Congenital prepubic sinus: Is it a residual cloacal membrane and umbilicophallic groove?

Abstract

We report two cases of congenital prepubic sinus (CPS) and discuss theories about its embryology and the etiology of its variants to improve the global understanding of this uncommon anomaly. Based on a review of the scant number of reported cases and our own experience, we postulate that CPS may be caused by a residual cloacal membrane and umbilicophallic groove and that its depth may determine the position of its ending.

Congenital prepubic sinus—An aborted dorsal urethral duplication or a cloacal remnant?

“…After the first report by Campbell et al, in 1987, around 50 cases are reported in literature [1][2][3][4][5]. Four theories are proposed to explain the etiology of CPS: 1) anomalous abdominal wall closure; 2) a variant of dorsal urethral duplication; 3) a fistula of primitive urogenital sinus and 4) a remnant of primitive cloaca [6][7][8][9]. Routine and immunohistochemical staining of tract in patients reported here and in literature; specifically, the reports by Huang et al, Chou et al and Balster et al, val-idates the presence of transitional, columnar and squamous epithelium in the lining.…”

“…Thus, the most likely source of 3 different type of epithelium in the lining of sinus is residual cloacal membrane [11]. It is postulated by Tsukamoto et al that during the embryonic development, the umbilicophallic groove traps a small share of cloacal membrane during its migration from the base of umbilical cord and tubularization following longitudinal growth of embryo [8]. The degree of depth of trapped cloacal membrane can explain the variable direction and location of blind ending tract at umbilicus, bladder or urethra.…”

Congenital Prepubic Sinus in an Adolescent Female: A Case Report and Review of the Literature

“…Table 1 summarizes the 17 female cases of CPS found in literature to date. Theories surrounding etiology include anomaly of abdominal wall closure 3 and persistent remnant of the cloaca, 8,12 but in most case reports, it appears to be a variant of dorsal urethral duplication. 14 Support for this arises from the presence of squamous epithelium distally and transitional epithelium in the proximal part of the sinus with surrounding smooth muscle ( Table 1).…”

Congenital tracheoesophageal, umbilical, vascular and meningeal fistulas

“…34 A congenital prepubic sinus (CPS) is another rare abnormality that is supposed to be due to persistence of a part of the cloacal membrane. 35 A case of midline CPS was described to extend from the prepubic skin to the superior surface of the urinary bladder, then to the umbilicus along the median umbilical ligament. 36 In a boy suffering from abnormal dribbling of urine from his perineum, a congenital posterior urethroperineal fistula was extending from the posterior urethra to the perineal skin.…”