SONOWORLD : Multicystic encephalomalacia

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Brain/Spine » Neonatal Brain

Multicystic encephalomalacia

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Author(s) :

Chaitali Shah, FRCR

Presentation A preterm neonate was born at 33 weeks of gestation to a 14 year old gravida 1, para 0, who presented with premature rupture of membranes. The neonate presented with respiratory failure. Portable cranial ultrasound was performed through the anterior fontanelle.

Caption: Coronal section of the brain

Description: Multiple thin-walled cysts are noted within the brain parenchyma bilaterally.

Caption: Coronal section through the frontal horns

Description: The numerous cysts replacing the brain parenchyma are seen again. The ventricles are mildly dilated, each measuring 5 mm at the frontal horns.

Caption: Coronal through the ventricular atria

Description: The brain parenchyma is reduced to thick septae between the cysts.

Caption: Sagittal scan through the temporal lobe

Description: The extent of the cystic lesions is again demonstrated.

Caption: Para-sagittal scan on the opposite side

Description: Slightly more brain tissue is present but the bulk of the intracranial space is occupied by the cystic lesions. Also to be noted is that there is no evidence of intracranial hemorrhage and no parenchymal calcifications.

Caption: Coronal color Doppler scan

Description: The cerebral vessels are seen traversing between the cysts. The cyst walls per se show no vascularity.

Caption: Non contrast enhanced axial CT scan

Description: This CT image demonstrates the numerous bilateral cysts. This pattern is compatible with extensive bilateral infarction and liquefaction of the brain parenchyma.

Differential Diagnosis Encephalomalacia

Final Diagnosis Severe multicystic encephalomalacia

Discussion

Multicystic encephalomalacia is a diffuse lesion involving the brain parenchyma in the perinatal period. The most common causative factor that is implicated in the pathogenesis of this condition is severe hypoxia causing ischemic insult and subsequent cerebral necrosis. Cystic encephalomalacia can also occur as a sequel to brain infections, especially herpes virus.

Role of ultrasound:
1. Diagnosis of the condition -characterized by presence of multiple, thin walled cysts replacing the brain parenchyma. There may or may not be associated ventriculomegaly or intraventricualr hemorrhage. If the encephalomalacia is secondary to intracranial infections, calcifications may be seen.
2. Assess the size of the cysts and the need for cyst shunts.
3. Monitor response to treatment.

The prognosis is usually dismal and if the infant survives, there is significant residual neurological deficit.

Case References

1. Stannard MW, Jimenez JF. Sonographic recognition of multiple cystic encephalomalacia. AJR. 1983 dec;141(6):1321-4.
2. Weidenheim KM, Bodhireddy SR, et al. Multicystic encephalopathy: review of eight cases with etiologic considerations. J Neuropathol Exp Neurol. 1995 Mar;54(2):268-75.
3. Ferrer I, Navarro C. Multicystic encephalomalacia of infancy: clinico-pathological report of 7 cases. J Neurol Sci. 1978 Sep;38(2):179-89.

Follow Up An EEG was performed which revealed diffuse decreased cerebral electric activity. The neonate was unable to maintain spontaneous respirations and died subsequently. An autopsy was declined by the parents. The cause of the multicystic encephalomalacia was postulated to be some global uterine insult.