BOSTON — Cyclic vomiting syndrome in adults often goes unrecognized for years after onset, despite its severe and disabling consequences.
The disorder may be the cause of repeat visits to emergency departments, unnecessary surgeries and diagnostic tests, and substance abuse, according to participants at the first-ever symposium devoted to cyclic vomiting syndrome (CVS) in adults. The gathering was held as a satellite meeting following a meeting on neurogastroenterology and motility.
Diagnosis of CVS in adults is complicated by the variability in age of onset and pattern of symptoms. The result is that the average delay in making the diagnosis is 8 years from the time symptoms first appear, noted Dr. B. U.K. Li, director of the Center for CVS at the Medical College of Wisconsin, Milwaukee, in a presentation at the meeting.
“Where were we? How did we miss these folks?” Dr. Richard McCallum of the Kansas University Medical Center, Kansas City, rhetorically asked the audience, composed of physicians, patients, and family members.
As the director of the first center for CVS in adults, Dr. McCallum said, “We're getting a continued trickle of patients coming to us, and an avalanche of phone calls from patients and physicians from around the world.”
According to the Rome 3 criteria H1b, the definition of CVS is two or more periods of intense nausea or unremitting vomiting or retching lasting hours to days, with a return to the usual state of health lasting weeks to months (J. Gastro. Liver Dis. 2006;15:237–41). In its mildest form, the symptoms of CVS do not interfere with a patient's ability to work or attend school, said Dr. David Fleisher of the University of Missouri, Columbia, who has studied 41 adults with CVS.
He found that about 8% of his sample had mild symptoms, 44% had moderate symptoms that caused them to worry about their ability to continue work or school, and about 49% had incapacitating symptoms so severe that they were sick more often than they were well.
Some of these patients experienced eight or more vomiting episodes per hour for months on end, leading in some cases to more than 100 visits to the emergency department or hospitalizations. As CVS progresses, episodes may become more frequent with less time for recovery, a process Dr. Fleisher terms “coalescence.”
When rushed to the emergency department during the emetic phase, CVS patients can present with blood in the vomitus due to prolapse gastropathy or Mallory-Weiss tears resulting from forceful heaves, erosive esophagitis, and aspiration. Dehydration and electrolyte imbalance, particularly hypokalemia, may accompany the GI symptoms.
Patients often experience intense abdominal pain, and may demand narcotics; they may show signs of narcotic dependence. Tooth decay can be evident, and patients may describe chronic weight loss, Dr. Fleisher said.
Patients also may show signs of a hyperadrenergic state, including low-grade fever, rapid pulse, and hypertension. Neutrophilia without bandemia, accompanied by abdominal pain and vomiting, may be mis- diagnosed as pancreatitis, peptic ulcer, appendicitis, or pyelonephritis. The periodicity of attacks, with or without hypertension, may lead to confusion and give the impression of porphyria, pheochromocytoma, abdominal epilepsy, intermittent small bowel obstruction, or endometriosis.
Patients also may present with unusual behaviors and mental states that compound the difficulty of identifying CVS. Normally pleasant and affable patients may become irritable and verbally abusive, demanding medications. Patients may describe thirst so intense that they drink surreptitiously from toilet bowls. They may engage in a guzzle-and-vomit sequence that can be mistaken for bulimia, and which may actually be explained by the transient relief provided by sudden emptying of the stomach.
Other patients may ask for repeated hot showers or baths. Some patients may appear so immobile that it is difficult to establish whether they are asleep or awake; they also may withdraw from social contact, a frightening condition Dr. Fleisher describes as “conscious coma.”
CVS patients often undergo batteries of tests, including upper GI series, abdominal ultrasound and CT scan, colonoscopy, barium enema, endoscopy, MRI of the head, sinus radiography, EEG, and lab work, usually with negative results.
In Dr. Fleisher's series, the 41 patients underwent almost 300 diagnostic studies—none of which were indicative of an organic etiology for CVS. Recent findings suggest that about 75% of patients with CVS show rapid gastric emptying on electrogastrograms, and this may help distinguish CVS from other vomiting disorders (Neurogastroenterol. Motil. 2006;18:728 [abstract 200]).
Unnecessary surgeries are also common. Of the 41 patients in the series, 10 had undergone cholecystectomies, 2 had appendectomies, 5 had laparoscopies, 1 had a hysterectomy, and others had undergone other GI procedures. None of these procedures relieved the CVS symptoms.