3. INTRODUCTION
One of the commonest surgical problems in males at
paediatric surgery clinic
Cause of cryptorchidism is multifactorial and it
exhibit wide variation in phenotypic expression
Has delitarious effects on the testis overtime when
left untreated
4. Cryptorchidism: A greek word which means ‘hidden testis’.It is
the absence of one or both testes in normal scrotal position
and during initial clinical evaluation.
• Normal scrotal position has been defined as positioning of
the midpoint of the testis at or below the midscrotum
(Wohlfahrt-Vejeet al, 2009)
• Although “high scrotal testes” are not routinely considered
undescended by most clinicians, they need observation and
may need surgical correction.
Retractile- 60%
Undescended- 35%
Ectopic- 3%
Ascending- <2%
5. Undescended testis: is arrested along its normal
path of descent
Ectopic testis : is located outside the normal path of
descent
Ascended (acquired cryptochidism): previously
descended, then “ascends” spontaneously
Testicular retraction (Secondry cryptochidism):
suprascrotal testis after inguinal exploration
Vanishing testis: present initially in development but
are lost owing to vascular accident or torsion, if it is
unilateral (monorchia), bilateral (anorchia).
Agenesis : never present and therefore associated
with ipsilateral mullerian duct persistence
6. HISTORICAL PERSPECTIVE
1786, John Hunter first drew attention to the mechanism
of descent (called as FATHER OF TESTICULAR
DESCENT) He found testes in belly at 7thmonths, and in
scrotum at 9thmonth and Coined term gubernaculum–a
ligament that guides the descent
1820, Rosenmerkal attempted the first surgical
orchidopexy but
1877, Annandale performed the first successful
orchiopexy
7. INCIDENCE/EPIDEMIOLOGY
1 to 4 % in term boys; 1 to 45% preterm neonates: by 1
year incidence is 0.8%
Occurs on the right-50%, left-35%, bilateral-10-15%
Approximately 40% of the nonpalpable testes are intra-
abdominal, 40% are inguinal, and 20% are atrophic or
absent
No definite racial differences in incidence are reported.
The familial cluster is 3.6-fold overall, 6.9-fold if a
brother is affected, and 4.6-fold if the father is affected.
The precise molecular and genetic mechanisms
underlying cryptorchidism in humans remain unknown.
8. INCIDENCE CONTD
Also more common in low-birth-weight newborns,
IUGR, and twin gestation.
Birth weight alone is the principal determinant of
cryptorchidism at birth and at 1 year of life,
independent of the length of gestation.
( Hjertkvist et al, 1989 ; Mayr et al, 1999 ).
10. VASCULAR SUPPLY
The 3 arteries- testicular, cremasteric and artery to the vas,
All anastomosed mainly at the head of epididymis hence ligation of the
testicular artery is not necessarily followed by testicular atrophy
(principle of stephen fowler’s surgery).
veins form pampiniform plexuses : condense into four veins at the SIR and
into two veins at the DIR ; later into single vein, they accompany testicular
artery and drain into IVC on the right side and into Left RENAL vein on the left
.
Lymphatic drainage: to the pre and para-aortic lymph nodes at the level of
the renal vessels (L2). Right side of lymphatic cross over to opposite side and
drain into contralateral lymph nodes.
NERVE SUPPLY
T10 sympathetic fibres via the renal and aortic plexus.
convey afferent (pain) fibres—hence referred pain from the testis to the loin.
convey efferent (vasomotor) to the blood vesels.
11. Spermatic cord
3 layers of fascia: external spermatic, cremasteric +cremaster muscle &
internal spermatic
3 arteries: testicular, cremasteric & artery to the vas
3 veins: pampiniform plexus of veins, cremasteric & vein of the vas
3 nerves — nerve to the cremaster from the genito-femoral nerve,
sympathetic fibres (T10–11 spinal segments) , & ilio-inguinal nerve (on and
not in the cord)
Scrotum
The skin of the scrotum is thin, pigmented & rugose
maintains a temperature 3-4⁰C lower than core body temperature
Its development depends on the descent of testis
Normally developed scrotum hang below on either side of midline raphe
12.
13. Embryology
The testes develop in the retroperitoneum
At 4 to 6 weeks' gestation, the genital ridges organize,
followed by migration of primordial germ cells
testicular differentiation is initiated in the 7th week by the SRY
gene
8 week testis is hormonally active
14.
15. Development of the cranial mesonephric ligament(CML) and
gonad (G) during embryonic regression of the mesonephros (M).
Asterisks denote the anlage of the diaphragm;long arrows show
the cranial mesonephric ligament.
CML
CML
M
G M
G
M
G
5mm 19mm 55mm
16. TWO STAGE DESCENT
Normal testicular descent occurs in two separate stages that have
different anatomy and hormonal control [Hutson and Hasthorpe, 2005].
After the onset of sexual differentiation in humans at 7–8 weeks’
gestation, the developing testis in the male secretes testosterone and
Müllerian inhibiting substance/anti-müllerian hormone (MIS/AMH).
MIS/AMH causes the Müllerian ducts to regress, so that the boy will not
form fallopian tubes, uterus and upper vagina (2/3rd).
The androgen is secreted in a probably exocrine manner down the
Wolffian duct and triggers its development into epididymis and vas
deferens, as well as formation of a distal bud to become the seminal
vesicle [Tong et al. 1996].
By contrast, in the female the lack of MIS/AMH allows Müllerian duct
development to proceed, to complete development into female internal
genitalia under the influence of maternal and fetal oestrogens. The
Wolffian duct regresses in the absence of androgens.
17. HUTSON AND HASTHORPE, 2005
TWO PHASES OF DESCENT
TRANSABDOMINAL PHASE : 8 TO 15 WKS
OF GESTATION
Primitive gonad in urogenital ridge turns into
testis by gene in short arm of Y chromosome
(SRY) around 5th to 6th;
Early Testis 3 hormones
1. Testosterone from Leydig cells
(CSL/CML regression)
2. Mullerian inhibiting substance from
Sertolli cells (Mullerian duct regression)
Insulin- like3 hormone Thickening of
caudal gubernaculum holds testis close to inguinal
abdominal wall Relative descend of testis
18. INGUINOSCROTAL PHASE : 28 TO 35 WKS
OF GESTATION
At 25 Wks Processus vaginalis elongates into gubernaculum
Distal end of gubernaculum elongates and reach scrotum between
30- 35 wks
Then testis descend through patent processus vaginalis
Testosterone GFN CGRP Migration of
gubernaculum along with testis to scrotum
19.
20. EMBRYOLOGY CONTD
By 28th weeks migrate through the inguinal canal
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
21. This descent occur as a result of a complex
interaction of hormonal and mechanical factors
Hormonal factors:
Testosterone
Dihydrotestosterone
Mullerian-inhibiting factor
Maternal HCG
Genital branch of genitofemoral nerve which secret
CGRP (elaborated by testosterone)
Non androgen–insulin like factor 3
22. Mechanical factors
Shortening and traction of the gubernaculum testis
Enlargement/elongation of processus vaginalis
Intra-abdominal pressure from increase visceral size
Straightening of fetus
Resolution of physiological hernia
Enlargement of testes/growth of epididymis
Propulsive force of the developing cremasteric muscle
23. Failure of any of these mechanisms may cause
testicular non-descent or maldescent.
Hormonal (inguinoscrotal) phase is more commonly
deranged
25. AETIOLOGY
Abnormal insertion of genital branch of genitofemoral
nerve
Poor intra-abdominal pressure: As in omphalocele,
diaphragmatic hernia, gastrochisis, Eagle-Barrett
syndrome, Beckwith-wiedeman’s syndrome, trisomies,
Extrophy of bladder
Retroperitoneal adhesions
Obstruction – adhesion at the deep ring
Short testicular vessel
26. AETIOLOGY CONTD
Hypothalamic-pituitary dysfunction>deficient
hormonal stimulation
Gubernaculum testis: abnormally inserted, poorly
or absence of extra-abdominal part ->> ineficient
pull
Defective folding
Severe intrauterine hypotonia
27. 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
28. CLASSIFICATION CONTD
B. Based on exploration findings:
Intra-abdominal 34%
Intracanalicular 27%
Peeping testis (near DIR) 12%
Extracanalicular 27%
Ectopic. (Docimo et al,
1995)
29. PATHOLOGICAL CHANGES
Often macroscopically normal in early childhood but by
puberty some degree of atrophy occur..
Microscopic evidence of tubular atrophy is evident by 5-6
years of age, & hyalinization is present by the time of puberty.
Loss of volume and progressive germ cell depletion starting at
6 months of age
Other histologic changes include:
Decreased tubular diameter, and
Decreased numbers of Leydig cells,
Atrophy of Leydig cells
Degeneration of Sertoli cells
30. PATHOLOGICAL CHANGES
CONTD
Abnormal germ cell development (Huff et al,1987)
Delayed disappearance of Gonocytes
Delayed appearance of Adult dark spermatogonia
Failure of primary spermatocytes to develop, and
Reduced total germ cell counts
31. Retractile testis:
Reduced androgens between 1-9yrs
Hyperactive cremasteric reflex
Often induced by cold, fear, pain
Stimulation of cutaneous branch of genitofemoral nerve.
Ascending testis:
Refers to testes that, having reached the scrotum,
eventually rise up again
Two main theories
• Ectopic but lax gubernaculum allow testes to drop into
scrotum, then pulls them out
• Failure of spermatic cord to elongate in proportion to body
growth +/-cremaster spasticity .
32. Ectopic testis: is located outside the normal path of descent. Most
common site superficial inguinal pouch (anterior to rectus muscle)
34. COMPARISON BETWEEN ECTOPIC &
UNDESCENDED TESTIS
Undescended testis
• The testis is arrested in its
normal path of descent
• Usually undeveloped
• Undeveloped & empty scrotum
on the affected side
• Shorter length of spermatic cord
• Poor spermatogenesis after 6
yrs
• Usually associated with indirect
inguinal hernia
• Treatment: surgery & HT
• Associated with a number of
complications
Ectopic testis
• The testis deviates from its
normal path of descent
• Fully developed testis
• Empty but usually fully
developed scrotum
• Longer length of spermatic
cord
• Spermatogenesis is perfect
• Never associated with indirect
inguinal hernia
• Treatment: basically surgical
• Complications: liability to injury
35. 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)
Presence of hypospadias. More proximal it is more
are the chances of UDT.
May present with attacks of pain in the groin due
either to recurrent torsion of the testis or strangulation
of an associated hernia.
36. CLINICAL FEATURES CONTD
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.
37. EXAMINATION
It include documentation of testicular palpability, position,,
mobility, size, and possible associated findings such as
hernia,hydrocele, penile size and urethral position.
Patient distraction, a warm room and hands, use of liquid soap
on the examiner’s hands and repeated examinations also help
to localise testis and to limit cremaster muscle activity
Abduction of the thighs contributes to inhibition of the cremaster
reflex
Begin examination of the UDT at the anterior superior iliac
spine and sweep the groin from lateral to medial with the
nondominant hand.
Maintain the position of the testis in the scrotum for a minute, so
that the cremaster muscle is fatigued.
38. EXAMINATION CONTD.
Release the testis and, if it remains in place, it is a retractile
testis. If it immediately pops back to a pre scrotal position, it is a
UDT.
For the difficult-to-examine patient (chubby 6 month-olds or
obese youths), the sitting cross-legged position can also help relax
the cremaster muscle.
Marked variation from the norm for height, weight & fat
distribution may suggest anorchia due to possible intersex or
pituitary deficiency
± Signs of syndromic features
Under developed scrotal skin with little or no rugae & appears
triangular in unilateral UDT or flat in bilateral UDTs
± hypertrophy of contralateral testis
39. EXAMINATION CONTD
Examination of potential ectopic sites- penile, femoral, &
perineal areas if the testicle cannot be felt.
If there is hypothalamic-pituitary dysfunction, the patient is
obese and the penis small for the age.
Examination under anaesthesia is also done for impalpable
testis before exploration
Clinical distinction between retractile and undescended testis
may be difficult, GOLD STANDARD is to carefully examine the
child in several positions and confirmation of incomplete descent
of the testis to a dependent scrotal position after induction of
anaesthesia.
40. CRYPTORCHIDISM IS ASSOCIATED
WITH:
inguinal hernia and/or patent processus vaginalis : 90%
hypospadias : 12 to 24%
cerebral palsy: 41 to 54%
mental retardation:
Down syndrome
Wilms tumor
Prune belly syndrome, and
Prader-Willi syndrome Prematurity
Low birth weight
Twin gestation
Gestational diabetes mellitus
Prenatal alcohol exposure
Hormonal abnormalities (fetus)
Toxic exposures in the mother
Mother younger than 20
A family history of undescended testes
41. Preterm and maternal history, including the use of
gestational steroids
Perinatal history, including documentation of a
scrotal examination at birth
The child's medical and previous surgical history
Family history of cryptorchidism or syndromes
All boys with nonpalpable testes and normal
serum gonadotropin levels must undergo
surgical exploration regardless of the results of
the hCG stimulation test.
41
42. The Human Chorionic Gonadotrophin (hCG)
Stimulation Test
HCG stimulation allows the identification of functioning testicular
tissue as well as biosynthetic defects in testosterone synthesis.
A weight-based single injection of hCG (100 IU/kg) is usually
sufficient to detect a rise in serum testosterone 4–5 days later.
A testosterone response to hCG may be labelled as normal if absolute
testosterone concentrations reach a level that is above the upper limit
of the normal prepubertal range, or rise by more than twice the
baseline value
A failure to see a measurable increase in testosterone in
combination with elevated LH and FSH is consistent with the
diagnosis of anorchia.
43. INVESTIGATION
Imaging
Abdominal USS ( done in case of chubby/obese
patients or ones not allowing to examine
MRA / MRV (for localisation of testicular vessels).
MRI may be identification of an ectopic abdominal
testis not localised by laproscopy
Because imaging has not been proved to be reliable in
demonstrating whether the testis is present or absent, its
routine use is discouraged ,and it did not influence the
management decision.
44. Laboratory Investigations
Karyotyping: when DSD(Disorders of sexual differentiation ) is
suspected, like when neither testis is palpable and also in boys with
associated hypospadias, especially proximal hypospadias chromosomal
and endocrinologic evaluation should be carried out
↑ FSH- likely represent bilateral anorchia.
HCG Stimulation tests- has clinical use where gonadothrophins are
normal
The levels of harmones should be drawn during the newborn
gonadotropin surge or at 60–90 days of life.
Diagnostic Laparoscopy (GOLD STANDARD)
45. COMPLICATIONS OF UNDESCENDED
TESTIS
Infertility
Associated hernia
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
46. COMPLICATION CONTD
Tumour:
10% of testicular cancer originate in cryptochid testis.
Torsion
Epididymo-orchitis in a chryptochid right testis
can mimic appendicitis
Psychologic effects of an empty scrotum
Testicular-Epididymal fusion abnormality
47. TREATMENT
A. Hormonal
Indications
Bilateral UDT
Hypothalamic-pituitary dysfunction
Patients unfit for surgery
When diagnosis of retractile testes is uncertain
LHRH and hCG are used with varying degrees of
success
Multiple dosage schedules have been proposed
Success rate low
50. ADVERSE EFFECTS OF
HORMONAL THERAPY:
Increase in scrotal rugae, pigmentation
Growth of pubic hair
Increased penile size
Priapism
Premature closure of epiphyseal plate (short
stature)
Increased appetite and weight gain
51. SURGICAL TREATMENT
Surgery remains the gold standard
Orchidopexy
Should be performed as early as 6months because
rarity of spontaneous descent after 6 months
Possible improvement in fertility
Interval of 6months in bilateral undescended testes
53. STANDARD ORCHIOPEXY.
The key steps in this procedure are ---
complete mobilization of the testis and spermatic
cord,
Repair of the patent processus vaginalis by high
ligation of the hernia sac,
Skeletonization of the spermatic cord without
sacrificing vascular integrity to achieve tension-
free placement of the testis within the dependent
position of the scrotum, and
Creation of a superficial pouch within the
hemiscrotum to receive the testis.
54. ORCHIDOPEXY FOR THE
PALPABLE UDT
General anesthesia; useful to re-examine the child
previously nonpalpable testis may become palpable.
A transverse inguinal skin incision is made in the
midinguinal canal, usually in a skin crease in children younger
than 1 year
The dermis is opened with electrocautery, and subcutaneous
tissue and Scarpa's fascia are opened sharply.
The skin and subcutaneous tissue are quite elastic in younger
children and allow for a tremendous degree of mobility by retractor
positioning for viewing the entire length of the inguinal canal.
Careful dissection to expose the external oblique
aponeurosis and the external ring.
60. Formation of a passage
to the scrotum.
B and C, Passage of
the testis into the scrotal
pouch
61.
62. COMPLICATIONS OF ORCHIOPEXY
Testicular retraction,
Hematoma formation,
Ilioinguinal nerve injury,
Postoperative torsion (either iatrogenic or
spontaneous),
Damage to the vas deferens, and
Testicular atrophy
Devascularization with atrophy of the testis can result
from skeletonization of the cord, from overzealous
electrocautery
63. IMPALPABLE UDTS
laparoscopy -best means Size,position and length of the
spermatic vessels and vas ; testicular size ,position and quality;
patency of internal inguinal ring, testicular-epididymal fusion
abnormalities, associated anamolies,
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
64. IMPALPABLE UDTS CONTD
If neither vas nor spermatic artery is found at the time of laproscopy ,
laproscopic or surgical dissection of the paravesical area and retroperitoneum
upto the level of the kidney is required to exclude the presence of a testis
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.
During laproscopy if length is found adequate ( means testis can be taken
upto the DIR on the other side) one stage lap. Orchidopexy can be
proceeded.
If length is inadequate staged procedure ( FOWLER STEPHEN’S) is
planned
65. Manoeuvres to gain sufficient length include:
Dissection of retroperitoneal attachments of the cord
Prentiss manoeuvre
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.
66.
67.
68. 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 around 6 months, when
collaterals have formed), the testis is brought down on
a wide pedicle of peritoneum containing the remaining
vessels.
72. 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.
73. 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
74. 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
75. 6. ORCHIDECTOMY :
Usually reserved for postpubertal men with a
contralateral normally positioned testis.
76. 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.
77.
78.
79.
80. OUTCOME
Early orchidopexy may improve fertility
No evidence that it reduces risk of malignancy but allows early
identification.
Even after surgery patients should be kept on follow up, councelled
regarding fertility issues and risk of malignancy
Testicular self-examination: which requires education and
counseling of the patient, remains a mainstay of testicular
cancer screening. It should be taught to all patients with a
history of cryptorchidism after they reach puberty available on
(http://www.cwpeds.com/pdfs/adolescents/TesticularExam.pdf)
81. UDT IN ADULTS
• Orchiedectomy is to be done in any patient presenting with
undescended testis after puberty (both palpable and non-palpable) as
testicle had alredy atrophied and risk of malignancy increases which is
around 1: 2500 (compared to 1:100000 in normal pop.)
• Both seminoma and non-seminomatous germ cell tumour develop
from CIS (precursors are gonocytes) also called as Intra Tubular Germ
Cell Neoplasia,Unclassified (ITGCNU)
• Seminoma is associated with persistently cryptorchid(abdominal)
testes (74%) and nonseminoma is present in the majority of scrotal
testes (63%) (Wood and Elder,2009).
• In non-palpable cases diagnostic lap followed by orchiedectomy is to
be done even if testis seem to be of normal size
• Patienta should be councelled and prosthesis should be offered
82. CONCLUSION
Undescended testis is relatively common and early
surgical correction should be done to prevent
complications
Awareness should be made among the masses so
that they will not present in later life with complications
and will get it corrected in the early life