Year : 1991 | Volume
: 39 | Issue : 3 | Page : 118--121
Keratomycotic malignant glaucoma
Thomas Kuriakose, Philip A Thomas
Institute of Ophthalmology, Joseph Eye Hospital, Tiruchirapalli- 620 001, India
Institute of Ophthalmology, Joseph Eye Hospital, Tiruchirapalli- 620 001
Malignant Glaucoma due to Keratomycosis is a devastating and poorly recognised complication occurring in a small percentage of treated patients. It is characterized, in cases of Keratomycosis by a raised tension, uniform shallowing of the anterior chamber and a fungus-exudate-iris mass covering the pupillary area. Three cases of «SQ»Keratomycotic Malignant Glaucoma«SQ» are discussed here. Two of these were successfully treated with therapeutic keratoplasty, extracapsular lens extraction and systemic antifungals. The development of malignant glaucoma after a therapeutic keratoplasty which occurred in one case has not previously been reported. All the three cases which developed malignant glaucoma had a pupillary size of 4 mm diameter or less and grew Fusarium from the cornea and anterior chamber.
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Kuriakose T, Thomas PA. Keratomycotic malignant glaucoma.Indian J Ophthalmol 1991;39:118-121
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Kuriakose T, Thomas PA. Keratomycotic malignant glaucoma. Indian J Ophthalmol [serial online] 1991 [cited 2022 Aug 15 ];39:118-121
Available from: https://www.ijo.in/text.asp?1991/39/3/118/24456
The classical concept of malignant glaucoma ,[2 ] has changed over the years and now includes a variety of secondary angle closure glaucomas unresponsive to the classical antiglaucoma measures 
With this broader definition `Fungal Malignant Glaucoma' was first described by Jones in four cases of mycotic keratitis ,. He noted iridolenticular adhesions causing aqueous diversion, and histologically demonstrated a lens-iris-fungal mass causing a pupillary block . This caused a uniform shallowing of the anterior chamber, as opposed to an iris bombe and associated elevation of intraocular pressure. Jones suggested a combination of penetrating keratoplasty, irrigation of the anterior chamber, lens removal and anterior vitrectomy to manage such cases ,. He isolated the same fungus from the cornea and anterior chamber.
In this paper, the importance of recognizing this condition during the management of fungal corneal ulcers is stressed. This is more so in developing countries where antifungal drugs are not easily available and thus there are more chances of developing this complication. The paper also highlights the importance of lens removal and a posterior chamber wash in managing this condition and suggests a less aggressive modality of treatment than what has been previously recommended.
A 25 year old male presented with a history of pain, redness and decreased vision of the left eye of 15 days duration, following sand entering his eyes. He was treated with native medicines prior to consulting an ophthalmologist, who gave antibiotics, including gentamicin, before referring him to us. Examination revealed a visual acuity of only hand movements in the left eye. The ulcer was 5 x 6 mm in size with irregular margins and involved only the superficial 2/3 of the cornea. The pupil was 3 mm in size with sluggish movements. A hypopyon of 3 mm height was present. The intraocular tension, as measured digitally, was found to be normal. Topical Econazole drops (5mg/ml) was started after demonstration of septate fungal hyphae in both Gram-stained smears and lactophenol cotton blue (LPCB) mounts of the corneal scrapings. Atropine drops were also given. On the third day of treatment, the intraocular pressure (IOP) was found to be raised, with uniform shallowing of the anterior chamber.
The hypopyon level increased to cover the pupillary area. Oral Ketoconazole 200mg twice daily and topical Natamycin (100 mg Natamycin) vaginal pessaries in 10 ml. Moisol were added to the therapeutic regimen. On the fifth day of treatment the intraocular pressure was still high in spite of maximal doses of Acetazolamide and Timolol, and the ulcer area increased to about 8 mm. size and had involved the full thickness of the cornea [Figure 1]. Since the tension had to be lowered quickly to save the eye and no donor cornea was available, a paracentesis, anterior chamber wash and a peripheral iridectomy were done to remove the pupillary block; the hypopyon was sent for culture. For one day following surgery, the tension was only borderline high but the hypopyon began to reform. Two days after the paracentesis, the anterior chamber again flattened and the tension rose.-The peripheral iridectomy was covered by exudate. The central cornea of the patient continued to thin out while donor corneas were awaited. The ulcer perforated on the fifth day after paracentesis, the iris and the exudates were seen bulging through the perforation. As the corneas were available on the day of perforation, keratoplasty was performed. A slightly eccentric 9 mm. button was removed from the cornea. As the iris and exudates were plastered to the back of the cornea, the central ring of iris, along with the infected cornea, was removed. As the anterior part of the lens also seemed to be infiltrated, a `Can Opener' type of capsulotomy and extracapsular lens extraction (ECCE) were performed. Since the red reflex was good and the vitreous seemed normal, no vitrectomy was done. The posterior chamber was irrigated thoroughly and a 9.5 mm. graft was sutured on. Postoperatively, systemic Ketoconazole and topical Natam7cin were continued. Initially, the graft was clear and tension was normal with a deep anterior chamber [Figure 2]. On the fifth post-operative day, the tension rose again and a gentle gonioscopy revealed diffuse peripheral anterior synechiae. The vitreous and fundus were normal, with a 0.2 cupping of the disc. As the IOP was not controlled with Acetazolamide and Timolol, a 180 degree cyclocryopexy was done 3 mm. behind the limbus. A week after cyclocryopexy, applanation tension was 8 mm. Hg and the vision was 6/60 with correction. The patient was discharged 9 days after cyclocryopexy with oral Ketoconazole 200 mg. twice daily, local Betnesol drops and Indomethacin. Two weeks after discharge, when the patient came for the first review, 2+ flare and cells, with large keratic precipitates on the back of the cornea, and corneal oedema, were present. Systemic steroids were started in addition to the local steroids. Ketoconazole was continued for another week and stopped. In spite of the systemic steroid therapy, the corneal oedema persisted and within two months, the donor cornea showed total opacification and mild vascularisation.
A 28 year-old male presented with a history of pain and decreased vision of his left eye of 15 days duration. The patient had sustained an injury in the left eye with a paddy stick prior to the start of his complaints. He was treated with gentamicin before being referred to us. Examination revealed a central ulcer of 5 mm size, surrounded by an immune ring.
The full thickness of the cornea was involved. There was a hypopyon filling nearly half the anterior chamber [Figure 3]. The pupil was not dilated and was 3 mm in diameter. Digitally, the IOP was high. Oral and topical Ketoconazole therapy, along with acetazolamide and atropine were started. Two days later, the anterior chamber was almost flat and the IOP was markedly raised. Adding timolol twice daily and oral glycerol did not improve the condition. A penetrating keratoplasty, using a 8 mm. donor graft, was done. At the time of surgery, since there was a haze in the anterior capsular region of the lens, an ECCE with a thorough wash of the anterior and posterior chambers was done. The hypopyon was sent for culture. Two peripheral iridectomies were done. The post-operative period was uneventful, except for a severe uveitis which subsided with steroids. Systemic ketoconazole 200 mg. twice daily was continued for two weeks.
A 40 year old man presented with a history of irritation and pain in the right eye of 5 days duration. There was no history of injury. Examination revealed a 4 x 5 mm ulcer involving the anterior 1/3 of the cornea in the central area. Cells were present in the anterior chamber but there was no hypopyon. The IOP as measured digitally was normal. Topical Saperconazole (2.5mg/ml) therapy was started and after 4 days Saperconazole was discontinued and topical Econozole (5mg/ml) was started as there was no response. The ulcer continued to worsen even with Econazole. Hypopyon developed and almost the entire thickness of the cornea was involved [Figure 4]. Due to lack of response to medical therapy a full thickness keratoplasty was done, using a 7.5 mm donor cornea. The hypopyon and button were sent for culture and the anterior chamber was then washed to remove all exudates. For 2 days postoperatively, the anterior chamber was formed and the graft was clear. From the third day the hypopyon started recollecting. Systemic steroid therapy was started, in addition to the oral Ketoconazole and local Econazole therapy. The anterior chamber flattened on the fourth day and the IOP became very high. The high IOP did not subside even with maximal doses of Diamox and Timolol. The donor cornea became opaque and oedematous on the fifth day. The iris prolapsed at about the 11 0' clock position. The patient refused any further surgery and absconded the following day and. was lost to further follow up.
Three cases of mycotic keratitis developed very high intraocular tension and shallowing of anterior chamber, which was unresponsive to conventional antiglaucoma measures. Forty eight cases of fungal corneal ulcers were diagnosed during the course of study. In case 1 and 2 the pupil was only 3 mm in size prior to the development of raised tension, in spite of atropinisation. Case 3 had a pupillary size of about
4 mm, and the pupil could not be dilated further. Fusarium sp. were isolated from the initial corneal scrapings and the corneal button in all three cases. The exudates from the anterior chamber and excised tissue from case 1 and the hypopyon of cases 2 and 3 also grew Fusarium. In case 1, 8 months after the initial infection, the eye was quite with no recurrence of infection. The visual acuity is only hand movements close to the face. The tension is under control without medications. His chance for visual recovery after regraft should be fair as his retina is normal. Case 2 also had no recurrence of infection; the eye is quiet and IOP is normal. Five months after the original infection, the visual acuity was counting fingers ten centimetres with correction ; there was deep vascularization of the donor cornea which was slightly hazy [Figure 4]. The posterior capsule was opacified and there were areas of anterior synechiae at the graft-host junction. Case 3 had a high intraocular tension when the patient left the hospital, with intraocular contents trying to escape between the sutures.
Barrie Jones first used the term `Fungal Malignant Glaucoma' to describe a malignant glaucoma secondary to fungal corneal ulcers leading to late perforation and rupture of the globe ,.He showed that the fungus penetrated into the anterior chamber and that the lens-iris-fungal mass at the pupillary area caused diversion of aqueous and subsequent angle-closure, leading to a high intraocular pressure . However, a malignant glaucoma-like picture has also been shown to occur secondary to fungal endophthalmitis, This can also be due to a vitreous abscess pushing the lens and iris forward  The causative mechanism of this glaucoma and its management is different from the glaucoma described by Jones. In corneal ulcers, the infecting fungus enters the anterior chamber and this, along with the hypopyon, occludes the pupil leading to an angle closure. Thus the term "Keratomycotic Malignant Glaucoma" may be more explanatory and specific than "Fungal Malignant Glaucoma".
The three cases presented here had the characteristics described by Jones to make a diagnoses of "Keratomycotic Malignant Glaucoma", that is raised tension unresponsive to standard antiglaucoma measures, uniform shallowing of the anterior chamber (unlike an iris bombe) and recovery of the fungus from the anterior chamber. We did not separately culture anterior and posterior chamber fluids since they are, in effect, a single compartment with organised material seen extending from the anterior to the posterior chamber via the pupil.
It is important to recognise the signs of this posterior chamber pathway to blindness in cases of fungal corneal ulcer  because at this stage, the eye will be lost unless the malignant glaucoma is also managed by surgical intervention. In case 1, we proceeded with an extracapsular lens extraction alone, as only the anterior part of the lens was involved. Anterior vitrectomy was considered unnecessary, as the clearing of cortical matter revealed a good red reflex. In addition, there were the advantages of an extracapsular extraction, including the barrier effect  if vitrectomy was avoided. Presumably cases 1 and 2 did not have any further infection, inspite of only an extracapsular extraction, either because the lens zonules prevented the vitreous getting infected, or the systemic ketoconazole, which achieves a higher concentration in the vitreous than in the aqueous sub may have taken care of the vitreous infection. Jones et al grew fungus from the vitreous of their first case after an intracapsular lens extraction . it is possible that this may have been contamination from the posterior chamber. The extracapsular surgery also ensures a good irrigation of the infected posterior chamber.
The development of malignant glaucoma in case 3 after a therapeutic keratoplasty raises certain questions that need to be studied further. What is the role of lens removal in therapeutic keratoplasty for keratomycosis? Should a good posterior chamber irrigation be done in all such cases if it has been decided to retain the lens? Factors that may help in arriving at a decision to do these two procedures are a full thickness involvement of the cornea, suggesting that the hypopyon may not be sterile, a raised IOP prior to surgery and a small nondilating pupil.
The possible reasons for rejection of the graft in case 1 include the inflamed state of the eye, anterior synechiae, larger size of the graft, a period of raised tension after surgery and the cyclocryotherapy performed for the raised tension.
An interesting point noted in this study is that of the pupillary size. None of the three cases had a pupillary size of more than 4 mm in spite of maximal cycloplegic therapy. Thus, we feel that, in spite of the disadvantages of a dilated fixed pupil, it is worthwhile to atropinize the eyes of cases of keratomycosis early in the course of the disease before the associated uveitis makes dilatation difficult. A small pupil increases the chance of a pupillary block leading to a malignant glaucoma.
The three cases reported here, and 3 out of 4 cases reported by Jones, all had Fusarium keratitis. This may be because of the marked virulence of Fusarium  or because the antifungal compounds currently available, especially the systemically active ones are ineffective against Fusarium. It would be
interesting to study whether Fusarium produces substances that help in its penetration into the cornea or that toxins produced by it [14 ] contribute to the production of malignant glaucoma.
Malignant glaucoma, as a complication of keratomycosis, occurs in a certain percentage of cases. There should be a reduction of its incidence since more effective antifungals are becoming available. Early recognition of this condition is important so that surgical intervention can be planned. Keratoplasty, with a good anterior and posterior chamber wash and an extra capsular lens extraction, and post-operative systemic antifungal therapy is recommended for management of this complication. A more invasive procedure, including anterior vitrectomy, may not be necessary. Full dilatation of pupils early in the course of the disease is recommended as a possible prophylaxis. The term "Keratomycotic Malignant Glaucoma" is suggested as a more specific term.
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