Bladder and bowel dysfunction in Down syndrome with neural tube defect: case report and review of the literature.


Journal

Italian journal of pediatrics
ISSN: 1824-7288
Titre abrégé: Ital J Pediatr
Pays: England
ID NLM: 101510759

Informations de publication

Date de publication:
20 Jul 2023
Historique:
received: 29 06 2022
accepted: 07 01 2023
medline: 24 7 2023
pubmed: 21 7 2023
entrez: 20 7 2023
Statut: epublish

Résumé

Down syndrome is a genetic disorder caused by trisomy of chromosome 21 and characterized by an increased risk of multiorgan involvement. In Down syndrome children, functional constipation and lower urinary tract infections have been described, together with higher risk for incontinence and delayed sphincter control. At present, to our knowledge, no clear association between Down syndrome, Bladder Bowel Dysfunction and neural tube defects has been previously described. We describe two female patients with Down syndrome presenting Bladder Bowel Dysfunction in association with neural tube defects, who both underwent personalized multidisciplinary intervention and pelvic floor rehabilitation, with good clinical outcomes. At present, no screening program has been established in order to rule out neural tube defects or neurogenic urinary anomalies in Down syndrome patients presenting bowel and/or bladder dysfunction. In our opinion, presence of spinal abnormalities, despite rare, may be contribute to urinary symptoms and should be ruled out in patients presenting progressive or persistent Bladder Bowel Dysfunction. Early diagnosis and management of spinal cord defects associated with neurogenic urinary dysfunction may allow to prevent possible complications.

Sections du résumé

BACKGROUND BACKGROUND
Down syndrome is a genetic disorder caused by trisomy of chromosome 21 and characterized by an increased risk of multiorgan involvement. In Down syndrome children, functional constipation and lower urinary tract infections have been described, together with higher risk for incontinence and delayed sphincter control. At present, to our knowledge, no clear association between Down syndrome, Bladder Bowel Dysfunction and neural tube defects has been previously described.
CASE PRESENTATION METHODS
We describe two female patients with Down syndrome presenting Bladder Bowel Dysfunction in association with neural tube defects, who both underwent personalized multidisciplinary intervention and pelvic floor rehabilitation, with good clinical outcomes.
CONCLUSION CONCLUSIONS
At present, no screening program has been established in order to rule out neural tube defects or neurogenic urinary anomalies in Down syndrome patients presenting bowel and/or bladder dysfunction. In our opinion, presence of spinal abnormalities, despite rare, may be contribute to urinary symptoms and should be ruled out in patients presenting progressive or persistent Bladder Bowel Dysfunction. Early diagnosis and management of spinal cord defects associated with neurogenic urinary dysfunction may allow to prevent possible complications.

Identifiants

pubmed: 37475046
doi: 10.1186/s13052-023-01412-z
pii: 10.1186/s13052-023-01412-z
pmc: PMC10360214
doi:

Types de publication

Review Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

89

Informations de copyright

© 2023. The Author(s).

Références

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Auteurs

Roberta Onesimo (R)

Rare Diseases Unit, Fondazione Policlinico Universitario Agostino Gemelli -IRCCS, Largo Agostino Gemelli 8, Rome, Italy. roberta.onesimo@policlinicogemelli.it.
Pediatric Unit, Fondazione Policlinico Universitario Agostino Gemelli -IRCCS, Rome, Italy. roberta.onesimo@policlinicogemelli.it.

Cristiana Agazzi (C)

Rare Diseases Unit, Fondazione Policlinico Universitario Agostino Gemelli -IRCCS, Largo Agostino Gemelli 8, Rome, Italy.
Pediatric Unit, Fondazione Policlinico Universitario Agostino Gemelli -IRCCS, Rome, Italy.

Luca Massimi (L)

Neurosurgery Unit - Fondazione Policlinico Universitario Agostino Gemelli -IRCCS, Rome, Italy.

Valentina Giorgio (V)

Neurosurgery Unit - Fondazione Policlinico Universitario Agostino Gemelli -IRCCS, Rome, Italy.

Chiara Leoni (C)

Rare Diseases Unit, Fondazione Policlinico Universitario Agostino Gemelli -IRCCS, Largo Agostino Gemelli 8, Rome, Italy.
Pediatric Unit, Fondazione Policlinico Universitario Agostino Gemelli -IRCCS, Rome, Italy.

Giuseppe Zampino (G)

Rare Diseases Unit, Fondazione Policlinico Universitario Agostino Gemelli -IRCCS, Largo Agostino Gemelli 8, Rome, Italy.
Pediatric Unit, Fondazione Policlinico Universitario Agostino Gemelli -IRCCS, Rome, Italy.
Università Cattolica del Sacro Cuore, Rome, Italy.

Claudia Rendeli (C)

Spina Bifida Center - Fondazione Policlinico Universitario Agostino Gemelli -IRCCS, Rome, Italy.

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Classifications MeSH