Clinical patterns

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CVS is distinguished by recurrent, severe, discrete episodes of vomiting. Episodes are stereotypical in regards to time of onset, duration and symptomatology. This disorder is also distinguished by an on-off pattern with intervals of returning to completely normal or baseline health between episodes.2 The duration of episodes is generally from hours to days, with a median duration of 27 h. The median frequency of episodes is 4 weeks. Because nearly 47% of all patients have regular intervals and the remaining have sporadic attacks at unpredictable intervals, 'cyclic' is a slight misnomer.

The most common time of onset is during nighttime or early morning hours with 42% of patients having onset from 01.00 to 07.00.10 Of patients, 67% have a well-described prodrome that precedes the episodes of vomiting. The parents often characterize both the onset and the resolution as sudden. The warning period typically lasts a median of 0.5-1.5 h. Despite the similarities to migraines, these prodromes rarely have visual disturbances and are characterized by pre-vomit-ing autonomic symptoms of pallor, nausea, abdominal pain and lethargy. Many parents have noted a rapid 'shut-off' of symptoms from the end of vomiting to the point of resuming oral intake and near-normal function that generally lasts a median of 8 h with a range of 0 h to 1 week.

The vomiting itself has been described as rapid-fire in nature, peaking at an average of six times per hour or once every 10min during the worst episode.4 The vomiting is typically projectile, and contains bile, mucus and occasionally blood. The latter generally occurs late in the episode as a result of prolapse gastropathy from repetitive vomiting which can also produce epigastric pain.11 Although a threshold of more than four emeses per hour at the peak originally identified 92% of those with CVS, we have detected increasing numbers who fit the consensus historical criteria with a lower intensity of vomiting of two to four times per hour.4 These children can appear remarkably debilitated during episodes. They are often curled up into the fetal position from pain, which is often accompanied by pallor, listlessness and unresponsiveness, and therefore appear much more ill than children with gastroenteritis.2

Even though children return to baseline health between episodes and are well about 90% of the time, CVS can have a significant impact on the quality of life of affected children.10 Over half (58%) of all affected patients require intravenous hydration during episodes and 19% require this with every episode. School-age children miss an average of 24 days of school per year, owing to episodes of vomiting. The high medical morbidity is reflected by the average annualized cost of management of $17000, including doctor visits, emergency department visits, in-patient hospital-izations, biochemical, radiographic, endoscopic testing and missed work by the parents.12

There are many symptoms that typically accompany vomiting during CVS episodes (Table 20.1). Abdominal pain, retching, anorexia and nausea are the most common gastrointestinal symptoms. The abdominal pain can be excruciating occasionally to the point of requiring narcotics and/or laparotomy.

By the patient's report, nausea is generally the most persistent and distressing symptom: it is minimally relieved by vomiting, often receding only while sleeping or with sedation. Typical behaviors (assuming a fetal position, social withdrawal, compulsive drinking, and avoiding lights and sounds) represent attempts to alleviate the nausea.10

The most common autonomic symptoms are lethargy and pallor. Other autonomic symptoms include fever, flushing, drooling, diarrhea, hypothermia and hypertension. Less than half of the patients have migraine features including headache (42%), photophobia (38%) and phono-phobia (30%). However, these numbers are significantly higher than in those with the chronic vomiting pattern who primarily have upper gastrointestinal tract disorders.13 Other symptoms

Table 20.1 Clinical features

Median age of onset: 4.8 years

Female/male: 57/43

Vomiting: 6 emesis/h at peak, with bile (81%), mucus (68.6%) and blood (34%)

Average emesis per episode: 31

Duration of episodes: 27h

Typical time of onset: 01.00-07.00

Median interval between episodes: 4 weeks

Associated symptoms: lethargy (93%), nausea (82%), abdominal pain (81%), anorexia (81%), retching (79%), headache (42%), photophobia (38%), phonophobia (30%)

Associated signs: pallor (91%), social withdrawal (54%), fever (30%), diarrhea (30%), drooling (27%)

Clinical patterns 291

during episodes include sensory hypersensitivity and vertigo.

The largest fraction (32%) have a seasonal clustering of episodes with more during the winter and fewer during the summer. Although this pattern correlates with the school year, we can only speculate that less school-related stress and less exposure to infections trigger fewer episodes. Winter holidays including Thanksgiving, Christmas and New Year can serve as a positive stress for some.

Various stressors have been noted to precipitate episodes of CVS. In 76% of patients, the parents can identify a recurring trigger preceding the vomiting episodes. These triggers consist of psychological, infectious and physical stressors (Table 20.2). Stress and infections are the most common triggers (44% and 31%, respectively). Interestingly, two-thirds of the stress is positive rather than negative. Various infections can trigger episodes, especially chronic sinusitis. Other triggers include dietary (23%), physical exhaustion (24%), atopic symptoms (6%), motion sickness (12%) and menses (22% of menstruating girls).

Evaluating the family history of patients with CVS is helpful in diagnosis because of the much higher rate of positive migraines (82%) compared to that obtained in those with chronic vomiting due to gastrointestinal disorders or to the general population (15%).5 Also, a family history of food allergies or atopy can be a clue for food triggers of attacks. In our series, we have found an unusually high percentage of patients with a family history of depression (40%) compared to the general population. How this plays a role in possible susceptibility to CVS of patients is currently unknown.

Cyclic versus chronic patterns of vomiting

An important clinical clue to the diagnosis of CVS is the pattern of vomiting. Based on a temporal pattern, children with recurrent vomiting can be delineated into cyclic and chronic groups (Figure 20.1). The cyclic group has an intense, but intermittent pattern of vomiting with peak emeses of > 4/h and 2 episodes per week.13 The chronic group has a low-grade, daily pattern of emeses with < 4 emeses/h and > 2 episodes per week13 (Table 20.3). These criteria are 92% sensitive and 100% specific for identifying children with CVS.2 Two-thirds of all children with recurrent vomiting fit into the chronic or continuous pattern of vomiting. These children rarely appear acutely ill or become dehydrated. Conversely, the cyclical pattern is associated with more intense vomiting and affected children more often require intravenous hydration (62% vs. 18%) compared with the chronic group.4

These two patterns are important because both of these groups differ in symptom and diagnostic profile. In those with the cyclical vomiting pattern,

Table 20.2 Common triggers for episodes of cyclic vomiting syndrome

Infections (urinary tract infection, streptococcal throat, flu, sinusitis, gastroenteritis) Positive stress (birthdays, holidays, vacations) Negative stress (school, family, deaths)

Dietary intake (cheese, chocolate, caffeine, monosodium glutamate)

Physical exhaustion (lack of sleep)

Motion sickness (car rides, roller-coasters, air travel)

Asthma

Allergies (seasonal, inhalants, foods) Menses

Surgery/anesthesia

Weather (temperature or pressure changes)

non-gastrointestinal disorders including neurological (including abdominal migraine), renal, endocrine and metabolic disorders predominate over gastrointestinal disorders by a ratio of 5 : 1.2,13 In contrast, in the chronic group, gastrointestinal disorders (mostly peptic disease) predominate over non-gastrointestinal causes of vomiting by a ratio of 7 : 1.2,13 This implies the need to center the diagnostic work-up on extraintestinal disorders in cyclic vomiting, and on upper gastrointestinal tract disorders in chronic vomiting. Consistent with proposed migraine association with CVS, the cyclic pattern of vomiting has a higher prevalence of family members with migraine headache (72% vs. 14%), associated headache (41% vs. 19%) and photophobia (18% vs. 4%).4

30 Days

Figure 20.1 Temporal patterns of vomiting: cyclic versus chronic. The number of emeses is plotted over a 2-month period. The chronic pattern represented by a dashed line has low-grade, nearly daily episodes, whereas the cyclic pattern represented by the solid line has many emeses over a 1-2-day period that recurs every few weeks. (Adapted from reference 46).

Differential diagnosis

Differentiating a cyclic versus a chronic pattern of vomiting is the first step in narrowing the differential diagnosis (Table 20.4). Although the majority of patients (88%) with a cyclical pattern ultimately are diagnosed with CVS, the remaining 12% have specific causes for vomiting found on diagnostic testing. The majority of disorders that can mimic CVS include non-gastrointestinal as well as gastrointestinal disorders.

Of the gastrointestinal disorders, the most serious are anatomic anomalies of the gastrointestinal tract including malrotation with intermittent volvulus, which can cause ischemic necrosis. Although not typically cyclical, we have found a few children with eosinophilic esophagitis related to significant food allergies to mimic CVS. Lucarelli et al have described seven children with a positive radio-allergosorbent test (RAST) to foods (milk, egg

Table 20.3 Characteristics of chronic and cyclic vomiting

Chronic pattern

Cyclic pattern

Time of onset

daytime

night-time or early morning

Number of emeses/h

<4 emeses

> 4 emeses

Frequency of recurrence

>2 episodes/week

< 2 episodes/week, typically

2-4 weeks

Family history of migraine

uncommon (14%)

common (82%)

Ill-appearing

no

yes (pale, lethargic)

Headaches

infrequent (19%)

frequent (41%)

Photophobia

infrequent (4%)

frequent (18%)

Vertigo

infrequent (7%)

frequent (24%)

Intravenous hydration required

uncommon (18%)

common (62%)

Esophagitis on EGD

common (59%)

uncommon (15%)

EGD, esophagogastroduodenoscopy

Table 20.4

Differential diagnosis of cyclic vomiting

Chronic pattern

Cyclic pattern

Gastrointestinal peptic injury (GERD esophagitis, gastritis, duodenitis) inflammatory bowel disease celiac disease chronic appendicitis pancreatitis eosinophilic gastroenteritis/esophagitis

anatomic (malrotation, volvulus, duplication cyst) pseudo-obstruction cholelithiasis/gallbladder dyskinesia

Infectious

chronic sinusitis giardiasis

sinusitis/other infections may be a trigger

Genitourinary

pyelonephritis, pregnancy

acute hydronephrosis due to uretopelvic junction obstruction or stones

Metabolic

rare

mitochondrial disorders (MELAS)

organic acidemias aminoacidurias fatty acid oxidation defects urea cycle defects acute intermittent porphyria

Endocrine

adrenal hyperplasia

Addison's disease diabetic ketoacidosis pheochromocytoma

Neurological

Chiari malformation subtentorial neoplasm

migraine (headaches/abdominal) abdominal epilepsy familial dysautonomia

Psychiatric

Munchausen-by-proxy pscyhogenic vomiting

Münchausen-by-proxy anorexia nervosa bulimia nervosa CVS

GERD, gastroesophageal reflux disease; CVS, cyclic vomiting syndrome

white and soy) whose episodes diminished after specific food elimination.14

The most common extraintestinal cause is acute hydronephrosis resulting from proximal or distal ureteral obstruction. Metabolic causes include mitochondrial disorders (disorders of fatty acid oxidation, mitochondrial encephalopathy, lactic acid and stroke-like syndrome), urea cycle defects (partial ornithine transcarbamylase deficiency), organic acidurias (proprionic acidemia), aminoacidurias and porphyrin degradation disorders (acute intermittent porphyria).15,16

Neurosurgical causes include various lesions of the subtentorial region including cerebellar medulloblastoma, brain stem glioma and Chiari malformation.2

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