Misdiagnosis of Brain Injury – Its Not Just a Headache – Remembering Natasha Richardson

Think about tort reform in the context of medical negligence – misdiagnosis of traumatic brain injury – on a national basis. It happens everywhere from Honolulu to Hilo to Washington, D.C. Why don’t the tort reform advocates talk about the 15,000 people in the united States whose life threatening head injury will be misdiagnosed by careless physicians this year?

Natasha Richardson died of a headache. She fell while skiing. She seemed OK afterward – there was no panic. All she had was a bad headache. Then in a few short hours she became very ill and then she slipped into a coma. Tragically she died. It was more than a headache. It was more than a bump. In her case I do not know if she was misdiagnosed. But her case draws attention to what every person needs to know when they bump their head.

http://www.youtube.com/watch?v=xWMiBoaJQp8

As doctors and their insurance companies and high paid lobbyists twist the arms of naive legislators in states across the country, begging for tort reform, about 15,000 people in the United States will be misdiagnosed at an emergency room or in a doctors office this year with a brain injury that may kill them. More than 30,000 people in the United States suffer this injury every year: Jonathan A. Edlow & Louis R. Caplan, Avoiding Pitfalls in the Diagnosis of Subarachnoid Hemorrhage, 342 New Eng. J. Med. 29, 29 (2000).

The medical diagnosis is:

• Ruptured brain aneurysm, or
• Subarachnoid hemorrhage

The plain language version is internal bleed. A ruptured brain aneurysm usually results in blood collecting at the base of the brain. A subarachnoid hemorrhage often is seen with blood collecting on the surface of brain at the site of the blow that caused the injury or at the opposite side of the brain.

Blood flows into the fixed space inside the skull and there is nowhere for it to go. Something has to give. The brain is crushed. It can take an hour or it can take a year. It depends on how fast the bleed is going. There are treatments – both surgical and new drugs – but time is crucial. Not a minute can be wasted and delay can be fatal as it was in the death of Natasha Richardson this past winter.

Now for the most frightening fact for the 30,000 people who will go to a doctor – usually an emergency room doctor – in 2009, with this condition: the common doctor negligence of failure to diagnose the condition is estimated at 53%:

Unfortunately, many will be discharged without receiving the correct diagnosis or the emergency medical treatment they desperately need.

Failure to diagnose is the most common negligence in the treatment of patients with aneurysmal subarachnoid hemorrhage,² and this failure may be as high as 53 percent in patients visiting the emergency room for the first time.³ Physicians fail to obtain an adequate history, perform a thorough neurological exam, or understand the significance of the history and physical exam findings. Jose I. Suarez et al., Aneurysmal Subarachnoid Hemorrhage, 354 New Eng. J. Med. 387, 388 (2006).

Less often, but still with troubling regularity, negligence involves failure to obtain or accurately interpret diagnostic tests.

Delay in diagnosis and treatment has dire consequences. In some cases, even minimal delay can mean the difference between life and death.”

Many of these patients will present with a severe headache, some will recall being unconscious and others with think they were only dazed (doctors call that a lucid interval) and every one of them needs a careful examination.

How does a doctor detect an internal bleed? CT Scan is the standard of care for seeing an internal bleed. Remember that: CT scan. The exact procedure will be described later in this article:

Q: What if internal bleeding of the brain occurs due to injury?

One of two things can happen; the blood can clot and block circulation to that part of the brain, or the blood can continue to accumulate an create a hemorrhagic area and pressure within the brain on a vessel or surrounding brain structure. In either case, it can lead to an "evolving" stroke with temporary or permanent disability, depending on the area involved and the time until the blockage or pressure is remedied. Also, a generalized response of fluid accumulation around the brain (cerebral edema) can occur to increase pressure around the brain as a whole.

A CT scan or MRI scan can determine the type, area and extent of bleeding to choose the appropriate treatment, which may include "clot-busting enzymatic" drugs, high-dose steroids (hydrocortisone or methylprednisolone) or diuretics to draw fluid away from the brain.

Time is critical, since the brain uses the greatest amount of oxygen in the body (about 20% for a 3-lb organ, far more than the heart), consequently it is exquisitely sensitive to anoxia.

An X-ray will not show an internal bleed. And remember, if you are sent home with instruction to call or come back if it gets worse, if the bleed gets to the point of crushing the brain it will not wait for you to drive back to the emergency room and even if you make it before you die, there is little that the doctors can do at that point – it is too late.

Here is an explanation of what happens inside the skull:

Aneurysms are ballooned areas on arteries. They are often referred to as berry, saccular, or congenital (existing at birth) aneurysms. The most likely cause is a weak area of the artery wall due to a congenital defect combined with degenerative changes that further weaken the tissue.

Aneurysms are typically located on large arteries at the base of the brain and usually occur at branching points. If they stretch and ultimately rupture, arterial pressure forces blood to leak from the aneurysm into the subarachnoid space—the area between the arachnoid membrane and the pia mater, a membrane adherent to the brain. The blood then quickly spreads into the cerebrospinal fluid surrounding the brain and spinal cord.

The problem is greater for women than for men, greater for blacks than for whites and is more common in pregnant women particularly in the third trimester accounting for from 6% to 25% of maternal deaths.Jonathan A. Edlow, Diagnosis of Subarachnoid Hemorrhage in the Emergency Department, 21 Emerg. Med. Clinics of N. Am. 73, 75 (2003),

The sequence is subtle. Many people will have only a small leak at first. At tiny bleed that grows and as the pressure builds causes other leaks and then a cascade due to mounting pressure that all of a sudden becomes critical. That first leak is called a sentinel leak by doctors:

Up to 70 percent of patients will experience a sentinel (warning) leak before suffering a larger hemorrhage. Often these patients have a headache but no other signs or symptoms. Half of the patients with sentinel leaks seek immediate attention, but up to 60 percent of them are misdiagnosed and later suffer a larger hemorrhage. If the sentinel leak is diagnosed and treated promptly, a more serious hemorrhage and the associated significant complications can be prevented.”

Rosen’s Emergency Medicine: Concepts in Clinical Practice (John A. Marx et al. eds., 6th ed. Mosby, Inc. 2006), (subscription required)

At the emergency room the doctor should look for the following signs and symptoms:

• severe headache with sudden onset,
• nausea, vomiting,
• sensitivity to light,
• neck pain.

The examination by the doctor must include looking for neck stiffness, an altered state of consciousness (for example, drowsiness, restlessness, or agitation), neurological impairments such as weakness or paralysis, visual problems, abnormal eye movements, or paralysis of eye movements. Rosen’s Emergency Medicine, n. 7.

The doctor should examine the back of the eye, called a funduscopic examination, to see if a retinal or preretinal hemorrhage exists.

Funduscopic examination is a routine part of every doctor’s examination of the eye, not

just the ophthalmologist’s. It consists exclusively of inspection. One looks through the ophthalmoscope, which is simply a light with various optical modifications, including lenses. The ophthalmoscope illuminates the retina through the normal iris defect that is the pupil. Light rays forming the image of the retina re-emerge through the pupil. The viewing aperture (window) of the ophthalmoscope contains a lens that modifies light rays to assist the user. In the procedure, one looks at structures lying in the innermost aspect of the globe, collectively known as the eyegrounds: retina, retinal blood vessels, optic nerve head (disk), and to a limited degree, subjacent choroid. The pupil is frequently dilated pharmacologically to render retinal inspection easier, and for examination of the macula. One paralyzes the pupilloconstrictor muscle of the iris with nonabsorbable, short-acting topical parasympatholytic drugs, resulting in a larger pupillary aperture. In comparison to the ophthalmologist, the internist, neurologist, or pediatrician concentrates particularly on funduscopic manifestations of systemic disease and less on local ocular disease. Synonyms for funduscopic examination include funduscopy, ophthalmoscopy, and direct ophthalmoscopy. Only ophthalmologists perform retinoscopy and indirect ophthalmoscopy, which require other equipment and provide different information.

Figure 117.4 The value of pupillodilation. (A) External examination (more…)

Figure 117.4

The value of pupillodilation. (A) External examination shows miotic pupil at baseline. (B) Scant fundus is revealed through this pupil. (Peripheral clouding is an artifact of photography through undilated pupil; actual funduscopy in this case showed retina only where photograph is vivid, and no image where photograph is cloudy or spotted.) (C) Pharmacologically dilated pupil provides much better aperture. (D) Retinal field of view enlarges after mydriatic treatment. Funduscopy grows easy. Almost all retinal photographs are taken through dilated pupils. The student expects a comparable view in undilated patients. The rude reality makes him wonder, unnecessarily, if his technique is defective.

The term temporal is used in describing ophthalmoscopic landmarks and findings, rather than "lateral"; and nasal replaces "medial." The optic nerve head or disk is seen when one looks through the pupil from an angle about 15 degrees temporal to the optical axis (the patient’s line of sight, "straight ahead"). The disk is a yellow-pink color that stands out from the redder, browner, or more orange retina proper.

The disk is sharply demarcated temporally and to a lesser degree nasally from the background retina, which is all the retina that is not disk, vessels, or macula. Frequently, a narrow crescent of stippled pigment adjoins the sides of the disk, especially the temporal side (house staff have called the author to see "lesions" that turned out to be this normal feature). The disk is slightly taller than wide. The central part of the disk is paler, and is called the optic cup or physiologic excavation; normally this occupies less than one-third the diameter of the disk. In glaucoma and in high myopia the cup is enlarged. The transverse diameter of the disk is a standard yardstick in fundal description, so that, for example, a lesion may be characterized as "one-half disk diameter out at two o"clock, and extending two disk diameters superiorly therefrom." Although some examiners realize that the disk is 1.5 mm wide, nobody describes a lesion as 3 mm across. Near mid-disk, the central retinal artery and vein emerge from the optic nerve, with which they have run forward into the orbit. Each soon bifurcates into superior and inferior branches, which run "flat," that is, parallel with the retinal surface. Beyond one disk diameter out, they are called arterioles and venules. With all retinal vessels, the artery/arteriole appears slightly smaller, and distinctly lighter, more orange-red and less purple than the vein/venule. The color difference reflects the contained blood column that is visualized: the vascular walls are transparent, and deoxygenated venous blood is darker than arterial blood. Before it crosses the disk edge, each large vessel divides into a nasal and a temporal branch. Thus the principal arteries, veins, and quadrants of any retina are the superior temporal, inferior temporal, inferior nasal, and superior nasal. The avascular, dusky area two disk diameters due temporal to the disk is the macula. This is the area of greatest visual acuity. Apart from this zone, the background retinal color will parallel the patient’s skin and hair pigmentation, from pale in light-skinned blondes to an umber shade in the darkest black people.

Normally, the largest veins pulsate slightly and the arteries do not, the reverse of the situation elsewhere in the body. No hemorrhage is seen in normal fundi. Any yellow, yellow-white, gray, or black interruptions of the background retinal color pattern suggest pathologic exudate, edema, or scar. No wrinkling of the retina should be seen. Chalky whiteness or erythema of the disk is abnormal, as are indistinct disk margins. Any sharp change in elevation that renders one area out of focus with the ophthalmoscope, while the remainder of the retina remains in focus, is abnormal. Tortuous blood vessels usually bespeak pathology.

About 50 percent of patients with aneurysmal hemorrhage will have no alteration in consciousness, and most will lack focal neurological signs.

After a person suffers a hemorrhage, his or her electrocardiogram may suggest acute myocardial infarction. This may complicate the patient’s evaluation and delay the correct diagnosis while an acute heart attack is being ruled out.

The physician should know that the patient may not have any of the physical or neurological findings discussed above:

About 12 percent of patients who arrive at the emergency room with severe acute-onset headaches and normal neurological exams have suffered a hemorrhage.¹⁷ Therefore, patients with sudden severe headaches should be thoroughly evaluated for subarachnoid hemorrhage even if they have no other signs or symptoms or the head­ache abates spontaneously or with pain medication.

Once the differential diagnosis includes subarachnoid hemorrhage, diagnostic testing should immediately follow. Computerized tomography (CT) of the head, without contrast material, is the diagnostic test of choice. It is about 95 percent sensitive for detecting acute subarachnoid hemorrhage if performed within hours of aneurysm rupture. CT is noninvasive, relatively inexpensive, and it can be completed in minutes. CT sensitivity decreases to about 50 percent one week after rupture. Rosen’s Emergency Medicine, supra n. 7.

When the patient is suspected of having suffered a hemorrhage but the CT scan is normal or inconclusive, a lumbar puncture should be performed to look for blood in the cerebrospinal fluid. This is an inexpensive and low-risk procedure that can be completed and evaluated in minutes.

Once a diagnosis of subarachnoid hemorrhage is made, complete diagnostic cerebral angiography should be performed as soon as possible to localize the source of bleeding. Angiography is the gold standard for identifying cerebral aneurysms. Surgical clipping (in which the neurosurgeon opens the skull and places a clip across the neck of the aneurysm to prevent rupture) or less-invasive endovascular treatment (in which coils are placed in the aneurysm via a catheter) should be performed as soon as possible.

Patients with a hemorrhage should be stabilized immediately with particular concern for airway maintenance, blood pressure treatment, correction of any coagulopathy (abnormal blood clotting), and pain and agitation control. They must be admitted to a critical care unit for monitoring and management.

Treatment involves preventing and managing secondary complications—rebleeding, vasospasm (constriction of large-capacity intracranial arteries), hydrocephalus (enlargement of the ventricles in the brain), hyponatremia (abnormally low sodium levels in the blood), and seizures.²¹ Testing of serum electrolyte levels, a complete blood cell count including platelets, and clotting time studies should be done to determine whether the patient has other conditions such as an infection or clotting abnormalities.

Frequent neurologic exams should be performed to evaluate the patient for changes in neurological status or evidence of rebleeding. For untreated ruptured aneurysms, there is a 3 percent or 4 percent risk of rebleeding in the first 24 hours, a 1 percent to 2 percent risk per day in the first month, and a long-term risk of 3 percent per year after 3 months. Jonathan A. Edlow & Louis R. Caplan, Avoiding Pitfalls in the Diagnosis of Subarachnoid Hemorrhage, 342 New Eng. J. Med. 29, 29 (2000).

Rebleeding may be due to sudden changes in blood pressure rather than absolute blood pressure. Bed rest, analgesics for headaches, and antihypertensive medications are generally recommended. Because blood in the subarachnoid space can cause seizures, patients should be monitored for seizure activity and given antiepileptic prophylaxis. To prevent vasospasm, the calcium channel blocker nimodipine should be used.

Vasospasm typically occurs between 5 and 14 days after the rupture and gradually resolves over 2 to 4 weeks. If the aneurysm has been treated, ischemia associated with vasospasm can be reduced or prevented using “triple H therapy” or angioplasty to dilate narrowed vessels. However, despite optimal therapy, 15 percent to 20 percent of patients with vasospasm die. Textbook of Clinical Neurology 1041 (Christopher G. Goetz ed., 3d ed., Elsevier 2007).

Hydrocephalus following a rupture occurs in about 20 percent of cases. The patient may undergo a ventriculostomy procedure to drain cerebrospinal fluid, but this intervention can lead to rebleeding and carries a small risk of infection. In symptomatic patients, chronic hydrocephalus may require a shunt to permanently divert cerebrospinal fluid.

Hyponatremia reduces blood sodium levels in 10 percent to 34 percent of patients, and seizures occur in 25 percent of these patients.

As many as 46 percent of the people who survive a ruptured brain aneurysm have long-term cognitive impairment, and one-third of those who survive require lifelong care. The average case fatality rate is 51 percent. Textbook of Clinical Neurology 1041 (Christopher G. Goetz ed., 3d ed., Elsevier 2007).

A patient’s prognosis largely depends on three factors: the patient’s age, level of consciousness on presentation, and the amount of subarachnoid blood seen on the initial CT scan. Younger patients with a good level of consciousness and minimal blood visible on the CT scan tend to do significantly better—and if diagnosed and treated without delay have dramatically better outcomes than patients who present later in the course of a hemorrhage or have diagnosis and treatment delayed.

In Textbook of Clinical Neurology 1041 (Christopher G. Goetz ed., 3d ed., Elsevier 2007) at page 1043 – 1044, they state that a retrospective study has demonstrated that rebleeding occurs in 48% of misdiagnosed patients:

In a major retrospective study involving patients who were treated at four different tertiary-care hospitals, the rate of rebleeding or other deterioration in patients who were initially misdiagnosed was 48 percent, while the rate in patients correctly diagnosed was under 3 percent. The authors concluded that “in patients in whom correct diagnosis was promptly established, the rates of subsequent deterioration and of untoward outcomes were significantly decreased.”

While the public is being bombarded by doctors asking not to be required to pay for their medical negligence as a part of their tort reform agenda, 15,000 Americans will be misdiagnosed with life threatening head and brain injuries this year. Perhaps the doctors should spend less time picketing the state house and spend more time with their patients. By the way, how much time did your doctor spend with you the last time you went in for treatment?