Published online June 1, 2006
PEDIATRICS Vol. 117 No. 6 June 2006, pp. 2119-2125 (doi:10.1542/peds.2005-1815)
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Cerebral Perfusion Abnormalities in Children With Sturge-Weber Syndrome Shown by Dynamic Contrast Bolus Magnetic Resonance Perfusion Imaging

Amlyn L. Evans, FRCRa, Elysa Widjaja, FRCRb, Daniel J. A. Connolly, FRCRa,c and Paul D. Griffiths, PhDb,c

a Department of Radiology, Royal Hallamshire Hospital, Sheffield, United Kingdom
b Unit of Academic Radiology, University of Sheffield, Sheffield, United Kingdom
c Department of Radiology, Sheffield Children's Hospital, Sheffield, United Kingdom


   ABSTRACT
TOP
 ABSTRACT
METHODS
 RESULTS
 DISCUSSION
 CONCLUSIONS
 REFERENCES
 
OBJECTIVE. Sturge-Weber syndrome is characterized by leptomeningeal angiomatosis and a facial naevus that is usually unilateral. Magnetic resonance imaging is the cornerstone of confirming the disease and judging the extent of the abnormalities. It has been shown, however, that brain perfusion abnormalities on nuclear medicine imaging often are more extensive than the abnormal leptomeningeal enhancement on magnetic resonance. In this article, we assess the utility of magnetic resonance perfusion in demonstrating perfusion abnormalities in pediatric cases of Sturge-Weber syndrome.

METHODS. Magnetic resonance perfusion studies were performed on 7 consecutive children who presented to our department with clinically suspected Sturge-Weber syndrome. The extent of time to peak abnormality on dynamic gadolinium bolus magnetic resonance perfusion imaging was compared with the extent of leptomeningeal enhancement and the presence of venous abnormalities.

RESULTS. Good magnetic resonance perfusion data were obtained in all 7 cases. Perfusion abnormalities were closely anatomically related to meningeal enhancement on postcontrast T1-weighted imaging. However, perfusion abnormalities were found consistently in the vicinity of developmental venous anomalies that were present in 4 of 7 cases. In 1 child, there was a perfusion deficit in the cerebellar lobe contralateral to the leptomeningeal angiomatosis, consistent with crossed cerebellar diaschisis.

CONCLUSIONS. Magnetic resonance perfusion is a sensitive indicator of perfusion abnormalities in Sturge-Weber syndrome and can be performed easily at the same time as the diagnostic scan. Magnetic resonance perfusion imaging therefore is useful in the assessment of this disease. This approach has the extra advantage of correlating the perfusion abnormalities with the high-resolution imaging that is provided from magnetic resonance imaging.

Key Words: cerebral blood flow • phakomatoses • magnetic resonance

Abbreviations: SWS—Sturge-Weber syndrome • FLAIR—fluid-attenuated inversion recovery • SPECT—single photon emission computed tomography • MR—magnetic resonance • FmMTT—first mean transit time

Sturge-Weber syndrome (SWS), otherwise known as encephalotrigeminal angiomatosis or encephalofacial angiomatosis, is 1 of the spectrum of diseases that are classified under the phakomatoses.1 It was probably first described by Schirmer in 18602; however, Sturge in 1879 is credited with the eponymous syndrome, having given a clinical account of the classic syndrome and postulated that an intracranial angiomatous malformation was responsible for the neurologic symptoms.3 Weber is credited with the first radiologic report of intracranial calcification on a plain skull radiograph.1 Although the syndrome is congenital, there is generally no heritability, although a few familial cases are described.1

The syndrome is characterized by a facial cutaneous naevus (the so-called naevus flammeus or port-wine stain) that is associated with neurologic symptoms such as focal seizures and/or hemiplegia that affects the opposite side of the body to the naevus. Hemianopsia and intellectual impairment also are common.4, 5 The cutaneous naevus is usually found in the territory of the trigeminal nerve, in particular the first division, and there also may be associated orbital abnormalities that consist of buphthalmos (congenitally enlarged globe), choroidal hemangioma, and glaucoma. The pathologic changes usually are unilateral, and although bilateral cutaneous involvement may be seen in 32.5% of cases, bilateral intracranial involvement is seen in only 7.5% of cases.1 Patients with bilateral intracranial disease usually are more severely affected with intractable seizures; therefore, the diagnosis of unexpected bilateral SWS is clinically important. It usually is a neurologically progressive disorder, ultimately associated with profound neurologic decline, the mechanism of which is incompletely understood.6

Findings on brain computed tomography and MRI reflect the underlying pathology. Superficial calcification,7 focal parenchymal atrophy, and the leptomeningeal enhancement are widely recognized, common findings.8 Leptomeningeal enhancement commonly is used to judge the extent of the disease process in SWS, and this has previously been assessed on MRI using T1-weighted imaging after gadolinium chelates have been injected.9 The T1 enhancement is thought to represent leakage of contrast medium into the interstitium between the leptomeningeal vessels and into the first cortical layer, which frequently undergoes astrogliotic scarring secondary to chronic hypoxia and results in breakdown of the blood–brain barrier. It has been reported that there is a diminishing amount of superficial enhancement over time, whereas the deep veins become more prominent. This is thought to be attributable to chronic progressive thrombosis, which is supported by post mortem studies that show thickened, fibrotic veins as a result of chronic venous hypertension.10, 11 Alternatively, leptomeningeal enhancement might represent slow flow throughout the abnormal, tortuous pial vasculature. Transient enhancement has been reported12 and is thought to be attributable to the reversibility of the venous hypertension. Seizures may be a causative factor in the venous hypertension or may be a consequence of the tissue hypoxia. As pial damage accumulates, the leptomeningeal enhancement would be expected to be permanent.

MRI, in particular postcontrast T1 or fluid-attenuated inversion recovery (FLAIR) imaging, is currently the cornerstone of diagnosis in SWS because of its ability to show abnormal leptomeningeal enhancement.9 Perfusion abnormalities also are seen on nuclear medicine imaging and are reported to be related to the leptomeningeal angiomatosis. In this article, we compare the extent of perfusion abnormalities in children with SWS against the extent of leptomeningeal disease that is detected on postcontrast MRI and with other abnormalities that are shown on MRI. Perfusion status is currently assessed using nuclear medicine studies, such as single photon emission computed tomography (SPECT) or positron emission tomography, which use relatively high doses of ionizing radiation.13–15 It is possible that MRI may be able to replace these methods.


   METHODS
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 ABSTRACT
 METHODS
RESULTS
 DISCUSSION
 CONCLUSIONS
 REFERENCES
 
Seven children who were referred for clinical MRI investigations because of high clinical suspicions of SWS were included; all had facial nevi. These were consecutive referrals during a 4-year period (from September 2000 to October 2004). The routine MRI assessment of children with SWS includes axial fast spin echo dual echo, sagittal and axial spin echo T1-weighted imaging, diffusion-weighted imaging, and time-of-flight magnetic resonance (MR) venography before the injection of gadolinium chelate. Dynamic contrast-enhanced perfusion imaging (described below) then is performed, and axial spin echo T1-weighted and fast spin echo T1-weighted FLAIR images are taken on a 1.5-T MR scanner (Infinion; Philips Medical Systems, Cleveland, OH). Dedicated orbital imaging was performed when ocular abnormalities were suspected on routine imaging. The dynamic bolus MRI perfusion scan used a gradient echo T2* -weighted echo planar imaging sequence with the following parameters: time to repeat 1400 ms, echo time 60 ms, flip angle 90 degrees, thickness 7 mm, field of view 24 cm, matrix 216 x 216 (Resolution-Aided Matrix factor 2), echo train length 108, and fat saturation, with images taken every 1.4 seconds after an intravenous bolus of gadolinium with a dose of 0.2 mL/kg body weight given as a 1.0 molar chelate (Gadovist; Schering, Berlin, Germany). The perfusion data were analyzed using the proprietary software making assessments of first mean transit time (FmMTT) and relative cerebral blood volume. Perfusion abnormalities were considered to be present when the FmMTT was increased by >1.5 seconds in a region when compared with the opposite side or with adjacent brain tissue in cases of bilateral abnormalities.


   RESULTS
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 ABSTRACT
 METHODS
 RESULTS
DISCUSSION
 CONCLUSIONS
 REFERENCES
 
The 7 children (4 girls, 3 boys) had an age range of 15 years (2 months to 15 years; mean: 5.5; SD: 6.3). All 7 patients had neuroimaging or ocular changes that were consistent with SWS, and the results are summarized in Table 1. One patient did not have intracranial abnormalities on MRI (patient 3) but had a diffuse choroidal angioma with choroidal detachment and naevus flammeus sufficient to confirm the diagnosis of SWS. This patient had no abnormalities on FmMTT or relative cerebral blood volume.


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  		TABLE 1 Details of the Imaging Findings in Patients With SWS

 
Of the 6 children with intracranial disease, 4 had unilateral disease and 2 had bilateral involvement, defined by the presence of bilateral leptomeningeal enhancement (eg, Fig 1). In both of these cases, the bilateral nature was not suspected clinically; a small contralateral naevus flammeus was recognized retrospectively in 1 case, and the cutaneous lesion was truly unilateral in the other. Four patients had venous anomalies (2 bilateral [eg, Fig 2], 1 of which had unilateral leptomeningeal enhancement only). The venous anomalies consisted of developmental venous anomalies and a radial transmedullary vein.


Figure 1
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  		FIGURE 1 Images from an 11-month-old with SWS. A, Axial T2-weighted images showing cerebral atrophy in the right posterior frontal and parietal regions (arrow). B and C, Axial FLAIR images after intravenous gadolinium chelate showing areas of leptomeningeal enhancement consistent with leptomeningeal angiomatosis in the right posterior frontal and parietal regions (C; arrow) and left medial parietal region (B; arrowhead). D, Axial T1-weighted image after intravenous gadolinium chelate at the same level as in B showing the reduced conspicuity of the leptomeningeal enhancement as compared with postcontrast T1-weighted FLAIR image (arrowhead). E and F, Axial FmMTT images from a dynamic contrast bolus MR perfusion study that was taken through the cerebrum and shows pronounced perfusion defect in the right parietal and frontal lobes and left occipital region areas comparable to the areas of leptomeningeal enhancement (B and C). Perfusion deficit: red, high; green, mid; blue, low.

 

Figure 2
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  		FIGURE 2 An 11-year-old child with SWS. A, Axial T2-weighted images showing developmental venous anomalies in the right frontal region and left basal ganglia. B, MR venogram showing developmental venous anomalies in right frontal region and the left basal ganglia. C and D, Axial FLAIR images after intravenous gadolinium chelate showing areas of leptomeningeal enhancement in the left parieto-occipital region and the right high frontal region. E and F, Axial regional cerebral blood volume (E) and FmMTT (F) images from a dynamic contrast bolus MR perfusion study showing the profound perfusion defect in the areas of enhancement shown in C and D and also perfusion defects along the course of the developmental venous anomalies.

 
The regions of the brain that were affected by leptomeningeal enhancement showed the most extensive FmMTT changes, and the area of abnormality broadly matched the extent of leptomeningeal enhancement in 5 of 6 cases. There was more extensive change than expected in 1 case (patient 4). However, the areas around venous anomalies also showed abnormal FmMTT in all cases in which they were present. This was less extensive than seen in the areas of leptomeningeal enhancement. FmMTT changes were seen bilaterally in 1 case, in which there was only unilateral leptomeningeal enhancement (patient 2) and the contralateral FmMTT change was along the course of a developmental venous anomaly. One patient showed increased FmMTT in the cerebellar hemisphere contralateral to the affected hemisphere. This was interpreted as showing crossed cerebellar diaschisis (patient 6; Fig 3).


Figure 3
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  		FIGURE 3 MRI scans of a 4-month-old infant with SWS. A and B, Axial FLAIR images after intravenous gadolinium chelate showing avid leptomeningeal enhancement in the temporal, parietal, and occipital regions. B shows in addition the rare finding of midbrain leptomeningeal enhancement. C, Axial T1 fat-saturated image after administration of intravenous gadolinium chelate showing enlargement of the left orbit (buphthalmos) and avid choroidal enhancement consistent with choroidal angioma (arrow). D, Axial apparent diffusion coefficient map from a diffusion-weighted sequence showing marked low diffusion coefficient in the white matter consistent with increased deoxyhemoglobin levels. E and F, FmMTT images from a dynamic contrast bolus MR perfusion study that was taken through the cerebrum (E) and cerebellum (F) and shows pronounced perfusion defect in the left cerebral hemisphere and a posterior perfusion defect in the right cerebellar hemisphere consistent with crossed cerebellar diaschisis secondary to the left cerebral changes (arrowhead).

 

   DISCUSSION
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 ABSTRACT
 METHODS
 RESULTS
 DISCUSSION
CONCLUSIONS
 REFERENCES
 
Most children with SWS have normal neurologic function for several months or even years after birth. Visual, motor, or developmental delay may be the first presentation of neurologic dysfunction, usually on 1 side of the body and frequently associated with seizures. The seizure activity was previously thought to be the major cause of progressive decline in children with SWS; however, in recent literature, it has been suggested that it is more likely to be attributable to progressive venous ischemia.

It is likely that the abnormalities that give rise to SWS are produced early in fetal development and account for the high frequency of association between unilateral involvement of facial ectoderm, globe, and occipital cortex of the brain. Facial and pial vascular anomalies are thought to result from persistence of primordial sinusoidal vascular channels that normally are present only from the fourth to the eighth gestational weeks. During that period, the ectoderm that is destined to become the skin of the upper face and the eye overlies the part of the dorsal neural tube that is destined to form the occipital lobes and adjacent structures of the cerebral hemispheres. How the cerebral angioma and facial naevus are formed from the persistence of the primordial structures is not clear, but superficial venous aplasia or early thrombotic occlusion may be the cause.16, 17 The leptomeningeal angiomatosis probably results from the abnormal persistence of an embryologic venous plexus as a result of venous blood being redirected through the developing leptomeninges. This mechanism also may explain the range of venous abnormalities seen in SWS (4 of 7 in the present study), such as persistent transmedullary radial veins, developmental venous anomalies, or dural fistulas. Other abnormalities consist of enlargement and calcification of the choroid plexus on the same side as the affected hemisphere.18

Venous abnormalities are probably attributable to aplasia of superficial cortical veins or venous obliteration early in the postconception period. The effect of absence of a functional superficial venous system is likely to be progressive, and, as the redirected venous pathways fail, ultimately this causes severe tissue hypoxia by venous hypertension. This is proposed as a possible explanation for the saltatory neurologic decline. Prolonged seizures are thought to cause injury by imposing an increased metabolic demand that cannot be met by the inadequate perfusion as a result of focal venous hypertension, and this can lead to venous infarction. Patients with SWS therefore are particularly susceptible to brain injury as a consequence of status epilepticus and require optimal seizure control.19 As the ischemic injury progresses, the cortex is reduced to a nonfunctioning calcified mantle, and the overlying leptomeningeal vessels regress, with capillaries no longer being perfused as a result of thrombosis; for this reason, many clinicians advocate the use of antiplatelet medication. The leptomeningeal angiomatosis is thought to result from the diversion of blood from the superficial cortex into the developing meninges in the first trimester.

Many authorities have described low-signal regions on T2-weighted images in parts of the brain that are affected by SWS, not associated with calcification. One possible explanation put forward for this observation is advanced or hypermyelination. This theory is not widely held, and the findings of case 6 in our study do not support hypermyelination. The child who is presented as case 6 was 5 months of age at the time of the MRI, an age when T1-weighted images can be used to assess myelination accurately. There was no evidence of advanced myelination in the affected area; in fact, myelination was shown to be delayed by the lack of high signal in the affected white matter. This is not surprising because many pathologies that produce hypoxia cause delay in myelination, such as birth asphyxia. In that case, we showed direct evidence of perfusion deficit to the area (Fig 3E), and the low signal on the apparent diffusion coefficient map of the diffusion-weighted imaging is likely to be attributable to high levels of deoxyhemoglobin.

There are several descriptions of the brain vasculature from both the older catheter-based literature and the more recent MRI literature.20, 21 The arterial circulation usually is not involved in SWS, but venous problems commonly are encountered. Transmedullary radial veins and developmental venous anomalies are readily visible on MR angiographic studies,22 but high-quality catheter angiography may be required to show fistulas. Delays in hemispheric transit times can be shown using high-temporal-resolution angiographic studies indicative of venous hypertension that is sufficient to reduce cerebral blood flow through the affected area. Various newer MRI techniques also have been used to identify the early venous anomalies, including bold oxygen level-dependent MR venography (a high-resolution, 3-dimensional, T2-weighted gradient echo sequence) that can visualize veins of <0.5 mm in diameter.23

Brain perfusion has been studied extensively using nuclear medicine scans such as 99mTc-hexamethylprophylene amine oxime SPECT,13, 14, 24–27 99mTc-pertechnetate, N-isopropyl-p [123I] iodoamphetamine SPECT,28 and 2-deoxy-2 [18F] fluoro-deoxyglucose positron emission tomography.15, 26, 29–31 Perfusion changes that are shown by these modalities have been reported to be present before the other structural abnormalities are demonstrable by neuroimaging, which suggests that "functional" studies may be more sensitive for early disease.25, 26 These perfusion changes also have been shown to track the neurologic decline.15, 32 We did not have the opportunity to compare our MR perfusion findings with nuclear medicine perfusion studies.

In our study of 7 patients with clinically suspected SWS, we have established that MR perfusion deficits are seen to be matched in relation to the areas of enhancement that correspond to leptomeningeal disease. In 1 case (patient 4), the area of perfusion abnormality was seen to be more extensive than the area of enhancement, suggesting that there may be early venous thrombosis that is as yet undetectable by postcontrast imaging. Perfusion deficits, as discussed above, have been demonstrated previously to be present earlier than other structural abnormalities. This study also may suggest that MR perfusion is more sensitive than the conventional postcontrast imaging in the detection of pathology.

There is a previously described case of crossed cerebellar diaschisis in SWS31 secondary to cerebral atrophic changes. We also present a case of crossed cerebellar diaschisis in which there is a perfusion deficit in the posterior right cerebellar hemisphere (patient 6) secondary to the extensive left cerebral changes (Fig 3). No changes were identified on conventional T2-weighted imaging; therefore, it is proposed that these changes are as a functional consequence of the supratentorial disease.

There was previously a single case report of MR perfusion in SWS that showed comparable changes to that shown on the T1 postcontrast scan.33 That report and the cases presented here show the advantages of using an integrated multimodality MR-based approach when imaging children with SWS. This takes advantage of combining "functional studies" directly with high-resolution imaging of the brain and vascular compartments.

In our experience, MR perfusion has been shown to be effective and easy to perform at the same time as the patients' diagnostic MRI. Our results show that, by and large, there is a good anatomic correlation between the extent of leptomeningeal disease and the extent of perfusion abnormalities. This is not perfect, and sometimes the lobar extent of perfusion defects is greater than the leptomeningeal enhancement. The more important finding of this study, however, was perfusion defects distant from the leptomeningeal disease both ipsilateral and contralateral. In all of our cases, when this was found, some form of venous anomaly was shown on MRI and venography. What is not known at present is how these distant abnormalities relate to neurologic dysfunction or seizure activity, if at all.


   CONCLUSIONS
TOP
 ABSTRACT
 METHODS
 RESULTS
 DISCUSSION
 CONCLUSIONS
REFERENCES
 
We have used perfusion MR for the radiologic assessment of SWS and found perfusion abnormalities in territories other than that suggested by postcontrast T1-weighted scanning. Demonstration of the full extent of perfusion abnormality is important for patients' prognosis and in planning surgery to remove the seizure foci. Perfusion MRI therefore also has a role in surgical management and preoperative counseling. The goal of surgery in SWS is to prevent seizure activity or to allow intractable seizures to come under medical control and to protect the normal brain from excitotoxic neural damage secondary to seizure activity.34 It therefore is essential to recognize the entirety of the abnormality on preoperative scanning. There has been concern that postcontrast T1 MRI may not reveal the true extent of the abnormality, and certainly there have been cases of children's having continued seizures after local resection but being cured after limited removal of tissue along the edge of their previous resection.35 This technique of performing perfusion MR to show areas of perfusion abnormality that is remote from the areas of presumed leptomeningeal angiomatosis therefore is important in the preoperative assessment. Use of this technique is particularly useful because it can be performed at the same time as the MRI study to assess the structural deficit. It does not require an additional appointment or possibly a general anesthetic to obtain the perfusion data.


   FOOTNOTES
 
Accepted Nov 10, 2005.

Address correspondence to Amlyn L. Evans, FRCR, Neuroradiology Department, Inpatient X-Ray, Royal Hallamshire Hospital, Sheffield S10 2JF, United Kingdom. E-mail: amlyn.evans{at}sth.nhs.uk

The authors have indicated they have no financial relationships relevant to this article to disclose.

* The use of the MR contrast agent for perfusion studies in this article is classed as an "off-label" use according to US Food and Drug Administration regulations. Back


   REFERENCES
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 REFERENCES
 

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