Grand Rounds Nanophthalmos

1
Grand RoundsNanophthalmos

• Mark Sherman MD
• University of Louisville
• Department of Ophthalmology and Visual Sciences
• 2/20/2015

2
Subjective

• CC Decreased vision OU x several years
• HPI 23 year old white female presented to the
  cornea clinic for evaluation of decreased vision
  and hazy corneas OU. The patient stated that the
  right eye has always had low vision since she had
  cataract surgery, but the left eye was okay
  until 2-3 years ago when the vision began to
  progressively deteriorate. The patient had not
  see an ophthalmologist since her previous
  surgeries.
• POH Nanophthalmos OU, congenital nystagmus,
  chronic angle closure glaucoma OU, sulcus IOL OD
  (2009), ACIOL OS (2009)
• PMH None

3
Exam

• OD
  OS
• BCVA CF _at_1 CF
  _at_3
• Pupils Irregular OU
• IOP 27 31
• EOM Full OU horizontal nystagmus
• CVF Mild Constriction OU

4
Exam

• SLE OD OS
• L/L WNL WNL
• Conjunctiva WNL WNL
• K Scar from previous Diffuse Haze and
  edema cataract surgery decompensated
• Scar from previous cataract
  surgery
• AC Shallow Shallow
• I/L Corectopia PCIOL Corectopia ACIOL
• DFE Pallor MVP WNL No view
  (B-scan flat)

5
Clinic Exam OD
Corneal diameter 9.0 mm
6
Clinic Exam OS
Corneal diameter 8.5 mm
7
Assessment/Plan

• Assessment 23 year old white female with
  nanophthalmos OU, chronic low vision OD secondary
  to chronic angle closure glaucoma, and a
  decompensated cornea OS secondary to bullous
  keratopahy
• Plan Penetrating keratoplasty OS
• Start topical glaucoma drops OU
• Refer to glaucoma clinic after surgery

8
Surgical Video
9
2 week follow up OS

• VA 20/125 IOP 25 mmHG

10
Nanophthalmos

• Rare disease characterized by a small eye
  secondary to compromised growth
• Typically have extreme axial hyperopia (15-20 mm
  axial length and 7-15 diopters) without any other
  obvious structure defects
• Usually a small, functional eye with relatively
  normal internal organization and proportions as
  apposed to microphthalmos which has a small
  disorganized globe with little to no visual
  potential

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Nanophthalmos

• Often have a high lens-to-eye volume leading to
  crowding of the anterior segment and
  angle-closure glaucoma
• Can be sporadic or hereditary
• both autosomal dominant and recessive inheritance
  patterns have been reported
• Autosomal dominant variant has been mapped to
  chromosome 11

12
Nanophthalmos

• Refractive error is managed with fully corrected
  cycloplegic refraction
• Often younger patient require bifocals due to
  incomplete accommodation ablility
• Angle-closure glaucoma is managed medically
• Often peripheral laser iridotomy with peripheral
  laser iridoplasty are effective in treatment of
  the angle-closure glaucoma
• Cataract surgery is often complicated by uveal
  effusion or hemorrhage and/or retinal detachment

13
References

• BCSC External Disease and Cornea. Nanophthalmos.
  Pgs 250-51.
• BCSC External Disease and Cornea.
  Microphthalmos. Pgs 249-250.
• Faucher A. Hasanee K, et al. Phacoemulsification
  and introcular lens implantation in nanophthalmic
  eye report of medium size series. J Cataract
  Refractive Surgery. 200228.
• Othman MI, Sullican SA, et al. Autosomal dominant
  nanophthalmos with high hyperopia and
  angle-closure glaucoma mapts to chromosome 11. Am
  J Hum Genetics. 199863(5) 1411-18.
• Yamani A. Wood I, et al. Abnormal collagen
  fibrils in nanophthalmos a clinical and
  hstiologic study. Am J Ophthamol.
  199127(1)106-8.