Tc-99m ECD Brain Perfusion Single-photon Emission Computed Tomography in Parry-Romberg Syndrome.

We report a 15-year-old girl referred to our department for a brain perfusion study with a diagnosis of Parry-Romberg syndrome with left hemifacial atrophy and left enophthalmos. In the presence of a normal magnetic resonance imaging, Tc-99m ECD brain perfusion revealed left temporal lobe hypoperfusion with preserved perfusion in rest of the cortical and subcortical regions and both cerebellar hemispheres.

A 15-year-old girl with progressive left hemifacial atrophy [Figure 1a and b] was diagnosed as Parry–Romberg syndrome (PRS) and referred to our department for a brain perfusion study. She had no neurological complaints and was being evaluated for right enophthalmos and any associated intracranial abnormality. The Tc-99m ECD brain perfusion single-photon emission computed tomography (SPECT) revealed left temporal hypoperfusion [Figure 1c and d-arrow and g and Figure 2]. Her magnetic resonance imaging (MRI), on the other hand, did not reveal any significant abnormality [Figure 1e and f].

Figure 1

(a and b) a 15-year-old girl with progressive left hemifacial atrophy, (c and d) transaxial and coronal Tc-99m ECD brain perfusion images reveal left temporal hypoperfusion, while (e and f) transaxial fluid-attenuated inversion recovery magnetic resonance imaging reveals no abnormality

Figure 2

Transaxial Tc-99m ECD perfusion single-photon emission computed tomography images showing left temporal hypoperfusion

Progressive hemifacial atrophy known as PRS is a rare self-limiting degenerative disorder affecting hemifacial skin, muscles, and bone.[123] The involvement of nervous system and ophthalmic system is common while pathogenesis is yet unexplained. Neurological symptoms include headache, trigeminal neuralgia, and focal epilepsy, and MRI is an excellent method to characterize intracranial involvement.[45] Intracranial calcification, brain atrophy, and leptomeningeal enhancement are some of the MRI findings that have been reported. Though PRS is characterised by hemifacial atrophy, the presence of cerebral hemiatrophy on imaging may require it to be differentiated from Rasmussems and Sturge Weber.

Brain perfusion changes have been described in PRS on SPECT. Hyperperfusion involving ipsilateral cerebral cortex with hypoperfusion in white matter of the same hemisphere[6] has been reported in a clinically asymptomatic patient with extensive white matter abnormalities on MRI. Hypoperfusion involving the right parietooccipital region has been described in a case of PRS with epilepsy.[7] Abnormal SPECT with unrevealing MRI has also been reported in progressive hemifacial atrophy.[8] Thus, addition of Tc-99m ECD brain perfusion SPECT may help explain the functional significance of structural abnormalities and details of central nervous system involvement in PRS, hereby prognosticating these patients.

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