{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T00:38:55Z","timestamp":1747183135942,"version":"3.40.5"},"reference-count":32,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2020,6,2]],"date-time":"2020-06-02T00:00:00Z","timestamp":1591056000000},"content-version":"vor","delay-in-days":1,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["ANZ Journal of Surgery"],"published-print":{"date-parts":[[2020,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The aim of this study was to report the contemporary management of Hirschsprung disease (HD) in New Zealand.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We undertook a national multi\u2010centre retrospective review of all newly diagnosed cases of HD during a 16\u2010year period (2000\u20132015). Demographics, genetic and syndromic associations, family history, radiology and histology results and surgical interventions were analysed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 246 cases (males:females 4:1) were identified, an incidence of 1:3870 live births. Short\u2010segment disease was present in 81.7%, long\u2010segment disease in 8.5%, total colonic aganglionosis in 6.5% and unknown in 3.3%. HD was diagnosed by 4\u2009weeks' corrected gestational age in 67%. Thirty cases (12%) also had Trisomy 21. Fifty\u2010three (21.5%) patients required a repeat rectal biopsy for definitive diagnosis. A contrast enema was performed in 55% and identified the transition zone with 69% accuracy. Primary pull\u2010through surgery was undertaken in 59% (65% of short\u2010segment cases) at a median age of 27\u2009days; others were initially managed by a defunctioning stoma. The commonest definitive procedure was a Soave\u2010Boley endorectal pull\u2010through (79%) (or similar variant). During a median follow\u2010up of 7.4\u2009years, six (2.5%) survivors underwent a redo pull\u2010through, 13 (5.5%) an appendicostomy, 16 (6.8%) a defunctioning stoma and 10 never had a definitive procedure. Total colonic aganglionosis was significantly more likely to be fatal (12.5% versus 0.5%, <jats:italic>P<\/jats:italic> &lt;\u20090.0005) or associated with a permanent end stoma (27.5% versus 4.5%, <jats:italic>P<\/jats:italic> &lt;\u20090.0005).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Most New Zealand born infants with short\u2010segment HD are currently managed by primary pull\u2010through, usually in the first months of life.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ans.15923","type":"journal-article","created":{"date-parts":[[2020,6,2]],"date-time":"2020-06-02T07:43:28Z","timestamp":1591083808000},"page":"1037-1040","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Contemporary management of Hirschsprung disease in New Zealand"],"prefix":"10.1111","volume":"90","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1837-9966","authenticated-orcid":false,"given":"Kiarash","family":"Taghavi","sequence":"first","affiliation":[{"name":"Department of Paediatric Surgery and Urology Starship Children's Health  Auckland New Zealand"},{"name":"Department of Surgery University of Auckland  Auckland New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lucy","family":"Goddard","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery and Urology Starship Children's Health  Auckland New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephen M.","family":"Evans","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery and Urology Starship Children's Health  Auckland New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrew","family":"Hobson","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery Christchurch Hospital  Christchurch New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7923-7064","authenticated-orcid":false,"given":"Spencer W.","family":"Beasley","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery Christchurch Hospital  Christchurch New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sasikumar","family":"Sankaran","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery Waikato Hospital  Waikato New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Askar","family":"Kukkady","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery Waikato Hospital  Waikato New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jonathan","family":"Stevenson","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery Wellington Children's Hospital  Wellington New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark D.","family":"Stringer","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery Wellington Children's Hospital  Wellington New Zealand"},{"name":"Department of Paediatrics and Child Health University of Otago  Wellington New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,6,2]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2005.07.053"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-002-0842-z"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.jp.7210997"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild-2016-311872"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4471-4721-3_40"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-018-04432-7"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.mpg.0000214164.90939.92"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1542\/neo.19-10-e577"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2009.02.050"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2004.09.052"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0036-1593991"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/ans.14857"},{"key":"e_1_2_6_14_1","first-page":"568","article-title":"A genetic study of Hirschsprung disease","volume":"46","author":"Badner JA","year":"1990","journal-title":"Am. J. Hum. Genet."},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-002-0934-9"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0038-1675771"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0038-1667040"},{"key":"e_1_2_6_18_1","first-page":"100","article-title":"Effectiveness of rectal suction biopsy in diagnosing Hirschsprung disease","volume":"26","author":"Keyzer\u2010Dekker CM","year":"2016","journal-title":"Eur. J. Pediatr. Surg."},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-015-3742-8"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/s003830050264"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2016.09.027"},{"key":"e_1_2_6_22_1","first-page":"207","article-title":"Contrast enema for Hirschsprung disease investigation: diagnostic accuracy and validity for subsequent diagnostic and surgical planning","volume":"26","author":"Frongia G","year":"2016","journal-title":"Eur. J. Pediatr. Surg."},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/jpsu.2003.50165"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-017-4064-9"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2015.03.019"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00247-004-1292-7"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0031-1271735"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-013-3361-1"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-008-2188-7"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1038\/ejhg.2012.94"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-012-3117-3"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-017-4065-8"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.sempedsurg.2017.09.008"}],"container-title":["ANZ Journal of Surgery"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fans.15923","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ans.15923","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/ans.15923","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ans.15923","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,6]],"date-time":"2023-09-06T11:32:19Z","timestamp":1693999939000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ans.15923"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,6]]},"references-count":32,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2020,6]]}},"alternative-id":["10.1111\/ans.15923"],"URL":"https:\/\/doi.org\/10.1111\/ans.15923","archive":["Portico"],"relation":{},"ISSN":["1445-1433","1445-2197"],"issn-type":[{"type":"print","value":"1445-1433"},{"type":"electronic","value":"1445-2197"}],"subject":[],"published":{"date-parts":[[2020,6]]},"assertion":[{"value":"2020-01-08","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-04-01","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-06-02","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}