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My friend's baby in india having poor vision. Doctors from sankara nethralaya suggested that there may be some solution for her problem in USA. We don't know whom to approach to find the solution. I am posting her case report. It will be helpful if someone tell us if some treatment exists for this problem.
Case Report is:
Case Summary:
a 3 month old child was seen
at Sankara Nethralaya initially on 22.04.2004 with the
parents complaining of noticing smaller appearance of
the left eye since birth and the child had been
diagnosed to have buphthalmos with leucocoria in both
the eyes elsewhere. The child had been diagnosed to
have PHPV on CT Scan examination and was on Timolet
GFS 0.5% once a day in both the eyes.
On examination, the child was noted to have
buphthalmos in both the eyes the right eye being
larger that left eye with a flat anterior chamber,
band shaped keratopathy in the right cornea, small
bound down pupil and clear lens. No mass lesion in
the anterior chamber, nodule, hypopyon or rubesosis
could be seen in either eyes. The cornea was hazy in
both the eyes. There was leukocoria at the pupil in
both the eyes. Fundus examination of both the eyes
showed a clear anterior vitreous and a dense white
reflex throughout the fundus in both the eyes but no
obvious mass lesion. The retina could not be seen in
either eye. Ultrasound examination of both eyes
should presence of a total retinal detachment highly
elevated with subretinal space full of moderate to
high reflective dot echoes and no obvious lesion or
clarification. The left eye axial length was smaller
that that of the right eye by 3 mm. Review of the CT
scan showed the presence of shifting fluid in both the
eyes but no obvious mass lesion or calcification.
Birth history of the child was not exactly
significant. The child was born of a full term
cesarian delivery with a normal neonatal period and no
history of oxygen administration. The elder sibling
of this child is 8 years of age and is normal and
there was no family history of retinoblastoma.
Baby was diagnosed to have secondary
buphthalmos and a remote possibility of retinoblastoma
exists in this child. However, clinical examinations
ultrasound and CT are not suggestive of
retinoblastoma. MRI has been suggested to evaluate
both eyes further.
The poor visual prognosis has been explained to parents and in case the doubt of
retinoblastoma still exists one can consider
enucleation of the right eye and histopathological
examination. These were discussed with the childs
parents and they are to decide about the same.
MRI Report Brain & Orbit
Name : Baby
Age : 3 months
Sex : Female
Referring consultants : Dr.MPS S/N
Technique:
BRAIN : AXIAL FLAIR, CORONAL T2
ORBIT : AXIAL T1 AND T2, CORONAL T2
FINDINGS:
The posterior fossa shows cerebellum. The fourth
ventricle shows normal size, shape and position. Both
the C.P angles are clear. The orbits and their
contents are normal.
The Medulla, pons and midbrain show normal signals in
both the sequences. The basal cisterns are normal.
The pituitary gland and optic chiasm are normal.
The third and lateral ventricles are of normal in
size, shape and position. No midline shift is noted.
The thalami, basal ganglia and internal capsules are
normal on both sides.
Both the cerebral hemispheres show normal intensities
of grey and white matter. The cerebral sulci are
normal. The extra cerebral spaces are clear.
The right globe appears slightly larger when compared
to the left with deep anterior chamber. There is
evidence of bilateral total retinal detachment. The
sub retinal fluid displays mixed hypo and hyperintense
signal in T1WI and hypointense in T2WI suggestive of
mixed acute and sub acute blood. No obvious mass
lesion seen within.
The extra ocular muscles are normal in thickness and
signal characteristics.
Both optic nerves display normal course, caliber and
signal characteristics.
Both lacrimal glands are normal. Retro orbital fat
shows normal signal intensity.
IMPRESSION:
MR FEATURES ARE SUGGESTIVE OF BILATERAL RETINAL
DETACHMENT.
Case Report is:
Case Summary:
a 3 month old child was seen
at Sankara Nethralaya initially on 22.04.2004 with the
parents complaining of noticing smaller appearance of
the left eye since birth and the child had been
diagnosed to have buphthalmos with leucocoria in both
the eyes elsewhere. The child had been diagnosed to
have PHPV on CT Scan examination and was on Timolet
GFS 0.5% once a day in both the eyes.
On examination, the child was noted to have
buphthalmos in both the eyes the right eye being
larger that left eye with a flat anterior chamber,
band shaped keratopathy in the right cornea, small
bound down pupil and clear lens. No mass lesion in
the anterior chamber, nodule, hypopyon or rubesosis
could be seen in either eyes. The cornea was hazy in
both the eyes. There was leukocoria at the pupil in
both the eyes. Fundus examination of both the eyes
showed a clear anterior vitreous and a dense white
reflex throughout the fundus in both the eyes but no
obvious mass lesion. The retina could not be seen in
either eye. Ultrasound examination of both eyes
should presence of a total retinal detachment highly
elevated with subretinal space full of moderate to
high reflective dot echoes and no obvious lesion or
clarification. The left eye axial length was smaller
that that of the right eye by 3 mm. Review of the CT
scan showed the presence of shifting fluid in both the
eyes but no obvious mass lesion or calcification.
Birth history of the child was not exactly
significant. The child was born of a full term
cesarian delivery with a normal neonatal period and no
history of oxygen administration. The elder sibling
of this child is 8 years of age and is normal and
there was no family history of retinoblastoma.
Baby was diagnosed to have secondary
buphthalmos and a remote possibility of retinoblastoma
exists in this child. However, clinical examinations
ultrasound and CT are not suggestive of
retinoblastoma. MRI has been suggested to evaluate
both eyes further.
The poor visual prognosis has been explained to parents and in case the doubt of
retinoblastoma still exists one can consider
enucleation of the right eye and histopathological
examination. These were discussed with the childs
parents and they are to decide about the same.
MRI Report Brain & Orbit
Name : Baby
Age : 3 months
Sex : Female
Referring consultants : Dr.MPS S/N
Technique:
BRAIN : AXIAL FLAIR, CORONAL T2
ORBIT : AXIAL T1 AND T2, CORONAL T2
FINDINGS:
The posterior fossa shows cerebellum. The fourth
ventricle shows normal size, shape and position. Both
the C.P angles are clear. The orbits and their
contents are normal.
The Medulla, pons and midbrain show normal signals in
both the sequences. The basal cisterns are normal.
The pituitary gland and optic chiasm are normal.
The third and lateral ventricles are of normal in
size, shape and position. No midline shift is noted.
The thalami, basal ganglia and internal capsules are
normal on both sides.
Both the cerebral hemispheres show normal intensities
of grey and white matter. The cerebral sulci are
normal. The extra cerebral spaces are clear.
The right globe appears slightly larger when compared
to the left with deep anterior chamber. There is
evidence of bilateral total retinal detachment. The
sub retinal fluid displays mixed hypo and hyperintense
signal in T1WI and hypointense in T2WI suggestive of
mixed acute and sub acute blood. No obvious mass
lesion seen within.
The extra ocular muscles are normal in thickness and
signal characteristics.
Both optic nerves display normal course, caliber and
signal characteristics.
Both lacrimal glands are normal. Retro orbital fat
shows normal signal intensity.
IMPRESSION:
MR FEATURES ARE SUGGESTIVE OF BILATERAL RETINAL
DETACHMENT.
