Phacomatosis Pigmentovascularis and Sturge-Weber Syndrome: Comparative Outcomes of Primary Combined Trabeculotomy with Trabeculectomy.

PURPOSE

To compare the outcomes of primary combined trabeculotomy with trabeculectomy (CTT) in children diagnosed with early-onset glaucoma associated with phacomatosis pigmentovascularis (PPV) and Sturge-Weber syndrome (SWS).

DESIGN

Retrospective cohort study.

PARTICIPANTS

Forty-seven eyes (47 children) with SWS and 48 eyes (31 children) with PPV, who underwent primary CTT with a minimum follow-up of 1 year between 1996 and 2020 were included.

METHODS

Preoperative and postoperative data were collected. Surgical success rates were estimated using Kaplan-Meier survival analysis, whereas comparisons between the groups were performed using mixed-effects Weibull proportional hazards models accounting for intrapatient correlation in bilateral cases. Risk factors for failure were assessed with multivariable models incorporating the same adjustment.

MAIN OUTCOME MEASURES

Intraocular pressure (IOP) control, complete and qualified success, need for additional medications, postoperative complications in 2 groups.

RESULTS

Preoperative ocular characteristics were comparable between the groups, except that PPV cases were more often bilateral. Neurological abnormalities, including epilepsy (P < 0.001) and central nervous system anomalies (P = 0.04), were more common in PPV. The median follow-up was 7 years in the PPV group and 6.5 years in the SWS group. At the final follow-up period, the IOP reduced significantly in both groups when compared with the preoperative values (P < 0.001). Complete success was higher in the the SWS group (60%) than in PPV (40%), though not statistically significant (P = 0.15). Qualified success was similar in both groups (P = 0.79). However, the PPV group required significantly more glaucoma medications (P = 0.03) to maintain the IOP. Older age at the time of surgery (P < 0.001), and higher grade of corneal haze at baseline (P = 0.03) were associated with failure (according to complete success criteria) in the PPV group. Although the overall rate of postoperative complications was similar (P = 0.98), sight-threatening complications were more frequent in the PPV group.

CONCLUSIONS

Primary CTT achieved good long-term IOP control in both the PPV and SWS groups. The PPV group exhibited a higher prevalence of neurological and systemic associations and a greater need for postoperative medications, indicating a more complex disease course. Early surgical intervention and close postoperative monitoring are essential to achieving optimal outcomes in these challenging phakomatoses. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any materials discussed in this article.