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26-07-2022 | Neurogenic Bladder | Original Article

Anorectal malformation, urethral duplication, occult spinal dysraphism (ARM-UD-OSD): a challenging uncommon association

Authors: Federica Lena, Chiara Pellegrino, Antonio Maria Zaccara, Maria Luisa Capitanucci, Giacomo Esposito, Barbara Daniela Iacobelli, Daniela Longo, Tamara Caldaro, Diletta Bruno, Francesca Bevilacqua, Francesca Santato, Giulia Lucignani, Carlo Efisio Marras, Enrico Castelli, Pietro Bagolan, Giovanni Mosiello

Published in: Pediatric Surgery International | Issue 10/2022

Abstract

Purpose

Urethral duplication (UD) is a rare malformation, which can be associated with other anomalies, like anorectal malformations (ARM). ARM has been described with occult spinal dysraphism (OSD). No ARM-UD-OSD combination has been reported.

Aim

To share our experience and to discuss the management of ARM-UD-OSD association.

Methods

We retrospectively reviewed records of five boys with UD. Four of these had ARM-UD-OSD association. ARM was the first diagnosis in all; OSD and UD was detected during screening for associated malformation.

Results

All patients underwent ARM correction, 3 after colostomy. All reached fecal continence, 3 are performing bowel management. Three patients underwent UD surgical correction. Because of symptoms’ worsening, 2 children had detethering surgery. At a mean follow-up of 9.5 years, all patients have normal renal function, 3 are on clean intermittent catheterization (CIC) for neurogenic bladder (1 has a cystostomy, another one an appendicostomy).

Conclusions

UD and OSD should be considered in patients with ARM. Children with these conditions associated must be centralized in a third-level Center and management carefully planned; in particular, urethral reconstruction should be weighed, considering CIC could be required. Suspicion of neurogenic bladder must be present in OSD patient.

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Title: Anorectal malformation, urethral duplication, occult spinal dysraphism (ARM-UD-OSD): a challenging uncommon association
Authors: Federica Lena
Chiara Pellegrino
Antonio Maria Zaccara
Maria Luisa Capitanucci
Giacomo Esposito
Barbara Daniela Iacobelli
Daniela Longo
Tamara Caldaro
Diletta Bruno
Francesca Bevilacqua
Francesca Santato
Giulia Lucignani
Carlo Efisio Marras
Enrico Castelli
Pietro Bagolan
Giovanni Mosiello
Publication date: 26-07-2022
Publisher: Springer Berlin Heidelberg
Keywords: Neurogenic Bladder
Posterior Sagittal Anorectoplasty
Spinal Dysraphia
Colostomy
Urinary Tract Infection
Published in: Pediatric Surgery International / Issue 10/2022
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI: https://doi.org/10.1007/s00383-022-05186-z

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Anorectal malformation, urethral duplication, occult spinal dysraphism (ARM-UD-OSD): a challenging uncommon association

Abstract

Purpose

Aim

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Purpose

Aim

Methods

Results

Conclusions

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