Congenital anorchia, acquired anorchia, testicular maldescent, and varicocele
Authors: Baker H. W. Gordon 
Published: July 2011

This chapter describes the causes, symptoms and clinical management strategies of three disorders affecting the testes; anorchia (both congenital and acquired), testicular maldescent, and varicocele.

Absence of both testes in baby boys (bilateral congenital anorchia) is infrequent. Unilateral anorchia or monorchidism is more common. Vascular accidents in gestation appear to be the major cause of anorchia. Bilateral anorchia is associated with changes in luteinizing hormone, follicle-stimulating hormone, and testosterone levels. Once the diagnosis of bilateral anorchia is made, both sterility and the requirement for androgen replacement therapy need to be considered. For treatment, androgen replacement therapy induces pubertal virilization and maintains it in adult life. Torsion and orchidectomy or failed orchiopexy for maldescent are the commonest causes of acquired anorchia. Clinical evaluation and androgen replacement therapy for acquired anorchia are as for congenital anorchia.

Normal testes may not complete descent into the scrotum until after birth, particularly in premature infants. The pathological condition of testicular maldescent generally includes incompletely descended or ectopic testes. Infertility is an important problem in patients with a past history of maldescended testes, though whilst the causes of maldescended testes may be multifactorial, the majority of infertile patients with maldescended testes have no other relevant clinical features. Clinical guidelines for treatment of maldescended testes recommend orchiopexy for congenital forms between 6 and 12 months of age, and as soon as possible for those discovered later and for acquired maldescent.

Varicocele is one of the most enigmatic and controversial areas in reproductive medicine; a dilation of the pampiniform plexus that usually affects the left side. Its pathogenesis, effects on the testis and, particularly, the benefits of treatment for infertility remain uncertain. Some adults with varicoceles complain of testicular discomfort, a feeling of weight or a dragging sensation in the scrotum. However, many men with a varicocele are unaware of its presence. The mechanism of development of the common varicocele is regarded as a missing or incompetent valve, although they can also result from portal hypertension or intra-abdominal venous obstruction. Asymmetrical testicular size is a frequent accompaniment to the presence of a varicocele, and on average poorer semen quality is present in affected men. They are most easily detected with the man standing upright. Inspection of the scrotum shows an enlargement of the left side of the scrotum, and the dilated veins maybe apparent. Most treatments involve venographic or surgical obstruction of the incompetent veins, though a variety of surgeries have also been performed. The association between varicoceles and infertility is controversial and a Cochrane Review concluded that there is insufficient evidence to support varicocele treatment for infertility. However, the field remains confused and contradictory.