This document discusses malignant glaucoma, a condition characterized by a shallow anterior chamber and elevated intraocular pressure despite a patent iridectomy. It occurs most commonly after glaucoma surgery. Several theories exist for its pathogenesis, involving aqueous misdirection posteriorly due to cilio-lenticular or cilio-vitreal blocks. Clinical features, diagnosis, differential diagnosis, and management approaches are described, including medical therapy with cycloplegics and hyperosmotics, laser treatments, and surgical interventions like vitrectomy. Preventing recurrence in the fellow eye is also addressed.

1. Malignant Glaucoma
DR SHYLESH B DABKE
GLAUCOMA FELLOW
ARAVIND EYE HOSPITAL, TIRUNELVELI
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2. Introduction
• Not one disease but a group of disease with a common phenotype.
• Flat AC + High IOP after surgery for ACG
• “Malignant Glaucoma” – Often refractory to treatment
• “Aqueous Misdirection” – Fluid flows down the easiest route
• Von graefe – 1869

3. • Most of the time aqueous flows forward into the trabecular meshwork however in aqueous
misdirection aqueous is directed posteriorly

4. Many possible mechanisms in aqueous misdirection
contact of ciliary body with lens and/or ant. hyaloid face
blockage at the level of ciliary sulcus
aqueous then flow the easiest way - Posteriorly
ant. Hyaloid act as a one way valve
trapping fluid in the posterior segment

5. A post-operative condition characterized by:
1. Axial as well as peripheral shallowing of the anterior chamber.
2. An accompanying rise of IOP due to secondary angle closure in the presence of
3. In the presence of a patent iridectomy
4. No response or even aggravation by miotics.

6. Other Names
• Ciliary block glaucoma
• Cilio -lenticular block glaucoma
• Aqueous misdirection syndrome
• Direct lens block angle closure
• Until a better understanding of the pathomechanism of the condition is attained,the term ‘malignant
glaucoma’ expresses its seriousness and will continue to be used.

7. Prevalence
• 2% to 4% - h/o of acute or chronic angle-closure glaucoma that have undergone
filtration surgery
• 1.3 % - Glaucoma surgery alone or combined with cataracts
• 2.3%- Penetrating surgery
• Women are three times more likely than men

8. Predisposing Factors
• Axial hyperopia
• Nanophthalmos
• Chronic angle closure with plateau iris configuration
• History of malignant glaucoma in the fellow eye

9. Theories
Shaffer and
Hoskins
Quigley et al
Epstein et al
Chandler et al

10. Shaffer and Hoskins* – Posterior pooling of
aqueous
Posterior diversion of aqueous flow
Accumulation of Aqueous
behind a posterior vitreous detachment
Secondary forward movement of the iris-lens diaphragm
R. N. Shaffer and H. D. Hoskins, “The role of vitreous detachment in aphakic and malignant glaucoma,” Transactions of
theAmerican Academy of Ophthalmology and Otolaryngology, vol. 58, pp. 217–228, 1954.

11. Cilio-lenticular(cilio-vitreal)block
Tips of the ciliary processes rotate forward and press
against the lens equator (in phakic eye) or against the
anterior hyaloid (in aphakia)
Obsruction to the forward flow of aqueous
• G. E. Trope, C. J. Pavlin, A. Bau, C. R. Baumal, and F. S. Foster, “Malignant glaucoma: clinical and ultrasound biomicroscopic
features,” Ophthalmology, vol. 101, no. 6, pp. 1030–1035, 1994.
• C. Tello, T. Chi, G. Shepps, J. Liebmann, and R. Ritch, “Ultrasound biomicroscopy in pseudophakic malignant glaucoma,”
Ophthalmology, vol. 100, no. 9, pp. 1330–1334, 1993.

12. Anterior Hyaloid Obstruction - Epstein and
colleagues
• May contribute to the cilio-lenticular block
Breaks in the hyalid near the vitreous base.
Possibly allow the posterior diversion of the aqueous
One-way valve effect : fluid coming posteriorly closes the
vitreous face against the ciliary body,preventing forward
flow

13. Chandler & Grant* - Slackness of lens zonules
Laxity of lens zonules coupled with pressure from the
vitreous leads to forward lens movement
A vicious circle is set up in that the higher the pressure in the
posterior segment, the more firmly the lens is held forward
P. A. Chandler, “Malignant glaucoma,” American Journal of Ophthalmology, vol. 34, no. 7, pp. 993–1000,1951
This concept that the lens
subsequently pushes the
peripheral iris into the AC angle
led to the proposed term
“Direct lens block angle
closure.”

14. Quigley et al* - Choroidal expansion
The precipitating event which increases
vitreous pressure is choroidal expansion
The initial compensatory outflow of aqueous along the
posteroanterior pressure gradient causes shallowing of the
anterior chamber
• H. A. Quigley, D. S. Friedman, and N. G. Congdon, “Possible mechanisms of primary angle-closure and malignant glaucoma,”
Journal of Glaucoma, vol. 12, no. 2, pp. 167–180, 2003.
• H. A. Quigley, “Angle-closure glaucoma—simpler answers to complex mechanisms: LXVI Edward Jackson Memorial Lecture,”
American Journal of Ophthalmology, vol. 148, no. 5, pp. 657–669.e1, 2009.

15. Final common pathway……..
Fluid build up behind the vitreous leads to vitreous condensation which exerts a
forward force
Establishment of a vicious cycle whereby the transvitreal pressure cannot be equalised by outflow of
aqueous humour
Anterior displacement of the lens-iris diaphragm
An attack of malignant glaucoma

16. Clinical Forms
• Classical Malignant glaucoma.
• Malignant glaucoma in Aphakia.
• Malignant glaucoma in Pseudophakia.
• Miotic induced malignant glaucoma.
• Malignant glaucoma associated with bleb needling.
• Malignant glaucoma associated with infection and inflammation.
• Malignant glaucoma associated with other ocular disorders.
• Spontaneous Malignant glaucoma.
¹ SHIELDS Textbook of Glaucoma -- 6th Edition

17. Classical Malignant Glaucoma
• Prototype and most common type.
• Typically occurs unilaterally following incisional surgical intervention for angle closure
glaucoma.
• Occurs in 0.6 to 4 % of these cases.¹
• R. J. Simmons, “Malignant glaucoma,” British Journal of Ophthalmology, vol. 56, no. 3, pp. 263–272, 1972.
• P. A. Chandler, “Malignant glaucoma,” American Journal of Ophthalmology, vol. 34, no. 7, pp. 993–1000, 1951

18. Risk factors
• Partial or total angle closure at the time of surgery.
• A previous angle closure attack.
• Shallow AC (axial hypermetropia) and PAS.
• Overfiltration following glaucoma surgery.
• Previous MG attack in other eye.
Classical Malignant Glaucoma
Never related to the type of incisional surgery or immediate pre-op IOP.

19. Pseudophakic malignant glaucoma
(A) aqueous pockets between the iris and anterior capsule;
(B) pockets within the capsular bag and lens implant;
(C) pockets between the posterior capsule and hyaloid face
(D) aqueous trapped within the vitreous cavity behind an intact hyaloid face.

20. Phakic / Aphakic malignant glaucoma

21. Miotic induced malignant glaucoma
• May correspond to the institution of miotic therapy.
• Exact mechanism not known.
• May be through contraction of the ciliary body or associated forward shift of the
lens with shallowing of the AC.

22. Clinical Features
• Symptoms – Pain, redness and photophobia.
• Myopic shift
• Shallowing or flattening of the AC, with an accompanying rise of IOP, despite patent PI.
• Recent history of intraocular surgery,laser surgery or use of miotics.
• No choroidal detachment or suprachoroidal hemorrhage, or iris bombe.
• No decrease of IOP in response to conventional antiglaucoma treatment

23. Diagnosis of Malignant glaucoma
• Essentially clinical.
• Can be confirmed by Fluoroscein Test.
- I.V Fluorescein is injected into the antecubital vein.
- In normal eyes,fluoroscein will not be seen to flow into the space behind the
lens,IOL or vitreous space.
- The diagnosis of MG is confirmed by noting the flow of fluoroscein tinged
aqueous into the posterior segment.

24. Imaging - UBM
• Swelling or anterior rotation of the ciliary body.
• Forward rotation of the lens-iris diaphragm.
• Direct angle closure by physical pushing of the iris against the trabecular
meshwork.

25. ASOCT

26. Differential diagnosis
1. Pupillary block glaucoma
2. Suprachoroidal hemorrhage
3. Choroidal effusion
4. Acute primary angle closure glaucoma
5. Phacomorphic glaucoma

27. Pupillary block glaucoma
• Closure of anterior chamber angle
• Laser peripheral iridotomy is the treatment of choice
• Unlike malignant glaucoma the anterior chamber usually remains deeper in the center
than on its circumference

28. Suprachoroidal hemorrhage
• Introperative or early post operative period
• IOP normal or elevated
• AC shallow- central and peripheral
• Fundus- bullous light brown choroidal elevations
• No relief by iridectomy

29. Choroidal effusion
• Introperative or early post operative period
• IOP low
• AC shallow- central and peripheral
• Fundus- bullous dark brown choroidal elevations
• No relief by iridectomy

30. Acute primary angle closure glaucoma
• Shallowing of the anterior chamber occurs symmetrically
• Sudden increase in IOP
• Microcystic edema of the cornea
• Conjunctival injection

31. Phacomorphic Glaucoma
• AC is more shallow in the affected eye.
• Associated with advanced cataract or a partially dislocated lens.
• No precipitating event such as the use of a miotic or surgery.

32. MANAGEMENT
MEDICAL
LASER
PROCEDURES
SURGICAL
TREATMENT OF
FELLOW EYE

33. • Serious potentially sight threatening condition
• Admission
• The first step is to make an accurate diagnosis and exclude the differential diagnoses
• If the patency of the iridectomy is in doubt, a repeat laser iridotomy can be performed to
exclude pupil block.

35. MEDICAL MANAGEMENT
• Initial medical therapy is directed at
Simmons RJ, Thomas JV, Yaqub Malignant glaucoma. In: Ritch R, Shields MB, Krupin T, eds. The glaucomas. St Louis: CV Mosby,
1251–63.
- lowering IOP with aqueous suppressants
- Attempting posterior displacement of the lens-iris diaphragm (and thus
helping to break the ciliary block) with a strong cycloplegic such as atropine.
- shrinking the vitreous with hyperosmotic agents
The effect of medical therapy is often not immediate, but approximately 50 percent of cases will be relieved
within five days.*

36. Recommended treatment regimen
• Cycloplegia:
- Atropine(1%) eye drop TDS
Paralysis sphincter muscle of ciliary body – leads to increased tension in zonules, flattens
lens, moves lens posteriorly – deepens AC.
In some cases, Atropine is needed upto one year to avoid recurrence.
- Phenylephrine(2.5%) eye drops QID
Tightens zonular complex by contraction of longitudinal muscle of ciliary body
- Subconjunctival mydricaine
• MIOTICS – CONTRAINDICATED promoting zonular relaxation and encourage forward lens
movement.
• Anti-Inflammatory Medication:
- Topical steroids can help to reduce inflammation

37. For shrinkage of vitreous and/or reduction of
aqueous production
• Beta blocker
• Alpha agonist-apraclonidine 1 % bd
• Oral acetazolamide 250 mg QID
• Oral Glycerol 50% 0.5-1 ml/kg
• IV mannitol 1 -2g/kg

38. Medical management - Principle
• Once the anterior chamber deepens and the intraocular pressure has been normalised,
medical treatment can be gradually withdrawn.
If high pressure continues after 5 days/
if lens-corneal touch occurs
Peripheral anterior synechiae, posterior
synechiae, cataract, and damage to the
corneal endothelium
surgical intervention should be considered

39. Laser treatment
1. Argon laser photocoagulation of ciliary processes.
2. Nd: YAG Laser assisted disruption of anterior hyaloid face or the posterior lens capsule
and hyaloid face.
3. Laser peripheral iridotomy.

40. Trans scleral cyclophotocoagulation
• If the ciliary processes are visible, argon laser photocoagulation can be used to shrink
the ciliary processes.
• Not a highly useful technique because the visibility of ciliary processes is rare.
• Limited to patients whose glaucoma has been resistant to medical and surgical
therapies, with no potential for improvement in visual acuity.

41. Nd: YAG laser assisted disruption of
posterior capsule/anterior hyaloid face
• Mechanism involves puncturing the anterior hyaloid face and providing a new communication
between sequestered aqueous and the anterior chamber.
• Through a large peripheral iridotomy or iridectomy, or through the pupil.
• The AC will start to deepen as soon as the anterior hyaloid face is disrupted

42. • Nd:YAG laser carries a high risk of lenticular damage. A patent PI, good visualization & precise
focusing are crucial.
Phakic
Eyes
Pseudophakic or
Aphakic eyes
• Nd:YAG laser capsulotomy with disruption of the anterior hyaloid face is often effective.

43. Surgical management
• Refractory to medical and laser therapy, surgical intervention to remove the vitreous is
necessary to increase aqueous flow into the anterior chamber.
• The success of this was first described by Chandler.

44. Chandler's Malignant Glaucoma Procedure
• Posterior sclerostomy
• An 18-gauge needle 1 to 1.5 mL of fluid is aspirated.
• AC formed with Air bubble

45. - If signicant cataract – limited core Vitrectomy – Phacoemulsification with lens implantation-
residual Vitrectomy – with hyaloido-zonulectomy-VPV)
Pseudophakic
Eyes
Phakic Eye
- Anterior pars plana vitrectomy + anterior hyaloidotomy
– pars plana vitrectomy ± lensectomy
Phakic Eye –
Cataractous

46. • Core vitrectomy surgery leads to resolution of malignant glaucoma
• This probably reflects the lack of effective removal of the anterior hyaloid in phakic eyes because of the
risk of lens damage and subsequent cataract formation.
Pseudophakic
eyes 65–90%
Phakic eyes
25–50%

47. • In the presence of a clear lens, treatment is rather more controversial
• Clear lens extraction should certainly be considered and discussed with the patient
Pseudophakic
eye
Phakic eye-
cataractous lens
Phakic eye with
clear lens
• Level of difficulty in surgical management

48. Management of fellow eye
• After an episode of malignant glaucoma in one eye, there is a high risk of this
complication occurring in the fellow eye after a surgical intervention.
• The patient should be warned of this when consent is taken for surgery on the fellow
eye.
• If the fellow eye has an occludable angle - perform a Laser PI.

49. Protection of the fellow eye from an attack
• Pre-op
- Avoid miotic usage.
• Intra -op
- Adminiser 4 % atropine and 2.5 % phenylephrine intraoperatively – pupil is
dilated and ciliary body is paralyzed before surgery is completed.
- Prevent AC shallowing intra-op – maintain a closed system or through the use of
a viscoelastic material at the time of surgery.
• Post -op
- Avoid hypotony and overfiltration.

50. Prevention of an attack in predisposed eyes
• Identify eyes at risk , preoperatively.
• In nanophthalmos, prophylactic sclerotomies may be required.
• Maintenance of anterior chamber depth both intra and post operatively.
• Aqueous overdrainage must be avoided in the early postoperative period by having a high
scleral flap resistance – tight scleral flap sutures and judicious removal.
• Topical atropine at the end of the surgery and postoperatively.
• Discontinuation of cycloplegics should be undertaken with care in any patient considered at
high-risk of MG.

51. Conclusion
• The precise mechanism of malignant glaucoma remains unclear but it is almost certainly
closely related to the anatomical relation between lens, zonules, anterior vitreous face, and
ciliary body.
• Reversal of aqueous misdirection is dependent on direct continuity between the vitreous cavity
and anterior chamber which is difficult to achieve in the presence of a lens which is often
relatively large.
• The management is controversial until the exact mechanism is more clearly understood.
• In the light of modern microsurgical techniques, reversal of ciliary block glaucoma can be
achieved with preservation of good visual acuity and intraocular pressure control