Perioperative Management and Long-Term Outcomes in Ocular Cicatricial Pemphigoid Patients Undergoing Cataract Surgery

Abstract

Objective: To observe the outcomes of cataract surgery in ocular cicatricial pemphigoid (OCP) patients and explore routine perioperative medical treatments.

Design: Retrospective case series.

Methods: Fourteen eyes of 8 patients were included in the study. Foster's stage 1-4 OCP patients were given human intravenous immunoglobulin, whereas patients with active inflammation were treated with prednisone tablets and methotrexate. Those who were intolerant to methotrexate and had severe inflammatory symptoms were treated with cyclophosphamide. Cataract surgery was performed for all patients after three months of systemic treatment under stable conditions. The conjunctival biopsy was evaluated by immunofluorescence microscopy. Then, patients were divided into individuals with or without ankyloblepharon. Records were reviewed for OCP stage, type of surgery, best-corrected visual acuity (BCVA), Schirmer I test, corneal fluorescein sodium staining, meibomian gland coloboma range, and ocular surface disease index (OSDI) scores. Follow-up was for the duration of taking topical and systemic medication.

Results: Nine female (64.29%) and 4 male (35.71%) eyes were diagnosed with OCP by biopsy. The mean follow-up time was 60.64 ± 35.62 months. Thirteen eyes (92.86%) of 7 patients underwent phacoemulsification. One eye underwent phacoemulsification combined with amniotic membrane transplantation. The intracapsular extraction of cataract was applied to one eye. The BCVA improved significantly in all the patients, which remained stable until the last follow-up. The Schirmer I test was higher than that before the surgery. Corneal fluorescein sodium staining after surgery showed a decrease in score compared to the preoperative score. The BCVA of the patients after surgery increased significantly. The OSDI scores of patients with ankyloblepharon were significantly higher than for those without it. Postoperative symblepharon showed no significant difference compared to the preoperative symblepharon.

Conclusions: In this series, OCP patients with cataracts were able to undergo phacoemulsification surgery, whereas routine use of immunosuppression and closed postoperative follow-up were necessary.

Figure 1

HE staining and immunofluorescence assay. (a) The histology of OCP conjunctiva. Multiple infiltrating immune cells were observed in the HE-stained slides from 10 eyes of 8 patients. (b) Control group. There was no staining along the conjunctival basement membrane zone. (c–e) Positive results of brilliant IgM, IgA, and IgG fluorescent staining along the basement membrane zone.

Figure 2

Preoperative and postoperative stage and score of OCP patients. (a) Foster's stage I-IV. (b) Keratopathy stage 1-4. (c) Symblepharon stage 1-3. (d) Meibomian gland coloboma range score 0-3.

Figure 3

Preoperative and postoperative radar images of the BCVA in OCP patients. The postoperative BCVA of the standard logarithmic visual acuity chart above 0.1 was significantly increased.

Figure 4

The BCVA, Schirmer I test, and corneal fluorescein sodium staining preoperative and postoperative. (a) The BCVA preoperative and postoperative (compared to preoperative, ^∗P < 0.001, n = 14). (b) The difference in BCVA between preoperative and postoperative patients (compared to the group without ankyloblepharon, ^∗P < 0.05). (c) The Schirmer I test preoperative and postoperative (compared to preoperative, ^∗P < 0.001, n = 14). (d) The difference in the Schirmer I test between preoperative and postoperative values. (e) Preoperative and postoperative corneal fluorescein sodium staining (compared to preoperative, ^∗P < 0.001, n = 14). (f) The difference in corneal fluorescein sodium dyeing between preoperative and postoperative samples.

Figure 5

The difference in OSDI score between preoperative and postoperative samples. There was statistically significant difference between the group without ankyloblepharon and the group with ankyloblepharon (^∗compared to preoperative P < 0.01, the group with ankyloblepharon n = 6, the group without ankyloblepharon n = 8).

Figure 6

Anterior segment image of the patient in the OCP stage of Foster's stage II and III. (a) Cortical and nuclear opacifications were found in the lens after dilatation before the operation of the eighth patient in the OCP stage of Foster's stage II. Sodium fluorescein staining before surgery of the eighth patient in the OCP stage of Foster's stage II, anterior segment image 1 month and 6 months after surgery. (b) Anterior segment image of the fourth patient in the OCP stage of Foster's stage III before surgery, the double frontal fornix becomes shallower preoperatively, and there are obvious symblepharons, which are especially evident in the inner and outer canthus. (c) Anterior segment image of the fourth patient in the OCP stage of Foster's stage III in 6 months after surgery. (d) Anterior segment image of the fourth patient in the OCP stage of Foster's stage III in 2 years after surgery.

Figure 7

The anterior segment image of the second patient in the OCP stage of Foster's stage IV.

Figure 8

Anterior segment image of the fifth patient in the OCP stage of Foster's stage IV. (a) Both eyes showed corneal neovascularization before surgery. (b) Anterior segment image and sodium fluorescein staining image of both eyes 2 years after ICCE surgery.