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 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.