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.

Pectus Arcuatum: A Pectus Unlike Any Other.

BACKGROUND: Pectus arcuatum is often mistaken for a type of pectus carinatum. However, pectus arcuatum is a unique clinical form of pectus caused by premature obliteration of the sternal sutures (manubrial sternum, four sternebrae and xiphoïd process), whereas pectus carinatum is due to abnormal growth of the costal cartilage. In order to better describe pectus arcuatum, we analysed the files of patients with pectus arcuatum followed in our centers. METHODS: Multicenter retrospective study of young patients' files diagnosed with pectus arcuatum. RESULTS: The clinical diagnosis of pectus arcuatum was made in 34 patients with a mean age at diagnosis of 10.3 years (4-23 years). A chest profile X-ray or a CT scan was performed in 16 patients (47%) and confirmed the diagnosis of PA by the presence of a sternal fusion. It was complete in 12 patients. A malformation was associated in 35% of cases (Noonan syndrome 33%, scoliosis 25% or cardiopathy 16%). 11 patients (32%) had a family history of skeletal malformation. Orthopedic treatment was initiated in 3 patients without any success. 11 patients underwent surgical correction, which was completed in 7 of them. CONCLUSION: The diagnosis of pectus arcuatum is based on clinical experience and if necessary, on a profile chest X-ray showing the fusion of the sternal pieces. It implies the search for any associated malformations (musculoskeletal, cardiac, syndromic). Bracing treatment is useless for pectus arcuatum. Corrective surgery, based on a sternotomy associated with a partial chondro-costal resection, can be performed at the end of growth. LEVEL OF EVIDENCE: IV.

3D imaging and urology: Why 3D reconstruction will be mandatory before performing surgery.

OBJECTIVE: We relate a single-center experience in virtual surgical planning to demonstrate interests and perspectives in pediatric urology. METHOD: From 2004 to April 2017, 4 patients were analyzed before intervention at our institution. All patients had undergone a low dose CT scan. The acquisition was then treated by a surface rendering software Pre-, per- and post-operative outcome were retrospectively collected.  RESULTS: 4 patients were operated on from 2004 to April 2017: two for oncological pathologies and two for congenital malformations. Mean age at intervention was 61 months (21-156 months). Two interventions were performed laparoscopically with one conversion. Mean operative time was 135 min (80-180 min). There were no complications.  CONCLUSION: 3D surgical planning should be mandatory in pediatric urology to perform the safest, the most accurate and effective surgery as possible.

ARTICULO SOLO EN INGLES.OBJETIVO: Relatamos la experiencia deun centro con la planificación quirúrgica virtual para demostrar los intereses y las perspectivas en urología pediátrica. MÉTODOS: Desde 2004 hasta abril 2017 se analizaron 4 pacientes antes de la intervención. Todos los pacientes habían sido sometidos a TAC de baja dosis. Laadquisición fue después tratada mediante un software de representación de superficie. Se recogieron los resultados pre-, peri- y postoperatorios  retrospectivamente. RESULTADOS: 4 pacientes fueron intervenidos entre 2004 y abril 2017: dos por patologías oncológicas y dos por malformaciones congénitas. La edad mediaen el momento de la intervención era de 61 meses (21-156 meses). Dos intervenciones fueron realizadas por vía laparoscópica con una conversión. El tiempo medio de operación fue 135 minutos (80-180 min). No hubo complicaciones. CONCLUSIONES: La planificación 3D debería ser obligatoria en urología pediátrica para la realización de la cirugía más segura, precisa y efectiva posible.

3D imaging and urology: why 3d reconstruction will be mandatory before performing surgery / Imagen 3D y Urología: Por qué la reconstrucción 3D será obligatoria antes de realizar la cirugía

Objective: We relate a single-center experience in virtual surgical planning to demonstrate interests and perspectives in pediatric urology. Method: From 2004 to April 2017, 4 patients were analyzed before intervention at our institution. All patients had undergone a low dose CT scan. The acquisition was then treated by a surface rendering software Pre-, per- and post-operative outcome were retrospectively collected. Results: 4 patients were operated on from 2004 to April 2017: two for oncological pathologies and two for congenital malformations. Mean age at intervention was 61 months (21-156 months). Two interventions were performed laparoscopically with one conversion. Mean operative time was 135 min (80-180 min). There were no complications. Conclusion:3D surgical planning should be mandatory in pediatric urology to perform the safest, the most accurate and effective surgery as possible

Objetivo: Relatamos la experiencia deun centro con la planificación quirúrgica virtual para demostrar los intereses y las perspectivas en urología pediátrica. Métodos: Desde 2004 hasta abril 2017 se analizaron 4 pacientes antes de la intervención. Todos los pacientes habían sido sometidos a TAC de baja dosis. La adquisición fue después tratada mediante un software de representación de superficie. Se recogieron los resultados pre-, peri- y postoperatorios retrospectivamente. Resultados: 4 pacientes fueron intervenidos entre 2004 y abril 2017: dos por patologías oncológicas y dos por malformaciones congénitas. La edad media en el momento de la intervención era de 61 meses (21-156 meses). Dos intervenciones fueron realizadas por vía laparoscópica con una conversión. El tiempo medio de operación fue 135 minutos (80-180 min). No hubo complicaciones. Conclusiones: La planificación 3D debería ser obligatoria en urología pediátrica para la realización de la cirugía más segura, precisa y efectiva posible