Understanding Malignant Hyperthermia: Causes, Symptoms, and Management

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Malignant hyperthermia (MH) is a rare, inherited muscle disorder aggravated by certain types of anesthesia that may cause a fast-acting, life threatening crisis. Individuals who are susceptible to MH have a mutation that results in the presence of abnormal proteins in the muscle cells of their body.

whenever vulnerable patients are exposed to certain anesthetic agents, or in extreme cases when they are exposed to high environmental heat or strenuous exercise, this causes an abnormal release of calcium from the sacroplasmic reticulum (an area that stores calcium in the muscle cells). This results in a sustained muscle contraction and an abnormal increase in metabolism and heat production. General signs of an MH crisis include increased heart rate, greatly increased body metabolism, muscle rigidity, and fever that can exceed 43.3 degrees celsius or 110 degrees farenheight, with muscle breakdown, derangements of body chemicals, and elevated acid content in the blood.

MH is generally not associated with other chronic medical problems, such as hypertension, diabetes, or similar diseases. MH have occurred in patients with underlying muscle diseases. There is a definite association with central core disease (CCD), an inherited neuromuscular disorder. Both CDD and MH are caused by a genetic mutation in the skeletal muscle ryanodine receptor type 1.

Individuals with CDD have persistent mild muscle weakness that does not worsen with time. This weakness affects the muscles near the center of the body (proximal muscles, generally muscles in the upper legs and hips. Muscle weakness causes affected infants to appear flacid and can delay the development of motor skills such as sitting,

standing, and walking. In severe cases, affected infants experience profoundly weak muscle tone (hupotonia) and serious or life-threatening breathing problems. CDD is also associated with skeletal abnormalities such as abnormal curvature of the spine (scoliosis), hip dislocation, and joint deformities called contractures that restrict the movement of certain joints

Management of Malignant hyperthermia

Clinical signs and symptoms of MH appear according to the swiftness of onset. The onset may be fast, occuring immediately after anesthesia induction, or it may occur after several hours of general anesthesia or may occur in the post operative recovery period.

Spasm of the jaw muscles with rigidity of the masseter muscles, or severe fasciculation after administration may suggest the possibility of MH occurence to the anesthesia provider.

Malignant hypertension may present with a variety of signs and symptoms during an acute phase or in a more subtle way, developing over a course of several hours.

If there is a likelihood of MH, the patient should be admitted to the hospital and be under close observation for at least 24 hrs after marked jaw rigidity.

If malignant hyperthermia is suspected, notify the surgeon to discontinue the procedure ASAP

Discontinue volatile agents and succinylcholine. If surgery must be performed, maintain general anesthesia with intra venous non-triggering anesthetics, for e.g., intravenous sedatives, narcotics, amnestic, and non-depolarizing neuromuscular blockers as needed. Hyperventilate the patient with 100% oxygen at flor of 10L/min. Administer dantrolene sodium 2.5mg/kg IV, given rapidly through a large-bore needle. Administer repeatedly until the crisis is resolved. Additionally, pack ice on the patient's head, axillae, groin, and underneath the patient. Infuse cold saline IV and initiate esophageal or rectal lavage.

Baker's cyst

This is a lump or bulge in the back of your knee. It is also called a popliteal synovial cyst. It occurs when extra joint fluid flows into a small sac behind the knee. This extra fluid occurs because of osteoarthritis or a torn cartilage, such as a meniscus tear. This irritates the knee joint, causing inflammation. The longer the inflammation process, the larger the cyst will become. This is because, fluid from the inflamed area pools into the knee joint, which subsequently increases the size of the cyst. Swelling in the posterior knee maybe an early sign. Pain that increases with flexion and extension is also reported by some patients.

Emergency Room

Diagnosis is based on adequate history taking, with physical examination. Several patients present with pain and swelling to the affected knee. Plain radiographs is a simple way to start. However, if thrombosis is suspected, then a doppler ultrasound is needed. An ultrasound has a number of advantages, one of the major ones, is its ability to confirm the diagnosis of Baker's cyst. Magnetic resonance imaging (MRI) is superior for diagnosis of Baker's cyst. This is because it can differentiate the cyst from other problems. It can rule out other conditions such as ligament tears and osteoarthritis. Getting an MRI from an emergency room visit may not be feasible, based on the available resources, and severity of condition. Patients often times are given referral for follow up with a specialist who can arrange an MRI form an out patient setting.

Treatment

The initial treatment of a Baker's cyst is pain management. Medications prescribed are usually NSAIDS such as Ibuprofen, in combination with oral steroids. Home care includes resting the affected limb, and applying ice. Ice should be applied no longer than 20 minutes. Never place ice directly on the skin. It is better to wrap into a towel before applying. Apply the ice every 3-4 hours, for a period of 24-48 hours. For added comfort, a knee brace may provide some pain relief as well. Make sure you talk with your health care provider before taking any over the counter medications. Always see an healthcare provider for professional diagnosis and treatment. Make sure to follow any guidelines and recommendations given.

Kidney Stones (Nephrolithiasis)

Kidney stones are hard and irregular in shape, and looks like actual stones. They may be comprised of calcium and oxalate, and more often uric acid that form in the kidneys. The stones may form in different areas of the kidneys. Namely, the renal pevlis, ureters or bladder. Depending on the area of stone formation, patients may complain of mild to severe pain. The presentation of any patients to the ER with suspected kidney stones is classic. They are usually in severe pain, wailing and unable to lie still. Nausea and vomiting usually accompanies the pain. Pain is usually felt in the lower back (flank) right or left. There may be visible hematuria (blood in the urine). Or seen on urinalysis. When the stone travels towards the bladder VIA the ureters, they may get stuck in the ureters. This condition is called obstructive urolithiasis. When this happens, urine backs up into the kidneys resulting in hydronephrosis, and swelling of the kidneys. There is a high risk of infection without antibiotics. There will aslo be damage to the kidney itself. When there is an obstructive stone, antibiotics should be promptly administered to prevent infection. A urologist will also need to relieve the obstruction to prevent long term kidney damage. This is usually done either by Lithotripsy (ultrasound waves are used to break up the stone), Ureteroscopy- this procedure involves a special device that is pushed through the urethra and bladder, and into the ureter to pull out the stone. Surgery may be needed as a last resort to remove the stone

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Treatment Of Kidney Stones

Treatment for kidney stones is based on the location, size and whether or not it is obstructing. In severe cases, over night hospital stay with urology consult is recommended. In the Emergency Room (ER), a non steroidal anti inflammatory (NSAID) such as toradol may be given intravenously (IV) with zofran for nausea. Sometimes a narcotic mediation such as morphine may be added if the pain is not resolved with NSAID alone. Also flomax 0.4 mg PO is given to aid the passage of the stone. Sodium chloride solution is also given to maintain hydration. If the stone is non obstructing, and small enough to pass on its own. The patient may be discharged from the ER to pass it at home. It is advised to strain each stream of urine, using a simple strainer to detect when it has passed. If the stones are too large, then a procedure called lithotripsy is done by an urologist.

Making The Diagnosis

Obtaining a history of the onset and nature of the pain is the first step. Pain is usually described as sudden, sharp, and severe, with tenderness in the flank area, that worsens with palpation. Microscopic urinalysis that reveals blood, and a plain (non-contrast) CT scan of the abdomen and pelvis. CT scans usually reveals location and size of the kidney stones.

Discharge Instructions

If you were seen in an ER, it is recommended to adhere to the discharge instructions, and follow up with an urologist for continued management. It is always advised to seek medical attention and return to the ER if symptoms worsens. In the meantime, drink plenty of fluids, This means at least 12, 8 ounce glasses of fluid- mostly water each day. Reduce salt intake. Each time you pass urinr, do it in a jar. Pour the urine from the jar through the strainer and into the toilet. Continue doing this until 24 hrs after your pain stops. By then, if there was a kidney stone, it should pass from your bladder. Some stones dissolve into sand-like particles and pass right through the strainer. In that case, you will not ever see a physical stone.

Save any stone that you find in the strainer and bring it to your healthcare provider to look at. It may be possible to stop certain types of stones from forming. For this reason, it is important to know what kind of stone you have.

Try to stay as active as possible. This will help the stone pass. Do not stay in bed unless your pain keeps you from getting around. You may notice a red, pink, or brown color to your urine. This is normal while passing a kidney stone.

Remember, always follow the advice of your medical provider.