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GRAND ROUNDS
Desirée Ong, M.D.
PGY-2
Vanderbilt Eye Institute
Patient CP
• 23 yo WM, passenger in MVA, was found
unconscious next to vehicle. Driver was
not at the scene
• In the ED, pt was noted to have swelling
and ecchymosis of the left peri-ocular area
• ED staff noted a unilateral irregular pupil
and were concerned about the possibility
of an open globe
Exam findings
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Gen: unconscious, unable to be awakened
No friends or family were present
No witnesses were at the scene
VA/VF: unable to obtain
Pupils: OD 3mm-->2mm, OS irregular
pupil with no constriction
• IOP: OD 17
OS 24,25,27
Differential Diagnosis for
Corectopia?
Differential Diagnosis
• Trauma: high suspicion for open globe, FOB, corneal
perforation, iridodialysis
• Iatrogenic: anterior segment surgery
• ICE syndrome
• Posterior synechiae/inflammatory changes
• Iris tumor
• Iris coloboma
• Iris stromal hypoplasia
• Ectropion uveae
• Posterior polymorphous dystrophy
• Posterior embryotoxin/Axenfeld-Rieger Syndrome
• Peter’s Anomaly
• Ectopia lentis et Pupillae
Family arrives to bedside..
• Pt has a h/o Sturge-Weber syndrome with
glaucoma; pt is s/p tube shunt placement at the
age of 6, his left pupil has looked like that since
the surgery. Pt has had laser treatments of his
port wine stain.
• POH: as above
FH: no eye disease or SWS
• PMH: none, no seizures or mental retardation
• SH: unknown tobacco/alcohol/drug use
• Meds: Alphagan and Azopt OS, pt has not been
compliant with his medications
• ALL: NKDA
Dilated exam
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Lens: clear both eyes
Ant vit: clear both eyes
C/D: 0.2 OD, 0.65-0.7 OS
D/M/V/P: margins sharp both
eyes, pale disc OS, vitreous clear,
macula flat, periphery flat 360
degrees of view both eyes
Follow-up
• Pt was discharged, declined to return
for f/u
• F/u with local eye care provider
• No visual changes
• VA (sc) OD – 20/20
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OS – 20/50-2 (unchanged)
• IOP OS 25-28 range
Sturge-Weber Syndrome
(encephalotrigeminal angiomatosis)
• Phakomatosis (“mother-spot”)
• Hamartomatous hemangiomas of face,
eyelid, choroid, retina, meninges, brain
• Abnormal proliferation of blood vessels
containing AV shunts
• Rarely bilateral
• Involves parietal and occipital lobes
Comi et al (2005):
• Found altered fibronectin expression in
fibroblasts taken from port wine stains and
brain compared to normal tissue from
same subjects
• Fibronectin has effects on angiogenesis,
vessel remodeling, and vessel innervation
density
• No known genetic abnormality, possibly a
somatic mutation
Comi AM, Weisz CJ, Highet BH, Skolasky RL, Pardo CA, Hess EJ.Sturge-Weber syndrome: altered blood
vessel fibronectin expression and morphology.
J Child Neurol. 2005 Jul;20(7):572-7.
Statistics
• No known incidence
• No known race or sex predilection
• No known prenatal or environmental
risk factors
• Normal life expectancy
• Symptoms worsen with age
• Prognosis excellent to guarded
“Catsup Fundus”
Doan A, Kwon YH: Sturge-Weber Syndrome: 4 y.o. child with a history of seizures and glaucoma. February
21, 2005 [cited –8/18/06-- ]; Available from: http://webeye.ophth.uiowa.edu/eyeforum/cases/case13.htm
Sturge-Weber Glaucoma
• Glaucoma in 30-70%, most common with
involvement of upper lid
• Congenital (60%), childhood (30%)
• Later onset of sx usually between age 8-20
• May present with photophobia, epiphora,
blepharospasm, Haab’s striae, clouding of
cornea, buphthalmos, amblyopia
• Retinal and choroidal detachments
Pathophysiology
In 1973, Weiss proposed the following:
• Congenital cases caused by abnormal
angles and increased episcleral venous
pressure
• Childhood/adulthood cases caused by
increased episcleral venous pressure only
• Elevated IOP directly related to
decreased aqueous outflow and/or
elevated EVP
Weiss DI. Dual origin of glaucoma in encephalotrigeminal haemangiomatosis. Trans Ophthalmol Soc UK.
1973;93:477-493
Angle Abnormalities
• Scleral spur poorly developed
• Thickening of uveal and trabecular
meshwork
• Displacement of iris root anteriorly
• Attachment of ciliary muscle directly
to TM
• Neovascularization or fibrosis of the
juxtacanalicular region
Aqueous outflow
• Aqueous drains from the anterior chamber
through two routes: the conventional route
(TM to SC) and uveoscleral outflow
• Hemangiomas upstream cause ↑ blood
flow and pressure leading to elevated EVP
Episcleral venous pressure
• Normal EVP = 9 +/- 1.5 mmHg
• SC fills with blood when EVP>IOP
(IOP is usually 5-6 mmHg higher)
• AV shunts
• Thyroid orbitopathy, artifact, body
position or idiopathic
• Ocular hypotony
Treatment
• Resistant, may need repeat surgery
• Congenital cases: surgical; initial or
repeated goniotomy/trabeculotomy, if
ineffective, filtration surgery, shunt
• Later-onset: medical tx first, then
trabeculectomy with MMC, shunt
• Diode cyclophotocoagulation last
resort
Complications
• Sudden hypotony can cause large choroidal
effusion or expulsive hemorrhage
• Prophylactic posterior sclerotomy,
viscoelastic to AC or tight scleral flap
sutures
• Increased risk for anesthesia complications
including intracerebral bleed and DIC
Mandal et al. (1999):
• Retrospective study
• 9 patients (10 eyes) with SWS glaucoma
• Primary combined trabeculotomytrabeculectomy without posterior
sclerotomy performed by one surgeon
• No intra-op complications
• Post-op one pt developed shallow AC with
choroidal detachment managed
conservatively
Mandal AM. Primary Combined Trabeculotomy-Trabeculectomy in Sturge-Weber Syndrome.
Ophthalmology. 1999;1621-27.
Agarwal et al (1993):
• Retrospective study
• 16 pts (19 eyes) with SWS glaucoma
• Primary combined trabeculotomy-trabeculectomy
performed in 18 eyes; repeat surgery in three eyes
• After average f/u of 42 mo, IOP was ≤ 22 mmHg in
11 eyes (61.1%)
• Intra-op complications: hyphema in four eyes
(22.2%), vitreous loss in three eyes (16.7%)
• Post-op complications: choroidal detachment in
three eyes (16.7%); vitreous hemorrhage in one
(5.6%)
Agarwal HC, Sandramouli S, Sihota R, and Sood NN. Sturge-Weber syndrome: management of glaucoma with combined
trabeculotomy-trabeculectomy. Ophthalmic Surg. 1993:24:399–402
Take home points
• Pre-existing conditions/interventions can
be confused for acute findings in the
trauma setting
• Certain conditions place pts at risk for
complications of surgery and anesthesia
References
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Agarwal HC, Sandramouli S, Sihota R, and Sood NN. Sturge-Weber syndrome: management of
glaucoma with combined trabeculotomy-trabeculectomy. Ophthalmic Surg. 1993:24:399–402.
Bellows AR, Chylack LT, Epstein DL, et al. Choroidal effusion during glaucoma surgery in
patients with prominent episcleral vessels. Arch Ophthalmol. 1979;97:493-497
Board, RJ, Shields, MB: Combined trabeculotomy-trabeculectomy for the management of
glaucoma in Sturge-Weber Syndrome. Ophthalmic Surg 12:813. 1981.
Cibis GW, Tripathi RC, Tripathi BJ. Glaucoma in Sturge-Weber syndrome. Ophthalmology.
1984;91:1061-1071
Comi AM, Weisz CJ, Highet BH, Skolasky RL, Pardo CA, Hess EJ.Sturge-Weber syndrome:
altered blood vessel fibronectin expression and morphology.
J Child Neurol. 2005 Jul;20(7):572-7.
Doan A, Kwon YH: Sturge-Weber Syndrome: 4 y.o. child with a history of seizures and
glaucoma. February 21, 2005 [cited –8/18/06-- ]; Available from:
http://webeye.ophth.uiowa.edu/eyeforum/cases/case13.htm
Kanski J. Glaucoma. In: Clinical Diagnosis in Ophthalmology. First ed. Philadelphia: Elsevier;
2006:265-98.
Mandal AM, and Gupta N. Patients with Sturge–Weber syndrome. Ophthalmology. 2004;111:
606.
Olsen KE, Huang AS, Wright MM. The efficacy of goniotomy/trabeculotomy in early-onset
glaucoma associated with the Sturge-Weber Syndrome. JAAPOS 1998;2:365-8.
Phelps CD. Arterial anastomosis with Schlemm's canal: a rare cause of secondary open-angle
glaucoma. Trans Am Ophthalmol Soc. 1985;83:304-15.
Phelps CD. The pathogenesis of glaucoma in Sturge-Weber syndrome. Ophthalmology
1978;85:276-86
Weiss DI. Dual origin of glaucoma in encephalotrigeminal haemangiomatosis. Trans
Ophthalmol Soc UK. 1973;93:477-493