Cause: Abnormality of testicular descent

Epidem: One of most common disorders of childhood; affects 3.4% of full-term infants and 30% of premature infants (Scorer CG, Farrington GH. Congenital Deformities of the Testis and Epididymis. New York: Appleton-Century Crofts; 1971). At age 1 yr, incidence is 0.8-1.5% (Pediatrics 1993;92:44). In 10% of children, defect is bilateral (Scorer & Farrington, 1971). About 3.5% of cryptorchid testes are absent (J Urol 1974;111:840).

Pathophys: Proposed etiologies of testicular descent include (1) traction of testis by gubernaculum and/or cremasteric muscle, (2) differential growth of body wall in relation to gubernaculum, (3) intra-abdominal pressure pushing testis through inguinal canal, (4) effect of genitofemoral nerve. These may play a role in testicular descent, but endocrine factors appear to play the major role (Endocrinology 1991;129:741,1409).

Sx: Asx

Si: Empty hemiscrotum, testis palpable or nonpalpable in inguinal canal or other sites of ectopia. Size of ipsilateral scrotum may be diminished.

Crs: If UDT is going to descend spontaneously, it often does so within the first year of life, with most descending by 6 mo of age. After age 1 yr, low likelihood of spontaneous descent.

Cmplc: Testicular Ca: about 10% of testicular tumors arise from UDTs (BJU 1992;70:656). UDT 35-48 times more likely than nl testis to undergo malignant degeneration (J Urol 1975;114:77). UDTs have increased risk of testicular torsion. There is risk of a symptomatic hernia because the processus vaginalis remains patent with UDT. Infertility: the higher and longer the testis resides away from the scrotum, the greater the risk of damage to seminiferous tubules (J Urol 1995;153:1255). Associated abnormality of vas deferens and epididymis may affect subsequent fertility (Urol Clin North Am 1982;9:339).

Diff Dx: Vanished testis, retractile testis, intersex abnormality in children with bilateral nonpalpable testes, and cryptorchidism may be associated with Klinefelter’s syndrome, hypogonadotropic hypogonadism, prune belly syndrome, myelomeningocele, hypospadias, Wilms’ tumor, Noonan’s syndrome, Prader-Willi syndrome, cystic fibrosis.

Lab: Not routinely indicated. Serum testosterone, LH, and FSH levels in children with bilateral nonpalpable UDTs. Other labs indicated if associated abnormality suspected.

Xray: Not indicated

Rx: Aimed at bringing testis into dependent position in scrotum, ideally by age 1 yr. Medical and surgical therapies may be used. Medical: HCG and GnRH. The International Health Foundation recommendations for HCG rx are biweekly injections of 250 IU for infants, 500 IU for children < 6 yr of age, and 1000 IU for those > 6 yr of age, for a total of 5 wk. GnRH at 1.2 mg/d as nasal spray for 4 wk successful in 6.18-70% of pts (Int J Urol 1992;15:135; Urol Clin North Am 1982;9:413; Klin Paediatr 1981;193:382; J Urol 1977;118:985). Hormone therapy does
not appear to be effective for truly undescended testis, but short-term therapy is effective in inducing descent of a retractile testis (N Engl J Med 1986; 314:466). Surgical rx includes laparoscopy, a useful diagnostic and therapeutic tool in the setting of a nonpalpable testis (Am Surg 1994;60:143; J Urol 1994;152:1249). If testis is palpable, inguinal approach is used and testis placed in subdartos scrotal pouch. Surgical cmplc include infection, injury to testicular vessels and/or vas deferens, and testicular ascent. Neoadjuvant GnRH treatment improves fertility index in prepubertal cryptorchidism (Urology 2009;73:1251; J Urol 2005;173:974).

Cause: Collection of fluid between parietal and visceral layers of the tunica albuginea

Epidem: Present in 6% of full-term boys. Risk of clinically evident contralateral hydrocele or hernia after unilateral repair is 7% (J Urol 1999;162:1169).

Pathophys: Patent processus vaginalis allows peritoneal fluid to pass into tunica vaginalis.

Sx: Typically asx

Si: Scrotal swelling that transilluminates; if a communicating hydrocele (patent processus vaginalis), then the scrotal swelling may fluctuate throughout the day

Crs: May resolve spontaneously within the first yr of life

Cmplc: If processus vaginalis is patent, pt may develop inguinal hernia.

Diff Dx: Inguinal hernia, testicular torsion, and testicular tumor

Xray: If testis is not palpable due to the size of the hydrocele, scrotal US will evaluate testes and confirm presence of hydrocele.

Rx: If the hydrocele remains after the child is 1 yr old or if it is communicating hydrocele or associated with an inguinal hernia, inguinal exploration, ligation of the patent processus vaginalis, and drainage of the hydrocele are indicated.

Cause: Patent processus vaginalis allows peritoneal contents to pass into inguinal canal. May also be related to abnormal collagen synthesis in inguinal area (Ann Surg 1993;218:754).

Epidem: Most pediatric hernias present within first year of life. Overall incidence 1-4.4%. Premature infants have increased risk of 13%. Male more than females. Few (6-10%) children with unilateral hernia develop clinical hernia on contralateral side (J Pediatr Surg 1993;28:1026). Of girls presenting with inguinal hernias, 1.6% are 46 XY with an intra-abdominal testes (see 13.2).