CONGENITAL GLAUCOMA They are a group of diverse disorders in which abnormal high intraocular pressure results due to the developmental abnormalities of the angle of anterior chamber obstructing the drainage of aqueous humour . EPIDEMIOLYOGY AND DEMOGRAPHICS > Incidence-1 in 10-15000 live births >75% cases are bilateral >65% are male >75% present in 1 st year of life
CLASSIFICATION 1.Primary congenital glaucoma(without associated anomalies of eye) >New born glaucoma(true congenital glaucoma)-IUL to birth. >Infantile glaucoma- upto 3 yrs >Juvenile glaucoma-3 to 15 yrs 2.Secondary congenital glaucoma(with associated anomalies) Like:-Glaucoma associated with iridocorneal dysgenesis -Glaucoma associated with aniridia -Glaucoma associated with ectopia lentis syndrome -Miscellaneous conditions
PATHOGENESIS Primary congenital glaucoma is due to failure of or abnormal development of the trabecular meshwork. Maldevelopment of trabeculum incuding the iridotrabecular junction( trabecular dysgenesis i s responsible for impaired aqueous outflow resulting in raised IOP. Trabeculodysgenesis is characterised by absence of the angle recess with iris having a flat concave direct insrtion into the surface. The iris may not completely separate from the cornea that the angle remains closed by persistent embryonic tissue.
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PATHOGENESIS OF GLAUCOMATOUS OCULAR DAMAGE Main theories are:- 1)Mechanical changes due to the raise of IOP 2)Vascular perfusion of optic nerve head 3)Defective autoregulation
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CLINICAL FEATURES Symtopms :Classsically characterised by triad of : photophobia,lacrimation,blepharospasm These are thought to be caused by irritation of corneal nerves, which occurs as result of elevated IOP. Photophobia is usually the initial sign,but is not enough by itself to arouse suspicion in most cases.
SIGNS: 1.Corneal signs- oedema,enlargement and descemets breaks( Habbs striae ) 2.sclera-becomes thin and appears blue due to underlying uveal tissues. 3.Anterior chamber-becomes deep. 4.Iris-May show Iridodonesis and atrophic patches in late stage. 5.Lens-Becomes flat due to stretching of zonules snd even subluxate . 6.Optic disc-May showvariable cupping and atrophy especially after 3 rd year. 7.IOP-Is raised which is nether marked nor acute. 8.Axial myopia 9.Eyeball-Shows Buphthalmos
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EAMINATION Shows:- reduced visual acuity - Buphthalmos -Corneal diam. >12mm -Corneal oedema - Haab’s striae * Perkins applanation tonometer -Elevated IOP(normal IOP-10to 15 mmHg) * Slit L amp Examination:-deep anterior chamber * Ophthalmoscopy :-optic disc cupping and atrophy later. * Gonioscopic examination of angle of anterior chamber reveals:- Trabeculodysgenesis with either flat or concave iris insertion.
DIFFERENTIAL DIAGNOSIS - Nasolacrimal duct obstruction -X-linked megalocornea without glaucoma -Birth trauma -Retinoblastoma -Corneal dystrophies and dysgenesis
MANAGEMENT Medical: -Temporary mesures to control IOP and to clear cloudy cornea prior to surgery. > Hyperosmotic agents like mannitol > Carbonic anhydrase inhibitors- mannitol > Beta blockers – timolol > Alpha 2 agonists- brimonidine
SURGICAL MANAGEMENT They are the mainstay of treatment. Options are-: Goniotomy -: Trabeculotomy -: Trabeculectomy -:Combined Trabeculectomy and Trabeculotomy -:Shunt procedures
GONIOTOMY Involves making a horizontal incision at the midpoint of the superficial layers of the trabecular meshwork. May need to be repeated. Eventual success rate is about 85%. Results are poor if the corneal diameter is 14mm or more because Sclemm’s canal obliterated.
PROCEDURE The goniotomy surgery involves entering the anterior chamber with a sharp goniotomy knife and making an opening incision through the abnormally developed trabecular meshwork to allow greater outflow o f the aqueous fluid and there by lowers the IOP.
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TRABECULOTOMY Done if corneal clouding prevents visualization of the angle or when repeated goniotomy has failed. Partial thickness sclera flap is fashioned. Schlemm canal is found and a trabeculotome is inserted into schlemm canal and then rotated into the anterior chamber. Technically highly demanding,requires previous experience,and good anatomical landmarks to achieve predictable results. Schlemm canal may be difficult to canalize because of hypoplasia or angle anomaly. Trabeculotomy surgery involves making an external incision and identifying the schlemm’s canal and breaking through the trabeculae meshwork to increase the aqueous outflow. Typically,120-140 degrees of trabecular meshwork can be treated by Trabeculotomy in a single surgery.