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'''Cyclic vomiting syndrome''' ('''CVS''') is a chronic [[Functional disorder|functional condition]] of unknown [[pathogenesis]]. CVS is characterized as recurring episodes lasting a single day to multiple weeks. Each episode is divided into four phases: inter-episodic, prodrome, vomiting, and recovery. Inter-episodic phase (symptom free phase), is characterized as no discernible symptoms, normal everyday activities can occur, and this phase typically lasts one week to one month. The prodrome phase is known as the pre-emetic phase, characterized by the initial feeling an approaching episode, still able to keep down oral medication. [[Vomiting|Emetic]] or vomiting phase is characterized as intense persistent [[nausea]], and repeated vomiting typically lasting hours to days. Recovery phase is typically the phase where vomiting ceases, nausea diminishes or is absent, and appetite returns. This syndrome is most commonly seen in children usually between ages 3 and 7, however adult diagnosis is quite common.<ref>{{Cite journal|last=Lee|first=Lennard Y. W.|last2=Abbott|first2=Laura|last3=Mahlangu|first3=Bruce|last4=Moodie|first4=Simon J.|last5=Anderson|first5=Simon|date=2012-9|title=The management of cyclic vomiting syndrome: a systematic review|url=https://journals.lww.com/eurojgh/Abstract/2012/09000/The_management_of_cyclic_vomiting_syndrome___a.1.aspx|journal=European Journal of Gastroenterology & Hepatology|language=en-US|volume=24|issue=9|pages=1001|doi=10.1097/MEG.0b013e328355638f|issn=0954-691X}}</ref> This disorder is thought to be closely related to migraines and family history of migraines. <ref>{{Cite journal|last=Fleisher|first=David R.|last2=Gornowicz|first2=Blake|last3=Adams|first3=Kathleen|last4=Burch|first4=Richard|last5=Feldman|first5=Edward J.|date=2005-12-21|title=Cyclic Vomiting Syndrome in 41 adults: the illness, the patients, and problems of management|url=https://doi.org/10.1186/1741-7015-3-20|journal=BMC Medicine|volume=3|issue=1|pages=20|doi=10.1186/1741-7015-3-20|issn=1741-7015|pmc=PMC1326207|pmid=16368014}}</ref><ref>{{Cite journal|last=Abell|first=T. L.|last2=Adams|first2=K. A.|last3=Boles|first3=R. G.|last4=Bousvaros|first4=A.|last5=Chong|first5=S. K. F.|last6=Fleisher|first6=D. R.|last7=Hasler|first7=W. L.|last8=Hyman|first8=P. E.|last9=Issenman|first9=R. M.|last10=Li|first10=B. U. K.|last11=Linder|first11=S. L.|date=2008|title=Cyclic vomiting syndrome in adults|url=https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1365-2982.2008.01113.x|journal=Neurogastroenterology & Motility|language=en|volume=20|issue=4|pages=269–284|doi=10.1111/j.1365-2982.2008.01113.x|issn=1365-2982}}</ref>
'''Cyclic vomiting syndrome''' ('''CVS''') is a chronic [[Functional disorder|functional condition]] of unknown cause characterized by recurring attacks of [[nausea]], [[vomiting]], and sometimes [[abdominal pain]], [[headaches]], or [[migraine]]s. CVS typically develops during [[childhood]], usually between ages 3 and 7; although it often remits during [[adolescence]], it can persist into adult life.<ref name="Lindley 2005">{{cite journal | vauthors = Lindley KJ, Andrews PL | title = Pathogenesis and treatment of cyclical vomiting | journal = Journal of Pediatric Gastroenterology and Nutrition | volume = 41 Suppl 1 | issue = Suppl 1 | pages = S38-40 | date = September 2005 | pmid = 16131963 | doi = 10.1097/01.scs.0000180299.04731.cb }}</ref><ref name=Li2008>{{cite journal | vauthors = Li BU, Lefevre F, Chelimsky GG, Boles RG, Nelson SP, Lewis DW, Linder SL, Issenman RM, Rudolph CD | display-authors = 6 | title = North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome | journal = Journal of Pediatric Gastroenterology and Nutrition | volume = 47 | issue = 3 | pages = 379–93 | date = September 2008 | pmid = 18728540 | doi = 10.1097/MPG.0b013e318173ed39 }}</ref><ref>{{cite journal | vauthors = Abell TL, Adams KA, Boles RG, Bousvaros A, Chong SK, Fleisher DR, Hasler WL, Hyman PE, Issenman RM, Li BU, Linder SL, Mayer EA, McCallum RW, Olden K, Parkman HP, Rudolph CD, Taché Y, Tarbell S, Vakil N | display-authors = 6 | title = Cyclic vomiting syndrome in adults | journal = Neurogastroenterology and Motility | volume = 20 | issue = 4 | pages = 269–84 | date = April 2008 | pmid = 18371009 | doi = 10.1111/j.1365-2982.2008.01113.x | url = https://deepblue.lib.umich.edu/bitstream/2027.42/72300/1/j.1365-2982.2008.01113.x.pdf | hdl = 2027.42/72300 }}</ref>

Treatment may include [[ondansetron]], [[sumatriptan]], or [[aprepitant]].<ref>{{cite journal |last1=Gui |first1=S |last2=Patel |first2=N |last3=Issenman |first3=R |last4=Kam |first4=AJ |title=Acute Management of Pediatric Cyclic Vomiting Syndrome: A Systematic Review. |journal=The Journal of pediatrics |date=November 2019 |volume=214 |pages=158-164.e4 |doi=10.1016/j.jpeds.2019.06.057 |pmid=31540764}}</ref>


==Signs and symptoms==
==Signs and symptoms==
{| class="wikitable"
⚫
Sufferers may vomit or retch 6–12 times in an hour and an episode may last from a few hours to over three weeks and in some cases months, with a median episode duration of 41 hours.<ref name=Li1999>{{cite journal | vauthors = Li BU, Fleisher DR | title = Cyclic vomiting syndrome: features to be explained by a pathophysiologic model | journal = Digestive Diseases and Sciences | volume = 44 | issue = 8 Suppl | pages = 13S–18S | date = August 1999 | pmid = 10490033 | doi = 10.1023/A:1026662402734 }}</ref> Acid, bile and, if the vomiting is severe, [[haematemesis|blood]] may be vomited. Some sufferers will ingest water to reduce the [[Gastroesophageal reflux disease|irritation of bile and acid on the esophagus]] during emesis. Between episodes, the sufferer is usually normal and healthy otherwise but can be in a weak state of [[Fatigue (medical)|fatigue]] or experience [[myalgia|muscle pain]]. In approximately half of cases 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.
!
!Adults
!Children
|-
|Mean age of diagnosis
|29-34 years old
|3-7 years old
|-
|Mean duration of episodes
|3-6 days
|few hours to 4 days
|-
|Mean Inter-episodic duration
|1-3 months
|1 week to 1 month
|-
|Presence of Prodrome phase
|common
|common
|-
|Recovery time
|lasting several days
|lasting hours to days
|-
|Vomiting
|universal up to 6 times an hour
|universal up to 6 times an hour
|-
|Abdominal pain
|common (57-70)%
|common (68-80)%
|-
|UGI Complications
|common (38%)
|common (22-32)%
|-
|Headaches
|common
|common
|-
|Fever
|not common
|not common
|-
|Dehydration needing IV fluids
|common
|common with longer attacks
|-
|Family history with migraines
|common (30-70)%
|common (40-89)%
|-
|Psychiatric disorders
|common
|common
|-
|Inter-episodic nausea/pain
|common
|rare
|-
|Mitochondrial DNA disorders
|not reported
|reported
|-
|Cannabis use
|reported
|not reported
|-
|Unpleasant triggers
|common (67%)
|common harder to pinpoint
|}


⚫
Episodes may happen every few days, every few weeks or every few months. For some sufferers, 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]], heightened sensitivity, especially to light, though sensitivity to smell, sound, pressure, and temperature, as well as oncoming muscle pain and fatigue, are also reported by some patients. The majority of sufferers can identify triggers that may precipitate an attack. The most common are various foods, [[infection]]s (e.g., [[colds]]), menstruation, extreme physical exertion, lack of sleep, and psychological [[stress (biological)|stress]]es, both positive and negative.


⚫
Sufferers may vomit or retch 6–12 times in an hour and an episode may last from a few hours to over three weeks and in some cases months, with a median episode duration of 41 hours.<ref name="Li1999">{{cite journal | vauthors = Li BU, Fleisher DR | title = Cyclic vomiting syndrome: features to be explained by a pathophysiologic model | journal = Digestive Diseases and Sciences | volume = 44 | issue = 8 Suppl | pages = 13S–18S | date = August 1999 | pmid = 10490033 | doi = 10.1023/A:1026662402734 }}</ref> Acid, bile and, if the vomiting is severe, [[haematemesis|blood]] may be vomited. Some sufferers will ingest water to reduce the [[Gastroesophageal reflux disease|irritation of bile and acid on the esophagus]] during emesis. Between episodes, the sufferer is usually normal and healthy otherwise but can be in a weak state of [[Fatigue (medical)|fatigue]] or experience [[myalgia|muscle pain]]. In approximately half of cases 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. Some sufferers experience episodes that progressively get worse when left untreated, occurring more frequently with reduced symptom free phase.<ref name=":0">{{Cite journal|last=Bhandari|first=Sanjay|last2=Jha|first2=Pinky|last3=Thakur|first3=Abhishek|last4=Kar|first4=Abhipsa|last5=Gerdes|first5=Harrison|last6=Venkatesan|first6=Thangam|date=2018-04-01|title=Cyclic vomiting syndrome: epidemiology, diagnosis, and treatment|url=https://doi.org/10.1007/s10286-018-0506-2|journal=Clinical Autonomic Research|language=en|volume=28|issue=2|pages=203–209|doi=10.1007/s10286-018-0506-2|issn=1619-1560}}</ref>
⚫
A sufferer may also be light-sensitive ([[photophobia|photophobic]]), sound-sensitive ([[phonophobia|phonophobic]]) or, less frequently, temperature- or pressure-sensitive during an attack.<ref name="Lindley 2005" /> Some sufferers also have a strong urge to bathe in warm or cold water. Some sufferers report 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 due to dehydration rather than the underlying cause of CVS.

⚫
Episodes may happen every few days, every few weeks or every few months, for some happening at common uniform times typically mornings.<ref name=":0" /> For other sufferers, there is not a pattern in time that can be recognized. Some sufferers have a warning of an episodic attack; they may experience a [[prodrome]], usually intense nausea and [[pallor]], heightened sensitivity, especially to light, though sensitivity to smell, sound, pressure, and temperature, as well as oncoming muscle pain and fatigue, are also reported by some patients. The majority of sufferers can identify triggers that may precipitate an attack. The most common are various foods, [[infection]]s (e.g., [[colds]]), menstruation, extreme physical exertion, lack of sleep, and psychological [[stress (biological)|stress]]es, both positive and negative.

⚫
A sufferer may also be light-sensitive ([[photophobia|photophobic]]), sound-sensitive ([[phonophobia|phonophobic]]) or, less frequently, temperature- or pressure-sensitive during an attack.<ref name="Lindley 2005">{{cite journal|vauthors=Lindley KJ, Andrews PL|date=September 2005|title=Pathogenesis and treatment of cyclical vomiting|journal=Journal of Pediatric Gastroenterology and Nutrition|volume=41 Suppl 1|issue=Suppl 1|pages=S38-40|doi=10.1097/01.scs.0000180299.04731.cb|pmid=16131963}}</ref> Some sufferers also have a strong urge to bathe in warm or cold water. Some sufferers experience [[Insomnia|insomnia,]] diarrhea (GI complications), hot and cold flashes, and excessive sweating before an episode. Some sufferers report 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 due to dehydration or [[hypokalemia]] from excessive vomiting, rather than the underlying cause of CVS.


==Genetics==
==Genetics==
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===Diagnostic criteria===
===Diagnostic criteria===
Although there are differences{{such as|date=December 2018}} between early-onset CVS (babies and children) and late-onset CVS (in adults),<ref>{{cite journal | vauthors = Boles RG, Zaki EA, Lavenbarg T, Hejazi R, Foran P, Freeborn J, Trilokekar S, McCallum R | display-authors = 6 | title = Are pediatric and adult-onset cyclic vomiting syndrome (CVS) biologically different conditions? Relationship of adult-onset CVS with the migraine and pediatric CVS-associated common mtDNA polymorphisms 16519T and 3010A | journal = Neurogastroenterology and Motility | volume = 21 | issue = 9 | pages = 936–e72 | date = September 2009 | pmid = 19368653 | doi = 10.1111/j.1365-2982.2009.01305.x }}</ref> there are established criteria to aid in diagnosis of CVS, namely:
Although there are differences{{such as|date=December 2018}} between early-onset CVS (babies and children) and late-onset CVS (in adults),<ref name=":0" /> there are established criteria to aid in diagnosis of CVS, namely:
#A history of three or more periods of intense, acute nausea and unremitting vomiting, as well as pain in some cases, lasting hours to days and even weeks or months<ref name="pmid28745840">{{cite journal | vauthors = Sagar RC, Sood R, Gracie DJ, Gold MJ, To N, Law GR, Ford AC | title = Cyclic vomiting syndrome is a prevalent and under-recognized condition in the gastroenterology outpatient clinic | journal = Neurogastroenterology and Motility : The Official Journal of the European Gastrointestinal Motility Society | volume = 30 | issue = 1 | pages = e13174| date = January 2018 | pmid = 28745840 | doi = 10.1111/nmo.13174 | url = http://eprints.lincoln.ac.uk/29512/1/29512%20CVS%20paper.pdf }}</ref>
#A history of three or more periods of intense, acute nausea and unremitting vomiting, as well as pain in some cases, lasting hours to days and even weeks or months<ref name="pmid28745840">{{cite journal | vauthors = Sagar RC, Sood R, Gracie DJ, Gold MJ, To N, Law GR, Ford AC | title = Cyclic vomiting syndrome is a prevalent and under-recognized condition in the gastroenterology outpatient clinic | journal = Neurogastroenterology and Motility : The Official Journal of the European Gastrointestinal Motility Society | volume = 30 | issue = 1 | pages = e13174| date = January 2018 | pmid = 28745840 | doi = 10.1111/nmo.13174 | url = http://eprints.lincoln.ac.uk/29512/1/29512%20CVS%20paper.pdf }}</ref>
#Intervening symptom-free or reduced-symptom intervals, lasting weeks to months
#Intervening symptom-free or reduced-symptom intervals, lasting weeks to months
Line 53: Line 126:


==Treatment==
==Treatment==
Treatment for Cyclic vomiting syndrome depends on the evident phase of the disorder.
There is no known cure for CVS, but there are medications that can be used for treatment, intervention, and prevention. There is a growing body of publications on both individual cases and the experiences of the CVS cohort. Treatment is usually on an individual basis, based on trial and error.

Because the symptoms of CVS are similar (or perhaps identical) to those of the disease well-identified as "[[ICHD classification and diagnosis of migraine#Abdominal migraine|abdominal migraine]]", beginning to establishing a [[Preventive healthcare|prophylactic]] migraine medications such as [[Topiramate|torpiramate]] and [[amitriptyline]], has seen recent success in treatment for the prodrome, and vomiting, phases, reducing the duration, severity, and frequency of episodes.<ref>{{Cite web|url=https://journals.lww.com/jpgn/Fulltext/2012/05000/Antimigraine__Low_Amine__Diet_May_Be_Helpful_in.28.aspx|title=Antimigraine (Low-Amine) Diet May Be Helpful in Children... : Journal of Pediatric Gastroenterology and Nutrition|website=LWW|language=en-US|access-date=2019-11-19}}</ref>

therapeutic treatment for the Prodromal phase, characterized by the anticipation of an episode, are [[Sumatriptan/naproxen sodium|sumatriptan]] (nasal or oral) an anti-migraine medication, [[Nonsteroidal anti-inflammatory drug|anti-inflammatory drugs]] to reduce abdominal pain, and possible [[Antiemetic|anti-emetic]] drugs. These options may be helpful in preventing an episode, or reducing the severity of an attack.

The most common therapeutic strategies for those already in the vomiting phase''',''' 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. For a number of patients, potent anti-emetic drugs such as [[ondansetron]] (Zofran) or [[granisetron]] (Kytril), and [[dronabinol]] (Marinol) may be helpful in either preventing an attack, aborting an attack, or reducing the severity of an attack. Many who comfort seek during episodes take prolonged showers and baths typically quite hot, sufferers only find relief while in the bath or shower, the use of a heating pad may help reduce abdominal pain.<ref>{{Cite journal|last=Fleisher|first=David R.|last2=Gornowicz|first2=Blake|last3=Adams|first3=Kathleen|last4=Burch|first4=Richard|last5=Feldman|first5=Edward J.|date=2005-12-21|title=Cyclic Vomiting Syndrome in 41 adults: the illness, the patients, and problems of management|url=https://doi.org/10.1186/1741-7015-3-20|journal=BMC Medicine|volume=3|issue=1|pages=20|doi=10.1186/1741-7015-3-20|issn=1741-7015|pmc=PMC1326207|pmid=16368014}}</ref>


Lifestyle changes may be recommended, such as extended rest, reduction of stress, frequent small meals, abstain from [[fasting]], and possibly cessation of [[cannabis]] use. A diet change may be recommended avoid food [[Allergen|allergens]], eliminating trigger foods such as, chocolates, cheese, bear, and red wine.<ref>{{Cite journal|last=Abell|first=T. L.|last2=Adams|first2=K. A.|last3=Boles|first3=R. G.|last4=Bousvaros|first4=A.|last5=Chong|first5=S. K. F.|last6=Fleisher|first6=D. R.|last7=Hasler|first7=W. L.|last8=Hyman|first8=P. E.|last9=Issenman|first9=R. M.|last10=Li|first10=B. U. K.|last11=Linder|first11=S. L.|date=2008|title=Cyclic vomiting syndrome in adults|url=https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1365-2982.2008.01113.x|journal=Neurogastroenterology & Motility|language=en|volume=20|issue=4|pages=269–284|doi=10.1111/j.1365-2982.2008.01113.x|issn=1365-2982}}</ref><ref>{{Cite journal|last=Li|first=B. Uk|last2=Lefevre|first2=Frank|last3=Chelimsky|first3=Gisela G.|last4=Boles|first4=Richard G.|last5=Nelson|first5=Susanne P.|last6=Lewis|first6=Donald W.|last7=Linder|first7=Steven L.|last8=Issenman|first8=Robert M.|last9=Rudolph|first9=Colin D.|date=2008-9|title=North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Consensus Statement on the Diagnosis and Management of Cyclic Vomiting Syndrome|url=https://journals.lww.com/jpgn/FullText/2008/09000/North_American_Society_for_Pediatric.22.aspx|journal=Journal of Pediatric Gastroenterology and Nutrition|language=en-US|volume=47|issue=3|pages=379|doi=10.1097/MPG.0b013e318173ed39|issn=0277-2116}}</ref>
The most common therapeutic strategies for those already in an attack are maintenance of salt balance by appropriate intravenous fluids<ref name="pmid24862509">{{cite journal | vauthors = Hejazi RA, McCallum RW | title = Cyclic vomiting syndrome: treatment options | journal = Experimental Brain Research | volume = 232 | issue = 8 | pages = 2549–52 | date = August 2014 | pmid = 24862509 | doi = 10.1007/s00221-014-3989-7 }}</ref> and, in some cases, sedation. Having vomited for a long period prior to attending a hospital, patients are typically severely dehydrated. For a number of patients, potent anti-emetic drugs such as [[ondansetron]] (Zofran) or [[granisetron]] (Kytril), and [[dronabinol]] (Marinol) may be helpful in either preventing an attack, aborting an attack, or reducing the severity of an attack. Lifestyle changes may be recommended, such as extended rest and reduction of stress.<ref name="NHS">{{cite web |url=https://www.nhs.uk/conditions/cyclical-vomiting-syndrome/ |title=Cyclical vomiting syndrome |author=<!--Not stated--> |date= 2017-10-18|website= |publisher=NHS Gov.UK |access-date= |quote=}}</ref> Because the symptoms of CVS are similar (or perhaps identical) to those of the disease well-identified as "[[ICHD classification and diagnosis of migraine#Abdominal migraine|abdominal migraine]]", treatment of CVS with a regimen of anti-migraine drugs, such as [[topiramate]] and [[amitriptyline]], is showing promise in preventing recurrent attacks.


Some patients experience relief from inhaled isopropyl alcohol.<ref name="NEJM">{{cite web |url=https://www.jwatch.org/na46286/2018/03/09/inhaled-isopropyl-alcohol-superior-oral-ondansetron |title=Inhaled Isopropyl Alcohol Superior to Oral Ondansetron as an Antiemetic |author=<!--Not stated--> |date= 2018-03-09|website= |publisher=New England Journal of Medicine Journal Watch jwatch.org |access-date= |quote=}}</ref>
Some patients experience relief from inhaled isopropyl alcohol.<ref name="NEJM">{{cite web |url=https://www.jwatch.org/na46286/2018/03/09/inhaled-isopropyl-alcohol-superior-oral-ondansetron |title=Inhaled Isopropyl Alcohol Superior to Oral Ondansetron as an Antiemetic |author=<!--Not stated--> |date= 2018-03-09|website= |publisher=New England Journal of Medicine Journal Watch jwatch.org |access-date= |quote=}}</ref>
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Fitzpatrick et al. (2007) identified 41 children with CVS. 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%).<ref name="pmid18070235">{{cite journal | vauthors = Fitzpatrick E, Bourke B, Drumm B, Rowland M | title = The incidence of cyclic vomiting syndrome in children: population-based study | journal = The American Journal of Gastroenterology | volume = 103 | issue = 4 | pages = 991–5; quiz 996 | date = April 2008 | pmid = 18070235 | doi = 10.1111/j.1572-0241.2007.01668.x }}</ref>
Fitzpatrick et al. (2007) identified 41 children with CVS. 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%).<ref name="pmid18070235">{{cite journal | vauthors = Fitzpatrick E, Bourke B, Drumm B, Rowland M | title = The incidence of cyclic vomiting syndrome in children: population-based study | journal = The American Journal of Gastroenterology | volume = 103 | issue = 4 | pages = 991–5; quiz 996 | date = April 2008 | pmid = 18070235 | doi = 10.1111/j.1572-0241.2007.01668.x }}</ref>


Most children who have this disorder miss on average 24 school days a year.<ref name="Li2008" /> The frequency of episodes is higher for some people during times of excitement.<ref name="Li2008"/> Charitable organizations to support sufferers and their families and to promote knowledge of CVS exist in several countries.
Most children who have this disorder miss on average 24 school days a year.<ref name="Li2008">{{cite journal|display-authors=6|vauthors=Li BU, Lefevre F, Chelimsky GG, Boles RG, Nelson SP, Lewis DW, Linder SL, Issenman RM, Rudolph CD|date=September 2008|title=North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome|journal=Journal of Pediatric Gastroenterology and Nutrition|volume=47|issue=3|pages=379–93|doi=10.1097/MPG.0b013e318173ed39|pmid=18728540}}</ref> The frequency of episodes is higher for some people during times of excitement.<ref name="Li2008"/> Charitable organizations to support sufferers and their families and to promote knowledge of CVS exist in several countries.


Complications can include dehydration, cavities, or an [[esophageal tear]].<ref name="NHS"/>
Complications can include dehydration, cavities, or an [[esophageal tear]].<ref name="NHS">{{cite web|url=https://www.nhs.uk/conditions/cyclical-vomiting-syndrome/|title=Cyclical vomiting syndrome|author=<!--Not stated-->|date=2017-10-18|website=|publisher=NHS Gov.UK|access-date=|quote=}}</ref>


==Epidemiology==
==Epidemiology==

Revision as of 16:37, 21 November 2019

Cyclic vomiting syndrome
Other namesCyclical vomiting syndrome
SpecialtyPediatrics

Cyclic vomiting syndrome (CVS) is a chronic functional condition of unknown pathogenesis. CVS is characterized as recurring episodes lasting a single day to multiple weeks. Each episode is divided into four phases: inter-episodic, prodrome, vomiting, and recovery. Inter-episodic phase (symptom free phase), is characterized as no discernible symptoms, normal everyday activities can occur, and this phase typically lasts one week to one month. The prodrome phase is known as the pre-emetic phase, characterized by the initial feeling an approaching episode, still able to keep down oral medication. Emetic or vomiting phase is characterized as intense persistent nausea, and repeated vomiting typically lasting hours to days. Recovery phase is typically the phase where vomiting ceases, nausea diminishes or is absent, and appetite returns. This syndrome is most commonly seen in children usually between ages 3 and 7, however adult diagnosis is quite common.[1] This disorder is thought to be closely related to migraines and family history of migraines. [2][3]

Signs and symptoms

Adults Children
Mean age of diagnosis 29-34 years old 3-7 years old
Mean duration of episodes 3-6 days few hours to 4 days
Mean Inter-episodic duration 1-3 months 1 week to 1 month
Presence of Prodrome phase common common
Recovery time lasting several days lasting hours to days
Vomiting universal up to 6 times an hour universal up to 6 times an hour
Abdominal pain common (57-70)% common (68-80)%
UGI Complications common (38%) common (22-32)%
Headaches common common
Fever not common not common
Dehydration needing IV fluids common common with longer attacks
Family history with migraines common (30-70)% common (40-89)%
Psychiatric disorders common common
Inter-episodic nausea/pain common rare
Mitochondrial DNA disorders not reported reported
Cannabis use reported not reported
Unpleasant triggers common (67%) common harder to pinpoint


Sufferers may vomit or retch 6–12 times in an hour and an episode may last from a few hours to over three weeks and in some cases months, with a median episode duration of 41 hours.[4] 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 emesis. Between episodes, the sufferer is usually normal and healthy otherwise but can be in a weak state of fatigue or experience muscle pain. In approximately half of cases 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. Some sufferers experience episodes that progressively get worse when left untreated, occurring more frequently with reduced symptom free phase.[5]

Episodes may happen every few days, every few weeks or every few months, for some happening at common uniform times typically mornings.[5] For other sufferers, there is not a pattern in time that can be recognized. Some sufferers have a warning of an episodic attack; they may experience a prodrome, usually intense nausea and pallor, heightened sensitivity, especially to light, though sensitivity to smell, sound, pressure, and temperature, as well as oncoming muscle pain and fatigue, are also reported by some patients. The majority of sufferers can identify triggers that may precipitate an attack. The most common are various foods, infections (e.g., colds), menstruation, extreme physical exertion, lack of sleep, and psychological stresses, both positive and negative.

A sufferer may also be light-sensitive (photophobic), sound-sensitive (phonophobic) or, less frequently, temperature- or pressure-sensitive during an attack.[6] Some sufferers also have a strong urge to bathe in warm or cold water. Some sufferers experience insomnia, diarrhea (GI complications), hot and cold flashes, and excessive sweating before an episode. Some sufferers report 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 due to dehydration or hypokalemia from excessive vomiting, rather than the underlying cause of CVS.

Genetics

There is no known genetic pathogenesis for CVS. Recent studies suggest many affected individuals have a family history of related conditions, such as migraines, psychiatric disorders and gastrointestinal disorders. Inheritance is thought to be maternal, a possible genetic mitochondrial inheritance. Adolescents show higher possible mitochondrial inheritance and maternal inheritance than found in adults. Single base-pair and DNA rearrangements in the mitochondrial DNA (mtDNA) have been associated with these traits.[7][8]

Diagnosis

The cause of CVS has not been determined and there are no diagnostic tests for CVS. Several other medical conditions, such as cannabinoid hyperemesis syndrome, 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 using Rome criteria by a physician may be appropriate.[9]

Once formal investigations to rule out gastrointestinal or other causes have been conducted, these tests do not need to be repeated in the event of future episodes.[6]

Diagnostic criteria

Although there are differences[example needed] between early-onset CVS (babies and children) and late-onset CVS (in adults),[5] there are established criteria to aid in diagnosis of CVS, namely:

  1. A history of three or more periods of intense, acute nausea and unremitting vomiting, as well as pain in some cases, lasting hours to days and even weeks or months[10]
  2. Intervening symptom-free or reduced-symptom intervals, lasting weeks to months
  3. There are repeated cycles of periods (of varying duration) with intense/acute nausea, with or without vomiting, with or without severe pain, followed by periods of reduced symptoms, followed by gradual increase in CVS symptoms until it peaks (peak intensity is generally relative to cycle intensity).
  4. Exclusion of metabolic, gastrointestinal, genitourinary or central nervous system structural or biochemical disease, e.g., individuals with specific physical causes (such as intestinal malrotation)

Treatment

Treatment for Cyclic vomiting syndrome depends on the evident phase of the disorder.

Because the symptoms of CVS are similar (or perhaps identical) to those of the disease well-identified as "abdominal migraine", beginning to establishing a prophylactic migraine medications such as torpiramate and amitriptyline, has seen recent success in treatment for the prodrome, and vomiting, phases, reducing the duration, severity, and frequency of episodes.[11]

therapeutic treatment for the Prodromal phase, characterized by the anticipation of an episode, are sumatriptan (nasal or oral) an anti-migraine medication, anti-inflammatory drugs to reduce abdominal pain, and possible anti-emetic drugs. These options may be helpful in preventing an episode, or reducing the severity of an attack.

The most common therapeutic strategies for those already in the vomiting phase, 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. For a number of patients, potent anti-emetic drugs such as ondansetron (Zofran) or granisetron (Kytril), and dronabinol (Marinol) may be helpful in either preventing an attack, aborting an attack, or reducing the severity of an attack. Many who comfort seek during episodes take prolonged showers and baths typically quite hot, sufferers only find relief while in the bath or shower, the use of a heating pad may help reduce abdominal pain.[12]

Lifestyle changes may be recommended, such as extended rest, reduction of stress, frequent small meals, abstain from fasting, and possibly cessation of cannabis use. A diet change may be recommended avoid food allergens, eliminating trigger foods such as, chocolates, cheese, bear, and red wine.[13][14]

Some patients experience relief from inhaled isopropyl alcohol.[15]

Prognosis

Fitzpatrick et al. (2007) identified 41 children with CVS. 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%).[16]

Most children who have this disorder miss on average 24 school days a year.[17] The frequency of episodes is higher for some people during times of excitement.[17] Charitable organizations to support sufferers and their families and to promote knowledge of CVS exist in several countries.

Complications can include dehydration, cavities, or an esophageal tear.[18]

Epidemiology

The average age at onset is 3–7 years, with described cases as young as 6 days and as old as 73 years.[19] Typical delay in diagnosis from onset of symptoms is 3 years.[19] Females show a slight predominance over males.[19]

One study found that 3 in 100,000 five-year-olds are diagnosed with the condition.[20] Two studies on childhood CVS suggest nearly 2% of school-age children may have CVS.[21][22]

History

Cyclic vomiting syndrome was first described in France by Swiss physician Henri Clermond Lombard[23] and first described in the English language by pediatrician Samuel Gee in 1882.[24]

It has been suggested that Charles Darwin's adult illnesses may have been due to this syndrome.[25][further explanation needed]

See also

References

  1. ^ Lee, Lennard Y. W.; Abbott, Laura; Mahlangu, Bruce; Moodie, Simon J.; Anderson, Simon (2012-9). "The management of cyclic vomiting syndrome: a systematic review". European Journal of Gastroenterology & Hepatology. 24 (9): 1001. doi:10.1097/MEG.0b013e328355638f. ISSN 0954-691X. {{cite journal}}: Check date values in: |date= (help)
  2. ^ Fleisher, David R.; Gornowicz, Blake; Adams, Kathleen; Burch, Richard; Feldman, Edward J. (2005-12-21). "Cyclic Vomiting Syndrome in 41 adults: the illness, the patients, and problems of management". BMC Medicine. 3 (1): 20. doi:10.1186/1741-7015-3-20. ISSN 1741-7015. PMC 1326207. PMID 16368014.{{cite journal}}: CS1 maint: PMC format (link) CS1 maint: unflagged free DOI (link)
  3. ^ Abell, T. L.; Adams, K. A.; Boles, R. G.; Bousvaros, A.; Chong, S. K. F.; Fleisher, D. R.; Hasler, W. L.; Hyman, P. E.; Issenman, R. M.; Li, B. U. K.; Linder, S. L. (2008). "Cyclic vomiting syndrome in adults". Neurogastroenterology & Motility. 20 (4): 269–284. doi:10.1111/j.1365-2982.2008.01113.x. ISSN 1365-2982.
  4. ^ Li BU, Fleisher DR (August 1999). "Cyclic vomiting syndrome: features to be explained by a pathophysiologic model". Digestive Diseases and Sciences. 44 (8 Suppl): 13S–18S. doi:10.1023/A:1026662402734. PMID 10490033.
  5. ^ a b c Bhandari, Sanjay; Jha, Pinky; Thakur, Abhishek; Kar, Abhipsa; Gerdes, Harrison; Venkatesan, Thangam (2018-04-01). "Cyclic vomiting syndrome: epidemiology, diagnosis, and treatment". Clinical Autonomic Research. 28 (2): 203–209. doi:10.1007/s10286-018-0506-2. ISSN 1619-1560.
  6. ^ a b Lindley KJ, Andrews PL (September 2005). "Pathogenesis and treatment of cyclical vomiting". Journal of Pediatric Gastroenterology and Nutrition. 41 Suppl 1 (Suppl 1): S38-40. doi:10.1097/01.scs.0000180299.04731.cb. PMID 16131963.
  7. ^ "What is cyclic vomiting syndrome?".
  8. ^ Venkatesan T, Zaki EA, Kumar N, Sengupta J, Ali M, Malik B, et al. (October 2014). "Quantitative pedigree analysis and mitochondrial DNA sequence variants in adults with cyclic vomiting syndrome". BMC Gastroenterology. 14 (1): 181. doi:10.1186/1471-230X-14-181. PMC 4287476. PMID 25332060.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  9. ^ Bhandari S, Jha P, Thakur A, Kar A, Gerdes H, Venkatesan T (April 2018). "Cyclic vomiting syndrome: epidemiology, diagnosis, and treatment". Clinical Autonomic Research. 28 (2): 203–209. doi:10.1007/s10286-018-0506-2. PMID 29442203.
  10. ^ Sagar RC, Sood R, Gracie DJ, Gold MJ, To N, Law GR, Ford AC (January 2018). "Cyclic vomiting syndrome is a prevalent and under-recognized condition in the gastroenterology outpatient clinic" (PDF). Neurogastroenterology and Motility : The Official Journal of the European Gastrointestinal Motility Society. 30 (1): e13174. doi:10.1111/nmo.13174. PMID 28745840.
  11. ^ "Antimigraine (Low-Amine) Diet May Be Helpful in Children... : Journal of Pediatric Gastroenterology and Nutrition". LWW. Retrieved 2019-11-19.
  12. ^ Fleisher, David R.; Gornowicz, Blake; Adams, Kathleen; Burch, Richard; Feldman, Edward J. (2005-12-21). "Cyclic Vomiting Syndrome in 41 adults: the illness, the patients, and problems of management". BMC Medicine. 3 (1): 20. doi:10.1186/1741-7015-3-20. ISSN 1741-7015. PMC 1326207. PMID 16368014.{{cite journal}}: CS1 maint: PMC format (link) CS1 maint: unflagged free DOI (link)
  13. ^ Abell, T. L.; Adams, K. A.; Boles, R. G.; Bousvaros, A.; Chong, S. K. F.; Fleisher, D. R.; Hasler, W. L.; Hyman, P. E.; Issenman, R. M.; Li, B. U. K.; Linder, S. L. (2008). "Cyclic vomiting syndrome in adults". Neurogastroenterology & Motility. 20 (4): 269–284. doi:10.1111/j.1365-2982.2008.01113.x. ISSN 1365-2982.
  14. ^ Li, B. Uk; Lefevre, Frank; Chelimsky, Gisela G.; Boles, Richard G.; Nelson, Susanne P.; Lewis, Donald W.; Linder, Steven L.; Issenman, Robert M.; Rudolph, Colin D. (2008-9). "North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Consensus Statement on the Diagnosis and Management of Cyclic Vomiting Syndrome". Journal of Pediatric Gastroenterology and Nutrition. 47 (3): 379. doi:10.1097/MPG.0b013e318173ed39. ISSN 0277-2116. {{cite journal}}: Check date values in: |date= (help)
  15. ^ "Inhaled Isopropyl Alcohol Superior to Oral Ondansetron as an Antiemetic". New England Journal of Medicine Journal Watch jwatch.org. 2018-03-09.
  16. ^ Fitzpatrick E, Bourke B, Drumm B, Rowland M (April 2008). "The incidence of cyclic vomiting syndrome in children: population-based study". The American Journal of Gastroenterology. 103 (4): 991–5, quiz 996. doi:10.1111/j.1572-0241.2007.01668.x. PMID 18070235.
  17. ^ a b Li BU, Lefevre F, Chelimsky GG, Boles RG, Nelson SP, Lewis DW, et al. (September 2008). "North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome". Journal of Pediatric Gastroenterology and Nutrition. 47 (3): 379–93. doi:10.1097/MPG.0b013e318173ed39. PMID 18728540.
  18. ^ "Cyclical vomiting syndrome". NHS Gov.UK. 2017-10-18.
  19. ^ a b c Li BU, Misiewicz L (September 2003). "Cyclic vomiting syndrome: a brain-gut disorder". Gastroenterology Clinics of North America. 32 (3): 997–1019. doi:10.1016/S0889-8553(03)00045-1. PMID 14562585.
  20. ^ Drumm BR, Bourke B, Drummond J, McNicholas F, Quinn S, Broderick A, et al. (October 2012). "Cyclical vomiting syndrome in children: a prospective study". Neurogastroenterology and Motility. 24 (10): 922–7. doi:10.1111/j.1365-2982.2012.01960.x. PMID 22762244.
  21. ^ Abu-Arafeh I, Russell G (November 1995). "Cyclical vomiting syndrome in children: a population-based study". Journal of Pediatric Gastroenterology and Nutrition. 21 (4): 454–8. doi:10.1097/00005176-199511000-00014. PMID 8583299.
  22. ^ Cullen KJ, Ma Cdonald WB (August 1963). "The periodic syndrome: its nature and prevalence". The Medical Journal of Australia. 50 (2): 167–73. PMID 14024194.
  23. ^ Lombard HC (1861). "Description d'une névrose de la digestion, caractérisée par des crises périodiques de vomissements et une profonde modification de l'assimilation". Gazette Medicale de Paris: 312.
  24. ^ Gee S (1882). "On fitful or recurrent vomiting". St Bartholomew Hospital Reports. 18: 1.
  25. ^ Hayman JA (December 2009). "Darwin's illness revisited". BMJ. 339: b4968. doi:10.1136/bmj.b4968. PMID 20008377.

Further reading

External links

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