F. I. Zokirov, B. B. Negmajanov and G. A. Khakimov
Samarkand State Medical Institute, Uzbekistan
Scientific Tracks Abstracts: Pediatr Therapeut
In spite of considerable progresses are achieved in understanding disorders of sex development, problem of gender assignment remains actual. A special attention deserve PAIS where in genetically determined males appearance of external genitalia might be ranging from almost normal female to nearly male that makes sex assignment challenging.
Taking into account the psychological and social impact we recommend gonadectomy and feminizing genitoplasty until the age of 2 years.
Rationality of current approach is explained by that until the age of 2 years children are psychosexually neutral and by such surgery genitals become unambiguous, what is required for a stable “normal” gender identity and relieves parental stress.
In addition even when the child is decided to be reared as male fertility problems arise because:
1. Th e testicles must be removed to avoid a risk of malignancy.
2. If there is a rudimental vagina the prostate may be underdeveloped which leads to reduced ejaculation
3. Normal sexual intercourse may not be possible due to underdeveloped penis.
4. Primary oligospermia is common for these patients
In the fi rst stage of surgical treatment resection of hypertrophied clitoris and if testicles are located in labia major gonadectomy are performed, which make the appearance of external genitalia normal. If testicles are undescended laparoscopic testectomy is preferable. In the second stage during the teenage years neovagina is created in order to create favorable capability for intercourse and hormone replacement therapy is ordered.
In conclusion it’s important to accentuate that even when these patients are infertile and cannot have children favorable conditions to realize normal sexual function are achieved, which plays a big role in social adaptation.
