GAURAV NAHARDNB UROLOGY

RESIDENT,MMHRC, MADURAI

UNDESCENDED TESTIS

INTRODUCTIONOne 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%

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.

DEFINITIONSNormal 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.

EPIDEMIOLOGYCryptorchidism 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.

ETIO-PATHOGENESISMultifactorial 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.

• Testicular descent occur as a result of a complex interactions of hormonal and mechanical factors

Hormonal factors:TestosteroneDihydrotestosterone Mullerian-inhibiting Substance(MIS/AMH)HCGGenital branch of genitofemoral nerve which

secret CGRP (elaborated by testosterone)Non androgen–insulin like factor 3(INSL-3)

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.

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.

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

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.

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.

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.

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.

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 herniaCerebral palsyArthrogryposisMyelomeningocele Omphalocele & GastroschisisImperforate anus Posterior urethral valveRenal and T10 to S5 spinal anomalies

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).

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.

Lifestyle factors may also interfere with testicular descent and function via hormonal or nonhormonal effects; ex. smoking is associated with cryptorchidism.

Presentation & Diagnosis

75% to 80%- palpable and60% 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.

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.

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?

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 HypospadiasIntersexualityPrecocious pubertyInfertilityConsanguinity

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.

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

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.

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%).

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.

Classification

A. Based on palpation (Kaplan-1993)Impalpable:High canalicularDeep inguinal ringIntra-abdominalAccounts for 20% of UDT.Palpable: Neck of scrotumSuperficial inguinal ringLow canalicularAccounts for 80% of UDT

Classification contd

B. Based on exploration findings: intra-abdominalintracanalicular extracanalicular (suprapubic or infrapubic), orectopic.

InvestigationImaging

Abdominal USSCT ScanMRIBecause imaging has not been proved to be

reliable in demonstrating whether the testis is present or absent, its routine use is discouraged

Laboratory InvestigationsKaryotyping↑ FSH- likely represent bilateral anorchiaHCG Stimulation tests- has clinical use

where gonadothrophins are normalFBC, Urinalysis, Serum electrolytes

Diagnostic Laparoscopy

Complications of Undescended testis

InfertilityAssociated herniao 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

Tumour: o 10% of testicular cancer originate in cryptochid

testis.TorsionEpididymo-orchitis in a cryptorchid right

testis can mimic appendicitisPsychologic effects of an empty scrotumTesticular-Epididymal fusion abnormality

ASSOCIATED PATHOLOGY

TESTICULAR MALDEVELOPMENT:Reduced total germ cell count

Impaired transformation of gonocytes to spermatogonia.

Delayed disappearance of gonocytes & appearance of Ad spermatogonia.

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.

OTHER ASSOCIATED TESTICULAR ANOMALIESPolyorchidismSplenogonadal fusionTransverse testicular ectopia

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.

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.

Surgical treatment

Surgery remains the gold standard.

OrchidopexyShould be performed as early as 6months

because of rarity of spontaneous descent after

6mnths possible improvement in fertilityInterval of 6months in bilateral undescended

testes.

Principles of orchidopexy (originally described by Bevan in

1899)

Adequate exposureHerniotomyMobilization of cordFixation of testis

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.

The external oblique aponeurosis is opened in line with the fascia

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.

The gubernaculum is divided

Patent processus is dissected off the vas and vessels.

A high ligation of the hernia sac is performed, and the remaining structures are skeletonised

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.

Superficial scrotal incision

Skin separated from dartos muscle

The testis is placed in a sub-dartos pouch.Fixation sutures to the testes nolonger

recommended

Subdartos pouch

Impalpable UDTsLaparoscopy -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

Impalpable UDTs contdIf 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

Intra-abdominal testis

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.

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.

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.

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.

6. Orchidectomy :

Reserved for postpubertal men with a contralateral normally positioned testis.

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.

Postoperative Complications

HaematomaInfectionUnsatisfactory position (requiring revision),Ilioinguinal nerve injuryDamage to the vasTesticular atrophyTorsion testis.

Outcome

Early orchidopexy may improve fertility

No evidence that it reduces risk of malignancy but allows early identification.

Thank You..