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Hypospadias in Children
the term testicular dysgenesis syndrome; this theory has recently Symptoms from meatal stenosis are rare, although macroscopically
been redefined.
8
Details of molecular mechanisms possibly interfering the meatus may appear to be narrow in many distal cases.
with normal urethral development and therefore causing hypospadias Hypospadias does not predispose to urinary tract infections; nor is
in humans are reported by Wang et al.
1
Fourteen per cent of siblings it a reason for incontinence. Severe forms with significant curvature
are affected and 8% of offspring.
9
In vitro fertilisation is associated of the penile shaft may interfere with vaginal intercourse, and more
with an increased risk of hypospadias. This may be directly related to proximal location of the meatus may interfere with fertility. The
maternal hormonal treatment or genuine maternal or foetal psychosexual development of patients and health-related quality of
endocrine abnormalities.
10
life (HRQoL) may be affected by malformation or by the treatment.
18
Beginning with the presence of a Y chromosome, the sex-determining Treatment
region Y (SRY region) and the corresponding protein testes-determining Treatment of patients with hypospadias needs to be more than simply
factor (TDF), a complex mechanism regulating male sexual surgical. Many patients and families need psychological support.
18
It is
differentiation begins. Under the influence of androgens (mainly important to follow these patients long-term, until after puberty, as
dihydrotestosterone), at one-month gestation the development of the surgical and psychological problems are likely to become apparent
genitalia is at an ambiguous stage; by 16–18 to 20 weeks, then.
19
Treatment goals include a functionally and cosmetically normal
masculinisation has occurred with the enlargement of genital tubercle penis, healthy psychosexual development and normal HRQoL. More
into the penis, fusion of urethral folds to form the urethra and fusion of precisely, the surgical goals are a vertical slit-like meatus in the distal
the labioscrotal folds into the scrotum, thereby creating the mid-line third of the glans, a normally shaped glans, a straight penile shaft, a
raphe. The prepuce cannot fuse if the glandular urethra is not correctly normal urethra, normal ventral skin coverage and a normal position of
formed. The origin of the glanular urethra is still controversial, but an the scrotum in relationship to the penile shaft. The optimal functional
endodermal origin of the whole urethra seems to be most likely.
11
outcome is a condensed urinary stream in the axes of the penis
achieved by normal bladder function and normal sexual function
Diagnosis/Classification including normal delivery of semen.
Diagnosis is usually made at birth; rarely, and only in severe cases,
pre-natal detection with ultrasound is possible. Since this Multiple surgical treatment options are available and depend on
malformation may cause significant stress in parents, it is mandatory individual cases and the surgeon’s preference. Generally, distal
that after diagnosis correct information about the consequences of hypospadias can be repaired as an outpatient procedure under
this malformation is given to the parents. general anaesthesia supported by a penile block or caudal
anaesthesia. Multiple variations of dressings and stents or catheters
Associated anomalies include undescended testes in 9% of distal cases exist. More severe forms may benefit from a hospital stay. There are
and up to 30% of severe cases, and inguinal hernia in 9%.
12
A prostatic about 250 methods described, many of which represent refinements
utriculus (Müllerian remnant) is found only in severe hypospadias.
13
of old ideas made possible by technological advances and by better
Classification of hypospadias uses the location of meatus; the material, including loops, etc.
concept of using the location of the meatus after orthoplasty is
important since it describes the actual urethral defect requiring repair. Judging by the number of recent publications, tubularised incised
Furthermore, in most cases the proximal hypoplastic urethra is plate repair (Snodgrass) has gained general acceptance for distal
diagnosed only during surgery: 50–60% are anterior, 20–30% penile forms; certainly, the superior cosmetic outcome and the simplicity of
and 20–30% between the penoscrotal junction and perineum.
14
the method have helped to promote this technique worldwide. The
debate as to whether the functional outcome stands the test of time
Work-up remains unresolved. Meatal advancement and glanduloplasty
As hypospadias and male DSD may be viewed as a spectrum of (MAGPI), tubularisation alone (glans approximation procedure [GAP]),
hormonal disease, karyotyping and endocrinology assessment are meatal-based flap techniques or onlay island flap are alternatives for
certainly needed in severe forms. The question of whether anterior distal and penile hypospadias without chordae and are described in
and penile forms associated with undescended testes also benefit detail in paediatric urology textbooks.
from these assessments remains unanswered; today, in the majority
of such cases no underlying pathology can be diagnosed and The repair of severe hypospadias is controversial, especially when
therapeutic consequences are rare.
15
onlay techniques are impossible. The question of whether resection
of the urethral plate is ever necessary to perfectly straighten the
Imaging penis without compromising penile length is hotly debated by
The majority of patients require no further imaging, as associated experts. The consequences, namely primarily tubularised (with or
anomalies are rare. In more severe forms, a urethrography to look without vascular pedicle) flaps, are prone to severe complications
for Müllerian remnants (prostatic utriculus) is indicated.
13
such as diverticula formation. Therefore, two-stage repairs have
Occasionally, post-operative imaging is needed.
16
Patients with gained more acceptance, as results seem to be very consistent. In
syndromes (i.e. VATER) or associated anomalies such as heart, limb such cases orthoplasty (dorsal plication in the mid-line or dorsal
malformations, cleft lip or pyloric stenosis may benefit from bilateral incision of tunica albuginea after mobilisation of
abdominal ultrasound (US).
17
neurovascular bundles or ventral patch repair) and transfer
of tissue (vascularised or free flap) to the ventral surface followed
Symptoms by urethoplasty – usually six months later – are performed. Salvage
Usually, the abnormal location of the meatus does not cause procedures are highly individualised and should be performed
symptoms, other than downward flection of the urinary stream. only by experts.
EUROPEAN UROLOGICAL REVIEW 83
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