Sturge-Weber syndrome is a sporadic congenital neurocutaneous disorder associated with a variable expression of a facial port-wine stain, leptomeningeal angioma of the brain, seizure disorder, intellectual delay, and glaucoma. Ocular manifestations of Sturge-Weber syndrome include choroidal hemangioma, conjunctival and episcleral vascular anomalies, heterochromia iridis, and filtration angle anomalies often associated with elevated intraocular pressure (IOP).
Glaucoma associated with Sturge-Weber syndrome is commonly found with an ipsilateral port-wine stain vascular lesion affecting the skin in the distribution of the ophthalmic branch of the trigeminal nerve. Glaucoma is reported to occur in 30% to 70% of patients with Sturge-Weber syndrome, with 60% occurring between the ages of 0 and 2 years and 40% in later childhood or adulthood.1 Glaucoma associated with Sturge-Weber syndrome is thought to be related to two potential mechanisms: anomalous filtration chamber angles or abnormal episcleral hemodynamics.2
Goniotomy and trabeculotomy are the most commonly used procedures for patients with infantile primary congenital glaucoma. On gonioscopy, the abnormalities seen in children with glaucoma associated with Sturge-Weber syndrome resemble those of children with primary congenital glaucoma and consist of the variable anterior insertion of the iris and increased opacification of the inner tissues of the angle.2 The long-term success of controlling IOP with goniosurgery has been reported as a success rate of 27% and a qualified success rate of 66%.3 This report shares what is currently the largest retrospective study of the surgical results of goniotomy and trabeculotomy for patients with glaucoma associated with Sturge-Weber syndrome.
Patients and Methods
The methodology of this study was approved by the New England Institutional Review Board (protocol #09-014-0010).
The medical records of 42 patients with glaucoma associated with Sturge-Weber syndrome who had a history of initial goniosurgery and were cared for by the authors (SAK and DSW) from 1990 to 2018 were reviewed. Thirty-eight patients with unilateral and 4 patients with bilateral glaucoma and a total of 46 operated eyes were studied. Fifty-one goniotomies and 11 trabeculotomies were performed. Thirty-three eyes received a single procedure, 11 eyes received 2 procedures, 1 eye received 3 procedures, and 1 eye had 4 procedures. A standard Barkan goniotomy procedure was performed, as described in a previous report.4
Inclusion criteria were clinical glaucoma associated with Sturge-Weber syndrome and a history of goniosurgery as their first surgical procedure. Exclusion criteria were patients with Sturge-Weber syndrome with no history of glaucoma, patients with glaucoma controlled by medications, and patients who did not undergo goniosurgery.
All patients had follow-up care for at least 6 months. Glaucoma was diagnosed by the presence of an initial elevation of IOP greater than 23 mm Hg without glaucoma medications. IOP was measured by Perkins applanation tonometry in the clinic setting without sedation and with significant assistance from supportive parents. The outcome result was determined by the IOP measurements from at least the last two follow-up examinations after surgery, by the addition of glaucoma medications, and by failure indicated by a persistent IOP elevation or need for filtration or drainage device surgery. Complete success was defined as an IOP of 22 mm Hg or less without medications, qualified success as an IOP of 22 mm Hg or less with the use of glaucoma medications, and failure as an IOP of greater than 22 mm Hg or the performance of a non-goniosurgery procedure.
The medical records of 42 patients (46 eyes) treated with 62 goniosurgery procedures were identified. Eighteen males and 24 females were included. Six procedures were performed by one surgeon (SAK) and 52 procedures were performed by the senior author (DSW). A senior referring physician performed four goniotomies for 1 eye. Thirty-seven eyes underwent 51 goniotomy procedures, 11 eyes underwent trabeculotomy procedures, and 2 eyes had both goniotomy and trabeculotomy procedures. The average age at goniosurgery was 1.5 years (range: 1 month to 23 years). The average IOP before goniosurgery was 35 mm Hg (range: 25 to 50 mm Hg) (Table 1).
Baseline Characteristics of Patients With Sturge-Weber Syndrome and Glaucoma Undergoing Goniosurgery
Ninety-eight percent of eyes (45 of 46 eyes) failed successful control of their IOP. Two percent of eyes (1 of 46 eyes) achieved qualified success. No eyes achieved complete success. The average postoperative interval to failure for the goniosurgery was 4 months (range: 1 to 48 months) (Figure 1). Among the 45 eyes with glaucoma associated with Sturge-Weber syndrome that failed, 19 eyes underwent trabeculectomy filtration surgery, 9 eyes had glaucoma drainage implant placement, and 4 eyes had cyclocryotherapy. Four eyes of 4 patients were lost to follow-up after failure. In our cohort of patients studied, there was no occurrence of a choroidal hemorrhage.
Results of goniosurgery for patients with Sturge-Weber syndrome and glaucoma.
Preoperative gonioscopy was recorded for all 41 eyes. All eyes had open angles with no vascular abnormalities. The trabecular meshwork was visible in all 41 eyes (100%). The scleral spur showed reduced visibility or was absent in 91% of eyes (37 of 41 eyes), and the ciliary body band was absent in 61% of examined eyes (25 of 41 eyes) (Figure 2).
Gonioscopy for patients with Sturge-Weber syndrome and glaucoma. Black arrow = trabecular meshwork visible in 100% of patients; gray asterisk = scleral spur reduced visibility or absent in 91% of patients; white arrow = ciliary body band absent in 61% of patients
The association of glaucoma with facial nevus flammeus was first recognized by Schirmer.5 The mechanism of this type of primary glaucoma related to Sturge-Weber syndrome is poorly understood, and a reliable procedure for its treatment has remained elusive.2 Barkan6 first reported the use and limited success of goniotomy for this type of glaucoma. Lister7 reported the failure of goniotomy in 5 of 5 cases of Sturge-Weber syndrome. Olsen et al3 reported 20 goniotomy operations for 12 eyes and 8 trabeculotomy procedures for 4 eyes with Sturge-Weber syndrome. Eight eyes that had goniotomy required a repeat procedure. Ten of 15 eyes (66%) were controlled with the addition of medications for the 6 of 10 eyes that experienced a qualified success.
Wu et al8 reported trabeculotomy ab externo with good surgical outcomes for early-onset glaucoma in patients with Sturge-Weber syndrome. Iwach et al9 reported the results of 46 goniotomies and expressed an awareness of less successful outcomes in younger patients with glaucoma associated with Sturge-Weber syndrome. They concluded that goniotomy was potentially an effective procedure to extend the period of stable IOP control when the procedure was combined with medication use. Sullivan et al10 reported 74 goniotomy procedures for 33 eyes. Only a single complete success and 24 (43%) eyes with a qualified success was achieved in their series of 57 eyes with Sturge-Weber syndrome having 103 glaucoma procedures. Herrygers11 noted the ineffectiveness of goniotomy and trabeculotomy for this type of glaucoma and suggested the need for eventual filtration surgery. The World Glaucoma Association meeting on childhood glaucoma reported the surgery preference for glaucoma associated with Sturge-Weber syndrome.12 Goniosurgery was recommended by 47% of physicians for patients younger than 3 years and 15% for patients older than 3 years.
The frequent failure of goniosurgery for glaucoma associated with Sturge-Weber syndrome compared to the high success rate for infantile primary congenital glaucoma is unexplained by preoperative or postoperative gonioscopy and by pathological examination of Sturge-Weber syndrome trabeculectomy specimens.2,13 Our gonioscopy findings confirm the observations of others that mild anomalies of the filtration angle are frequently present at the level of the scleral spur and ciliary body band, which resemble the findings seen in primary congenital glaucoma. The angle may be more normal in appearance in late-onset glaucoma associated with patients with Sturge-Weber syndrome.14 Increased episcleral venous pressure has been reported in glaucoma associated with Sturge-Weber syndrome. It may be an important element in the mechanism of this glaucoma resistant to goniosurgery.15 The advantage of goniosurgery as the primary surgical intervention is the low risk of prolonged hypotony and related posterior segment postoperative complications.16 Goniotomy also has a low risk of surgical complications and allows for the preservation of the conjunctiva for future filtration or drainage tube surgery, if needed.
Awad et al17 reported the success of medical control of glaucoma associated with Sturge-Weber syndrome for 7 of 22 eyes (32%) without acetazol-amide use and recommended medical therapy as the initial treatment of choice. Glaucoma implant surgery was successfully performed for 10 eyes of 9 children with glaucoma associated with Sturge-Weber syndrome at an average age of 6 years without the occurrence of significant complications or need for additional glaucoma surgery.18 Cibis et al2 reported the successful use of trabeculotomy and trabeculectomy, and Mandal19 reported success with trabeculotomy-trabeculectomy for glaucoma associated with Sturge-Weber syndrome in 9 children with an average age of 1.5 years. Akhter and Salim20 reported that glaucoma associated with Sturge-Weber syndrome often requires drainage tube surgery or trabeculectomy for early-onset glaucoma associated with Sturge-Weber syndrome and initial trabeculectomy for late-onset cases.
We now recommend initial medical treatment for patients of all ages with glaucoma associated with Sturge-Weber syndrome. Early-onset glaucoma associated with Sturge-Weber syndrome treated with goniosurgery may be helpful when combined with the continued use of medications. Before 1 year of age, tube surgery should be first considered when additional surgery is required. For 18 patients with glaucoma associated with Sturge-Weber syndrome in our reported cohort who failed goniosurgery, trabeculectomy was successfully performed for 14 of 19 eyes (74%) at an average age of 5.1 years with a mean postoperative follow-up interval of 6.4 years (range: 0.5 to 13 years). It is our preference to perform trabeculectomy for initial surgery for patients older than 1 year with secondary tube shunt surgery when this has failed at the site of the failed trabeculectomy. For those younger than 1 year who fail goniosurgery and require further glaucoma surgery, our preference is primary implantation of glaucoma tube shunt.
- Patrianakos TD, Nagao K, Walton DS. Surgical management of glaucoma with the Sturge Weber syndrome. Int Ophthalmol Clin. 2008;48(2):63–78. doi:10.1097/IIO.0b013e3181693582 [CrossRef]
- Cibis GW, Tripathi RC, Tripathi BJ. Glaucoma in Sturge-Weber syndrome. Ophthalmology. 1984;91(9):1061–1071. doi:10.1016/S0161-6420(84)34194-X [CrossRef]
- Olsen KE, Huang AS, Wright MM. The efficacy of goniotomy/trabeculotomy in early-onset glaucoma associated with the Sturge-Weber syndrome. J AAPOS. 1998;2(6):365–368. doi:10.1016/S1091-8531(98)90036-2 [CrossRef]
- Ho CL, Walton DS. Goniosurgery for glaucoma secondary to chronic anterior uveitis: prognostic factors and surgical technique. J Glaucoma. 2004;13(6):445–449. doi:10.1097/01.ijg.0000141368.96950.3c [CrossRef]
- Schirmer R. Ein Fall von Teleangiektasie. Arch Für Ophthalmol Vol. 1860;7(1):119–121.
- Barkan O. Goniotomy for glaucoma associated with nevus flammeus; successful operation in two cases. Am J Ophthalmol. 1957;43(4 Part 1):545–549. doi:10.1016/0002-9394(57)90807-3 [CrossRef]
- Lister A. The prognosis in congenital glaucoma. Trans Ophthalmol Soc U K. 1966;86:5–18.
- Wu Y, Yu R, Chen D, et al. Early trabeculotomy ab externo in treatment of Sturge-Weber syndrome. Am J Ophthalmol. 2017;182:141–146. doi:10.1016/j.ajo.2017.08.002 [CrossRef]
- Iwach AG, Hoskins HD Jr, Hetherington J Jr, Shaffer RN. Analysis of surgical and medical management of glaucoma in Sturge-Weber syndrome. Ophthalmology. 1990;97(7):904–909. doi:10.1016/S0161-6420(90)32483-1 [CrossRef]
- Sullivan TJ, Clarke MP, Morin JD. The ocular manifestations of the Sturge-Weber syndrome. J Pediatr Ophthalmol Strabismus. 1992;29(6):349–356.
- Herrygers L. Sturge-Weber syndrome and glaucoma. Glaucoma Today. 2004;(September/October):16–17.
- World Glaucoma Association, eds. Childhood Glaucoma: The 9th Consensus Report of the World Glaucoma Association [Vancouver, July 17–20, 2013]. Kugler; 2013.
- Akabane N, Hamanaka T. Histopathological study of a case with glaucoma due to Sturge-Weber syndrome. Jpn J Ophthalmol. 2003;47(2):151–157. doi:10.1016/S0021-5155(02)00702-5 [CrossRef]
- Asano Y, Kimura R. Glaucoma associated with the Sturge-Weber Syndrome: a case report. Glaucoma. 1983;5:186–189.
- Shiau T, Armogan N, Yan DB, Thomson HG, Levin AV. The role of episcleral venous pressure in glaucoma associated with Sturge-Weber syndrome. J AAPOS. 2012;16(1):61–64. doi:10.1016/j.jaapos.2011.09.014 [CrossRef]
- Eibschitz-Tsimhoni M, Lichter PR, Del Monte MA, et al. Assessing the need for posterior sclerotomy at the time of filtering surgery in patients with Sturge-Weber syndrome. Ophthalmology. 2003;110(7):1361–1363. doi:10.1016/S0161-6420(03)00456-1 [CrossRef]
- Awad AH, Mullaney PB, Al-Mesfer S, Zwaan JT. Glaucoma in Sturge-Weber syndrome. J AAPOS. 1999;3(1):40–45. doi:10.1016/S1091-8531(99)70093-5 [CrossRef]
- Budenz DL, Sakamoto D, Eliezer R, Varma R, Heuer DK. Two-staged Baerveldt glaucoma implant for childhood glaucoma associated with Sturge-Weber syndrome. Ophthalmology. 2000;107(11):2105–2110. doi:10.1016/S0161-6420(00)00381-X [CrossRef]
- Mandal AK. Primary combined trabeculotomy-trabeculectomy for early-onset glaucoma in Sturge-Weber syndrome. Ophthalmology. 1999;106(8):1621–1627. doi:10.1016/S0161-6420(99)90462-1 [CrossRef]
- Akhter K, Salim S. Sturge-Weber syndrome and secondary glaucoma. EyeNet. 2014;(October):49–51.
Baseline Characteristics of Patients With Sturge-Weber Syndrome and Glaucoma Undergoing Goniosurgery
|Eyes undergoing goniosurgery (total)||46|
| Goniotomy and trabeculotomy||2|
|Average age at goniosurgery (years)||1.5 (range: 0.2 to 23)|
|Average IOP prior to goniosurgery (mm Hg)||35 (range: 25 to 50)|