Aposthia: A Congenitally Deficient Prepuce without Hypospadias

A 3-day-old male neonate presented with his grandfather to our urology clinic for an anomalous prepuce. Parents were instructed to consult a urologist for the nature of the anomaly. The baby was full term with 3.5 kg birth weight and unremarkable other findings, breast-feed, and started regular urinary voiding. Local examination revealed normal sized and positioned testis in each hemiscrotum with bilateral mild hydroceles. The penis was normal in length without curvatures. However, the prepuce was deficient all around and covered only less than the half of the glans penis. The external urethral meatus was slit-like and normally positioned but was relatively wide (Figures 1 & 2). Abdominal ultrasonography revealed no urinary abnormalities. Relatives were assured as there were no needed further interventions. Accordingly, we should declare the differences between a deficient prepuce without hypospadias which is known as Aposthia and a hypospadias with ventrally abnormal meatus and ventrally deficient prepuce. While the latter anomaly is relatively common, the former one is very rare and is known as Aposthia, microposthia or natural circumcision [1]. Different postulations have been proposed for its explanation up to familial and abnormal genetic expression hypothesis [2].

Figure 1:Panel A: View of the deficient prepuce while the penis is supported. Note: the relatively wide urethral meatus.

Figure 2:Panel B: A confirmatory view of the deficient prepuce while the penis is unsupported.

In conclusion, Aposthia is a very rare condition of congenitally deficient prepuce without hypospadias. It is a spot diagnosis and usually needs no interventions other than assurance of the parents.

1. Palacios GM, Gallart MR, Barca RP, Martinez EE, Casasnovas BA (2013) Congenital absence of preputial foreskin: An extremely uncommon anomaly. J Pediatr Surg 48(2): e13-e15.
2. Din AUM, Salam A, Rafiq MA, Khaliq I, Ansar M, et al. (2007) Aposthia: a birth defect or normal quantitative recessive human genetic trait? East Mediterr Health J 13(2): 280-286.