UNDESCENDED TESTIS

BYDr. Tufail khan

Dr. Gowhar Nazir MuftiAsst. Professor Deptt. Of

Pediatric surgery

OUTLINE Introduction Historical perspective Incidence Relevant Embryology /Anatomy Aetiopathogenesis Classification Pathology Presentation/Clinical feature Management Complications Conclusion

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

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%

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

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

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.

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 ).

ANATOMY

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.

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

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

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.

CMLCML

M G MG

MG

5mm 19mm 55mm

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.

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 ofcaudal gubernaculum holds testis close to

inguinal abdominal wall Relative descend of testis

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

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

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

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

Failure of any of these mechanisms may cause testicular non-descent or maldescent.

Hormonal (inguinoscrotal) phase is more commonly deranged

GERM CELL MATURATION 8 wk: gonocytes (fetal stem cells) 15 wk: spermatogonia 3 months of age: adult dark spermatogonia

(adult stem cells) appear and remain Neonatal surge in LH, FSH 4-5years: primary spermatocytes Puberty: spermatogenesis

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

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

CLASSIFICATIONA. 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

CLASSIFICATION CONTDB. Based on exploration findings: Intra-abdominal 34% Intracanalicular 27% Peeping testis (near DIR) 12% Extracanalicular 27% Ectopic. (Docimo et

al, 1995)

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

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

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 .

Ectopic testis: is located outside the normal path of descent. Most common site superficial inguinal pouch (anterior to rectus muscle)

ECTOPIC TESTIS

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

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.

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.

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.

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

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.

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

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.

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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.

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.

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)

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

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

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 successMultiple dosage schedules have been proposedSuccess rate low

HORMONAL REGIMINE

HCG GNRH PLACEBO SURGERY0%

10%20%30%40%50%60%70%80%90%

100%

Comparison of modalities

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

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

Principles of orchidopexy (originally described by Bevan in 1899)

Adequate exposure Herniotomy Mobilization of cord Fixation of testis

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.

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.

The external ring is opened.

Cremasteric fibers are dissected from the cord

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High ligation of the processus vaginalis at the internal inguinal ring.

The ligated processus and the cord structures

Separation of the internal spermatic fascia from the cord structures after ligation of the processus vaginalis

Formation of a dartos pouch

SUBDARTOS POUCH

Formation of a passage to the scrotum.

B and C, Passage of the testis into the scrotal pouch

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

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

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

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.

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.

LAPAROSCOPIC FOWLER STEPHENS

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.

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

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 :

Usually 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.

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)

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

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

Thank you

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