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Esophageal Achalasia

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Textbook of Pediatric Gastroenterology, Hepatology and Nutrition

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Abstract

Achalasia is a rare esophageal motility disorder characterized by impaired relaxation of the lower esophageal sphincter (LES) during swallowing combined with various degrees of esophageal body motor dysfunction. It results from a progressive degeneration of the myenteric neurons which leads to defective LES relaxation and ultimately aperistalsis of the esophageal body. Dysphagia, for solid and liquid, vomiting, chest pain, regurgitation of undigested food, cough, and weight loss are the cardinal symptoms of the disease. The diagnosis is based on a combination of imaging studies (upper gastrointestinal (GI) contrast series), esophagogastroduodenoscopy (EGD), and esophageal manometry which ultimately confirms the presence of impaired LES relaxation and abnormalities of esophageal body peristalsis. The management of childhood achalasia includes a range of options such as pharmacologic therapy, endoscopic botulinum toxin injection (EBTI) into the LES, pneumatic dilatation (PD), and surgery (myotomy of the LES, also known as Heller myotomy (HM), with or without an associated antireflux procedure). None of the abovementioned therapies are curative but focus on symptomatic relief by reducing the LES pressure. With the implementation of modern interventional/surgical therapeutic modalities, the majority of children achieve adequate long-lasting improvement of symptoms. Long-term surveillance with endoscopy although not advocated, it may be indicated in certain cases particularly in adulthood, as there is a risk of esophageal cancer.

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References

  1. Willis T. Pharmaceutice rationalis sive diatriba do medicamentorum operationibus in humano corpore. London: Hagae Comitis; 1674.

    Google Scholar 

  2. Hurst AF, Rowlands RP. Case of achalasia of the cardia relieved by operation. Proc R Soc Med. 1924;17:45–6.

    PubMed Central  CAS  PubMed  Google Scholar 

  3. Kun L, Herbella FA, Dubecz A. 1913: annus mirabilis of esophageal surgery. Thorac Cardiovasc Surg. 2013;61:460–3.

    Article  PubMed  Google Scholar 

  4. Vaezi MF, Pandolfino JE, Vela MF. ACG clinical guideline: diagnosis and management of achalasia. Am J Gastroenterol. 2013;108:1238–49, quiz 50.

    Article  PubMed  Google Scholar 

  5. Ghoshal UC, Daschakraborty SB, Singh R. Pathogenesis of achalasia cardia. World J Gastroenterol. 2012;18:3050–7.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  6. Azizkhan RG, Tapper D, Eraklis A. Achalasia in childhood: a 20-year experience. J Pediatr Surg. 1980;15:452–6.

    Article  CAS  PubMed  Google Scholar 

  7. Mayberry JF, Mayell MJ. Epidemiological study of achalasia in children. Gut. 1988;29:90–3.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  8. Marlais M, Fishman JR, Fell JM, et al. UK incidence of achalasia: an 11-year national epidemiological study. Arch Dis Child. 2011;96:192–4.

    Article  CAS  PubMed  Google Scholar 

  9. Myers NA, Jolley SG, Taylor R. Achalasia of the cardia in children: a worldwide survey. J Pediatr Surg. 1994;29:1375–9.

    Article  CAS  PubMed  Google Scholar 

  10. Vaezi MF, Richter JE. Diagnosis and management of achalasia. American college of gastroenterology practice parameter committee. Am J Gastroenterol. 1999;94:3406–12.

    Article  CAS  PubMed  Google Scholar 

  11. Francis DL, Katzka DA. Achalasia: update on the disease and its treatment. Gastroenterology. 2010;139:369–74.

    Article  PubMed  Google Scholar 

  12. Dayalan N, Chettur L, Ramakrishnan MS. Achalasia of the cardia in sibs. Arch Dis Child. 1972;47:115–8.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  13. Hernandez A, Reynoso MC, Soto F, et al. Achalasia microcephaly syndrome in a patient with consanguineous parents: support for a.m. being a distinct autosomal recessive condition. Clin Genet. 1989;36:456–8.

    Article  CAS  PubMed  Google Scholar 

  14. Mayberry JF, Atkinson M. A study of swallowing difficulties in first degree relatives of patients with achalasia. Thorax. 1985;40:391–3.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  15. Bohl J, Gockel I, Sultanov F, et al. Childhood achalasia: a separate entity? Z Gastroenterol. 2007;45:1273–80.

    Article  CAS  PubMed  Google Scholar 

  16. Park W, Vaezi MF. Etiology and pathogenesis of achalasia: the current understanding. Am J Gastroenterol. 2005;100:1404–14.

    Article  PubMed  Google Scholar 

  17. Crist J, Gidda JS, Goyal RK. Intramural mechanism of esophageal peristalsis: roles of cholinergic and noncholinergic nerves. Proc Natl Acad Sci U S A. 1984;81:3595–9.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  18. Sivarao DV, Mashimo HL, Thatte HS, et al. Lower esophageal sphincter is achalasic in nNOS(−/−) and hypotensive in W/W(v) mutant mice. Gastroenterology. 2001;121:34–42.

    Article  CAS  PubMed  Google Scholar 

  19. Goyal RK, Chaudhury A. Physiology of normal esophageal motility. J Clin Gastroenterol. 2008;42:610–9.

    Article  PubMed Central  PubMed  Google Scholar 

  20. Goldblum JR, Rice TW, Richter JE. Histopathologic features in esophagomyotomy specimens from patients with achalasia. Gastroenterology. 1996;111:648–54.

    Article  CAS  PubMed  Google Scholar 

  21. Csendes A, Smok G, Braghetto I, et al. Gastroesophageal sphincter pressure and histological changes in distal esophagus in patients with achalasia of the esophagus. Dig Dis Sci. 1985;30:941–5.

    Article  CAS  PubMed  Google Scholar 

  22. Csendes A, Smok G, Braghetto I, et al. Histological studies of Auerbach’s plexuses of the oesophagus, stomach, jejunum, and colon in patients with achalasia of the oesophagus: correlation with gastric acid secretion, presence of parietal cells and gastric emptying of solids. Gut. 1992;33:150–4.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  23. Wattchow DA, Costa M. Distribution of peptide-containing nerve fibres in achalasia of the oesophagus. J Gastroenterol Hepatol. 1996;11:478–85.

    Article  CAS  PubMed  Google Scholar 

  24. Cassella RR, Brown AL Jr, Sayre GP, et al. Achalasia of the esophagus: pathologic and etiologic considerations. Ann Surg. 1964;160:474–87.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  25. Khajanchee YS, VanAndel R, Jobe BA, et al. Electrical stimulation of the vagus nerve restores motility in an animal model of achalasia. J Gastrointest Surg. 2003;7:843–9, discussion 9.

    Article  PubMed  Google Scholar 

  26. Qualman SJ, Haupt HM, Yang P, et al. Esophageal Lewy bodies associated with ganglion cell loss in achalasia. Similarity to Parkinson's disease. Gastroenterology. 1984;87:848–56.

    CAS  PubMed  Google Scholar 

  27. Eckardt VF, Krause J, Bolle D. Gastrointestinal transit and gastric acid secretion in patients with achalasia. Dig Dis Sci. 1989;34:665–71.

    Article  CAS  PubMed  Google Scholar 

  28. Atkinson M, Ogilvie AL, Robertson CS, et al. Vagal function in achalasia of the cardia. Q J Med. 1987;63:297–303.

    CAS  PubMed  Google Scholar 

  29. Kahrilas PJ, Boeckxstaens G. The spectrum of achalasia: lessons from studies of pathophysiology and high-resolution manometry. Gastroenterology. 2013;145:954–65.

    Article  PubMed  Google Scholar 

  30. Jones DB, Mayberry JF, Rhodes J, et al. Preliminary report of an association between measles virus and achalasia. J Clin Pathol. 1983;36:655–7.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  31. Robertson CS, Martin BA, Atkinson M. Varicella-zoster virus DNA in the oesophageal myenteric plexus in achalasia. Gut. 1993;34:299–302.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  32. Niwamoto H, Okamoto E, Fujimoto J, et al. Are human herpes viruses or measles virus associated with esophageal achalasia? Dig Dis Sci. 1995;40:859–64.

    Article  CAS  PubMed  Google Scholar 

  33. Birgisson S, Galinski MS, Goldblum JR, et al. Achalasia is not associated with measles or known herpes and human papilloma viruses. Dig Dis Sci. 1997;42:300–6.

    Article  CAS  PubMed  Google Scholar 

  34. Dantas RO, Alves LM, Nascimento WV. Effect of bolus volume on proximal esophageal contractions of patients with Chagas’ disease and patients with idiopathic achalasia. Dis Esophagus. 2010;23:670–4.

    Article  CAS  PubMed  Google Scholar 

  35. Clark SB, Rice TW, Tubbs RR, et al. The nature of the myenteric infiltrate in achalasia: an immunohistochemical analysis. Am J Surg Pathol. 2000;24:1153–8.

    Article  CAS  PubMed  Google Scholar 

  36. Raymond L, Lach B, Shamji FM. Inflammatory aetiology of primary oesophageal achalasia: an immunohistochemical and ultrastructural study of Auerbach’s plexus. Histopathology. 1999;35:445–53.

    Article  CAS  PubMed  Google Scholar 

  37. Verne GN, Hahn AB, Pineau BC, et al. Association of HLA-DR and -DQ alleles with idiopathic achalasia. Gastroenterology. 1999;117:26–31.

    Article  CAS  PubMed  Google Scholar 

  38. Wong RK, Maydonovitch CL, Metz SJ, et al. Significant DQw1 association in achalasia. Dig Dis Sci. 1989;34:349–52.

    Article  CAS  PubMed  Google Scholar 

  39. Latiano A, De Giorgio R, Volta U, et al. HLA and enteric antineuronal antibodies in patients with achalasia. Neurogastroenterol Motil. 2006;18:520–5.

    Article  CAS  PubMed  Google Scholar 

  40. Goin JC, Sterin-Borda L, Bilder CR, et al. Functional implications of circulating muscarinic cholinergic receptor autoantibodies in chagasic patients with achalasia. Gastroenterology. 1999;117:798–805.

    Article  CAS  PubMed  Google Scholar 

  41. Zilberstein B, de Cleva R, Gabriel AG, et al. Congenital achalasia: facts and fantasies. Dis Esophagus. 2005;18:335–7.

    Article  CAS  PubMed  Google Scholar 

  42. Rao PS, Vijaykumar Rao PL. Achalasia in siblings in infancy. Indian J Pediatr. 2001;68:887–8.

    Article  CAS  PubMed  Google Scholar 

  43. Senocak ME, Hicsonmez A, Buyukpamukcu N. Familial childhood achalasia. Z Kinderchir. 1990;45:111–3.

    CAS  PubMed  Google Scholar 

  44. Tryhus MR, Davis M, Griffith JK, et al. Familial achalasia in two siblings: significance of possible hereditary role. J Pediatr Surg. 1989;24:292–5.

    Article  CAS  PubMed  Google Scholar 

  45. Di Nardo G, Tullio-Pelet A, Annese V, et al. Idiopathic achalasia is not allelic to alacrima achalasia adrenal insufficiency syndrome at the ALADIN locus. Dig Liver Dis. 2005;37:312–5.

    Article  CAS  PubMed  Google Scholar 

  46. Jung KW, Yoon IJ, Kim do H, et al. Genetic evaluation of ALADIN gene in early-onset achalasia and alacrima patients. J Neurogastroenterol Motil. 2011;17:169–73.

    Article  PubMed Central  PubMed  Google Scholar 

  47. Nihoul-Fekete C, Bawab F, Lortat-Jacob S, et al. Achalasia of the esophagus in childhood. Surgical treatment in 35 cases, with special reference to familial cases and glucocorticoid deficiency association. Hepatogastroenterol. 1991;38:510–3.

    CAS  Google Scholar 

  48. Johnston BT, Colcher A, Li Q, et al. Repetitive proximal esophageal contractions: a new manometric finding and a possible further link between Parkinson’s disease and achalasia. Dysphagia. 2001;16:186–9.

    Article  CAS  PubMed  Google Scholar 

  49. Okawada M, Okazaki T, Yamataka A, et al. Down's syndrome and esophageal achalasia: a rare but important clinical entity. Pediatr Surg Int. 2005;21:997–1000.

    Article  PubMed  Google Scholar 

  50. De Giorgio R, Di Simone MP, Stanghellini V, et al. Esophageal and gastric nitric oxide synthesizing innervation in primary achalasia. Am J Gastroenterol. 1999;94:2357–62.

    Article  CAS  PubMed  Google Scholar 

  51. de Leon AR, de la Serna JP, Santiago JL, et al. Association between idiopathic achalasia and IL23R gene. Neurogastroenterol Motil. 2010;22:734–8, e218.

    Article  CAS  PubMed  Google Scholar 

  52. Nunez C, Garcia-Gonzalez MA, Santiago JL, et al. Association of IL10 promoter polymorphisms with idiopathic achalasia. Hum Immunol. 2011;72:749–52.

    Article  CAS  PubMed  Google Scholar 

  53. Santiago JL, Martinez A, Benito MS, et al. Gender-specific association of the PTPN22 C1858T polymorphism with achalasia. Hum Immunol. 2007;68:867–70.

    Article  CAS  PubMed  Google Scholar 

  54. Paladini F, Cocco E, Cascino I, et al. Age-dependent association of idiopathic achalasia with vasoactive intestinal peptide receptor 1 gene. Neurogastroenterol Motil. 2009;21:597–602.

    Article  CAS  PubMed  Google Scholar 

  55. Gupta V, Lal A, Sinha SK, et al. Leiomyomatosis of the esophagus: experience over a decade. J Gastrointest Surg. 2009;13:206–11.

    Article  PubMed  Google Scholar 

  56. Gockel I, Eckardt VF, Schmitt T, et al. Pseudoachalasia: a case series and analysis of the literature. Scand J Gastroenterol. 2005;40:378–85.

    Article  PubMed  Google Scholar 

  57. Herbella FA, Oliveira DR, Del Grande JC. Are idiopathic and chagasic achalasia two different diseases? Dig Dis Sci. 2004;49:353–60.

    Article  PubMed  Google Scholar 

  58. Lukens FJ, Machicao VI, Woodward TA, et al. Esophageal sarcoidosis: an unusual diagnosis. J Clin Gastroenterol. 2002;34:54–6.

    Article  PubMed  Google Scholar 

  59. Kelly JL, Mulcahy TM, O'Riordain DS, et al. Coexistent Hirschsprung’s disease and esophageal achalasia in male siblings. J Pediatr Surg. 1997;32:1809–11.

    Article  CAS  PubMed  Google Scholar 

  60. Taketomi T, Yoshiga D, Taniguchi K, et al. Loss of mammalian Sprouty2 leads to enteric neuronal hyperplasia and esophageal achalasia. Nat Neurosci. 2005;8:855–7.

    Article  CAS  PubMed  Google Scholar 

  61. Assor P, Negreanu L, Picon L, et al. Slowly regressing acute pandysautonomia associated with esophageal achalasia: a case report. Gastroenterol Clin Biol. 2008;32:46–50.

    Article  CAS  PubMed  Google Scholar 

  62. Murphy MS, Gardner-Medwin D, Eastham EJ. Achalasia of the cardia associated with hereditary cerebellar ataxia. Am J Gastroenterol. 1989;84:1329–30.

    CAS  PubMed  Google Scholar 

  63. Buyukpamukcu M, Buyukpamukcu N, Cevik N. Achalasia of the oesophagus associated with Hodgkin’s disease in children. Clin Oncol. 1982;8:73–6.

    CAS  PubMed  Google Scholar 

  64. Ghosh P, Linder J, Gallagher TF, et al. Achalasia of the cardia and multiple endocrine neoplasia 2B. Am J Gastroenterol. 1994;89:1880–3.

    CAS  PubMed  Google Scholar 

  65. Mandaliya R, DiMarino AJ, Cohen S. Association of achalasia and eosinophilic esophagitis. Indian J Gastroenterol. 2013;32:54–7.

    Article  PubMed  Google Scholar 

  66. Simila S, Kokkonen J, Kaski M. Achalasia sicca–juvenile Sjogren’s syndrome with achalasia and gastric hyposecretion. Eur J Pediatr. 1978;129:175–81.

    Article  CAS  PubMed  Google Scholar 

  67. Jung C, Michaud L, Mougenot JF, et al. Treatments for pediatric achalasia: heller myotomy or pneumatic dilatation? Gastroenterol Clin Biol. 2010;34:202–8.

    Article  CAS  PubMed  Google Scholar 

  68. 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.

    Article  CAS  PubMed  Google Scholar 

  69. Hussain SZ, Thomas R, Tolia V. A review of achalasia in 33 children. Dig Dis Sci. 2002;47:2538–43.

    Article  CAS  PubMed  Google Scholar 

  70. Shettihalli N, Venugopalan V, Ives NK, et al. Achalasia cardia in a premature infant. BMJ Case Rep. 2010. doi:10.1136/bcr. 05.2010.3014.

    Google Scholar 

  71. Polk HC Jr, Burford TH. Disorders of the distal esophagus in infancy and childhood. Am J Dis Child. 1964;108:243–51.

    PubMed  Google Scholar 

  72. Nurko S. Other motor disorders. In: Walker W, Durie P, Hamilton J, editors. Pediatric gastrointestinal disease, pathophysiology, diagnosis and management, vol. 1, 3rd edn. St Louis: Mosby; 2000. p. 317–50.

    Google Scholar 

  73. Rudolph CD, Sood MR. Achalasia and other motor disorders. In: Wyllie R, Hyams JS, Kay M, editors. Pediatric gastrointestinal and liver disease. Philadelphia: Elsevier; 2011. p. 248–54.

    Google Scholar 

  74. Rosseneu S, Afzal N, Yerushalmi B, et al. Topical application of mitomycin-C in oesophageal strictures. J Pediatr Gastroenterol Nutr. 2007;44:336–41.

    Article  CAS  PubMed  Google Scholar 

  75. Sorser SA, Barawi M, Hagglund K, et al. Eosinophilic esophagitis in children and adolescents: epidemiology, clinical presentation and seasonal variation. J Gastroenterol. 2013;48:81–5.

    Article  PubMed  Google Scholar 

  76. Katzka DA, Smyrk TC, Chial HJ, et al. Esophageal leiomyomatosis presenting as achalasia diagnosed by high-resolution manometry and endoscopic core biopsy. Gastrointest Endosc. 2012;76:216–7.

    Article  PubMed  Google Scholar 

  77. Kessing BF, Smout AJ, Bredenoord AJ. Clinical applications of esophageal impedance monitoring and high-resolution manometry. Curr Gastroenterol Rep. 2012;14:197–205.

    Article  PubMed Central  PubMed  Google Scholar 

  78. MacKalski BA, Keate RF. Rumination in a patient with achalasia. Am J Gastroenterol. 1993;88:1803–4.

    CAS  PubMed  Google Scholar 

  79. 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.

    Article  PubMed  Google Scholar 

  80. Desseilles M, Fuchs S, Ansseau M, et al. Achalasia may mimic anorexia nervosa, compulsive eating disorder, and obesity problems. Psychosomatics. 2006;47:270–1.

    Article  PubMed  Google Scholar 

  81. Vicentine FP, Herbella FA, Allaix ME, et al. Comparison of idiopathic achalasia and Chagas' disease esophagopathy at the light of high-resolution manometry. Dis Esophagus. 2014;27:128–33

    Google Scholar 

  82. Bode CP, Schroten H, Koletzko S, et al. Transient achalasia-like esophageal motility disorder after candida esophagitis in a boy with chronic granulomatous disease. J Pediatr Gastroenterol Nutr. 1996;23:320–3.

    Article  CAS  PubMed  Google Scholar 

  83. Howard PJ, Maher L, Pryde A, et al. Five year prospective study of the incidence, clinical features, and diagnosis of achalasia in Edinburgh. Gut. 1992;33:1011–5.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  84. Goldenberg SP, Burrell M, Fette GG, et al. Classic and vigorous achalasia: a comparison of manometric, radiographic, and clinical findings. Gastroenterology. 1991;101:743–8.

    CAS  PubMed  Google Scholar 

  85. Camacho-Lobato L, Katz PO, Eveland J, et al. Vigorous achalasia: original description requires minor change. J Clin Gastroenterol. 2001;33:375–7.

    Article  CAS  PubMed  Google Scholar 

  86. Hansford BG, Mitchell MT, Gasparaitis A. Water flush technique: a noninvasive method of optimizing visualization of the distal esophagus in patients with primary achalasia. AJR Am J Roentgenol. 2013;200:818–21.

    Article  PubMed  Google Scholar 

  87. Levine MS, Rubesin SE, Laufer I. Barium esophagography: a study for all seasons. Clin Gastroenterol Hepatol. 2008;6:11–25.

    Article  PubMed  Google Scholar 

  88. Miller DL, Allen MS, Trastek VF, et al. Esophageal resection for recurrent achalasia. Ann Thorac Surg. 1995;60:922–5, discussion 5–6.

    Article  CAS  PubMed  Google Scholar 

  89. Devaney EJ, Lannettoni MD, Orringer MB, et al. Esophagectomy for achalasia: patient selection and clinical experience. Ann Thorac Surg. 2001;72:854–8.

    Article  CAS  PubMed  Google Scholar 

  90. Spitz L. Gastric transposition for esophageal substitution in children. J Pediatr Surg. 1992;27:252–7, discussion 7–9.

    Article  CAS  PubMed  Google Scholar 

  91. Peters JH, Kauer WK, Crookes PF, et al. Esophageal resection with colon interposition for end-stage achalasia. Arch Surg. 1995;130:632–6, discussion 6–7.

    Article  CAS  PubMed  Google Scholar 

  92. Orringer MB, Stirling MC. Esophageal resection for achalasia: indications and results. Ann Thorac Surg. 1989;47:340–5.

    Article  CAS  PubMed  Google Scholar 

  93. Gockel I, Junginger T, Eckardt VF. Effects of pneumatic dilation and myotomy on esophageal function and morphology in patients with achalasia. Am Surg. 2005;71:128–31.

    PubMed  Google Scholar 

  94. Neyaz Z, Gupta M, Ghoshal UC. How to perform and interpret timed barium esophagogram. J Neurogastroenterol Motil. 2013;19:251–6.

    Article  PubMed Central  PubMed  Google Scholar 

  95. Gheorghe C, Bancila I, Tutuian R, et al. Predictors of short term treatment outcome in patients with achalasia following endoscopic or surgical therapy. Hepatogastroenterol. 2012;59:2503–7.

    Google Scholar 

  96. Vaezi MF, Baker ME, Achkar E, et al. Timed barium oesophagram: better predictor of long term success after pneumatic dilation in achalasia than symptom assessment. Gut. 2002;50:765–70.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  97. Andersson M, Lundell L, Kostic S, et al. Evaluation of the response to treatment in patients with idiopathic achalasia by the timed barium esophagogram: results from a randomized clinical trial. Dis Esophagus. 2009;22:264–73.

    Article  CAS  PubMed  Google Scholar 

  98. Pasha SF, Acosta RD, Chandrasekhara V, et al. The role of endoscopy in the evaluation and management of dysphagia. Gastrointest Endosc. 2014;79:191–201.

    Google Scholar 

  99. O'Neill OM, Johnston BT, Coleman HG. Achalasia: a review of clinical diagnosis, epidemiology, treatment and outcomes. World J Gastroenterol. 2013;19:5806–12.

    Article  PubMed Central  PubMed  Google Scholar 

  100. Savarino E, Gemignani L, Zentilin P, et al. Achalasia with dense eosinophilic infiltrate responds to steroid therapy. Clin Gastroenterol Hepatol. 2011;9:1104–6.

    Article  PubMed  Google Scholar 

  101. 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.

    Article  CAS  PubMed  Google Scholar 

  102. Nurko S, Rosen R. Esophageal dysmotility in patients who have eosinophilic esophagitis. Gastrointest Endosc Clin N Am. 2008;18:73–89, ix.

    Article  PubMed Central  PubMed  Google Scholar 

  103. Roman S, Hirano I, Kwiatek MA, et al. Manometric features of eosinophilic esophagitis in esophageal pressure topography. Neurogastroenterol Motil. 2011;23:208–14, e111.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  104. 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 Gastroenterol. 2010;105:460–7.

    Article  PubMed  Google Scholar 

  105. Staiano A, Boccia G, Miele E, et al. Segmental characteristics of oesophageal peristalsis in paediatric patients. Neurogastroenterol Motil. 2008;20:19–26.

    CAS  PubMed  Google Scholar 

  106. Rohof WO, Myers JC, Estremera FA, et al. Inter- and intra-rater reproducibility of automated and integrated pressure-flow analysis of esophageal pressure-impedance recordings. Neurogastroenterol Motil. 2014;26:168–75.

    Google Scholar 

  107. Bredenoord AJ, Fox M, Kahrilas PJ, et al. Chicago classification criteria of esophageal motility disorders defined in high resolution esophageal pressure topography. Neurogastroenterol Motil. 2012;24 Suppl 1:57–65.

    Article  PubMed Central  PubMed  Google Scholar 

  108. Rohof WO, Salvador R, Annese V, et al. Outcomes of treatment for achalasia depend on manometric subtype. Gastroenterology. 2013;144:718–25, quiz e13–4.

    Article  PubMed  Google Scholar 

  109. Scherer JR, Kwiatek MA, Soper NJ, et al. Functional esophagogastric junction obstruction with intact peristalsis: a heterogeneous syndrome sometimes akin to achalasia. J Gastrointest Surg. 2009;13:2219–25.

    Article  PubMed Central  PubMed  Google Scholar 

  110. 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.

    Article  PubMed  Google Scholar 

  111. Perretta S, Dallemagne B, McMahon B, et al. Video. Improving functional esophageal surgery with a “smart” bougie: endoflip. Surg Endosc. 2011;25:3109.

    Article  PubMed  Google Scholar 

  112. Rohof WO, Hirsch DP, Kessing BF, et al. Efficacy of treatment for patients with achalasia depends on the distensibility of the esophagogastric junction. Gastroenterology. 2012;143:328–35.

    Article  PubMed  Google Scholar 

  113. Rieder E, Swanstrom LL, Perretta S, et al. Intraoperative assessment of esophagogastric junction distensibility during per oral endoscopic myotomy (POEM) for esophageal motility disorders. Surg Endosc. 2013;27:400–5.

    Article  PubMed  Google Scholar 

  114. Yoneyama F, Miyachi M, Nimura Y. Manometric findings of the upper esophageal sphincter in esophageal achalasia. World J Surg. 1998;22:1043–6, discussion 6–7.

    Article  CAS  PubMed  Google Scholar 

  115. Dudnick RS, Castell JA, Castell DO. Abnormal upper esophageal sphincter function in achalasia. Am J Gastroenterol. 1992;87:1712–5.

    CAS  PubMed  Google Scholar 

  116. Tatum RP, Wong JA, Figueredo EJ, et al. Return of esophageal function after treatment for achalasia as determined by impedance-manometry. J Gastrointest Surg. 2007;11:1403–9.

    Article  PubMed  Google Scholar 

  117. Vaezi MF, Richter JE. Current therapies for achalasia: comparison and efficacy. J Clin Gastroenterol. 1998;27:21–35.

    Article  CAS  PubMed  Google Scholar 

  118. Bortolotti M, Coccia G, Brunelli F, et al. Isosorbide dinitrate or nifedipine: which is preferable in the medical therapy of achalasia? Ital J Gastroenterol. 1994;26:379–82.

    CAS  PubMed  Google Scholar 

  119. 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.

    Article  CAS  PubMed  Google Scholar 

  120. Khoshoo V, LaGarde DC, Udall JN Jr. Intrasphincteric injection of Botulinum toxin for treating achalasia in children. J Pediatr Gastroenterol Nutr. 1997;24:439–41.

    Article  CAS  PubMed  Google Scholar 

  121. 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.

    Article  CAS  PubMed  Google Scholar 

  122. Emami MH, Raisi M, Amini J, et al. Pneumatic balloon dilation therapy is as effective as esophagomyotomy for achalasia. Dysphagia. 2008;23:155–60.

    Article  PubMed  Google Scholar 

  123. Di Nardo G, Rossi P, Oliva S, et al. Pneumatic balloon dilation in pediatric achalasia: efficacy and factors predicting outcome at a single tertiary pediatric gastroenterology center. Gastrointest Endosc. 2012;76:927–32.

    Article  PubMed  Google Scholar 

  124. Pastor AC, Mills J, Marcon MA, et al. A single center 26-year experience with treatment of esophageal achalasia: is there an optimal method? J Pediatr Surg. 2009;44:1349–54.

    Article  PubMed  Google Scholar 

  125. Lee CW, Kays DW, Chen MK, et al. Outcomes of treatment of childhood achalasia. J Pediatr Surg. 2010;45:1173–7.

    Article  PubMed Central  PubMed  Google Scholar 

  126. Fan Y, Song HY, Kim JH, et al. Fluoroscopically guided balloon dilation of benign esophageal strictures: incidence of esophageal rupture and its management in 589 patients. AJR Am J Roentgenol. 2011;197:1481–6.

    Article  PubMed  Google Scholar 

  127. Adams H, Roberts GM, Smith PM. Oesophageal tears during pneumatic balloon dilatation for the treatment of achalasia. Clin Radiol. 1989;40:53–7.

    Article  CAS  PubMed  Google Scholar 

  128. Miller RE, Tiszenkel HI. Esophageal perforation due to pneumatic dilation for achalasia. Surg Gynecol Obstet. 1988;166:458–60.

    CAS  PubMed  Google Scholar 

  129. Zhang Y, Xu CD, Zaouche A, et al. Diagnosis and management of esophageal achalasia in children: analysis of 13 cases. World J Pediatr. 2009;5:56–9.

    Article  PubMed  Google Scholar 

  130. Askegard-Giesmann JR, Grams JM, Hanna AM, et al. Minimally invasive Heller’s myotomy in children: safe and effective. J Pediatr Surg. 2009;44:909–11.

    Article  PubMed  Google Scholar 

  131. Heller E. Extramukose kardioplastic bein chronischen kardiospasmus mit dilitation des oesophagus. Mitt Grenzgeb Med Chir. 1914;27:141–9.

    Google Scholar 

  132. Ali A, Pellegrini CA. Laparoscopic myotomy: technique and efficacy in treating achalasia. Gastrointest Endosc Clin N Am. 2001;11:347–58, vii.

    CAS  PubMed  Google Scholar 

  133. Vaos G, Demetriou L, Velaoras C, et al. Evaluating long-term results of modified Heller limited esophagomyotomy in children with esophageal achalasia. J Pediatr Surg. 2008;43:1262–9.

    Article  PubMed  Google Scholar 

  134. Ellis FH Jr, Crozier RE, Watkins E Jr. Operation for esophageal achalasia. Results of esophagomyotomy without an antireflux operation. J Thorac Cardiovasc Surg. 1984;88:344–51.

    PubMed  Google Scholar 

  135. Donahue PE, Samelson S, Schlesinger PK, et al. Achalasia of the esophagus. Treatment controversies and the method of choice. Ann Surg. 1986;203:505–11.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  136. Roberts KE, Duffy AJ, Bell RL. Controversies in the treatment of gastroesophageal reflux and achalasia. World J Gastroenterol. 2006;12:3155–61.

    PubMed Central  PubMed  Google Scholar 

  137. Illi OE, Stauffer UG. Achalasia in childhood and adolescence. Eur J Pediatr Surg. 1994;4:214–7.

    Article  CAS  PubMed  Google Scholar 

  138. Buick RG, Spitz L. Achalasia of the cardia in children. Br J Surg. 1985;72:341–3.

    Article  CAS  PubMed  Google Scholar 

  139. Molena D, Yang SC. Surgical management of end-stage achalasia. Semin Thorac Cardiovasc Surg. 2012;24:19–26.

    Article  PubMed  Google Scholar 

  140. Inoue H, Minami H, Kobayashi Y, et al. Peroral endoscopic myotomy (POEM) for esophageal achalasia. Endoscopy. 2010;42:265–71.

    Article  CAS  PubMed  Google Scholar 

  141. Von Renteln D, Fuchs KH, Fockens P, et al. Peroral endoscopic myotomy for the treatment of achalasia: an international prospective multicenter study. Gastroenterology. 2013;145:309–11, e1–3.

    Article  PubMed  Google Scholar 

  142. Familiari P, Marchese M, Gigante G, et al. Peroral endoscopic myotomy for the treatment of achalasia in children. J Pediatr Gastroenterol Nutr. 2013;57:794–7.

    Article  PubMed  Google Scholar 

  143. Maselli R, Inoue H, Misawa M, et al. Peroral endoscopic myotomy (POEM) in a 3-year-old girl with severe growth retardation, achalasia, and down syndrome. Endoscopy. 2012;44 Suppl 2:E285–7.

    PubMed  Google Scholar 

  144. Li YD, Cheng YS, Li MH, et al. Temporary self-expanding metallic stents and pneumatic dilation for the treatment of achalasia: a prospective study with a long-term follow-up. Dis Esophagus. 2010;23:361–7.

    Article  PubMed  Google Scholar 

  145. Zeng Y, Dai YM, Wan XJ. Clinical remission following endoscopic placement of retrievable, fully covered metal stents in patients with esophageal achalasia. Dis Esophagus. 2014;27:103–8.

    Google Scholar 

  146. Eckardt VF, Aignherr C, Bernhard G. Predictors of outcome in patients with achalasia treated by pneumatic dilation. Gastroenterology. 1992;103:1732–8.

    CAS  PubMed  Google Scholar 

  147. Eckardt VF, Hoischen T, Bernhard G. Life expectancy, complications, and causes of death in patients with achalasia: results of a 33-year follow-up investigation. Eur J Gastroenterol Hepatol. 2008;20:956–60.

    Article  PubMed  Google Scholar 

  148. Leeuwenburgh I, Scholten P, Alderliesten J, et al. Long-term esophageal cancer risk in patients with primary achalasia: a prospective study. Am J Gastroenterol. 2010;105:2144–9.

    Article  CAS  PubMed  Google Scholar 

  149. Sandler RS, Nyren O, Ekbom A, et al. The risk of esophageal cancer in patients with achalasia. A population-based study. JAMA. 1995;274:1359–62.

    Article  CAS  PubMed  Google Scholar 

  150. Hirota WK, Zuckerman MJ, Adler DG, et al. ASGE guideline: the role of endoscopy in the surveillance of premalignant conditions of the upper GI tract. Gastrointest Endosc. 2006;63:570–80.

    Article  PubMed  Google Scholar 

  151. Eckardt AJ, Eckardt VF. Editorial: cancer surveillance in achalasia: better late than never? Am J Gastroenterol. 2010;105:2150–2.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of Neurogastroenterology and Motility, Department of Pediatric Gastroenterology, Great Ormond Street Hospital for Children, NHS Foundation Trust; UCL Institute of Child Health, 30 Guilford Street, WC1N 3JH, London, UK

    Efstratios Saliakellis, Keith J. Lindley & Osvaldo Borrelli

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  1. Efstratios Saliakellis
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  2. Keith J. Lindley
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  3. Osvaldo Borrelli
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Correspondence to Osvaldo Borrelli .

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  1. Department of Pediatrics, University of Chicago, Chicago, Illinois, USA

    Stefano Guandalini

  2. Clinical Director Child Health King’s College Hospital, Professor and Director Pediatric Liver, GI and Nutrition Center, London, United Kingdom

    Anil Dhawan

  3. Department of Pediatrics, Hebrew University Hadassah Hospital, Jerusalem, Israel

    David Branski

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Saliakellis, E., Lindley, K., Borrelli, O. (2016). Esophageal Achalasia. In: Guandalini, S., Dhawan, A., Branski, D. (eds) Textbook of Pediatric Gastroenterology, Hepatology and Nutrition. Springer, Cham. https://doi.org/10.1007/978-3-319-17169-2_11

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