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Familial achalasia in children

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Abstract

Achalasia is rare in the pediatric age group and in most cases it is idiopathic with no family history. Familial achalasia is very rare. This report describes two families with achalasia: in one, six children were affected while in the other a brother and a sister had Allgrove’s syndrome (triple-A syndrome consisting of achalasia, adrenal insufficiency, and alacrima). Familial achalasia suggests that it is hereditary and may be transmitted as an autosomal recessive trait. The management of achalasia in children is still controversial. With the recent advances in minimal invasive surgery, laparoscopic Heller’s myotomy is the procedure of choice in the management of achalasia in children.

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Authors and Affiliations

  1. Division of Pediatric Surgery, Department of Surgery, Tawam Hospital, Al-Ain, Abu Dhabi, United Arab Emirates

    Fawaz Chikh Torab, Moustafa Hamchou & Gabriel Ionescu

  2. Department of Pediatric Surgery, Maternity and Children Hospital, Dammam, Saudi Arabia

    Ahmed H. Al-Salem

Authors
  1. Fawaz Chikh Torab
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  2. Moustafa Hamchou
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  3. Gabriel Ionescu
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  4. Ahmed H. Al-Salem
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Correspondence to Ahmed H. Al-Salem.

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Torab, F.C., Hamchou, M., Ionescu, G. et al. Familial achalasia in children. Pediatr Surg Int 28, 1229–1233 (2012). https://doi.org/10.1007/s00383-012-3186-3

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  • DOI: https://doi.org/10.1007/s00383-012-3186-3

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