2. INTRODUCTION
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%
3. HISTORICAL PERSPECTIVE
1786: Hunter first drew attention to the mechanism
of descent.
1820: Rosenmerkal attempted the first surgical
orchidopexy but,
1877: Annandale performed the first successful
orchiopexy.
4. 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).
5. 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.
6. 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.
7. EPIDEMIOLOGY
Cryptorchidism is one of the most common
congenital anomalies.
1% to 4% of full-term and 1% to 45% of preterm male
neonates.
a component of over 390 syndromes.
familial cluster is 3.6-fold overall, 6.9-fold if a
brother is affected, and 4.6-fold if the father is
affected.
8. ETIO-PATHOGENESIS
Multifactorial pathogenesis.
Birth weight is the principal determining factor, at
birth to age one year, independent of the length of
gestation.
Premature infants- 30%
More common in low-birth-weight male newborns,
IUGR, and twin gestation.
9. • Testicular descent occur as a result of a complex
interactions of hormonal and mechanical factors
Hormonal factors:
Testosterone
Dihydrotestosterone
Mullerian-inhibiting Substance(MIS/AMH)
HCG
Genital branch of genitofemoral nerve which secret
CGRP (elaborated by testosterone)
Non androgen–insulin like factor 3(INSL-3)
10. Mechanical factors
Shortening and traction of the gubernaculum testis.
Enlargement/elongation of processus vaginalis.
Intra-abdominal pressure from increased visceral size.
Straightening of fetus.
Resolution of physiological hernia.
Enlargement of testes/growth of epididymis.
Propulsive force of the developing cremasteric muscle.
11. Testicular Descent
Testicular descent occurs in two phases- transandominal
& transinguinal.
INSL3(Insulin-like 3, Leydig cell origin) & Testosterone-
key hormones required for testicular descent.
Transabdominal descent involves differential growth of
vertebrae and pelvis until 23 weeks’ gestation. Afterward
facilitated by the development of the gubernaculum,
processus vaginalis, spermatic vessels, and scrotum.
A normal hypothalamic-pituitary-gonadal (HPG)axis is a
prerequisite for testicular descent.
12. Testosterone and its conversion to
dihydrotestosterone (DHT) are also necessary for
continued migration, especially during the
inguinoscrotal phase.
Release of calcitonin gene-related peptide (CGRP)
from genitofemoral nerve stimulates development
and function of the gubernaculum.
Enlargement, distal detachment and migration of the
gubernaculum are key events that facilitate and
direct caudal movement of the testis
13. Intra-abdominal pressure also appears to play a role
in testicular descent most significant during
transinguinal migration to the scrotum, probably in
conjunction with androgens and a patent processus
vaginalis.
14. Transabdominal descent complete by 10 weeks.
Traverses inguinal canal between 20-28 weeks.
32nd week – emerges from superficial inguinal ring.
35-40th week –descends into the scrotum.
Left testis descends before the right.
About 96% of testes have descended at birth.
15. Nonsyndromic Congenital Cryptorchidism
Perinatal risk factors associated with cryptorchidism
include prematurity, low birth weight/small for
gestational age, breech presentation, and maternal
diabetes.
Extrascrotal testes - much less likely to descend by 1
year of age (50%) than high scrotal testes defined as
cryptorchid at birth.
Spontaneous descent is more likely and may occur
later in premature Infants.
16. Syndromic Cryptorchidism
Undescended testes are frequently present in
diseases associated with reduced androgen
production and/or action, such as androgen
biosynthetic defects, androgen insensitivity, Leydig
cell agenesis, and gonadotropin deficiency disorders,
AMH biosynthesis or receptor defects.
Most commonly bilateral.
17. Certain anomalies are associated with increased risk of
cryptorchidism: Musculoskeletal, central nervous
system( CNS), or abdominal wall/gastrointestinal defects
include
Classic prune-belly (triad or Eagle-Barrett) syndrome;
Spigelian hernia & Umbilical hernia
Cerebral palsy
Arthrogryposis
Myelomeningocele
Omphalocele & Gastroschisis
Imperforate anus
Posterior urethral valve
Renal and T10 to S5 spinal anomalies
18. Genetic Susceptibility
Polygenic & multifactorial.
Most probable mode of inheritance- autosomal
dominant with reduced penetrance.
INSL3, its receptor, relaxin/insulin-like family
peptide receptor 2 (RXFP2), HOXA10, and HOXA11-
most likely candidate genes for human
nonsyndromic cryptorchidism(mouse models).
19. Environmental Risk Factors
Exposure to antiandrogenic and/or endocrine-
disrupting chemicals(EDCs) may contribute to
cryptorchidism.
EDCs include phthalates, pesticides, brominated
flame retardants, diethylstilbestrol, and dioxins.
A subset of boys with cryptorchidism have
measurable abnormalities in pituitary and/or
gonadal hormone secretion during infancy without
syndromic endocrine dysfunction.
20. Lifestyle factors may also interfere with testicular
descent and function via hormonal or nonhormonal
effects; ex. smoking is associated with
cryptorchidism.
21. Presentation & Diagnosis
75% to 80%- palpable and
60% to 70% are unilateral;
involvement of the right side is more common
overall but less frequent in series of nonpalpable
testes.
8% of testes-abdominal, 63% canalicular, 24%
prescrotal, and 11% in the superficial inguinal pouch
or ectopic.
22. Clinical features
Most patients presents in infancy and around school age.
A 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.
23. HISTORY: should cover the following questions:
Has the testis ever been palpable in the scrotum?
Was the patient born prematurely?
Has the patient undergone prior inguinal surgery?
Is or was the patient's mother on a vegetarian diet?
Was the patient fed soy formula during infancy?
What was the patient's birth weight?
24. PRENATAL HISTORY:
Did the patient's parents used an assisted
reproductive technique?
Did his mother receive hormonal treatment?
Were there multiple gestations?
FAMILY HISTORY:
Cryptorchidism
Hypospadias
Intersexuality
Precocious puberty
Infertility
Consanguinity
25. PHYSICAL 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.
26. 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
27. Gold standard for diagnosis remains careful
examination of a child in several positions and
confirmation of incomplete descent of the testis to a
dependent scrotal position after induction of
anesthesia.
28. Nonpalpable testes
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%).
29. Diagnosis of a vanishing testis requires
documentation of blind-ending spermatic vessels in
the abdomen, inguinal canal, or scrotum.
Endocrine evaluation in cases of suspected bilateral
vanishing testis (anorchia) include elevated basal
serum gonadotropin levels and no response to hCG
stimulation.
30. Classification
A. Based on palpation (Kaplan-1993)
Impalpable:
High canalicular
Deep inguinal ring
Intra-abdominal
Accounts for 20% of UDT.
Palpable:
Neck of scrotum
Superficial inguinal ring
Low canalicular
Accounts for 80% of UDT
31. Classification contd
B. Based on exploration findings:
intra-abdominal
intracanalicular
extracanalicular (suprapubic or infrapubic), or
ectopic.
32. 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
33. Laboratory Investigations
Karyotyping
↑ FSH- likely represent bilateral anorchia
HCG Stimulation tests- has clinical use where
gonadothrophins are normal
FBC, Urinalysis, Serum electrolytes
Diagnostic Laparoscopy
34. Complications of Undescended testis
Infertility
Associated hernia
o indirect inguinal hernia usually accompanies a
congenital undescended testis in about 90% cases but
rarely symptomatic.
Testicular atrophy: due to pressure effects and
histological changes.
Trauma
35. Tumour:
o 10% of testicular cancer originate in cryptochid testis.
Torsion
Epididymo-orchitis in a cryptorchid right testis
can mimic appendicitis
Psychologic effects of an empty scrotum
Testicular-Epididymal fusion abnormality
36. ASSOCIATED PATHOLOGY
TESTICULAR MALDEVELOPMENT:
Reduced total germ cell count
Impaired transformation of gonocytes to
spermatogonia.
Delayed disappearance of gonocytes & appearance of
Ad spermatogonia.
37. ANOMALIES OF EPIDIDYMIS, PROCESSUS
VAGINALIS & GUBERNACULUM:
Anomalies of fusion between the caput and/or cauda
epididymis, elongation and/or looping, and atresia.
Failure of closure of processus vaginalis &
Aberrant lateral attachment of gubernaculum.
39. TREATMENT
GOALS of treatment:
to optimize testicular function,
potentially reduce and/or facilitate diagnosis of
testicular malignancy,
provide cosmetic benefits, and
prevent complications such as clinical hernia or
torsion.
40. Observation is indicated for the first 6 postnatal
months to allow spontaneous testicular descent.
If descent does not occur in the postnatal period
surgical treatment at 6 months of age.
41. Surgical treatment
Surgery remains the gold standard.
Orchidopexy
Should be performed as early as 6months because of
rarity of spontaneous descent after 6mnths
possible improvement in fertility
Interval of 6months in bilateral undescended testes.
43. Orchidopexy for the palpable UDT
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.
44.
45. The external oblique aponeurosis is opened in line with
the fascia
46. Rolling the cord structures under a finger may help
confirm the exact site of the canal.
Care inside the canal is taken to identify and preserve
the ilioinguinal nerve.
The cord is isolated by sweeping the cremasteric
fibres off it.
49. A high ligation of the hernia sac is performed, and the
remaining structures are skeletonised
50. Manoeuvres to gain sufficient length include:
Dissection of retroperitoneal attachments of the cord .
Divide (or pass the testis under) the inferior epigastric
vessels after opening the floor of the canal (transversalis
fascia), allowing a more medial and thus direct route to
the scrotum.(Prentiss manoeuvre).
Cranial extension of the incision.
55. Impalpable UDTs
Laparoscopy -best means of identifying intra-
abdominal testis, vas and vessels.
If laparoscopy indicates blind-ending gonadal vessels
and vas deferens, the patient is said to have vanishing
testis syndrome and no further action is necessary
56. Impalpable UDTs contd
If intra-abdominal testis identified, consider staged
orchidopexy or microvascular transfer.
If vas vessels seen entering inguinal canal, the groin
should be explored.
The length of the gonadal vessels is the limiting factor to
getting the intra-abdominal testis into the scrotum
58. Options for intra-abdominal UDT
1. Standard inguinal orchidopexy(a high failure rate)
2. A two-stage Fowler-Stephens orchidopexy (open
or laparoscopy).
The testicular artery is sacrificed.
The rationale is that the testicular arterial supply comes
from three sources.
At a 2nd stage (after 6 months of age, when collaterals
have formed), the testis is brought down on a wide
pedicle of peritoneum containing the remaining vessels.
59. Options for intra-abdominal UDT contd
3. Microvascular testicular
autotransplantation
employs microsurgical techniques.
reserved for older children with internal spermatic
artery large enough to be anastomosed to inferior
epigastric artery.
60. 4. Refluo Testicular
Autotransplantation
Provides only venous drainage by microvascular
anastomosis of testicular veins to inferior epigastric
veins
Based on discovery that failure in Fowler-Stephens
was due to testicular congestion
Reduced operating time and increased success.
61. 5. Jones Preperitoneal Approach
Preperitoneal cavity accessed by splitting
abdominal obliques.
Testes mobilized transperitoneally and passed to
the scrotum through the inguinal canal or
posterior wall.
63. Bilateral impalpable testis
Raise suspicion of an intersex condition.
Karyotype and hormonal profile should be characterized
Can involve measurement of MIS or an HCG stimulation
test to detect the presence or absence of functioning
testicular tissue.
64. Postoperative Complications
Haematoma
Infection
Unsatisfactory position (requiring revision),
Ilioinguinal nerve injury
Damage to the vas
Testicular atrophy
Torsion testis.
65. Outcome
Early orchidopexy may improve fertility
No evidence that it reduces risk of malignancy but allows
early identification.