1. Congenital absence of preputial foreskin: An extremely
uncommon anomaly
Maria Garcia-Palacios⁎, Roberto Mendez-Gallart, Pablo Rodriguez-Barca,
Elina Estevez-Martinez, Adolfo Bautista-Casasnovas
Hospital Clinico Universitario de Santiago de Compostela, Department of Pediatric Surgery, 15706, Santiago de Compostela,
A Coruña. Spain
Received 27 July 2012; revised 29 August 2012; accepted 19 October 2012
Key words:
Aposthia;
Preputial anomalies;
Hypospadias
Abstract The part of the penile skin that covers the glans penis is named prepuce or foreskin. The
embryologic development of the prepuce and urethra is related. Several congenital anomalies of the
prepuce have been previously reported, but the absence of the prepuce with a normal development of the
urethra is a very rare association. We report a sporadic case with absence of the prepuce and normal
urethral development.
© 2013 Elsevier Inc. All rights reserved.
The prepuce is a common structure of genitalia that forms
the covering of the glans penis. Natural circumcision is a
very rare congenital condition in which the prepuce is absent.
It is referenced in Jewish law of 1567 CE as regards a child
born circumcised [1,2].
On the basis of embryologic studies, it is generally
accepted that normal preputial development is required for
successful canalization of the glandular urethra, so the
absence of the prepuce would result in an abnormal
development of the glandular urethra [3].
1. Case report
A term male neonate was born to a 30 year-old
mother. The pregnancy was controlled and no alterations
were found. The absence of the prepuce was noted on
physical examination in the delivery room in our
institution. In a detailed examination of the external
genitalia, the meatus was noted at the end of the glans
penis with the prepuce completely missing. The scrotal
sac had an abnormal appearance with a red skin area; a
dermatologist evaluated it and the diagnosis was erythema
toxicum neonatorum. In a few days the redness of the
skin area had disappeared. The urethral structure was
evaluated with a 6 CH catheter and it was normal. No
voiding problems were observed. There were no other
cases of missing prepuce or hypospadias documented in
the family history (Figs. 1, 2, 3).
This is the unusual sporadic presentation case of natural
circumcision or true aposthia with normal anatomic position
of the urethra. This case looks like an auto amputation of the
prepucium which occurred right before the delivery since
there is some granulation tissue between the skin and the
glans. The absence of decoloration of the glans may be
explained as a consequence of the amputation late in
gestation (Fig. 1).
⁎ Corresponding author: Tel.: +34 981 950 000.
E-mail address: maria.garcia.palacios@gmail.com (M. Garcia-Palacios).
www.elsevier.com/locate/jpedsurg
0022-3468/$ – see front matter © 2013 Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1016/j.jpedsurg.2012.10.072
Journal of Pediatric Surgery (2013) 48, E13–E15

2. 2. Discussion
The prepuce is a common anatomical covering of the
glans penis. Several congenital external genital anomalies
related to the prepuce have been reported previously.
However, natural circumcision or aposthia (the absence of
the prepuce) with a normal development of the urethra is
very rare [3].
The development of the prepuce begins at the third month
of gestation as a fold of the skin at the base of the glans with
the dorsal portion growing at a more rapid rate than the
ventral component. The proper development of the prepuce
depends on the presence of androgen and androgen
receptors. The closure of the ventral portion of the prepuce
is completed by the fifth month of gestation after the closure
of the glanular urethra. So in embryologic development the
prepuce is formed at the same time as the glanular urethra
and is dependent on the normal distal urethra development. If
the glanular urethra fails to develop, the preputial tissue is not
generally formed ventrally as is the case in hypospadias [4].
Most of patients who have been referred to pediatric
surgeons as natural circumcision have an associated
hypospadias. Their preputial tissue is absent on the ventrum
but it is excessive dorsally. The glanular urethra develops
incompletely and the meatus is formed ventrally.
Radojici and Perovic found 6 various morphological
forms of the prepuce. The length of the prepuce varies from
large to very small or absent like in natural circumcision [5].
Kim et al. have explained the term naturally circumcised
in 4 conditions: (A) Having no phimosis (B) Having a
relatively a small prepuce without phimosis (C) Fully
Fig. 1 Absence of preputial foreskin and discoloration of the
glans. Granulation tissue between the skin and the glans.
Fig. 2 Absence of the preputial foreskin.
Fig. 3 Normal development of the urethra without hypospadias.
E14 M. Garcia-Palacios et al.

3. retractable prepuce during erection and (D) A penis looks
circumcised without erection. Only the last condition is
actually a natural circumcision [6].
Amin-Ud-Dim et al. found sporadic and familiar cases of
natural circumcision. In sporadic cases all of them had a
hypospadias associated and the familiar case had a normal
development of the urethra. Both groups had a history of
consanguinous marriage and the study suggested that
certain linked modifier loci as well as a number of
autosomal recessive genes are required to express natural
circumcision [1].
The present case had a normal urethra with the prepuce
missing and no familial history of consanguineous marriage
or hypospadias.
References
[1] Amin-Ud-Din M, Salam A, Rafiq MA, et al. Aposthia: a birth defect or
normal quantitative recessive human genetic trait? East Mediterr Health
J 2007;13:280-6.
[2] Cold CJ, Taylor JR. The prepuce. BJU Int 1999;83:34-44.
[3] Altemus AR, Hutchins GM. Development of the human anterior
urethra. J Urol 1991;146:1085-93.
[4] Kim KS, Liu W, Cunha GR, et al. Expression of the androgen receptor
and 5 alpha-reductase type 2 in the developing human fetal penis and
urethra. Cell Tissue Res 2002;307:145-53.
[5] Radojicic ZI, Perovic SV. Classification of prepuce in hypospadias
according to morphological abnormalities and their impact on
hypospadias repair. J Urol 2004;172:301-4.
[6] Kim DS, Lee JY, Pang MG. Male circumcision: a South Korean
perspective. BJU Int 1999;83:28-33.
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Congenital absence of preputial foreskin