Tumors

1. Tumors

2. Tumors affecting the visual system are the source of the largest liability claimsand judgments against optometrists. Claims are due to misdiagnosis of intracranial or intraocular tumors. Record award $6.5 million ruling possibly largest in optometry case A Calhoun County jury’s decision Thursday to award $6.5 million to the father of 11-year-old Joshua Medders is the largest malpractice award ever against an Alabama optometrist and possibly the largest in the country, says an optometry

3. Intracranial tumors with ocular manifestations are a rare, but significant, cause of liability claims involving optometrists. The most litigious tumors with ocular manifestations are pituitary adenomas for adults and craniopharyngiomas for children. Pituitary adenoma in coronal section MRI Cranial pharyngioma in sagittal section MRI

4. In the presence of symptoms, optometrists may be held liable for the detection of brain tumors, even rare tumors occurring in children: • decreased visual acuity • hemianopic visual field defect • acute strabismus (particularly incomitant strabismus occurring in children) • papilledema • history of worsening headache that is of unexplained origin • history of worsening neurological symptoms

5. The most common brain tumor affecting vision in adults is pituitary adenoma; the patient may be symptomatic or asymptomatic.

6. The pituitary gland sits in the sella turcica immediately behind the sphenoid sinus and below the optic chiasm. Pituitary adenomas are usually slow-growing and invariably benign, and impingement on the chiasm results in visual symptoms.The prevalence of thesetumors is about 20 cases annually per 100,000 population. Pituitarytumors account for 10%-15% of all intracranial neoplasms. The highest incidence occurs between the 3rd to6th decades. Women of child-bearing years are at greatest risk.

7. Symptoms are dependent on whether the tumor is "secreting" or "non-secreting"; these latter, non-functional tumors are more likely to be detected by optometrists because of the compressive effect they exert on the optic chiasm, causing visual field and visual acuity loss and headache. They constitute about 25% of all pituitary adenomas.

8. The most common field defects produced by pituitary adenomas are bitemporal hemianopias, central scotomas, and homonymous hemianopias.

9. Headaches can be a significant symptom. Clinicians should be suspicious whenever a patient complains of an acute onset recurring headache, pain unlike previous headaches, a headache poorly responsive to analgesics, and headache sufficiently painful to awaken the patient at night while asleep.In suspiciouscases,MRI with gadolinium (toenhance thetumor) should be ordered.

10. Craniopharyngiomas are the most litigious brain tumors affecting the visual system in children. The signs and symptoms of disease are similar to those described for pituitary adenomas.

11. Craniopharyngiomas are slow growing, benign tumors arising from embryonic cells that extend from the pharynx to the floor of the sella tursica, as well as above and within it.They constitute 3% of adult and 10% of childhood brain tumors, and about 50% of adult and 15% of childhood suprasellar tumors. About half of cases occur before the age of 15, but rarely before 5 years of age. They are also most likely between the 5th to 7th decades of life.

12. Example case: pediatric brain tumor • Six-year-old boy having trouble with headaches has +1.50 error OU • A fundus evaluation is performed at the exam, but not a visual field test • Doctor prescribes glasses, but child’s acuities get worse at 2 subsequent exams over about a 6 month period • One year after first exam, acuities are 20/100 and 20/200 • Craniopharyngioma is diagnosed

13. CLAP Traps • Attempt a visual field, even with a young patient • Make sure the refractive error will cause amblyopia before calling it that

14. Example case: adult brain tumor • Long-term contact lens wearer complains of decreased distance acuity (to 20/40 from 20/25) • Patient has mild refractive amblyopia and early cataract • Doctor attempts refit unsuccessfully, refers to a contact lens specialist, who advises use of a different type of lens • Pituitary adenoma is diagnosed by MD about 6 months later

15. CLAP Traps • Always determine the cause of reduced acuity or have a plan to determine the cause—and document it • Seek a second opinion if called for—but there is joint liability in such cases, so make sure the consultant is knowledgeable

16. If a patient requires referral for MRI, the appointment should be timely scheduled and appropriately documented. The use of gadolinium, which is absorbed by the cancerous cells, better reveals the tumor.

17. "Silent" intraocular tumors present the greatest diagnostic challenge to clinicians. In the majority of liability claims, however, the patient is symptomatic. The most common cause of claims in adults is malignant melanoma; for children, it is retinoblastoma. Metastatic tumors to the eye are rarely a cause of claims. Malignant melanoma tumor Retinoblastoma tumor

18. In symptomatic patients, the standard of care requires a dilated fundus examination, but even for asymptomatic, first-presenting patients, a dilated fundus examination should be performed.

19. In the presence of symptoms, optometrists have been held liable for failure to detect intraocular tumors, even tumors as rare as malignant melanoma, which occur in 5 to 7 people per million annually in the US.

20. An optometrist examined a Caucasian woman in her early 60s who complained of decreased vision in one eye. Visual acuity was 20/40 in the affected eye and 20/25 in the other. Although the OD performed ophthalmoscopy and tonometry, he did not perform a dilated fundus examination or a test of the visual field, attributing the decreased acuity to cataracts, which were present in both eyes. About nine months later the patient's continued difficulties with her vision caused her to seek another examination from a different doctor, and the diagnosis of ocular malignant melanoma was made. The tumor, located anterior to the equator, was 12 mm in size. Because of the size of the tumor, the eye had to be enucleated. The patient sued, alleging that a timely diagnosis of the tumor might have spared the eye and would have decreased the risk of metastatic disease.

21. Important indications of disease include reduced visual acuity, visual field loss, and acute strabismus. The cause of such symptoms and signs of disease must always be determined; if a cause cannot be identified at initial examination, subsequent examination (or referral) is necessary.

22. The most likely means of detecting a "silent" intraocular tumor is through a dilated fundus examination performed with a binocular indirect ophthalmoscope; for this reason, all new patients, at initial examination, should receive an ophthalmoscopic assessment of the posterior pole and retinal periphery.

23. Choroidal nevi can—in rare instances—develop into melanomas (21 in 100,000 over a 10 year period). Suspicious lesions are those 4-5 DD in size or larger, with elevation (2 mm), and orange (lipofuscin) pigment.

24. A middle-aged, Caucasian male went to an ophthalmologist for a “routine” examination. During the fundus evaluation the ophthalmologist detected a large nevus in the right eye, but she undertook no specialized testing and merely instructed the patient to return in a year for another exam. About 11 months later the man experienced sudden decreased acuity in the eye and returned to the ophthalmologist, who made an evaluation and immediately arranged for referral to a retinal specialist for treatment of a detachment. The specialist determined that the cause of the detachment was an underlying malignant melanoma, and treatment was instituted to treat the tumor, but it had metastasized and subsequently the man died. A suit for wrongful death was brought against the ophthalmologist and the clinic she worked for, alleging that her failure to diagnose the melanoma was the proximate cause of the man’s death.

25. Metastasis to the eye from a primary tumor diagnosed elsewhere (e.g., lung for men, breast for women) is much more common than choroidal melanoma (50,000 cases annually vs. 1,500). Therefore, a thorough history should always be taken to determine if a patient has been diagnosed and treated for cancer. Patients with such a history should receive a dilated fundus examination.

26. The most common intraocular tumor encountered in children is retinoblastoma; the most frequently affected age group is 4 years and younger; the most likely presenting signs are leukocoria and acute strabismus.

27. In symptomatic patients, the standard of care requires a dilated fundus examination, but by age 3 even an asymptomatic child should be given a dilated fundus examination (AOA ClinicalPractice Guidelines).

28. A 4 ½-year-old girl who had been wearing spectacles since age 2 for accommodative esotropia was examined by an optometrist. He found that acuity was 20/30 in each eye, that both eyes were well aligned with the spectacles, and that there was no pathology by direct ophthalmoscopy through an undilated pupil. He suggested that the child’s past eyecare records be obtained and that the child return for reassessment in 3 months. At this next exam he found acuity to be reduced to 20/40 in one eye, for which he prescribed patching. The child was scheduled to return in 3 months, and at this examination— 20/30 was found in each eye—the optometrist performed cycloplegic retinoscopy and prescribed new spectacles. The optometrist discontinued the patching and asked the mother to bring the child back for reevaluation in 3 months, but because of complications related to pregnancy the mother was not able to return with the child until more than 6 months had elapsed. At this examination, which was approximately 13 months after the initial one, the optometrist found that the child had developed unilateral leukocoria. A subsequent dilated fundus examination performed under anesthesia revealed the cause to be a 15 DD size retinoblastoma, located at the equator of the eye; the tumor was growing anteriorly and had seeded into the vitreous. The child was referred to an eye hospital and given irradiation, which destroyed the tumor but left acuity in the eye at 20/300. A lawsuit was filed, alleging that the optometrist had been negligent in failing to perform a dilated fundus examination with a binocular indirect ophthalmoscope at the initial examination and that this omission had allowed the tumor to grow, adversely affecting the likely outcome of therapy.

29. Retinoblastoma is a congenital retinal tumor, and in 25%–33% of cases it is bilateral due to genetic mutation, with an affected parent having a 50% chance of passing the disease to a child. Incidence is estimated as being 1 in 18,000–30,000 live births. The tumor may grow either under the retina, causing retinal detachment, or over the retina into the vitreous humor. It may then extend through the sclera into the orbit or through the optic nerve intracranially.

30. Infants and children with acute strabismus must be given a thorough eye health assessment, including examination of the retinal periphery through a dilated pupil, to rule out an intraocular tumor. Leukocoria requires a similar examination.

31. If a patient requires referral for further evaluation, the appointment should be timely scheduled and appropriately documented.

32. The mother of an 18-month-old infant noticed that the child's eye occasionally turned out and took her to an optometrist for evaluation of the strabismus. The optometrist found that eye movements were full, the exotropia was intermittent, and the interior of the eye was normal by direct ophthalmoscopy. The optometrist reported these findings to the mother, but because she still seemed concerned he offered to refer the infant to a nearby pediatric ophthalmologist. The mother asked who the physician was and stated that she would make the appointment herself. It was 8 months later before the infant was seen, however, and at that examination she was found to have bilateral retinoblastoma. The optometrist was sued for negligence in failing to perform a dilated fundus examination or refer the infant to a practitioner who would perform a dilated fundus examination. Substantial damages were paid.

33. Binocular vision claims involve failure to treat amblyopia or strabismus during the "critical period" (birth to 6-8 years of age) or failure to detect brain or intraocular tumors that cause binocular vision anomalies.

34. Liability claims involving loss of binocular vision allege that treatment was not timely provided, thereby permanently reducing visual acuity potential or limiting or preventing binocularity. Thus a significant percentage of claims involves young children.

35. A 6-year-old boy was examined by an optometrist and found to have anisometropic amblyopia and constant unilateral esotropia that reduced his acuity in the affected eye to 20/200. The optometrist prescribed spectacles but made no effort to treat the amblyopia. Another optometrist examined the child two years later and obtained similar results, but likewise instituted no amblyopia therapy. A year and a half later (two months short of the child's tenth birthday) an ophthalmologist performed an examination and commented to the child's parents that patching, if instituted four years earlier, could have improved vision to 20/40 in the amblyopic eye. The child subsequently sued both optometrists, alleging that they had been negligent in failing to institute or recommend amblyopia therapy on a timely basis.

36. Most liability claims involving strabismus also involve amblyopia. Intermittent exotropia is common in infants; constant esotropia is more frequently a cause of liability claims. Any constant strabismus in an infant is potentially amblyopiagenic and thus requires assessment and treatment.

37. Refractive amblyopia requires treatment during the "critical period" of child development (to 6 to 8 years of age) or visual acuity will be irrevocably reduced. Anisometropic hyperopia is the usual presenting condition; strabismus may also be present.

38. If a child has significantly reduced monocular acuity due to strabismus or amblyopia or both, protective lenses should be prescribed. Polycarbonate plastic is the only ophthalmic lens material that provides adequate protection against injury. Accommodative esotropia

39. Children may present with signs or symptoms of a binocular vision disorder that are due to a tumor. Keys to making a correct diagnosis: • Make sure the refractive error is amblyopiagenic before deciding that reduced acuity is due to amblyopia • Be suspicious of acute strabismus, especially if it is noncomitant • Don’t hesitate to run a visual field test, even on young children