OPTOMETRIC EDUCATION CONSULTANTS CLINICAL CASE CHALLENGE
The first patient is a 16 year old male whose vision has been fluctuating for 6 weeks. He also complains about headaches. His primary care physician feels it’s a normal growth spurt and Mom feels it’s migraines as there is a strong family history, but she still wants eyes checked. His vision is 20/20 OD/OS uncorrected and he has bilateral optic disc edema.
The second patient is a 55 year old woman with progressively increasing hyperpigmentation of her lower legs for the past 6 months.
While these two patients are seemingly unrelated, they do have an underlying, unifying cause for their maladies. He had severe acne and was using minocycline 50 mg BID PO and she had meibomian gland disease was using doxycycline 100 mg PO QD.
There are several potential adverse effects from tetracycline and its derivatives. Enhanced photosensitivity is common and dental staining is well known, making this medication one to avoid in children and during pregnancy (Category D drug). Tetracycline can enhance the effects of coumadin and digoxin. Intracranial hypertension resulting in pseudotumor cerebri has been reported, albeit uncommonly. Cutaneous hyperpigmentation is a well-known side effect of tetracyclines, but doxycycline-induced cutaneous hyperpigmentation has only been rarely reported.
The 16 year old male was evaluated (with serology and neuroimaging) for potential causes of bilateral disc edema. When no other etiology was discovered, he was diagnosed with medically induced pseudotumor cerebri. No further evaluation was undertaken for the 55 year old female as the hyperpigmentation was quickly understood to be medically induced. Both had their medications immediately discontinued. His disc edema resolved without complications and her pigmentation diminished over several months.
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Case Challenge 01 - June 2018
Case 1: A 27 year old woman presents urgently complaining of painful vision loss in her right eye. She has no known medical history and this has never occurred before. She has an edematous optic nerve with hemorrhaging, an afferent pupil defect, superior arcuate scotoma, pain when she moves her eye, and 20/70 visual acuity. A clear-cut case of optic neuritis possibly as the first manifestation of multiple sclerosis? Perhaps…perhaps not.
While it is tempting to look at an acute optic neuropathy with pain on eye movement in a young female and immediately surmise optic neuritis and a possible demyelinating disease such as multiple sclerosis, there are inconsistencies which must be considered. Demyelinating optic neuritis presents with a normal optic disc in the majority of cases. Additionally, disc and juxtapapillary hemorrhages are extremely rare in optic neuritis, so other causes must be considered.
Non-arteritis anterior ischemic optic neuropathy (NAAION) can have a similar optic disc appearance. NAAION will typically have inferior visual field defects. More importantly, NAAION is a painless condition. Arteritic anterior ischemic optic neuropathy would not be considered because of the patient’s age.
Infectious optic neuropathy needs to be strongly investigated in a case like this. Neuroretinitis, often associated with cat-scratch disease, will present with a macular star of exudates, but this finding may be missing early in the disease. However, optical coherence tomography (OCT) will show a serous macular detachment early in the disease course.
In this case, contrast-enhanced MRI of the orbits/chiasm and brain should be ordered to rule out demyelination and neural sheath swelling in perineuritis. Macular OCT should be performed to look for a serous detachment suggestive of neuroretinitis. The patient should also be tested for numerous infectious agents including Bartonella, syphilis, Lyme, tuberculosis, herpes, Epstein-Barr, and rickettsioses, to name a few. This can best be done in concert with the patient’s primary care physician or an infectious disease specialist.
MRI findings showed optic nerve enhancement possibly consistent with infectious, autoimmune, or granulomatous disease with no evidence of demyelination. Serological testing subsequently revealed very high titers of Epstein Barr Nuclear Antibody IgG and Epstein Barr Capsid Antibody IgG.
The pathogenesis of infectious optic neuropathies may involve direct involvement of the optic nerve by a pathogen and /or indirect involvement with inflammatory, degenerative, or vascular mechanisms.
Thanks to Dr. Nikki Rook for sharing this case
Case Challenge 02 - June 2018
A 13 year old female was referred for painless reduced vision (20/40) in her left eye with a concurrent abnormal screening visual field, reportedly elevated intraocular pressure (IOP), and an afferent pupillary defect. Her previous exam was 3 weeks earlier and she had been previously referred to an ophthalmologist over a year earlier by another optometrist, but her mother did not know why and did not take her. When presented with painless vision loss in a young patient with these findings, there are numerous diagnostic possibilities.
The key piece of diagnostic information was her IOP: she measured 28 mm Hg OD and 43 mm Hg OS by Goldmann applanation. Her pachymetry was slightly thick at 593µ OD and 595µ OS, but these values will not significantly impact an IOP of 43 mm Hg. There were no biomicroscopic or gonioscopic abnormalities and both angles were open. The reason for the pronounced visual field loss, reduced vision, and left afferent pupillary defect was asymmetric glaucomatous damage. She was diagnosed with juvenile open angle glaucoma (JOAG).
Glaucoma can afflict infants and children in several ways. Conditions such as inflammation or trauma can contribute to elevated intraocular pressure in secondary glaucoma, while congenital abnormalities of the trabecular meshwork development can result in infantile glaucoma. Lesser known, however, is juvenile open angle glaucoma, which afflicts children and young adults similar to primary open angle glaucoma, with no identifiable trabecular meshwork abnormalities or other secondary causes. Intraocular pressure elevation develops between ages 3 and 16 years. Typically the IOP level is quite high; there are no “normotensive” JOAG cases. The disease tends to be more aggressive and progressive than adult POAG.
Treating children and young adults with JOAG presents challenges and medical therapy isn’t the same as with older adults. Pediatric use is considered to be off label, though it is commonly done. Topical beta blockers are a safe and effective class when used in children. Prostaglandin analogs (PGA) are safe and well tolerated, but unfortunately not very effective in the pediatric glaucoma population. Children where PGA efficacy is best demonstrated are older, typically commensurate with puberty. When used in children, topical carbonic anhydrase inhibitors (CAIs) are a safe and effective means by which to lower IOP. Brimonidine, though effective in lowering IOP in children, crosses the blood-brain barrier and can potentially affect the central nervous system (CNS). This medication has demonstrated an unacceptable level of adverse events in children (even inducing coma) and should not be used if possible, and definitely not before the age of 8 years.
In the young girl presented here, prostaglandin analogs predictably had no effect. She eventually ended up using dorzolamide/timolol fixed combination (Cosopt), which lowered her IOP to a range of 15-17 mm in each eye.
Case Challenge 03 - July 2018
A 35 year old man presents wanting another opinion due to “blood on my right eye”. He says that everything happened 3 days ago after violently vomiting, reportedly due to food poisoning from chicken Caesar salad. He still feels a little nauseated and somewhat “not himself”. He saw another practitioner 3 days ago, was told he had a ‘bruise’ on his eye and it should go away in 1-2 weeks. From the outward appearance, he seems to simply have just a subconjunctival hemorrhage…or does he?
Further examination revealed visual acuity of 20/100 OD and 20/70 OS, certainly not explained by a subconjunctival hemorrhage. He didn’t mention anything about his vision because he was more concerned about the blood on his right eye. He then revealed headaches occurring for the past 2 weeks. Fundus examination revealed a bilateral “Blood and Thunder” appearance initially suggestive of central retinal vein occlusion…or is it
Careful inspection showed that the hemorrhages were subretinal, intraretinal, subhyaloid, and vitreous in location rather than the typical intraretinal location in retinal vein occlusion. The bilateral appearance, location of the hemorrhages, and history of headache were more indicative of Terson’s syndrome.
Terson’s syndrome originally was defined by the occurrence of vitreous hemorrhage in association with subarachnoid hemorrhage. Terson’s syndrome now encompasses any intraocular hemorrhage associated with intracranial hemorrhage and elevated intracranial pressures.
Intraocular hemorrhage includes the development of subretinal, retinal, subhyaloidal, or vitreal blood. The classic presentation is in the subhyaloidal space. This fundus appearance in the context of severe head injury should alert clinicians to the possibility of a traumatic intracranial bleed. In the absence of head trauma, headache could be indicative of a pre-existing ruptured intracranial aneurysm.
This patient was sent directly to a hospital emergency room due to the high suspicion of intracranial hemorrhage and elevated intracranial pressure, which was subsequently confirmed with MRI. Ultimately, he was diagnosed with Hairy Cell Leukemia and cryptococcal meningitis
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