eMedicine Specialties > Ophthalmology > Phakomatoses

Sturge-Weber Syndrome: Multimedia

Author: Monte A Del Monte, MD, Skillman Professor of Pediatric Ophthalmology, Professor of Ophthalmology, Pediatrics and Communicable Diseases, Director of Pediatric Ophthalmology and Strabismus, W K Kellogg Eye Center, University of Michigan Medical School
Coauthor(s): Maya Eibschitz-Tsimhoni, MD, Assistant Professor of Ophthalmology, Pediatric Ophthalmology and Adult Strabismus, Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan Medical Center
Contributor Information and Disclosures

Updated: Feb 15, 2007

Multimedia

A child with Sturge-Weber syndrome that primarily...
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Media file 1: A child with Sturge-Weber syndrome that primarily affects the distribution of cranial nerve V2-3 with milder involvement of cranial nerve V1. Secondary glaucoma is evident. Ocular melanocytosis involving the sclera of both eyes is an associated finding. Image courtesy of Dr. Lamia Salah Elewa.
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A child with Sturge-Weber syndrome that primarily...

A child with Sturge-Weber syndrome that primarily affects the distribution of cranial nerve V2-3 with milder involvement of cranial nerve V1. Secondary glaucoma is evident. Ocular melanocytosis involving the sclera of both eyes is an associated finding. Image courtesy of Dr. Lamia Salah Elewa.

Same patient as in Image 1. Close-up view of the ...
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Media file 2: Same patient as in Image 1. Close-up view of the left eye, showing the Ahmed valve implanted in the inferotemporal quadrant after multiple failed filtration procedures induced severe superior conjunctival scarring. Intraocular pressure was controlled. Image courtesy of Dr. Lamia Salah Elewa.
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Same patient as in Image 1. Close-up view of the ...

Same patient as in Image 1. Close-up view of the left eye, showing the Ahmed valve implanted in the inferotemporal quadrant after multiple failed filtration procedures induced severe superior conjunctival scarring. Intraocular pressure was controlled. Image courtesy of Dr. Lamia Salah Elewa.

Same patient as in Image 1. T1-weighted axial MRI...
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Media file 3: Same patient as in Image 1. T1-weighted axial MRI images demonstrate left cerebral hemiatrophy associated with leptomengeal angiomatosis. Image courtesy of Dr. Lamia Salah Elewa.
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Same patient as in Image 1. T1-weighted axial MRI...

Same patient as in Image 1. T1-weighted axial MRI images demonstrate left cerebral hemiatrophy associated with leptomengeal angiomatosis. Image courtesy of Dr. Lamia Salah Elewa.

Same patient as in Image 1. Ocular ultrasound ima...
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Media file 4: Same patient as in Image 1. Ocular ultrasound image of the posterior segment demonstrating the diffuse choroidal thickening seen in the diffuse choroidal hemangioma with "tomato-catsup fundus." Image courtesy of Dr. Lamia Salah Elewa.
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Same patient as in Image 1. Ocular ultrasound ima...

Same patient as in Image 1. Ocular ultrasound image of the posterior segment demonstrating the diffuse choroidal thickening seen in the diffuse choroidal hemangioma with "tomato-catsup fundus." Image courtesy of Dr. Lamia Salah Elewa.

More on Sturge-Weber Syndrome

Overview: Sturge-Weber Syndrome
Differential Diagnoses & Workup: Sturge-Weber Syndrome
Treatment & Medication: Sturge-Weber Syndrome
Follow-up: Sturge-Weber Syndrome
Multimedia: Sturge-Weber Syndrome
References
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References

  1. Audren F, Abitbol O, Dureau P. Non-penetrating deep sclerectomy for glaucoma associated with Sturge-Weber syndrome. Acta Ophthalmol Scand. Oct 2006;84(5):656-60. [Medline].

  2. Board RJ, Shields MB. Combined trabeculotomy-trabeculectomy for the management of glaucoma associated with Sturge-Weber syndrome. Ophthalmic Surg. Nov 1981;12(11):813-7. [Medline].

  3. Cheng KP. Ophthalmological manifestations of Sturge-Weber syndrome. In: Brodensteiner JB, Roach ES, eds. Sturge-Weber Syndrome. 1999.

  4. Cibis GW, Tripathi RC, Tripathi BJ. Glaucoma in Sturge-Weber syndrome. Ophthalmology. Sep 1984;91(9):1061-71. [Medline].

  5. Eibschitz-Tsimhoni M, Lichter PR, Del Monte MA, et al. Assessing the need for posterior sclerotomy at the time of filtering surgery in patients with Sturge-Weber syndrome. Ophthalmology. Jul 2003;110(7):1361-3. [Medline].

  6. Iwach AG, Hoskins HD, Hetherington J, Shaffer RN. Analysis of surgical and medical management of glaucoma in Sturge-Weber syndrome. Ophthalmology. Jul 1990;97(7):904-9. [Medline].

  7. MacDonald IM, Bech-Hansen NT, Britton WA, et al. The phakomatoses: recent advances in genetics. Can J Ophthalmol. Feb 1997;32(1):4-11. [Medline].

  8. Ritch R. Serous retinal detachment after glaucoma filtration surgery in Sturge-Weber Syndrome. J Glaucoma. 1992;1(1):58-62.

  9. Schirmer R. Ein fall von telangiektasie. Albrecht von Graefes Arch Ophthalmol. 1860;7:119-121.

  10. Sturge, WA. A case of partial epilepsy, apparently due to a lesion of one of the vaso-motor centers of the brain. Trans Clin Soc Lond. 1897;12:162-167.

  11. Sullivan TJ, Clarke MP, Morin JD. The ocular manifestations of the Sturge-Weber syndrome. J Pediatr Ophthalmol Strabismus. Nov-Dec 1992;29(6):349-56. [Medline].

  12. Susac JO, Smith JL, Scelfo RJ. The "tomatoe-catsup" fundus in Sturge-Weber syndrome. Arch Ophthalmol. Jul 1974;92(1):69-70. [Medline].

  13. Weber FP. Right-sided hemi-hypotrophy resulting from right-sided congenital spastic hemiplegia with a morbid condition of the left side of the brain, revealed by radiograms. Neurol Psychopathol. 1922;3:134-139.

  14. Weiss DI. Dual origin of glaucoma in encephalotrigeminal haemangiomatosis. Trans Ophthalmol Soc U K. 1973;93(0):477-93. [Medline].

  15. Witschel H, Font RL. Hemangioma of the choroid. A clinicopathologic study of 71 cases and a review of the literature. Surv Ophthalmol. May-Jun 1976;20(6):415-31. [Medline].

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Further Reading

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Keywords

SWS, encephalotrigeminal hemangiomatosis, port wine stain, port-wine stain, nevus flammeus

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Contributor Information and Disclosures

Author

Monte A Del Monte, MD, Skillman Professor of Pediatric Ophthalmology, Professor of Ophthalmology, Pediatrics and Communicable Diseases, Director of Pediatric Ophthalmology and Strabismus, W K Kellogg Eye Center, University of Michigan Medical School
Monte A Del Monte, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Medical Association, Association for Research in Vision and Ophthalmology, International Society for Genetic Eye Diseases and Retinoblastoma, Pan-American Association of Ophthalmology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Coauthor(s)

Maya Eibschitz-Tsimhoni, MD, Assistant Professor of Ophthalmology, Pediatric Ophthalmology and Adult Strabismus, Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan Medical Center
Maya Eibschitz-Tsimhoni, MD is a member of the following medical societies: American Academy of Ophthalmology and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Medical Editor

Gerhard W Cibis, MD, Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas, Kansas City
Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

J James Rowsey, MD, Former Director of Corneal Services, St Luke's Cataract and Laser Institute, Florida
J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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