TY - JOUR
T1 - Embryological and clinical implications of the association between anorectal malformations and spinal dysraphisms
AU - Totonelli, Giorgia
AU - Messina, Raffaella
AU - Morini, Francesco
AU - Mosiello, Giovanni
AU - Palma, Paolo
AU - Scuglia, Marianna
AU - Iacobelli, Barbara D.
AU - Bagolan, Pietro
PY - 2017/8/1
Y1 - 2017/8/1
N2 - 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.
AB - 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.
KW - Anorectal malformations
KW - Embryogenesis
KW - Neurulation
KW - Spinal dysraphism
KW - Spinal lipoma
KW - Tethered cord
UR - http://www.scopus.com/inward/record.url?scp=85020636103&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85020636103&partnerID=8YFLogxK
U2 - 10.1007/s00383-017-4104-5
DO - 10.1007/s00383-017-4104-5
M3 - Article
AN - SCOPUS:85020636103
VL - 33
SP - 843
EP - 847
JO - Pediatric Surgery International
JF - Pediatric Surgery International
SN - 0179-0358
IS - 8
ER -