Medico-Legal Aspects of Neuro-Ophthalmology

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Introduction

The ever-evolving landscape of healthcare is significantly influenced by the growing resource burden of healthcare disputes and medico-legal claims. The most substantial medical malpractice settlement in US history reached a staggering $229,000,000[1]. Latest trends highlight an increase in medical malpractice payouts and the frequency of claims[2]. The mean compensation and the number of claims exceeding $1 million have surged with significant disparities across specialties[3].


Ophthalmologists are subject to these risks for litigation with 5–10% of practitioners facing liability claims annually and average payments may be in the hundreds of thousands of US dollars[4]. Interestingly, 30% of all malpractice claims have the trainee (resident or fellow) described as an additional defendant[5]. High payouts have resulted following litigation for cataract surgical complications ($12.1 million), medical retina ($5.7 million) especially retinopathy of prematurity ($1.18 million), and glaucoma ($1.08 million)[6]. In a retrospective review of online legal databases from Canada, most cases were surgical (46.2%) or involved misdiagnosis (32.7%).[7]


Malpractice in neuro-ophthalmology carries special risks for ophthalmologists because of the risk of missing underlying systemic or neurologic disease, the higher potential morbidity and mortality, and other implications for delayed diagnosis and/or treatment. We review these potential challenges and the broader medico-legal landscape in neuro-ophthalmology.

Impact on Neuro-Ophthalmology

Medical errors in neuro-ophthalmology can lead not just to irreversible and/or bilateral vision loss but also systemic morbidity and even death. The risk of neuro-ophthalmic misdiagnoses among ophthalmologists can be as high as 60% to 70% and the issue highlights the need for appropriate recognition, triage, and referral of urgent and emergent cases for neuro-ophthalmic evaluation[8]. A prospective observational study spanning a year highlighted the concerning access and manpower shortage issues related to neuro-ophthalmology in both the emergency department, outpatient, and inpatient settings[9]. Thus, general ophthalmologists should be aware of both the medical presentations and medico-legal risks of neuro-ophthalmic disorders.

Risk Management Issues in Neuro-Ophthalmology

In one series of 43 malpractice cases from the Westlaw Legal Database involving neuro-ophthalmologic diagnostic errors, the most frequent cited reason for malpractice was failure to diagnose[10]. Further insights from the LexisNexis Academic legal database (spanning January 1, 1989 - December 31, 2018), focusing specifically on malpractice lawsuits involving ophthalmology trainees, reveal the following distribution of etiologies among 28 cases:

  •  Delay in evaluation: 6 (21.4%) cases[11].
  •  Incorrect diagnosis or treatment: 13 (46.4%) cases[11].
  • Improper informed consent: 5 (17.9%) cases[11].
  • Unawareness of trainee involvement: 2 (7.1%) cases[11].
  • Failure to supervise the trainee: 6 (21.4%) cases[11].
  • Error in surgical technique: 16 (57.1%) cases[11].
  • Prolonged operative time: 4 (14.3%) cases[11].
  • Trainee inexperience: 9 (32.1%) cases[11].

Neuro-Ophthalmic Diagnoses Causing Trouble

In analyzing malpractice trials from a comprehensive database, several specific diagnoses in neuro-ophthalmology were identified as particularly prone to litigation:

  • Cerebrovascular Pathology: In one study, 30.2% of malpractice cases occurred from missed or delayed diagnoses of cerebrovascular disease. Stroke was the most frequently misdiagnosed condition[10]. Additional cerebrovascular complications included unrecognized arteriovenous malformations, aneurysms, and venous sinus occlusions, each comprising 7.7% of the cases[10]. A significant challenge faced by practitioners was the misinterpretation of transient monocular blindness (TMB), which was commonly mistaken for a less serious etiology[10].
  • Intracranial Tumor: In this study, 27.9% of the malpractice cases were missed diagnosis of brain tumor with pituitary tumors representing 50% of the cases[10]. Other misdiagnosed tumors included meningiomas, pilocytic astrocytomas, optic nerve tumors, and other non-specific benign and malignant brain tumors[10]. The main omission was failure to order appropriate neuroimaging and/or additional visual testing (e.g., automated perimetry)[10].
  • Giant cell arteritis (GCA): In 25.6% of cases, delayed diagnosis of GCA was the cause[10]. Failure to recognize clinical signs and symptoms associated with GCA (e.g., new headaches, acute visual changes, polymyalgia rheumatica, jaw claudication) was a common allegation[10].
  • Other Diagnostic Oversights in this medico-legal series included idiopathic intracranial hypertension (IIH) and other optic neuropathy, with respective figures standing at 9.3% and 7.0%[10].

Neuro-Ophthalmic Recommendations in High-Risk Cases

It has been our experience (AGL) that neuro-ophthalmic conditions which lead to medical malpractice suits share common features. Although failure to diagnose is a common root cause, the failure to appropriately triage and refer specific neuro-ophthalmic disorders to the hospital or emergency department is a common cause of delay of treatment. Some urgent and emergent conditions for which earlier diagnosis and treatment makes a difference in final outcomes are listed below. We have divided the recommendations into key presenting clinical stems (chief complaint), red flags, and “red herrings” (distractors from real diagnosis) to aid the reader in recognizing these potentially dangerous medical and medico-legal scenarios. Lee’s A’s (“arteritis, apoplexy, aneurysm, abscess, and arterial dissection”) are emphasized but acute, fulminant IIH is also included for completeness.

  1. Arteritis (Giant Cell)
    • Key clinical stem: "Acute headache in elderly with visual complaints."[12]
    • Red Flags: An acute optic neuropathy in older adults is typically due to non-arteritic anterior ischemic optic neuropathy (NAION) but clinicians should be aware of arteritic (GCA) AION (A-AION) in the differential diagnosis in every elderly patient[12].  Severe visual loss, simultaneous bilateral visual loss, transient visual loss (amaurosis fugax) or diplopia (an underrecognized presentation of GCA), and visual symptoms in the setting of PMR should be considered red flags for GCA[12]. Serum erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) with or without platelet count (thrombocytosis) are recommended, but temporal artery ultrasound and/or temporal artery biopsy may be necessary to confirm the diagnosis[12].
    • Red herring: The presence of other vasculopathic risk factors (e.g., hypertension, diabetes mellitus, hyperlipidemia) in older patients may lead to misdiagnosis of a non-arteritic etiology (e.g., NAION) for the complaints and can lead to delayed diagnosis[12]. In addition, some patients with biopsy proven GCA have no systemic symptoms (e.g., headache, jaw claudication) at onset and may even have normal serum acute phase reactants (e.g., ESR, CRP)[12]. In these “occult” GCA cases, a high index of suspicion should be maintained for GCA. In addition, a normal fundus (with a relative afferent pupillary defect) in these cases suggests posterior ION (PION) and PION as opposed to AION is much more likely to be GCA (arteritic PION) than non-arteritic (NAION)[12].
  2. Apoplexy (Pituitary)
    • Key clinical stem: "Acute, painful, bitemporal hemianopsia."[12]
    • Red flag: Pituitary apoplexy can occur in any age and either gender but patients who are pregnant or post-partum have additional susceptibility to apoplexy (Sheehan syndrome).
    • Red herring: Patients with pituitary apoplexy may have severe visual loss that precludes standard automated perimetry with the default stimulus III test object size (e.g., Humphrey visual field) or severe headache or other patient factors may lead to an unreliable or uninterpretable perimetry[12]. In these cases, identification of the bitemporal hemianopsia (or other visual field defects) with confrontation visual field testing is recommended[12]. A normal fundus exam in these cases does not exclude an intracranial process and in the acute setting, optic atrophy may not have had sufficient time to develop in the patient[12]. In addition, the bilateral and often symmetric nature of the visual loss may lead to the absence of an RAPD because the defect is not significantly different (i.e., relative) between the two eyes[12].
  3. Abscess (Mucor)
    • Key clinical stem: "Acute, painful orbital apex syndrome with diabetic ketoacidosis."[12]
    • Red flag: Patients with diabetes mellitus, especially those in diabetic ketoacidosis are prone to infection with fungus[12]. Admission to the hospital for neuroimaging and to rule out the life-threatening fungal infection with mucormycosis is recommended. Computed tomography (CT) scan may show sinus disease and prompt otolaryngologic evaluation and biopsy can confirm the diagnosis (hyphae)[12]. Aggressive anti-fungal therapy can be vision or life saving in these circumstances.
    • Red herring: Unfortunately, magnetic resonance imaging (MRI) may be misleading in fungal sinus disease and fungi may appear hypointense (mimicking air) on T2 MRI[12]. In addition, patients with diabetes mellitus may have visual loss from diabetic retinopathy and diplopia from diabetic ocular motor mononeuropathy[12]. The combination of afferent (optic neuropathy) and efferent (ophthalmoplegia) presentations in diabetes however should prompt attention to and imaging of the orbital apex for fungal disease. In addition, diabetic ophthalmoplegia is an isolated ocular motor mononeuropathy (i.e., cranial nerve III, IV, VI) and the presence of combination or multiple cranial neuropathies should also prompt consideration for Mucor in such cases[12].
  4. Aneurysm (Pupil-involved Third Nerve Palsy)
    • Key clinical stem: "Acute painful ophthalmoplegia and anisocoria (big pupil)."[12]
    • Red flags: The 'rule of the pupil' states that a pupil-involved third nerve palsy is an aneurysm of the posterior communicating artery until proven otherwise[12]. Red herring: Although cranial MRI is generally the imaging study of choice in the outpatient setting for neuro-ophthalmic conditions, in the emergency setting, a non-contrast CT scan followed by CTA might be more appropriate in acute, pupil involved third nerve palsy to look for subarachnoid hemorrhage from a ruptured aneurysm followed by angiography to exclude aneurysm[12].
    • Red herring: Cranial MRI or CT scan alone however without MR or CT angiography (MRA, CTA) might miss an underlying aneurysm[12]. Standard catheter angiography may still be necessary in such cases to identify an underlying aneurysm[12].  In addition, elderly patients with third nerve palsy (or any acute ophthalmoplegia) should be evaluated for underlying GCA (e.g., headache, scalp or temporal tenderness, jaw claudication)[13].
  5. Arterial (Carotid or Vertebral) Dissection
    • Key clinical stem: "Acute painful anisocoria (small pupil: Horner syndrome)."[12]
    • Red flag: Patients with trauma may have a Horner syndrome due to arterial dissection of the internal carotid artery or the vertebral artery[12]. In the acute setting, CT and CTA of the head and neck are reasonable initial imaging studies but follow up MRI and MRA of the head and neck may demonstrate the blood in the dissected false lumen in carotid and vertebral artery dissections (crescent sign)[12]. Prompt admission and neurologic consultation as well as consideration for antiplatelet or other treatment may be necessary to reduce the risk for stroke.
    • Red herring: Although the pain of carotid or vertebral dissection is often in the neck (most arterial dissections are extracranial and cervical in origin), sometimes the Horner syndrome is accompanied by referred eye pain[12]. This pain arises because the vagus nerve carries general visceral afferent (GVA) fibers that supply the carotid artery but the pain mislocalizes (referred) to the general somatic afferent of the trigeminal nerve (eye pain)[12]. Cranial MRI of the head localizing to the trigeminal V1 pain and the oculosympathetic pathway (Horner syndrome) may lead to misdiagnosis. The imaging of the Horner syndrome should include the entire oculosympathetic axis form hypothalamus (first order neuron) to the neck and upper chest (second order neuron to thoracic level 2) and rostrally to the cavernous sinus and orbit[12].
  6. Acute, fulminant idiopathic intracranial hypertension (IIH)
    • Key clinical stem: “Acute and severe IIH.”
    • Red flags: An acute (less than a few weeks) and severe (loss of visual acuity or significant visual field loss) with papilledema from IIH constitutes a special form of IIH termed fulminant IIH. Admission to the hospital, temporizing of the intracranial pressure with a lumbar drain, aggressive medical treatment (e.g., acetazolamide, corticosteroids), and urgent surgical consultation is recommended for fulminant IIH. The surgical options include optic nerve sheath fenestration, cerebrospinal fluid shunting, and cerebrovascular stenting. Cranial CT and CTV (to exclude venous sinus thrombosis) and MRI and MRV with and without contrast are recommended as well as lumbar puncture to confirm the diagnosis of IIH.
    • Red herring: Although most cases of IIH are not fulminant and can be evaluated and managed medically as an outpatient, fulminant IIH typically requires surgical intervention to prevent irreversible visual loss. The differentiation of typical from fulminant IIH depends on the acute onset (weeks) and severe nature of the visual loss.  

Patient-Physician Relationship

The Ophthalmic Mutual Insurance Company (OMIC) has highlighted the underestimated power of patient relationships[14]. Surgeons who spend more time with their patient's pre-surgery were notably less prone to malpractice claims[14]. Often, a patient's decision to litigate is rooted less in clinical errors and more in feelings of being undervalued or misunderstood. Genuine care, effective communication, and empathy, especially after unfavorable outcomes, play a monumental role in litigation prevention[15].

Conclusion

Clinicians should be aware of the urgent/emergent conditions (Lee’s A’s) in neuro-ophthalmology including arteritis, apoplexy, aneurysm, abscess, arterial dissection and acute fulminant IIH. Earlier diagnosis and treatment of these neuro-ophthalmic conditions is mandatory to prevent permanent and irreversible visual loss but also potential systemic morbidity and mortality. Failure to diagnose but more importantly failure to recognize, triage, and refer in a timely manner are the main root causes for malpractice in neuro-ophthalmology for these conditions.

References

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  7. Salimi A, Saed Aldien A, ElHawary H, Ambikkumar A, Kapusta MA. Canadian legal cases in ophthalmology: a closer look at 40 years' worth of data. Can J Ophthalmol. 2024 Apr;59(2):83-88. doi: 10.1016/j.jcjo.2023.02.007. Epub 2023 Mar 14. PMID: 36931321.
  8. Stunkel L, Newman-Toker DE, Newman NJ, Biousse V. Diagnostic error of neuro-ophthalmologic conditions: State of the science. J Neuroophthalmol. 2021;41(1):98-113. doi: 10.1097/WNO.0000000000001031.
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  13. Fang C, Leavitt JA, Hodge DO, Holmes JM, Mohney BG, Chen JJ. Incidence and etiologies of acquired third nerve palsy using a population-based method. JAMA Ophthalmol. 2017;135(1):23-28. doi: 10.1001/jamaophthalmol.2016.4456.
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