Journal of Pediatric Gastroenterology and Nutrition

Original Article: Gastroenterology

Short and Long-Term Outcomes in Hirschsprung Disease: Are the Syndrome-Associated Patients Really Doing Worse?

Corresponding Author

Hugo Gagnon MD

[email protected]

Department of Pediatrics, Centre Mère-Enfant Soleil du Centre Hospitalier de l'Université Laval, Quebec, Canada

Address correspondence and reprint requests to Hugo Gagnon, MD, Department of Pediatrics, Centre Mère-Enfant Soleil du Centre Hospitalier de l'Université Laval, Quebec, Canada G1V 4G2 (e-mail: [email protected]).Search for more papers by this author

Sophie Duguay MD,

Sophie Duguay MD

Department of Pediatrics, Centre Mère-Enfant Soleil du Centre Hospitalier de l'Université Laval, Quebec, Canada

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Pascale Prasil MD, FRCSC,

Pascale Prasil MD, FRCSC

Department of Pediatrics, Centre Mère-Enfant Soleil du Centre Hospitalier de l'Université Laval, Quebec, Canada

Department of Surgery, Centre Mère-Enfant Soleil du Centre Hospitalier de l'Université Laval, Quebec, Canada

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Julie Castilloux MD, FRCPC,

Julie Castilloux MD, FRCPC

Department of Pediatrics, Centre Mère-Enfant Soleil du Centre Hospitalier de l'Université Laval, Quebec, Canada

Department of Pediatric Gastro-Enterology, Centre Mère-Enfant Soleil du Centre Hospitalier de l'Université Laval, Quebec, Canada

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Hugo Gagnon MD,

Corresponding Author

Hugo Gagnon MD

[email protected]

Department of Pediatrics, Centre Mère-Enfant Soleil du Centre Hospitalier de l'Université Laval, Quebec, Canada

Sophie Duguay MD,

Sophie Duguay MD

Department of Pediatrics, Centre Mère-Enfant Soleil du Centre Hospitalier de l'Université Laval, Quebec, Canada

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Pascale Prasil MD, FRCSC,

Pascale Prasil MD, FRCSC

Department of Pediatrics, Centre Mère-Enfant Soleil du Centre Hospitalier de l'Université Laval, Quebec, Canada

Department of Surgery, Centre Mère-Enfant Soleil du Centre Hospitalier de l'Université Laval, Quebec, Canada

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Julie Castilloux MD, FRCPC,

Julie Castilloux MD, FRCPC

Department of Pediatrics, Centre Mère-Enfant Soleil du Centre Hospitalier de l'Université Laval, Quebec, Canada

Department of Pediatric Gastro-Enterology, Centre Mère-Enfant Soleil du Centre Hospitalier de l'Université Laval, Quebec, Canada

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First published: 27 July 2023

https://doi.org/10.1097/MPG.0000000000003896

Citations: 1

The authors report no conflicts of interest.

This article has been developed as a Journal CME and MOC Part II Activity by NASPGHAN. Visit https://learnonline.naspghan.org/ to view instructions, documentation, and the complete necessary steps to receive CME and MOC credits for reading this article.

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Abstract

Introduction:

Given the lack of data to help caregivers in the follow-up of Hirschsprung disease (HD), this study aimed to compare the functional outcomes of isolated Hirschsprung disease (I-HD) to syndrome-associated Hirschsprung disease (SA-HD) at 1, 3, 5, and 10 years.

Methods:

A retrospective chart review of patients diagnosed with HD between January 1990 and May 2021 at our pediatric center was performed to collect data on patient characteristics, investigations, and treatments. Ninety-five patients were identified, of whom 76 were included in the study. SA-HD is defined as a syndrome known to be associated with HD or cognitive impairment.

Results:

Patient characteristics were comparable between groups (P > 0.05). There were 52 patients with I-HD and 24 with SA-HD. The patients median age was 9 days at diagnosis and 1.5 month at surgery. SA-HD patients became bowel continent at a significantly older age (mean age 8.43 vs 4.94 years, P = 0.0471) and received more bowel continence medications. At 5 years, SA-HD patients requiring ≥2 medications for bowel continence represented 54.5% versus 11.1% of I-HD patients (P = 0.009). Lastly, SA-HD patients had urinary incontinence at a significantly older age (P = 0.0136, 5 years).

Conclusion:

Clinicians should be aware that SA-HD patients are more prone to bladder dysfunction and became bowel continent at an older age than I-HD patients. They need more and prolonged bowel management medications, and other important complications need to be addressed in patient care. These results should prompt a longer follow-up period for these patients, especially in SA-HD.

Graphical Abstract

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Citing Literature

Volume77, Issue5

November 2023

Pages 592-596