The presence of a uterus by pelvic ultrasound suggests either persistent Müllerian duct syndrome (AMH deficiency or insensitivity) or a severely virilized genetic female with congenital adrenal hyperplasia. An unambiguous micropenis, especially accompanied by hypoglycemia or jaundice, suggests congenital hypopituitarism. Treatment
The primary management of cryptorchidism is watchful waiting, due to the high likelihood of self-resolution. Where this fails, orchiopexy is effective if inguinal testes have not descended after 4–6 months. Surgery is often performed by a pediatric urologist or pediatric surgeon, but in many communities still by a general urologist or surgeon. When the undescended testis is in the inguinal canal, hormonal therapy is sometimes attempted and very occasionally successful. The most commonly used hormone therapy is human chorionic gonadotropin (hCG). A series of hCG injections (10 injections over five weeks is common) is given and the status of the testis/testes is reassessed at the end. Although many trials have been published, the reported success rates range widely, from roughly 5% to 50%, probably reflecting the varying criteria for distinguishing retractile testes from low inguinal testes. Hormone treatment does have the occasional incidental benefits of allowing confirmation of Leydig cell responsiveness (proven by a rise of the testosterone by the end of the injections) or inducing additional growth of a small penis (via the testosterone rise). Some surgeons have reported facilitation of surgery, perhaps by enhancing the size, vascularity, or healing of the tissue.
This uncommon syndrome involves vertical gaze palsy associated with pupils that “accommodate but do not react." The causes of Parinaud syndrome include brain tumors (pinealomas), multiple sclerosis and brainstem infarction. Due to the lack of detail in the older literature and the scarcity of AR pupils at the present time, it is not known whether syphilis can cause Parinaud syndrome. It is not known whether AR pupils are any different from the pupils seen in other dorsal midbrain lesions. The condition is diagnosed clinically by a physician. Treatment
There is no definite treatment, but, because syphilis may be an underlying cause, it should be treated. However, because this sign is associated with neurosyphilis, it should be treated with crystalline penicillin 24 mU intravenous per day for 10 to 14 days. If the patient is allergic to penicillin, they should undergo desensitization and then be treated. History
Argyll Robertson pupils were named after Douglas Argyll Robertson (1837–1909), a Scottish ophthalmologist and surgeon who described the condition in the mid-1860s in the context of neurosyphilis. In the early 20th century, William John Adie described a second type of pupil that could "accommodate but not react". Adies tonic pupil is usually associated with a benign peripheral neuropathy (Adie syndrome), not with syphilis.When penicillin became widely available in the 1940s, the prevalence of AR pupils (which develop only after decades of untreated infection) decreased dramatically. AR pupils are now quite rare. A patient whose pupil “accommodates but does not react” almost always has a tonic pupil, not an AR pupil.