Cryptorchidism
Anton Sharapov
PGY4
Outline
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Anatomical considerations
Embryology
Definition of terms
Incidence
Significance
Diagnosis
Management
Unresolved issues
Case
2 month old boy with no testicles in the
scrotum
’ Options?
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Anatomical
considerations
Anatomical
considerations
Historical perspective
1786, John Hunter, found testes in
belly at 7th months, and in scrotum at
9th month
’ Coined term gubernaculum – a
ligament that guides the descent
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Embryology
Develop retroperitoneally
’ 3 weeks - germ cells from yolk sack
migrate to genital ridge
’ Testis determining factor, 7 weeks
’ 4-8 weeks – primordial testes form
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Embryology
bHCG -> Leydig cells secrete
testosterone
’ 9 weeks: scrotum, mesonephric
(Wolfian) duct develop
’ Epididymus, vas deference, seminal
vesicles develop.
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Embryology
Seroli cells secrete MIS and cause
involution of Mullerian Duct structures
’ Gonads migrate to internal Inguinal
ring under the influence of MIS
’ 12 week – processus vaginalis forms
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Embryology
Testes poised at opening of PV and
cleft btw ext and int oblique mm.
’ Intraabdominal pressure and
gubernaculum move testes through at
26-36 weeks
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Embryology
Second stage of migration is governed
by testosterone
’ sensory nucleus of the genitofemoral
n. controlled by androgen stimulation
’ releases CGRP (calcitonin gene
related peptide)
’ CGRP guides the gubernaculum
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Embryology
Gubernaculum – contractile ligament
’ Epididymis precedes the testes
’ Fusion of testis with post wall of
scrotum
’ Proximal mesonephric duct persists as
appendix epididymus
’ 37-40 weeks PV closes
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40% inguinal canal
25% prescrotal
10% abdominal
25% ectopic
Definition of terms
Undescended
’ Ectopic
’ Retractile
’ Ascended
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Undescended
Arrest of normal descend along normal
pathway
’ Intraabdominal, Inguinal, Prescrotal
’ Secondary to disruption of normal
hormonal influence on the
gubernaculum or its innervation
(genitofemoral n.)
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Ectopic
Abnormal destination of migration
’ Secondary to abnormal distal insertion
of gubernaculum – or so we think…
’ Will never descend…
’ The superficial inguinal location is by
far the most common.
’ Suprapubic, femoral, perineal,
contralateral position.
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Retractile
Cremaster m. spasm
’ Most common in 5-6 yo – Leydig cells’
function is at its minimum
’ Creamasteric reflex is inversely
proportional to androgen level.
’ … but 20% 12 yom have evidence of
those…
’ Not to be “treated”…
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Ascended
Refers to testes that, having reached
the scrotum, eventually rise up again
’ Two main theories
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Ascended
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 (CP patients)
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Incidence
Most cases present at birth
’ All races, all places
’ 30% prems
’ 3-5 % term
’ At 3 months to adults– 0.8%
’ Then no change
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Incidence
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80% palpable
– 25% retractile
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20% non-palpable
– 8% abdominal
– 8% retractile
– 2% canalicular
’ Significant
proportion is atrophic
’ In utero torsion
Incidence
Unilateral twice as common as
bilateral
’ R >L (L descends first)
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Natural History
Ectopics never descend
’ True undescended testicle rarely
descend spontaneously after 3 months
of age
’ Descent usually associated with rise in
androgens
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Natural History
Testicular ascent
’ Error in exam?
’ Controversial management
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Significance
Key – Prevention of potential tragedies
’ Testicular neoplasm
’ Subfertility
’ Testicular torsion
’ Inguinal hernia
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32 yom with abdo pain
Testicular cancer
Le Conte, 1851 first report
’ Observe higher then expected
incidence of germ cell cancers
’ 1:1000-2500
’ General population 1:100,000
’ Most common cancer 25-34 yoa group
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Testicular cancer
10% of cryptorchid testes undergo
degeneration
’ 10% of all seminomas are secondary
to cryptorchidism
’ Still, testicular cancer is relatively rare
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Testicular cancer
Level of risk does not warrant excision
of intraabdominal testes
’ 20% cancers in unilateral
cryptorchidism in normally descended
testicle
’ Hence, goal – proper monitoring
’ No role of biopsy
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Subfertility
Need 33 degrees C to thrive
’ Testicular-epididymal fusion
abnormality
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Subfertility
Lower sperm counts
’ Lower fertility rates
’ Worse with b/l involvement
’ Worse with increasing age at
orchidopexy
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Subfertility
Will fertilize but won’t support division
of embryonic cells
’ Germ cell density decrease beginning
at 1 yoa.
’ Infertility
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– 40% unilateral
– 70% bilateral cryptorchidism
Inguinal hernia
Patent processus vaginalis
’ Treated at the time of surgical
intervention
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Testicular Torsion
Little solid evidence
’ Usually as a result of tumor
’ Cause of “acute abdomen” when
located intraabdominally
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Diagnosis
History & Physical exam
’ Laboratory investigations
’ EUA
’ laparoscopy
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Diagnosis- imaging
Usually radiologic evaluation not
warranted
’ Not sensitive enough to detect
intraabdominal testes
’ Not specific enough to exclude
intraabdominal testes
’ Consider in obese and b/l ND
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Diagnosis
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Important to ID retractile type…
Important to ID associated anomalies
– Hypospadias
– Intersex states
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Mixed gonadal dysgenesis
True hermaphrodism
Important to ID other disorders
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Prader-Willi
Kallmann’s
Laurence-Moon-Biedl
Prune belly
Physical Exam
Relaxed warm room
’ Supine, legs elevated
’ Two hand technique
’ Retractile will stay in scrotum
momentarily with cremasteric reflex
’ Ectopic – won’t go to scrotum or will
spring back immediately
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Physical Exam
Physical Exam
Feels atrophic?
’ “Atrophic” tissue may represent
gubernaculum and dissociated
epididymus and vas deferens
’ may coexist with intrabdominal testicle
’ Hence don’t give on this testicle just yet
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Physical Exam
Physical Exam
Normal size
’ Before 12 yoa – 1-2 cm3
’ At 16 yoa – 14 cm3
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Unilateral
Establish viability (exam/lab)
’ Surgery or hormone
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Bilateral Non-palpable
Newborn phenotypical “male”
’ consider genetic female with CAH until
proven otherwise
’ Life threatening
’ rare
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Bilateral Non-palpablenewborn “male”
US pelvis – look for uterus
’ Karyotyping
’ Lytes/testosterone/MIH/LH/FSH
’ bHCG stimulation
’ Adrenal hormones/metabolites
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Bilateral Non-palpable –
older child
Evaluate for Testicular absence/atrophy
’ Testosterone, LH, FLS, MIS
’ Consider thyroid hormone/cortisol –
associated pituitary aplasia
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Bilateral Non-palpable –
older child
In the presence of normal
gonadotropin levels and detectable
MIS, ignore results of bHCG
stimulation
’ Proceed with surgical exploration
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Management - Goals
Ease of detection of testicular cancer,
not reduction of incidence
’ Preservation of fertility
’ Treatment of inguinal hernia
’ Prevention of torsion
’ Psychological benefits
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Management
Hormonal
’ Surgical
’ both
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Timing
Traditionally, if won’t descend by 1
year of age – explore/investigate
’ Adjust age if premature
’ Between 6 months and 2 years
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Management- medical
Descent hormonally mediated
’ Paracrine effect, hence can’t use
testosterone
’ Hence gonadotropins
’ bHCG, im, 1500-2000 u twice weekly
for four weeks. FDA approved.
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Management- medical
Greatest success with the most distal
’ Ectopics won’t respond by definition
’ High testes will ascend when hormone
is discontinued
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Management- medical
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Side-effects
– penile enlargement
– Pubic hair growth
– Increased testicular size
– Aggressive behavior during
administration
Management- medical
Some claim GhRH is more effective
’ Not FDA approved
’ At best 20% success rate…
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Management-surgical
Well established efficacy
’ Palpable –
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– open inguinal
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Nonpalpable –
– Open inguinal vs laparoscopic assisted
Techniques
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Simple Dartos (Koop) pouch technique
– Superficial pouch between scrotal skin
and Dartos fascia
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Multistage technique
Technique
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Fowler Stephens technique
– Division of main testicular pedicle
Microvascular technique (free flap)
’ Refluo technique
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– FS technique plus venous drainage
Laparoscopy
Laparoscopy
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TOP: Normal Vas
and spermatic
vessels, R, exit
from ring
Bottom: blind
vessel/vas.
Vanishing testes.
Laparoscopy
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Low intraabdominal
testicle
Can salvage
spermatic vessels.
Laparoscopy
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High testicle
Clip testicular
vessels
Complications
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Atrophy
– 8% failure rate even in distal
– >25% for intraabdominal
Ascent
’ Infection
’ Bleeding
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Follow up
No straddling activities for 2/52 post op
’ R/a early post op (sutures) and at least
3/12 post surgery for position and size
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Unresolved issues
Timing of surgery
’ Pathogenesis
’ Follow up of retractile testes
’ Many other…
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Literature
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6.
Docimo SG. The results of surgical therapy for
cryptorchidism: a literature review and analysis. J Urol
1995;154:1148-52.
Docimo SG, Silver R. The Undescended Testicle: diagnosis
and management. Am Fam Physician 2000, Nov 1, 2000
www.emedicine.com/radio/topic201.htm June 21, 2002
Ferrer and McKenna Current Approach to the undescended
testicle. Contemporary Pediatrics January 2000
Jawdeh, Akel. American University of Beirut, vol 1, No 3,
2002
www.projectlinks.org/cryptorchidism