TY - JOUR
T1 - Internal anal sphincter achalasia
T2 - data from a nationwide survey of allied disorders of Hirschsprung’s disease in Japan
AU - Obata, Satoshi
AU - Fukahori, Suguru
AU - Yagi, Minoru
AU - Suzuki, Makoto
AU - Ueno, Shigeru
AU - Ushijima, Kosuke
AU - Taguchi, Tomoaki
N1 - Funding Information:
This study was supported by a Grant from The Ministry of Health, Labour and Welfare of Japan [Health and Labour Sciences Research Grants for Research on Intractable Diseases (H23-042, H24-037, and H26-045)]. We thank all members of The Japanese Society of Pediatric Surgeons, The Japanese Society of Pediatric Nutrition, Gastroenterology, and Hepatology, and The Japanese Study Group of Pediatric Constipation.
Publisher Copyright:
© 2017, Springer Japan.
PY - 2017/12/1
Y1 - 2017/12/1
N2 - Purpose: To investigate the incidence and treatment of internal anal sphincter achalasia (IASA) in Japan based on an analysis of data from a nationwide retrospective cohort study of the allied disorders of Hirschsprung’s disease. Methods: Five cases of definitive IASA were collected from a nationwide retrospective cohort study conducted from 2001 to 2010 and a search of the Japanese literature. Results: Symptoms developed during the neonatal period in two patients, during early childhood in two, and at school age in one. Symptoms included abdominal distension with severe constipation (n = 4) and enterocolitis (n = 1). Rectocolonography showed megarectum and no narrow segment in most of the patients. All patients were negative for rectosphincteric reflex. The presence of ganglion cells was demonstrated by H&E or AChE staining from rectal mucosal biopsies or resected full-thickness segments. Two patients were treated conservatively, and three were treated surgically by internal anal sphincter myotomy (n = 2) or Lynn procedure (n = 1), with satisfactory outcomes. Conclusion: IASA is a rare but distinct entity in Japan. Although the clinical features of IASA resemble those of short- and ultrashort-segment HD, characteristic pathological findings include the presence of ganglion cells. The outcomes of both conservative and surgical treatment are good.
AB - Purpose: To investigate the incidence and treatment of internal anal sphincter achalasia (IASA) in Japan based on an analysis of data from a nationwide retrospective cohort study of the allied disorders of Hirschsprung’s disease. Methods: Five cases of definitive IASA were collected from a nationwide retrospective cohort study conducted from 2001 to 2010 and a search of the Japanese literature. Results: Symptoms developed during the neonatal period in two patients, during early childhood in two, and at school age in one. Symptoms included abdominal distension with severe constipation (n = 4) and enterocolitis (n = 1). Rectocolonography showed megarectum and no narrow segment in most of the patients. All patients were negative for rectosphincteric reflex. The presence of ganglion cells was demonstrated by H&E or AChE staining from rectal mucosal biopsies or resected full-thickness segments. Two patients were treated conservatively, and three were treated surgically by internal anal sphincter myotomy (n = 2) or Lynn procedure (n = 1), with satisfactory outcomes. Conclusion: IASA is a rare but distinct entity in Japan. Although the clinical features of IASA resemble those of short- and ultrashort-segment HD, characteristic pathological findings include the presence of ganglion cells. The outcomes of both conservative and surgical treatment are good.
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U2 - 10.1007/s00595-017-1532-8
DO - 10.1007/s00595-017-1532-8
M3 - Article
C2 - 28455798
AN - SCOPUS:85018259282
SN - 0941-1291
VL - 47
SP - 1429
EP - 1433
JO - Surgery today
JF - Surgery today
IS - 12
ER -