Juxtapapillary capillary hemangiomas. Clinical features and visual acuity outcomes.
OBJECTIVE
To describe the clinical features, association with von Hippel-Lindau (VHL) disease and visual acuity outcomes of patients with a juxtapapillary capillary hemangioma.


METHODS
Retrospective observational case series.


METHODS
Seventy-two eyes of 68 patients identified with a juxtapapillary capillary hemangioma. Follow-up data of at least 6 months duration were available for 60 eyes.


METHODS
A retrospective chart review of patients diagnosed with a juxtapapillary capillary hemangioma examined at four medical centers.


METHODS
Age at diagnosis, visual acuity (VA) at first examination and at last follow-up, tumor growth pattern and location, associated clinical features, type of treatment, association with VHL, and presence of peripheral hemangiomas were recorded for each patient.


RESULTS
On initial examination, VA was >/=20/40 in 43 of 70 eyes (61%) and was >/=20/200 in 60 eyes (86%). At an average follow-up of 5.4 years (range, 0.5-19 years), VA of >/=20/40 was achieved in 21 eyes (35%) and >/=20/200 in 33 eyes (55%). Patients with VHL had poorer initial VA (48% vs. 70% with VA >/=20/40, and 74% vs. 93% with VA >/=20/200) and final VA (26% vs. 41% with VA >/=20/40, and 39% vs. 65% with VA >/=20/200) compared with patients without VHL. Patients with VHL more commonly were seen at an earlier age (average, 20 vs. 44 years, P: < 0.001), with bilateral (17% vs. 0%), and/or peripheral (39% vs. 0%) (P: < 0.001) tumors that more often had an endophytic growth pattern (63% vs. 22%, P: = 0.001) compared with patients without VHL. Patients selected for laser treatment generally had poorer initial (52% vs. 74% with VA >/=20/40, 79% vs. 96% with VA >/=20/200) and final VAs (18% vs. 56% with VA >/=20/40, 45% vs. 67% with VA >/=20/200) compared with patients not treated with laser.


CONCLUSIONS
On long-term follow-up of patients with a juxtapapillary capillary hemangioma, the VA generally worsens. Patients with VHL and a juxtapapillary hemangioma more often present at a younger age, have tumors with an endophytic growth pattern, and have bilateral, multiple tumors. Treatment with laser photocoagulation results in variable VA outcomes.
McCabe
C M
CM
Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami School of Medicine, Miami, Florida, USA.
Flynn
H W
HW
Shields
C L
CL
Shields
J A
JA
Regillo
C D
CD
McDonald
H R
HR
Berrocal
M H
MH
Gass
J D
JD
Mieler
W F
WF
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D005451:Fluorescein Angiography; D005500:Follow-Up Studies; D018324:Hemangioma, Capillary / Q000150:complications / Q000473:pathology* / Q000601:surgery; D006801:Humans; D017075:Laser Coagulation; D008297:Male; D008875:Middle Aged; D009898:Optic Disk / Q000473:pathology* / Q000601:surgery; D019574:Optic Nerve Neoplasms / Q000150:complications / Q000473:pathology* / Q000601:surgery; D012189:Retrospective Studies; D014792:Visual Acuity; D006623:von Hippel-Lindau Disease / Q000150:complications