30th ESPU Congress - Lyon, France - 2019

S9: DSD

Moderators: Katja Wolffenbuttel (Netherlands), Daniela Gorduza (France)

ESPU Meeting on Thursday 25, April 2019, 15:18 - 15:38


15:18 - 15:23
S9-1 (LO)

★ SEXUAL FUNCTION AND QUALITY OF LIFE IN ADULT PATIENTS WITH CONGENITAL ADRENAL HYPERPLASIA

Barbara DOBROWOLSKA-GLAZAR 1, Anna GUZIK 1, Ireneusz HONKISZ 1, Janusz SULISŁAWSKI 1, Katarzyna TYRAWA 2, Michał WOLNICKI 1 and Rafał CHRZAN 1
1) Jagiellonian University Medical College, University Children's Hospital, Paediatric Urology, Cracow, POLAND - 2) Jagiellonian University Medical College, University Children's Hospital, Paediatric Endocrinology, Cracow, POLAND

PURPOSE

This is to assess sexual function and Quality of Life (QoL) in adult women with Congenital Adrenal Hyperplasia (CAH) who had feminizing genitoplasty in childhood.

MATERIAL AND METHODS

This prospective study including a control group was approved by the Ethical Committee. Fifty adult patients who had genitoplasty in childhood were identified and 9 (18%) agreed to participate in the study (mean age -30.6 years). Mean age at operation was 5.4 years and mean follow-up was 10.9 years. Female Sexual Function Index (FSFI) was used to evaluate sexual function and Short Form Health Survey (SF-36) to value the QoL. A FSFI score < 26,55 was classified as Female Sexual Dysfunction (FSD). The control group (GC) consists of 10 adult females in comparable age, without any oncological and chronic diseases. Fisher's exact test was used for statistical analysis.

RESULTS

All patients in CAH group had female gender identity. One was homosexual and one reported having no sexual activity. In CG all patients had female gender identity and all was heterosexual and one reported having no sexual activity.
The FSD (FSFI < 26.55) was found in 5/9 patients of the CAH group vs 4/10 CG patients. The difference was statistically not significant (p value - 0.66). Mean SF-36 score in the CAH group was 47.1 and 46.7 in the CG.
Mean serum testosterone in CAH group was 0.46 ng/ml (norm:0,1-1,12ng/ml) and in the CG was 0.40 ng/ml, 17OH progesterone was 6.41ng/ml (norm:0,2-5,2ng/ml) and 2.26ng/ml, respectively.

CONCLUSIONS

The traceable adult CAH patients has a good QoL and sexual function. Remarkable only a small number is willing to participate in such evaluation.


15:23 - 15:26
S9-2 (PP)

★ VIRILIZED FEMALES WITH CONGENITAL ADRENAL HYPERPLASIA AND PERSISTENT URO-GENITAL SINUS (UGS), INTERFERE EARLY BETTER THAN DEFER

Ahmed ELHELALY 1, Abdulhakim AL OTAY 2, Mustafa AL GHANBAR 2, Faris AL HARBI 2, Moahmmed AL HAGBANI 2, Osama SARHAN 3 and Ziad NAKSHABANDI 2
1) Prince Sultan Military Medical City, Urology, Riyadh, SAUDI ARABIA - 2) Prince Sultan Military Medical City, Urology department, Riyadh, SAUDI ARABIA - 3) King Fahad Specialist Hospital, Urology department, Dammam, SAUDI ARABIA

PURPOSE

Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive disorders found in 1 every 15,000 births. Female virilisation and persistent UGS are common features and surgical correction usually needed. We aimed to study effect of timing of surgery on the perioperative outcomes.

MATERIAL AND METHODS

Twenty sex female patients with (CAH) were identified retrospectively in the period (2000-2010).  Nine patients were excluded due to previous surgical correction and or incomplete data. We included 17 patients, 9 (group A), underwent surgical correction before  age of 2 years ( range 9 months -2 years) and 8 patients (group B),  underwent surgery after the age of 2 years (range 2.5-15 years). Patients with, low confluence were, 5 and 4, and high confluence, were 4 and 4, in group A and B respectively. All patients underwent total uro-genital sinus mobilization (TUM), with distal or proximal dissection. Mean follow up period was 10 years, (range 8-18 years). Peri-operative outcomes were assessed.

RESULTS

Mean operative time was 115 and 181 minutes (range 90 to 185 and 120 to 235 minutes; p value =.001) for group A and group B respectively. None in group A, received blood transfusions, however 37.5% of group B needed, with a mean Hemoglobin drop 1.2 and 2.3 grams for group A and group B respectively. After a mean follow up period of 10 years (range 6 to 14 years), three patients in group B needed re-surgery for vaginal stenosis, but none in group (A).  All patients were continent.

CONCLUSIONS

Early surgery for virilized females with CAH and persistent UGS is preferable due to less intraoperative morbidity, (shorter operative time, and easier tissue handling with less intraoperative bleeding) and better post-surgical outcome.


15:26 - 15:29
S9-3 (PP)

LONG-TERM SURGICAL RESULTS IN CONGENITAL ADRENAL HYPERPLASIA

Javier SERRADILLA RODRIGUEZ 1, Susana RIVAS VILA 2, María José MARTÍNEZ URRUTIA 2, Roberto LOBATO 2, Alba BUENO JIMÉNEZ 1, Solon CASTILLO 2, Virginia AMESTY 2 and Pedro LÓPEZ PEREIRA 2
1) Hospital La Paz, Pediatric Surgery, Madrid, SPAIN - 2) Hospital La Paz, Pediatric Urology, Madrid, SPAIN

PURPOSE

Surgical treatment in congenital adrenal hyperplasia (CAH) is very debated due to the patients’ psycho-physical impact.

Our aim is to evaluate our patients’ current sexual situation and satisfaction according to the treatment that they received.

MATERIAL AND METHODS

Retrospective study of our CAH patients treated between 1977-2012, assessing their baseline situation (physical examination, genetics, family history), the surgery performed (resection or plication of corpora cavernosa (CC), glanuloplasty, vaginoplasty) and their secondary sexual development.

The long-term surgical results were analyzed through a subjective questionnaire about their current sexual situation, satisfaction and self-steem, excluding patients below 16.

RESULTS

Thirty-three patients (20 years, [6-43]) were reviewed. The surgical procedure differed depending on the historical period: plication of CC was performed in 7 of our oldest patients, requiring 3 of them a resection later; resection of CC was performed in 21 in the later years and 5 did not require any intervention in the erectile tissue. Glans reduction was practiced in 7 and vaginoplasty in 30.

Twenty-two patients met the questionnaire inclusion criteria. The response rate was 68% (15). While resection or plication of CC did not show differences in sexual satisfaction (p>0.05), glans reduction showed a significant decrease in clitoral sensitivity (p<0.05). Although the majority (10) were satisfied with their genitalia appearance, 3 referred not being satisfied with their female assignment.

CONCLUSIONS

Glans reduction in CAH patients cause a decrease in genital sensitivity. The surgical consequences for sexual and social development in this condition should lead us to a multidisciplinary, more conservative management.


15:29 - 15:38
Discussion