title: Visual outcomes and late complications in paediatric orbital rhabdomyosarcoma.
abstract: BACKGROUND
  Orbital rhabdomyosarcoma is a rare but important malignancy for an ophthalmologist. We aimed to review the management and outcome, including late orbital complications and visual acuity over 25 years from a specialist paediatric ophthalmology department.
  
  
  METHODS
  This was a retrospective longitudinal case series.
  
  
  METHODS
  All patients presenting to our institution between December 1989 and December 2014 with a histopathological diagnosis of rhabdomyosarcoma originating from (primary) or invading into the orbit (paranasal) were included.
  
  
  METHODS
  The oncology and ophthalmology databases were cross referenced to identify patients.
  
  
  METHODS
  Baseline demographics, chemotherapy, surgical and radiation dose, visual acuity, ocular and systemic complications, local and distant recurrence and mortality were recorded for each patient. Outcomes were reported with descriptive statistics.
  
  
  RESULTS
  Eighteen patients were included. Median age was 4.3 years (range 4 months to 16 years) with average follow-up of 9 years. The 5-year disease-specific survival was 100% for the orbital group and 25% for the paranasal group; 29% of the orbital group maintained vision better than 6/12 in their treated eye, and the overall globe conservation rate was 71%. The most common ocular complications were cataract and keratopathy in both the orbital and paranasal groups. Other ocular complications included orbital hypoplasia or fat atrophy, eyelid malposition and lacrimal duct stenosis.
  
  
  CONCLUSIONS
  Ophthalmic late effects are seen in a significant proportion of patients with orbital rhabdomyosarcoma. There is excellent survival in these patients, and continued efforts should be made to reduce the late effects of therapy.
authors.affiliation: Department of Ophthalmology, Children's Hospital Westmead, Sydney, New South Wales, Australia.
authors.forename: Edwina
authors.initials: E
authors.lastname: Eade
authors.affiliation: Department of Ophthalmology, Children's Hospital Westmead, Sydney, New South Wales, Australia.
authors.forename: Krishna
authors.initials: K
authors.lastname: Tumuluri
authors.affiliation: Department of Ophthalmology, Children's Hospital Westmead, Sydney, New South Wales, Australia.
authors.forename: Helen
authors.initials: H
authors.lastname: Do
authors.affiliation: Department of Ophthalmology, Children's Hospital Westmead, Sydney, New South Wales, Australia.
authors.forename: Neil
authors.initials: N
authors.lastname: Rowe
authors.affiliation: Department of Ophthalmology, Children's Hospital Westmead, Sydney, New South Wales, Australia.
authors.forename: James
authors.initials: J
authors.lastname: Smith
mesh_terms: D000293:Adolescent; D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use*; D015353:Eye Enucleation / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology*; D009517:New South Wales / Q000453:epidemiology; D009918:Orbital Neoplasms / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D014792:Visual Acuity
chemical_list: D018906:Antineoplastic Agents, Alkylating; D003520:Cyclophosphamide
keywords: complications; orbit; rhabdomyosarcoma; treatment; vision