Approximately 100 cases described. The incidence is estimated at 1/30.106 births. It may be associated with VACTERL syndrome or a malformative sequence of the urogenital septum (anorectal and external genital malformations, vertebral anomalies). The Skoog classification is used, based on the position of the ectopic urethra in relation to the anus and the number of associated anomalies. In addition, it may be pre-sphincteric, post-sphincteric or consist of urethral atresia. 

Treatment: either neophalloplasty or transformation into female sex.

Anesthetic implications: 

cardiac and renal ultrasound (possible association with VACTERL syndrome), early bladder drainage.

References : 

-         Kian B, Esmaeilian S, Kayedi M.
A rare case report of aphallia with hypoplastic kidney and vesicorectal fistula.
Radiology Case Reports 2022 ; 17 : 4656-9

-        Blachman-Braun R, Galvez C, Guevara C, Quintanilla R, Alam A.
Aphallia associated with anorectal malformation.
Sultan Qaboos University Med J 2022 ; 22 :428-9

-        Krishnamoorthya K, Sethuramanb G, Usha Devib U.
Aphallia in a neonate with VACTERL malformation: report of a rare association.
Clinical Dysmorphology 2023, 32:74-6

Updated: November 2023