A review of juvenile glaucoma including screening, diagnostic approach, and management. Patient details have been anonymised to protect the individual.

1. Ophthalmic Case
Presentation
LYNDON WOYTUCK
MBBS4 PROGRAMME AT ST GEORGE’S UNIVERSITY OF
LONDON DELIVERED BY THE UNIVERSITY OF NICOSIA
SHEBA MEDICAL CENTER AT TEL HASHOMER

2. Presentation to Emergency Department
• I.S. 32 year old male, married
• Presenting Complaint on Feb 27: Sharp pain in left eye since Feb 25, nausea,
headache, blurred vision
• Background: NKDA, no comorbidities
• History of Presenting Complaint: 4 years prior had blurred vision and IOP
was assessed at 42 in both eyes in community.Went on to haveTRAB
surgery. Bleb needling performed in right eye 10/2015 (IOP 14R, 31L) with
local treatment following surgery.
• LE CC 6/20, RE CC 6/8.5 (Myopia -8)
• Prescribed Alphagan (brimonidine) 2x1/day, and Xalacom
(latanoprost/timolol) 1x1/day among others

3. Differential
• Primary developmental glaucoma (in children)
• Primary open angle glaucoma
• Angle closure glaucoma (acute or chronic)
• Secondary acquired glaucoma
• Inflammatory, Phacogenic,Traumatic, Drug induced, Intraocular haemorrhage,
Neovascular

4. Juvenile Glaucoma
• Rare juvenile onset open angle glaucoma with autosomal dominant
transmission
• Epidemiology: 1 in 50 000 in USA
• Pathophysiology:
• Impaired outflow of aqueous humour through the canal of Schlemm causes increased
IOP, with thickened and abnormal deposition of extracellular tissue between the
anterior chamber and canal on pathology.
• The myocilin gene relates to the trabecular meshwork inducible glucocorticoid
response (TIGR) in patients with juvenile glaucoma.This codes for the glycoprotein
myocilin that is found in the trabecular meshwork and other ocular tissues.The
normal function of myocilin (MYOC) and its role in causing glaucoma is
undetermined. Also, CYP1B1 familial may relate to consanguineous cases.

5. Juvenile Glaucoma
• Presentation:
• When carefully monitored in families known to have the condition, increased IOP begins at 5-10
years, but in sporadic or previously unknown cases onset is usually found in adolescence or
early adulthood
• Family history of glaucoma over 2 generations or in a parent and sibling (early diagnosis)
• Asymptomatic until glaucoma is advanced
• Myopia in 50%
• On exam:
• Increased IOP bilaterally
• Myopia
• Optic disc damage (cupping)
• Visual field loss
• Slit lamp examination - Normal
• Gonioscopy - Normal, open angles, occasionally prominent uveal processes

6. Management
• Workup: Glaucoma assessment –Tonometry, Field testing (perimetry), fundus
photography or ophthalmoscopy, and retinal tomography
• Emergent care may include Acetazolamide, glycerol 50% solution PO, Mannitol IV
• Medical:Topical glaucoma medications may temporally control IOP.The rising IOP
may become resistant to all medications and dictate a need for eye surgery (1-3
years after symptom onset)
• Beta-blockers (Timolol), Prostaglandin agonists (Latanoprost), or combination with an alpha2-
agonist (brimonidine [Combigan]) or carbonic anhydrase inhibitor (brinzolamide, [Simbrinza])
• Surgical: Laser trabeculoplasty,Trabeculectomy, Drainage implant, Deep
sclerectomy, Goniotomy
• This patient had trabeculectomy with a needling revision in the right eye, now
having a drainage implant surgery in his left eye after being given emergent care,
brimonidine, dorzolamide/timolol (Cosopt), latanoprost and multiple analgesics

7. Tube shunt surgery
• Refractory glaucoma in which medications and other
procedures have failed
• Glaucoma tubes or shunt devices come in many different
varieties (ie. Ahmed, Molteno, Baerveldt, Krupin, Schocket,
etc.). All are implanted in the eye to provide an artificial
drainage from the eye to equalize the IOP
• An incision is made underneath the conjunctiva and the body
of the device is placed over the sclera within the orbit and
sutured in place.The tube extends from the body of the device,
and is inserted into the anterior chamber.
• Regular follow up by the surgeon until the eye completely
heals: 6 to 8 weeks. Meanwhile, frequent and multiple eye
drops and restriction from strenuous activities.
• The need for further IOP lowering treatment after surgery
varies

8. Prognosis
• This patient
• Careful observation after surgery
• Inform of risk for children
• In general
• Regular eye exams indefinitely
• Regular follow up for candidate children or with myopia and borderline IOP
• Early diagnosis is key

9. References
• Medscape. “Juvenile Glaucoma”Accessed from:
http://emedicine.medscape.com/article/1207051-overview
• Teresa Chen. Harvard “GlaucomaTube Shunt Procedures”Accessed
from: http://www.djo.harvard.edu/site.php?url=/patients/pi/421