Surgical Management of Perineal Groove.

Alberto Peña's contributions to our understanding of pediatric perineal surgery and anatomy make this the rarest of anomalies both discernable and reparable. We diagnosed, managed and operated on the 24 th ever reported case of pure perineal groove. We review here the embryology, workup and surgical management necessary to successfully dispatch this disabling defect. Perineal grooves are so rare that a discussion of them is not included in Peña's masterpiece, "Surgical Management of Anorectal Malformations." 1 Indeed, only 23 cases of perineal groove have been previously reported in the surgical literature. Others that are referenced to are derivatives of imperforate anus anomalies. A perineal groove is a wet mucus membrane that courses the perineal space from the anus to the vagina in a female, or, far more rarely, from the anus to the penoscrotal junction in a male. 2 Most clinicians, including those in the realm of pediatrics, are unfamiliar with the presentation of this rare anomaly and thus, its management. It is often misdiagnosed as an anal fissure, perineal trauma, diaper dermatitis, infection, or sexual abuse. 3 We present to you not only a description of the condition, but also a treatment paradigm on how to surgically excise it without damaging the normal anus and the normal vagina.