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Paediatric Urology
Hypospadias in Children
Rita Gobet
Head, Division of Paediatric Urology, University Children’s Hospital, Zurich
Abstract
The objective of this article is not only to summarise surgical and technical facts about hypospadias, but also to put the understanding
and management of children with this very common defect of the masculinisation process into perspective with our modern European
way of life. Environmental factors may influence the reported incidence of hypospadias, but certainly our way of life, asking and
expecting perfect bodies for everybody, also increases the number of surgical procedures performed for minor defects. Psychosocial
and psychosexual outcomes and long-term follow-up are most likely as important as short-term surgical outcomes. Finding valid
answers to ethical questions is relevant to enable us to professionally counsel families and patients and to issue recommendations
based on evidence.
Keywords
Hypospadias, psychosocial outcome, psychosexual outcome
Disclosure: The author has no conflicts of interest to declare.
Received: 9 December 2008 Accepted: 22 February 2009
Correspondence: Rita Gobet, Division of Paediatric Urology, University Children's Hospital, Steinwiestr. 75, 8032 Zürich, Switzerland.
E: Rita.Gobet@kispi.uzh.ch
Hypospadias is one of the most common congenital malformations. Smith’s Recognizable Patterns of Human Malformation, 15 syndromes
It affects the male external genitalia, mainly the ventral side of the are listed in which hypospadias is a frequent feature;
3
the most likely
penis. The most obvious feature is an ectopic location of the meatus cause of hypospadias is multifactorial. Environmental or other
urethrae externus, resulting from an incomplete urethral endocrine disruption, inborn endocrinological disorders including
development. The term hypospadias labels a wide spectrum of local tissue unresponsiveness or a developmental arrest of other
penile malformations ranging from very mild to more devastating origin may be essential factors.
4
Most importantly, hypospadias can
forms. It is caused by a ventral fusion defect of the urethral folds, be considered as one side of a spectrum of genital anomalies ranging
resulting in incomplete formation of the penile urethra; therefore, from simple distal hypospadias with unknown aetiology to XY
the meatus urethrae externus is located anywhere between the disorders of sex development (DSD) with underlying disorders of the
glans and the perineum. In severe forms the orifice is located in sex chromosomes and/or androgen metabolism.
between the two halves of the bifid scrotum, while in mild forms the
meatus may lay glanular just proximal to its usual place and is often There is evidence of an increasing reported incidence of hypospadias
associated with an otherwise completely normal penis. in several European countries and the US from the 1960s to the
1980s, as presented in 1999 by Paulozzi.
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Continuation of this rising
In addition to the ectopic location of the orifice, the penile shaft can be trend was not observed in European countries as reported by Dolk et
short and/or bent and there may be a glanular tilt, i.e. an angle al.,
6
and the authors severely doubted the methodological accuracy of
between the axis of the penile shaft and the glans penis. The curvature the means applied to actually screening this trend worldwide.
6
Today,
of the penile shaft may be caused by skin traction alone or by chordae concerns with data quality still severely hamper attempts to answer
(fibrotic tissue) causing a ventral curvature of the shaft, which is most the burning question of whether increased exposure to endocrine
often associated with a hypoplastic urethra (urethra not surrounded by disruptors (exogenous substances that possibly interfere with the
a normal corpus spongiosum), or the corpora cavernosa themselves physiological functions of endogenous hormones) is affecting male
may be ventrally malformed, causing significant curvature. The ventral sex differentiation.
deficit of the penis often causes a paucity of ventral skin coverage and
almost always incomplete closure of the foreskin, causing the typical Male reproductive health is reported to be negatively affected in
preputial dorsal hood. Bifid scrotum or penoscotal transposition is several ways by environmental chemicals acting as anti-androgens,
associated with more severe forms. such as increased incidence of testicular cancer, decreased semen
quality and increased incidence of undescended testes and
This anomaly affects between 1:125 and 1:300 live male births.
1,2
It is hypospadias. Skakkebaek et al.
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suggested that all of these
usually an isolated malformation but may be part of a syndrome. In observations may be related and indicated a common cause, coining
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