Familial achalasia in children

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 s...

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Published inPediatric surgery international Vol. 28; no. 12; pp. 1229 - 1233
Main Authors Torab, Fawaz Chikh, Hamchou, Moustafa, Ionescu, Gabriel, Al-Salem, Ahmed H.
Format Journal Article
LanguageEnglish
Published Berlin/Heidelberg Springer-Verlag 01.12.2012
Springer Nature B.V
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Summary: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.
Bibliography:ObjectType-Case Study-2
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ISSN:0179-0358
1437-9813
DOI:10.1007/s00383-012-3186-3