Imaging findings of Sturge-Weber syndrome

Sturge-Weber syndrome (SWS), also known as craniofacial hemangioma, is a rare congenital neurocutaneous syndrome, first reported by Sturge in 1879, Weber described its typical intracranial calcification manifestations in 1929, so it is called Sturge-Weber syndrome, mainly characterized by skin vascular nevi and ipsilateral leptomeningeal hemangioma in the distribution area of the facial trigeminal nerve, It is characterized by irregular vascular nevus in the distribution of the trigeminal nerve of one face, contralateral hemiplegia, hemiplegia, ipsilateral intracranial calcification, glaucoma, seizures, and mental decline
.
The disease is a congenital disease, the cause is not fully understood, it is generally believed that the embryo 4-8 weeks of the original blood vessel development abnormalities
.

Pathological changes

The pathological changes of SWS are leptomeningeal hemangiomas covering the cerebral cortex, often involving the occipital and parietal lobes, thickening of the pia mater, often composed of dilated and tortuous thin-walled veins, dilated and tortuous venous walls often thickened, calcification, blood stasis and thrombosis
can be seen.

Clinical manifestations

1.
Facial hemangioma: mostly unilateral, hemangiomas in the brain are generally on the same side as facial hemangiomas, if facial hemangiomas are distributed in the trigeminal nerve innervation area, especially the eyelids, the patient's chance of suffering from Sturge-Weber syndrome is increased, if it is bilateral facial hemangiomas, the probability of suffering from Sturge-Weber syndrome is higher

2.
Seizures: is the main clinical manifestation of SWS, patients with bilateral cerebral hemisphere involvement have a high incidence of epilepsy, typical epilepsy is focal seizures or secondary generalized seizures

3.
Mental decline: intelligence can range from mild learning difficulties to severe intellectual disabilities

4.
Glaucoma

5.
Stroke-like seizures
.

Classification of Sturge-Weber syndrome

Sturge-Weber syndrome is divided into 3 types:

Type I is facial and pimater hemangiomas, which may have glaucoma, epilepsy and intracranial hemangioma imaging typical manifestations, that is, classic Sturge-Weber syndrome

Type II is a facial hemangioma, with glaucoma, and no intracranial lesions are found

Type III is only leptomeningeal hemangioma, no facial hemangioma, and often no glaucoma
.

Radiographic changes

CT shows tortuous ceral-like calcifications under the cerebral cortex of the temporoparietal occipital region; Cerebral cortical atrophy, usually occurring on the ipsilateral side of facial hemangiomas, is mostly located in the parietal or frontoparietal lobes
.
Enhanced scan may show pia mater and choroid plexus lesions
.
MRI can clearly show the extent of the hemangioma, particularly MRI with enhanced T1-weighted images showing pia mater hemangioma and associated vascular abnormalities and hypertrophic choroid plexus, and T2-weighted images showing cerebral cortical atrophy, white matter area involvement, and glial hyperplasia
.

differential diagnosis

SWS needs to be differentiated
from calcification after treatment of hypoxic-ischemic encephalopathy, meningeal calcifications caused by meningitis, tuberous sclerosis, Fahr disease, hyperparathyroidism, oligodendroglioma, meningioma calcification, and post-traumatic hematoma calcification.

Diagnosis and treatment of Sturge-Weber syndrome

The disease can be diagnosed
according to typical clinical manifestations (facial purple-red hemangioma, epilepsy, mental retardation, glaucoma, hemiplegia, etc.
) and characteristic imaging findings.
Treatment is mainly symptomatic treatment, such as treatment of glaucoma, control of stroke-like seizures, migraine, anti-epileptic treatment, etc
.

SWS patients

Male 16 years old, seizures, cutaneous hemangioma
of the left face.
CT shows patchy calcifications of the cerebral cortex of the left parietal occipital lobe
.

Female 52 years old
.
The right side of the head is tight, dizzy, tinnitus for half a month
.
Plain CT scan shows calcification of the right parietal lobe, and contrast scan shows increased tortuous venous angiography
.