Contemporary management of Hirschsprung disease in New Zealand
© 2020 Royal Australasian College of Surgeons..
BACKGROUND: The aim of this study was to report the contemporary management of Hirschsprung disease (HD) in New Zealand.
METHODS: We undertook a national multi-centre retrospective review of all newly diagnosed cases of HD during a 16-year period (2000-2015). Demographics, genetic and syndromic associations, family history, radiology and histology results and surgical interventions were analysed.
RESULTS: A total of 246 cases (males:females 4:1) were identified, an incidence of 1:3870 live births. Short-segment disease was present in 81.7%, long-segment disease in 8.5%, total colonic aganglionosis in 6.5% and unknown in 3.3%. HD was diagnosed by 4 weeks' corrected gestational age in 67%. Thirty cases (12%) also had Trisomy 21. Fifty-three (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-through surgery was undertaken in 59% (65% of short-segment cases) at a median age of 27 days; others were initially managed by a defunctioning stoma. The commonest definitive procedure was a Soave-Boley endorectal pull-through (79%) (or similar variant). During a median follow-up of 7.4 years, six (2.5%) survivors underwent a redo pull-through, 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%, P < 0.0005) or associated with a permanent end stoma (27.5% versus 4.5%, P < 0.0005).
CONCLUSIONS: Most New Zealand born infants with short-segment HD are currently managed by primary pull-through, usually in the first months of life.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2020 |
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| Erschienen: |
2020 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:90 |
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| Enthalten in: |
ANZ journal of surgery - 90(2020), 6 vom: 03. Juni, Seite 1037-1040 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Taghavi, Kiarash [VerfasserIn] |
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| Links: |
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| Themen: |
Contrast enema |
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| Anmerkungen: |
Date Completed 29.04.2021 Date Revised 29.04.2021 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1111/ans.15923 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM310642183 |
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| 520 | |a © 2020 Royal Australasian College of Surgeons. | ||
| 520 | |a BACKGROUND: The aim of this study was to report the contemporary management of Hirschsprung disease (HD) in New Zealand | ||
| 520 | |a METHODS: We undertook a national multi-centre retrospective review of all newly diagnosed cases of HD during a 16-year period (2000-2015). Demographics, genetic and syndromic associations, family history, radiology and histology results and surgical interventions were analysed | ||
| 520 | |a RESULTS: A total of 246 cases (males:females 4:1) were identified, an incidence of 1:3870 live births. Short-segment disease was present in 81.7%, long-segment disease in 8.5%, total colonic aganglionosis in 6.5% and unknown in 3.3%. HD was diagnosed by 4 weeks' corrected gestational age in 67%. Thirty cases (12%) also had Trisomy 21. Fifty-three (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-through surgery was undertaken in 59% (65% of short-segment cases) at a median age of 27 days; others were initially managed by a defunctioning stoma. The commonest definitive procedure was a Soave-Boley endorectal pull-through (79%) (or similar variant). During a median follow-up of 7.4 years, six (2.5%) survivors underwent a redo pull-through, 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%, P < 0.0005) or associated with a permanent end stoma (27.5% versus 4.5%, P < 0.0005) | ||
| 520 | |a CONCLUSIONS: Most New Zealand born infants with short-segment HD are currently managed by primary pull-through, usually in the first months of life | ||
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| 700 | 1 | |a Kukkady, Askar |e verfasserin |4 aut | |
| 700 | 1 | |a Stevenson, Jonathan |e verfasserin |4 aut | |
| 700 | 1 | |a Stringer, Mark D |e verfasserin |4 aut | |
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