Zhao Shimeng,Cui Xiaowei,Cao Yanyan,et al.Analysis of postoperative complications and surgical outcomes of feminizing genitoplasties in girls with congenital adrenal hyperplasia and masculinized external genitalia across different age groups[J].Journal of Clinical Pediatric Surgery,2025,(03):236-242.[doi:10.3760/cma.j.cn101785-202308044-007]
不同年龄段先天性肾上腺皮质增生合并外生殖器男性化女童术后并发症与手术效果分析
- Title:
- Analysis of postoperative complications and surgical outcomes of feminizing genitoplasties in girls with congenital adrenal hyperplasia and masculinized external genitalia across different age groups
- 关键词:
- 先天性肾上腺皮质增生症; 性别发育异常; 女性化外生殖器成形术; 阴蒂成形术; 阴道成形术
- Keywords:
- Congenital Adrenal Hyperplasia; Disorders of Sex Development; Feminizing Genitoplasty; Clitoroplasty; Vaginoplasty
- 摘要:
- 目的 比较不同年龄段先天性肾上腺皮质增生(congenital adrenal hyperplasia,CAH)合并外生殖器男性化女童行女性化外生殖器成形术(feminizing genitoplasties,FG)后并发症与手术效果。方法 回顾性分析河北省儿童医院2010年3月至2022年8月收治的32例CAH合并外生殖器男性化女童的临床资料,以3岁为界将患儿分为婴幼儿组和非婴幼儿组。其中婴幼儿组15例,14例采用阴蒂成形术,1例采用阴蒂+阴道成形术;非婴幼儿组17例,16例采用阴蒂成形术,1例采用阴蒂+阴道成形术。两组患儿基线资料比较,除手术年龄差异有统计学意义外(P＜0.001),其余资料差异均无统计学意义(P＞0.05)。观察指标包括Prader分级、手术方式、手术时长、随访时间、随访时年龄、术后早期与晚期并发症及患儿监护人对术后外生殖器的满意度。结果 是否于婴幼儿期接受手术是术后外生殖器外观满意度的独立影响因素(OR=0.068,95%CI:0.005~0.849,P＜0.05)。接受随访的32例患儿中,共7例(21.88%)出现术后并发症;婴幼儿组2例,其中切口出血1例,早期切口感染伴开裂、晚期外阴瘢痕1例;非婴幼儿组5例,其中切口出血3例,留置尿管合并泌尿道感染2例;两组并发症发生率差异无统计学意义(P＞0.05)。两组外生殖器外观满意度比较结果显示,外观对称性、阴蒂大小、阴蒂位置、阴道口外观、阴唇外观、生殖器皮肤质量评分比较,差异均无统计学意义(P＞0.05);但两组外生殖器外观满意度总得分差异有统计学意义(P＜0.05)。结论 是否于婴幼儿期接受手术是CAH合并外生殖器男性化女童行FG术后外生殖器外观总体满意度的独立影响因素,3岁前行FG患儿其监护人的外生殖器外观满意度评分高于3岁后手术者,且术后并发症发生率较低。
- Abstract:
- Objective To compare the postoperative complications and outcomes of feminizing genitoplasties (FG) in girls with congenital adrenal hyperplasia (CAH) and masculinized external genitalia across different age groups.Methods We conducted a retrospective analysis of 32 girls with CAH and masculinized genitalia who underwent FG procedures at Hebei Children’s Hospital from March 2010 to August 2022.Patients were divided into two groups based on age:infants and young children (≤3 years) and older children group (>3 years).The infant group included 15 cases-14 underwent clitoroplasty and 1 underwent clitoroplasty plus vaginoplasty.The older group included 17 cases-16 received clitoroplasty and 1 received both clitoridoplasty and vaginoplasty.Clinical characteristics were compared between the groups,with significant differences only in surgical age ; other factors showed no statistically significant differences (P>0.05).Outcome measures included Prader staging,type of surgical procedure,operation time,follow-up duration,follow-up age,early and late postoperative complications,and caregiver satisfaction with postoperative genital appearance.Results Age at surgery was an independent predictor of overall genital appearance satisfaction postoperatively (OR=0.068,95%CI:0.005-0.849,P<0.05).Among the 32 girls,7(21.88%) experienced postoperative complications:2 cases (13.33%) in the infant group had complications,including incision bleeding (1 case) and early infection with later vulvar scarring (1 case).In the older group,5 cases (29.41%) experienced complications:incision bleeding (3 cases) and catheter-associated urinary infection (2 cases).There were no statistically significant differences between groups in complication rate (P>0.05).Comparative analysis of satisfaction with genital appearance showed no significant differences between the groups for symmetry,clitoral size,clitoral position,vaginal introitus appearance,labial appearance,and genital skin quality (P>0.05).However,the total appearance satisfaction score was significantly higher in the infant group.Conclusions Surgery during infancy is associated with higher overall satisfaction in postoperative genital appearance.FG performed before age 3 yields higher appearance satisfaction scores and lower complication rates than surgeries conducted later.
参考文献/References:
[1] Auer MK,Nordenstr?m A,Lajic S,et al.Congenital adrenal hyperplasia[J].Lancet,2023,401(10372):227-244.DOI:10.1016/S0140-6736(22)01330-7.
[2] Claahsen-van der Grinten HL,Speiser PW,Ahmed SF,et al.Congenital adrenal hyperplasia-current insights in pathophysiology,diagnostics,and management[J].Endocr Rev,2022,43(1):91-159.DOI:10.1210/endrev/bnab016.
[3] 中华预防医学会出生缺陷预防与控制专业委员会新生儿筛,中国医师协会青春期医学专业委员会临床遗传学组,中华医学会儿科学分会内分泌遗传代谢学组.先天性肾上腺皮质增生症新生儿筛查共识[J].中华儿科杂志,2016,54(6):404-409.DOI:10.3760/cma.j.issn.0578-1310.2016.06.003. The Subspecialty Group of Newborn Screening,Society of Birth Defects Prevention and Control,Chinese Preventive Medicine Association,The Subspecialty Group of Clinical Genetics,Society of Adolescent Medicine,Chinese Medical Doctor Association,The Subspecialty Group of Endocrinologic,Hereditary and Metabolic Diseases,The Society of Pediatrics,Chinese Medical Association.Consensus statement on neonatal screening for congenital adrenal hyperplasia[J].Chin J Pediatr,2016,54(6):404-409.DOI:10.3760/cma.j.issn.0578-1310.2016.06.003.
[4] 中华医学会小儿外科学分会泌尿外科学组.性别发育异常中国专家诊疗共识[J].中华小儿外科杂志,2019,40(4):289-297.DOI:10.3760/cma.j.issn.0253-3006.2019.04.001. Group of Urological Surgery,Branch of Pediatric Surgery,Chinese Medical Association.Chinese expert consensus on disorders of sexual development[J].Chin J Pediatr Surg,2019,40(4):289-297.DOI:10.3760/cma.j.issn.0253-3006.2019.04.001.
[5] Shalaby M,Chandran H,Elford S,et al.Recommendations of patients and families of girls with 46XX congenital adrenal hyperplasia in the United Kingdom regarding the timing of surgery[J].Pediatr Surg Int,2021,37(1):137-143.DOI:10.1007/s00383-020-04780-3.
[6] Harris RM,Chan YM.Ethical issues with early genitoplasty in children with disorders of sex development[J].Curr Opin Endocrinol Diabetes Obes,2019,26(1):49-53.DOI:10.1097/MED.0000000000000460.
[7] Fisher RS,Espeleta HC,Baskin LS,et al.Decisional regret about surgical and non-surgical issues after genitoplasty among caregivers of female infants with CAH[J].J Pediatr Urol,2022,18(1):27-33.DOI:10.1016/j.jpurol.2021.10.001.
[8] Merke DP,Auchus RJ.Congenital adrenal hyperplasia due to 21-hydroxylase deficiency[J].N Engl J Med,2020,383(13):1248-1261.DOI:10.1056/NEJMra1909786.
[9] 中华医学会儿科学分会内分泌遗传代谢病学组.先天性肾上腺皮质增生症21-羟化酶缺陷诊治共识[J].中华儿科杂志,2016,54(8):569-576.DOI:10.3760/cma.j.issn.0578-1310.2016.08.003. The Subspecialty Group of Endocrinologic,Hereditary and Metabolic Diseases,the Society of Pediatrics,Chinese Medical Association.Consensus statement on diagnosis and treatment of congenital adrenal hyperplasia due to 21-hydroxylase deficiency[J].Chin J Pediatr,2016,54(8):569-576.DOI:10.3760/cma.j.issn.0578-1310.2016.08.003.
[10] 尤祥云,向振东,易成,等.Clavien-Dindo分级系统在泌尿外科的应用现状及前景[J].国际泌尿系统杂志,2021,41(1):160-163.DOI:10.3760/cmajcn431460-20190000-00046. You XY,Xiang ZD,Yi C,et al.Current situation and prospect of Clavien-Dindo grading system in urology[J].Int J Urol Nephrol,2021,41(1):160-163.DOI:10.3760/cmajcn431460-20190000-00046.
[11] Creighton SM,Minto CL,Steele SJ.Objective cosmetic and anatomical outcomes at adolescence of feminising surgery for ambiguous genitalia done in childhood[J].Lancet,2001,358(9276):124-125.DOI:10.1016/S0140-6736(01)05343-0.
[12] Fernandez N,Chavarriaga J,Pérez J.Complete corporeal preservation clitoroplasty:new insights into feminizing genitoplasty[J].Int Braz J Urol,2021,47(4):861-867.DOI:10.1590/S1677-5538.IBJU.2020.0839.
[13] Kudela G,Gawlik A,Koszutski T.Early feminizing genitoplasty in girls with congenital adrenal hyperplasia (CAH)-analysis of unified surgical management[J].Int J Environ Res Public Health,2020,17(11):3852.DOI:10.3390/ijerph17113852.
[14] Mouriquand PDE,Gorduza DB,Gay CL,et al.Surgery in disorders of sex development (DSD) with a gender issue:If (why),when,and how?[J].J Pediatr Urol,2016,12(3):139-149.DOI:10.1016/j.jpurol.2016.04.001.
[15] Szymanski KM,Whittam B,Kaefer M,et al.Parental decisional regret and views about optimal timing of female genital restoration surgery in congenital adrenal hyperplasia[J].J Pediatr Urol,2018,14(2):156.e1-156.e7.DOI:10.1016/j.jpurol.2017.11.012.
[16] De Sutter P.DSD:A discussion at the crossroads of medicine,human rights,and politics[J].Front Pediatr,2020,8:125.DOI:10.3389/fped.2020.00125.
[17] Bennecke E,Bernstein S,Lee P,et al.Early genital surgery in disorders/differences of sex development:patients’perspectives[J].Arch Sex Behav,2021,50(3):913-923.DOI:10.1007/s10508-021-01953-6.
[18] Burgu B,Duffy PG,Cuckow P,et al.Long-term outcome of vaginal reconstruction:comparing techniques and timing[J].J Pediatr Urol,2007,3(4):316-320.DOI:10.1016/j.jpurol.2006.09.008.
[19] Zainuddin AA,Grover SR,Soon CH,et al.Malaysian females with congenital adrenal hyperplasia:surgical outcomes and attitudes[J].Front Pediatr,2019,7:144.DOI:10.3389/fped.2019.00144.
[20] Roth JD,Casey JT,Whittam BM,et al.Characteristics of female genital restoration surgery for congenital adrenal hyperplasia using a large-scale administrative database[J].Urology,2018,115:162-167.DOI:10.1016/j.urology.2018.02.025.
[21] 李佳,摆俊博,王文光,等.尿生殖窦部分游离术治疗女性先天性肾上腺皮质增生症所致性发育异常的疗效评估[J].临床外科杂志,2022,30(2):134-138.DOI:10.3969/j.issn.1005-6483.2022.02.011. Li J,Bai JB,Wang WG,et al.Evaluation of partial urogenital sinus mobilization of sex development due to congenital adrenal hyperplasia in female children[J].J Clin Surg,2022,30(2):134-138.DOI:10.3969/j.issn.1005-6483.2022.02.011.
[22] Marei MM,Fares AE,Musa N,et al.Timing and outcome concerns regarding feminizing genitoplasty from the perspective of Egyptian families of girls with virilized external genitalia[J].Horm Res Paediatr,2016,85(1):49-57.DOI:10.1159/000442200.
[23] Bernabé KJ,Nokoff NJ,Galan D,et al.Preliminary report:surgical outcomes following genitoplasty in children with moderate to severe genital atypia[J].J Pediatr Urol,2018,14(2):157.e1-157.e8.DOI:10.1016/j.jpurol.2017.11.019.
[24] Ellerkamp V,Rall KK,Schaefer J,et al.Surgical therapy after failed feminizing genitoplasty in young adults with disorders of sex development:retrospective analysis and review of the literature[J].J Sex Med,2021,18(10):1797-1806.DOI:10.1016/j.jsxm.2021.07.008.
[25] Almasri J,Zaiem F,Rodriguez-Gutierrez R,et al.Genital reconstructive surgery in females with congenital adrenal hyperplasia:a systematic review and meta-analysis[J].J Clin Endocrinol Metab,2018,103(11):4089-4096.DOI:10.1210/jc.2018-01863.
[26] Bogdanska M,Morris A,Hutson JM,et al.Long-term urinary symptoms in adolescent and adult women with congenital adrenal hyperplasia[J].J Pediatr Urol,2018,14(3):240.e1-240.e5.DOI:10.1016/j.jpurol.2018.01.006.
[27] Trachta J,Mushtaq I,Petrasova N,et al.Lower urinary tract symptoms in adult females after feminizing genitoplasty for congenital adrenal hyperplasia[J].J Urol,2021,205(5):1483-1489.DOI:10.1097/JU.0000000000001509.
[28] Stites J,Bernabé KJ,Galan D,et al.Urinary continence outcomes following vaginoplasty in patients with congenital adrenal hyperplasia[J].J Pediatr Urol,2017,13(1):38.e1-38.e7.DOI:10.1016/j.jpurol.2016.10.012.
[29] Lean WL,Deshpande A,Hutson J,et al.Cosmetic and anatomic outcomes after feminizing surgery for ambiguous genitalia[J].J Pediatr Surg,2005,40(12):1856-1860.DOI:10.1016/j.jpedsurg.2005.08.045.
[30] Szymanski KM,Salama AK,Whittam B,et al.Beyond changing diapers:stress and decision-making among parents of girls with congenital adrenal hyperplasia seeking consultation about feminizing genital restoration surgery[J].J Pediatr Urol,2019,15(6):653-658.DOI:10.1016/j.jpurol.2019.09.022.
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备注/Memo
收稿日期:2023-8-30。
基金项目:河北省重点研发计划卫生健康创新专项项目(21377710D);河北省医学科学研究课题计划(20231130)
通讯作者:周云,Email:13803376203@163.com