Cyclic vomiting syndrome
From Wikipedia, the free encyclopedia
Cyclic vomiting syndrome (US English) or cyclical vomiting syndrome (UK English) (CVS) is a condition whose symptoms are recurring attacks of intense nausea, vomiting and sometimes abdominal pain and/or headaches or migraines. Cyclic vomiting usually develops during childhood usually ages 3–7; although it often remits during adolescence, it can persist into adult life.[1][2][3]
Episodes may happen every few days or every few months. For some there is not a pattern in time that can be recognized. Some sufferers have a warning of an attack: they may experience a prodrome, usually intense nausea and pallor sometimes heightened sensitivity to especially light but also smell, sound, pressure, and temperature with sometimes oncoming muscle pain and fatigue is reported by some patients. The majority of sufferers, but not all, can identify "triggers" that may precipitate an attack. The most common are various foods, infections (such as colds), menstruation, extreme physical exertion, lack of sleep, and psychological stresses both positive and negative.
During an attack a sufferer may be light sensitive (photophobic), sound sensitive (phonophobic), and, less frequently, temperature or pressure sensitive.[1] Some sufferers also have a strong urge to bathe in warm or cold water. Some sufferers reports that they experience a restless sensation or stinging pain along the spine, hands, and feet followed by weakness in both legs. Some of these symptoms may be caused by dehydration rather than any underlying cause for the CVS.
There are established criteria to aid diagnosis of CVS; essential criteria are:
The prevalence of the condition is not clear. Two published studies on childhood CVS suggest nearly 2% of school age children may have CVS. However, diagnosis is problematic and as knowledge of CVS has increased in recent years more and more cases are emerging. This suggests a tendency to underdiagnosis, and thus the true figure may be higher.
The most common therapeutic strategies for those already in an attack are maintenance of salt balance by appropriate intravenous fluids and, in some cases, sedation. Having vomited for a long period prior to attending a hospital, patients are typically severely dehydrated. Abortive therapy has limited success, but for a number of patients potent anti-emetic drugs such as ondansetron (Zofran) or granisetron (Kytril), dronabinol (Marinol), and more recently dextromethorphan may be helpful in either preventing an attack, aborting an attack or reducing the severity of an attack. In some instances, sedatives or painkillers (particularly morphine based) can be helpful.[citation needed] Lifestyle changes may also be recommended, such as extended rest and/or reduction of stress.[citation needed] Because the symptoms of CVS are similar (or perhaps identical) to those of the disease well-identified as "abdominal migraine," treatment of CVS with a regimen of anti-migraine drugs, such as Topirimate and amitryptiline, is showing promise in preventing recurrent attacks.
Most children who have this disorder miss on average 24 school days a year.[9] The frequency of episodes is higher, for some people, during times of excitement.[9] Charitable organizations to support sufferers and their families and to promote knowledge of CVS exist in several countries.
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[edit] Patient characteristics
The average age at onset is 3–7 years, but CVS has been seen in infants who are as young as 6 days and in adults who are as old as 73 years.[4] Typical delay in diagnosis from onset of symptoms is 2.7 – 3 years.[4] Females show a slight predominance over males; the female-to-male ratio is 57:43.[5] CVS occurs in all races but seems to disproportionately affect caucasians.[edit] Genetics
Many affected individuals have a family history of related conditions, such as migraines, in their mothers and maternal relatives suggesting mitochondrial inheritance. Single base-pair and DNA rearrangements in the mitochondrial DNA have been associated with these traits. [6] Charles Darwin's adult illnesses have been suggested to be due to this syndrome.[7][edit] Background
Sufferers may vomit or retch six to twelve times an hour, and an episode may last from a few hours to well over three weeks and in some cases into months, with a median episode duration of 41 hours.[8] Acid, bile, and (if the vomiting is severe) blood may be vomited. Some sufferers will ingest water to reduce the irritation of bile and acid on the esophagus during emeses. Between episodes the sufferer is usually otherwise normal and healthy but can be in a weak state of fatigue or have muscle pain. In approximately half of sufferers the attacks, or episodes, occur in a time related manner. Each attack is stereotypical: that is, in any given individual the timing, frequency and severity of attacks is similar.Episodes may happen every few days or every few months. For some there is not a pattern in time that can be recognized. Some sufferers have a warning of an attack: they may experience a prodrome, usually intense nausea and pallor sometimes heightened sensitivity to especially light but also smell, sound, pressure, and temperature with sometimes oncoming muscle pain and fatigue is reported by some patients. The majority of sufferers, but not all, can identify "triggers" that may precipitate an attack. The most common are various foods, infections (such as colds), menstruation, extreme physical exertion, lack of sleep, and psychological stresses both positive and negative.
During an attack a sufferer may be light sensitive (photophobic), sound sensitive (phonophobic), and, less frequently, temperature or pressure sensitive.[1] Some sufferers also have a strong urge to bathe in warm or cold water. Some sufferers reports that they experience a restless sensation or stinging pain along the spine, hands, and feet followed by weakness in both legs. Some of these symptoms may be caused by dehydration rather than any underlying cause for the CVS.
[edit] Diagnostic criteria and investigations
The cause of CVS has not been determined; there are no diagnostic tests for CVS. Several other medical conditions can mimic the same symptoms, and it is important to rule these out. If all other possible causes have been excluded a diagnosis of CVS may be appropriate.There are established criteria to aid diagnosis of CVS; essential criteria are:
- A history of three or more periods of intense, acute nausea, and unremitting vomiting and sometimes pain lasting hours to days and even into months are reported.
- Intervening symptom-free intervals, lasting weeks to months
- Exclusion of metabolic, gastrointestinal or central nervous system structural or biochemical disease e.g. individuals with specific physical causes (e.g. intestinal malrotation)
The prevalence of the condition is not clear. Two published studies on childhood CVS suggest nearly 2% of school age children may have CVS. However, diagnosis is problematic and as knowledge of CVS has increased in recent years more and more cases are emerging. This suggests a tendency to underdiagnosis, and thus the true figure may be higher.
[edit] Treatment
There is no known cure for CVS, but there are medications that can be used to treat, intervene in, and prevent attacks. There is a growing body of publications on either individual cases or experiences of cohorts of CVS patients. Treatment is usually on an individual basis, based on trial and error.The most common therapeutic strategies for those already in an attack are maintenance of salt balance by appropriate intravenous fluids and, in some cases, sedation. Having vomited for a long period prior to attending a hospital, patients are typically severely dehydrated. Abortive therapy has limited success, but for a number of patients potent anti-emetic drugs such as ondansetron (Zofran) or granisetron (Kytril), dronabinol (Marinol), and more recently dextromethorphan may be helpful in either preventing an attack, aborting an attack or reducing the severity of an attack. In some instances, sedatives or painkillers (particularly morphine based) can be helpful.[citation needed] Lifestyle changes may also be recommended, such as extended rest and/or reduction of stress.[citation needed] Because the symptoms of CVS are similar (or perhaps identical) to those of the disease well-identified as "abdominal migraine,"
[edit] Course and outcome
Fitzpatrick et al. (2007) identified 41 children with cyclic vomiting. The mean age of the sample was 6 years at the onset of the syndrome, 8 years at first diagnosis, and 13 years at follow-up. As many as 39% of the children had resolution of symptoms immediately or within weeks of the diagnosis. Vomiting had resolved at the time of follow-up in 61% of the sample. Many children, including those in the remitted group, continued to have somatic symptoms such as headaches (in 42%) and abdominal pain (in 37%).[citation needed]Most children who have this disorder miss on average 24 school days a year.[9] The frequency of episodes is higher, for some people, during times of excitement.[9] Charitable organizations to support sufferers and their families and to promote knowledge of CVS exist in several countries.
