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PubMed This is a summary of 21 peer-reviewed journal articles Updated
Pediatric Ophthalmology

Protecting Your Child's Vision: Glaucoma and Eye Care in SWS

At a Glance

Children with Sturge-Weber syndrome need lifelong eye monitoring because glaucoma can be present at birth or develop later, especially when a port-wine birthmark affects the eyelid or forehead. Early treatment helps protect the optic nerve and vision.

Because Sturge-Weber syndrome (SWS) is a vascular condition, it can affect the blood vessels within and around the eye. A significant number of children with SWS will develop some form of eye involvement, most commonly glaucoma (high pressure inside the eye) [1][2].

The risk is highest if the facial port-wine birthmark involves the eyelids or the forehead [3]. This is because the same genetic variant that caused the birthmark can also cause abnormal blood vessels in the eye’s drainage system and the choroid (the layer of blood vessels behind the retina) [4][5].

Glaucoma: Monitoring and Pressure

Glaucoma in SWS happens because the eye cannot drain fluid (aqueous humor) efficiently. This may be due to a malformed drainage angle or high pressure in the veins outside the eye [4][6]. If the pressure stays too high, it can damage the optic nerve, which is the “cable” that sends visual information to the brain. Children may have few or no visible symptoms even when pressure is at harmful levels, making surveillance critical.

Surveillance Schedule

Because glaucoma can be present at birth or develop years later, lifelong monitoring is essential [7]. While an evaluation in the first month of life and frequent check-ins during the first year are common, there is no single universal schedule [3]. Your child’s surveillance depends on their age, birthmark pattern, eye findings, and whether glaucoma is already present. A child with glaucoma may need much more frequent visits, and a normal examination does not remove the lifelong risk.

Signs of Eye Issues

In infants, the eye is still flexible. High pressure can actually stretch the eye, making it look larger. Look for:

  • Buphthalmos: An enlargement of the entire globe of the eye [8].
  • Corneal Clouding: The clear front surface of the eye (cornea) may look hazy, milky, or gray [9].
  • Excessive Tearing and Light Sensitivity (Photophobia): Excessive crying, tearing, or squinting in bright light.

Beyond glaucoma, children with SWS are also at risk for refractive errors (needing glasses) and amblyopia (a “lazy eye” where the brain favors one eye over the other). Routine eye care should include monitoring visual development and addressing these with glasses or patching as needed [10].

Treating SWS Glaucoma

Treatment aims to lower the intraocular pressure (IOP) to a safe level to protect the optic nerve [11]. Treatment is selected by a pediatric glaucoma specialist according to age, pressure, optic-nerve findings, anatomy, and prior treatment.

  • Medications: Eye drops may be used to lower pressure, particularly in older children, but they are sometimes less effective in SWS than in other types of glaucoma and may only serve as a bridge to surgery [6][12].
  • Angle Surgery (Trabeculotomy): Often required as a primary treatment for infants and young children, this procedure opens the eye’s natural drainage canal [11].
  • Tube Shunts (Glaucoma Drainage Devices): For older children or more resistant cases, a tiny tube may be implanted to drain fluid out of the eye [13][14].

A Note on Surgical Risks: Surgery in SWS carries a unique risk of postoperative hypotony (pressure that drops too low) and choroidal effusions (fluid buildup behind the retina) [15][16]. This happens because the abnormal blood vessels in SWS can leak when the eye pressure is suddenly lowered during surgery [11]. Your surgeon will take specific precautions to manage these risks.

Choroidal Hemangiomas

A diffuse choroidal hemangioma (DCH) is a thick layer of abnormal blood vessels behind the retina [5]. It is often “silent,” meaning it doesn’t cause pain, but it can cause the retina to leak fluid, leading to blurry vision or even a retinal detachment [17][18].

  • Monitoring: Doctors use specialized imaging like OCT (a detailed eye scan) and B-scan ultrasound to “see” through the back of the eye and monitor the thickness of these vessels [19][20].
  • Treatment: If the hemangioma starts leaking fluid, treatments like Photodynamic Therapy (PDT) use a light-activated drug to “shrink” the abnormal vessels without damaging the retina [18][21].

Ophthalmologic Red Flags

Contact your ophthalmologist immediately or go to an emergency room if your child experiences:

  1. Sudden Eye Pain: Often described as a deep ache in or around the eye.
  2. Redness and Nausea: A red eye accompanied by unexplained vomiting can be a sign of a sudden pressure spike (acute glaucoma).
  3. Sudden Vision Loss: A noticeable change in how well your child can see or track objects.
  4. Corneal Haze: A sudden “foggy” or “steamy” look to the clear front surface of the eye.

Common questions in this guide

Why are children with Sturge-Weber syndrome at risk for glaucoma?
Sturge-Weber syndrome can cause abnormal blood vessels in the eye’s drainage system or in the veins around the eye. These changes can prevent normal fluid drainage and raise pressure inside the eye, with higher risk when a port-wine birthmark affects the eyelid or forehead.
How often should a child with Sturge-Weber syndrome have eye exams?
There is no single schedule for every child. An evaluation during the first month and frequent visits during the first year are common, followed by lifelong monitoring based on age, birthmark pattern, eye findings, and whether glaucoma is present.
What signs of glaucoma should I watch for in my child?
Warning signs in infants can include an unusually large eye, a hazy or cloudy cornea, excessive tearing, and sensitivity to light. A painful or red eye with nausea or vomiting, sudden vision loss, or a new corneal haze requires immediate contact with an ophthalmologist or emergency evaluation.
What treatments can lower eye pressure in Sturge-Weber glaucoma?
Treatment may include pressure-lowering eye drops, trabeculotomy to open the eye’s natural drainage canal, or a tube shunt to drain fluid. A pediatric glaucoma specialist chooses the approach based on the child’s age, eye anatomy, pressure, optic-nerve findings, and previous treatment.
What is a choroidal hemangioma, and how is it treated in Sturge-Weber syndrome?
A diffuse choroidal hemangioma is a layer of abnormal blood vessels behind the retina. It may cause no symptoms, but leaking fluid can blur vision or contribute to retinal detachment; doctors can monitor it with OCT and ultrasound and may use photodynamic therapy if leakage develops.
Why does glaucoma surgery have special risks in Sturge-Weber syndrome?
Abnormal blood vessels in Sturge-Weber syndrome can leak when eye pressure falls quickly during surgery. This may cause pressure that is too low, called hypotony, or fluid buildup behind the retina, called a choroidal effusion, so the surgeon uses precautions to manage these risks.
How are glasses and lazy eye treated in a child with Sturge-Weber syndrome?
Routine eye care checks visual development for refractive errors and amblyopia, sometimes called lazy eye. Glasses can correct focusing problems, and patching may be recommended when the brain is favoring one eye over the other.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Does my child's port-wine birthmark involve the upper face or eyelid, and what does that mean for their lifetime glaucoma risk?
  2. 2.What was my child's eye pressure today, and how does it compare to their previous readings?
  3. 3.Is my child currently showing any signs of choroidal thickening or a choroidal hemangioma on imaging like OCT or ultrasound?
  4. 4.If my child needs surgery for glaucoma, which procedure (angle surgery vs. tube shunt) do you recommend based on their age and eye anatomy?
  5. 5.How are we monitoring for amblyopia ('lazy eye') or the need for glasses, and should we be patching?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (21)
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    Ocular manifestations of Sturge-Weber syndrome: pathogenesis, diagnosis, and management.

    Mantelli F, Bruscolini A, La Cava M, et al.

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    PMID: 27257371
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    Incidence of Sturge-Weber syndrome and associated ocular involvement in Olmsted County, Minnesota, United States.

    Rihani HT, Dalvin LA, Hodge DO, Pulido JS

    Ophthalmic genetics 2020; (41(2)):108-124 doi:10.1080/13816810.2020.1731834.

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    Glaucoma Management in Sturge-Weber Syndrome Using the Delphi Process.

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    Ophthalmology. Glaucoma 2026; (9(3)):336-342 doi:10.1016/j.ogla.2025.11.003.

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    Pathophysiology, diagnosis, and management of glaucoma associated with Sturge-Weber syndrome.

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    International ophthalmology 2018; (38(1)):409-416 doi:10.1007/s10792-016-0412-3.

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    Pathophysiology and management of glaucoma associated with phakomatoses.

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    Sturge-Weber syndrome: an update for the pediatrician.

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    Management of Glaucoma in an Adult Presentation of Sturge-Weber Syndrome.

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    Clinical characteristics of infants with port-wine stain and glaucoma secondary to Sturge-Weber Syndrome.

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    Sturge-Weber Syndrome: A Review of Pathophysiology, Genetics, Clinical Features, and Current Management Approache.

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    Surgical Outcomes of Early Versus Late Onset Glaucoma Associated With Sturge-Weber Syndrome.

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    Sturge-Weber syndrome secondary glaucoma: From Pathogenesis to Treatment.

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    Eye and vision (London, England) 2025; (12(1)):16 doi:10.1186/s40662-025-00432-6.

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    Outcome of trabeculectomy versus Ahmed glaucoma valve implantation in the surgical management of glaucoma in patients with Sturge-Weber syndrome.

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    A Case of Refractory Childhood Glaucoma Associated With Sturge-Weber Syndrome Treated With Baerveldt Glaucoma Implant.

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This page is for informational purposes only and does not constitute medical advice. Your child’s ophthalmologist or pediatric glaucoma specialist should interpret eye findings and recommend care for your child.

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