International Ophthalmology

, Volume 31, Issue 1, pp 25–28

Induction of bilateral ligneous conjunctivitis with the use of a prosthetic eye

Authors

    • Department of OphthalmologyUludag University
  • Meral Yıldız
    • Department of OphthalmologyUludag University
  • Tayfun İrfan
    • Dismer Dental Health Center
Case Report

DOI: 10.1007/s10792-010-9381-0

Cite this article as:
Yazıcı, B., Yıldız, M. & İrfan, T. Int Ophthalmol (2011) 31: 25. doi:10.1007/s10792-010-9381-0

Abstract

The purpose of this article to report a case of ligneous conjunctivitis in an anophthalmic socket, in respect of a 20-year-old woman. The subject woman had a history of left enucleation surgery presented with bilateral palpebral ligneous conjunctivitis and ligneous gingivitis. The hematologic study revealed a severe plasma plasminogen deficiency. The eyelid lesions were successfully treated with surgical excision, topical heparin and corticosteroid eyedrops. However, the ligneous lesions recurred bilaterally after she was fitted with a prosthetic eye and were refractory to intensive topical treatment with heparin and cyclosporin A eye drops. This case shows that the use of a prosthetic eye may induce ligneous conjunctivitis in an anophthalmic socket and normal eye which is refractory to topical treatment.

Keywords

Ligneous conjunctivitisAnophthalmic socketOcular prosthesis

Introduction

Ligneous conjunctivitis is a rare, autosomal recessive disease characterized by the formation of fibrin-rich recurrent membranes on the palpebral conjunctiva and other mucosal sites. The underlying pathologic mechanism is related to systemic plasminogen deficiency [1]. It is well known that ligneous conjunctivitis may develop after several ocular surgeries or conjunctival infections [2]. To our knowledge, no case of ligneous conjunctivitis induced by the use of a prosthetic eye has been reported before.

Case report

A 20-year-old immigrant woman from Azerbaijan presented with redness, discomfort, discharge, eyelid masses, and inability to wear eye prosthesis. A medical history was obtained from the patient and her parents. Her left eye had received a perforating trauma when she was 3 years old and was enucleated at 6 years of age because of painful glaucoma. The patient had been unable to wear a prosthetic eye because of the conjunctival masses that developed in her right and left eyelids. It was not known whether these lesions first occurred after enucleation or after wearing the eye prosthesis. These lesions had recurred despite their surgical removal four times within 1 year. The patient had regular menstruations after removal of a mass lesion from her uterine cervix at 16 years of age. Neither her parents nor her siblings had a history of similar problems. An informed consent was obtained from the patient for all procedures.

The patient’s visual acuity was 20/25 in the right eye. On the left side, the orbital socket was partially contracted and there was no palpable implant in the orbit. We noted yellow-white pedunculated membranes that were 10 × 10 mm in dimension on the temporal sides of upper and lower palpebral conjunctiva on both sides. We also observed thick, membranous lesions in the gingiva around the tooth insertions in both upper and lower jaws (Fig. 1). The gynecologic examination revealed no genital mucosal abnormality. Excisional biopsy of the conjunctival lesions and incisional biopsy of the gingival lesions showed histopathologic findings that were consistent with the diagnosis of ligneous conjunctivitis. Plasma plasminogen activity was 26% (reference range 80–120%).
https://static-content.springer.com/image/art%3A10.1007%2Fs10792-010-9381-0/MediaObjects/10792_2010_9381_Fig1_HTML.jpg
Fig. 1

Ligneous gingival membranes were seen in the upper jaw before treatment

After excision of the conjunctival membranes, the patient received intensive treatment with topical heparin (5,000 IU/ml every 30 min to 1 h) in combination with 1% prednisolone acetate (four times a day) in both eyes [3]. The ligneous membranes regressed noticeably at 3 weeks and completely disappeared at 3 months. Heparin eye drops were gradually tapered to twice a day during the next 12 months, and corticosteroid eye drops were tapered off within a month. The patient continued to instill heparin eye drops twice a day.

Three months after initiation of the ocular treatment, four teeth with alveolar resorption were extracted and a local gingivectomy was performed. The patient then applied an oral rinse with heparin solution (1/5,000 IU heparin diluted in 200 ml saline solution) three times daily for 3 months and thereafter once a day for 3 months. The gingival lesions showed a marked improvement with this treatment and she experienced no systemic or local side-effects of this treatment.

Eighteen months after initiation of the ophthalmic treatment, the patient received a small, impression-fitted eye prosthesis. One month later, ligneous membranes were noted in the upper and lower tarsal conjunctiva bilaterally (Fig. 2a). Despite treatment with heparin (six times a day) and prednisolone (four times a day) eye drops, the membranes persisted. We excised the membranes and advised the patient to instill the heparin eye drops bilaterally 12 times a day and to begin using her eye prosthesis 10 days later. At the examination 1 month later, we noted a further recurrence. After excision of the membranes, removal of the prosthesis, and maintenance treatment with topical heparin, the patient had a stable remission without recurrence on both sides. One year later, the prosthesis was placed in the socket again and the patient received heparin drops bilaterally five times a day together with 0.05% cyclosporin A and prednisolone eye drops twice a day on the anophthalmic side only. Unfortunately, new conjunctival membranes developed in both eyes after 1 month and the lesion in the normal eye was larger than that in the anophthalmic socket (Fig. 2b). Because the use of the ocular prosthesis induced the recurrence of ligneous conjunctivitis despite topical drug treatment, the patient was advised not to have further operations on the anophthalmic socket.
https://static-content.springer.com/image/art%3A10.1007%2Fs10792-010-9381-0/MediaObjects/10792_2010_9381_Fig2_HTML.jpg
Fig. 2

a Ligneous membranes in the lower tarsal plate 1 month after the patient began wearing the ocular prosthesis. b Ligneous membranes recurred in both sides despite the topical treatment with heparin and cyclosporin A, and the lesion in the right eye was larger than the one in the anophthalmic side

Discussion

Recent studies have shown that ligneous conjunctivitis is the result of type 1 plasminogen deficiency caused by inherited or sporadic mutations of the plasminogen gene [1, 2]. Plasminogen is synthesized primarily in the liver; it converts into a potent proteolytic enzyme, plasmin, through either plasma or tissue activators, and breaks down the fibrin clot to its soluble degradation products. Patients with plasminogen deficiency are unable to remove fibrin deposits in mucosal sites secondary to injury or inflammation.

There is no reported experience of an anophthalmic eye prosthesis in patients with ligneous conjunctivitis. In the patient described here, the disease might have first occurred either after enucleation or after fitting an ocular prosthesis. Nevertheless, the use of a prosthetic eye was exacerbating ligneous conjunctivitis in both the anophthalmic side and the opposite eye. The ligneous lesions responded well to surgical excision and topical heparin treatment. Low-dose heparin eye drops were also effective in preventing recurrence of the disease; however, when the patient fitted her eye prosthesis, ligneous membranes recurred in both eyes, and did not resolve despite intensive topical treatment with heparin, cyclosporin A, and corticosteroids, until the prosthesis was removed.

Ligneous conjunctivitis in the anophthalmic socket was possibly activated by conjunctival microtrauma and irritation elicited by the prosthesis; however, it is difficult to explain the development of simultaneous, sympathetic ligneous attacks in the normal eye. Ligneous conjunctivitis occurs bilaterally in about half of the patients [2]. Involvement of the fellow eye, which was not traumatized previously, has been observed in some cases. It has been suggested that an abnormal immune response, in association with plasminogen deficiency, may play a role in development of the disease [4].

For treatment of ligneous conjunctivitis, variable results have been reported with the topical use of drugs such as corticosteroids, heparin, cyclosporin A, plasminogen, fresh frozen plasma, and direct thrombin inhibitor [3, 58]. De Cock et al. [3] observed clinical improvement in 13 (76%) of 17 patients with heparin treatment. Heparin functions by accelerating the activity of antithrombin III, which itself inhibits thrombin formation, thereby blocking the conversion of fibrinogen to fibrin. In cases involving multiple mucosal sites along with the conjunctiva, systemic treatments with high-dose corticosteroids or oral contraceptive medications may have good results [9, 10].

Oral heparin rinse treatment for ligneous gingivitis was reported to have been unsuccessful in one patient [11]. In our case, however, this treatment, combined with membrane excision, provided a noticeable improvement. The use of heparin in both topical eye drop and oral rinse formulations may have increased the systemic dose of the medication and its local effect. Recently, a successful result was reported with the use of a warfarin rinse, which, similar to heparin, acts to reduce thrombin generation [12].

Ligneous conjunctivitis may cause serious ocular complications requiring enucleation or evisceration surgery. Patients should be informed that the use of an ocular prosthesis may induce exacerbations of ligneous conjunctivitis in both the anophthalmic socket and the contralateral eye that is refractory to topical treatment. These ligneous lesions can be treated successfully with discontinuation of the prosthesis, surgical excision and heparin eye drops.

Copyright information

© Springer Science+Business Media B.V. 2010