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Cryptorchidism
Amir Saad Aljboory
Urological resident
Iraqi board urology
Arabic board urology
DEFINITIONS
• Normal scrotal position: positioning of midpoint of the testis at or
below midscrotum.
• Undescended testis: absence of one or both testes in normal scrotal
position.
• Vanishing testes: present initially in development but are lost owing to
vascular accident or torsion unilaterally (monorchia) or, very rarely,
bilaterally (anorchia).
Agenesis: testis that was never present and therefore associated with
ipsilateral müllerian duct persistence.
• Congenital cryptorchidism: testes that are extrascrotal at birth.
• Recurrent cryptorchidism is when testes descend spontaneously
postnatally but subsequently return to a nonscrotal position.
• Testicular ascent or acquired cryptorchidism : Testes are intrascrotal at
birth but subsequently identified in an extrascrotal position
• Secondary cryptorchidism- testes that are suprascrotal after inguinal
hernia repair; testicular retraction- as a complication of orchidopexy.
• Retractile testes are scrotal testes that retract easily out of scrotum but
can be manually replaced in a stable scrotal position and remain there at
least temporarily.
Introduction:
• Development of the Testis
Initial differentiation of male and female gonad development is dependent
on: Presence of Y chromosome
SRY gene ( protein product of the Y chromosome)
steroidogenic factor 1 (SF1/Nr5a1)
Sox9 (SRY box-containing 9)
- Within the developing testis the three main differentiating cell types are:
- -spermatogonia ( gamete forming cells) –
- Sertoli cells (support cells) and
-Leydig or interstitial cells (hormone secreting cells)
Phases of testicular descent
• Phase 1: 5 weeks - The caudal mesonephros contacts the future gubernaculum at the
internal inguinal ring.
• Phase 2: 7 weeks -The genitofemoral nerve accompanies the newly formed gubernaculum
and processus vaginalis.
• Phase 3: 10 to 12 weeks - Gubernaculum remains a thin cord in both sexes.
Phase 3a: 12 to 14 weeks - The testis overrides the genital ducts and contacts the
gubernaculum.
• Phase 4: 14 to 20 weeks swelling of the gubernaculum, further development of the
cremaster muscle, and migration of the processus vaginalis produce widening of the
inguinal canal
• Phase 5: 20 to 28 weeks - Release of the distal subcutaneous attachment of the
gubernaculum and transinguinal passage of the testis.
Phase 5a : after 7th month – Caudal movement of the testis, regression of the
gubernaculum
Regulation of testicular descent
• Mechanical factors
Intra-abdominal Pressure
Gubernaculum tension
Processus vaginalis patency
• Growth factors
Insulin-like 3 (INSL3) growth factor (Abdominal phase)
Calcitonin gene related peptide (Inguinoscrotal Phase)
Epidermal growth factor (EGF) may promote by activating the
androgen responsive systems
• Hormonal factors
Testosterone (Inguinoscrotal Phase)
Undescended testis
• One of the most common pediatric disorders of male endocrine glands
& Most common genital disorder identified at birth.
Cryptorchidism: A greek word which means ‘hidden testis’
• Retractile- 60%
• Undescended- 35%
• Ectopic- 3%
• Ascending- <2%
Epidemiology & Risk factors
• Incidence of Undescended testis
1–9% of full-term infants , by 1 year incidence is 0.8%
30% of premature infants
Occurs on the right-50%, left-35%, bilateral-10-15%
Prevalence reported to be (possibly) increasing
• Risk factors
• Maternal & Gestational Factors
– Maternal Obesity
– Low birth weight
– Prematurity
• Maternal smoking- small-to-moderate increased risk for cryptorchidism is
present in offspring
• Genetic Factors
• 14% of cryptorchid boys – have positive family history.
• Multifactorial pattern transmission
• Father affected – 4%
• Sibiling affected – 6-10 %
• Gene mutation have identified -cryptorchidism
– INSL3
– LGR8
– Androgen receptor polymorphism
– HOXA10
– HOXD13
• Environmental
Prenatal exposure - endocrine disrupters
• – DES
• – Pesticide (DDT)
• – Nonylphenol
• – Natural phytoestrogens
• – Phthalates etc.
•Associated conditions
• Abdominal wall defects (eg, prune belly)
• Neural tube defects (eg, myelomeningocele)
• Cerebral palsy ~50%
• Disorders of sexual development
• Genetic disorders
• • Diminished testosterone secretion (eg, Klinefelter)
• • Diminished testosterone action (eg, Androgen insens.)
• • Primary hypogonadism (eg, Noonan)
• • Chromosomal disorders (eg, trisomy 18, trisomy 13)
Classification
A. Based on palpation (Kaplan-1993)
Impalpable: Accounts for 20% -30% of UDT.
• High canalicular
• Deep inguinal ring
• Intra-abdominal
Palpable: Accounts for 70% - 80 % of UDT
• Neck of scrotum
• Superficial inguinal ring
• Low canalicular
Classification contd
B. Based on exploration findings:
• intra-abdominal
• intracanalicular
• extracanalicular (suprapubic or infrapubic), or
• ectopic.
Pathological changes
• often macroscopically normal in early childhood but by puberty some
degree of atrophy occur..
• Microscopic evidence of tubular atrophy is evident by 5-6years of age,
& hyalinization is present by the time of puberty.
• loss of volume and progressive germ cell depletion starting at 6 months
of age
histologic changes include:
• decreased tubular diameter, and
• decreased numbers of Leydig cells,
• atrophy of Leydig cells
• degeneration of Sertoli cells
• Abnormal germ cell development
o Delayed disappearance of Gonocytes
o Delayed appearance of Adult dark spermatogonia
o failure of primary spermatocytes to develop, and
o reduced total germ cell counts
Clinical features
• Most patients presents in infancy and around school age.
• few present after puberty.
• Absence of one or both testes
• swelling in the groin (may be the testis or a hernia)
• May present with attacks of pain in the groin due either to recurrent torsion of the
testis or strangulation of an associated hernia
• gestational age at birth- usually preterm
• Determining if the testis was palpable in the scrotum at any time is important
• past history of inguinal surgery should be noted
• family history of cryptorchidism and other associated conditions.
Examination
• Patient should be warm and relaxed for the examination
• Observation should precede the examination.
• Supine and, if possible, upright cross-legged and standing positions.
• Abduction of the thighs contributes to inhibition of the cremaster reflex.
• Document testicular palpability, position, mobility, size, and possible
associated findings such as hernia, hydrocele, penile size, and urethral
position.
• Palpable Testes
• • Undescended testes may be located along the line of normal descent
between the abdomen and scrotum or in an ectopic position.
• • Ectopic:  Superficial inguinal pouch(m.c.)  Perirenal  Prepubic 
Femoral  Peripenile  Perineal  Contralateral scrotal
Nonpalpable testes
• Examination under anaesthesia is done for impalpable testis before
exploration
• When a testis is nonpalpable, possible clinical findings at surgery
include:
1. abdominal or transinguinal “peeping” location (25% to 50%),
2. complete atrophy (“vanishing” testis, 15% to 40%), and
3. extra-abdominal location but nonpalpable due to body habitus,
testicular size, and/or limited pts.’cooperation(10-30%).
Investigation
• Imaging
• Abdominal USS
• CT Scan
• MRI
Because imaging has not been proved to be reliable in demonstrating
whether the testis is present or absent, its routine use is discouraged
•Laboratory Investigations
• Karyotyping
• ↑ FSH- likely represent bilateral anorchia
• HCG Stimulation tests- has clinical use where gonadothrophins are
normal
• FBC, Urinalysis, Serum electrolytes
•Diagnostic Laparoscopy
Management of undescended testis
• Cryptorchid testis should be treated – between 6 month to 1 year of
age.
• 12-18 months – histological deterioration of the testis noted.
• Testis rarely descends – after 6 months.
• Surgical advantage to Orchiopexy- within 6 months specially in high
undescended testis.
• Hormonal therapy
hCG (human Chorionic Gonadotropin)
• Stimulate endogenous secretion of testosterone.
• Therapeutic dose – 1500 U/ m2 body surface area
• twice in a week for 4 weeks (FDA approved).
• Total dose should not exceed 15,000 units.
• Testicualar descent rate
• – 25% with hCG
• – 18 % with GnRH
LHRH- 1.2 mg/ day in divided doses intranasal for 4 weeks .
• • Testicular descent rate –about 20%.
• • Not FDA approved
• • Boserelin – superanalogue of LHRH
• – Small dose- 10μg every other day for 6 months.
• – Descent rate – 17%
•Surgical Management
• Palpable testes
one stage orchidopexy
• Non- palpable testes
• Laparoscopy / open
• High incidence of congenital inguinal hernia (hernia repair)
• Retractile or ectopic testes
Cremasterotomy
Inguinal Orchidopexy
Principles of orchidopexy
• Adequate exposure
• Herniotomy
• Mobilization of cord
• Fixation of testis
• Most commonaly performed – creation of subdartos pouch and placing
the testis.
• General anesthesia; useful to re-examine the child- previously
nonpalpable testis may become palpable.
• Groin crease incision is made Careful dissection to expose the external
oblique aponeurosis and the external ring
Cryptorchidism: A Guide for Urological Residents
Cryptorchidism: A Guide for Urological Residents

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Cryptorchidism: A Guide for Urological Residents

  • 1. Cryptorchidism Amir Saad Aljboory Urological resident Iraqi board urology Arabic board urology
  • 2. DEFINITIONS • Normal scrotal position: positioning of midpoint of the testis at or below midscrotum. • Undescended testis: absence of one or both testes in normal scrotal position. • Vanishing testes: present initially in development but are lost owing to vascular accident or torsion unilaterally (monorchia) or, very rarely, bilaterally (anorchia). Agenesis: testis that was never present and therefore associated with ipsilateral müllerian duct persistence. • Congenital cryptorchidism: testes that are extrascrotal at birth.
  • 3. • Recurrent cryptorchidism is when testes descend spontaneously postnatally but subsequently return to a nonscrotal position. • Testicular ascent or acquired cryptorchidism : Testes are intrascrotal at birth but subsequently identified in an extrascrotal position • Secondary cryptorchidism- testes that are suprascrotal after inguinal hernia repair; testicular retraction- as a complication of orchidopexy. • Retractile testes are scrotal testes that retract easily out of scrotum but can be manually replaced in a stable scrotal position and remain there at least temporarily.
  • 4. Introduction: • Development of the Testis Initial differentiation of male and female gonad development is dependent on: Presence of Y chromosome SRY gene ( protein product of the Y chromosome) steroidogenic factor 1 (SF1/Nr5a1) Sox9 (SRY box-containing 9) - Within the developing testis the three main differentiating cell types are: - -spermatogonia ( gamete forming cells) – - Sertoli cells (support cells) and -Leydig or interstitial cells (hormone secreting cells)
  • 5. Phases of testicular descent • Phase 1: 5 weeks - The caudal mesonephros contacts the future gubernaculum at the internal inguinal ring. • Phase 2: 7 weeks -The genitofemoral nerve accompanies the newly formed gubernaculum and processus vaginalis. • Phase 3: 10 to 12 weeks - Gubernaculum remains a thin cord in both sexes. Phase 3a: 12 to 14 weeks - The testis overrides the genital ducts and contacts the gubernaculum. • Phase 4: 14 to 20 weeks swelling of the gubernaculum, further development of the cremaster muscle, and migration of the processus vaginalis produce widening of the inguinal canal • Phase 5: 20 to 28 weeks - Release of the distal subcutaneous attachment of the gubernaculum and transinguinal passage of the testis. Phase 5a : after 7th month – Caudal movement of the testis, regression of the gubernaculum
  • 6. Regulation of testicular descent • Mechanical factors Intra-abdominal Pressure Gubernaculum tension Processus vaginalis patency • Growth factors Insulin-like 3 (INSL3) growth factor (Abdominal phase) Calcitonin gene related peptide (Inguinoscrotal Phase) Epidermal growth factor (EGF) may promote by activating the androgen responsive systems • Hormonal factors Testosterone (Inguinoscrotal Phase)
  • 7. Undescended testis • One of the most common pediatric disorders of male endocrine glands & Most common genital disorder identified at birth. Cryptorchidism: A greek word which means ‘hidden testis’ • Retractile- 60% • Undescended- 35% • Ectopic- 3% • Ascending- <2%
  • 8. Epidemiology & Risk factors • Incidence of Undescended testis 1–9% of full-term infants , by 1 year incidence is 0.8% 30% of premature infants Occurs on the right-50%, left-35%, bilateral-10-15% Prevalence reported to be (possibly) increasing • Risk factors • Maternal & Gestational Factors – Maternal Obesity – Low birth weight – Prematurity • Maternal smoking- small-to-moderate increased risk for cryptorchidism is present in offspring
  • 9. • Genetic Factors • 14% of cryptorchid boys – have positive family history. • Multifactorial pattern transmission • Father affected – 4% • Sibiling affected – 6-10 % • Gene mutation have identified -cryptorchidism – INSL3 – LGR8 – Androgen receptor polymorphism – HOXA10 – HOXD13 • Environmental Prenatal exposure - endocrine disrupters • – DES • – Pesticide (DDT) • – Nonylphenol • – Natural phytoestrogens • – Phthalates etc.
  • 10. •Associated conditions • Abdominal wall defects (eg, prune belly) • Neural tube defects (eg, myelomeningocele) • Cerebral palsy ~50% • Disorders of sexual development • Genetic disorders • • Diminished testosterone secretion (eg, Klinefelter) • • Diminished testosterone action (eg, Androgen insens.) • • Primary hypogonadism (eg, Noonan) • • Chromosomal disorders (eg, trisomy 18, trisomy 13)
  • 11. Classification A. Based on palpation (Kaplan-1993) Impalpable: Accounts for 20% -30% of UDT. • High canalicular • Deep inguinal ring • Intra-abdominal Palpable: Accounts for 70% - 80 % of UDT • Neck of scrotum • Superficial inguinal ring • Low canalicular
  • 12. Classification contd B. Based on exploration findings: • intra-abdominal • intracanalicular • extracanalicular (suprapubic or infrapubic), or • ectopic.
  • 13. Pathological changes • often macroscopically normal in early childhood but by puberty some degree of atrophy occur.. • Microscopic evidence of tubular atrophy is evident by 5-6years of age, & hyalinization is present by the time of puberty. • loss of volume and progressive germ cell depletion starting at 6 months of age
  • 14. histologic changes include: • decreased tubular diameter, and • decreased numbers of Leydig cells, • atrophy of Leydig cells • degeneration of Sertoli cells • Abnormal germ cell development o Delayed disappearance of Gonocytes o Delayed appearance of Adult dark spermatogonia o failure of primary spermatocytes to develop, and o reduced total germ cell counts
  • 15. Clinical features • Most patients presents in infancy and around school age. • few present after puberty. • Absence of one or both testes • swelling in the groin (may be the testis or a hernia) • May present with attacks of pain in the groin due either to recurrent torsion of the testis or strangulation of an associated hernia • gestational age at birth- usually preterm • Determining if the testis was palpable in the scrotum at any time is important • past history of inguinal surgery should be noted • family history of cryptorchidism and other associated conditions.
  • 16. Examination • Patient should be warm and relaxed for the examination • Observation should precede the examination. • Supine and, if possible, upright cross-legged and standing positions. • Abduction of the thighs contributes to inhibition of the cremaster reflex. • Document testicular palpability, position, mobility, size, and possible associated findings such as hernia, hydrocele, penile size, and urethral position. • Palpable Testes • • Undescended testes may be located along the line of normal descent between the abdomen and scrotum or in an ectopic position. • • Ectopic:  Superficial inguinal pouch(m.c.)  Perirenal  Prepubic  Femoral  Peripenile  Perineal  Contralateral scrotal
  • 17. Nonpalpable testes • Examination under anaesthesia is done for impalpable testis before exploration • When a testis is nonpalpable, possible clinical findings at surgery include: 1. abdominal or transinguinal “peeping” location (25% to 50%), 2. complete atrophy (“vanishing” testis, 15% to 40%), and 3. extra-abdominal location but nonpalpable due to body habitus, testicular size, and/or limited pts.’cooperation(10-30%).
  • 18. Investigation • Imaging • Abdominal USS • CT Scan • MRI Because imaging has not been proved to be reliable in demonstrating whether the testis is present or absent, its routine use is discouraged
  • 19. •Laboratory Investigations • Karyotyping • ↑ FSH- likely represent bilateral anorchia • HCG Stimulation tests- has clinical use where gonadothrophins are normal • FBC, Urinalysis, Serum electrolytes •Diagnostic Laparoscopy
  • 20.
  • 21. Management of undescended testis • Cryptorchid testis should be treated – between 6 month to 1 year of age. • 12-18 months – histological deterioration of the testis noted. • Testis rarely descends – after 6 months. • Surgical advantage to Orchiopexy- within 6 months specially in high undescended testis.
  • 22.
  • 23. • Hormonal therapy hCG (human Chorionic Gonadotropin) • Stimulate endogenous secretion of testosterone. • Therapeutic dose – 1500 U/ m2 body surface area • twice in a week for 4 weeks (FDA approved). • Total dose should not exceed 15,000 units. • Testicualar descent rate • – 25% with hCG • – 18 % with GnRH LHRH- 1.2 mg/ day in divided doses intranasal for 4 weeks . • • Testicular descent rate –about 20%. • • Not FDA approved • • Boserelin – superanalogue of LHRH • – Small dose- 10μg every other day for 6 months. • – Descent rate – 17%
  • 24. •Surgical Management • Palpable testes one stage orchidopexy • Non- palpable testes • Laparoscopy / open • High incidence of congenital inguinal hernia (hernia repair) • Retractile or ectopic testes Cremasterotomy
  • 25. Inguinal Orchidopexy Principles of orchidopexy • Adequate exposure • Herniotomy • Mobilization of cord • Fixation of testis • Most commonaly performed – creation of subdartos pouch and placing the testis. • General anesthesia; useful to re-examine the child- previously nonpalpable testis may become palpable. • Groin crease incision is made Careful dissection to expose the external oblique aponeurosis and the external ring