Post-operative complications following masculinizing genitoplasty in moderate to severe genital atypia: results from a multicenter, observational prospective cohort study.
Post-operative complications following masculinizing genitoplasty in moderate to severe genital atypia: results from a multicenter, observational prospective cohort study.
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中到重度生殖器异型性的男性化生殖器成形术术后并发症：一项多中心观察性前瞻性队列研究的结果。
DOI：
10.1016/j.jpurol.2021.02.006
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发表时间：
2021-06
影响因子：
2
通讯作者：
Kolon TF
中科院分区：
文献类型：
--
作者：
Long CJ;Van Batavia J;Wisniewski AB;Aston CE;Baskin L;Cheng EY;Lakshmanan Y;Meyer T;Kropp B;Palmer B;Nokoff NJ;Paradis A;VanderBrink B;Scott Reyes KJ;Yerkes E;Poppas DP;Mullins LL;Kolon TF
性发育差异 (DSD) 是存在非典型染色体、性腺和/或表型性别的先天性疾病。作为男性抚养的患者中，严重生殖器异型性的表型相对罕见，并且缺乏治疗标准。在这些罕见病例中，早期手术计划的决策包括但不限于异型性程度、睾丸位置和苗勒氏管残余的存在。在这项研究中，我们描述了针对中度至重度生殖器异型性的年轻男性患者进行男性化生殖器成形术的手术方法和短期结果，这些患者的父母选择了早期手术。这项由 NIH 赞助的研究是一项持续、观察性、多中心调查，评估因 DSD 导致非典型生殖器受影响的儿童及其父母的医疗、手术和心理结果。参与者前瞻性地从美国 12 家专门从事 DSD 护理的儿童医院招募。儿童入组的标准是奎格利评分为 3-6，具有 46, XY 或 45,X/46, XY 染色体补体，年龄 <3 岁，以前没有做过生殖器整形术；患者的纳入与是否进行生殖成形术无关。美容根据 4 点 Likert 量表进行分级，并发症根据 Clavian-Dindo 分类进行分级。在 31 名参与者中，30 名患者接受了尿道下裂修复术，1 名患者未接受生殖器成形术。大多数参与者 (22) 接受了分阶段尿道下裂修复术。在 12 个月的随访中，31 名儿童中的 12 名（41%）发现了 17 种并发症。龟头裂开和尿道皮肤瘘是最常见的并发症。 14 名（44%）参与者进行了睾丸固定术，4 名（13%）参与者进行了条纹性腺切除。父母和外科医生都报告说，与基线相比，手术后美容效果有所改善。大多数符合学习条件的儿童都选择了生殖器整形术。所有外科医生都没有采用单一的手术方法来将 46, XY 或 45,X/46, XY DSD 的年轻患者中度至重度生殖器模糊性男性化。因严重尿道下裂而接受生殖器成形术的患者中有 41% 出现并发症。总体而言，根据父母和外科医生的判断，生殖器整形术后生殖器的外观有所改善。早期生殖器成形术的结果需要指导家庭为年幼的孩子做出此类手术的决定。河图比较了母亲、父亲和外科医生随时间变化的李克特美容评分。 A 组包括队列中的所有患者。
Differences of sex development (DSD) are congenital conditions in which there is atypical chromosomal, gonadal and/or phenotypic sex. A phenotype of severe genital atypia in patients raised as male is a relatively rare occurrence and standards for management are lacking. Decision making for early surgical planning in these rare cases includes, but is not limited to, degree of atypia, location of testes, and presence of Müllerian remnants. In this study we describe surgical approaches and short-term outcomes for masculinizing genitoplasty in moderate to severe genital atypia in young patients raised male, for whom parents opted for early surgery. This NIH-sponsored study is an ongoing, observational, multicenter investigation assessing medical, surgical and psychological outcomes in children and their parents affected by atypical genitalia due to DSD. Participants were prospectively enrolled from 12 children’s hospitals across the United States that specialize in DSD care. Criteria for child enrollment were a Quigley score of 3–6 in those with a 46, XY or 45,X/46, XY chromosome complement, age <3 years with no previous genitoplasty; patients were included independent of whether genitoplasty was performed. Cosmesis was graded according to a 4-point Likert scale and complications per the Clavian-Dindo classification. Of the 31 participants, 30 underwent hypospadias repair and 1 patient did not undergo a genitoplasty procedure. The majority of participants (22) received a staged hypospadias repair. Seventeen complications were identified in 12 of the 31 children (41%) at 12 months of follow up. Glans dehiscence and urethrocutaneous fistula were the most common complications. Orchiopexy was performed in 14 (44%) and streak gonads were removed in 4 (13%) participants. Both parents and surgeons reported improved cosmesis after surgery when compared to baseline. Genitoplasty was chosen by parents for the majority of children eligible for study. No single surgical approach for masculinizing moderate to severe genital ambiguity in young patients with 46, XY or 45,X/46, XY DSD was adopted by all surgeons. Complications occurred in 41% of those who underwent genitoplasty for severe hypospadias. Overall, appearance of the genitals, as determined by parents and surgeons, improved following genitoplasty. Outcomes of early genitoplasty are needed to guide families when making decisions about such procedures for their young children. River plot comparing Likert cosmesis score over time for mothers, fathers, and surgeons. Panel A includes all patients in the cohort.
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影响因子：
2.1
作者：
Perlmutter, Adam E.;Morabito, Rocco;Tarry, William F.
通讯作者：
Tarry, William F.
影响因子：
2.6
作者：
Park, Kwan Hyun;Lee, Joo Hwan;Song, Sang Yong
通讯作者：
Song, Sang Yong
影响因子：
6.6
作者：
Hensle, TW;Tennenbaum, SY;Pollard, J
通讯作者：
Pollard, J
影响因子：
6.6
作者：
Rusnack, SL;Wu, HY;Canning, DA
通讯作者：
Canning, DA
影响因子：
3.3
作者：
Nistal, M;Riestra, ML;Paniagua, R
通讯作者：
Paniagua, R