Traumatic Ghost Cell Glaucoma with Successful Resolution of Corneal Blood Staining Following Pars Plana Vitrectomy.

Ghost cell glaucoma (GCG) was first described in 1976. It is a type of a secondary open angle glaucoma, which occurs following long-standing vitreous hemorrhage. The ghost cells are rigid and less pliable than fresh red blood cells; therefore, they may cause direct obstruction of the trabecular meshwork and secondary increase in the intraocular pressure (IOP). This case report presents the diagnosis and management of a rare case of traumatic GCG after vitreous hemorrhage in a phakic child. Pars plana vitrectomy was done after unsuccessful medical therapy and the diagnosis was confirmed by cytopathology. Surprisingly, spontaneous resolution of the corneal blood staining occurred. The outcome in this case was favorable with controlled IOP in the affected eye.

INTRODUCTION

Ghost cell glaucoma (GCG) was first described in 1976 by Campbell GCC is a secondary open angle glaucoma, which occurs following long-standing vitreous hemorrhage. We report our experience in the diagnosis and management of a case of traumatic GCG postvitreous hemorrhage, which was recalcitrant to medical therapy. Pars plana vitrectomy (PPV) was performed and the diagnosis was confirmed by cytopathology. The outcome in this case was favorable, and the intraocular pressure (IOP) in the affected eye was eventually controlled without medication.

CASE REPORT

A 10-year-old male presented with decreased vision in the right eye for 1-month duration following blunt trauma by a stone to this right eye. He was referred to King Khaled Eye Specialist Hospital in Riyadh, Saudi Arabia, as a case of right eye posttraumatic dense vitreous hemorrhage with resolved hyphema and controlled IOP on medications. On examination, visual acuity was Hand motion close to face (HM) in the right eye and 20/20 in the left eye. The IOP was 20 mmHg in the right eye and 20 mmHg in the left eye. Examination of the left eye was unremarkable. Anterior segment examination of the right eye revealed mild ciliary injection, inferotemporal blood corneal staining, 3+ khaki-colored cells in the anterior chamber, and early cataract [Figure 1]. Gonioscopic examination revealed 30% angle recession. The right eye fundus could not be visualized, so B-scan ultrasonography was performed, which confirmed dense vitreous hemorrhage and a flat retina. The IOP in the affected eye was controlled with the following topical medications: Brinzolamide 1% BID, timolol maleate 1% BID, and apraclonidine 0.5% TID. After 2 weeks, the IOP increased to 37 mmHg despite maximum tolerated medical therapy. The case was diagnosed clinically as GCG. We elected to perform PPV and anterior chamber washout. The aqueous sample was sent for cytopathology analysis and diagnosis. The cytology smears showed old hemorrhage, red blood cells, and degenerated red blood cells consistent with the clinical diagnosis of GCG [Figure 2a–c]. One week postoperatively, the IOP in the right eye was controlled with one medication and vision in the eye improved to 20/40. Two years after PPV, there were lens changes for which cataract extraction was performed. Postoperative examination 4 weeks following cataract surgery indicated spontaneous resolution of corneal staining and the IOP was normal without medications [Figure 3].

Figure 1

The clinical appearance of the right eye at presentation showing inferotemporal blood corneal staining and early cataract

Figure 2

(a) Cytology smear of the aqueous of the right eye showing numerous red blood cells, some of which are degenerated representing ghost cells (black arrow) (Giemsa stain, ×1000). (b) Ghost cell appearance with routine staining (H and E, ×1000). (c) The characteristic appearance of the ghost cell with Heinz bodies (black arrow) (methylene B, ×1000)

Figure 3

The postoperative clinical photo of the right eye 2 years later showing complete resolution of the blood corneal staining and a stable intraocular lens

DISCUSSION

Campbell and Essigmann characterized GCG postvitreous hemorrhage as a type of secondary open angle glaucoma that involved obstruction of the trabecular meshwork due to aged red blood cells known as ghost cells. Ghost cells are formed within 1–3 weeks after vitreous hemorrhage. The red blood cells become degenerated and spherical in shape, and less pliable. They partially lose their intracellular hemoglobin to the extracellular vitreous space forming clumps that adhere to vitreous strands. The remaining part of the intracellular hemoglobin binds to the internal surface of the cell membrane forming characteristic clumps called Heinz bodies. This process leads to the development of ghost cells that remain in the vitreous for months following a resolved hemorrhage. Ghost cells do not form in the cases of anterior chamber hemorrhage due to the rapid circulation and high levels of oxygen in the anterior chamber.12 Ghost cells can reach the anterior chamber through a disrupted anterior hyaloid face, after traumatic injury, cataract extraction, vitrectomy, or even spontaneously. Ghost cells in the anterior chamber have been reported to be associated with a snake bite.123 Ghost cells are rigid, less pliable than fresh red blood cells; therefore, they cause a secondary increase in IOP by direct obstruction of the trabecular meshwork.1

The cause of elevated IOP after trauma to the right eye was initially attributed to hyphema. However, following complete resolution of the hyphema and normalization of the IOP, there was another episode of elevated IOP, which was attributed to outflow obstruction representing GCC. Delayed IOP elevation has been observed and explained in the literature.4

GCG is often self-limited and is managed in a stepwise approach, starting with medical therapy. In the absence of a good response and the presence of dense vitreous hemorrhage - as in our patient - PPV is recommended since it works by removing the reservoir for the ghost cells.4 Repeated anterior chamber irrigation and trabeculectomy have been also reported as additional surgical options in certain cases.15

Corneal blood staining is caused by the breakdown products of hemoglobin that are deposited within the cornea in the presence of hyphema and elevated IOP. However, cases of corneal staining with normal IOP have been reported.6 In these cases, once the hyphema is resolved and the IOP is controlled, the corneal blood staining can clear spontaneously, similar to our case.6

In conclusion, we present an interesting case of posttraumatic GCC in a phakic child. The diagnosis was clinically suspected and confirmed histologically. High clinical suspicion in similar cases has allowed us to provide appropriate management with excellent outcome including spontaneous resolution of corneal blood staining and normal IOP without medications.

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Conflicts of interest

There are no conflicts of interest.