Embryological and clinical implications of the association between anorectal malformations and spinal dysraphisms.

Abstract:

PURPOSE:To describe the association of anorectal malformation (ARM) and spinal dysraphism (SD) in terms of impact on the management of SD and embryogenetic implications. METHODS:Patients with SD associated with (A) or without (B) ARM were included. The two groups were further divided into operated on (A1/B1) or not (A2/B2) for SD. Groups A and B were compared for type of SD (embryogenetic classification) and prevalence of neurosurgery; Groups A1 vs. A2 for type of ARM (Wingspread classification); Groups A1 vs. B1 for age at neurosurgery, neurophysiology, and clinical symptoms. MAIN RESULTS:One hundred twenty-one patients with SD, 83 with and 38 without ARM were consecutively treated (1999-2015). Group A was associated only with SDs developing after primary neurulation, corresponding to the period of cloacal septation and organogenesis (p = 0.0007). Untethering surgery was significantly less frequent in Group A (p < 0.0001 and p = 0.04, respectively). Higher ARMs were not associated with increased risk for neurosurgery. No other significant differences were detected. CONCLUSIONS:In our series, ARMs were associated only with SD developing after primary neurulation, suggesting a single insult leading to both SD and the associated ARM. Neurosurgery is indicated less frequently in patients with ARM-associated SD, despite the similar preoperative clinical features.

journal_name

Pediatr Surg Int

authors

Totonelli G,Messina R,Morini F,Mosiello G,Palma P,Scuglia M,Iacobelli BD,Bagolan P

doi

10.1007/s00383-017-4104-5

subject

Has Abstract

pub_date

2017-08-01 00:00:00

pages

843-847

issue

8

eissn

0179-0358

issn

1437-9813

pii

10.1007/s00383-017-4104-5

journal_volume

33

pub_type

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