<p>Congenital jejunoileal atresia is a rare intestinal malformation with favourable survival in high-income settings, but affected infants remain at risk of prolonged intestinal failure and dependence on parenteral nutrition (PN). Data on nutritional outcomes by anatomical subtype remain limited. We aimed to quantify the risk of discharge with home PN according to the type of congenital jejunoileal atresia and to compare neonatal outcomes across anatomical subtypes. We conducted a single-centre retrospective cohort study including infants with congenital jejunoileal atresia managed between 2013 and 2022. Atresia was classified according to the Grosfeld classification and grouped as simple (types I–IIIa), multiple (type IV), or apple-peel (type IIIb). Sixty-four children were included. The most common type of atresia was single (<i>n</i> = 42, 66%), followed by multiple (<i>n</i> = 13, 20%) and apple-peel atresia (<i>n</i> = 9, 14%). Two infants died during hospitalisation. Compared with simple atresia, multiple (OR [95% CI] = 20 [2.0 – 203]) and apple-peel atresia (OR [95% CI] = 32 [3.0 – 345]) were associated with a significantly increased risk of home PN. These associations remained robust after excluding syndromic cases. Cholestasis increased with anatomical severity.</p><p><i>Conclusion:&#xa0;</i>Anatomical complexity is a major determinant of nutritional prognosis in congenital jejunoileal atresia. The type of atresia strongly predicts the risk of discharge with home PN, with a clear severity gradient from simple to multiple and apple-peel atresia. These findings support early parental counselling, optimized discharge planning and long-term follow-up strategies.<Table Float="No" ID="Taba"> <tgroup cols="2"> <colspec align="left" colname="c1" colnum="1" /> <colspec align="left" colname="c2" colnum="2" /> <tbody> <row> <entry align="left" nameend="c2" namest="c1"> <p><b>What is Known:</b></p> </entry> </row> <row> <entry align="left" nameend="c2" namest="c1"> <p>• <i>Congenital jejunoileal atresia has a good overall survival but is frequently complicated by intestinal failure requiring parenteral nutrition.</i></p> </entry> </row> <row> <entry align="left" nameend="c2" namest="c1"> <p>• <i>The influence of anatomical subtype on postoperative nutritional outcomes remains insufficiently characterized.</i></p> </entry> </row> <row> <entry align="left" nameend="c2" namest="c1"> <p><b>What is New:</b></p> </entry> </row> <row> <entry align="left" nameend="c2" namest="c1"> <p>• <i>Apple-peel and multiple atresia are associated with a substantially higher risk of home parenteral nutrition at discharge compared with simple atresia.</i></p> </entry> </row> <row> <entry align="left" nameend="c2" namest="c1"> <p>• <i>There is a clear stepwise increase in nutritional risk according to anatomical complexity, making subtype a key prognostic factor.</i></p> </entry> </row> </tbody> </tgroup> </Table></p>

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The anatomical type of atresia drives the risk of home parenteral nutrition of infants with congenital jejunoileal atresia

  • Gwénaëlle Bultez,
  • Victor Sartorius,
  • Nicolas Vinit,
  • Claire Bellanger,
  • Benjamin Pariente,
  • Véronique Rousseau,
  • Elsa Kermorvant-Duchemin,
  • Alexandre Lapillonne

摘要

Congenital jejunoileal atresia is a rare intestinal malformation with favourable survival in high-income settings, but affected infants remain at risk of prolonged intestinal failure and dependence on parenteral nutrition (PN). Data on nutritional outcomes by anatomical subtype remain limited. We aimed to quantify the risk of discharge with home PN according to the type of congenital jejunoileal atresia and to compare neonatal outcomes across anatomical subtypes. We conducted a single-centre retrospective cohort study including infants with congenital jejunoileal atresia managed between 2013 and 2022. Atresia was classified according to the Grosfeld classification and grouped as simple (types I–IIIa), multiple (type IV), or apple-peel (type IIIb). Sixty-four children were included. The most common type of atresia was single (n = 42, 66%), followed by multiple (n = 13, 20%) and apple-peel atresia (n = 9, 14%). Two infants died during hospitalisation. Compared with simple atresia, multiple (OR [95% CI] = 20 [2.0 – 203]) and apple-peel atresia (OR [95% CI] = 32 [3.0 – 345]) were associated with a significantly increased risk of home PN. These associations remained robust after excluding syndromic cases. Cholestasis increased with anatomical severity.

Conclusion: Anatomical complexity is a major determinant of nutritional prognosis in congenital jejunoileal atresia. The type of atresia strongly predicts the risk of discharge with home PN, with a clear severity gradient from simple to multiple and apple-peel atresia. These findings support early parental counselling, optimized discharge planning and long-term follow-up strategies.

What is Known:

Congenital jejunoileal atresia has a good overall survival but is frequently complicated by intestinal failure requiring parenteral nutrition.

The influence of anatomical subtype on postoperative nutritional outcomes remains insufficiently characterized.

What is New:

Apple-peel and multiple atresia are associated with a substantially higher risk of home parenteral nutrition at discharge compared with simple atresia.

There is a clear stepwise increase in nutritional risk according to anatomical complexity, making subtype a key prognostic factor.