Congenital infiltrating lipomatosis of face associated with ipsilateral cerebellar hypertrophy
Presentation
Left cheek hypertrophy and facial deformation. Normal neurological examination.
Patient Data
Facial MRI showing diffuse, ill defined, high T1 and T2 soft tissue infiltration of the left cheek and ipsilateral parotid gland, with loss of signal after fat suppression.
There are low signal strands within it, in keeping with a fibrous component, as well as underlying bony hypertrophy.
After gadolinium-based contrast media injection, we can see vascular enlargement with no signs of malignancy.
On the T2 weighted sequences, there is ipsilateral cerebellar hypertrophy with an abnormal pattern of foliation/fissuration.
Case Discussion
Congenital infiltrating lipomatosis of the face is a very rare congenital, nonhereditary disease manifesting with prominent unilateral facial overgrowth and deformity, first described by Slavin et al. in 1983 1.
Histopathologically, congenital infiltrating lipomatosis of the face is characterized by diffuse infiltration of nonencapsulated, mature adipocytes into the soft tissues.
Rare cases of associated brain lesions have been described in the literature, including hemimegalencephaly, band heterotopia, and hemihypertrophy of the brainstem and cerebellum 2.
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