Rare Diseases Symptoms Automatic Extraction

Long-term results after proton beam therapy for retinal papillary capillary hemangioma.

[von hippel-lindau disease]

To evaluate the potential benefit and risk of proton beam therapy in the treatment of symptomatic retinal papillary capillary hemangioma.Retrospective interventional case series.This study included patients presenting with symptomatic exudative retinal papillary capillary hemangioma with or without association with von Hippel-Lindau disease. All patients were treated either as a first or a secondary treatment option by proton beam therapy between 2001 and 2009. The minimum follow-up was 30 months.Eight eyes of 8 patients (3 male and 5 female, with a mean age of 36 years [range 22-80 years]) were treated for symptomatic papillary retinal hemangioma. The median interval between onset of macular edema and proton beam therapy was 1.7 months (range 0.5-3.3 months). The median follow-up period was 84 months (range 32-106 months) between proton beam treatment and last follow-up. Exudation completely resolved in all but 1 patient after 4.2 months on average (range 2.8-7.2 months). Mean visual acuity prior to proton beam irradiation was 0.7 logMAR (0.2 DIN (DIN 58220 norm)) (range 2-0.3 logMAR) and declined to 0.8 logMAR (0.16 DIN; range 2-0.1 logMAR) at last follow-up examination (no statistical significance, P=.071).The anatomic outcome after proton beam therapy for retinal papillary hemangioma is convincing, whereas functional outcome may be compromised because of tumor location, long-persisting macular edema, extensive exudation, and poor initial visual acuity. In patients with extended retinal detachment surgical intervention was still necessary. Although proton beam therapy is proven to be a therapeutic option, treatment will remain challenging.

Diseases presenting "edema" symptom

  • acute rheumatic fever
  • adrenomyeloneuropathy
  • aniridia
  • cadasil
  • canavan disease
  • classical phenylketonuria
  • coats disease
  • congenital toxoplasmosis
  • cushing syndrome
  • cutaneous mastocytosis
  • erythropoietic protoporphyria
  • esophageal carcinoma
  • fabry disease
  • familial mediterranean fever
  • focal myositis
  • harlequin ichthyosis
  • homocystinuria without methylmalonic aciduria
  • inclusion body myositis
  • lamellar ichthyosis
  • liposarcoma
  • lymphangioleiomyomatosis
  • malignant atrophic papulosis
  • pleomorphic liposarcoma
  • systemic capillary leak syndrome
  • trochlear dysplasia
  • von hippel-lindau disease
  • waldenström macroglobulinemia

This symptom has already been validated