Ovarian tissue cryopreservation for fertility preservation before hematopoietic stem cell transplantation in patients with sickle cell disease: safety, ovarian function follow-up, and results of ovarian tissue transplantation.

PURPOSE: To describe the experience of performing ovarian tissue cryopreservation (OTC) before hematopoietic stem cell transplantation (HSCT), among girls/women with severe sickle cell disease (SCD)(SS or S/ß0-thalassemia) who are, besides the usual surgical risk, at risk of SCD-related complications during the fertility preservation procedure for improving their counseling and management. METHODS: This retrospective study included 75 patients (girls/women) with SCD who have had OTC before myeloablative conditioning regimen (MAC) for HSCT. Characteristics of patients and data on OTC, ovarian status follow-up, and results of ovarian tissue transplantation (OTT) were collected in medical records. RESULTS: At OTC, the median (IQR 25-75; range) age of the patients was 9.6 (6.9-14.1; 3.6-28.3) years, 56/75 were prepubertal, and no SCD or surgery-related complications occurred. The median follow-up post-HSCT was > 9 years. At the last follow-up, among prepubertal patients at HSCT, 26/56 were ≥ 15 years old and presented with a premature ovarian insufficiency (POI), except 2, including the patient who had received an OTT to induce puberty. Eight were 13-15 years old and presented for POI. The remaining 22 patients were under 13. Among the 19 patients who were menarche at HSCT, 2 died 6 months post-HSCT and we do not have ovarian function follow-up for the other 2 patients. All the remaining patients (n = 15) had POI. Five patients had OTT. All had a return of ovarian function. One patient gave birth to a healthy baby. CONCLUSION: OTC is a safe fertility preservation technique and could be offered before MAC independent of the patient's age.

Genito-urinary Reconstruction in Female Children With Congenital Adrenal Hyperplasia: Favorable Surgical Outcomes can be Achieved by Contemporary Techniques and a Dedicated Multidisciplinary Management.

INTRODUCTION: Congenital adrenal hyperplasia (CAH) is the most common cause of genital atypia in females. A dedicated multidisciplinary team (MDT) should be included for an optimal management. Here, we aimed to review our surgical experience and to assess long-term urinary, gynecological and endocrine outcomes after primary genitoplasty in this specific cohort. METHODS: Patients born with CAH and who underwent feminizing genitoplasty in our institution were retrospectively identified (2001-2021). We analyzed patients' characteristics, intraoperative details, and postoperative urinary, gynecological, and endocrine outcomes. RESULTS: Forty patients were included and followed-up for a median (IQR) time of 7 (1-19) years. Thirty-eight (95%) had 21-hydroxylase deficiency. After multidisciplinary decision and written consent from patient and/or family, a single-stage reconstructive surgery was performed at a median age of 10 (3-165) months. Median length of hospital stay was 5 (1-7) days. Procedures were: PUM (N = 35 (87.5%)), TUM (N = 3 (7.5%)), urogenital mobilization was unnecessary in 2 (5%). Reduction clitoroplasty was done in 33 (82.5%) patients. Only 3 (7.5%) experienced significant Clavien-Dindo complications requiring additional surgery during the follow-up period. Recurrent urinary tract infections (UTI) occurred in 6 (15%), one required ureteric reimplantation for symptomatic high-grade vesicoureteric reflux. All patients over 3 years were toilet-trained without incontinence. Severe vaginal stenosis occurred in 1 (2.5%) patient. In patients who achieved puberty, 6/9 had vaginal calibration at a median age of 17.3 (16-21) years without detected stenosis. One (2.5%) had major hypertrophy of the right labia minora requiring labiaplasty. Nine (22.5%) reached puberty. Two (5%) patients developed acne/hirsutism. Short stature was noted in 11 (27.5%) and obesity in 18 (45%). CONCLUSION: Based on our contemporary series, genitourinary reconstructive surgery for female patients born with CAH is technically feasible and safe with a low complication rate. A regular follow-up with a MDT to assess long-term complications is necessary, and it is vital to inform patients and families about the different management options with all the risks and benefits of surgery. TYPE OF THE STUDY: original research, clinical research. LEVEL OF EVIDENCE: Level 3 retrospective study.

A catheterizable serous-lined urinary outlet associated with the ileal bladder augmentation Abol-Enein and Ghoneim procedure: a safe and reliable procedure in children.

Purpose: This study aims to evaluate the long-term outcome of the serous-lined extramural continent catheterizable outlet procedure (SLECCOP) associated with ileal bladder augmentation in children. Methods: This was a monocentric and retrospective study (2002-2021) that included children (<18 years) undergoing the SLECCOP associated with W-shaped ileocystoplasty with a catheterizable channel (Abol-Enein and Ghoneim procedure). Patients who received other types of bladder augmentation or W-shaped ileocystoplasty without a catheterizable channel were excluded. Patient records were reviewed for demographic information, surgical data, and long-term outcomes. Results: This study included 52 children [33 boys, median age: 8.5 (0.8-18) years]. Pathological conditions included 28 children with the bladder exstrophy and epispadias complex (BEEC), 11 with neurogenic bladders, and 13 with other pathologies. Two patients underwent total bladder substitution. Thirty-four (65%) patients had bladder neck reconstruction (BNR), with 23 undergoing the SLECCOP and ileocystoplasty and 11 having prior BNR. All stomas, except for two, were umbilical, and were associated with omphaloplasty in 28 patients with the BEEC. A total of 40 stomas were created using the appendix (77%) and 12 with a Monti tube (23%). Stoma-related complications included cutaneous strictures (n = 2, 4%) and leaks (n = 10, 19%), all treated by dextranomer/hyaluronic acid copolymer injection (n = 10). A redo surgery was required in three patients: extraserosal wrapping was performed for persistent leakage (n = 2, 4%), and surgical revision was required for the Monti tube procedure (n = 1, 2%). Three patients (6%) underwent dilatation for transient stoma stenosis. Leakage occurred in 20% of appendix channels (n = 8/40) and 17% of Monti tubes (n = 2/12). Strictures were reported in 3% of appendix channels (n = 1/40) and 8% of Monti tubes (n = 1/12). Bladder stones developed in four patients (8%). Channel leakage persisted in one patient (2%) at a median follow-up of 4.4 years (IQR 1.4-9.7). Conclusion: W-Ileal bladder augmentation with the SLECCOP is an efficient technique for treating children with incontinence caused by different etiologies. The rate of channel complication is very low, specifically for strictures, in this complex population of patients.

Evaluating pediatric ureteropelvic junction obstruction: Dynamic magnetic resonance urography vs renal scintigraphy 99m-technetium mercaptoacetyltriglycine.

BACKGROUND: Ureteropelvic junction obstruction (UPJO) is a common congenital urinary tract disorder in children. It can be diagnosed as early as in utero due to the presence of hydronephrosis or later in life due to symptomatic occurrence. AIM: To evaluate the discrepancy between dynamic contrast-enhanced magnetic resonance urography (dMRU) and scintigraphy 99m-technetium mercaptoacetyltriglycine (MAG-3) for the functional evaluation of UPJO. METHODS: Between 2016 and 2020, 126 patients with UPJO underwent surgery at Robert Debré Hospital. Of these, 83 received a prenatal diagnosis, and 43 were diagnosed during childhood. Four of the 126 patients underwent surgery based on the clinical situation and postnatal ultrasound findings without undergoing functional imaging evaluation. Split renal function was evaluated preoperatively using scintigraphy MAG-3 (n = 28), dMRU (n = 53), or both (n = 40). In this study, we included patients who underwent surgery for UPJO and scintigraphy MAG-3 + dMRU but excluded those who underwent only scintigraphy MAG-3 or dMRU. The patients were divided into groups A (< 10% discrepancy) and B (> 10% discrepancy). We examined the discrepancy in split renal function between the two modalities and investigated the possible risk factors. RESULTS: The split renal function between the two kidneys was compared in 40 patients (28 boys and 12 girls) using scintigraphy MAG-3 and dMRU. Differential renal function, as determined using both modalities, showed a difference of < 10% in 31 children and > 10% in 9 children. Calculation of the relative renal function using dMRU revealed an excellent correlation coefficient with renal scintigraphy MAG-3 for both kidneys. CONCLUSION: Our findings demonstrated that dMRU is equivalent to scintigraphy MAG-3 for evaluating split renal function in patients with UPJO.

Staged laparoscopic orchiopexy of intra-abdominal testis: Spermatic vessels division versus traction? A multicentric comparative study.

BACKGROUND: Staged laparoscopic management of intra-abdominal testes using pedicular section is recognized as gold standard technique, successful in 85 % of cases for scrotal testicular position with less than 10 % testicular atrophy. Recently, Shehata proposed a new technique without pedicular division for these testes, using spermatic vessels traction, but did not provide a comparative study of the two techniques. OBJECTIVE: To evaluate the laparoscopic spermatic pedicular traction (Shehata technique, ST) for the treatment of intra-abdominal testis, as an alternative to gold standard pedicular section (2-stage Fowler-Stephens, FS). STUDY DESIGN: Intra-abdominal testes of 129 patients in two tertiary pediatric urology centers were managed laparoscopically (2011-2019) either by 2-stage FS orchidopexy or ST according to the surgeon preference. Testicular position and size were statistically compared. RESULTS: A total of 147 testes were pulled down by 80 ST and 67 FS, including 18 bilateral cases. Median (IQR) age at surgery was 24.2 (15.6-46.4) months (ST) and 18.3 (13.1-38.2) months (FS) (p = 0.094). Scrotal pulling-down of the testis was performed after a median (IQR) period of 2.3 (1.6-3.4) months (ST) and 6.1 (4.7-8.3) months (FS), respectively (p < 0.005). Although ST had collapsed in 17 cases (21.3 %), only one (1.3 %) redo procedure was required. After a median (IQR) follow-up of 22 (12-40) and 19 (8.75-37) months (p = 0.59), the testis was in the scrotum in 85 % and 81 % of ST and FS cases, respectively (p = 0.51). Testicular atrophy occurred in 10 % of ST and 13.4 % of FS (p = 0.61). Multivariate analysis using the propensity score analysis did not identify any difference between the two techniques. DISCUSSION: Our results seem to confirm that FS and ST achieve the same results regarding final testicular position and testicular atrophy rate, with a long-term follow-up. Our study supports pediatric surgeons to favor laparoscopic spermatic pedicular traction (ST) which preserves the testicular vascularization and may ensure better spermatogenesis after puberty. More details on the size and position of the testicle at the beginning of the first laparoscopy seem however essential to assess more accurately the outcomes of each surgical technique. Our outcomes will also be re-evaluated when our patients have reached puberty, from an exocrine and endocrine points of view. CONCLUSIONS: This study showed similar results after laparoscopic traction or section of spermatic vessels for intra-abdominal testis in a long-term follow-up, providing more evidence for the use of ST as a valuable alternative to FS.