OPEN ACCESS TEXTBOOK OF GENERAL SURGERY

UNDESCENDED TESTIS & AJW MILLAR RA BROWN

UNDESCENDED TESTIS the gonad to the developing inguinal Incomplete testicular descent is one of region and future . the most common malformations Meanwhile the processus vaginalis encountered in the male. Controversy testis, an outpouching of the remains regarding the aetiology, invades the pathophysiology and optimal . Testicular descent treatment. Testicular development from its intra-abdominal position, and descent is a complex event and is through the takes place effected by multiple factors, during the 8th intra-uterine month with mechanical and hormonal, for its concomitant increase in the length of normal completion. Abnormalities in the and testicular one or more of these can lead to an vessels. abnormal final gonadal location. The left testis is said to precede the Definition right in descent. An undescended testis must be After descent the bulky gelatinous regarded as a testis that is not in the gubernaculum shrinks to thin fascial scrotum, was never there and cannot attachments. be brought down into the scrotum. Hormonal factors play a major role in Embryology: Development and descent, e.g. maternal chorionic descent. Testicular development is gonadotrophic hormones and foetal determined at the time of conception interstitial cell activity with androgenic by the presence of the SRY (sex- hormone production. Other hormones determining region on the short arm of implicated are: the Y chromosome). Sexual · Descendin. an androgen differentiation begins at 7-8 weeks independent factor that provides gestation when the developing testis gubernacular cellular growth and arising from the genital ridge secretes · Estradiol. high levels in mothers testosterone (Leydig cells) and may inhibit gubernacular growth. Mullerian inhibiting substance (MIS) (Sertoli cells). Testosterone allows the Man is the only animal in which the Wolffian ducts to develop into an testis descends before birth and with , vas deferens and seminal the chimpanzee are the only 2 in vesicle, while MIS causes regression which the testis resides permanently in of Mullerian duct structures. Distal to the scrotum. The current concept of the developing gonad the the hormonal control of testicular gubernaculum proliferates, connecting descent is that transabdominal

descent is mediated by gubernaculum structures of testicular parenchyma enlargement under the control of MIS. become apparent and become more Inguinoscrotal migration is indirectly abnormal as the malposition persists. under testicular control through The changes are present in bilateral androgen secretion. Disturbance of and unilateral undescended testis, and the hypothalamo-pituitary testicular in the latter situation, also in the axis will interfere with the androgen contralateral scrotal testis. In dependent transinguinal descent to a summary there is decreased tubular variable extent. size, decreased number of spermatogonia, atrophy of Leydig cells Aetiology of incomplete descent and interstitial fibrosis. Orchidopexy Failure of descent is likely to stem should be done early, before changes from two factors: become irreversible and theoretically · Inherent defect in the testis, or this should improve maturation after failure of hormonal environment the testis is brought down. (vide supra) · Mechanical defect preventing the Diagnosis otherwise normal testis from descending correctly, as genital Undescended testis must be development is usually normal. differentiated from a retractile testis Occasionally a malpositioned testis where there is: is noted which is not really `undescended', but has been 1. a history of a testis being felt or hitched up out of the scrotum seen in the scrotum following herniotomy. 2. a normally developed scrotum on that side Side: R/L 55%/45% 3. a testis of normal size that can Bilateral: 15-20% be manipulated into the scrotum Incidence Preterm infant 21% 75% of undescended testes referred Full-term 2.7% for a specialist opinion are retractile Age one year 1% NOTE that both squatting and the Family history 14% `chair test' where the knees are pulled up against the chest and the patient is The testis should be in the scrotum of examined relax the a full-term baby. If not, then some allowing a retractile testis to be spontaneous late descent may occur manipulated into the scrotum. The in two-thirds of full-term babies by 6 cremaster reflex is absent in the weeks of age, and in 3 months for newborn and maximum at age 7-8 premature infants. Minimal descent years. occurs after one year. Types Histology of Undescended Testes a. Incomplete descent - arrested Normally age dependent in its normal line of descent morphological changes are seen from b. Ectopic - deviated from its infancy to adulthood. These changes normal line of descent after are caused by a congenital defect or traversing the external ring. by secondary alteration due to the malposition. Morphological changes Incomplete descent are not seen within the first 12 months · Abdominal - between the lower of life. Beginning with the second pole of the kidney and the year histological changes involving all internal ring

· Entrant inguinal - above the The `Ascending' testis internal ring, but may enter the inguinal canal (impalpable) An apparently normally descended · Inguinal - lying in the inguinal testis in infancy but is later (± 4 yrs) canal (usually impalpable) noted to be incompletely descended. · Emergent inguinal - can be This is thought to be due to partial milked from the inguinal canal absorption of the patent processus to emerge from the external vaginalis into the parietal peritoneum ring when it becomes palpable. in the first years of life. · High scrotal - testis cannot be manipulated to the bottom of Treatment the scrotum, and lies at the entry to the scrotum. Orchidopexy - that is mobilising the testis, its vascular supply with vas Ectopic deferens and placing it in the scrotum · Superficial inguinal ectopic - usually in a sub- pouch. Age at lies in the superficial inguinal operation: approximately 2 years. In pouch lateral to the external bilateral impalpable testis plus atrophic ring and scrotum - evidence of · Pubopenile - at the base of the testicular tissue should be proven prior penis to operation (Testosterone levels with · Perineal - in the perineum HCG Stimulation Test). Laparoscopy outside the scrotum is an excellent method of identifying the presence or absence of an intra- · Femoral - in the upper thigh abdominal testes when it is

impalpable. Clinical examination

Reasons for operating Should be conducted in a warm room having gained the confidence of the 1. To improve fertility. patient. Look first for development or Unilateral undescent - 80% fertile abnormality of the genitalia. Warm (after orchidopexy) hands. Gently bring the hand down Bilateral undescent - 30% fertile from the ASIS towards the to (after orchidopexy) ?less for intra- milk the testis out of the inguinal canal. abdominal testis If the testis is visible in the it is 2. Torsion is more likely in an probably ectopic. undescended testis

(10% torsion occur in UDT) If the testis is difficult to feel or pick up 3. Prone to trauma (uncommon) between the fingers, it is probably in 4. 70% associated with patent the inguinal canal. processus vaginalis therefore

predisposes to hernia The Impalpable Testis 5. Psychological When a testis is impalpable, it may be 6. Malignant potential 20-50 times · An incompletely descended testis greater with abdominal testis: lying in the or inguinal approximately 6-10 times greater canal. in inguinal testis · Testicular agenesis Note in 20% of contralateral · `Vanished' testis - vas and vessels descended testes in unilateral are present, but the testis is absent undescent, dysplastic change or replaced by fibrous tissue. shown. Therefore slight increased Probably due to a vascular incidence in malignancy of the accident to the testis, idiopathic `normal' testis. infarction or antenatal torsion.

Hormonal treatment Incidence

Either HCG or LHRH or both have Approx. 1-3%, (4% in premature been used. Little benefit is gained in births) truly undescended testes. Most Male/female ratio : 6:1 testes, which have descended with 60% right-sided presentation, 30% left- hormonal therapy, have been high sided scrotal or retractile testes. 10-15% bilateral presentation The hernia most frequently comes to notice in the first year of life and particularly during the first three A hernia is defined as the protrusion of months. Direct and femoral in a viscus from the cavity in which it children are extremely rare. normally resides. Clinical evidence: Bowel containing Indirect inguinal hernia is a hernia, hernia which emerges through the internal inguinal ring within the cremasteric The symptoms and signs of inguinal extending down the inguinal hernia vary with the type, size, canal for various distances. duration and presence of complications. In the groin of a child, If the hernia remains within the canal, a mass, which comes and goes with it is incomplete. If it extends beyond straining, must be a hernia. At times, the external ring, into the scrotum the it is necessary to base the diagnosis hernia is complete. on a reliable history alone. Symptoms referable to hernia, eg. a feeding Direct inguinal hernia. problem, pain, irritability, or colic, may This is a hernia presenting in the be the first indication of a hernia. triangle of Hesselbach. There is disruption of the posterior wall of the On palpation inguinal canal medial outside the resulting from Thickening of the cord, a reducible insufficiency of the internal oblique tense fluctuant cylindrical or globular muscle and transversus abdominis inguino-scrotal swelling with a blunt, muscle or in this area. rounded shape and sometimes with an Very uncommon in paediatric patients. expansile impulse when the abdominal pressure is increased. Ability to During embryonic development the reduce the swelling frequently with a processus vaginalis testis (canal of gurgling sensation. In infants the Nuck in a female), invades the internal ring is palpable on rectal gubernaculum. The processus examination and this test may be used vaginalis usually becomes obliterated. to confirm reduction of a hernia In a considerable number of infants following an episode of irreducibility. failure of obliteration occurs in part or in toto, with a persistent peritoneal Congenital hydrocoele pouch. It is this sac of peritoneum, which is the congenital predisposition Here, the fluid formed by the serosa of essential for the development of an the peritoneal cavity trickles down a indirect inguinal hernia in life. At the narrow patent processus vaginalis and time of birth the sac is open in 80-94% collects in the tunica, around the testis. of male infants. At the end of 2 years Clinically, one can get above it and it 56% remain open and in adults the transilluminates. An encysted patency rate varies between 15-35%. hydrocoele of the cord is where fluid is Once a hernia has occurred trapped within the tunica above the spontaneous resolution is not possible. testis.

Fluid hernia CAUSE: due to enzymatic defect so that testosterone cannot exert This implies the presence of a wider its effect on the target organ with patency of an attenuated processus incomplete masculinization. vaginalis and usually presents in the toddler with increasing scrotal swelling during the day, which settles Management of androgen overnight. Sometimes it is difficult to insensitivity distinguish from a bowel containing hernia but it is usually confined to the · Herniotomy - if hernia contains a scrotum, is a non-tender, gonad – biopsy transilluminable fluid collection around · Gonadectomy (controversial as to the . whether gonadectomy should be done in infancy Exclude undescended testis in boys · Provide endocrine function with a suspected hernia (6%). artificially at puberty

Inguinal hernias in girls Complications

Very similar to the male with a few Irreducibility/Obstruction/Strangulat exceptions. ion Complications may be serious and the 1. Incidence of complications higher incidence is inversely related to age. 2. 25% of hernias are sliding hernias, The younger the child the more likely it where the broad ligament, bladder is for the hernia to become or vascular pedicle make up part of complicated - 30% present with the wall of the sac irreducibility. 3. The ovary and commonly (>75% at RXH) 1. Irreducibility may be the first prolapse into the hernial sac. This manifestation of a hernia with 60% may present as an `irreducible' of them occurring within the first hernia, but without bowel three months of life. This provides symptoms. Torsion of the ovary in further strong support for the trend the sac may occur towards repairing hernias when 4. Always the possibility of a they are diagnosed regardless of testicular feminizing syndrome age. (Androgen insensitivity) viz: 2. Obstruction: Evidence of Incidence 1% of `girls' with inguinal gastrointestinal tract involvement hernia (cramping pain, bile stained Hernias are usually bilateral and vomiting and abdominal contain gonads distension) together with groin mass. Phenotype - female 3. Strangulation: this implies External genitalia - female, vascular compromise. This is presenting with primary infertility if clinically evident with an inguino- missed as children scrotal mass, which is tender. On examination the vagina will be Eventually oedema and erythema found to be short, the cervix, of the overlying skin develops. uterus, fallopian tubes rudimentary Nausea, vomiting with abdominal or absent, and the gonad - distension is indicative of small testicular on histology bowel obstruction. Constitutional Genotype of child - male with XY upset with fever, and shock may chromosomal pattern complicate the clinical situation.

Testicular ischaemia with potential As soon as the condition has been necrosis of the testis is a further stabilised, proceed with operative relief complicating factor. Testicular of the obstruction, assessment of atrophy occurs in ± 10% of testes viability of the bowel and herniotomy. following an episode of irreducibility. ACUTE SCROTUM

Treatment Sudden onset of pain and swelling of the scrotal contents. Uncomplicated- Herniotomy Differential diagnosis of an "acute All hernias should be repaired when scrotum" they are diagnosed. The only contra- indication to immediate surgery 1. Complicated hernia - excluded irrespective of age, is concomitant by clinical examination disease or serious impairment of the (inguino-scrotal swelling) patient's general condition. Only the 2. (50%) symptomatic side is repaired in males. 3. Epididymo-orchitis (30%) Surgery on the opposite side will 4. Torsion of appendix of testis or depend on local evidence of patency epididymis (15%) of the processus vaginalis. Both sides are done in females as the 5. Idiopathic scrotal incidence of a sac on the opposite side oedema(<2%) is 70% and there is no hazard of 6. Other rare causes reproductive structure damage. There is often a vague history of A congenital hydrocoele and trauma - which should be ignored hydrocoele of the cord not associated unless there is obvious evidence on with a hernia will usually resolve by the clinical examination. It is extremely age of 1 year; if not, then herniotomy. difficult to clinically differentiate testicular torsion from epididymo- Irreducible / Obstructed hernia orchitis. All "acute " should therefore be explored surgically This needs urgent attention. The without delay. In torsion, in addition to baby is sedated and a short attempt at detorsion and fixation of the affected reducing the hernia is made (<10 testis, the testis on the opposite side mins) using gentle, but never forceful should also be explored and fixed, as pressure. If successful, elective the underlying anatomical defect of a herniotomy is then performed within 48 high investment of the hours. If taxis fails, then immediate around the testis (so-called bell and operation. There is a higher clapper testes) usually affects both incidence of progression to emergency sides. If epididymo-orchitis is found a surgery in Cape Town (>50%) than in pus swab is taken for culture and First World referral centres (<20%). antibiotic sensitivity. The testis may be very swollen and a releasing Strangulated hernia incision in the will alleviate pain and protect the testis Evidence of strangulation must be from atrophy. Post-operative regarded as an emergency, the underlying genito-urinary abnormality earliest sign being oedema of the shoud be excluded by investigation in overlying skin. Taxis should not be children <2yrs or with recurrent attempted. The infant requires urgent attacks. resuscitation and correction of electrolyte and metabolic abnormality.

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