Sturge-Weber Syndrome Radiology: MRI, CT and the 8 C's Mnemonic

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Axial unenhanced CT and axial gradient-echo MRI of Sturge-Weber syndrome showing gyriform tram-track cortical calcification and hemispheric atrophy — Sturge-Weber syndrome. Left: axial unenhanced CT shows dense gyriform cortical and subcortical calcification in the left parieto-occipital region, the tram-track appearance, with underlying volume loss and widened sulci. Right: axial gradient-echo MRI at the same level shows the calcification as corresponding gyriform blooming hypointensity. Susceptibility-weighted imaging shows this better than CT and without radiation.

Sturge-Weber syndrome is a sporadic capillary-venous malformation syndrome of the skin, brain and eye, caused by a somatic activating mutation in GNAQ (most often p.R183Q) that arises after fertilisation, so the child is a genetic mosaic and nothing is inherited [7]. Current consensus defines it as at least two of a facial capillary malformation, a cerebral vascular malformation and ocular involvement [2] — which means a child can have Sturge-Weber syndrome with no port-wine birthmark at all.

Two things about the imaging have changed enough since the classical description to matter in daily reporting. First, the findings most textbooks list — pial enhancement, tram-track calcification, atrophy — are late, and a normal-looking scan in an infant does not exclude the diagnosis. Second, the lesion itself is not an angioma. This page covers the mnemonic, the early and late imaging findings separately, who should be scanned and when, and where the classical teaching is now wrong.

Key points

Sturge-Weber syndrome needs two of three: facial capillary malformation, cerebral vascular malformation, ocular involvement [2].
The brain lesion is a subarachnoid varicose network of dilated veins compensating for absent cortical veins. “Leptomeningeal angioma” and “pial angiomatosis” are misnomers that should be retired [1].
Pial-arachnoid enhancement is often not visible before 1 year of age; a negative early MRI does not exclude the diagnosis [1,3].
Early, non-contrast markers carry the diagnosis in infancy: choroid plexus thickness 5.6 mm or more on the affected side (about 92 percent sensitive, 100 percent specific), enlarged deep medullary veins on SWI, and asymmetric accelerated myelination [1,5].
Tram-track calcification is a late, insensitive finding. SWI beats CT for detecting it [1].
Risk of brain involvement is predicted by a birthmark involving any part of the forehead, following embryonic facial vasculature rather than the trigeminal divisions [4].
Arterial spin labelling shows hyperperfusion early and hypoperfusion late; the direction of the perfusion abnormality dates the disease [1,9].

The 8 C’s mnemonic for Sturge-Weber syndrome

Visual mnemonic showing the 8 C's of Sturge-Weber syndrome features — The 8 C’s of Sturge-Weber syndrome.

The C	What it means	How it looks
Cutaneous port-wine birthmark	Facial capillary malformation, the cutaneous hallmark	Clinical. Risk of brain involvement rises with forehead and upper-eyelid involvement [4]
Congested subarachnoid veins	The dilated venous network in the subarachnoid space, formerly miscalled pial angiomatosis	Pial-arachnoid enhancement on post-contrast T1 and post-contrast FLAIR; serpiginous flow voids on T2 [1]
Choroid plexus enlargement	Congested plexus from diverted deep venous drainage	Ipsilateral, measurable on non-contrast T2 or T1. Cut-off 5.6 mm on the affected side [5]
Cortical tram-track calcification	Gyriform cortical and subcortical calcification from chronic venous ischaemia	Parallel gyriform lines on CT and radiographs; blooming on SWI or gradient echo [1]
Cerebral atrophy	Progressive volume loss in the affected territory	Hemispheric or lobar, usually parieto-occipital, with widened sulci
Calvarial thickening	Compensatory Dyke-Davidoff-Masson type change to the shrinking hemisphere	Ipsilateral diploic thickening, elevated petrous ridge, hyperpneumatised sinuses and mastoid
Choroidal haemangioma	Diffuse choroidal vascular malformation in the eye	Diffuse choroidal thickening on ocular ultrasound, MRI or OCT; occurs in roughly 40 to 50 percent
Chronically raised intraocular pressure	Glaucoma, in 30 to 70 percent, and buphthalmos when it starts in infancy	Clinical and tonometric. Needs lifelong ophthalmology follow-up [2]

The 8 C’s of Sturge-Weber syndrome, with the imaging correlate of each.

One correction to the classical list. Older versions of this mnemonic include “cavernous sinus enlargement”. That is not a recognised Sturge-Weber feature. The venous structures that enlarge are the choroid plexus, the deep medullary veins and the subependymal veins, all of them part of the centripetal drainage that takes over when the cortical veins fail [1]. The eighth C is better spent on the choroid plexus.

Roach classification and where it falls short

Roach type	Facial capillary malformation	Brain involvement	Glaucoma
Type I (classic)	Present	Present	May be present
Type II	Present	Absent	May be present
Type III	Absent	Present (isolated)	Usually absent

The Roach scale (1992). Type III has no birthmark and is the type most often missed, or mislabelled as migraine or meningitis.

The Roach scale is still the vocabulary of most reports, but it is now regarded as oversimplified, particularly for Type II, because it treats brain involvement as a binary and ignores the fact that the risk is stratified by the size and site of the birthmark [1]. The 2025 ISSVA classification instead places the condition among the syndromic slow-flow port-wine capillary malformations. In practice, use Roach for communication and describe the phenotype in full.

Early versus late MRI findings

This is the section that changes reporting. The disease is a progressive venous congestion, so imaging findings move through a sequence, and the ones every textbook illustrates sit at the end of it. Seizures usually begin before the age of 2, which means the diagnostically useful window is the one in which the classical findings are still absent [1].

Stage	Finding	Sequence	Why it happens
Early (neonate to about 1 year)	Enlarged deep medullary veins; subarachnoid varicose network	SWI, T2	Cortical veins absent or dysplastic; flow redirected into the deep system. Reported as early as 5 to 11 days of life [1]
Early	Choroid plexus enlargement, 5.6 mm or more on the affected side	Non-contrast T1 or T2	Subependymal veins drain the plexus; once overwhelmed the plexus congests. About 92 percent sensitive, 100 percent specific [5]
Early	Accelerated myelination: asymmetric low T2 signal, high T1 signal in subcortical white matter	T1, T2	Transient hyperperfusion and hypermetabolism. Usually identifiable before 6 months [1]
Early	Hyperperfusion of the affected cortex	Arterial spin labelling	Compensatory phase, before tissue loss. Seen in younger, asymptomatic patients with little atrophy [1,9]
Established	Pial-arachnoid enhancement following sulci and cisterns	Post-contrast T1, post-contrast FLAIR	The subarachnoid varicose network. First reported as early as 35 days but frequently not visible before 1 year [1]
Late	Gyriform cortical and subcortical calcification (tram-track)	SWI, gradient echo, CT	Chronic ischaemic cortical injury. Accumulates with age [1]
Late	Cortical laminar necrosis, gliosis, lobar or hemispheric atrophy	T1, FLAIR, DWI	Irreversible injury from sustained venous congestion
Late	Hypoperfusion of the affected cortex	Arterial spin labelling	Burn-out stage; associated with extensive calcification and severe epilepsy [1]
Late	Ipsilateral calvarial thickening, sinus and mastoid hyperpneumatisation, elevated petrous ridge	CT, T1	Compensatory change to the atrophic hemisphere (Dyke-Davidoff-Masson pattern)

Sturge-Weber syndrome imaging findings by stage. The classical triad of enhancement, calcification and atrophy is the end of the sequence, not the start.

Suggested MRI protocol

3.0 T where available, with sedation planned rather than improvised. In infants a feed-and-wrap, non-contrast study is a reasonable first pass [2].
Volumetric T1 pre- and post-contrast for pial-arachnoid enhancement and for the choroid plexus.
SWI or T2* — the single highest-yield sequence. It shows the deep medullary veins, the subarachnoid varicose network and calcification, all without contrast [1].
Arterial spin labelling, acquired before gadolinium, with a tailored post-label delay. Interpret with caution close to a seizure, since ictal and post-ictal perfusion changes mimic disease [1].
Post-contrast FLAIR is more sensitive than post-contrast T1 for subtle leptomeningeal enhancement.
T2 and DWI for myelination pattern, gliosis and acute stroke-like injury.
Venous MR angiography and high-resolution orbital sequences are included in the 2025 consensus protocol [2].

Why leptomeningeal angioma is the wrong name

Radio-pathological correlation finds no angioma. What is present is a network of dilated, varicose veins in the subarachnoid space, compensating for absent or dysplastic superficial cortical drainage. The 2025 Pediatric Radiology review states the term is outdated and should be avoided, preferring subarachnoid varicose network or leptomeningeal capillary-venous malformation [1].

This is not pedantry, because the name drives the mental model. “Angioma” implies a proliferative mass lesion and invites questions about haemorrhage and resection. “Venous congestion” explains, in one mechanism, the whole imaging sequence: diverted deep venous drainage gives choroid plexus enlargement and enlarged medullary veins; early congestion gives hyperperfusion and accelerated myelination; sustained congestion gives ischaemia, calcification, laminar necrosis, atrophy and finally hypoperfusion. It also explains why these are low-flow malformations that do not need catheter angiography unless surgery is planned.

Which birthmarks to work up, and when to scan

The old teaching that the birthmark follows the V1 trigeminal division is wrong. In 192 children with a facial port-wine stain, the strongest predictor of seizures, abnormal neurodevelopment, glaucoma and abnormal MRI was involvement of any part of the forehead, its lower border a line from the outer canthus of the eye to the top of the ear, including the upper eyelid. That area crosses all three trigeminal divisions but corresponds closely to the embryonic vasculature of the face. Bilateral distribution was not independently significant [4].

Question	US consensus, 2021 [3]	Italian consensus, 2025 [2]
Which birthmark triggers workup	High-risk facial port-wine birthmark: refer to paediatric neurology and paediatric ophthalmology for baseline assessment and periodic follow-up	Frontal region bordered inferiorly by outer canthus to upper ear, including upper eyelid, plus midline frontonasal. About 81 percent of children with that distribution had the syndrome in the cited series
Screening MRI in an asymptomatic infant	Not routinely recommended. May be performed in selected cases, for example where presymptomatic treatment is being considered	Recommended, preferably after 12 months, with gadolinium. Non-contrast feed-and-wrap within 4 weeks if suspicion is high
Repeat imaging	Routine follow-up neuroimaging not recommended if symptoms are stable	If negative after 12 months and the child stays asymptomatic, wait to around age 6 to avoid repeat sedation
EEG	Not specified	Baseline sleep EEG in the first 3 months for high-risk birthmarks; 6-monthly for the first 2 years once confirmed
Ophthalmology	Baseline evaluation and periodic follow-up; intervals not defined	Complete examination at diagnosis, then annual review from birth if uncomplicated; tonometry under anaesthesia until the child cooperates; lifelong review in adults
Presymptomatic drugs	Retrospective data suggest benefit from antiseizure medication plus aspirin; no completed prospective trial	Antiseizure medication may be offered where brain involvement is extensive and early-onset epilepsy risk is high, with the empirical basis explained to the family

The two consensus statements disagree about screening MRI in asymptomatic infants. Both agree that early enhancement is unreliable.

The disagreement is real and worth stating in a report or a multidisciplinary discussion rather than papering over. It comes down to the same fact read two ways: a negative MRI before the first birthday does not exclude the diagnosis. Negative neuroimaging in infants with a high-risk birthmark who later proved to have the syndrome was reported in 3 to 23 percent of retrospective cases [3], and the leptomeningeal malformation was undetectable on the initial scan in 21 percent of children imaged at 3 months or younger [5]. The US group concludes that an unreliable test should not be done routinely; the Italian group concludes it should be delayed until it becomes reliable. What rescues the early scan is the indirect signs: a direct or at least one indirect sign on a first MRI before 3 months gave 100 percent sensitivity and 94 percent specificity for the diagnosis [6].

Ocular involvement

Glaucoma in 30 to 70 percent, with a bimodal onset — infancy, from a maldeveloped anterior chamber angle, and later childhood or adulthood, from raised episcleral venous pressure. Infantile onset produces buphthalmos, an enlarged globe visible on any brain MRI.
Diffuse choroidal haemangioma in roughly 40 to 50 percent, ipsilateral to the birthmark. On MRI it is diffuse choroidal thickening that enhances; it is the cause of the “tomato ketchup fundus” and can cause exudative retinal detachment and vision loss.
Episcleral and conjunctival vascular lesions and dilated tortuous retinal vessels.
Look for these on every brain MRI in a child with a port-wine birthmark. Globe asymmetry and choroidal thickening are easy to see and easy to skip past.

Differential diagnosis

Entity	What overlaps	What separates it
PHACE syndrome	Segmental facial vascular lesion with intracranial abnormality	The skin lesion is an infantile haemangioma, not a capillary malformation, and the intracranial findings are posterior fossa malformation, arterial anomalies, coarctation and eye anomalies
Meningioangiomatosis	Cortical and leptomeningeal lesion with gyriform calcification and seizures	Focal, often frontal or temporal, associated with neurofibromatosis type 2; no birthmark, no hemispheric atrophy pattern
Klippel-Trenaunay syndrome	Capillary malformation, sometimes PIK3CA-related	Limb capillary-lymphatic-venous malformation with overgrowth; no leptomeningeal disease
Coeliac disease with epilepsy and occipital calcification	Bilateral occipital gyriform calcification with seizures	No enhancement, no birthmark, no atrophy of the classical Sturge-Weber pattern; gluten enteropathy
Cortical laminar necrosis from hypoxic-ischaemic injury	Gyriform T1 hyperintensity and later calcification	Follows a vascular or watershed distribution with a clear insult; no leptomeningeal enhancement
Congenital infection (CMV, toxoplasmosis)	Intracranial calcification with atrophy and seizures	Periventricular or basal ganglia calcification, migrational anomaly, microcephaly; not gyriform and cortical
Dyke-Davidoff-Masson syndrome	Hemiatrophy with ipsilateral calvarial thickening and sinus hyperpneumatisation	The compensatory skull change is shared. Sturge-Weber adds enhancement, gyriform calcification and the birthmark; Dyke-Davidoff-Masson is the end-state of any early hemispheric insult

Differential diagnosis of gyriform cortical calcification and hemispheric atrophy in a child.

Management in brief

Problem	Treatment	Evidence
Seizures	Standard antiseizure medication; levetiracetam and oxcarbazepine are the most used. Drug-resistant epilepsy: focal resection, hemispherotomy or vagus nerve stimulation	Cross-sectional multicentre survey data; surgery reserved for medically refractory cases [8]
Stroke-like episodes	Low-dose aspirin, 3 to 5 mg/kg/day up to 100 mg	Offered by many but not all centres; retrospective data suggest fewer stroke-like episodes and seizures. Offered early once neurological symptoms appear in extensive disease [2,8]
Presymptomatic prevention	Antiseizure medication plus aspirin before the first seizure, in selected infants with extensive brain involvement	May delay seizure onset; hypothesis-generating retrospective data, no completed prospective trial [3,10]
Cognition	Sirolimus	Prospective open-label trial showing a signal for improved processing speed [11]
Refractory seizures	Cannabidiol	Prospective trial support [8]
Port-wine birthmark	Pulsed dye laser, starting in the first year of life, sessions 4 to 6 weeks apart	Earlier treatment gives better outcomes; sedation or general anaesthesia preferred under 1 year to avoid pain memory [2]
Glaucoma	Medical intraocular pressure control, then angle or filtration surgery; lifelong monitoring	Tonometry under anaesthesia until the child cooperates; visual field testing in adults [2]

Management of Sturge-Weber syndrome. Most of the drug evidence is retrospective or open-label.

What to put in the report

Which lobes and which hemisphere, and whether involvement is unilateral or bilateral. Bilateral disease predicts earlier seizures and worse neurodevelopment.
Pial-arachnoid enhancement: present or absent, and its extent along sulci. If the child is under 1 year and the study is negative, say explicitly that absent enhancement does not exclude the diagnosis at this age [1,3].
Choroid plexus thickness on each side, in millimetres. This is a measurement, not an impression, and 5.6 mm or more on the affected side is the published threshold [5].
Deep medullary and subependymal vein prominence on SWI, and any subarachnoid varicose network.
White matter signal: asymmetric accelerated myelination early, gliosis and volume loss later.
Calcification and its distribution, ideally described on SWI rather than requesting a CT.
Perfusion: hyper- or hypoperfused, and the caveat that a recent seizure confounds it.
Atrophy and the compensatory skull changes, with a comparison to any prior study, since progression drives surgical decisions.
The orbits: globe size asymmetry and diffuse choroidal thickening.

Tram-track sign — the sign itself, and the other conditions that produce it.
Von Hippel-Lindau syndrome — the other classic phakomatosis, with its surveillance schedule.
PHACE syndrome — the segmental facial vascular lesion that is not a capillary malformation.
Neurofibromatosis — imaging findings across types 1 and 2.
Illustrated radiology mnemonics — the full collection.

Frequently asked questions

What is Sturge-Weber syndrome?

Sturge-Weber syndrome is a sporadic neurocutaneous disorder caused by a somatic activating mutation in GNAQ, most often p.R183Q, and less commonly in GNA11 or GNB2. The mutation arises in a progenitor cell early in embryogenesis, so the patient is a mosaic and the disease is not inherited. It produces capillary-venous malformations in three territories: the skin (a facial port-wine birthmark), the brain (an abnormal vascular network in the subarachnoid space with absent or dysplastic cortical veins) and the eye (glaucoma and diffuse choroidal haemangioma). Current consensus defines the syndrome as at least two of those three features, so a child can have Sturge-Weber syndrome with no birthmark at all.

What are the imaging findings of Sturge-Weber syndrome?

The classic combination is unilateral pial-arachnoid enhancement on post-contrast T1, ipsilateral choroid plexus enlargement, cortical and subcortical gyriform calcification producing the tram-track appearance, progressive hemispheric atrophy with ipsilateral calvarial thickening and sinus hyperpneumatisation, and enlarged deep medullary and subependymal veins. That combination is late disease. In the first year of life the findings are different and quieter: an asymmetric area of accelerated myelination seen as low T2 signal in subcortical white matter, hyperperfusion on arterial spin labelling, enlarged deep medullary veins and a subarachnoid varicose network on susceptibility-weighted imaging, and choroid plexus thickening on non-contrast sequences.

What is tram-track calcification and how specific is it?

Tram-track calcification is the pair of parallel gyriform lines produced when cortical and subcortical calcification follows the walls of adjacent gyri, seen on skull radiographs and CT. It is neither early nor specific. It reflects chronic venous congestion and cortical injury, so it is typically absent in infancy and accumulates with age, which means a normal CT in a symptomatic infant excludes nothing. Similar gyriform or serpiginous calcification occurs in treated or chronic infections, coeliac disease with epilepsy and occipital calcification, mitochondrial disease, radiation injury, cortical laminar necrosis and meningioangiomatosis. Susceptibility-weighted MRI shows the same calcification with far better sensitivity than CT and without ionising radiation, so CT is rarely the right first test in a child.

Which port-wine birthmarks need a brain MRI?

Distribution matters more than the old teaching suggested. Waelchli and colleagues showed that facial port-wine stains follow embryonic facial vasculature, not the trigeminal divisions, and that the best predictor of seizures, abnormal neurodevelopment, glaucoma and abnormal MRI was involvement of any part of the forehead, defined by a line from the outer canthus of the eye to the top of the ear and including the upper eyelid. Bilateral distribution added nothing independently. A birthmark confined to the lower face or to the V2 or V3 territory alone carries a low risk. Infants with a forehead or midline frontonasal birthmark should have urgent ophthalmology review and be referred for neurology assessment and brain imaging.

When should the brain MRI be done in an infant with a port-wine birthmark?

The two published consensus statements do not agree, and it is worth knowing both. The 2021 US statement says routine screening for brain involvement is not recommended in a newborn or infant with a high-risk birthmark and no seizures or neurological symptoms, and that imaging can be performed in selected cases, particularly where presymptomatic treatment is being considered. The 2025 Italian multidisciplinary consensus recommends contrast-enhanced MRI in asymptomatic patients preferably after 12 months of age, with a non-contrast feed-and-wrap study within four weeks where suspicion is high, and no repeat before around 6 years if the first study after 12 months was negative and the child stays asymptomatic. Both agree on the underlying reason: pial-arachnoid enhancement is often not visible before the first birthday, so an early negative scan does not exclude the diagnosis.

Can the MRI be normal in Sturge-Weber syndrome?

Yes, and this is the single most important caveat in reporting these studies. Pial-arachnoid enhancement is not reliably detectable before 1 year of age. Negative neuroimaging in infants with a high-risk port-wine birthmark who later proved to have Sturge-Weber syndrome was reported in 3 to 23 percent of cases in retrospective series, and in one cohort the leptomeningeal capillary malformation could not be discerned on the initial scan in 21 percent of children imaged at 3 months or younger. The way round it is to look at the indirect signs. In a prospective series, a direct sign or at least one indirect sign on the first MRI performed before 3 months identified Sturge-Weber syndrome with 100 percent sensitivity and 94 percent specificity, and choroid plexus thickness of 5.6 mm or more on the affected side gave a sensitivity of about 92 percent with 100 percent specificity.

Why is choroid plexus enlargement seen in Sturge-Weber syndrome?

Because the superficial venous drainage fails and the blood has to go somewhere else. In an affected region the cortical veins are absent or dysplastic, so venous flow is redirected centripetally into the deep system through enlarged deep medullary veins, which converge on the subependymal veins. The subependymal veins also drain the choroid plexus, so once they are overwhelmed the choroid plexus becomes congested and enlarges. It is a downstream marker of the same haemodynamic problem that causes the cortical injury, which is why it is useful early, before enhancement or calcification appear, and why it can be measured on a non-contrast scan in a sleeping infant.

Is leptomeningeal angioma the correct term?

No. It is a long-standing misnomer and the 2025 Pediatric Radiology review argues it should be abandoned. Radio-pathological correlation shows no angioma: what is present is a network of dilated, varicose veins lying in the subarachnoid space, compensating for absent or dysplastic superficial cortical drainage. The preferred term is subarachnoid varicose network, or leptomeningeal capillary-venous malformation. The distinction is not pedantry. Calling it an angioma implies a mass-like proliferative lesion, when the pathology and every downstream imaging finding, from hyperperfusion to hypoperfusion, calcification and atrophy, follow from progressive venous congestion.

What is the Roach classification of Sturge-Weber syndrome?

The Roach scale, from 1992, divides the syndrome into three types. Type I, classic Sturge-Weber syndrome, has both facial and leptomeningeal involvement and may have glaucoma. Type II has the facial capillary malformation alone with no intracranial disease and may have glaucoma. Type III is isolated leptomeningeal involvement with no birthmark and usually no glaucoma. It remains in wide use but is now regarded as oversimplified, particularly for Type II, because it does not capture how the risk of brain and eye involvement is stratified by the size and site of the birthmark. The 2025 ISSVA classification places the condition among the syndromic slow-flow port-wine capillary malformations instead.

How is Sturge-Weber syndrome treated?

Treatment is symptom-directed and multidisciplinary. Seizures are managed with standard antiseizure medication, most often levetiracetam or oxcarbazepine, with epilepsy surgery including focal resection, hemispherotomy or vagus nerve stimulation for drug-resistant cases. Low-dose aspirin at 3 to 5 mg/kg/day, capped at 100 mg, is offered by many centres to reduce stroke-like episodes and seizure clusters, although the evidence remains retrospective. Presymptomatic antiseizure medication with aspirin may delay seizure onset and is offered selectively where brain involvement is extensive. Sirolimus and cannabidiol have been studied in prospective trials with signals for cognition and seizure frequency respectively. The birthmark is treated with pulsed dye laser starting in the first year of life, and glaucoma needs lifelong ophthalmological follow-up with medical or surgical intraocular pressure control.

References

Cerron-Vela CR, Manteghinejad A, Andronikou S. Beyond the leptomeningeal angioma: a comprehensive review of MR imaging features of Sturge-Weber syndrome, from early vascular responses to tissue necrosis. Pediatr Radiol. 2025;55(13):2704-2715. PMID 41137931
El Hachem M, Diociaiuti A, Latorre S, Ficcadenti A, Kiener A, Marinelli F, et al. Multidisciplinary, multicenter consensus for the care of patients affected with Sturge-Weber syndrome. Orphanet J Rare Dis. 2025;20(1):28. PMID 39819452
Sabeti S, Ball KL, Bhattacharya SK, Bitrian E, Blieden LS, Brandt JD, et al. Consensus statement for the management and treatment of Sturge-Weber syndrome: neurology, neuroimaging, and ophthalmology recommendations. Pediatr Neurol. 2021;121:59-66. PMID 34153815
Waelchli R, Aylett SE, Robinson K, Chong WK, Martinez AE, Kinsler VA. New vascular classification of port-wine stains: improving prediction of Sturge-Weber risk. Br J Dermatol. 2014;171(4):861-867. PMID 24976116
Catsman-Berrevoets CE, Koudijs SM, Buijze MSJ, de Laat PCJ, Pasmans SGMA, Dremmen MHG. Early MRI diagnosis of Sturge Weber syndrome type 1 in infants. Eur J Paediatr Neurol. 2022;38:66-72. PMID 35461064
Bar C, Pedespan JM, Boccara O, Garcelon N, Levy R, Grevent D, et al. Early magnetic resonance imaging to detect presymptomatic leptomeningeal angioma in children with suspected Sturge-Weber syndrome. Dev Med Child Neurol. 2020;62(2):227-233. PMID 31050360
Shirley MD, Tang H, Gallione CJ, Baugher JD, Frelin LP, Cohen B, et al. Sturge-Weber syndrome and port-wine stains caused by somatic mutation in GNAQ. N Engl J Med. 2013;368(21):1971-1979. PMID 23656586
Yeom S, Comi AM. Updates on Sturge-Weber syndrome. Stroke. 2022;53(12):3769-3779. PMID 36263782
Pouliquen G, Fillon L, Dangouloff-Ros V, Kuchenbuch M, Bar C, Chemaly N, et al. Arterial spin-labeling perfusion imaging in the early stage of Sturge-Weber syndrome. AJNR Am J Neuroradiol. 2022;43(10):1516-1522. PMID 36137664
Day AM, Hammill AM, Juhasz C, Pinto AL, Roach ES, McCulloch CE, et al. Hypothesis: presymptomatic treatment of Sturge-Weber syndrome with aspirin and antiepileptic drugs may delay seizure onset. Pediatr Neurol. 2019;90:8-12. PMID 30482419
Sebold AJ, Day AM, Ewen J, Adamek J, Byars A, Cohen B, et al. Sirolimus treatment in Sturge-Weber syndrome. Pediatr Neurol. 2021;115:29-40. PMID 33316689
Ramirez EL, Julich K. Sturge-Weber syndrome: an overview of history, genetics, clinical manifestations, and management. Semin Pediatr Neurol. 2024;51:101151. PMID 39389653

Sturge-Weber Syndrome Radiology: MRI, CT and the 8 C’s Mnemonic

Key points

The 8 C’s mnemonic for Sturge-Weber syndrome

Roach classification and where it falls short

Early versus late MRI findings

Suggested MRI protocol

Why leptomeningeal angioma is the wrong name

Which birthmarks to work up, and when to scan

Ocular involvement

Differential diagnosis

Management in brief

What to put in the report

Frequently asked questions

References

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Hepatic Cystic Lesions- Visual Mnemonics

Causes of Apical Lung Fibrosis – Visual Mnemonics

About The Author

Dr. Amar Udare, MD

2 thoughts on “Sturge-Weber Syndrome Radiology: MRI, CT and the 8 C’s Mnemonic”

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Key points

The 8 C’s mnemonic for Sturge-Weber syndrome

Roach classification and where it falls short

Early versus late MRI findings

Suggested MRI protocol

Why leptomeningeal angioma is the wrong name

Which birthmarks to work up, and when to scan

Ocular involvement

Differential diagnosis

Management in brief

What to put in the report

Related reading on RadioGyan

Frequently asked questions

References

Causes Of Cerebral Rim Enhancing Lesions – Visual Mnemonics

Hepatic Cystic Lesions- Visual Mnemonics

Causes of Apical Lung Fibrosis – Visual Mnemonics

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About The Author

Dr. Amar Udare, MD

2 thoughts on “Sturge-Weber Syndrome Radiology: MRI, CT and the 8 C’s Mnemonic”

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