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Editors Selection IGR 21-4

Surgical Treatment: Goniosurgery is not effective in the presence of Sturge-Weber syndrome

Elena Bitrian
Consuelo Gajardo

Comment by Elena Bitrian & Consuelo Gajardo on:

90993 Failure of Goniosurgery for Glaucoma Associated With Sturge-Weber Syndrome, Yeung HH; Kane SA; Turlapati N et al., Journal of Pediatric Ophthalmology & Strabismus, 2020; 57: 384-387


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Glaucoma associated with Sturge-Weber syndrome (SWS) is one of the most challenging types of childhood glaucoma due to limited response to certain topical medications and surgical treatments and higher risk of surgical complications.

Angle surgery is the procedure of choice for the treatment of most types of pediatric glaucoma. It achieves success rates of 85-90 % in primary open angle glaucoma, but in SWS the success rate is lower.1

In this paper, Yeung et al. report their results of angle surgery as a primary treatment for glaucoma associated with SWS in 46 eyes. They found a failure rate of 98% and interval of failure of four months (range 1-48 months). However, the average age of patients at time of surgery was 1.5 years (with a wide range, 1 month-23 years). There are two peaks of incidence of glaucoma in SWS: congenital and juvenile onset. The congenital type is commonly associated with angle dysgenesis and it is preferably treated with angle surgery because of the angle anomalies and because of the risks associated with more invasive surgery. In the juvenile form, elevation of episcleral venous pressure can be the cause of glaucoma.2

The authors state that goniosurgery is not an effective treatment of pediatric glaucoma. However, although the rate of success was not as high as in primary open-angle glaucoma, some patients can achieve long periods of controlled pressure with angle surgery and coadjuvant eyedrops. As well, it can be a temporizing measure allowing their eye to grow and increase the success and predictability of a later filtrating surgery, while decreasing the risk of surgical complications.3

In conclusion, even if angle surgery in SWS does not achieve success rates as high as in other types of childhood glaucoma, it delays the need for more invasive filtration surgery (trabeculectomy of tube shunt). Surgeons caring for SWS patients should be proficient at performing surgery in pediatric glaucoma and postoperative care for the patient.

Even if angle surgery in SWS does not achieve success rates as high as in other types of childhood glaucoma, it delays the need for more invasive filtration surgery

References

  1. Shaffer RN. Prognosis of goniotomy in primary infantile glaucoma (trabeculodysgenesis). Trans Am Ophthalmol Soc. 1982;80:321-325.
  2. Shiau T, Armogan N, Yan DB, et al. The role of episcleral venous pressure in glaucoma associated with Sturge-Weber syndrome. JAAPOS. 2012;16:61-64.
  3. Levin A, Maul E, Chang TC, et al. Glaucoma Associated with Non-acquired Systemic Disease or Syndrome. Childhood Glaucoma Consensus Series 9. Weinreb RN et al. (eds). Amsterdam: Kugler Publications, 2013.


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