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Diagnostic value of the preoperatively detected radiological transition zone in Hirschsprung’s disease

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Abstract

Background and objective

The cardinal diagnostic sign of congenital aganglionic megacolon, or Hirschsprung’s disease (HD), is an aganglionic segment of the distal colon or rectum. To determine the surgical planning of a radiological transition zone (TZ) in HD, this study investigated the association between a radiological TZ and the bowel resection length.

Methods

A prospective observational study was conducted in children (n = 192) with suspected HD determined by radiological TZ on contrast barium enema, and who underwent pull-through operations. The bowel resection length was ≥10 cm above the proximal radiological TZ levels and confirmed by intraoperative frozen sections. In the contrast enema, the presence and level of a radiological TZ were recorded. Correlation of the TZ features with ganglion cells assessed by immunostaining of neuronal nuclei (NeuN) and the odds ratio were calculated.

Results

The sensitivity and specificity for diagnosing HD by the presence of a radiological TZ were 86.9 and 92.1%, respectively; Youden’s index was 79.0%. The positive and negative predictive values were 91.7 and 87.6%. The kappa value indicating an association between TZ and HD was 0.776 (P < 0.05). The correlation rate between a radiological TZ and the pathological results was 88.5% in the rectosigmoid colon and 44.4% in the descending colon, and was higher in children older than 3 months (85.3%) than in infants (69.0%).

Conclusion

A preoperatively determined radiological TZ has potential value to identify the length of resected bowel in patients with HD, and it also has a high predictive value for diagnosis of HD.

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Abbreviations

HD:

Hirschsprung’s disease

PPV:

Positive predictive value

NPV:

Negative predictive value

TZ:

Transition zone

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Acknowledgements

This study was supported by Grants from the National Natural Science Foundation of China (No. 81270441).

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Authors and Affiliations

  1. Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1095 Jiefang Ave, Wuhan, 430030, China

    Xuyong Chen, Wu Xiaojuan, Hongyi Zhang, Chunlei Jiao, Kechi Yu, Tianqi Zhu & Jiexiong Feng

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  1. Xuyong Chen
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  2. Wu Xiaojuan
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  3. Hongyi Zhang
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  4. Chunlei Jiao
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  5. Kechi Yu
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  6. Tianqi Zhu
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  7. Jiexiong Feng
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Correspondence to Tianqi Zhu or Jiexiong Feng.

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Chen, X., Xiaojuan, W., Zhang, H. et al. Diagnostic value of the preoperatively detected radiological transition zone in Hirschsprung’s disease. Pediatr Surg Int 33, 581–586 (2017). https://doi.org/10.1007/s00383-017-4064-9

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  • DOI: https://doi.org/10.1007/s00383-017-4064-9

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