Aphallia

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Aphallia
Other namesPenile agenesis[1]

Aphallia is a congenital malformation in which the phallus (penis or clitoris) is absent. It is the female counterpart of penile agenesis and testicular agenesis.[2] The word is derived from the Greek a- for "not", and phallos for "penis". It is classified as an intersex condition.

Causes[edit]

Aphallia has no known cause. It is not linked to deficient hormone amounts or action, but rather to a failure of the fetal genital tubercle to form between 3 and 6 weeks after conception. The urethra of an affected child opens on the perineum.

Diagnosis[edit]

Treatment[edit]

Congenital anomalies like cryptorchidism, renal agenesis/dysplasia, musculoskeletal and cardiopulmonary anomalies are also common (>50% cases), hence evaluation of the patient for internal anomalies is mandatory. Although aphallia can occur in any body type, it is considered a substantially more troublesome problem with those who have testes present, and has in the past sometimes been considered justification for assigning and rearing a genetically male infant as a girl. After the theory in the 1950s that gender as a social construct was purely nurture and so an individual child could be raised early on and into one gender or the other regardless of their genetics or brain chemistry. Intersex people generally advocate harshly against coercive genital reassignment however, and encourage infants to be raised choosing their own gender identity. The nurture theory has been largely abandoned and cases of trying to rear children this way have not proven to be successful transitions.[3]

Nowadays consensus recommends male gender assignment[4][5]

Recent advances in surgical phalloplasty techniques have provided additional options for those still interested in pursuing surgery.[6][7]

Incidence[edit]

It is a rare condition, with only approximately 60 cases reported as of 1989,[8] and 75 cases as of 2005.[9] However, due to the stigma of intersex conditions and the issues of keeping accurate statistics and records among doctors, it is likely there are more cases than reported.

See also[edit]

  • David Reimer - a born male child whose parents were advised by a psychologist to raise as a girl after a botched circumcision.
  • Perineal urethra

References[edit]

  1. ^ RESERVED, INSERM US14-- ALL RIGHTS. "Orphanet: Penile agenesis". www.orpha.net. Retrieved 16 March 2019.
  2. ^ "Aphallia | Intersex Society of North America". Archived from the original on 4 January 2008. Retrieved 2007-12-08.
  3. ^ "What's wrong with the way intersex has traditionally been treated?". Intersex Society of North America.
  4. ^ Horm Res Paediatr 2016;85:112-118
  5. ^ Venkatesh, S. K; Babu, P. R; Joseph, T. P; Varma, K. K (2017). "Male Gender Assignment of a Child with Aphallia and Associated Complex Urological Anomaly". Journal of Indian Association of Pediatric Surgeons. 22 (1): 38–39. doi:10.4103/0971-9261.194619. PMC 5217138. PMID 28082775.
  6. ^ De Castro R, Merlini E, Rigamonti W, Macedo A (2007). "Phalloplasty and urethroplasty in children with penile agenesis: preliminary report". J. Urol. 177 (3): 1112–6, discussion 1117. doi:10.1016/j.juro.2006.10.095. PMID 17296424.
  7. ^ Descamps MJ, Hayes PM, Hudson DA (2007). "Phalloplasty in complete aphallia: pedicled anterolateral thigh flap". J Plast Reconstr Aesthet Surg. 62 (3): e51–4. doi:10.1016/j.bjps.2007.04.014. PMID 17574944.
  8. ^ Skoog SJ, Belman AB (1989). "Aphallia: its classification and management". J. Urol. 141 (3): 589–92. doi:10.1016/S0022-5347(17)40903-7. PMID 2918598.
  9. ^ Chibber PJ, Shah HN, Jain P, Yadav P (2005). "Male gender assignment in aphallia: a case report and review of the literature". Int Urol Nephrol. 37 (2): 317–9. doi:10.1007/s11255-004-7974-0. PMID 16142564.

External links[edit]

Classification
External resources