J Korean Ophthalmol Soc.  2013 Nov;54(11):1737-1747. 10.3341/jkos.2013.54.11.1737.

Clinical Features and Surgical Outcomes of Sturge-Weber Syndrome with Glaucoma

• Park JH
• Lim SH
• Cha SC

• KMID: 2217922
• DOI: http://doi.org/10.3341/jkos.2013.54.11.1737

Abstract

PURPOSE
To report clinical manifestations including neurocutaneous and ocular findings and to evaluate outcomes of trabeculectomy in patients with Sturge-Weber syndrome.
METHODS
The medical records of 10 eyes of 8 glaucoma patients with Sturge-Weber syndrome who were followed up for at least 1 year after trabeculectomy were reviewed retrospectively. We analyzed neurocutaneous and ocular findings, cumulative surgical success rates, and complications in patients with Sturge-Weber syndrome.
RESULTS
The mean patient age at the time of surgery was 12.6 +/- 13.0 years and mean follow-up period was 71.6 +/- 81.8 months. All patients showed various clinical findings including facial hemangioma (8 patients), seizure (6 patients), intracranial lesion (6 patients), developmental delay (4 patients), conjunctival/episcleral hemangioma (4 eyes), and choroidal hemangioma (4 eyes). Postoperative success was achieved in 8 out of 10 eyes (80.0%). Postoperatively, serous retinal detachment occurred in 2 out of 4 eyes with preoperative diffuse choroidal hemangioma.
CONCLUSIONS
Management of glaucoma associated with Sturge-Weber syndrome requires multidisciplinary treatment because of systemic involvement. Trabeculectomy appears to be an effective and relatively safe surgical option for glaucoma associated with Sturge-Weber syndrome. However, serious complications such as serous retinal detachment should be considered when planning trabeculectomy for patients with diffuse choroidal hemangioma.

Keyword

Glaucoma; Neurocutaneous findings; Sturge-Weber syndrome; Trabeculectomy

MeSH Terms

Choroid
Follow-Up Studies
General Surgery
Glaucoma*
Hemangioma
Humans
Medical Records
Retinal Detachment
Retrospective Studies
Seizures
Sturge-Weber Syndrome*
Trabeculectomy

Figure

• 

  Figure 1. Conjunctival and/or episcleral hemangioma of patient. (A) Mildly engorged conjunctival and/or episcleral vessel with hypovascular bleb. (B) Moderately engorged conjunctival and/ or episcleral vessel with avascular bleb. (C, D) Severely engorged conjunctival and/or episcleral vessel.

• 

  Figure 2. Diffuse choroidal hemangioma in patients with Sturge-Weber syndrome and Klippel-Trenaunay- Weber syndrome. (A) Right eye of patient 6. (B) Left eye of patient 2. (C) Left eye of patient 8.

• 

  Figure 3. Kaplan-Meier survival curve showing the cumulative success rates of trabeculectomy for patients with Sturge-Weber syndrome.

• 

  Figure 4. Patient 6 presented choroidal detachment with serous retinal detachment and optic disc swelling after trabeculectomy. (A, B) Severe choroidal detachment with serous retinal detachmnet and optic disc swelling on th 19th postoperative day. (C, D) Decreased choroidal detachment with serous retinal detachemnt and remained optic disc swelling on the 60th postoperative day. (E) No choroidal effusion or retinal detachment was noted 5 years after trabeculectomy.

• 

  Figure 5. Immediately after removal of releasable suture at postoperative third days (Patient 1), (A, B) almost total hyphema was developed, and (C) massive subconjuntival hemorrahge was seen. (D) Hemorrhagic bleb was noted. (E, F) Two-year postoperative appearance of the bleb on the right eye of patient 1. The figures show diffuse moderately elevated, hypovascular bleb.

• 

  Figure 6. Brain MRI in a patient with Sturge- Weber syndrome. (A) Leptomeningeal angioma (arrow) and choroid plexus hemangioma (arrow head) of patient 1. (B) Dominant choroidal plexus hemangioma (arrow) of patient 3. (C) Diffuse cortical atrophy (arrow) of patinet 8.

• 

  Figure 7. Cutaneous manifestations of Sturge-Weber syndrome and Klippel- Trenaunay-Weber syndrome. (A) Portwine stain in patient 2 with Sturge- Weber syndrome. Note the hypertrophy and nodularity of facial angioma. (B) Bilateral involvement of facial hemangioma (Patient 5) (C) Limbal and truncal hemangioma in patient 7 with Klippel- Trenaunay-Weber syndrome. (D) Limbal soft tissue hypertrophy in patient 7 with Klippel-Trenaunay-Weber syndrome.

Reference

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