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DermAtlas: EYE - Sturge-Weber syndrome
© 2001-2009, DermAtlas
Image Name: sws_dilated_vessels_1_080308   File Type: jpg
Diagnosis: STURGE-WEBER SYNDROME /
VASCULAR MALFORMATION /
TELANGIECTASIA
  Category: ophthalmology /
vascular malformation /
congenital malformation
Body Site: eye / conjunctiva   Age: 15 years
Contributor: Cameron Parsa    
Description: dilated conjunctival and subconjunctival vessels
Comments: This 15-year-old boy was evaluated for a left-side port-wine stain involving the left side of his face, seizures, and mental retardation. Dilated conjunctival and subconjunctival vessels were noted on examination of his left eye.
Related Images: sws_dilated_vessels_2_080308 

DermAtlas: EYE - Sturge-Weber syndrome
© 2001-2009, DermAtlas
Image Name: sws_corkscrew_vessels_1_080308   File Type: jpg
Diagnosis: STURGE-WEBER SYNDROME /
TELANGIECTASIA /
VASCULAR MALFORMATION
  Category: /
vascular malformation /
ophthalmology
Body Site: eye / conjunctiva   Age: 25 years
Contributor: Cameron Parsa    
Description: dilated corkscrew vessels
Comments: This 25-year-old man had a history of Sturge-Weber syndrome with an extensive left facial portwine stain, increased intraocular pressure, and seizures. Note the dilated corkscrew conjunctival vessels on the left eye.

DermAtlas: EYE - Sturge-Weber syndrome
© 2001-2009, DermAtlas
Image Name: sws_dilated_vessels_2_080308   File Type: jpg
Diagnosis: STURGE-WEBER SYNDROME /
VASCULAR MALFORMATION /
TELANGIECTASIA
  Category: ophthalmology /
vascular malformation /
congenital malformation
Body Site: eye / conjunctiva
limbus
  Age: 15 years
Contributor: Cameron Parsa    
Description: dilated perilimbal vessels
Comments: This 15-year-old boy was evaluated for a left-side port-wine stain involving the left side of his face, seizures, and mental retardation. Dilated conjunctival and subconjunctival vessels were noted on examination of his left eye. Note the prominent perilimbal vessels in this image.
Related Images: sws_dilated_vessels_1_080308 

DermAtlas: EYE - Sturge-Weber syndrome
© 2001-2009, DermAtlas
Image Name: sws_tomato_catsup_fundus_2_080308   File Type: jpg
Diagnosis: STURGE-WEBER SYNDROME /
VASCULAR MALFORMATION /
TELANGIECTASIA
  Category: ophthalmology /
vascular malformation
Body Site: eye / retina   Age: 6 years
Contributor: Cameron Parsa    
Description: choroidal details readily visible on normal uninvolved retina
Comments: This 6-year-old boy was evaluated for an extensive portwine stain involving most of the right side of the face and scalp and glaucoma in the right eye. The involved right fundus appears bright red from thickening of the choroidal blood vessels. This is oftern referred to the "tomato catsup" fundus of Sturge-Weber syndrome.
Related Images: All related Images  sws_tomato_catsup_fundus_1_080308  sws_tomato_catsup_fundus_2_080308 

DermAtlas: EYE - Sturge-Weber syndrome
© 2001-2009, DermAtlas
Image Name: sws_tomato_catsup_fundus_1_080308   File Type: jpg
Diagnosis: STURGE-WEBER SYNDROME /
VASCULAR MALFORMATION /
TELANGIECTASIA
  Category: ophthalmology /
vascular malformation
Body Site: eye / retina   Age: 6 years
Contributor: Cameron Parsa    
Description: choroidal details not visible on involved retina
Comments: This 6-year-old boy was evaluated for an extensive portwine stain involving most of the right side of the face and scalp and glaucoma in the right eye. The involved right fundus appears bright red from thickening of the choroidal blood vessels. This is oftern referred to the "tomato catsup" fundus of Sturge-Weber syndrome.
Related Images: sws_tomato_catsup_fundus_2_080308 

DermAtlas: EYE LID - stye
© 2001-2009, DermAtlas
Image Name: hordeolum_1_061030   File Type: jpg
Diagnosis: STYE /
HORDEOLUM
  Category: infections and infestations /
ophthalmology
Body Site: eye lid / eye brow   Age: 12 years
Contributor: Naeem Alhayani, DVD    
Description: red nodule on the right upper eyelid with edema of both right lids and a small crusted papule on the left eye brow
Comments: This 12-year-old boy complained of a red painful nodule on the right upper eyelid. He was treated with oral antibiotics and warm compresses.

DermAtlas: FACE - Goldenhar syndrome
© 2001-2009, DermAtlas
Image Name: epibulbar_dermoid_4_060429   File Type: jpg
Diagnosis: GOLDENHAR SYNDROME /
OCULO-AURICULO-VERTEBRAL SPECTRUM (OAVS) /
DERMOID, EPIBULBAR /
MALFORMATION, CONGENITAL
  Category: Genetic Disorder /
congenital malformation /
ophthalmology
Body Site: face / conjunctiva, bulbar
eye / cheek
  Age: 12 months
Contributor: Bernard Cohen, MD    
Description: unilateral white papule on right bulbar conjunctiva and scar on cheek lateral to right corner of mouth
Comments: This otherwise healthy demonstrates some of the typical findings of oculo-auriculo-vertebral syndrome including a unilateral epibulbar dermoid and scar near the right corner of the mouth where an accessory tragus was removed shortly after birth. OAVS is probably a phenotypically and genetically heterogeneous disorder characterized by anomalies of the ear, hemifacial microsomia, and vertebral defects. Associated findings include anomalies of the eye, brain, and developmental delay. These findings represent anomalies of the first branchial arch and contiguous structures. Although most cases are sporadic, some families manifest an autosomal dominant inheritance pattern.
Related Images: All related Images  epibulbar_dermoid_1_060429  epibulbar_dermoid_2_060429  epibulbar_dermoid_3_060429 

DermAtlas: FACE - Goldenhar syndrome
© 2001-2009, DermAtlas
Image Name: epibulbar_dermoid_3_060429   File Type: jpg
Diagnosis: GOLDENHAR SYNDROME /
OCULO-AURICULO-VERTEBRAL SPECTRUM (OAVS) /
DERMOID, EPIBULBAR /
MALFORMATION, CONGENITAL
  Category: Genetic Disorder /
congenital malformation /
ophthalmology
Body Site: face / conjunctiva, bulbar
eye / cheek
  Age: 12 months
Contributor: Bernard Cohen, MD    
Description: unilateral white papule on right bulbar conjunctiva and scar on cheek lateral to right corner of mouth
Comments: This otherwise healthy demonstrates some of the typical findings of oculo-auriculo-vertebral syndrome including a unilateral epibulbar dermoid and scar near the right corner of the mouth where an accessory tragus was removed shortly after birth. OAVS is probably a phenotypically and genetically heterogeneous disorder characterized by anomalies of the ear, hemifacial microsomia, and vertebral defects. Associated findings include anomalies of the eye, brain, and developmental delay. These findings represent anomalies of the first branchial arch and contiguous structures. Although most cases are sporadic, some families manifest an autosomal dominant inheritance pattern.
Related Images: All related Images  epibulbar_dermoid_1_060429  epibulbar_dermoid_2_060429  epibulbar_dermoid_4_060429 

DermAtlas: FACE - Goldenhar syndrome
© 2001-2009, DermAtlas
Image Name: epibulbar_dermoid_2_060429   File Type: jpg
Diagnosis: GOLDENHAR SYNDROME /
OCULO-AURICULO-VERTEBRAL SPECTRUM (OAVS) /
DERMOID, EPIBULBAR /
MALFORMATION, CONGENITAL
  Category: Genetic Disorder /
congenital malformation /
ophthalmology
Body Site: face / conjunctiva, bulbar
eye / cheek
  Age: 12 months
Contributor: Bernard Cohen, MD    
Description: unilateral white papule on right bulbar conjunctiva and scar on cheek lateral to right corner of mouth
Comments: This otherwise healthy demonstrates some of the typical findings of oculo-auriculo-vertebral syndrome including a unilateral epibulbar dermoid and scar near the right corner of the mouth where an accessory tragus was removed shortly after birth. OAVS is probably a phenotypically and genetically heterogeneous disorder characterized by anomalies of the ear, hemifacial microsomia, and vertebral defects. Associated findings include anomalies of the eye, brain, and developmental delay. These findings represent anomalies of the first branchial arch and contiguous structures. Although most cases are sporadic, some families manifest an autosomal dominant inheritance pattern.
Related Images: All related Images  epibulbar_dermoid_1_060429  epibulbar_dermoid_3_060429  epibulbar_dermoid_4_060429 

DermAtlas: FACE - Goldenhar syndrome
© 2001-2009, DermAtlas
Image Name: epibulbar_dermoid_1_060429   File Type: jpg
Diagnosis: GOLDENHAR SYNDROME /
OCULO-AURICULO-VERTEBRAL SPECTRUM (OAVS) /
DERMOID, EPIBULBAR /
MALFORMATION, CONGENITAL
  Category: Genetic Disorder /
congenital malformation /
ophthalmology
Body Site: face / conjunctiva, bulbar
eye / cheek
  Age: 12 months
Contributor: Bernard Cohen, MD    
Description: unilateral white papule on right bulbar conjunctiva and scar on cheek lateral to right corner of mouth
Comments: This otherwise healthy demonstrates some of the typical findings of oculo-auriculo-vertebral syndrome including a unilateral epibulbar dermoid and scar near the right corner of the mouth where an accessory tragus was removed shortly after birth. OAVS is probably a phenotypically and genetically heterogeneous disorder characterized by anomalies of the ear, hemifacial microsomia, and vertebral defects. Associated findings include anomalies of the eye, brain, and developmental delay. These findings represent anomalies of the first branchial arch and contiguous structures. Although most cases are sporadic, some families manifest an autosomal dominant inheritance pattern.
Related Images: All related Images  epibulbar_dermoid_2_060429  epibulbar_dermoid_3_060429  epibulbar_dermoid_4_060429 

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© DermAtlas, Johns Hopkins University; 2000-2009
Bernard A. Cohen, MD, Christoph U. Lehmann, MD

DermAtlas was last updated: Oct-28-2009
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