PHTHISIS BULBI

Signs and Symptoms

Phthisis bulbi is an ocular condition defined by atrophy and disorganization of the intraocular structures that leads to a soft and anatomically disfigured, shrunken globe.1-3 The term is derived from the Greek word phthiein, meaning shrinkage or consuming.3 Ocular atrophy and phthisis bulbi connote consecutive stages in the degeneration process of a severely damaged eye.3,4 The process is often set into motion following an event of blunt or penetrating trauma with catastrophic functional and anatomic consequences.1-9 The condition is known to develop in eyes experiencing a protracted, non-remitting inflammatory course.1-8 It may also occur from tumor, ischemia, complications from cataract surgery, infection, rampant proliferative vitreoretinopathy, complicated retinal detachment and ocular inflammatory disease.1-13

The condition itself has no symptoms per se; the diagnosis is a term given to the end-stage degeneration of the globe and its contents.1-12 The first classification system of phthisis bulbi was based simply on observed structural changes: (1) ocular atrophy without shrinkage, (2) ocular atrophy with shrinkage and (3) ocular atrophy with shrinkage and ocular tissue disorganization.4 The classification system has since been modified to include the amount and description of the cosmetic complications:3

(Ia) Corneal opacity with no enophthalmos and normal sclera without corneal sensitivity.

(Ib) Corneal opacity with no enophthalmos and normal sclera with corneal sensitivity.

(IIa) Corneal opacity with mild enophthalmos and normal sclera without corneal sensitivity.

(IIb) Corneal opacity with mild enophthalmos and normal sclera with corneal sensitivity.

(III) Moderate enophthalmos with disfigured sclera.

(IV) Severe enophthalmos with disfigured sclera and loss of orbital fat.

The symptoms that are experienced during the chronic process are related to the underlying cause and the inflammation occurring (pain, severe vision loss); the eye is failing after the fact.1-11 Symptoms also include loss of color perception and loss of acuity. Signs include enophthalmos, variable ossification of ocular structures, variable injection of the conjunctiva, corneal-scleral sequelae such as sclerocornea and the formation of band keratopathy.

Other features that accompany the degeneration include anterior chamber collapse, iris neovascularization, hypoyon uveitis, hyphema, posterior synechiae and peripheral anterior synechiae.1,2,5-12 In the beginning stages, dense vitritis, vitreous hemorrhage, massive intraretinal exudation, florid proliferative retinopathy, choroidal rupture, subretinal hemorrhage or signs of tumor (choroidal folds) may be seen.14-16

Pathophysiology

Phthisis bulbi can follow any situation in which the normal neurology, cytokines and chemo-attractants responsible for routine ocular homeostatic signals are interrupted.17,18 The condition itself is enigmatic, usually beginning as errant wound healing.17,18 Here, a runaway cascade of tissue reactions is seen, similar to other aggressive inflammatory wound healing scenarios elsewhere in the body.

What makes the phthisis bulbi reaction unique is that tractional changes are induced by fibroproliferative mechanisms set off by exposure to intraocular bleeding.17,18 Wound or injury bleeding induces fibro-ingrowth, creating mechanical forces on adjacent structures perpetuating the course.17,18

Ocular hypotensive induction is another factor. This provokes an environment of ischemia that is a critical player in the pathology.17

Phthisis bulbi reactions reach a plateau within the first three months, but can go on for years.17 Cell proliferation and transformation are important features of the process; these eventually culminate in retinal and ciliochoroidal detachment, hypotony and marked shrinkage of the globe.17 The process has been considered an “intraocular fluoride proliferative reaction.”17 The prominent area of this proliferative reaction centers in the pars plana, the optic disc and the base of the iris.17

Management

The management of phthisis bulbi is rooted in maintaining patient comfort and slowing the degenerative process. Cycloplegic medications such as atropine 1% QD-BID, topical steroids and topical nonsteroidal anti-inflammatory drugs QD-QID are all employed. Topical artificial tear drops and ointments can provide lubrication and soothing comfort. Oral analgesics can also be prescribed. While chronic use of oral steroids is rarely undertaken, timely use of injectable steroids (e.g., triamcinolone) can be attempted to quell inflammation and improve or maintain comfort.19 If the phthisical eye has visual ability in the setting of a failed fellow eye, low vision rehabilitation can be attempted.19

Scleral cosmetic contact lenses can be fitted, with or without surgical resection of the conjunctiva, so long as the enophthalmos is not significant.3,20 Some patients report irritation from the lenses, leading to decreased wear time. The surgical resection is designed to create a protective flap (mucous membrane vs. Gunderson flap), covering sensitive corneas, improving function and comfort.21,22

If the enophthalmos is significant, a spacer can be implanted and the scleral contact lens subsequently fitted over top.3 When the globe is painful, evisceration or enucleation can be performed.23-26 Since evisceration leaves the scleral shell with extraocular muscle connections intact, a well-designed prosthesis can move naturally, creating an excellent cosmetic result.23-26 While the thought of evisceration may conjure an association with sympathetic ophthalmia, the risk is actually quite small.26 Enucleation with placement of a hydroxyapatite spacer or simple acrylic sphere implant to build orbital volume yields few complications.25

Clinicians encountering individuals who have undergone these procedures should be familiar with removal and replacement of the prosthesis. This will permit the remaining ocular contents to be examined and maintained. It will also permit examination of the prosthetic, to ensure quality, proper placement and integrity. Orbital implant movement, flap and spacer tissue erosion and chronic mucous formation or conjunctival infection are possible complications.

Retrobulbar alcohol or chlorpromazine injection remains a possibility for patients with blind, painful eyes.27 However, the procedure is rarely used as it is painful, often ineffective and—when it does work—its effect is often only temporary, making repeat injections necessary. The procedure often shrinks the orbital fat, increasing the enophthalmic posture. Today, it is only recommended for patients in persistent pain who cannot accept the thought of losing their eye (evisceration/enucleation).27

Clinical Pearls

While phthisis bulbi is most common in cases of penetrating trauma, it can occur following any injury, infection, complicated surgery, inflammation or retinal detachment when the healing system fails.

Treatment is directed at preserving comfort, maintaining the integrity of the socket and preserving the function of the prosthesis to maximize cosmesis.

Prostheses are fabricated by crafts-persons known as ocularists.

1. Naveen HC, Porwal A, Nelogi S. Prosthetic rehabilitation of phthisis bulbi by digital imaging technique–a case report. Cont Lens Anterior Eye. 2010;33(5):231-4.

2. Tan LT, Isa H, Lightman S, Taylor SRJ. Prevalence and causes of phthisis bulbi in a uveitis clinic. Acta Ophthalmologica 2012;90(5):e417-18.

3. Aggarwal H, Singh RD, Kumar P, et al. Prosthetic guidelines for ocular rehabilitation in patients with phthisis bulbi: A treatment-based classification system. J Prosthet Dent. 2014;1(1);pii: S0022-3913(13)00320-X.

4. Hogan MJ, Zimmerman LE. Phthisis bulbi. In Hogan MJ, Zimmerman LE Eds. Ophthalmic pathology. An atlas and textbook, 2nd ed. Philadelphia, PA, WB Saunders, 1962:168-221.

5. Semba RD, Bloem MW. Measles blindness. Surv Ophthalmol. 2004;49(2):243-55.

6. Ostler HB, Thygeson P. The ocular manifestations of herpes zoster, varicella, infectious mononucleosis, and cytomegalovirus disease. Surv Ophthalmol. 1976;21(2):148-59.

7. Kim PS, Choi CW, Yang YS. Outcome and significance of silicone oil tamponade in patients with chronic serous retinal detachment. Korean J Ophthalmol. 2014;28(1):26-31.

8. Coşkun M, Ataş M, Akal A, et al. The factors affecting the development of phthisis bulbi after penetrating eye injuries. Ulus Travma Acil Cerrahi Derg. 2012;18(4):317-20.

9. Huamont FU, Peyman GA, Goldberg MF. Complicated retinal detachment and its management with pars plana vitrectomy.Br J Ophthalmol. 1977;61(12):754-60.

10. Hadjistilianou T, De Francesco S, Marconcini S et al. Phthisis bulbi and buphthalmos as presenting signs of retinoblastoma: A report of two cases and literature review. European Journal of Ophthalmology 2006;16(3):465-9.

11. Mullaney PB, Karcioglu ZA, Al-Mesfer S, Abboud EB. Presentation of retinoblastoma as phthisis bulbi. Eye.1997;11(1)403–8.

12. Vemuganti GK, Honavar SG, Jalali S. Intraocular osseous metaplasia. A clinico-pathological study. Indian J Ophthalmol. 2002;50(3):183-8.

13. Limaiem R, Merdassi A, Lahdhiri, et al. Orbital cellulitis and endophthalmitis after cataract surgery. Bull Soc Belge Ophtalmol. 2008;(309-310):27-30.

14. Phillips BN, Chun DW, Colyer M. Closed globe macular injuries after blasts in combat. Retina. 2013;33(2):371-9.

15. Kashyap S, Meel R, Pushker N, et al. Phthisis bulbi in retinoblastoma. Clin Experiment Ophthalmol. 2011;39(2):105-10.

16. Qureshi UA, Wani NA, Altaf U. Parry-Romberg syndrome associated with unusual intracranial vascular malformations and Phthisis bulbi. J Neurol Sci. 2010;15;291(1-2):107-9.

17. Stefani FH. Phthisis bulbi–an intraocular fluoride proliferative reaction. Dev Ophthalmol. 1985;10 (1):78-160.

18. Kreiger AE. Wound complications in pars plana vitrectomy. Retina. 1993;13(4):335-44.

19. Rodriguez ML1, Juarez CP, Luna JD. Intravitreal triamcinolone acetonide injection in blind painful eyes. Intraocular steroids as a treatment for blind painful red eyes. Eur J Ophthalmol. 2003;13(3):292-7.

20. Froiman PC, Villas Bôas Júnior CE, et al. Visual rehabilitation in pre phthisis bulbi’s patient: case report. Arq Bras Oftalmol. 2009;72(4):540-2.

21. Ding J, Chen T, Hou Z, et al. Cosmetic shell fitting over a sensitive cornea in mild phthisis bulbi using total conjunctival flap. Aesthetic Plast Surg. 2013;37(2):398-401.

22. Ma’luf RN, Awwad ST. Mucous membrane graft versus Gunderson conjunctival flap for fitting a scleral shell over a sensitive cornea. Ophthal Plast Reconstr Surg. 2005;21(5):356-8.

23. Georgescu D, Vagefi MR, Yang CC, et al. Evisceration with equatorial sclerotomy for phthisis bulbi and microphthalmos. Ophthal Plast Reconstr Surg. 2010;26(3):165-7.

24. Aggarwal H, Singh RD, Kumar P, et al. Prosthetic guidelines for ocular rehabilitation in patients with phthisis bulbi: A treatment-based classification system. J Prosthet Dent. 2014; S0022-3913(13)00320.

25. Tanenbaum M. Enucleation, eviseraction and exenteration. In: Yanoff M, Duker JS. Ophthalmology. Mosby-Elsevier, St. Loius, MO 2009:752-760.

26. Zheng C, Wu AY. Enucleation versus evisceration in ocular trauma: a retrospective review and study of current literature. Orbit. 2013;32(6):356-61.

27. al-Faran MF, al-Omar OM. Retrobulbar alcohol injection in blind painful eyes. Ann Ophthalmol. 1990;22(12):460-2.