April 2014
Volume 55, Issue 13
ARVO Annual Meeting Abstract  |   April 2014
Children and young adults with anophthalmia/microphthalmia treated with ocular prosthesis
Author Affiliations & Notes
  • Marita Andersson Gronlund
    Pediatric Ophthalmology, Inst Neurosci & Phys/Ophthal, Goteborg, Sweden
  • Marie Odersjo
    Dept of Otolaryngology, Gothenburg, Sweden
  • Gun Lindgren
    Pediatric Ophthalmology, Inst Neurosci & Phys/Ophthal, Goteborg, Sweden
  • Alexandra Topa
    Dept of Clinical Genetics, Gothenburg, Sweden
  • Ylva Jugard
    Pediatric Ophthalmology, Inst Neurosci & Phys/Ophthal, Goteborg, Sweden
  • Footnotes
    Commercial Relationships Marita Gronlund, None; Marie Odersjo, None; Gun Lindgren, None; Alexandra Topa, None; Ylva Jugard, None
  • Footnotes
    Support None
Investigative Ophthalmology & Visual Science April 2014, Vol.55, 4491. doi:
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      Marita Andersson Gronlund, Marie Odersjo, Gun Lindgren, Alexandra Topa, Ylva Jugard; Children and young adults with anophthalmia/microphthalmia treated with ocular prosthesis. Invest. Ophthalmol. Vis. Sci. 2014;55(13):4491.

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      © ARVO (1962-2015); The Authors (2016-present)

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Purpose: Anophthalmia and microphthalmia (A/M) are rare congenital malformations due to genetic defects, infections or drug exposure during pregnancy. A/M often occurs with other disorders, both ocular and general. Early intervention with ocular prosthesis can stimulate orbital growth and prevent facial asymmetry. The aim of this study was to describe a group of children/young adults with A/M emphasizing on investigation, treatment and follow-up.

Methods: A review of medical records of 18 individuals (10 females, 8 males) with a mean age of 8.7 years (range 0.8 - 31.8) with A/M treated with ocular prosthesis at Sahlgrenska University Hospital, Gothenburg, Sweden between year 2000 and 2012.

Results: The median age by diagnosis was 10 days (1-110 days); n=16. In this group of 18 individuals, three have anophthalmia (A) and 15 microphthalmia (M). A majority has other malformations in the A/M eye and eight individuals have findings in the fellow eye. MRI/CT revealed CNS pathology in 2 out of 14 cases. Visual evoked potential (VEP) was performed in seven cases, all pathological. Seven individuals have subnormal visual acuity in the fellow eye, s ix are in contact with vision support services and 15 have a follow-up at an eye clinic. Almost half of the group wears glasses. Eleven individuals have extra ocular findings (i.e. cardiac defects, autism, septo-optic dysplasia, and hearing impairment), which indicate a syndromic condition. Genetic tests (karyotype, array and/or targeted gene analysis) were done in five cases without significant results. Intrauterine varicella infection caused one M. Ocular prosthesis was started at a median age of 7.8 months (22 days-16 years). Treatment with ocular prosthesis was tolerated well, no complication but local irritation in form of redness and secretion were noted; especially when the child had a cold. All, but two were treated locally by antibiotics one or several times. Four children, (A, n=2; M, n=2) had ocular plastic surgery.

Conclusions: The investigation of A/M children should include MRI of CNS and orbit, ultrasound of the bulb and VEP. The ophthalmologist who makes the diagnosis A/M needs to monitor the visual development of the fellow eye but also initiate treatment with prosthesis, pediatric assessment, hearing test and genetic counseling.

Keywords: 421 anterior segment • 462 clinical (human) or epidemiologic studies: outcomes/complications • 419 anatomy  

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