|Year : 2005 | Volume
| Issue : 4 | Page : 258
DK Gupta, Shilpa Sharma
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India
D K Gupta
Department of Pediatric Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110 029
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Gupta D K, Sharma S. Editorial comments. J Indian Assoc Pediatr Surg 2005;10:258
Gender assignment is a common dilemma in newborns with aphallia. Though in most cases, the urethra is short and opens in the anorectum yet it may open in the perineum with good urinary control. Female gender has often been assigned for technical reasons and also for the belief of psychosexual neutrality at birth with female genitoplasty.
Patients with aphalia are truly males and are expected to behave so with male mental makeup in their adult life. If they are assigned a female gender, the child may not be expected to behave normally in society due to abnormal sexual orientation. The brain gender makeup has been currently proved to affected by antenatal androgens which can not be altered by psychosexual and endocrine treatment. Also the long term results in patients converted to females are not available in the literature.
Henceforth, the present consensus is that patients affected by aphalia are better raised as males. Recently, successful phallic reconstruction in two patients with aphalia were presented from Bologna (Italy), using the lower abdominal wall skin flap for making the shaft and the bladder/labial mucosa free graft for making the urethra. The procedures were completed at 9 and 17 months of age.
However, all is not that well. Male genitoplasty in such cases requires a long urethroplasty and it is not supported by corpus spongiosum. Due to lack of urethral resistance, the long and the short term results of the long urethroplasty are not satisfactory. Patients are not able to expel urine at the meatus in a forceful stream. This problem was encountered in one out of the two cases from Bologna and a scrotal urethrostomy had to be resorted to later on. We also had the similar experience in one case in whom male genitoplasty was performed.
The initial management should include dismantling of the urethra from the anorectum and placing the same in the perineum (as preliminary urethrostomy). This may be done even during the newborn period or infancy so as to avoid urinary complications in future. One should not allow the child brought early to go without a urethrostomy as death due to chronic renal failure has been reported. If the parents are not sure of the gender, they may be given time to think it over.
In Indian society, the family accepts an inadequate male better than an incomplete female along with psychosocial disturbance. If a male gender assignment is planned, the phalloplasty should preferably be done after puberty as the results following phalloplasty performed in infancy and childhood may not be satisfactory due to inadequate growth of the reconstructed phallus. If the child presents late after puberty or the parents had refused treatment in the past, as may be the case in our country, phallic and urethral reconstruction should be done with the hope to achieve satisfactory results.
In our experience, three cases of aphalia presented during the newborn period, in infancy and at 7 years of age (with urethra opening in the anorectum in the last case). One seen during the newborn period was converted to female gender. The second case was from Madagascar (Africa) and insisted only for male gender assignment. In this case, a successful but a smaller sized phalloplasty using lower abdominal wall flaps was performed. The urethra is short but quite effective in expelling the urine. There were no backpressure changes noted during follow up at 2 and 5-year age. The third one did not agree to female sex assignment and refused treatment.
| References|| |
|1.||Chibber PJ, Shah HN, Jain P, Yadav P. Male gender assignment in aphallia: a case report and review of the literature. Int Urol Nephrol 2005;37:317-9. [PUBMED] [FULLTEXT]|
|2.||Castro R De, Farhoud R, Ross F. Male gender assignment in newborns with aphallia: Two cases of early and preliminary reconstruction of penis and urethra. Presented at the First World Congress on Hypospadias and Intersex Disorders. Istanbul; 2005. |