What is Tapia Syndrome?
Tapia syndrome was initially identified by a Spanish ear, nose, and throat specialist, Antonio Garcia Tapia, in 1904. He described it as a specific condition causing neurological signs and symptoms that originated outside the brain and spinal cord. Today, it’s recognized as a rare complication that can occur during procedures that require manipulation of the airway. To date, less than 100 cases have been reported.
Tapia syndrome leads to one-sided paralysis of the tongue and vocal cord due to damage to specific nerves – the hypoglossal nerve (which controls tongue movements) and the recurrent laryngeal nerve (which helps voice production and breathing). The symptoms can involve speaking difficulties, voice changes, tongue deviation, and problems with swallowing. Some procedures can increase the risk of developing this condition. However, Tapia syndrome can happen during any surgery that requires general anesthesia and a medical procedure called orotracheal intubation (insertion of a tube through the mouth and into the airway).
What Causes Tapia Syndrome?
Tapia syndrome is a rare condition that involves a combined, one-sided weakness of two nerves in the head – the hypoglossal nerve and the recurrent laryngeal branch of the vagus nerve. These nerves can experience damage along any point in their path – either within the brain (intracranially) or outside the brain (extracranially). Mostly, this damage occurs outside the brain in the neck area where the two nerves are very close to one another.
Typically, Tapia syndrome presents as damage to the peripheral nerves due to direct harm to the hypoglossal and recurrent laryngeal nerves, which are located at the base of the tongue. Most instances of this are iatrogenic, meaning they happen as a result of a medical treatment or procedure. This often occurs during procedures that involve inserting a tube into the patient’s airway (orotracheal intubation) for anaesthesia during surgery. It may also occur in the intensive care setting with prolonged use of a breathing tube and assisted ventilation. The nerve injury might come from stretching or squeezing these nerves.
Neck bending or extension during anaesthesia procedures might make the nerve injury worse. Certain anatomical irregularities, such as a long styloid process or an enlarged hyoid bone (both located in the neck area), may increase the risk of developing this syndrome. Other causes may include any disease in the neck region, including cancers, trauma, or tearing of neck arteries. Interestingly, Tapia syndrome has been reported more often in patients with COVID-19, likely due to the increased use of orotracheal intubation for assisted ventilation in these patients.
The central type of Tapia syndrome typically forms part of a brainstem syndrome affecting certain areas within the brain and is very rare. Causes may include bleeding, restricted blood supply, abscess formation, malignancies, and other space-occupying lesions. Central Tapia syndrome has been attributed to brainstem infarctions (areas of dead tissue due to insufficient blood supply), meningovascular syphilis, and metastatic hemangiosarcoma (a type of cancer).
Risk Factors and Frequency for Tapia Syndrome
Tapia syndrome is often linked with surgeries that require general anesthesia and placing a tube into the patient’s mouth and windpipe. Several factors influence the likelihood of developing the syndrome, including how long the tube is left in, the way the patient’s neck is positioned, and how the patient’s head is moved during surgery. This pattern is backed up by data from shoulder arthroscopy, cervical spine surgery, and critical care units. Tapia syndrome has also been found in patients who have had heart and chest surgery, ear, nose and throat surgery, and jaw and face surgery.
- Tapia syndrome is usually related to surgeries requiring general anesthesia and a tube inserted into the windpipe.
- Various factors affect the risk of this syndrome: duration of tube insertion, neck positioning, and head manipulation.
- Support for this comes from shoulder, spine surgeries, and intensive care units.
- Cases have also been identified after heart, chest, ear, nose and throat, as well as jaw and face surgeries.
Signs and Symptoms of Tapia Syndrome
Tapia syndrome generally diagnosed based on clinical signs and symptoms. It typically appears suddenly in patients who have been on breathing support through a tube in the windpipe. Common symptoms include hoarseness, problems with speech and swallowing, and the tongue veering to one side when sticking out.
Getting a thorough patient history and conducting a detailed neurological check-up is key to ruling out other possibilities and determining whether the issue arises from in the brain or the peripheral nerves. It’s quite rare to have injuries to the base of the brain. More commonly, there may be signs indicative of involvement of the vagus and hypoglossal nerves on one side, along with sensory and motor symptoms affecting the other side of the body.
Damage to nerves inside the skull without involving the connections between the nervous system and the muscles, a condition usually caused by inflammation or tumors of brain lining, might also occur. A single-sided issue with the vagus nerve and the recurrent laryngeal branch of the hypoglossal nerve is quite unusual. Damage outside the brain generally causes single nerve weaknesses. Any additional signs pointing to a specific location should be discussed and the patient referred to the appropriate specialist for more investigations and treatment.
In 2010, Aktas and Boga suggested a grading system for Tapia syndrome that gives a practical guideline for diagnosis, but it hasn’t been approved yet. It classifies the syndrome into three grades depending on the severity of the symptoms and recommends associated treatment strategies.
- Grade I: Mild – Unilateral cord and tongue paralysis, no uvula distortion, minimal slowdown in speaking, no tongue swelling, no swallowing problems. Corticosteroid treatment not recommended.
- Grade II: Moderate – Unilateral cord and tongue paralysis, no uvula distortion, a mild slowdown in speaking, tongue swelling, pharynx dryness, trouble swallowing, cracked speech. 15 days of corticosteroid treatment recommended (first 3 days intravenously, followed by oral route).
- Grace III: Severe – Unilateral cord and tongue paralysis, significant uvula distortion, great difficulty in speaking, tongue swelling, pharynx dryness, trouble swallowing, challenges in feeding and drinking. 1 week of intravenous corticosteroid treatment recommended (1 mg/kg per day).
Testing for Tapia Syndrome
If your doctor suspects you have Tapia syndrome, a condition that affects nerves in your tongue and voice box, they may need to use certain imaging techniques to confirm the diagnosis. Computed tomography (CT) scans and magnetic resonance imaging (MRI) are two commonly used tools that can help do this.
Doctors usually opt for CT scans first, even though they may not be as detailed as MRI. This is because CT scans are easier to access in most medical facilities. In some cases, contacting a neurologist or performing an electromyography, which measures the electrical activity in your muscles, may add extra information but these are usually not necessary for the diagnosis or the treatment plan.
In addition to these imaging tests, your doctor may also use endoscopy to visually check your airway while you swallow. If Tapia syndrome is affecting your nerves, saliva may gather in the piriform sinus, a part of your throat. This test is a safe and reliable way to confirm the diagnosis. If the suspicion of Tapia syndrome is high, using airway endoscopy might even eliminate the need for other tests such as CT scan, MRI, barium swallow, or electromyography.
Treatment Options for Tapia Syndrome
Tapia syndrome is typically managed with supportive care and engaging in active rehabilitation. This recovery process may take several months. It is vital to participate in speech and language therapy early on, along with implementing a well-structured program for swallowing rehabilitation to ensure the best possible recovery. For managing Tapia syndrome, it’s generally agreed that a team approach with various healthcare professionals is most effective.
There is ongoing debate about the use of steroids in treating Tapia syndrome. There’s no concrete evidence as of now, so any decisions about steroid use should be made in consultation with the person’s neurologist. The theory behind using steroids is that they might help to reduce swelling of the nerves, which could then speed up recovery. For those situations where the Tapia syndrome symptoms are mild, steroids are usually not recommended. In more severe cases, steroids may be considered, as they could potentially provide benefits. Although, switching from intravenous to oral administration after the initial few days is usual.
What else can Tapia Syndrome be?
Tapia syndrome is typically linked to procedures like mouth-to-trachea intubation (a breathing tube) and surgery. Therefore, if someone shows symptoms of this condition without having been through any sort of direct physical trauma, it can raise some alarm bells. It’s essential to rule out other health problems, particularly those caused by growths or infections in the body. Some of these include:
- Lung cancer, particularly tumors on the left side, which could press on a nerve in the larynx (voice box).
- Ortner syndrome, which is a weakness or paralysis in a nerve of the larynx, caused by something growing inside the heart or major blood vessels.
- Cancerous lymph nodes
- Tumors in the head and neck
- Formation of abscesses (pockets of pus)
Proper clinical assessments and relevant tests can help eliminate these possibilities and guide the management of the condition.
What to expect with Tapia Syndrome
The time it takes to recover can vary greatly, usually between 3 and 22 months, with most people recovering around 9 to 12 months. It’s important to remember that recovery isn’t always the same for everyone. In fact, about 30% of patients fully recover, while 39% achieve incomplete recovery. Unfortunately, over 26% of patients do not achieve recovery at all.
Possible Complications When Diagnosed with Tapia Syndrome
Tapia syndrome, unfortunately, may not always be fully curable. This can lead to a significant decline in a person’s quality of life. A patient might not entirely recover, which could result in long-term problems with speaking and swallowing. This could affect their ability to eat properly, resulting in low nutrition intake and a high chance of choking on food. In severe cases, Tapia syndrome can also cause both vocal cords to become paralyzed. This could potentially block the airway, becoming an emergency that may require an opening to be made in the windpipe to facilitate breathing.
Common effects and complications include:
- Persistent decline in the quality of life
- Chronic difficulty speaking and swallowing
- Poor nutrition due to inadequate food intake
- Increased choking risk
- Potential complete vocal cord paralysis
- Emergency requiring an opening to be made in the windpipe
Preventing Tapia Syndrome
Patients who are having procedures that might lead to Tapia syndrome, a rare condition that can damage the nerves, should be informed about the possible risks of nerve injury. This is an important step in the process of obtaining patient consent for the procedure. Understanding the disease can help doctors avoid unnecessary tests and give patients a clear idea about what they can expect in terms of recovery and outcomes.