 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.)
 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
 In
all developmental glaucoma, there is
maldevelopment of iridocorneal angle
(goniodysgenesis) which includes:1) Trabeculodysgenesis: maldevelopment of
the trabecular meshwork
2) Corneodysgenesis: maldevelopment of the
cornea
3) Iridodysgenesis: maldevelopment of the
iris.
 The hallmark for developmental glaucoma is
isolated trabeculodysgenesis.
Normal trabecular meshwork
Underdeveloped trabecular meshwork (Trabeculodysgenesis)
 Early:


Triad of photophobia, epiphora and blepharospasm
Due to corneal edema
 Late:



Hazy cornea
Large eye “ox eye”
Defective vision
 Cornea



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
 Anterior

chamber
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
 Fundus

Glaucomatous optic cupping (late)
 Refraction

Axial myopia, but less than expected, due to:1) Flat cornea
2) Flat lens
3) Deep anterior chamber and the lens is
relatively become more posterior in position
Increased tear lake in the left eye
Increased corneal diameter of the left eye in
comparison with the right eye
Hazy cornea
Haab's striae as seen on retroillumination
Haab’s striae
Haab’s striae
Dense opacification of cornea in
advanced cases
Stromal edema superiorly (arrow)
Positive family history
 Corneal assessment





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:1) Effect of general anaesthesia
2) Low scleral rigidity
3) Flat cornea

 Gonioscopy


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
Tono Pen
Examination of a 3 months
infant with buphthalmos
under general anesthesia
using Schiotz tonometer.
Perkins handheld tonometer
Treatment of buphthalmos is only surgical :
 Goniotomy
 Trabeculotomy
 Trabeculectomy
 Drainage devices
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.
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.
Goniotomy
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.

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.
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 :
1. Blurring of vision
2. Bleeding in the eye
3. Sudden, permanent loss of central vision
4. Infection in the eye
5. Malignant glaucoma is rare
Trabeculotome
Trabeculotomy
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.
Procedures of trabeculectomy
Trabeculectomy
 such
as Molteno- seton implant or Ahmad’s
valve are indicated in difficult recurrent
cases where conventional procedure above
failed.
Molteno implant