juvenile glaucoma: a case study and disease review
TRANSCRIPT
Ophthalmic Case PresentationLYNDON WOYTUCKMBBS4 PROGRAMME AT ST GEORGE’S UNIVERSITY OF LONDON DELIVERED BY THE UNIVERSITY OF NICOSIASHEBA MEDICAL CENTER AT TEL HASHOMER
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 have TRAB 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
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
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.
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
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
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
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
References• Medscape. “Juvenile Glaucoma” Accessed from:
http://emedicine.medscape.com/article/1207051-overview• Teresa Chen. Harvard “Glaucoma Tube Shunt
Procedures” Accessed from: http://www.djo.harvard.edu/site.php?url=/patients/pi/421