Alejandra Vilanova-Sanchez, Anna Morandi, Dalia Aminoff, Maria Marcela Bailez, Andrea Bischoff, Fenna Braam, Lesley Breech, Ivo de Blaauw, Hannah Feldtblad, Margit Fisch, Michel Haanen, Gundela Holmdahl, Belinda Hsi Dickie, Willemijn Irvine, Moniek Ter Kuile, Allison Mayhew, Erin R McNamara, Paola Midrio, Mikko P Pakarinen, Luna Pivi, Tomas Wester, Dan Wood, Pernilla Stenström, European Reference Network (ERN) eUROGEN and ARM-net
In ARM patients with vaginal agenesis and identifiable Müllerian structures, no consensus currently exists regarding early versus deferred vaginal replacement. This report is the first to summarize expert perspectives on the advantages and disadvantages of both approaches and highlights the need for further research on long-term outcomes to inform future practice.
BACKGROUND: Traditionally, vaginal replacement in patients with anorectal malformations (ARM) has been performed concomitantly with anorectal reconstruction during childhood when the native vagina does not reach the perineum. Concerns regarding long-term outcomes have led to increasing debate about whether reconstruction should instead be deferred until adolescence or adulthood. This consensus report aimed to establish best-practice recommendations regarding the timing of vaginal replacement, follow-up, and patient-centered aspects of care in patients with ARM, vaginal agenesis, and identifiable Müllerian structures for whom vaginal replacement is required.
METHODS: An international multidisciplinary panel used the Nominal Group Technique to gather evidence from a literature review, digital expert case discussions, a global survey, and an in-person meeting involving 22 specialists and patients.
RESULTS: Evidence from 27 studies revealed only low-level evidence studies. Most reports favored early vaginal replacement. Responses from 71 multidisciplinary experts worldwide indicated growing acceptance of delayed reconstruction and highlighted the importance of shared decision-making with the parents. In the in-person meeting no consensus on the optimal timing of vaginal replacement was achieved, while consensus was reached regarding: (1) comprehensive counseling and multidisciplinary care; (2) structured follow-up, including imaging, and psychosexual support; and (3) patient and parent education to support self-management.
CONCLUSIONS: In ARM patients with vaginal agenesis and identifiable Müllerian structures, no consensus currently exists regarding early versus deferred vaginal replacement. This report is the first to summarize expert perspectives on the advantages and disadvantages of both approaches and highlights the need for further research on long-term outcomes to inform future practice.