Abstract
While achalasia is fairly common in the adult age, it is very rare in the pediatric population. In children, achalasia presents specific challenges in term of diagnosis and treatment. In fact, the rarity of achalasia and presence of symptoms that mimic common pediatric diagnoses may lead to misdiagnosis in children with this disease. In addition, in the pediatric population, there are currently no definitive guidelines for treating achalasia. The general principles of treatment are similar to those utilized in adults, although responses to specific interventions differ somewhat. The goal of this chapter is to illustrate the challenges in diagnosis and treatment of children with achalasia.
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References
Marlais M, Fishman JR, Fell JM, Haddad MJ, Rawat DJ. UK incidence of achalasia: an 11-year national epidemiological study. Arch Dis Child. 2011;96:192–4.
Walzer N, Hirano I. Achalasia. Gastroenterol Clin North Am. 2008;37:807–25, viii.
Zarate N, Mearin F, Gil-Vernet JM, Camarasa F, Malagelada JR. Achalasia and Down’s syndrome: coincidental association or something else? Am J Gastroenterol. 1999;94:1674–7.
Upadhyaya VD, Gangopadhyaya AN, Gupta DK, Sharma SP, Kumar V, Gopal SC. Esophageal achalasia of unknown etiology in infants. World J Pediatr. 2008;4:63–5.
Hallal C, Kieling CO, Nunes DL, et al. Diagnosis, misdiagnosis, and associated diseases of achalasia in children and adolescents: a twelve-year single center experience. Pediatr Surg Int. 2012;28:1211–7.
Sharp NE, St Peter SD. Treatment of idiopathic achalasia in the pediatric population: a systematic review. Eur J Pediatr Surg. 2015.
Lee CW, Kays DW, Chen MK, Islam S. Outcomes of treatment of childhood achalasia. J Pediatr Surg. 2010;45:1173–7.
Tannuri AC, Tannuri U, Velhote MC, Romao RL. Laparoscopic extended cardiomyotomy in children: an effective procedure for the treatment of esophageal achalasia. J Pediatr Surg. 2010;45:1463–6.
Boeckxstaens GE, Jonge WD, van den Wijngaard RM, Benninga MA. Achalasia: from new insights in pathophysiology to treatment. J Pediatr Gastroenterol Nutr. 2005;41 Suppl 1:S36–7.
Torab FC, Hamchou M, Ionescu G, Al-Salem AH. Familial achalasia in children. Pediatr Surg Int. 2012;28:1229–33.
Shteyer E, Edvardson S, Wynia-Smith SL, et al. Truncating mutation in the nitric oxide synthase 1 gene is associated with infantile achalasia. Gastroenterology. 2015;148:533–6.e4.
Motamed F, Modaresi V, Eftekhari K. Familial achalasia, a case report. Iran J Pediatr. 2010;20:233–6.
Zimmerman FH, Rosensweig NS. Achalasia in a father and son. Am J Gastroenterol. 1984;79:506–8.
Okawada M, Okazaki T, Yamataka A, Lane GJ, Miyano T. Down’s syndrome and esophageal achalasia: a rare but important clinical entity. Pediatr Surg Int. 2005;21:997–1000.
Rozycki DL, Ruben RJ, Rapin I, Spiro AJ. Autosomal recessive deafnessassociated with short stature, vitiligo, muscle wasting and achalasia. Arch Otolaryngol. 1971;93:194–7.
Allgrove J, Clayden GS, Grant DB, Macaulay JC. Familial glucocorticoid deficiency with achalasia of the cardia and deficient tear production. Lancet. 1978;1:1284–6.
Fernbach SK, Poznanski AK. Pediatric case of the day. Triple A syndrome: achalasia, alacrima and ACTH insensitivity. Radiographics. 1989;9:563–4.
Goswami S, Roy A, Bhattacharjee R, et al. The “Double A” phenotype: portending Allgrove’s syndrome and averting adrenal crisis. Indian J Endocrinol Metab. 2012;16:S367–8.
Li Y, Fallon SC, Helmrath MA, Gilger M, Brandt ML. Surgical treatment of infantile achalasia: a case report and literature review. Pediatr Surg Int. 2014;30:677–9.
Betalli P, Carretto E, Cananzi M, et al. Autism and esophageal achalasia in childhood: a possible correlation? report on three cases. Dis Esophagus. 2013;26:237–40.
Hussain SZ, Thomas R, Tolia V. A review of achalasia in 33 children. Dig Dis Sci. 2002;47:2538–43.
Pyun JE, da Choi M, Lee JH, Yoo KH, Shim JO. Achalasia previously diagnosed as gastroesophageal reflux disease by relying on esophageal impedance-pH monitoring: use of high-resolution esophageal manometry in children. Pediatr Gastroenterol Hepatol Nutr. 2015;18:55–9.
Hampasandra Madan Kumar HV, Fishbein A, Nevin MA. Could it be achalasia if not asthma? Am J Respir Crit Care Med. 2012;186:e1.
Akhter J, Newcomb RW. Tracheal obstruction secondary to esophageal achalasia. J Pediatr Gastroenterol Nutr. 1988;7:769–72.
Al-Abdoulsalam T, Anselmo MA. An 11-year-old male patient with refractory asthma and heartburn. Can Respir J. 2011;18:81–3.
Cools-Lartigue J, Chang SY, McKendy K, et al. Pattern of esophageal eosinophilic infiltration in patients with achalasia and response to Heller myotomy and Dor fundoplication. Dis Esophagus. 2013;26:766–75.
Dabritz J, Domagk D, Monninger M, Foell D. Achalasia mistaken as eating disorders: report of two children and review of the literature. Eur J Gastroenterol Hepatol. 2010;22:775–8.
Richterich A, Brunner R, Resch F. Achalasia mimicking prepubertal anorexia nervosa. Int J Eat Disord. 2003;33:356–9.
Lam CR. Diagnosis and surgical treatment of “H-type” tracheoesophageal fistulas. World J Surg. 1979;3:651–7.
Ng J, Bartram J, Antao B, Everard M, Shawis R. H-type tracheoesophageal fistula masquerading as achalasia cardia in a 13-year-old child. J Paediatr Child Health. 2006;42:215–6.
Boybeyi O, Kose M, Ersoz DD, Haliloglu M, Karnak I, Senocak ME. Achalasia-like findings in a case with delayed diagnosis of H-type tracheoesophageal fistula. Pediatr Surg Int. 2008;24:965–9.
Franklin AL, Petrosyan M, Kane TD. Childhood achalasia: a comprehensive review of disease, diagnosis and therapeutic management. World J Gastrointest Endosc. 2014;6:105–11.
Khan MQ, AlQaraawi A, Al-Sohaibani F, Al-Kahtani K, Al-Ashgar HI. Clinical, endoscopic, and radiologic features of three subtypes of achalasia, classified using high-resolution manometry. Saudi J Gastroenterol. 2015;21:152–7.
Zhang Y, Xu CD, Zaouche A, Cai W. Diagnosis and management of esophageal achalasia in children: analysis of 13 cases. World J Pediatr. 2009;5:56–9.
Morera C, Nurko S. Heterogeneity of lower esophageal sphincter function in children with achalasia. J Pediatr Gastroenterol Nutr. 2012;54:34–40.
Vaezi MF, Pandolfino JE, Vela MF. ACG clinical guideline: diagnosis and management of achalasia. Am J Gastroenterol. 2013;108:1238–49; quiz 50.
Goldani HA, Staiano A, Borrelli O, Thapar N, Lindley KJ. Pediatric esophageal high-resolution manometry: utility of a standardized protocol and size-adjusted pressure topography parameters. Am J Gastroenterol. 2010;105:460–7.
Singendonk MM, Smits MJ, Heijting IE, et al. Inter- and intrarater reliability of the Chicago classification in pediatric high-resolution esophageal manometry recordings. Neurogastroenterol Motil. 2015;27:269–76.
Arun S, Senthil R, Bhattacharya A, Thapa BR, Mittal BR. Incidental detection of pediatric achalasia cardia during gastroesophageal scintigraphy. Clin Nucl Med. 2013;38:228–9.
Efrati Y, Horne T, Livshitz G, Broide E, Klin B, Vinograd I. Radionuclide esophageal emptying and long-acting nitrates (Nitroderm) in childhood achalasia. J Pediatr Gastroenterol Nutr. 1996;23:312–5.
Bergami GL, Fruhwirth R, Di Mario M, Fasanelli S. Contribution of ultrasonography in the diagnosis of achalasia. J Pediatr Gastroenterol Nutr. 1992;14:92–6.
Maksimak M, Perlmutter DH, Winter HS. The use of nifedipine for the treatment of achalasia in children. J Pediatr Gastroenterol Nutr. 1986;5:883–6.
Morreau P. Achalasia. London: Springer; 2009.
Khoshoo V, LaGarde DC, Udall Jr JN. Intrasphincteric injection of Botulinum toxin for treating achalasia in children. J Pediatr Gastroenterol Nutr. 1997;24:439–41.
Storr M, Born P, Frimberger E, et al. Treatment of achalasia: the short-term response to botulinum toxin injection seems to be independent of any kind of pretreatment. BMC Gastroenterol. 2002;2:19.
Hurwitz M, Bahar RJ, Ament ME, et al. Evaluation of the use of botulinum toxin in children with achalasia. J Pediatr Gastroenterol Nutr. 2000;30:509–14.
Pastor AC, Mills J, Marcon MA, Himidan S, Kim PC. A single center 26-year experience with treatment of esophageal achalasia: is there an optimal method? J Pediatr Surg. 2009;44:1349–54.
Berquist WE, Byrne WJ, Ament ME, Fonkalsrud EW, Euler AR. Achalasia: diagnosis, management, and clinical course in 16 children. Pediatrics. 1983;71:798–805.
Nakayama DK, Shorter NA, Boyle JT, Watkins JB, O’Neill Jr JA. Pneumatic dilatation and operative treatment of achalasia in children. J Pediatr Surg. 1987;22:619–22.
Hamza AF, Awad HA, Hussein O. Cardiac achalasia in children. Dilatation or surgery? Eur J Pediatr Surg. 1999;9:299–302.
Newcomer MK, Brazer SR. Complications of upper gastrointestinal endoscopy and their management. Gastrointest Endosc Clin N Am. 1994;4:551–70.
Gershman G, Ament ME, Vargas J. Frequency and medical management of esophageal perforation after pneumatic dilatation in achalasia. J Pediatr Gastroenterol Nutr. 1997;25:548–53.
Jung C, Michaud L, Mougenot JF, et al. Treatments for pediatric achalasia: Heller myotomy or pneumatic dilatation? Gastroenterol Clin Biol. 2010;34:202–8.
Azizkhan RG, Tapper D, Eraklis A. Achalasia in childhood: a 20-year experience. J Pediatr Surg. 1980;15:452–6.
Patti MG, Albanese CT, Holcomb 3rd GW, et al. Laparoscopic Heller myotomy and Dor fundoplication for esophageal achalasia in children. J Pediatr Surg. 2001;36:1248–51.
Pachl MJ, Rex D, Decoppi P, et al. Paediatric laparoscopic Heller’s cardiomyotomy: a single centre series. J Pediatr Surg. 2014;49:289–92; discussion 92.
Rebecchi F, Giaccone C, Farinella E, Campaci R, Morino M. Randomized controlled trial of laparoscopic Heller myotomy plus Dor fundoplication versus Nissen fundoplication for achalasia: long-term results. Ann Surg. 2008;248:1023–30.
Adikibi BT, MacKinlay GA, Munro FD, Khan LR, Gillett PM. Intraoperative upper GI endoscopy ensures an adequate laparoscopic Heller’s myotomy. J Laparoendosc Adv Surg Tech A. 2009;19:687–9.
Jafri M, Alonso M, Kaul A, et al. Intraoperative manometry during laparoscopic Heller myotomy improves outcome in pediatric achalasia. J Pediatr Surg. 2008;43:66–70; discussion 70.
Corda L, Pacilli M, Clarke S, Fell JM, Rawat D, Haddad M. Laparoscopic oesophageal cardiomyotomy without fundoplication in children with achalasia: a 10-year experience: a retrospective review of the results of laparoscopic oesophageal cardiomyotomy without an anti-reflux procedure in children with achalasia. Surg Endosc. 2010;24:40–4.
Esposito C, Riccipetitoni G, Chiarenza SF, et al. Long-term results of laparoscopic treatment of esophageal achalasia in children: a multicentric survey. J Laparoendosc Adv Surg Tech A. 2013;23:955–9.
Caldaro T, Familiari P, Romeo EF, et al. Treatment of esophageal achalasia in children: today and tomorrow. J Pediatr Surg. 2015;50:726–30.
Inoue H, Minami H, Kobayashi Y, et al. Peroral endoscopic myotomy (POEM) for esophageal achalasia. Endoscopy. 2010;42:265–71.
Swanstrom LL, Kurian A, Dunst CM, Sharata A, Bhayani N, Rieder E. Long-term outcomes of an endoscopic myotomy for achalasia: the POEM procedure. Ann Surg. 2012;256:659–67.
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Shariff, F., Langer, M. (2016). Pediatric Achalasia. In: Fisichella, P., Herbella, F., Patti, M. (eds) Achalasia. Springer, Cham. https://doi.org/10.1007/978-3-319-13569-4_19
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DOI: https://doi.org/10.1007/978-3-319-13569-4_19
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