Transcript Grand Rounds

Grand Rounds
Nanophthalmos
Mark Sherman MD
University of Louisville
Department of Ophthalmology and Visual Sciences
2/20/2015
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
Exam
BCVA:
OD
CF @1’
Pupils:
IOP:
EOM:
CVF:
OS
CF @3’
Irregular OU
27
31
Full OU +horizontal nystagmus
Mild Constriction OU
Exam
SLE:
OD
OS
L/L
 Conjunctiva
 K
WNL
WNL
WNL
WNL
AC
 I/L
Shallow
Shallow
Corectopia +PCIOL
Corectopia +ACIOL
++Pallor MVP WNL
No view (B-scan flat)

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DFE:
Scar from previous
cataract surgery
Diffuse Haze and edema
decompensated
Scar from previous
cataract surgery
Clinic Exam OD
Corneal diameter ~9.0 mm
Clinic Exam OS
Corneal diameter ~8.5 mm
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
Surgical Video
2 week follow up OS

VA: 20/125 IOP: 25 mmHG
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
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

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both autosomal dominant and recessive inheritance patterns
have been reported
Autosomal dominant variant has been mapped to
chromosome 11
Nanophthalmos

Refractive error is managed with fully corrected
cycloplegic refraction


Angle-closure glaucoma is managed medically


Often younger patient require bifocals due to incomplete
accommodation ablility
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
References
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BCSC: External Disease and Cornea. Nanophthalmos. Pgs: 250-51.
BCSC: External Disease and Cornea. Microphthalmos. Pgs: 249250.
Faucher A. Hasanee K, et al. Phacoemulsification and introcular
lens implantation in nanophthalmic eye: report of medium size
series. J Cataract Refractive Surgery. 2002;28.
Othman MI, Sullican SA, et al. Autosomal dominant
nanophthalmos with high hyperopia and angle-closure glaucoma
mapts to chromosome 11. Am J Hum Genetics. 1998;63(5) 141118.
Yamani A. Wood I, et al. Abnormal collagen fibrils in
nanophthalmos: a clinical and hstiologic study. Am J Ophthamol.
199;127(1):106-8.