ABSTRACT: Diffuse esophageal spasm (DES) causes chest pain and/or dysphagia in adults. We reviewed charts of 278 subjects 0 to 18 years of age after esophageal manometry to describe the frequency and characteristics of DES in children. Patient diagnoses included normal motility (61%), nonspecific esophageal motility disorder (20%), DES (13%, n = 36), and achalasia (4%). Of patients with DES, the most common chief complaint was food refusal in subjects younger than 5 years (14/24, 58%) and chest pain in subjects older than 5 years (4/12, 33%). Comorbid medical conditions, often multiple, existed in 33 subjects. DES should be considered when young children present with food refusal.
*Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL
†University of Kansas, Kansas City, KS
‡Children's Mercy Hospital, Kansas City, MO
§Louisiana State University and Children's Hospital, New Orleans, LA.
Address correspondence and reprint requests to Paul E. Hyman, MD, 200 Henry Clay Ave, New Orleans, LA 70118 (e-mail: email@example.com).
Received 11 April, 2012
Accepted 22 October, 2012
The authors report no conflicts of interest.
Diffuse esophageal spasm (DES) is an esophageal motility disorder characterized by simultaneous contractions in the esophageal body. Adult patients with DES often present with chest pain and/or dysphagia (1). DES incidence is estimated to be 1 in 500,000 adults per year (2) and is found in 3% to 5% of adults assessed for esophageal motility disorders (3). The precise diagnostic criteria of DES are a subject of debate with recent studies revealing that spasms are much more frequent in the distal compared with proximal esophagus (4). Use of high-resolution manometry provides further data delineating features of esophageal spastic disorders including DES in adults (5) and children (6).
There are few prior descriptions of children with DES. A chart review of 83 children presenting with chest pain found 7 with DES (7). DES was also diagnosed in 5 adolescents ages 13 to 18 years presenting with chest pain and/or dysphagia (8). Two children with cerebral palsy, 20 and 30 months old, had a lifelong history of food aversion and retching or vomiting associated with DES (9). A 22-month old mentally retarded girl with DES had symptoms of paroxysmal posturing, irritability, and inadequate oral intake (10), and 1 newborn experienced DES-associated apnea and bradycardia (11). Our aims were to describe the frequency of DES in children undergoing esophageal manometry and to assess patterns of illness in 36 children with DES.
The present study was completed while all authors were affiliated with the University of Kansas Medical Center and was approved by the University of Kansas Human Subjects Committee. Data collection was completed according to the standards of institutional Human Subjects Committee and Health Insurance Portability and Accountability Act regulations. In this retrospective chart review, we assessed 278 subjects 0 to 18 years of age who had esophageal manometry for 10 years before (1994–2004) as part of their diagnostic evaluation. We determined the characteristics and clinical presentations of the 36 subjects meeting manometric criteria for DES. We defined DES as simultaneous esophageal body contractions associated with >10% of swallows and mean simultaneous contraction amplitude >30 mmHg (12).
Subjects with contractions >100 mmHg or prolonged duration contractions all had abnormal peristalsis with simultaneous contractions and were included in the DES group. Esophageal manometry was performed according to the standards for children (13).
We used a 4- to 8-lumen water-perfused manometry catheter with recording sites 3 or 4 cm apart, placed such that at least 2 sites recorded from the esophageal body, 1 from the lower esophageal sphincter, and 1 from the stomach. We evaluated >10 swallows (range 11–44). GraphPad Prism (GraphPad, La Jolla, CA) was used for statistical analyses. All P values were calculated by Fisher exact test. P values <0.05 were considered significant.
Of 278 subjects undergoing esophageal manometry, 61% had normal motility (n = 169). Abnormal results included nonspecific esophageal motility disorder in 20% (n = 55), DES in 13% (n = 36), and achalasia in 4% (n = 11). Two percent of subjects (n = 6) had uninterpretable studies because of lack of patient cooperation.
DES subject's age ranged from 2 months to 18 years at diagnosis (mean 5.4 years) and 67% were girls. Manometry results associated with DES included simultaneous contractions in all (represented in Fig. 1), contractions >100 mmHg in 10 of 36, and multipeaked contractions in 7 of 36. There were 6 subjects with DES with incomplete lower esophageal sphincter relaxation. Three of those subjects had a history of fundoplication. Five subjects, ranging from 2 to 8 years, had simultaneous contractions while upset, but not after calming.
The chief complaints of subjects with DES are summarized in Table 1 and stratified by age. Food refusal was more common in infants and toddlers than in children and teens (P < 0.05). There were no complaints of chest pain in subjects younger than 9 years.
Coexistent symptoms included chronic constipation (9/36), malnutrition (9/36), and irritability (5/36). Chronic vomiting occurred in 16 subjects (73%) younger than 4 years and only 4 subjects (29%) older than 4 years (P < 0.05). Chronic retching presented in 10 subjects (45%) younger than 4 years and 3 subjects (21%) older than 4 years. Chronic constipation occurred 4 times (17%) in those younger than 5 years and 5 times (42%) in those older than 5 years. Malnutrition in 9 subjects was distributed through all ages.
Of 22 subjects with antroduodenal (n = 15) or colonic manometry (n = 3) or both (n = 4), 13 were diagnosed as having more generalized motility disorders. Before manometry, 25 subjects (69%) had surgery for symptoms: 20 gastrostomies, 13 fundoplications, 3 pyloroplasties, 1 Collis gastroplasty, and 1 gastrojejunostomy. Seven subjects had no symptomatic improvement with intensive behavioral treatment by a multidisciplinary feeding team before diagnostic manometry.
Comorbid disorders complicated all but 3 subjects with DES. Comorbid conditions included developmental delay (n = 7), Down syndrome (n = 6), and cerebral palsy (n = 4). Seven subjects had structural heart abnormalities, 4 were premature, and 2 had a history of seizures. There were 3 subjects with previously diagnosed gastrointestinal neuropathies including 2 with neuropathic pseudo-obstruction and 1 with Hirschsprung disease.
In 2 subjects undergoing 24-hour esophageal manometry, oral nifedipine (0.2 mg/kg nifedipine liquid aspirated from gelcaps every 6 hours) reduced the amplitude of contractions and the number of simultaneous contractions for about 90 minutes, beginning 10 to 15 minutes after drug administration. Treatment of these 2 toddlers with nifedipine resulted in advancing nutrition support and reducing choking and food refusal. Withdrawal of nifedipine after 1 year resulted in prompt return of symptoms.
This retrospective analysis of esophageal manometry demonstrated that DES occurred in chronically ill infants and toddlers presenting with food refusal. Infants and toddlers experiencing pain or discomfort avoid repeating activities they associate with these symptoms. Food aversion has been correlated to dysphoric eating experiences in gastroesophageal reflux (14) and other upper gastrointestinal motor and sensory disorders (9). DES was not recognized in the studied infants and toddlers as they underwent unsuccessful surgical and psychological treatments for food refusal, choking, and gagging. Diagnosis of DES ended inappropriate testing and ineffective treatment trials. Appropriate evaluation of DES may reduce anxiety in patients and their parents by providing an explanation for previously unexplained symptoms.
The present study reviewed 36 examples of DES in children. We found DES in 24 infants, toddlers, and preschool children, a population rarely described with this condition. In a cohort of 278 patients ages 0 to 18 years undergoing esophageal manometry, DES was present in 13%, a rate higher than that of adults undergoing esophageal manometry. We considered whether to eliminate or include 5 subjects who had simultaneous contractions only when agitated, and only normally propagating swallows when calm. Only when these children were offered food would they become agitated and develop symptoms of gagging and choking. We speculated that the emotional distress associated with eating was associated with DES, and so the children learned to avoid eating. Therefore, treatment may be directed at treating esophageal spasm, or treating anxiety and arousal to prevent DES.
Unlike adults with DES, a majority of children with DES experienced coexisting disease. In most cases, the comorbid conditions affected the central and/or enteric nervous system. Although the children appeared to have a separate condition affecting eating and swallowing, it was likely that DES was part of a systemic neuropathy.
Abnormalities on manometry have been reported in patients with eosinophilic esophagitis (EoE) (15–17). Esophageal biopsies were not available for review in our patient cohort; however, despite reports in a small number of adults, DES has not been reported concurrently in children with EoE.
In 2 cases of DES, oral nifedipine improved manometric abnormalities and clinical symptoms without altering blood pressure. Oral nifedipine may be a useful drug to treat symptoms of DES in children despite unclear efficacy data in adult trials (18,19).
In summary, DES should be considered in children with food refusal, dysphagia, chest pain, or unexplained vomiting and may be more prevalent in the pediatric population than previously thought. Diagnosis by esophageal manometry can alleviate patient concern, direct pharmacotherapy, and may prevent unnecessary tests or surgery.
1. Richter JE, Castell DO. Diffuse esophageal spasm: a reappraisal. Ann Intern Med
2. Storr M, Allescher HD, Classen M. Current concepts on pathophysiology, diagnosis and treatment of diffuse oesophageal spasm. Drugs
3. Dekel R, Pearson T, Wendel C, et al. Assessment of oesophageal motor function in patients with dysphagia or chest pain—the Clinical Outcomes Research Initiative experience. Aliment Pharmacol Ther
4. Sperandio M, Tutuian R, Gideon RM, et al. Diffuse esophageal spasm: not diffuse but distal esophageal spasm (DES). Dig Dis Sci
5. Pandolfino JE, Roman S, Carlson D, et al. Distal esophageal spasm in highresolution esophageal pressure topography: defining clinical phenotypes. Gastroenterology
6. Goldani HA, Staiano A, Borrelli O, et al. Pediatric esophageal high-resolution manometry: utility of a standardized protocol and size-adjusted pressure topography parameters. Am J Gastroenterology
7. Glassman MS, Medow MS, Berezin S, et al. Spectrum of esophageal disorders in children with chest pain. Dig Dis Sci
8. Milov DE, Cynamon HA, Andres JM. Chest pain and dysphagia in adolescents caused by diffuse esophageal spasm. J Pediatr Gastroenterol Nutr
9. Zangen T, Ciarla C, Zangen S, et al. Gastrointestinal motility and sensory abnormalities may contribute to food refusal in medically fragile toddlers. J Pediatr Gastroenterol Nutr
10. Wyllie E, Wyllie R, Rothner AD, et al. Diffuse esophageal spasm: a cause of paroxysmal posturing and irritability in infants and mentally retarded children. J Pediatr
11. Fontan JP, Heldt GP, Heyman MB, et al. Esophageal spasm associated with apnea and bradycardia in an infant. Pediatrics
12. Spechler S, Castell D. Nonachalasia esophageal motility abnormalities. In: Castell DO, Richter JE, eds. The Esophagus
. 4th ed. Philadelphia: Lippincott Williams & Wilkins; 2004:262–274.
13. Di Lorenzo C, Hillemeier C, Hyman P, et al. Manometry studies in children: minimum standards for procedures. Neurogastroenterol Motil
14. Hyman PE. Gastroesophageal reflux: one reason why baby won’t eat. J Pediatr
1994; 125 (6 Pt 2):S103–S109.
15. Martin Martin L, Santander C, Lopez Martin MC, et al. Esophageal motor abnormalities in eosinophilic esophagitis identified by high-resolution manometry. J Gastroenterol Hepatol
16. Nurko S, Rosen R, Furuta GT. Esophageal dysmotility in children with eosinophilic esophagitis: a study using prolonged esophageal manometry. Am J Gastroenterol
17. Nurko S, Rosen R. Esophageal dysmotility in patients who have eosinophilic esophagitis. Gastrointest Endosc Clin N Am
18. Davies HA, Lewis MJ, Rhodes J, et al. Trial of nifedipine for prevention of oesophageal spasm. Digestion
19. Drenth JP, Bos LP, Engels LG. Efficacy of diltiazem in the treatment of diffuse oesophageal spasm. Aliment Pharmacol Ther