EN
TÜRKÇE
Poster - 225
ARM in ARM? Investigating the co-occurrence of anorectal malformations and labioscrotal anomalies
Patrick Sharman, Maria Randazzo, Ingo Jester, Ian Jones
Birmingham Children's Hospital
Purpose Anorectal malformations (ARMs) are known to be associated with other defects, however there is no evidence addressing the co-occurrence of ARMs with anomalies of structures derived from the labioscrotal folds. A systematic review of current literature was performed to investigate patterns of association between different ARMs and labioscrotal anomalies (LSAs) when presenting simultaneously.
Method Five online databases (Cochrane, MEDLINE, Embase, CINAHL Plus, ProQuest) were searched for articles reporting co-occurrence of ARMs with LSAs; relevant data was tabulated according to type of defect. ARMs were grouped by increasing severity as ‘Low’ (perineal (cutaneous)/vestibular/rectovaginal fistula; anal stenosis), ‘High’ (rectourethral prostatic/rectourethral bulbar/rectobladder neck/rectovesical fistula; rectal atresia) and ‘Complex’ (cloaca/no fistula/pouch colon/H-type fistula). Both the number of individuals with at least one ARM-LSA co-occurrence and the total number of LSAs associated with each type of ARM were recorded.
Results 1642 articles were identified. In compliance with PRISMA guidelines, 144 papers met inclusion criteria, reporting co-occurrence of ARMs with LSAs (319 patients). Patients with complex ARMs had proportionally the most LSAs associated with their ARM - 58.4% of these were associated with only a single LSA. For high and low ARMs, 66.0% and 77.0% of cases were associated with a single LSA respectively. The absence of data reporting prevalence of each type of ARM without LSA limits the potential to predict the presence of LSAs according to level of ARM.
Conclusion This systematic review indicates that more severe ARMs are associated with a greater number of LSAs. More importantly, a significant proportion of low (i.e. less severe) ARMs may co-occur alongside at least one LSA. This study emphasises the need to establish a close collaboration between paediatric surgeon and urologist, ideally in a joint clinic setting, in the management of patients with any type of ARM.