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Phallic Urethra in Female Pseudohermaphroditism FREE

WILLIAM A. REILLY, M.D.; FRANK HINMAN, M.D.; DONALD E. PICKERING, M.D.; JACKSON T. CRANE, M.D.
[+] Author Affiliations

Received for publication Sept. 11, 1957; accepted Oct. 15.


AMA Am J Dis Child. 1958;95(1):9. doi:10.1001/archpedi.1958.02060050011002.
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The presence of a urethra coursing through the phallus would seem to be evidence of maleness. However, certain female patients with intersexual external genitalia (female pseudohermaphrodites) have been observed either with a single urethra running through the clitoris or with this, plus a second urethra, emptying into the urogenital sinus. Recently, five cases of phallic urethra have been observed and the findings of one other unreported case has been studied. These will be presented and discussed in detail in order to point out the factors involved in differential diagnosis.

The study of intersexuality would be more rewarding if it could be approached from an etiological standpoint. In any given case, however, exact etiologic factors cannot always be determined, but if consideration is given to each of the possible causes (developmental anomaly, fetal adrenal hyperplasia, and exogenous androgens) a reasonable basis can be constructed for diagnosis and treatment.

Theoretically, intersexuality with

REFERENCES

Burns, R. K., Jr.:  Bisexual Differentiation of Sex Ducts in Opossums as Result of Treatment with Androgen , J. Exper. Zool. 100:119-140 ( (Oct.) ) 1945;.
van Wagenen, G., and Hamilton, J. B.:  The Experimental Production of Pseudohermaphroditism in the Monkey , in Essays in Biology , Berkeley and Los Angeles, University of California Press, 1943;, pp. 581-607.
Jost, A.:  Problems of Fetal Endocrinology: The Gonadal and Hypophyseal Hormones , Recent Progr. Hormone Res. 8:379-418, 1953;.
Howard, F. S., and Hinman, F., Jr.:  Female Pseudohermaphroditism with Supplementary Phallic Urethra: Report of 2 Cases , J. Urol. 65:439-452 ( (March) ) 1951;.
Prader, A.:  Der Genitalbefund beim Pseudohermaphroditismus femininus des kongenitalen adrenogenitalen Syndroms: Morphologie, Häufigkeit, Entwicklung und Vererbung der verschiedenen Genitalformen , Helvet. pædiat. acta 9:231-248 ( (July) ) 1954;.
Wilkins, L.: The Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence , Springfield, Ill., Charles C Thomas, Publisher, 1950;, pp. 240 and 264.
Bentinck, R. C.; Lisser, H., and Reilly, W. A.:  Female Pseudohermaphrodism with Penile Urethra, Masquerading as Precocious Puberty and Cryptorchidism , J. Clin. Endocrinol. 16:412-418 ( (March) ) 1956;.
Felicissimo Paula Xavier, J., and de Abreu Junqueira, M.:  Sobre um caso de arrhenoblastoma do ovario e gravidez topica simultanea: Virilisação da gestante e do feto feminino , Rev. gynéc. e obst. 1:356-377 ( (March) ) 1938;.
Brentnall, C. P.:  A Case of Arrhenoblastoma Complicating Pregnancy , J. Obst. & Gynaec. Brit. Empire 52:235-240 ( (June) ) 1945;.
Grunwaldt, E., and Bates, T.:  Phallic Urethra: Report of a Case , Pediatrics 20:503-505 ( (Sept.) ) 1957;.
Clarke, J. J.:  A Case of Pseudo-Hermaphroditism , Tr. Path. Soc. London 44:120-122, 1892-1893;.
Atkinson, W., and Mason, J. C.: " Pseudohermaphroditism ," S. Clin. North America 14:573-576, 1934;.
Gross, R. E., and Meeker, I. A., Jr.:  Abnormalities of Sexual Development: Observations from 75 Cases , Pediatrics 16:303-324 ( (Sept.) ) 1955;.
Wilkins, L.: Personal communication to W. A. Reilly, 1956.
Fibiger, J.:  Bidrag til Læren om den kvindelige Pseudohermafroditisme , Hospitalstid. 13:825; 857; 889, 1905;.
Lehmann, K.:  Lidt om Pseudohermafroditers Arvelightedsforhold og Levedygtighed , Ugesk. Læger 81:751-757, 1919;.
Allibone, E. C.; Baar, H. S., and Cant, W. H. P.:  The Interrenal Syndrome in Childhood , Arch. Dis. Childhood 22:210-225 ( (Dec.) ) 1947;.
Marie, J.; Barbet, J.; Hebert, S.; Roussel, A., and Horowitz, F.:  Le Syndrome d'hyperplasie cérébriforme des surrénales de la période néonatale (syndrome de Debré-Fibiger) , Arch. franç. pédiat. 9:785-817, 1952;.
Matheson, W. J., and Ward, E. M.:  Hormonal Sex Reversal in a Female , Arch. Dis. Childhood. 29:23-27 ( (Feb.) ) 1954;.
Krokiewicz, A.:  Ein Fall von Hermaphroditismus spurius completus femininus , Arch. path. Anat. 146:525-530, 1896;.
Benda, C.:  Fall von Pseudohermaphroditismus femininus externus (Pseudarrhenie) , Berl. klin. Wchnschr. 51:66-69, 1914;.
Paquin, A. J., Jr.; Evans, J.; Finby, N., and Baker, D.: The Urogenital Sinus: Its Demonstration and Significance, to be published.
Perloff, W.; Conger, K. B., and Levy, L. M.:  Female Pseudohermaphroditism: Description of 2 Unusual Cases , J. Clin. Endocrinol. 13: 783-790 ( (July) ) 1953;.
Hayles, A. B., and Nolan, R. B.:  Female Pseudohermaphroditism: Report of a Case in an Infant Born of a Mother Receiving Methyltestosterone During Pregnancy , Proc. Staff Meet. Mayo Clin. 32:41-44 ( (Jan. 23) ) 1957;.
Javert, C. T., and Finn, W. F.:  Arrhenoblastoma: The Incidence of Malignancy and the Relationship to Pregnancy, to Sterility, and to Treatment , Cancer 4:60-77 ( (Jan.) ) 1951;.
Grubenmann, I.:  Eine sagittale Verdoppelung der weiblichen Harnröhre , Frankfurt Ztschr. Path. 10:101-128, 1912;.
Grossman, M.: Personal communication to the authors, 1957.
Hubble, D. V., and Illingworth, R. S.:  Adrenocortical Hyperplasia in Childhood , Arch. Dis. Child. 32:285-292 ( (Aug.) ) 1957;.

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References

Burns, R. K., Jr.:  Bisexual Differentiation of Sex Ducts in Opossums as Result of Treatment with Androgen , J. Exper. Zool. 100:119-140 ( (Oct.) ) 1945;.
van Wagenen, G., and Hamilton, J. B.:  The Experimental Production of Pseudohermaphroditism in the Monkey , in Essays in Biology , Berkeley and Los Angeles, University of California Press, 1943;, pp. 581-607.
Jost, A.:  Problems of Fetal Endocrinology: The Gonadal and Hypophyseal Hormones , Recent Progr. Hormone Res. 8:379-418, 1953;.
Howard, F. S., and Hinman, F., Jr.:  Female Pseudohermaphroditism with Supplementary Phallic Urethra: Report of 2 Cases , J. Urol. 65:439-452 ( (March) ) 1951;.
Prader, A.:  Der Genitalbefund beim Pseudohermaphroditismus femininus des kongenitalen adrenogenitalen Syndroms: Morphologie, Häufigkeit, Entwicklung und Vererbung der verschiedenen Genitalformen , Helvet. pædiat. acta 9:231-248 ( (July) ) 1954;.
Wilkins, L.: The Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence , Springfield, Ill., Charles C Thomas, Publisher, 1950;, pp. 240 and 264.
Bentinck, R. C.; Lisser, H., and Reilly, W. A.:  Female Pseudohermaphrodism with Penile Urethra, Masquerading as Precocious Puberty and Cryptorchidism , J. Clin. Endocrinol. 16:412-418 ( (March) ) 1956;.
Felicissimo Paula Xavier, J., and de Abreu Junqueira, M.:  Sobre um caso de arrhenoblastoma do ovario e gravidez topica simultanea: Virilisação da gestante e do feto feminino , Rev. gynéc. e obst. 1:356-377 ( (March) ) 1938;.
Brentnall, C. P.:  A Case of Arrhenoblastoma Complicating Pregnancy , J. Obst. & Gynaec. Brit. Empire 52:235-240 ( (June) ) 1945;.
Grunwaldt, E., and Bates, T.:  Phallic Urethra: Report of a Case , Pediatrics 20:503-505 ( (Sept.) ) 1957;.
Clarke, J. J.:  A Case of Pseudo-Hermaphroditism , Tr. Path. Soc. London 44:120-122, 1892-1893;.
Atkinson, W., and Mason, J. C.: " Pseudohermaphroditism ," S. Clin. North America 14:573-576, 1934;.
Gross, R. E., and Meeker, I. A., Jr.:  Abnormalities of Sexual Development: Observations from 75 Cases , Pediatrics 16:303-324 ( (Sept.) ) 1955;.
Wilkins, L.: Personal communication to W. A. Reilly, 1956.
Fibiger, J.:  Bidrag til Læren om den kvindelige Pseudohermafroditisme , Hospitalstid. 13:825; 857; 889, 1905;.
Lehmann, K.:  Lidt om Pseudohermafroditers Arvelightedsforhold og Levedygtighed , Ugesk. Læger 81:751-757, 1919;.
Allibone, E. C.; Baar, H. S., and Cant, W. H. P.:  The Interrenal Syndrome in Childhood , Arch. Dis. Childhood 22:210-225 ( (Dec.) ) 1947;.
Marie, J.; Barbet, J.; Hebert, S.; Roussel, A., and Horowitz, F.:  Le Syndrome d'hyperplasie cérébriforme des surrénales de la période néonatale (syndrome de Debré-Fibiger) , Arch. franç. pédiat. 9:785-817, 1952;.
Matheson, W. J., and Ward, E. M.:  Hormonal Sex Reversal in a Female , Arch. Dis. Childhood. 29:23-27 ( (Feb.) ) 1954;.
Krokiewicz, A.:  Ein Fall von Hermaphroditismus spurius completus femininus , Arch. path. Anat. 146:525-530, 1896;.
Benda, C.:  Fall von Pseudohermaphroditismus femininus externus (Pseudarrhenie) , Berl. klin. Wchnschr. 51:66-69, 1914;.
Paquin, A. J., Jr.; Evans, J.; Finby, N., and Baker, D.: The Urogenital Sinus: Its Demonstration and Significance, to be published.
Perloff, W.; Conger, K. B., and Levy, L. M.:  Female Pseudohermaphroditism: Description of 2 Unusual Cases , J. Clin. Endocrinol. 13: 783-790 ( (July) ) 1953;.
Hayles, A. B., and Nolan, R. B.:  Female Pseudohermaphroditism: Report of a Case in an Infant Born of a Mother Receiving Methyltestosterone During Pregnancy , Proc. Staff Meet. Mayo Clin. 32:41-44 ( (Jan. 23) ) 1957;.
Javert, C. T., and Finn, W. F.:  Arrhenoblastoma: The Incidence of Malignancy and the Relationship to Pregnancy, to Sterility, and to Treatment , Cancer 4:60-77 ( (Jan.) ) 1951;.
Grubenmann, I.:  Eine sagittale Verdoppelung der weiblichen Harnröhre , Frankfurt Ztschr. Path. 10:101-128, 1912;.
Grossman, M.: Personal communication to the authors, 1957.
Hubble, D. V., and Illingworth, R. S.:  Adrenocortical Hyperplasia in Childhood , Arch. Dis. Child. 32:285-292 ( (Aug.) ) 1957;.

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