Yasunari T, Shiraki K, Hattori H, Miki T: Frequency of choroidal abnormalities in neurofibromatosis type 1. Lancet 356:988–992, 2000.
105 STURGE–WEBER SYNDROME
759.6
(Encephalotrigeminal Angiomatosis
Syndrome)
Monte A. Del Monte, MD
Ann Arbor, Michigan
Sturge–Weber syndrome (SWS) belongs to a group of disorders collectively known as phakomatoses (‘mother-spot’ diseases). It consists of congenital hamartomatous malformations that may affect the eyes, skin, and central nervous system at different times. SWS is classified into three categories:
1.Complete trisymptomatic SWS, in which all three organ systems are involved;
2.Incomplete bisymptomatic SWS, in which the involvement is either oculocutaneous or neurocutaneous;
3.Incomplete monosymptomatic SWS, in which there is only neural or cutaneous involvement.
Patients without cutaneous involvement appear to be spared the ocular manifestations of the syndrome.
ETIOLOGY/INCIDENCE
The clinical manifestations of SWS have a common embryologic basis, the primary defect being a developmental insult affecting precursors of tissues that originate in the proand mesencephalic neural crest. These affected precursors then give rise to vascular and other tissue malformations in the meninges, the eyes and the dermis. Sources of the insult have been suggested to be:
●A somatic mutation in the precursors that may result in overproduction of an angiogenic factor; or
●A lethal gene, surviving by mosaicism.
Incomplete SWS results from the same developmental defect but affects only those cells whose progeny are destined for the affected tissues.
Unlike the other phakomatoses, which often have clear-cut hereditary patterns, the role of heredity in SWS has not been documented; to date, no gene defect has been associated with the syndrome. Several types of chromosomal abnormalities have been reported, but most SWS patients have normal karyotypes. The majority of SWS patients have a sporadic, nonfamilial disease.
The syndrome occurs in all races, with no significant predilection for either sex. The incidence of SWS in the general population is unknown.
DIAGNOSIS
Clinical signs and symptoms
Systemic
The hallmark of SWS is a facial cutaneous venous dilation, also referred to as nevus flammeus or port-wine stain, that occurs
in as many as 96% of patients and is visible at birth. The nevus appears as one or more dull red patches of irregular outline, usually in the areas of the trigeminal nerve branches. Although it does not increase in size and is not medically threatening, the nevus does darken with age, and as a cosmetic blemish, it may carry psychologic impact.
A leptomeningeal congenital venous angiomatosis, usually ipsilateral to the facial lesion and located most commonly in the meninges overlying the occipital and posterior parietal lobes, results in involvement of the central nervous system (CNS). Characteristic progressive calcifications in the external layers of the cerebral cortex beneath the angiomatosis are associated with cortical atrophy; occasionally, they extend anteriorly to the frontal and temporal lobes.
Focal or generalized motor seizures occur in up to 85% of patients; they usually begin in the first year of life and may become profound, resulting in further neurologic and developmental deterioration. Early diagnosis and treatment are necessary to minimize permanent brain damage.
Some degree of mental retardation is seen in about 60% of patients, as well as neurologic deficits such as:
●Hemiplegia;
●Homonymous hemianopsia.
Ocular
●Eyelid hemangioma-like superficial changes that show, on histology, only venous dilation.
●Glaucoma, which is a significant cause of morbidity because of its early onset and resistance to conventional forms of treatment.
●Conjunctival and episcleral hemangiomas.
●Prominent, tortuous conjunctival and episcleral vascular plexi.
●Diffuse choroidal hemangiomas.
●Iris heterochromia.
●Tortuous retinal vessels, with occasional arteriovenous communications.
Glaucoma is estimated to occur in 30% to 70% of patients, with early onset (at birth or in early infancy) the rule, although it may present at any time. The glaucoma is almost always unilateral and ipsilateral to the port-wine stain, although contralateral or bilateral glaucoma with unilateral skin lesions have been reported. Occurrence of glaucoma has been noted especially when the facial skin changes involve both the upper and lower eyelids.
As soon as SWS is suspected, a complete ophthalmic evaluation is essential to rule out glaucoma or to institute effective measures to control it, as the infant eye is quickly damaged by increased intraocular pressure (IOP). The earlier glaucoma can be detected and controlled, the less likely the patient is to suffer the secondary glaucomatous changes, including buphthalmos, increased corneal diameter, tears in Descemet’s membrane, corneal edema, and optic nerve damage that results in myopia, anisometropia, amblyopia, strabismus and visual field defects. All SWS patients require regular ophthalmic examination throughout life, even when no ocular abnormalities are initially detected, to avoid vision loss secondary to later-onset glaucoma.
Amblyopia is an important cause of poor vision in patients with infantile glaucoma. It is usually anisometropic because of glaucoma-induced myopia or secondary to unilateral or bilateral pattern deprivation caused by cloudy corneas. Even when glaucomatous optic nerve damage is present, amblyopia may be
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superimposed on the organic damage and a trial of amblyopia therapy is indicated.
Prominent tortuous conjunctival and episcleral vascular plexi affect up to 70% of SWS patients and often correlate with increased episcleral venous pressure, probably resulting from arteriovenous shunts within the episcleral hemangiomas.
Diffuse choroidal hemangioma (which appears as a circumscribed, isolated form in otherwise normal adults) is present in up to 50% of patients with SWS. Almost always unilateral, and ipsilateral to the port-wine stain, choroidal hemangiomas also have been reported as bilateral in association with bilateral nevus flammeus. These hemangiomas:
●Are flat;
●Commonly cover more than half the fundus;
●Involve the posterior pole;
●Extend into the equatorial zone;
●May show diffuse involvement of the entire uvea;
●Can have a striking reddish ‘tomato ketchup fundus’ appearance.
Often a focal, perimacular area of the angioma is thickened and elevated, with dilation and tortuosity of the overlying retinal vessels and peripheral arteriovenous communications.
Choroidal angiomatosis grows slowly and is usually asymptomatic in childhood, but during adolescence or adulthood a marked thickening of the choroid may become evident, with secondary changes to overlying ocular structures. These changes can range from mild atrophy of retinal pigment epithelium (RPE) and focal RPE proliferation with drusen formation to severe fibrous transformation and focal ossification of the RPE.
The retina over the hemangioma may be attached and well preserved, attached but degenerated, or detached. Early-stage choroidal thickening and lifting of the retina may produce an increasing ipsilateral hyperopia. Degenerative retinal changes include focal chorioretinal adhesions, loss of photoreceptors, severe cystoid degeneration of the outer layers, and marked gliosis. Widespread serous detachment, retinal leakage, and edema may occur. Subretinal fibrosis near the macula and cystoid macular edema are associated with the most severe visual loss.
Glaucomatous damage, degenerative changes in the outer retinal layers, and vascular abnormalities in the occipital lobe may cause visual field defects; careful examination with visual field perimetry is indicated.
Iris heterochromia occurs in about 10% of SWS patients; the more deeply pigmented iris usually is ipsilateral to the portwine stain, indicating an increase in melanocyte numbers or activity.
Laboratory findings
The three forms of SWS are generally diagnosed on clinical grounds by the association of the typical cutaneous, CNS, and ocular abnormalities.
When a typical facial vascular skin lesion is found in a newborn, it should alert the physician to perform a complete ophthalmologic and systemic assessment for the potentially serious associated disorders. Neonates with bisymptomatic or trisymptomatic SWS may initially seem neurologically normal, without symptoms of glaucoma or other ocular problems; thus, in some instances the diagnosis may not become clear for some time.
In young patients with suspected glaucoma, examination under anesthesia or deep sedation is necessary to confirm the
diagnosis; careful assessment in each eye of intraocular pressure, corneal diameter, cycloplegic refraction, axial length, gonioscopy, and optic nerve cupping is mandatory.
Ocular signs of infantile glaucoma associated with SWS include:
●Corneal diameter >12.0 mm during the first year of life;
●Corneal edema;
●Tears in Descemet’s membrane (Haab’s striae);
●Unilateral or bilateral myopic shift;
●Optic nerve cupping >0.3 or any cup asymmetry;
●Intraocular pressure >18 or 19 mm Hg.
Increased conjunctival vascularity can be observed on a slitlamp examination or can be seen by the naked eye as a pinkish discoloration. However, the abnormal plexus of episcleral vessels may be hidden by the overlying tissue of Tenon’s capsule in infancy and appreciated clinically only in later childhood.
Diagnosis of diffuse choroidal hemangioma is based on:
●Tumor appearance on binocular indirect ophthalmoscopy;
●A- and B-scan ultrasonography; A-scan shows high internal reflectivity, whereas B-scan typically shows a solid echogenic mass;
●Fluorescein angiography may reveal;
●Only a heightened background choroidal fluorescence early in the disease;
●Widespread irregular hyperfluorescence as dye leaks from the surface of a progressing tumor;
●A diffuse multilocalized pattern of fluorescein accumulation in the outer retina characteristic of polycystic degeneration and edema in more advanced disease.
Diffuse choroidal hemangioma may easily be missed on ophthalmoscopic examination, especially in children, because the color of the hemangioma resembles that of the normal fundus, and the elevation may be minimal; comparison of the red reflex with the normal opposite eye can help in confirming the diagnosis.
Central nervous system involvement can be confirmed by various neuroimaging methods:
●Plain skull radiographs are often adequate for diagnosis.
●Magnetic resonance imaging (MRI) allows detection of malformations affecting the CNS, including abnormal venous drainage and abnormal pial contrast enhancement associated with SWS angiomatous malformations, and it can detect cerebral volume reduction and ipsilateral choroid plexus enlargement; in addition, the technique can demonstrate the curvilinear posterior contrast enhancement of ocular choroidal angiomas, using intravenous contrast.
●Computed tomography (CT) is superior to MRI in detecting the characteristic double-lined gyroform pattern of calcifications paralleling cerebral convolutions, which are known to radiologists as the ‘railroad track’ sign; however, these calcifications usually are not detectable before 1 year of age and may not be seen until several years of age.
Differential diagnosis
Disorders with clinical presentations similar to those of SWS must be included in differential diagnosis. They include the following:
●Klippel–Trenaunay–Weber (KTW) syndrome consists of port-wine stains of the extremities and face and hemihypertrophy of soft and bony tissues, as well as all the characteristics of SWS; KTW syndrome is sporadic, as is SWS. There is also an association in KTW syndrome between hemi-
196
hypertrophy and solid visceral tumors that most commonly |
or if the child develops strabismus, treatment to prevent ambly- |
affect the kidney, adrenal gland, or liver. |
opia and treat the strabismus should be initiated. Anisome- |
●Beckwith–Wiedemann syndrome consists of a facial porttropic amblyopia may require occlusion therapy along with wine stain, macroglossia, omphalocele, and visceral hypercorrection of the refractive error; in some patients, a contact
plasia; note that there is also some associated risk of visceral neoplasia. Also, severe hypoglycemia resulting from pancreatic islet cell hyperplasia is very common and may be life threatening.
Neuroimaging findings similar to those of SWS may be found in several other conditions and should also be considered in the differential diagnosis:
lens may be required to treat fusion difficulty due to aniseikonia.
Medical treatment for SWS glaucoma usually fails with time but may be tried initially, as significant (albeit temporary) reduction in IOP may be achieved. Reduced IOP may clear the cornea and permit surgery to proceed or, in younger patients, delay the need for filtration surgery. This is especially important considering the excessive difficulties of operating on a
●In Dyke–Davidoff–Masson syndrome, one cerebral hemismall eye, as well as the tendency of the younger patient to scar
sphere is partially or completely atrophic as a result of an intrauterine or perinatal carotid artery infarction; because the cerebral atrophy in SWS also occurs during infancy, changes similar to those of Dyke–Davidoff–Masson syndrome may be seen, including cerebral hemiatrophy with ipsilateral calvarial diploic space enlargement.
●Severe siderosis, prior to injection of contrast material, has MRI findings similar to those in SWS with cerebral hemiatrophy. The typical contrast enhancement and the abnormal veins seen with contrast injection easily differentiate the two conditions.
●Calcification secondary to intrathecal methotrexate therapy and meningitis also must be included in the CT differential diagnosis of cortical pattern calcification; neither of these, however, would demonstrate the specific unilateral geographic localization.
When assessing the status of a uveal mass in a patient with SWS, the ophthalmologist must consider the possibility that the lesion may be something other than a choroidal hemangioma.
at the site of the scleral flap, which may reduce long-term success.
Medical therapy can also be used as an adjunct to surgery. Topical antiglaucoma therapy for extended periods is sometimes helpful postoperatively to further reduce borderline elevations in IOP without the need to reoperate. Initial medical therapy with a:
●Topical β-blocker, followed sequentially by the addition of a
carbonic anhydrase inhibitor (systemic in infants and topical in older children), and an α-adrenergic agonist (bromonidine), or a topical prostaglandin receptor agonist (latanoprost) is a reasonable protocol in SWS patients.
In recent years, few patients with diffuse choroidal hemangiomas associated with a bullous nonrhegmatogenous retinal detachment have been treated with radiation therapy such as:
●Brachytherapy; or
●External beam irradiation.
Preliminary reports suggest that radiation therapy may be a reasonable alternative to the currently preferred photocoagula-
●The major clinical difficulty can be distinguishing a choroition in selected patients, but the ultimate risk-to-benefit ratio dal hemangioma from a choroidal melanoma. There are a of this form of therapy is still unknown, as are its precise indi-
few reports of patients with SWS who developed a choroidal tumor in the eye ipsilateral to the port-wine stain, and it eventually proved to be a malignant melanoma rather than a hemangioma. Simultaneous occurrence of uveal melanoma and choroidal hemangioma in a patient with SWS also has been described. The reddish-orange color of choroidal hemangiomas as viewed by binocular indirect ophthalmoscopy is an important diagnostic sign that differentiates them from metastatic carcinomas and amelanotic melanomas which have a white or creamy appearance. When uveal melanoma is suspected, both fluorescein angiography and A- and B-scan ultrasonography are essential.
cations and contraindications.
Surgical
Surgery, rather than medical therapy, is still considered by most ophthalmologists to be the mainstay for glaucoma therapy in SWS, with antiglaucoma medications being used primarily as adjuncts. The selection of surgical technique remains controversial, however, because the long-term results are often disappointing with any of the procedures, and none of them enjoys the success with SWS glaucoma that it may have when initially performed for primary infantile glaucoma.
Because of the rarity of SWS, the published accounts of surgi-
●Other orange-colored fundus tumors that must be considcally treated cases have been uncontrolled, and no standard ered in a differential diagnosis of a diffuse choroidal hemanguidelines exist. The objective of therapy is rapid and perma-
gioma include. |
nent lowering of the IOP into the normal range (generally, |
●Serous or partly organized detachment of the retinal pigment <20 mm Hg) or to a level slightly higher but without progres-
epithelium.
●Osteoma of the choroids.
●Nodular scleritis.
●Exophytic retinal capillary hemangioma.
TREATMENT
Ocular
For small degrees of anisometropia, full optical correction of both eyes or at least full correction of the refractive difference between the eyes is desirable. In higher degrees of anisometropia
sion of other signs, such as corneal enlargement, increased myopia, or increased optic nerve cupping.
Postoperative care for infants and young children commonly requires repeated examinations under anesthesia to assess surgical success. If continued borderline IOP elevation is found, then a trial of medical therapy with close follow-up may be safely continued as long as no progression of glaucoma damage is observed. However, if IOP remains clearly elevated or progressive glaucomatous damage is detected, repeat glaucoma surgery should be performed.
The anesthesiologist should be made aware that the patient has SWS, because the presence of a spinal cord or brain heman-
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Phakomatoses • 11 SECTION
gioma may increase the risk of intracerebral bleeding or disseminated intravascular coagulation with anesthesia. In addition, an anesthesia protocol should be planned to prevent the development of hypertension that could result in hemorrhage.
●Goniotomy or trabeculotomy is believed by some to be the treatment of choice for early-onset glaucoma in infancy, when the probable mechanism for pressure elevation is an abnormal outflow angle. However, these procedures often are unsuccessful in infants with SWS, or they succeed only after being repeated several times and with the addition of adjunct medical therapy; in patients over the age of 4, an even shorter duration of pressure control is the rule. Nevertheless, some physicians prefer to perform these procedures initially because they are occasionally successful and goniotomy is also thought to be less likely to cause the complications (especially expulsive choroidal hemorrhage or choroidal effusion) that are associated with a precipitous drop in IOP.
●Trabeculectomy (either fullor partial-thickness) is more likely to be successful in treating glaucoma of later onset, when the outflow angle appears clinically normal, as it bypasses any component of the glaucoma possibly caused by elevated episcleral venous pressure. Combined trabec- ulotomy-trabeculectomy may be a reasonable compromise in the older patient with SWS, in view of the possible combination of angle abnormality and raised episcleral pressure in causing SWS glaucoma.
●Adjunctive antimetabolites, used in conjunction with a filtering surgery, may achieve a more satisfactory degree of IOP control in this patient population by slowing wound healing and scar formation. The most commonly used clinical agents are 5-fluorouracil (5-FU), given as a series of subconjunctival injections postoperatively, and mitomycin C, usually applied intraoperatively using a sponge saturated with the solution. Postoperative subconjunctival injections are usually impossible in very young patients, so the intraoperative application of mitomycin C is frequently required for these cases.
Both 5-fluorouracil and mitomycin C are associated with thinner, more cystic blebs and may carry a higher rate of complications such as wound leak, chronic hypotony, and possibly late endophthalmitis.
●Corticosteroids should be used after filtration surgery to minimize postoperative inflammation and scarring of the bleb. A sub-Tenon’s injection of a short-acting corticosteroid such as dexamethasone or triamcinolone at the completion of surgery and the use of topical corticosteroid drops or ointment after surgery are recommended.
●Cyclocryotherapy is difficult to control and has a high complication rate; therefore, it should be used to save useful vision or to prevent or relieve severe pain only when all other procedures have failed or are not feasible. New types of cyclodestructive procedures, such as Nd : YAG transscleral laser and therapeutic ultrasound, have had only limited trial in pediatric and SWS glaucoma, and their potential for long-term success as well as the complications they may cause in the young patient is not yet fully understood.
●Seton devices also are being used when routine filtration surgery has failed. Encouraging initial results have been reported using various posterior tube shunt implant devices, but long-term follow-up results are not yet available.
●Neodymium-YAG laser goniotomy and argon laser trabeculoplasty have been used only to a limited extent in pediatric glaucoma, but favorable results in some SWS patients have been reported.
●Eye muscle surgery is the best treatment for any significant strabismus still present after completion of amblyopia therapy, refractive lens correction, and orthoptics. Avoidance or careful cauterization of the dilated subconjunctival and episcleral vessels during strabismus surgery is important in order to prevent bleeding and scarring so that the conjunctiva and anterior sclera can be preserved for future glaucoma procedures.
Unfortunately, no treatment has yet been shown to be effective in preventing or reversing the visual deterioration associated with the secondary changes of ocular structures overlying a diffuse choroidal hemangioma. Management of affected eyes emphasizes the reduction of subretinal fluid as the main therapeutic aim in an attempt to stabilize or reverse, if possible, visual impairment caused by nonrhegmatogenous retinal detachment. However, no reliable treatment for the retinal detachment that develops in these patients has been found, and even in the exceptional case in which the retina can be reattached, fibrous metaplasia of the retinal pigment epithelium and cystoid degeneration of the retina overlying the choroidal hemangioma prevent good visual results. Many such eyes eventually become blind and painful and must be enucleated. Attempts to repair the nonrhegmatogenous retinal detachment involve:
●Cryotherapy and diathermy;
●Xenon arc or argon laser photocoagulation;
●Draining of the subretinal fluid;
●Radiation therapy.
A factor critical to a successful outcome appears to be early initiation of treatment.
●Laser photocoagulation is generally considered to be the preferred therapeutic intervention. Light photocoagulation scars are placed over the entire tumor in an attempt to strengthen the adhesion of the retina to the underlying pigment epithelium and thus prevent the spread of the retinal detachment; this form of treatment has afforded limited success. However, retinal detachment often recurs even after photocoagulation therapy, and in some patients it is not possible to attain complete reattachment of the retina. Furthermore, with large hemangiomas and diffuse infiltrating tumors of the macula, treatment success is limited.
●External drainage of subretinal fluid, with or without scleral buckling in conjunction with xenon photocoagulation, has been used to treat diffuse choroidal hemangiomas associated with large exudative retinal detachments in SWS.
●Pars plana vitrectomy, endolaser and internal drainage of subretinal fluid can be performed.
●Cryotherapy and penetrating diathermy are of limited use because of the posterior location of the tumor.
●Dye laser photocoagulation has been very helpful in reducing the cosmetic blemish of the cutaneous port-wine stain.
PRECAUTIONS
Surgical management of secondary open-angle glaucoma in SWS using filtering surgery and Seton procedures bears an
198
increased risk for a number of surgical complications, the most sight-threatening and feared being expulsive choroidal hemorrhage and intraoperative massive choroidal effusion.
●Sudden change in the IOP gradient when the eye is opened may result in expulsive choroidal hemorrhage from the choroidal hemangioma. Treatment involves rapid closure of all scleral incisions, with restoration of IOP. Transscleral drainage of suprachoroidal blood also may be indicated.
●The intraoperative formation of a massive choroidal effusion without hemorrhage also occurs frequently during filtering surgery in SWS patients. It is assumed that the increased episcleral venous pressure in these patients causes a similar increase in the venous pressure within the ciliary body and choroid. During surgery, when the eye is opened and IOP falls, rapid transudation of fluid from the intravascular to the extravascular space results. This extravasation of fluid may be massive enough to cause choroidal detachment instantaneously, as well as later serous retinal detachment; however, it seems that once the intraoperative effusion is managed with immediate drainage, the postoperative prognosis becomes excellent despite the persistence of some degree of choroidal and serous retinal detachment.
●Postoperatively, smaller serous choroidal detachment may develop.
●Serous retinal detachment often occurs in association with choroidal effusion and hypotony; it is possible that a choroidal effusion temporarily interferes with the metabolic transport systems of the retinal pigment epithelium. These serous retinal detachments usually resolve spontaneously as the IOP normalizes.
Various preoperative and perioperative measures have been suggested to counteract or prevent these complications, including hyperosmotics, maximum antiglaucoma therapy preoperatively, prophylactic posterior sclerotomy, prophylactic radiotherapy or laser photocoagulation of the choroidal hemangioma, and electrocautery of the anterior episcleral vascular anomaly.
Suggested steps to minimize the intraoperative and postoperative hypotony are preplacement of scleral flap sutures, injection of a viscoelastic prior to excision of the trabecular meshwork, and tight suturing of the scleral flap with a releasable suture that could be lysed after surgery with an argon laser or removed using a slit lamp or at the time of examination under anesthesia.
Any intraocular surgery predisposes the eye to the risk of bacterial endophthalmitis. Patients with filtering blebs, especially the thin avascular blebs seen with the use of mitomycin C, are at increased risk for developing bacterial endophthalmitis months or even years after surgery. Because this risk is intensified by contact lens wear, the use of any type of contact lenses by these patients is discouraged. Other potential sources of infection include normal conjunctival flora, episodes of bac-
terial conjunctivitis, and contaminated medication dropperbottle tips.
SUPPORT GROUP
Sturge–Weber Foundation P.O. Box 460931
Aurora, CO 80046 (303) 690-9735
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