Surgical Evaluation Of Using The Inner Surface Of The Prepuce In Feminizing Genitoplasty In Cases Of Verilized Females With Congenital Adrenal Hyperplasia
试验速览
- 阶段
- 不适用
- 发起方
- 入组人数
- 5
- 试验地点
- 2
- 主要终点
- vaginal size calibration
研究概览
简要总结
Background and Rationale: The vaginoplasty remains a challenge for the surgeon. The most commonly used techniques have been: the Y-V plasty described by fortunoff for low vagina, the pullthrough operation described by Hendren and Crawford for high vagina, and the passerini-Glazer technique . None of these procedures is entirely satisfactory; with the first two, the neovagina has a tendency to become stenotic in most cases. Moreover, in the third, in addition to the high rate of stenosis, which is encountered in a third of case , the high rate of urethro-vaginal fistulas is unacceptable
Objectives :
To describe and evaluate a surgical technique for vaginoplasty that is easy to realize with fewer complications especially vaginal stenosis.
Study population & Sample size : 24 patients suffering from congenital adrenal hyperplasia (CAH) presenting to outpatient clinic of diabetis Endocrine And Metabolism Pediatric Unit (DEMPU) of Cairo University Specialized Pediatric Hospital will be considered.
Study Design : non-controlled prospective clinical trial with all patients included in single group
Methods: Cystoscopy will be done promptly before proceeding to surgery, Confluence depth more than 20 mm is considered high anomaly, feminizing genitoplasty will be done as a one-stage procedure, One month after operation, examination under anesthesia will be done with calibration of vagina.
Possible Risk (s) to study population : The risk of this study is involving a vulnerable group of females which exposed to lengthy operation may complicate with bleeding and need for blood transfusion, infection early after surgery or vaginal stenosis.
Outcome parameter (s):
Vaginal calibration using hegars dilators Urodynamics for females older than 3 years and complaining from incontinence
详细描述
• Background and Rationale: Disorders of sex development(DSD) are medical conditions in which the development of chromosomal, gonadal, or anatomic sex varies from normal and may be incongruent with each other. Five main groups of DSD patients may be identified, submitted to the gender assignment process, and may be considered for a surgical genital reconstruction. (Lee Pa et al, 2006) Congenital adrenal hyperplasia (CAH) refers to a group of autosomal recessive disorders resulting from deficiency of one of the five enzymes required for synthesis of cortisol in the adrenal cortex. CAH is the most common cause of genital ambiguity. (van der Zwan, 2013) About 90-95% of individuals with CAH have a mutation in CYP21A2 gene, encoding 21-hydroxylase enzyme. The worldwide incidence of classical 21 OHD is 1:15,000 to 1:16,000 live births of which approximately 75% are salt wasters. That leads to a block of normal production of cortisone and results in overproduction of steroid metabolism by-products through pituitary feedback. These androgenic steroids lead to a variable degree of masculinization of normal female fetus.
The goal of therapy in CAH is to both correct the deficiency in cortisol secretion and suppresses adrenocorticotropic hormone (ACTH) overproduction. Proper treatment with glucocorticoid reduces stimulation of the androgen pathway, thus preventing further virilization and allowing normal growth and development. In addition to hormonal therapy, feminizing reconstructive surgery targets the correction of anatomical disturbance. The feminizing genitoplasty operation consists of reducing the size of the phallus (clitoroplasty), creating labia minora and vaginoplasty.
• Sample size: Sample size calculation: n = 2(Za+Z1-β)2σ2/Δ2 n sample size Za = 1.65 ( p<0.05 as acceptable and a study with 80% power; using the below table, we get the following values: Zα, is 1.65 a error 5% 1% .1% 2 sided 1.96 2.5758 3.2905
1 sided 1.65 2.33 Z1-,β= 0.8416 power 80% 85% 90% 95% value 0.8416 1.0364 1.2816 1.6449 σ standard deviation =0 .5 Δ effect size 36% =0.36 Effect of using the prepuce in minimizing vaginal stenosis is 100% - effect of using other techniques 64%
N =2(1.65+0.8416)2 0.52 / 0.362 = 24 patients.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Treatment
- 盲法
- None
入排标准
- 年龄范围
- 18 Months 至 14 Years（Child）
- 性别
- Female
- 接受健康志愿者
- 否
入选标准
- •Females (46, XX genotype identified by karyotype
- •Age: infants and children from 18 months old age to 14 years
- •Virilized external genitalia.
- •Persistent urogenital sinus
- •Congenital adrenal hyperplasia.
- •Controlled by glucocorticoid and mineralocorticoid replacement therapy and clinically and hormonally judged fit for surgical interventions.
- •Voluntarily provided informed written consent of the parents for the planned procedure and possible risks.
- •Informed written approval of the parents of the included children to use clinical data collected in a research program.
排除标准
- •Male disorders of sexual development (46 XY)
- •Debatable sex of rearing; Mixed gonadal dysgenesis, Ovotesticular disorders of sexual development and partial androgen insensitivity syndrome.
- •Inadequate compliance with corticosteroid therapy, rendering surgery or interventions clinically hazardous or unsuitable.
- •Inadequately controlled hormonal assay: elevated androgens.
- •Patients who did any corrective surgery before.
结局指标
主要结局
vaginal size calibration
时间窗: 6 months to one year
the new vagina will be assessed for stenosis 1 month after the procedure using Hegar dilators under general anaesthesia, further calibration will be done over 6 months to ensure patency of the vagina and to avoid stenosis by dilatation if stenosis encountered
次要结局
- urinary incontinence(6 months)
研究者
Mahmoud Tarek Mohamed Bahget
Assistant lecturer
Kasr El Aini Hospital