Prepared by Khansa Mohd Rashid Norhana Rahmat Definition Increase in IOP restricted to developmental abnormalities in trabecular meshwork in the angle of the eye in absence of other secondary causes or related systemic congenital anomalies ID: 193823
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BUPHTHALMOS
Prepared by :Khansa’ Mohd RashidNorhana RahmatSlide2
Definition
Increase in IOP restricted to developmental abnormalities in trabecular meshwork in the angle of the eye in absence of other secondary causes or related systemic congenital anomalies (e.g: Sturge-Weber syndrome, Neurofibromatosis, Lowe syndrome etc.) Slide3
Incidence
Only 1% from all glaucoma75% Bilateral65% Male80% diagnoses in the first year of lifeMost cases appear to be sporadicHowever, an autosomal recessive pattern of transmission of the disease is evidencedSlide4
Etiology
In all developmental glaucoma, there is maldevelopment of iridocorneal angle (goniodysgenesis) which includes:-Trabeculodysgenesis: maldevelopment of the trabecular meshwork Corneodysgenesis: maldevelopment of the cornea Iridodysgenesis: maldevelopment of the iris. The hallmark for developmental glaucoma is isolated trabeculodysgenesis. Slide5
Normal trabecular meshwork
Underdeveloped trabecular meshwork (Trabeculodysgenesis)Slide6
Symptoms
Early:Triad of photophobia, epiphora and blepharospasmDue to corneal edemaLate:Hazy corneaLarge eye “ox eye”Defective visionSlide7
Signs
CorneaLarge & hazyIncreased corneal diameter Normal = 10.5 mm Buphthalmos = > 12 mmHaab’s striae: transverse, tears in Descemet’s membraneScleraBluish discoloration due to thinning of scleraSlide8
Anterior chamber
Deep: due to bulging of cornea & flattening of lensIrisIridodenesis (tremulous)May have stromal hypoplasiaPupilSluggish reactionLensPhacodenesis (tremulous)Relatively small and displaced posteriorlyFlattened due to stretching of the suspensory ligamentsSlide9
Fundus
Glaucomatous optic cupping (late)RefractionAxial myopia, but less than expected, due to:-Flat cornea Flat lens Deep anterior chamber and the lens is relatively become more posterior in position Slide10
Increased tear lake in the left eye
Increased corneal diameter of the left eye in comparison with the right eyeSlide11
Haab’s
striaeHaab’s striaeHaab's striae as seen on retroillumination
Hazy corneaSlide12
Dense
opacification of cornea in advanced casesStromal edema superiorly (arrow)Slide13
Diagnosis
Positive family historyCorneal assessmentCorneal diameter more than 12 mmHaab’s striaeHazy corneaTonometryPerkins handheld or Tono Pen is preferable in childrenIOP more than 21 mmHgIOP measured under general anaesthesia is less than real IOP due to:-Effect of general anaesthesiaLow scleral rigidityFlat corneaSlide14
Gonioscopy
Abnormal angle structures or membraneIris is directly inserted into trabecular meshwork (either flat insertion or concave insertion)OphthalmoscopyGlaucomatous cuppingCup to disc ratio is more than 0.3Slide15
Examination of a 3 months infant with buphthalmos
under general anesthesia using Schiotz tonometer. Tono PenPerkins handheld tonometerSlide16
Treatment
Treatment of buphthalmos is only surgical :Goniotomy TrabeculotomyTrabeculectomyDrainage devicesSlide17
Goniotomy
Indication : Clear cornea with diameter less than 13mm.How?Goniotomy knife introduced in the A.C under gonioscopic control to cut the abnormal mesodermal membrane at the angle in 2/5 of the circumference.Slide18
Result
Goniotomy can successfully treat congenital glaucoma 80% to 90% of the time in cases the symptoms start when the child is 1 month to 2 years old. Complication The greatest complication after goniotomy is a return of high pressure in the eyes. If the pressure in the eye increases, the procedure may need to be repeated. Slide19
GoniotomySlide20
Trabeculotomy
Indication Corneal diameter more than 13mm or less than 13mm with hazy cornea.How?Trabeculotomy is performed from the scleral side at the limbus under the conjunctival flap. A fine wire-like instrument (trabeculotome) is
inserted into
Schlemm’s
canal from an external
incision and the
trabecular
meshwork is torn
by rotating the instrument into the anterior
chamber.Slide21Slide22
Result
Trabeculotomy can successfully treat congenital glaucoma 80% to 90% of the time in cases the symptoms start at the age of one month to two years old. Trabeculotomy is not as successful in children whose glaucoma was present at birth or began late in childhood.Slide23
Complication
The most common problem after trabeculotomy is scarring of the new opening in the eye which prevents fluid from draining out of the eye. Other complications :Blurring of visionBleeding in the eyeSudden, permanent loss of central visionInfection in the eyeMalignant glaucoma is rareSlide24
Trabeculotomy
TrabeculotomeSlide25
Trabeculectomy
Indication After failure of trabeculotomy or in advanced cases with corneal diameter more than 13mm.How ?Trabeculectomy is a surgical procedure involves removal of part of the trabeculum in the eye to relieve pressure caused by glaucoma.Slide26
Procedures of trabeculectomySlide27
TrabeculectomySlide28
Drainage devices
such as Molteno- seton implant or Ahmad’s valve are indicated in difficult recurrent cases where conventional procedure above failed.Molteno implantSlide29
Thank you