I saw Max Kimpland for neuro-ophthalmic consultation regarding optic pathway gliomas.

He is 5 years of age and right-handed. He has been given a diagnosis of Asperger’s syndrome and he was noted to have cafe-au-lait spots in March 2004. A CAT scan of the head on May 14th  showed left orbital and chiasmal optic pathway gliomas. We reviewed the MRI of the brain from 6/3/04 and there was a fairly significant sellar mass and involvement of both optic nerves.

He got glasses in May after seeing Dr. Chen due to poor vision in both eyes.

There is no family history of neurofibromatosis. He has no cutaneous neurofibromas. 

With correction he saw 20/100 with the right eye and 20/250 with the left. The best that we could get him to do with the color plates was 3/8 with the right and 1/8 with the left. He appeared to have full fields to confrontation techniques. Both pupils are 4mm, but I thought he had an afferent pupillary defect on the left.

He had trace ptosis on the left, but normal eyelid function and no palpable masses.  Ocular ductions are full and alignment is normal. He had occasional rotary nystagmus in the left eye. Slit lamp examination is unremarkable without Lisch nodules. The dilated funduscopic examination was remarkable for optic atrophy, left more than right.

Given the cafe-au-lait spots and optic pathway glioma I suspect that he has neurofibromatosis.

He has bilateral vision loss related to the optic pathway involvement, with likely contributions from both the optic nerve and the sellar components. I discussed with the family that the options are to observe because we do not know how long he has had this, versus chemotherapy. I would favor treatment because the lesion is quite large and he is certainly a risk for further vision loss.  I communicated my opinion to Dr. Belasco. She told me after their visit with her that she is planning chemotherapy. 

I discussed with the family that the goal of chemotherapy is to prevent worsening of the vision loss and unfortunately not necessarily to regain vision.

I can see him back in a few months and recheck his visual status.

I saw Max Kimpland for neuro-ophthalmic follow-up regarding his optic pathway glioma in the setting of neurofibromatosis.

I reviewed the MRI from 1/11/05.  The optic nerve gliomas were stable, but there was increased prominence of the pontine glioma on the left.  A hypothalamic glioma was unchanged.

He is on Carboplatin, Vincristine, Zofran, Glycolax, Bactrim and Fluconazole.  His father feels his vision function is about the same.

Using Snellen lines he saw 20/70 with the right eye and 20/400, difficulty with the left.  He saw 4/8 color plates but 1.5/8 with the left.  He had constriction of the visual field with the left eye.  He had a trace afferent pupillary defect  on the left versus no APD.  This was difficult because the lack of cooperation.  Penlight examination of the anterior segment is unremarkable.  Ocular ductions are full.  On the undilated funduscopic examination he has pale optic nerves bilaterally.  Facial sensation and strength were normal.

His visual acuity on the left is a little bit worse than it was on December 2nd, but his examination is comparable to the way it looked on 7/29/04.  Perhaps this represents variation in a child who is not fully cooperative.  it is hard to say whether there is definite worsening or not.  I suspect the best thing to do again is to repeat the examination in six weeks.

I saw Max Kimpland for neuro-opthalmic follow-up regarding his optic pathway glioma in the setting of neurofibromatosis. 

The patient is 5 years of age.  He has an MRI scheduled for tomorrow.  His vision has remained stable.  He has had Vincristine neuropathy with loss of reflexes.

He saw 20/60 with the right eye and 20/400 with the left.  He saw 6/7 color plates with the right eye, but 0/5 with the left.  He had a temporal field defect with the left eye.  He had an afferent pupillary defect on the left.  Ocular ductions are full and he had a trace of exodeviation.  He has flat colored lesions on his irises.  He had a trace exodeviation in primary gaze.  He has 1 or 2mm of ptosis on the left, but there is no palpable mass.  On the dilated funduscopic examination he has pale optic nerves bilaterally.  Cycloplegic automated refraction was -0.50+0.50X149 O.D. and -0.50+2.00X177 O.S. 

His vision in his right eye has improved.  I got a sense from his visual behavior today that his difficulties on previous examinations may have been reflective of simple poor visual function, or it could have been a combination of poor visual function plus lack of cooperation.  In any case, he is better today.  I will see him back in six months.