1. Prepared by : Khansa’ Mohd Rashid Norhana Rahmat
BUPHTHALMOS
Prepared by :
Khansa’ Mohd Rashid
Norhana Rahmat

2. 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.)

3. Incidence Only 1% from all glaucoma 75% Bilateral 65% Male
80% diagnoses in the first year of life
Most cases appear to be sporadic
However, an autosomal recessive pattern of transmission of the disease is evidenced

4. 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.

5. Underdeveloped trabecular meshwork (Trabeculodysgenesis)
Normal trabecular meshwork
Underdeveloped trabecular meshwork (Trabeculodysgenesis)

6. Symptoms Early: Late: Triad of photophobia, epiphora and blepharospasm
Due to corneal edema
Late:
Hazy cornea
Large eye “ox eye”
Defective vision

7. Signs Cornea Sclera Large & hazy Increased corneal diameter
Normal = 10.5 mm
Buphthalmos = > 12 mm
Haab’s striae: transverse, tears in Descemet’s membrane
Sclera
Bluish discoloration due to thinning of sclera

8. Anterior chamber Iris Pupil Lens
Deep: due to bulging of cornea & flattening of lens
Iris
Iridodenesis (tremulous)
May have stromal hypoplasia
Pupil
Sluggish reaction
Lens
Phacodenesis (tremulous)
Relatively small and displaced posteriorly
Flattened due to stretching of the suspensory ligaments

9. Fundus Refraction Glaucomatous optic cupping (late)
Axial myopia, but less than expected, due to:-
Flat cornea
Flat lens
Deep anterior chamber and the lens is relatively become more posterior in position

10. Increased tear lake in the left eye
Increased corneal diameter of the left eye in comparison with the right eye

11. Haab's striae as seen on retroillumination
Hazy cornea
Haab’s striae
Haab’s striae
Haab's striae as seen on retroillumination

12. Dense opacification of cornea in advanced cases
Stromal edema superiorly (arrow)

13. Diagnosis Positive family history Corneal assessment Tonometry
Corneal diameter more than 12 mm
Haab’s striae
Hazy cornea
Tonometry
Perkins handheld or Tono Pen is preferable in children
IOP more than 21 mmHg
IOP measured under general anaesthesia is less than real IOP due to:-
Effect of general anaesthesia
Low scleral rigidity
Flat cornea

14. Gonioscopy Ophthalmoscopy Abnormal angle structures or membrane
Iris is directly inserted into trabecular meshwork (either flat insertion or concave insertion)
Ophthalmoscopy
Glaucomatous cupping
Cup to disc ratio is more than 0.3

15. Perkins handheld tonometer
Tono Pen
Examination of a 3 months infant with buphthalmos under general anesthesia using Schiotz tonometer.
Perkins handheld tonometer

16. Treatment Treatment of buphthalmos is only surgical : Goniotomy
Trabeculotomy
Trabeculectomy
Drainage devices

17. Goniotomy Indication : How? Clear cornea with diameter less than 13mm.
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.

18. Result Complication 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.

19. Goniotomy

20. Trabeculotomy How? 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.

22. 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.

23. 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 vision
Bleeding in the eye
Sudden, permanent loss of central vision
Infection in the eye
Malignant glaucoma is rare

24. Trabeculotome
Trabeculotomy

25. Trabeculectomy Indication How ?
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.

26. Procedures of trabeculectomy

27. Trabeculectomy

28. Drainage devices
such as Molteno- seton implant or Ahmad’s valve are indicated in difficult recurrent cases where conventional procedure above failed.
Molteno implant

29. Thank you