Download Atypical asymptomatic presentation of advanced pituitary adenoma

Atypical asymptomatic presentation of advanced pituitary adenoma that advances
Rapidly to Apoplexy
I. Case History
A. Patient Demographics 48 yo/AA/F
B. Referred in for glaucoma consultation, no complaints
C. OCULAR HISTORY: questionable past history of glaucoma, was taking
Timoptic 0.5% (in mid 1990's) but stopped drops as recommended by
another doctor. Borderline IOP, high myopia OU.
D. Medical history: cardiac arrhythmia, hypothyroidism, partial hysterectomy,
carpel tunnel, breast Bx. June 2009 (-)
II. Pertinent findings
A. Clinical
1. DVA CC 20/30 OD Ph 20/25
20/25 OS Ph 20/20
2 Pupils: PERRL (-) APD
3. SLE: Nml OU Cl cornea, conjunctiva, A/C, lens, vitreous OU
4. Ta OD 23 OS 23 @ 9:45 AM
5. DFE: Vitreous and macula clear OU, C/D .85/.70 OD .70/.75 OS
with slight temporal pallor OU, drance heme OS
6. Initial VF 24-2: high false positives essentially full OU; retest OS 2
months later paracentral scotoma OS
7. OCT/NFL looked slightly thinned OD; nml OS
8. Neuro-imaging study ordered MRI of the brain and orbits with
contrast thin sections through orbits.
B. Physical
1.Patient in normal shape, slightly overweight,(-) headaches,normal
gait, nothing significant on Neuro-symptom inventory .
C. lab studies: CBC with differential, prolactin levels elevated
D. Neuro-imaging studies: notable for a large sellar mass that extends into
the suprasellar region compressing the intracranial portion of the left ON. Likely
represents a large pituitary macroadenoma. See films
III. Differential Dx.
A. primary diagnosis: Bilateral optic atrophy subtle secondary to chiasmal
compression.
B. others within differential: Likely COAG OS>OD
IV. Diagnosis and Discussion: The patient was brought back in a month after the
initial visit for diurnal IOP check and gonio and to review the results of MRI with the
patient. The patient was told she had a large macroadenoma that would likely need
surgery rather soon. What is atypical about this case is despite the enormous
macroadenoma there was no significant bitemporal VF loss. This was because the
tumor growth was in a downward direction into the soft palate and not entirely
upward compressing the mid-chiasm, although there was obviously some ON
compression as the pallor would indicate. It also appears that the patient has a
glaucoma diagnosis overlapping this compressive atrophy.
She presented a month later when she was told the news, and she was started on
Travatan-Z for COAG. She was referred to neuro-surgery for surgical
consultation.The neurosurgeon she was referred to did not feel comfortable with
doing her surgery as the tumor was very large and extending in a downward
(atypical direction). He then referred her to an academic institution and she had to
wait another month until she could be seen. In the meantime our patient came
back for an unscheduled urgent visit with acute onset of the worst headache of her
life, nausea and vomiting profusely and diplopia. On motility she presented with
multiple CN deficits and ophthalmoplegia OS>OD, and ptosis OS. She was Dx. with
a cavernous sinus syndrome, likely due to pituitary apoplexy, and she was rushed
to the ER for emergent imaging studies. These studies confirmed apoplexy and the
patient was air lifted to the regional hospital with neuro-surgical back-up. She was
urgently operated on that day and is in recovery.
V. Treatment: The patient underwent complete removal of the hemorrhagic
pituitary, and will require hormone replacement therapy. She also has constant
diplopia due to persistent ophthalmoplegia which is improving slowly. We suggested
patching one eye for now to eliminate diplopia. She has continued her Travatan -Z
for her glaucoma. It is possible the increased cupping is due at least in part to the
compression of the chiasm, but given the borderline IOP, and compromised ON I
decided to leave her on it for now.
VI. Conclusion/ Take Away Points
A. Pituitary apoplexy is a vision threatening and life threatening condition
that occurs when pituitary adenoma grows suddenly and rapidly outstripping its
blood supply. This causes a sudden and profound intracranial hemorrhage which
leads to the headache and ultimately the cavernous sinus syndrome.
The two key things to point out here are the fact that you can have an enormous
pituitary macroadenoma and not have bi-temporal VF loss. Infact from the
standpoint of the tumor there was essentially only central scotoma OS, which could
also be from old glaucoma.
The other key point is to always investigate pallor of the ON. Glaucoma does not
cause pallor, and if you right optic atrophy in your chart you better have a reason
for it ;either ischemia or compression.
Lastly a sudden onset cavernous sinus syndrome, in a patient with known pituitary
neoplasm, is apoplexy until proven otherwise.
Resources:
Walsh and Hoyt's Clinical Neuro-Ophthalmology 5th Edition
Neuro-Ophthalmology 2nd Edition ,Bert Glaser, MD