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CYCLIC VOMITING SYNDROME: UNDERSTANDING THE SYMPTOMS, CAUSES, AND TREATMENT OPTIONS

CYCLIC VOMITING SYNDROME: UNDERSTANDING THE SYMPTOMS, CAUSES, AND TREATMENT OPTIONS

Medically Reviewed by Dr. Rosmy Barrios

Cyclic Vomiting Syndrome (CVS) is a disorder that affects children and adults.

The hallmark feature of Cyclic Vomiting Syndrome is recurrent severe vomiting episodes, often starting at the same time of day. These episodes can last for hours or even days, occurring between long periods without symptoms. They are frequently debilitating and significantly impact a person's quality of life. There is no known cause, yet the episodes are likely the result of a dysfunction in the nervous system.

CVS is a rare disorder, with an estimated prevalence of 1.9%-2.3% across the global population, diagnosed in an average of 3 children per 100,000 per annum. A diagnosis is more common in children between the ages of 3 to 7 years. However, it can also occur in adults, infants, and older people.[1]

This article will explore the symptoms, treatment, and latest advancements in CVS research.

Symptoms of Cyclic Vomiting Syndrome

The main symptom of CVS is severe vomiting episodes. The vomiting is extreme and can occur with such frequency that it significantly disrupts daily activities.

The symptoms of CVS can vary, yet they typically follow a pattern of four phases[2]:

  1. Prodrome (Warning Phase): The initial phase involves a feeling that an episode is about to begin. People with CVS may experience:
    • Intense sweating
    • Nausea, lasting from minutes to hours
    • Appearing pale
  2. Vomiting Phase: This phase is characteristic of intense nausea and uncontrollable vomiting, often occurring multiple times per hour. Episodes can last from hours to up to ten days. Additional symptoms during this phase include:
    • Abdominal pain
    • Inability to speak or move
    • Diarrhea
    • Slightly elevated temperature
    • Dizziness
    • Headache
    • Sensitivity to light
    • Drowsiness
    • Drooling or excessive saliva
  3. Recovery Phase: The vomiting, retching, and nausea gradually subside, and other symptoms improve. This recovery phase can take anywhere from a few hours to a few days.
  4. Well Phase (Symptom-Free Period): This is the period between episodes where individuals with CVS experience no symptoms and feel entirely well. The duration of this phase is unpredictable and can vary from weeks to months.

How Long Does CVS Last?

Episodes can persist for a few hours or extend to several days. The cycle of these phases is often regular and predictable, with episodes recurring at the same time of day or night and lasting for a similar duration. The time between episodes is unpredictable, with "remission" lasting from weeks to months. Reports indicate that when left untreated, episodes occur 4-12 times per year on average.[3]

While CVS often begins in childhood, many adults continue to experience episodes, even with treatment to lessen their severity and frequency.

Complications

Beyond the immediate discomfort of a CVS episode, the condition can eventually lead to more serious health complications. These include[4]:

  • Dehydration and electrolyte imbalance: Severe and repeated vomiting causes significant loss of fluids and essential electrolytes. The resultant dehydration can induce dizziness, weakness, muscle cramps, and lead to potentially dangerous organ problems.
  • Nutritional deficiencies: Prolonged vomiting and the inability to eat normally during episodes can deprive the body of vital nutrients, further impacting health.
  • Impact on mental health: CVS is a physically and emotionally taxing condition. The unpredictability of episodes and their disruptive nature can contribute to significant anxiety and depression.
  • Esophagitis: Stomach acid repeatedly forced back into the esophagus can cause inflammation and irritation, leading to pain and difficulty swallowing.
  • Mallory-Weiss tear: The force of vomiting can cause tears in the lining of the esophagus[5], leading to bleeding, which may be mild or severe.
  • Child Growth and Development: The frequent vomiting and dehydration seen in CVS can lead to weight loss and malnutrition, which can affect a child's physical and cognitive development.

Children with CVS need to receive proper treatment and management to prevent these complications.

Is CVS Life Threatening?

While CVS can be debilitating and leave a lasting impact on a person's quality of life, it is not typically life-threatening.

However, complications can arise from frequent vomiting, such as dehydration and electrolyte imbalance, and these can provoke a critical condition if left untreated for a substantial period. A person with CVS requires proper treatment and management to prevent these complications.

Cyclic Vomiting Syndrome vs. Other Conditions

CVS is often difficult to distinguish from other conditions. The differential diagnoses for CVS include:

  • Gastroesophageal Reflux Disease (GERD): GERD is the result of a weak valve that allows stomach contents to travel back up into the esophagus. This leads to symptoms like heartburn, chest pain, and acid reflux. Unlike CVS, GERD symptoms are generally less severe, tied to acid irritation, and lack the distinct cyclical pattern of CVS.[6]
  • Food Allergies and Intolerances: These conditions involve adverse reactions to specific foods. Food allergies trigger an immune response, while intolerances cause digestive difficulties. Symptoms usually occur shortly after eating and differ from CVS, which has a more cyclical pattern with episodes of greater intensity.[7]
  • Volvulus (malrotation): Volvulus is a birth defect where the intestines are malformed, causing a dangerous blockage. Symptoms include greenish vomit, severe abdominal pain, and possibly bloody stools. This structural problem requires surgery, which fundamentally differs from the episodic nature of CVS.[8]
  • Ureteropelvic (UPJ) obstruction: This obstruction in the urinary tract induces urinary build-up that impacts the kidney. Symptoms center around flank pain, urinary issues, nausea, and vomiting. UPJ obstruction is diagnosed through imaging and differs from CVS due to its focus on urinary symptoms.[9]
  • Metabolic disorders: This is a broad category of inherited conditions affecting how the body processes nutrients. Symptoms vary widely but can include digestive disturbances, vomiting, abdominal pain, and other issues[10]. Unlike CVS, metabolic disorders typically involve many symptoms beyond vomiting and are diagnosed through specialized blood tests.
  • Cyclic Vomiting Syndrome vs. Cannabinoid Hyperemesis Syndrome: Symptoms of CVS are similar to cannabinoid hyperemesis syndrome (CHS), a condition caused by long-term marijuana use. CHS has three stages symptomatic of severe nausea and vomiting, with episodes occurring multiple times a day. The symptoms resolve entirely once cannabis use discontinues, whereas CVS episodes are unrelated to cannabis[11], can be sporadic or at a set time, and are more common in children.

Causes of Cyclic Vomiting Syndrome

The exact cause of CVS is unknown. Researchers theorize it is the result of a dysfunction in the nervous system.

Scientists are actively researching several potential mechanisms underpinning CVS, including:

  • Neurotransmitter Imbalances: The condition may result from hyperexcitability in the nervous system, leading to hyperemesis. Defects in the nervous system or brain circuits governing vomiting and nausea may play a prime role.[12]
  • Mast Cell Activation: Mast cells are vital players in the immune system and are responsible for contributing to several cases of CVS[13].
  • Genetic Predisposition: There appears to be a hereditary component to CVS, as many people with the condition have family members with migraines or other related symptoms. Research is ongoing to pinpoint the specific genes that might increase a person's vulnerability.
  • Hormonal Changes: In some women, CVS episodes seem linked to menstrual periods, suggesting a potential role for hormonal fluctuations as a trigger. A hyperactive stress response system (involving the hypothalamic-pituitary-adrenal axis) may also contribute to CVS episodes.
  • Mitochondrial disorders: Research reveals that CVS biomarkers in children overlap with those seen in mitochondrial disorders. The condition may be related to a dysfunction in Kreb's cycle, governing cellular respiration, and resembles the gastrointestinal component of mitochondrial metabolism disorders[14], such as MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes).
  • Gastroparesis: In some cases, CVS can damage nerves controlling stomach function. This condition, called gastroparesis, causes delayed stomach emptying, exacerbating nausea, vomiting, and nutritional concerns.

Risk Factors

The following factors may contribute to acquiring CVS[15]:

  • Family history of CVS, CHS, or migraines
  • Sensitivity to light or sound
  • Gastrointestinal disorders
  • Mitochondrial disorders
  • Female gender

CVS is also related to several other comorbidities, including:

What Are the Triggers of CVS?

Some potential triggers of CVS include:

  • Migraines
  • Physical exhaustion
  • Stress and panic attacks
  • Hot weather
  • Allergenic foods
  • Motion sickness
  • Menstruation
  • Infections
  • Sleep deprivation
  • Fasting

Diagnosis of CVS

Diagnosing CVS is demanding, as there is no clear test for the disorder. A doctor typically conducts a physical exam and reviews the person's medical history and symptoms.

They may also order diagnostic tests to rule out other conditions:

  • Imaging techniques like endoscopy, ultrasound, or computed tomography (CT) are used to identify any obstructions in the gastrointestinal tract or indications of different digestive diseases.
  • Motility tests to monitor the way food passes through the digestive system and possible digestive disorders.
  • Blood and urine tests to assess the patient’s degree of dehydration, anemia, infection, inflammation markers, and liver complications. These can also reveal signs of a mitochondrial disorder[16].

The patient may need to keep a diary that tracks their episodes to aid the diagnostic process.

Treatment for Cyclic Vomiting Syndrome

There is currently no cure for CVS. Available treatment can help manage the symptoms and prevent future episodes.

While the condition is more common in children, treatment for Cyclic Vomiting Syndrome in adults is identical. Patients with CVS need to work closely with their healthcare team to manage their symptoms effectively and prevent future episodes.

Treatment for CVS may include:

Medications

Medications can help manage the symptoms of CVS, such as:

  • Anti-nausea medications
  • Antidepressants
  • Anticonvulsants
  • Medications for controlling excess stomach acids
  • Pain relievers[17]

Prokinetics may help if delayed stomach emptying is a factor. These medications promote gut motility (movement).

Supplements like Coenzyme Q10, L-carnitine, and Riboflavin have shown potential benefits for CVS in limited studies. However, larger-scale research is needed to confirm their effectiveness[18].

Hospitalization

Some patients may require intravenous fluids in a hospital setting to recover from a severe episode.

Alternative and Complementary Therapies

Several alternative and complementary approaches show some promise, with more research being necessary to confirm their potential benefits. These include:

  • Ginger: Studies reveal that ginger retains potent anti-nausea effects[19].
  • Acupressure: This technique might help with nausea and vomiting in some individuals[20].
  • Relaxation Techniques: Stress can be a trigger. Strategies like deep breathing or meditation might be beneficial.

Avoiding Triggers

Recognizing and avoiding triggers can lower the probability or severity of future CVS episodes. Keeping a journal of symptoms and potential triggers can help a person identify what may be causing their episodes.

Management and Prevention of Future Episodes

In addition to avoiding triggers, there are other steps a person can take to manage and prevent future episodes of CVS. These may include[21]:

  • Eating small, frequent meals
  • Staying hydrated
  • Getting enough rest
  • Managing stress
  • Taking medications as prescribed

What Foods Should You Avoid with Cyclic Vomiting Syndrome?

While there is no specific diet for CVS, some foods may trigger episodes in some people. These may include:

  • Foods high in fat or sugar
  • Dairy products
  • Monosodium glutamate (MSG)[22]
  • Processed foods
  • Caffeine
  • Chocolate
  • Alcohol

A person with CVS needs to keep a journal of their symptoms and potential triggers to identify which foods may be causing their episodes.

Latest Advancements in CVS Research

Research on CVS is ongoing, and there have been some recent advancements in understanding and treating the disorder. Some areas of current research include:

  • Identifying genetic factors that may contribute to CVS.
  • Studying the role of the nervous system and mitochondrial dysfunction in CVS.
  • Investigating the use of cannabinoids in treating CVS[23].
  • Developing new medications to prevent and manage CVS episodes, such as monoclonal antibodies and calcitonin gene-related peptide antagonists that can manage migraines and may help treat CVS episodes[24].

Conclusion

Cyclic Vomiting Syndrome is a rare disorder that causes recurrent episodes of severe vomiting, often starting at the same time of day. While there is no cure for CVS, there are treatments available to help manage the symptoms and prevent future episodes. A person with CVS needs to work closely with their healthcare team to identify triggers and develop a management plan to improve their quality of life. With ongoing research and advancements in understanding and treating CVS, there is hope for a better future for those living with this disorder.

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