
  7. Approximately 70-90% of patients have hydrocephalus, which often develops postnatally


Other Causes of posterior fossa cystic malformations are Dandy-Walker variant, mega cisterna magna, and posterior fossa arachnoid cyst


Predisposing factors for Dandy-Walker malformation include gestational exposure to rubella (in the first trimester), cytomegalovirus, toxoplasmosis, warfarin, alcohol, and isotretinoin.

CLINICAL FEATURES

  • Enlarged head circumference
  • Developmental delay
  • Signs of cerebellar dysfunction
  • Signs and symptoms of hydrocephalus
  • Interference with respiratory control centers in the brainstem may cause respiratory failure
  • Seizures
  • Hearing or visual difficulties
  • Intellectual impairment has been observed in patients with agenesis of the corpus callosum

INVESTIGATIONS

Ultrasonography - routinely used during the antenatal period as a screening method, and it is particularly used for postnatal follow-up studies of hydrocephalus.
CT scan
MRI for detailed evaluation of Dandy-Walker malformation lesions

TREATMENT

Dandy Walker Syndrome is treated by excision of the cyst or with a cystoperitoneal shunt.

PROGNOSIS

Prognosis depends upon the extent of neurological impairment prior to treatment and the presence of other developmental abnormalities.
Overall mortality rates of 12-50%



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6 comments

  1. Dandy water malformation

     
  2. Anonymous Says:
  3. its dandy walker malformation!!!!

     
  4. Anonymous Says:
  5. Galen malformation

     
  6. Anonymous Says:
  7. dandy walker. (cystic dilatation of the fourth ventricle)

     
  8. Anonymous Says:
  9. dandy walker cyst

     
  10. Anonymous Says:
  11. dandy walker malformation (agenesis of the vermis, dilatation of the fourth ventricle, enlarged posterior fossa)

     

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