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Diagnosis, misdiagnosis, and associated diseases of achalasia in children and adolescents: a twelve-year single center experience

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Abstract

Purpose

Although achalasia is a rare disorder in children, its symptom may mimic common childhood diseases. This study aimed to assess the diagnosis and management of achalasia in children and adolescents in a Brazilian single center during a 12-year period.

Methods

Patients with achalasia were identified from a database built during the period of January 2000–January 2012 from a Pediatric Gastroenterology reference center. Information regarding demographic data, clinical symptoms, diagnosis, treatment, and long-term follow-up were described.

Results

Thirteen patients were studied; median age was 7 (1–14) years. Most frequent symptoms were vomiting (84.6 %) and dysphagia (69.2 %). Weight loss occurred in 46.0 % of patients and chronic cough in 46.1 %. Associated disorders were Down’s syndrome, Allgrove syndrome, and congenital central hypoventilation syndrome. Achalasia was misdiagnosed with anorexia nervosa. Six patients were previously treated as having gastroesophageal reflux disease and asthma. Five patients had pneumatic balloon dilation as initial therapy whereas five had esophageal myotomy. Finally, 11 patients had surgical therapy with a favorable follow-up.

Conclusion

Achalasia symptoms may mimic common diseases in children, and therefore, may delay the diagnosis. This study emphasizes the importance of the clinical symptoms for the diagnosis of achalasia, mainly in those cases with associated disorders.

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References

  1. Berquist WE, Byrne WJ, Ament ME, Fonkalsrud EW, Euler AR (1983) Achalasia: diagnosis, Management, and Clinical Course in 16 Children. Pediatrics 71:798–805

    PubMed  CAS  Google Scholar 

  2. Patient Care Committee, Society for Surgery of the Alimentary Tract (2004) Esophageal achalasia. J Gastroint Surg 8(3):367–368

    Article  Google Scholar 

  3. Marlais M, Fishman JR, Fell JME, Haddad MJ, Rawat DJ (2010) UK incidence of achalasia: an 11-year national epidemiological study. Arch Dis Child 96(2):192–194

    Article  PubMed  Google Scholar 

  4. Walzer N, Hirano I (2008) Achalasia. Gastroenterol Clin North Am 37(4):807–825

    Article  PubMed  Google Scholar 

  5. Hussain SZ, Thomas R, Tolia V (2002) A Review of Achalasia in 33 Children. Dig Dis Sci 47:2538–2543

    Article  PubMed  CAS  Google Scholar 

  6. Aichbichler BW, Eherer AJ, Petritsch W, Hinterleitner TA, Krejs GJ (2001) Gastric Adenocarcinoma Mimicking Achalasia in a 15-Year-Old Patient: a Case Report and Review of the Literature. J Pediatr Gastroenterol Nutr 32:103–106

    Article  PubMed  CAS  Google Scholar 

  7. Khan AA, Shah SW, Alam A, Butt AK, Shafqat F (2002) Efficacy of rigiflex ballon dilatation in 12 children with achalasia: a 6-month prospective study showing weight gain and symptomatic improvement. Dis Esophagus 15:167–170

    Article  PubMed  CAS  Google Scholar 

  8. Upadhyaya M, Fataar S, Sajwany MJ (2002) Achalasia of the cardia: experience with hydrostatic balloon dilatation in children. Pediatr Radiol 32(6):409–412

    Article  PubMed  Google Scholar 

  9. Vaos G, Demetriou L, Velaoras C, Skondras C (2008) Evaluating long-term results of modified Heller limited esophagomyotomy in children with esophageal achalasia. J Pediatr Surg 43(7):1262–1269

    Article  PubMed  Google Scholar 

  10. Pastor AC, Mills J, Marcon MA, Himidan S, Kim PC (2009) A single center 26-year experience with treatment of esophageal achalasia: is there an optimal method? J Pediatr Surg 44(7):1349–1354

    Article  PubMed  Google Scholar 

  11. Babu R, Grier D, Cusick E, Spicer RD (2001) Pneumatic dilatation for childhood achalasia. Pediatr Surg Int 17:505–507

    Article  PubMed  CAS  Google Scholar 

  12. Di Nardo G, Rossi P, Oliva S, Aloi M, Cozzi DA, Frediani S, Redler A, Mallardo S, Ferrari F, Cucchiara S (2012) Pneumatic balloon dilation in pediatric achalasia: efficacy and factors predicting outcome at a single tertiary pediatric gastroenterology center. Gastrointest Endosc 76(5):927–932

    Google Scholar 

  13. Tannuri AC, Tannuri U, Velhote MC, Romão RL (2010) Laparoscopic extended cardiomyotomy in children: an effective procedure for the treatment of esophageal achalasia. J Pediatr Surg 45(7):1463–1466

    Article  PubMed  Google Scholar 

  14. Eckardt VF, Köhne U, Junginger T, Westermeier T (1997) Risk factors for diagnostic delay in achalasia. Dig Dis Sci 42(3):580–585

    Article  PubMed  CAS  Google Scholar 

  15. Zhang Y, Xu CD, Zaouche A, Cai W (2009) Diagnosis and management of esophageal achalasia in children: analysis of 13 cases. World J Pediatr 5(1):56–59

    Article  PubMed  Google Scholar 

  16. Däbritz J, Domagk D, Monninger M, Foell D (2010) Achalasia mistaken as eating disorders: report of two children and review of the literature. Eur J Gastroenterol Hepatol 22(7):775–778

    Article  PubMed  Google Scholar 

  17. Lee CW, Kays DW, Chen MK, Islam S (2010) Outcomes of treatment of childhood achalasia. J Pediatr Surg 45(6):1173–1177

    Article  PubMed  Google Scholar 

  18. Nihoul-Fékété C, Bawab F, Lortat-Jacob S, Arhan P, Pellerin D (1989) Achalasia of the Esophagus in Childhood: surgical Treatment in 35 Cases With Special Reference to Familial Cases and Glucocorticoid Deficiency Association. J Pediatr Surg 24(10):1060–1063

    Article  PubMed  Google Scholar 

  19. Alhussaini B, Gottrand F, Goutet JM, Scaillon M, Michaud L, Spyckerelle C, Viola S, Lamblin MD (2011) Clinical and Manometric Characteristics of Allgrove Syndrome. J Pediatr Gastroenterol Nutr 53(3):271–274

    Article  PubMed  Google Scholar 

  20. Milenkovic T, Zdravkovic D, Savic N, Todorovic S, Mitrovic K, Koehler K, Huebner A (2010) Triple A syndrome: 32 years experience of a single centre (1977–2008). Eur J Pediatr 169(11):1323–1328

    Article  PubMed  Google Scholar 

  21. Zárate N, Mearin F, Hidalgo A, Malagelada JR (2001) Prospective Evaluation of Esophageal Motor Dysfunction in Down’s Syndrome. Am J Gastroenterol 96(6):1718–1724

    PubMed  Google Scholar 

  22. Okawada M, Okazaki T, Yamataka A, Lane GJ, Miyano T (2005) Down’s syndrome and esophageal achalasia: a rare but important clinical entity. Pediatr Surg Int 21(12):997–1000

    Article  PubMed  Google Scholar 

  23. Faure C, Viarme F, Cargill G, Navarro J, Gaultier C, Trang H (2002) Abnormal Esophageal Motility in Children With Central Hypoventilation Syndrome Congenital. Gastroenterology 122:1258–1263

    Article  PubMed  Google Scholar 

  24. Nurko S, Rosen R (2008) Esophageal Dysmotility in Patients Who Have Eosinophilic Esophagitis. Gastrointest Endosc Clin N Am 18(1):73–89

    Article  PubMed  Google Scholar 

  25. Eckardt VF, Gockel I, Bernhard G (2004) Pneumatic dilation for achalasia: late results of a prospective follow up investigation. Gut 53(5):629–633

    Article  PubMed  CAS  Google Scholar 

  26. Askegard-Giesmann JR, Grams JM, Hanna AM, Iqbal CW, Teh S, Moir CR (2009) Minimally invasive Heller’s myotomy in children: safe and effective. J Pediatr Surg 44(5):909–911

    Article  PubMed  Google Scholar 

  27. Lelli JL Jr, Drongowski RA, Coran AG (1997) Efficacy of the transthoracic Modified Heller Myotomy in children with Achalasia–A 21-Year Experience. J Pediatr Surg 32(2):338–341

    Article  PubMed  Google Scholar 

  28. Corda L, Pacilli M, Clarke S, Fell JM, Rawat D, Haddad M (2009) Laparoscopic oesophageal cardiomyotomy without fundoplication in children with achalasia: a 10-year experience. Surg Endosc 24(1):40–44

    Article  PubMed  Google Scholar 

  29. Mattioli G, Esposito C, Pini Prato A, Doldo P, Castagnetti M, Barabino A, Gandullia P, Staiano AM, Settimi A, Cucchiara S, Montobbio G, Jasonni V (2003) Results of the laparoscopic Heller-Dor procedure for pediatric esophageal achalasia. Surg Endosc 17:1650–1652

    Article  PubMed  CAS  Google Scholar 

  30. Maksimak M, Perlmutter DH, Winter HS (1986) The Use of Nifedipine for the Treatment of Achalasia in Children. J Pediatr Gastroenterol Nutr 5(6):883–886

    Article  PubMed  CAS  Google Scholar 

  31. Jafri M, Alonso M, Kaul A, Dierig J, Racadio J, Inge T, Brown R, Ryckman F, Tiao G (2008) Intraoperative manometry during laparoscopic Heller myotomy improves outcome in pediatric achalasia. J Pediatr Surg 43:66–70

    Article  PubMed  Google Scholar 

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Conflict of interest

All authors declare no conflict of interest.

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Authors and Affiliations

  1. Post-Graduate Program Sciences in Gastroenterology, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil

    Cristiane Hallal, Carlos O. Kieling, Sérgio G. S. Barros & Helena A. S. Goldani

  2. Pediatric Gastroenterology Unit, Rua Ramiro Barcelos 2350, Porto Alegre, RS, CEP 90035-903, Brazil

    Cristiane Hallal, Carlos O. Kieling, Daltro L. Nunes, Cristina T. Ferreira & Helena A. S. Goldani

  3. Pediatric Surgery Service, Faculdade de Medicina, Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil

    Guilherme Peterson & José C. Fraga

  4. Gastroenterology Service, Faculdade de Medicina, Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil

    Sérgio G. S. Barros, Cristina A. Arruda & Helena A. S. Goldani

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  1. Cristiane Hallal
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  2. Carlos O. Kieling
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Correspondence to Cristiane Hallal.

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Hallal, C., Kieling, C.O., Nunes, D.L. et al. Diagnosis, misdiagnosis, and associated diseases of achalasia in children and adolescents: a twelve-year single center experience. Pediatr Surg Int 28, 1211–1217 (2012). https://doi.org/10.1007/s00383-012-3214-3

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  • DOI: https://doi.org/10.1007/s00383-012-3214-3

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