Clinical Features and Surgical Outcomes of Sturge-Weber Syndrome with Glaucoma

Jae Hwi Park; Su Ho Lim; Soon Cheol Cha

doi:10.3341/jkos.2013.54.11.1737

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Park, Lim, and Cha: Clinical Features and Surgical Outcomes of Sturge-Weber Syndrome with Glaucoma

Original Article

J Korean Ophthalmol Soc 2013;54(11):1737-1747.

Published online: 2 September 2013

DOI: https://doi.org/10.3341/jkos.2013.54.11.1737

Clinical Features and Surgical Outcomes of Sturge-Weber Syndrome with Glaucoma

Jae Hwi Park, MD, Su Ho Lim, MD, Soon Cheol Cha, MD, PhD

Department of Ophthalmology, Yeungnam University College of Medicine, Daegu, Korea

Address reprint requests to Soon Cheol Cha, MD, PhD, Department of Ophthalmology, Yeungnam University Medical Center, #170 Hyeonchung-ro, Nam-gu, Daegu 705-703, Korea, Tel: 82-53-620-3442, Fax: 82-53-626-5936, E-mail: sccha@yumail.ac.kr

Received 25 April 2013 Revised 20 May 2013 Accepted 1 October 2013

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

To report clinical manifestations including neurocutaneous and ocular findings and to evaluate outcomes of tra- beculectomy in patients with Sturge-Weber syndrome.

Methods

The medical records of 10 eyes of 8 glaucoma patients with Sturge-Weber syndrome who were followed up for at least 1 year after trabeculectomy were reviewed retrospectively. We analyzed neurocutaneous and ocular findings, cumulative surgical success rates, and complications in patients with Sturge-Weber syndrome.

Results

The mean patient age at the time of surgery was 12.6 ± 13.0 years and mean follow-up period was 71.6 ± 81.8 months. All patients showed various clinical findings including facial hemangioma (8 patients), seizure (6 patients), intra- cranial lesion (6 patients), developmental delay (4 patients), conjunctival/episcleral hemangioma (4 eyes), and choroidal hemangioma (4 eyes). Postoperative success was achieved in 8 out of 10 eyes (80.0%). Postoperatively, serous retinal detachment occurred in 2 out of 4 eyes with preoperative diffuse choroidal hemangioma.

Conclusions

Management of glaucoma associated with Sturge-Weber syndrome requires multidisciplinary treatment because of systemic involvement. Trabeculectomy appears to be an effective and relatively safe surgical option for glaucoma associated with Sturge-Weber syndrome. However, serious complications such as serous retinal detachment should be considered when planning trabeculectomy for patients with diffuse choroidal hemangioma.

Keywords: Glaucoma, Neurocutaneous findings, Sturge-Weber syndrome, Trabeculectomy

References

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Figure 1.

Conjunctival and/or episcleral hemangioma of patient. (A) Mildly engorged conjunctival and/or episcleral vessel with hypovascular bleb. (B) Moderately engorged conjunctival and/ or episcleral vessel with avascular bleb. (C, D) Severely engorged conjunctival and/or episcleral vessel.

Figure 2.

Diffuse choroidal hemangioma in patients with Sturge-Weber syndrome and Klippel-Trenaunay- Weber syndrome. (A) Right eye of patient 6. (B) Left eye of patient 2. (C) Left eye of patient 8.

Figure 3.

Kaplan-Meier survival curve showing the cumulative success rates of trabeculectomy for patients with Sturge-Weber syndrome.

Figure 4.

Patient 6 presented choroidal detachment with serous retinal detachment and optic disc swelling after trabeculectomy. (A, B) Severe choroidal detachment with serous retinal detachmnet and optic disc swelling on th 19th postoperative day. (C, D) Decreased choroidal detachment with serous retinal detachemnt and remained optic disc swelling on the 60th postoperative day. (E) No choroidal effusion or retinal detachment was noted 5 years after trabeculectomy.

Figure 5.

Immediately after removal of releasable suture at postoperative third days (Patient 1), (A, B) almost total hyphema was developed, and (C) massive subconjuntival hemorrahge was seen. (D) Hemorrhagic bleb was noted. (E, F) Two-year postoperative appearance of the bleb on the right eye of patient 1. The figures show diffuse moderately elevated, hypovascular bleb.

Figure 6.

Brain MRI in a patient with Sturge- Weber syndrome. (A) Leptomeningeal angioma (arrow) and choroid plexus hemangioma (arrow head) of patient 1. (B) Dominant choroidal plexus hemangioma (arrow) of patient 3. (C) Diffuse cortical atrophy (arrow) of patinet 8.

Figure 7.

Cutaneous manifestations of Sturge-Weber syndrome and Klippel- Trenaunay-Weber syndrome. (A) Portwine stain in patient 2 with Sturge- Weber syndrome. Note the hypertrophy and nodularity of facial angioma. (B) Bilateral involvement of facial hemangioma (Patient 5) (C) Limbal and truncal hemangioma in patient 7 with Klippel- Trenaunay-Weber syndrome. (D) Limbal soft tissue hypertrophy in patient 7 with Klippel-Trenaunay-Weber syndrome.

Table 1.

Patient demographics and clinical characteristics

Characteristics	Number (%)
Total patients	8
Total Eyes	10
Age (years)
Mean ± SD	12.6 ± 13.0
Range	0-39
Sex
Male	6 (75%)
Female	2 (25%)
Laterality of glaucoma
Unilateral	6 (75%)
Bilateral	2 (25%)
Preoperative IOP (mm Hg)	27.3 ± 5.3
Numbers of preoperative medications	2.8 ± 1.5
Follow-up period (months)	71.6 ± 81.8

Values are presented as mean ± SD.

SD = standard deviation; IOP = intraocular pressure.

Table 2.

Distribution of facial hemangioma in patients with Sturge-Weber syndrome

Patient No.	Side	Location
Patient No.	Side	V1	V2	V3
Patient 1	Right	+	+
Patient 2	Left	+	+	+
Patient 3	Left	+	+
Patient 4	Right	+	+
	Left	+	+
Patient 5	Right	+	+	+
	Left	+	+	+
Patient 6	Right	+	+	+
Patient 7	Right	+	+	+
	Left	+
Patient 8	Left	+	+

V1 = Ophthalmic branch of trigeminal nerve; V2 = Maxillary branch of trigeminal nerve; V3 = Mandibular branch of trigeminal nerve.

Table 3.

Ocular manifestations in Sturge-Weber syndrome (N = 8)

Ocular manifestation	Number (%)
Conjunctival and/or Episcleral hemangioma	4 (50)
Choroidal hemangioma	4 (50)
Ocular melanosis	3 (37.5)
Iris heterochromia	0 (0)

Table 4.

Details of patients with Sturge-Weber syndrome treated with trabeculectomy

	Diagnosis	Age^*	Sex	Side	Onset	Preop. IOP	Final IOP	Preop C/D	Final C/D	Postoperative complications
Patient 1	SWS	39 years	M	OD	J	24	8	0.95	0.95	Hyphema
Patient 2	SWS, KTW	27 years	M	OS	I	23	19	0.9	0.9	Wound leak
Patient 3	SWS	1 month	M	OS	I	25	27	0.85	0.8	SRD, Shallow A.C.
Patient 4	SWS, KTW	9 years	M	OS	J	23	16	0.95	Total cup	(-)
Patient 5	SWS, KTW	32 months	M	OD	I	23	33	0.95	0.7	Shallow A.C.
		7 years	M	OS	I	32	14	0.8	0.55	Shallow A.C.
Patient 6	SWS, KTW	5 years	F	OD	I	28	8	0.7	0.6	SRD, Shallow A.C., Hypotony
Patient 7	SWS, KTW	6 years	M	OD	I	35	12	0.8	0.6	Shallow A.C.
		6 years	M	OS	I	36	13	0.8	0.6	Shallow A.C.
Patient 8	SWS	25 years	F	OS	J	26	10	0.95	0.95	(-)

IOP = intraocular pressure; C/D = cup to disc ratio; SWS = Sturge-Weber syndrome; J = juvenile onset (above 2 years); KTW = Klippel-Trenaunay-Weber syndrome; I = infantile onset (below 2 years); SRD = serous retinal detachment; A.C. = anterior chamber.

^* Age at surgery.

Table 5.

Neurologic manifestations in Sturge-Weber syndrome (N = 8)

Neurologic manifestation	Number (%)
Seizure	6 (75)
Leptomeningeal hemangioma	6 (75)
Cerebral atrophy or calcification	5 (62.5)
Developmental delay	4 (50)
Mental retardation	3 (37.5)
Hemiparesis	2 (25)
Headache	2 (25)
Growth hormone deficiency	1 (12.5)

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