Undescended Testes and Hernia

Undescended Testis

Undescended Testis (Cryptorchidism): A condition where one or both of the testicles fail to move into the scrotum before birth, remaining in the abdomen or inguinal canal, which can lead to fertility problems and increased risk of testicular cancer if not corrected.

Varicocele

An abnormal enlargement of the pampiniform venous plexus in the scrotum, similar to a varicose vein, which can lead to reduced fertility and testicular atrophy due to impaired blood drainage and elevated scrotal temperature.

Hernia

The protrusion of an organ or tissue through an abnormal opening, often occurring in the inguinal region where the intestine or other abdominal contents push through the inguinal canal, potentially causing pain and complications if left untreated.

Hydrocele

The accumulation of serous fluid within the tunica vaginalis, the sac surrounding the testicles, leading to painless swelling of the scrotum that may resolve on its own or require surgical intervention if persistent.

Acute Scrotum in Children

A sudden onset of scrotal pain and swelling in children, which can be caused by various conditions requiring prompt evaluation and treatment to prevent complications.

• Testicular Torsion: A urological emergency where the spermatic cord twists, cutting off blood supply to the testicle, causing severe pain and swelling, and requiring immediate surgical intervention to prevent testicular loss.
• Obstructed Hernia: A type of hernia where the protruding organ or tissue becomes trapped and its blood supply is compromised, leading to tissue ischemia and necrosis if not urgently treated.
• Torsed Appendages: The twisting of testicular or epididymal appendages (small vestigial structures), causing localized pain and swelling that can mimic testicular torsion but is typically less severe and self-limiting.
• Hydrocele: As previously defined, it is the collection of fluid around the testicle within the tunica vaginalis, causing painless swelling.
• Infection: In the context of acute scrotum, this typically refers to epididymitis or orchitis, where bacterial or viral infection leads to inflammation, pain, and swelling of the epididymis or testicle, often requiring antibiotics and supportive care.

Causes of acute scrotum

• Acute testicular torsion
• Torsion of epididymal and testicular appendages
• Epididymo-orchitis
• Incarcerated inguinal hernia
• Idiopathic scrotal edema
• Acute hydrocele
• Henoch-Schonlein purpura
• Tumors
• Trauma
• Scrotal/Fournier's gangrene
• Symptomatic varicocele

• Gonads - Intra-Abdominal Organs
• Undifferentiated gonads are bipotential for either gender within the first 3 to 5 weeks of gestation.
• Differentiation of the testis is initiated by the SRY gene located in the short arm of the Y chromosome by week 7 of gestation.
• The SRY gene induces Sertoli cells - MIS which causes involution of Müllerian duct cells.
• During the 8th week of gestation, testicular androgens produced by fetal testicular Leydig cells and MIS produced by fetal testicular Sertoli cells are responsible for the development of normal male internal genitalia by weeks 10 to 13 of gestation.

The Transabdominal Descent of the Testes

• Occurs between 10 and 23 weeks of gestation under hormonal direction.
• From urogenital ridge to the inguinal region simultaneously:
  • Shortening of the gubernaculum
  • Testicular differentiation
  • Eversion of the cremasteric muscle
• The Leydig cell produced:
  • Insulin-like hormone (Insl3) termed descendin mediates this transabdominal migration.
  • Its receptor, relaxin family peptide 2 (RXFP2 gene), is expressed on the gubernaculum.

The Inguinoscrotal Descent

• Occurs between 24 and 34 weeks of gestation and is androgen dependent:
  • Androgens act on the genitofemoral nerve inducing the release of calcitonin gene-related peptide (CGRP), which promotes rhythmic contractions of the gubernaculum, leading to its extension and protrusion into the scrotal sac.
• As the gubernaculum testis protrudes towards the scrotum, it pulls the testis down into the same direction.
• Processus vaginalis - an evagination of the parietal peritoneum - elongates through the internal inguinal ring between the internal and external oblique muscles, creating a path for the descending testis to reach the scrotum.
• Once this process is complete, the inguinal canal is dilated by the gubernacular bulb and the testis pushed through the canal by an increase in intra-abdominal pressure.

Regulators of Testicular Descent

• Hormones - early & migratory phases:
  • Androgens - regression of cranial suspensory ligament
• Inguinoscrotal migration:
  • MIS - enlargement of gubernaculum
• Neurogenic:
  • Genitofemoral Nerve - gubernaculum migration
  • Calcitonin gene-related peptide (CGRP)
• Physical force:
  • Intra-abdominal pressure

• Definition:
  • A testis that cannot be manipulated to the bottom of the scrotum without tension on the spermatic cord.
  • Cryptorchidism literally means hidden or obscure testis.
  • Can be unilateral or bilateral.
  • The term encompasses palpable, nonpalpable, and ectopic testicles.

Testicular Retraction

• A testicle can be milked down to the bottom of the scrotum.
• It is common in boys 5-6 years old and is due to a hyperactive cremaster muscle reflex. This is basically a variation of normal.
• In children from 1 year to 11 years of age, 80% of fully descended testes can withdraw from scrotum and leave an empty scrotum behind due to the cremaster reflex.
• This phenomenon usually disappears by puberty.

Testicular Retraction

• A testicle can be milked down to the bottom of the scrotum.
• It is common in boys 5-6 years old and is due to a hyperactive cremaster muscle reflex. This is basically a variation of normal.
• In children from 1 year to 11 years of age, 80% of fully descended testes can withdraw from scrotum and leave an empty scrotum behind due to cremaster reflex.
• This phenomenon usually disappears by puberty.

Cannilicular Testis

• The testicle is located above its natural position in the scrotum, but still outside the abdominal cavity.
• Tension from the external musculature of the body wall prevents normal descent into the scrotum.

Intra-Abdominal Testes

• The testicle is located inside the abdominal cavity residing in a position along its pathway of natural descent.
• In such a position, it is not amenable to future examination by a physician, and it is at risk of becoming cancerous.

Ectopic Testicle

• The testicle may be found in regions not in the usual pathway of descent into the scrotum.
• Five major sites of ectopia are perineum, femoral canal, superficial inguinal pouch, suprapubic area, and contralateral scrotal pouch.
• The etiology is believed to be misdirected attachment to the scrotum.

Absent Testicle

• Such a phenomenon of an absent testicle can be bilateral (affecting both sides).
• It is believed to be associated with in utero torsion, vascular insult, or agenesis.

Vanishing Testis Syndrome?

Disorder of Sexual Differentiation?

• The position of testis can be abdominal, inguinal, prescrotal, or gliding.
• Incidence is 3-5% in full term boys, and 1.8% at one year of age.
• The testicles descend to a scrotal position in human beings in order to optimize sperm production.

• Multifactorial
• Usually affects the migratory phase:
• Androgenic hormone deficiency (hCG, MIS, testosterone, receptor)
• GFN functional failure (abnormal location)
• Excessive mobility of gonads

Specific Aetiology

• Inherited syndromes
  • Microcephaly
  • Anthrogryposis multiplex congenital
  • Prune belly syndrome
• Obstructive causes
  • PUV
  • Anterior abd wall defect
  • Neural tube defects
  • Epididymal def..
  • Abnormal vas deferens
  • Klinefelter syndrome

Other Risks

• Premature boys - most will descend to the scrotum by 6 months corrected gestational age. If the testis is not in the scrotum by 8 months of age, then it will not descend spontaneously.
• Low birth weight (IUGR)
• Maternal risk factors associated with higher risk of UDT are:
  • Low birth parity, intrauterine insemination, complicated pregnancy such as pre-eclampsia, peripartum asphyxia, exposure to diethylstilbestrol (an anti-androgen), and nicotine during pregnancy.
  • Father and brother 3-4 times in the newborn

• X-Ray Studies: Generally, radiologic imaging is not reliable.
• Ultrasound: Can help identify a testicle located in the inguinal canal but is of limited use for intrabdominal testes.
• MRI and CT Scan: Can be useful for intrabdominal testes, but they are often difficult to use on small children and have a high rate of false-negative results.

• The most common problems associated with undescended testicles are:
• Testicular neoplasm - seminoma after 15 years
• Subfertility
• Testicular torsion
• Inguinal hernia
• Trauma

• Bilateral Undescended Testes
  • First, intersex (females with adrenal hyperplasia) should be ruled out.
  • Bilateral undescended testes in boys less than 9 years old - hormonal work-up is needed to exclude bilateral anorchia which means the testes never formed on either side.
  • If the hormonal work-up is normal, an HCG stimulation test is applied and testosterone is subsequently measured.
  • Patients with bilateral anorchia will not make testosterone in response to HCG.

• Retractile Testis
  • This is a normal variant.
  • This phenomenon usually disappears by 13 years of age.

• Surgical Therapy
  • Surgery is immediately performed on ectopic testes, cryptorchids with coexisting hernias, and boys at pubertal age.
  • When a testis is felt in the groin area we usually explore the area through a small incision made in the skin above the scrotum called the inguinal region.
  • Most undescended testes are associated with a hernia that must be repaired. After this is done, the testis is brought down into the scrotum and anchored in a space created in the scrotum (orchiopexy). Both Incisions (in the inguinal region and scrotum) are closed with absorbable sutures.

• Concealed Laparoscopic Orchiopexy
  • Laparoscopy can be used to localize nonpalpable, undescended testes.
  • The laparoscopy is performed first to find out if the testicle is located in the abdomen or if it is congenitally absent.

• Persistence or patent processus vaginalis
• Overriding rings in infants
• May accompany undescended testis
• Commoner in premature births
• Spontaneous resolution in hydrocele
• Hernias must be repaired ASAP to avoid complications