Schäfer Frank-Mattias, Schwab-Eckhardt Benjamin, Voß Egbert, Schroth Michael, Staudt Franz, Stehr Maximilian
Department of Pediatric Surgery and Pediatric Urology, Cnopfsche Kinderklinik, 90419 Nürnberg, Germany.
Clinic of Urology and Pediatric Urology, University Hospital Erlangen, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg, 91054 Erlangen, Germany.
Children (Basel). 2024 Sep 9;11(9):1104. doi: 10.3390/children11091104.
BACKGROUND/OBJECTIVES: In recent years, changing paradigms, both culturally and scientifically, have fundamentally altered the approach to the treatment of children with Disorders of Sexual Development (DSD) prior to reaching the age of legal consent. In Germany, the situation changed with the introduction of legislation that includes a partial ban on DSD surgery in children in 2021. This study aims to analyze the impact of this legislation on clinical practice.
From 2014 to 2024, all patients with DSD in our institution were included. The study group comprised all patients operated on after the legislation. All patients operated on before the legislation served as the control group. Karyotype, phenotype, resulting type of DSD, age at presentation and age at operation were recorded.
A total of 35 patients were included in this study, with 15 in the study group and 20 in the control group. The operation was authorized by the family court for all patients in the study group. A total of 46,XY patients with severe hypospadias and clinical aspect of intersexual outer genitalia were the largest proportion (25 patients, 71.4%). Nine patients (25.7%) were 46,XX girls with classical congenital adrenal hyperplasia (CAH) type. One patient (2.9%) showed a mixed gonadal dysgenesis. The mean age of the patients at first presentation in our institution was 10.7 months in the control group and 11.0 months in the study group. The mean age at operation was significantly higher in the study group (20.1 months) compared to the control group (15.1 months; = 0.032, unpaired -test).
The introduction of the legislation with a partial ban of genital surgery in DSD children in Germany has led to a significant delay in surgery. Since the majority of the patients comprise severe hypospadias and 46,XX CAH patients, further amendments of the law are proposed to minimize potential harm.
背景/目的：近年来，文化和科学范式的转变从根本上改变了对性发育障碍（DSD）儿童在达到法定同意年龄之前的治疗方法。在德国，随着2021年引入包括部分禁止对儿童进行DSD手术的立法，情况发生了变化。本研究旨在分析该立法对临床实践的影响。
纳入2014年至2024年在我们机构的所有DSD患者。研究组包括立法后接受手术的所有患者。所有在立法前接受手术的患者作为对照组。记录核型、表型、DSD的最终类型、就诊年龄和手术年龄。
本研究共纳入35例患者，研究组15例，对照组20例。研究组所有患者的手术均得到家庭法院的授权。共有46，XY且患有严重尿道下裂和两性畸形外生殖器临床表现的患者占比最大（25例，71.4%）。9例患者（25.7%）为46，XX型经典先天性肾上腺皮质增生症（CAH）女孩。1例患者（2.9%）表现为混合性性腺发育不全。对照组患者首次在我们机构就诊的平均年龄为10.7个月，研究组为11.0个月。研究组的平均手术年龄（20.1个月）明显高于对照组（15.1个月；P = 0.032，非配对t检验）。
德国引入部分禁止对DSD儿童进行生殖器手术的立法导致手术显著延迟。由于大多数患者为严重尿道下裂和46，XX CAH患者，建议进一步修订法律以尽量减少潜在危害。
