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[Aphallia - report of two cases].
Urologe A. 2020 Jul; 59(7):825-828.U

Abstract

Aphallia is an extremely rare congenital malformation of unknown cause. The incidence is reported in the literature to be 1 in 10-30 million live births. Almost 100 cases have been described to date. Aphallia is associated with other congenital malformations (in particular urogenital and gastrointestinal anomalies) in >50% of cases. The diagnosis is made clinically and shows the complete absence of the corpora cavernosa and the corpus spongiosum with a urethral opening along the perineal midline (most frequently ventral to the anus and in the ventral rectal wall). Two case reports from the authors' department: The first child was a male newborn (46,XY) with penis agenesis and additional bilateral intraabdominal testis, an anorectal malformation (ARM) with a rectovesical fistula, as well as left renal duplication and grade III vesico-ureteral reflux on the right side. The second child was a male newborn (46,XY) with aphallia without further urological or anorectal malformation. Only right inguinal hernia was present. In the first patient, several corrective surgeries were performed in the further course in view of the additional malformations. With regard to the aphallia, the various temporary treatment options (scrotal or parascrotal phalloplasty or penis prosthesis) were discussed with the parents. Masculinizing surgery by means of definitive phalloplasty was planned once the patient has reached puberty. Due to the technical demands of phallus reconstruction, feminization is still favored in some countries in the literature, which nowadays, however, cannot be justified medically or legally.

Authors+Show Affiliations

Klinik für Kinderchirurgie und Kinderurologie, Kinderkrankenhaus Amsterdamer Straβe, Amsterdamer Straβe 59, 50735, Köln, Deutschland. hagelschuerp@kliniken-koeln.de.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

ger

PubMed ID

32472223

Citation

Hagelschuer, P, et al. "[Aphallia - Report of Two Cases]." Der Urologe. Ausg. A, vol. 59, no. 7, 2020, pp. 825-828.
Hagelschuer P, Mack-Detlefsen B, Korsch E, et al. [Aphallia - report of two cases]. Urologe A. 2020;59(7):825-828.
Hagelschuer, P., Mack-Detlefsen, B., Korsch, E., Ekamp, A., & Boemers, T. M. (2020). [Aphallia - report of two cases]. Der Urologe. Ausg. A, 59(7), 825-828. https://doi.org/10.1007/s00120-020-01244-3
Hagelschuer P, et al. [Aphallia - Report of Two Cases]. Urologe A. 2020;59(7):825-828. PubMed PMID: 32472223.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Aphallia - report of two cases]. AU - Hagelschuer,P, AU - Mack-Detlefsen,B, AU - Korsch,E, AU - Ekamp,A, AU - Boemers,T M, PY - 2020/5/31/pubmed PY - 2020/7/9/medline PY - 2020/5/31/entrez KW - Androgens KW - Anorectal malformation KW - Duplex kidney KW - Penile agenesis KW - Phalloplasty SP - 825 EP - 828 JF - Der Urologe. Ausg. A JO - Urologe A VL - 59 IS - 7 N2 - Aphallia is an extremely rare congenital malformation of unknown cause. The incidence is reported in the literature to be 1 in 10-30 million live births. Almost 100 cases have been described to date. Aphallia is associated with other congenital malformations (in particular urogenital and gastrointestinal anomalies) in >50% of cases. The diagnosis is made clinically and shows the complete absence of the corpora cavernosa and the corpus spongiosum with a urethral opening along the perineal midline (most frequently ventral to the anus and in the ventral rectal wall). Two case reports from the authors' department: The first child was a male newborn (46,XY) with penis agenesis and additional bilateral intraabdominal testis, an anorectal malformation (ARM) with a rectovesical fistula, as well as left renal duplication and grade III vesico-ureteral reflux on the right side. The second child was a male newborn (46,XY) with aphallia without further urological or anorectal malformation. Only right inguinal hernia was present. In the first patient, several corrective surgeries were performed in the further course in view of the additional malformations. With regard to the aphallia, the various temporary treatment options (scrotal or parascrotal phalloplasty or penis prosthesis) were discussed with the parents. Masculinizing surgery by means of definitive phalloplasty was planned once the patient has reached puberty. Due to the technical demands of phallus reconstruction, feminization is still favored in some countries in the literature, which nowadays, however, cannot be justified medically or legally. SN - 1433-0563 UR - https://www.unboundmedicine.com/medline/citation/32472223/[Aphallia___report_of_two_cases]_ L2 - https://dx.doi.org/10.1007/s00120-020-01244-3 DB - PRIME DP - Unbound Medicine ER -