Top

Published in:

01-12-2012 | Original Article

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

Authors: Cristiane Hallal, Carlos O. Kieling, Daltro L. Nunes, Cristina T. Ferreira, Guilherme Peterson, Sérgio G. S. Barros, Cristina A. Arruda, José C. Fraga, Helena A. S. Goldani

Published in: Pediatric Surgery International | Issue 12/2012

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.

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

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

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):192–194PubMedCrossRef

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

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

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 32:103–106PubMedCrossRef

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 15:167–170PubMedCrossRef

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

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):1262–1269PubMedCrossRef

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):1349–1354PubMedCrossRef

11.

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

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

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):1463–1466PubMedCrossRef

14.

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

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):56–59PubMedCrossRef

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):775–778PubMedCrossRef

17.

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

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):1060–1063PubMedCrossRef

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):271–274PubMedCrossRef

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):1323–1328PubMedCrossRef

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):1718–1724PubMed

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):997–1000PubMedCrossRef

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 122:1258–1263PubMedCrossRef

24.

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

25.

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

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):909–911PubMedCrossRef

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):338–341PubMedCrossRef

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):40–44PubMedCrossRef

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 17:1650–1652PubMedCrossRef

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):883–886PubMedCrossRef

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 43:66–70PubMedCrossRef

Title: Diagnosis, misdiagnosis, and associated diseases of achalasia in children and adolescents: a twelve-year single center experience
Authors: Cristiane Hallal
Carlos O. Kieling
Daltro L. Nunes
Cristina T. Ferreira
Guilherme Peterson
Sérgio G. S. Barros
Cristina A. Arruda
José C. Fraga
Helena A. S. Goldani
Publication date: 01-12-2012
Publisher: Springer-Verlag
Published in: Pediatric Surgery International / Issue 12/2012
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI: https://doi.org/10.1007/s00383-012-3214-3

Keynote webinar | Spotlight on medication adherence

Springer Medicine

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

Abstract

Purpose

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusion

Please log in to get access to this content

Other articles of this Issue 12/2012

Comparing beneficial effects of inhaled nitric oxide to l-arginine in necrotizing enterocolitis model in neonatal rats

Perinatal neuroblastoma of the pancreas

Laparoscopy-assisted surgery for neonatal intestinal atresia and stenosis: a report of 35 cases

Implantable versus cuffed external central venous catheters for the management of children and adolescents with acute lymphoblastic leukaemia

Laparoscopy versus ultrasonography for the evaluation of Mullerian structures in children with complex disorders of sex development

Re: Laparoscopy-assisted percutaneous endoscopic gastrostomy using a “Funada-kit II”device by Takahashi T, Miyano G, Shiyanagi S, Lane GJ, Yamataka A [Pediatr Surg Int 2012 Sep 1 (Epub ahead of print)]