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.
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