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Rare Congenital Genitourinary Anomalies

Abstract

Between the 4th and 7th weeks, the mesodermal mesenchyme migrates to the cranial aspect of the cloacal membrane to form the future penis. Congenital penile anomalies can be isolated variations of external genitalia development or they can represent significant underlying malformations. Prompt diagnosis and potential surgical planning are essential to allay the anxieties of parents as well as to identify other potentially clinically significant conditions. Congenital penile anomalies show an increasing incidence. The weighted incidence of anomalies including hypospadias has increased from 7 of every 1,000 newborns (1988–1991) to 8.3 of every 1,000 newborns (1997–2000), but this may be a result of improved reporting. Multiple etiologies in abnormal development have been proposed including genetic, hormonal, and environmental influences. Whereas molecular pathology has aided in the identification of key steps in abnormal development, modern imaging techniques continue to refine the evaluation and treatment of such anomalies.

Aphallia is a congenital absence of the penis, which is a rare anomaly caused by developmental failure of the genital tubercle reported for the first time in 1853.

The term “microphallus” is applicable only to a normally formed yet abnormally short penis; this condition may be considered a minor form of ambiguous genitalia with correlated medical and psychological problems. The scrotum usually is normal, but sometimes the testes are small or undescended. Abnormal largeness of the penis is an anomaly whereby the phallus enlarges rapidly in childhood due to high levels of production of testosterone, and it is known as megalopenis. Duplication of the penis, or diphallia, is a rare anomaly resulting from incomplete fusion of the genital tubercle. It is an extremely rare but well-documented anomaly. Penile torsion is a rotational defect of the penile shaft. Almost always, the shaft is rotated in a counterclockwise direction. Penoscrotal transposition was first reported by Appleby in 1923. Mcllvoy and Harris in 1955 reported the first performed surgery to move the penis into a more cranial position through a subcutaneous tunnel beneath the prepenile scrotum. Penile lymphoedema is an uncomfortable and potentially disfiguring disorder. It may be congenitally inherited in 15 % of the cases. Penile cysts are a very rare congenital anomaly; few cases are recorded and published as a case report; congenital epidermal cysts or dermoid cyst occasionally form along the median penile raphe on the ventral aspect of the penile shaft or on the glans.

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Authors and Affiliations

  1. Al Agouza, Cairo, PO, Egypt

    Mohamed A. Baky Fahmy

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  1. Mohamed A. Baky Fahmy
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Fahmy, M.A.B. (2015). Penis. In: Rare Congenital Genitourinary Anomalies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-43680-6_2

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  • DOI: https://doi.org/10.1007/978-3-662-43680-6_2

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