maldescended testis in adults - up.ac.za 2015/sat/s4/bg-gaudji... · bilateral • fowler stephens...
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Maldescended testis in Adults
Dr. BG GAUDJIUrologist STEVE BIKO ACADEMIC HOSPITAL
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Definitions
• Cryptorchid: testis neither resides nor can be manipulated into the scrotum
• Ectopic: aberrant course• Retractile: can be manipulated into
scrotum where it remains without tension• Gliding: can be manipulated into upper
scrotum but retracts when released• Ascended: previously descended, then
“ascends” spontaneously
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Epidermiology
Cryptorchidism is the most common genital disorder encountered in paediatrics.
• 1% to 4% of full-term and • 1% to 45% of preterm male neonates (Sijstermans et al,
2008)Spontaneous descent is more likely and may occur later in
premature infants. Spontaneous descent after the first year of life is
uncommon.
How common is it?
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Is the incidence of cryptorchidism increasing?
• Literature controversial– Cryptorchidism, hypospadias, micropenis– Decreasing semen quality– Increasing testicular cancer– Increasing demand for assisted reproduction
• Impact of environmental xenoestrogens– Herbicides, pesticides, PCBs, polystyrenes
• Environmental antiandrogens– Linuron, vinclozolin, pp’DDE, polyaromatic
hydrocarbons
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Testicular development
• 6 wk primordial germ cells migrate to genital ridge
• 7 wk testicular differentiation• 8 wk testis hormonally active
– Sertolis secrete MIF• 10-11 wk Leydig cells secrete T• 10-15 wk external genital differentiation
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Testicular descent
• 5-8 wk processus vaginalis– Gubernaculum attaches to lower
epididymis• 12 wk transabdominal descent to internal
inguinal ring• 26-28 wk gubernaculum swells to form
inguinal canal, testis descends into scrotum
• Insulin-3 (INSL3) effects gubernacular growth
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Genetic Susceptibility
• Genetic studies of cryptorchidism suggest that the disease is heritable but that susceptibility is likely polygenic and multifactorial.
• Autosomal dominance with reduced penetrance probable mode of inheritance
• Recurrence risk ratio (RR) was 10.1 in twins, 3.5 in brothers, and 2.3 in offspring and were significantly higher in maternal than in paternal half-brothers. (Schnack et al, 2008).
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• An adult is not a big child!
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Presentating History
• Pain• Hernia • Testicular malignancy• Infertility• Micropenis• Delayed puberty
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Hypogonadism
• Loss of libido ( desire )• Fatigue / depression / loss of well-being• ↓ Lean body muscle and mass• ↑ Visceral fat / mass• Sleep disturbances• ↓ Virility• ↑ Sweating / dry skin / anaemia• Osteoporosis
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An Adult with empty scrotum
Congenital• Undescended• Retractile• Ectopic• Vanished• DSD• Agenesis
Acquired• Torsion• Orchidectomy
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Evaluation
– Role of Sonar – Baseline
investigations ?–Testosterone–Semen analysis
• To do a biopsy or not
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Age At Presentation!
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Hypothalamus
Pituitary
Sertoli
Androgen resistance
Dysgenesis/anorchiaTestosterone biosynthetic problems
Hypopituitarism
Low/absent GnRHKallmann’sPrader Willi
Testosterone
dihydrotestosterone
Androgen receptor
Post-receptor effects
Leydig Germ cells
GnRH
MIF
FSH LH
5 α reductase 5 α reductase deficiency
MIF deficiency/persistent Mullerian ducts
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THE LINK BETWEEN ERECTILE DYSFUNCTION, TESTOSTERONE AND METABOLIC SYNDROME
Testosterone deficiency syndrome is a proven risk factor for all the metabolic syndrome components
All components of metabolic syndrome are underlying conditions for erectile dysfunction
Erectiledysfunction
Abdominal obesity represents a “vicious circle”: abdominal fat tissue reduces testosterone andtestosterone reduces the fat tissues.
Testosterone deficiency syndrome
Metabolicsyndrome
(abdominal fat, diabetes, obesity, hypertension....)
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Increased risk of neoplasia
• Cortes 2001: 1638 testicular samples from 1335 patients (23% bilateral, 77% unilateral)
• Mean age @ surgery 11.7 yo (0.1-18.9 yr)
• 1 invasive germ cell tumor• 6 carcinoma in situ• 1 Sertoli cell tumor
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Neoplasia & cryptorchidism
• 3 neoplasms in intra-abdominal testes• 4 neoplasms in boys with abnormal
external genitalia• 2 neoplasms in boys with known
abnormal karyotype• Risk of neoplasia 5% with intraabdominal
testes, abnormal external genitalia or abnormal karyotype (Cortes 2001)
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When to operate?
• Lee 2002• Inverse correlation between age at
surgery and T• Inverse correlation between body wt and
T• Direct correlation between T and sperm
density, motility, morphology• Indicates direct relationship between
spermiogenesis and T in cryptorchid men
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Is further treatment after surgery indicated?
• Subfertility correlates with reduced total germ cell counts
• Defects in germ cell maturation associated with blunting of normal surges LH/FSH
• Prepubertal treatment with GnRH could theoretically trigger normal germ cell maturation & proliferation
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Palpable UDT Orchiolysis pexy
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Laparoscopic findings
Unilateral • Absent testis and
cord structures• Absent testis with
cord structures exiting the inguinal canal
• High testis • Testicular Nubbin
– Orchidectomy
Bilateral • Fowler Stephens• Prentis Maneuvre• Orchidectomy
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Laparoscopy
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INTRA ABDOMINAL TESTIS
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Adults !
• Modifications have been discussed regarding orchiolysis
• Challenge on mobilization• Adult with bilateral undcended testis!
– Intra-abdominal testis– Fowler -Stevens– When to do orchidectomy?– When to do nothing?
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Absent testis
• Treat the complications• Hypogonadism
– Testosterone supplement• Family interest• Self image
– Testicular prosthesis
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Thank you!