By Marcello Cherchi, MD PhD
For patients
Cyclic vomiting syndrome (CVS) is pretty much what it sounds like: a condition in which a patient cycles through a period of vomiting, then a period without vomiting, that repeats. The duration and interval of symptoms is variable. The underlying cause of this disease is unknown, though much of the speculation views this as a variant of migraine.
For clinicians
Overview
Cyclic vomiting syndrome (CVS) manifests with recurrent episodes of severe nausea and emesis lasting hours to days, occurring at erratic intervals. A patient can be afflicted with these episodes over months to decades. CVS can occur at any age; it is slightly more common in females. Gastrointestinal workups (endoscopy, gastric emptying studies) and otovestibular workups are unremarkable. The mechanism of disease is unknown; some practitioners view it as a version of migraine, and may attempt treatment as such.
Introduction
Cyclic vomiting was first described by Samuel Gee in 1882 (Gee 1882). There have been several attempts at proposing diagnostic criteria (Li, Lefevre et al. 2008, Hyams, Di Lorenzo et al. 2016), but these have been neither uniform nor universally accepted.
Clinical presentation
Cyclic vomiting syndrome (CVS) manifests with recurrent episodes of severe nausea and emesis lasting hours to days, occurring at erratic intervals. A patient can be afflicted with these episodes over months to decades (Fleisher, Gornowicz et al. 2005). The duration of the episodes roughly exhibits a Gaussian distribution, as shown in the following table from Fleisher and Matar (Fleisher and Matar 1993):
Most descriptions of CVS comment on its intermittent quality, but some authors have noticed patients can develop increasingly frequent and prolonged episodes, to the point that symptoms appear nearly continuous; Fleisher and colleagues refer to this as “episode coalescence” (Fleisher, Gornowicz et al. 2005).
Some studies focus on the variability among patients (Dipasquale, Falsaperla et al. 2022), while others focus on similarities (Prakash, Staiano et al. 2001).
Demographics
Cyclic vomiting was originally thought to affect only children, but it has become clear that it can also affect adults (Fleisher, Gornowicz et al. 2005).
The age of onset, and the overall duration of the illness, are quite variable, as shown in the following table from Fleisher and Matar (Fleisher and Matar 1993):
It is slightly more common in females than in males (Ailani, Kaiser et al. 2022, Dipasquale, Falsaperla et al. 2022).
Mechanism of disease
The underlying mechanism of CVS is unknown (Frazier and Venkatesan 2022) and it remains a diagnosis of exclusion (Goldman 2021). Repeated gastrointestinal studies have failed to find any consistent structural abnormalities that would account for CVS, so it is generally believed to reflect a disorder of physiologic function (rather than due to a structural or anatomical cause). For decades there has been speculation that it is a variant of migraine (Li, Murray et al. 1999, Yu, Priyadharsini et al. 2018), and one study (Li, Murray et al. 1999) observed that 70% of adult patients with CVS also reported a history of migraine. Another study (Li, Murray et al. 1999) reported that 82% of pediatric patients report a history of migraine. Some studies speculate that CVS is an autonomic disorder (Rashed, Abell et al. 1999).
Differential diagnosis
The symptoms of CVS can be distressing to the point that clinicians cast a wide net seeking etiologies. One study (Li, Murray et al. 1999) reported patients to have undergone numerous laboratory tests, imaging studies, endoscopic procedures and laparoscopies, and had even undergone various surgeries (cholecystectomy, appendectomy, pyloroplasty, fundoplication) in an attempt to treat the problem.
Numerous diseases can present with episodic emesis (porphyria, pheochromocytoma, intermittent bowel obstruction). Fleisher and Matar (Fleisher and Matar 1993) provide the following table listing a very broad differential diagnosis:
Testing
One study observed that patients with CVS and migraine exhibit white matter intensities on brain MRI (Rashid, Weaver et al. 2022), but this is unsurprising and patients with a history of migraine alone can have such MRI findings.
The majority of patients with CVS do not complain of a disturbance of equilibrium, so commonly their evaluation and management are undertaken by gastrointestinal specialists.
Nevertheless, occasionally these patients are referred to an otoneurologist, often after having undergone a gastrointestinal workup, sometimes with endoscopy or a gastric motility study, with unremarkable results.
For most otovestibular conditions, gastrointestinal symptoms (nausea and vomiting) will accompany or will follow the symptom of disequilibrium. However, in some patients, the gastrointestinal symptoms may predominate the clinical picture. For this reason, we will usually consider undertaking a screening otovestibular workup (usually with vestibular evoked myogenic potentials and video head impulse testing).
Treatment
Treatment guidelines have been suggested (Venkatesan, Levinthal et al. 2019), though based on low quality evidence.
Given the apparent overlap between CVS and migraine, it has become somewhat common to attempt treatment with migraine prophylactic strategies (Ailani, Kaiser et al. 2022), such as amitriptyline (Goldman 2021), topiramate (Sezer and Sezer 2016, Goldman 2021, Mooers, Srivastava et al. 2021), valproate (Hikita, Kodama et al. 2009), flunarizine or cyproheptadine (Dipasquale, Falsaperla et al. 2022).
Overall the prognosis of CVS is favorable (Dipasquale, Falsaperla et al. 2022).
References
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