ESPU Meeting on Friday 10, June 2022, 11:10 - 11:45
Mahmoud M. MAREI 1, Mohamed S. ABDELMONSIF 2, Noha Arafa HUSSEIN 3 and Ahmed K. WISHAHY 4
1) Cairo University: Faculty of Medicine - Cairo University Hospitals, Paediatric Surgery Unit/Section, Cairo, EGYPT - 2) Ghamra Military Hospital, Department of Pediatric Surgery, Cairo, EGYPT - 3) Cairo University, Diabetes, Endocrine and Metabolism Pediatric Unit (DEMPU), Cairo, EGYPT - 4) Cairo University: Faculty of Medicine - Cairo University Hospitals, Department of Pediatric Surgery, Cairo, EGYPT
Müllerian duct remnants (MDRs) in male DSD have variable degrees of development and Wolffian duct relations. We analysed such variations and correlation with External Masculinization Score (EMS) (range:0–12) to guide operative decision making.
MATERIAL AND METHODS
We prospectively recruited a 3-years series of 20 laparoscopically-managed male-reared DSD cases with MDRs, approached with either longitudinal splitting/division or near-total excision. We generated a classification of internal anatomy (GVMDR) based on: (A) gonadal status (G0:absent; G1:unfavourable/undesired [dysgenetic/ovary]; G2:ovotestis; G3:normal right/left testis). (B) relation to vasa differentia (V0:absent relation bilaterally; V1:unilateral relation; V2:bilateral relation). (C) degree of development towards normal female (M1-M5:uterus with bilateral Fallopian tubes). The predictivity of surgical approach from external genitalia using EMS was jointly studied.
Bilateral complex connections between MDRs and vasa deferentia (V2) e.g. PMDS (with a uterus and bilateral fallopian tubes), rendering excision hazardous, received a laparoscopic longitudinal splitting/division (6 cases), to permit tension-free orchidopexy (G3). Situations which allowed safe laparoscopic excision without jeopardizing the male duct system, were either (V0) no relation with the Vas; (V1) unilateral relation e.g. MGD; or (V2) bilateral relation with either (G0) one side connected to an absent gonad or (G1) unfavourable/undesired gonad i.e. dysgenetic or against gender/sex of rearing (ovary). The latter group (12 cases) were less virilized and the MDR-duct connection was more distal and less extensive. Chi-Square Test=14.25 for GVMDR predictivity/impact on surgery i.e. division (12 cases) vs. excision (6 cases), p<0.05. Two cases needed neither procedures.
The mean EMS value was 8.58±1.74SD for division cases and 5.33±1.98SD for excision cases (Independent t-test=–3.403, p=0.004). The vasa deferentia were preserved in all cases, with no visceral or ureteric injuries.
Combining GVMDR and EMS is predictive for feasibility of excision vs. division. The male duct system is more separable/dissectable in unilateral involvement. Excision is feasible with less virilization.
Gabriela GROCHOWSKA and Malgorzata BAKA-OSTROWSKA
Children's Memorial Health Institute in Warsaw, Paediatric Urology, Warsaw, POLAND
To present results of feminizing genitoplasty (FG) in 225 patients with DSD.
MATERIAL AND METHODS
Analysis includes: 1) type of DSD 2) age at the time of surgery 3) aesthetic results (vulva appearance, shape of labia, urethral orifice localization, clitoris position and size) 4) reoperations rate 5) incidence of UTI's and urine incontinence after FG.
Congenital adrenal hyperplasia was diagnosed in 209 cases (Prader I - 1%; II - 7%; III - 37%; IV - 46%; V - 9%;), 46XY DSD in 10 patients, mixed gonadal dysgenesis in 4 subjects and ovotesticular DSD in 2 cases.
Median age at the time of surgery was 2 years (range from 2 mo to 16 ys).
One-stage procedure was performed in 219 cases and two-stage in 6.
Physical examination under general anesthesia was performed at least 3 times after operation: 1) 1 year after FG, 2) before menarche (+/- 10 ys), 3) in pubertal age.
Excellent aesthetic results was achieved in 210 cases, medium in 11 and poor in 5 subjects.
Further treatment to permit penetrative intercourse was necessary in 135/225 patients (60%) in pubertal age; vaginal calibrations were enough in 78% and 22% needed surgery of vaginal outlet by using skin or buccal mucosa flaps, because of severe vaginal stenosis.
There was 5 cases of urethral orifice stenosis required either dilatations or meatotomy.
Recurrent UTI's occured in 17/225 patients after FG.
Continence problems (nocturnal enuresis or day-time incontinence) were noticed in 21/225.
1. Feminizing genitoplasty allows to create female external genitalia regardless of reason of masculinization.
2. Vaginal, clitoral and labial surgery should be performed as one-stage procedure.
3. Aesthetic results of feminizing genitoplasty in childhood are generally very good, but additional surgery due to vaginal stenosis is sometimes needed in teenage patients.
Medical university Vienna, Paediatric Urology, Vienna, AUSTRIA
Disorders of Sexual Development (DSD) is an umbrella term and an ever evolving topic. Genital surgery is the most controversial part of DSD management. Over the last 30 years there was formation of a movement made up of different political groups, patients, activists, and the LGBT movement demanding de-medicalization, officialization of medical treatment and even a ban of medical procedures. This was largely ignored by the medical community. In Austria this has led to governmental action and an intersex law is currently under preparation.
MATERIAL AND METHODS
The author is member of a national task force for the preparation of an intersex law proposal led by the Austrian Department of Justice of and is reporting of the task force’s work.
Austria is a small western European country with roughly 8.5 million inhabitants. Currently, there is a political coalition of the conservative people’s party and the green party. The task-force was composed as follows: 2 jurists as leads (judges, not aware of the DSD issue in any way), 4 intersex activists, 1 LGBT group representative, 2 specialists from the ministry of health and social affairs, 1 pediatric endocrinologist, 1 psychologist, 1 pediatric urologist, and 2 CAH support group members. Over weeks there were tough discussions on all the controversial topics (are DSD, CAH and hypospadias diseases? what is the role of LGBT? when DSD is no disease why then access to health care funded by the society, ban of surgery? ban of hormones? etc.). There was largely no consensus.
In 2022 Austria most probably will be the second country in Europe having an intersex law. The process of law-making should be of interest for everybody active in the field of DSD and hypospadias. Passivity and lack of interest by the medical community may be precarious.