scholarly journals Genital Reconstructive Surgery in Females With Congenital Adrenal Hyperplasia: A Systematic Review and Meta-Analysis

2018 ◽  
Vol 103 (11) ◽  
pp. 4089-4096 ◽  
Author(s):  
Jehad Almasri ◽  
Feras Zaiem ◽  
Rene Rodriguez-Gutierrez ◽  
Shrikant U Tamhane ◽  
Anoop Mohamed Iqbal ◽  
...  
Keyword(s):  
Sexual Function ◽  
Congenital Adrenal Hyperplasia ◽  
Reconstructive Surgery ◽  
Health Care Professionals ◽  
Surgical Outcomes ◽  
Sexual Experience ◽  
Sexually Active ◽  
Adrenal Hyperplasia ◽  
Tract Infections

Abstract Background Females with congenital adrenal hyperplasia (CAH) and atypical genitalia often undergo complex surgeries; however, their outcomes remain largely uncertain. Methods We searched several databases through 8 March 2016 for studies evaluating genital reconstructive surgery in females with CAH. Reviewers working independently and in duplicate selected and appraised the studies. Results We included 29 observational studies (1178 patients, mean age at surgery, 2.7 ± 4.7 years; mostly classic CAH). After an average follow-up of 10.3 years, most patients who had undergone surgery had a female gender identity (88.7%) and were heterosexual (76.2%). Females who underwent surgery reported a sexual function score of 25.13 using the Female Sexual Function Index (maximum score, 36). Many patients continued to complain of substantial impairment of sensitivity in the clitoris, vaginal penetration difficulties, and low intercourse frequency. Most patients were sexually active, although only 48% reported comfortable intercourse. Most patients (79.4%) and treating health care professionals (71.8%) were satisfied with the surgical outcomes. Vaginal stenosis was common (27%), and other surgical complications, such as fistulas, urinary incontinence, and urinary tract infections, were less common. Data on quality of life were sparse and inconclusive. Conclusion The long-term follow-up of females with CAH who had undergone urogenital reconstructive surgery shows variable sexual function. Most patients were sexually active and satisfied with the surgical outcomes; however, some patients still complained of impairment in sexual experience and satisfaction. The certainty in the available evidence is very low.

scholarly journals Surgical Outcomes of Clitoroplasty in Children with Congenital Adrenal Hyperplasia and Clitoral Hypertrophy: A 19-Year Experience of a Single Surgeon

2021 ◽  
Vol 18 (21) ◽  
pp. 11152
Author(s):  
Jaebeom Jun ◽  
Sang Hoon Song ◽  
Sungchan Park ◽  
Jae Hyeon Han ◽  
Kun Suk Kim
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Surgical Outcomes ◽  
Medical Records ◽  
Perioperative Complications ◽  
Surgical Techniques ◽  
Operation Time ◽  
Adrenal Hyperplasia ◽  
Tertiary Referral Hospital ◽  
The Mean

This study aimed to describe the experience with clitoroplasty for clitoral hypertrophy in patients with congenital adrenal hyperplasia of a single surgeon. The medical records of female pediatric patients with congenital adrenal hyperplasia who underwent clitoroplasty at a tertiary referral hospital between 2002 and 2020 were retrospectively analyzed. Three different surgical techniques were applied for clitoroplasty: recession without reduction, reduction and recession, and girth reduction and recession. A total of 104 patients underwent clitoroplasty for clitoral hypertrophy. The median patient age at the time of surgery was 10 months (range, 4 months to 10 years). The operation time was longer in reduction clitoroplasty than in recession clitoroplasty without reduction (median, 153 vs. 111 min, p = 0.003). The mean postoperative pain score of the patients did not differ among the different clitoroplasty techniques. During the mean follow-up of 37.7 months, nine (8.6%) patients underwent reperformed clitoroplasty. The rate of reperformed operation was significantly higher in patients who underwent reduction clitoroplasty (17.3%) than in those who underwent recession without reduction (2%) or girth reduction and recession (0%) (p = 0.031). Early clitoroplasty in patients with congenital adrenal hyperplasia yielded good mid-term surgical outcomes in terms of cosmesis and recurrence rate, with minimal perioperative complications.

scholarly journals Feminizing genitoplasty in childhood: aiming for achievable outcomes

2020 ◽  
Vol 16 (1) ◽  
Author(s):  
Amr Abdelhamid AbouZeid
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
External Genitalia ◽  
Urogenital Sinus ◽  
Sexually Active ◽  
Adrenal Hyperplasia ◽  
Vaginal Reconstruction ◽  
Feminizing Genitoplasty ◽  
Early Reconstruction ◽  
Different Cultures

Abstract Background Early genital reconstruction may be recommended in cases of congenital adrenal hyperplasia to avoid the stigma that can affect these individuals with variable degrees through different cultures. However, the separation and mobilization of a high vagina has remained challenging with less satisfactory surgical outcomes. Therefore, the value of early vaginal reconstruction has been questioned in favour of delaying the whole repair after puberty. In this report, the author has adopted the third option in-between, which comprises early reconstruction of the external genitalia and delaying the more challenging vaginal reconstruction (if needed) to be performed after puberty. Results The study included ten consecutive cases of CAH who underwent feminizing genitoplasty during the period 2016 through 2019. Their age at operation ranged from 8 to 84 months (mean 31; median 15). In five cases (50%), the technique of limited urogenital sinus mobilization adopted in this report succeeded in bringing the vaginal introitus down to the perineum. Those cases had originally a low vagina. In the rest of cases, labial retraction showed a common but wide urogenital introitus perfectly lined by urogenital mucosa. The outcomes have been considered satisfactory to a great extent for both parents and doctors regarding cosmesis and lack of functional complications (voiding problems). Longer follow up is still needed to assess the sexual function when these girls become sexually active. Conclusion In cases of congenital adrenal hyperplasia, reduction clitoroplasty combined with partial urogenital sinus mobilization can achieve predictable and satisfactory outcomes. In about 50% of cases, this approach is sufficient to bring the vagina down to the perineum. In the other half of cases with higher vagina, the possibility of satisfactory sinus intercourse can be studied when these girls grow and become sexually active; otherwise, a delayed vaginal reconstruction may turn to be necessary.

scholarly journals Concurrence of Meningomyelocele and Salt-Wasting Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
Heves Kırmızıbekmez ◽  
Rahime Gül Yesiltepe Mutlu ◽  
Serdar Moralıoğlu ◽  
Ahmet Tellioğlu ◽  
Ayşenur Cerrah Celayir
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Growth Retardation ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Deficiency ◽  
Salt Wasting ◽  
Significant Regression ◽  
One Year ◽  
The One ◽  
21 Hydroxylase Deficiency

Congenital adrenal hyperplasia (CAH) is a group of inherited defects of cortisol biosynthesis. A case of classical CAH due to 21-hydroxylase deficiency (21-OHD) with early onset of salt waste and concurrence of meningomyelocele (MMC) was presented here. The management of salt-wasting crisis which is complicated by a postrenal dysfunction due to neurogenic bladder was described. Possible reasons of growth retardation in the one-year follow-up period were discussed. A significant regression of the phallus with proper medical treatment was also mentioned.

scholarly journals The role of imaging in congenital adrenal hyperplasia

2014 ◽  
Vol 58 (7) ◽  
pp. 701-708 ◽  
Author(s):  
Sara Reis Teixeira ◽  
Paula Condé Lamparelli Elias ◽  
Marco Túlio Soares Andrade ◽  
Andrea Farias Melo ◽  
Jorge Elias Junior
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adrenal Tumors ◽  
Adrenal Hyperplasia ◽  
Salt Wasting ◽  
Testicular Adrenal Rest Tumors ◽  
High Incidence ◽  
Adrenal Rest ◽  
Hormonal Evaluation

Congenital adrenal hyperplasia (CAH) is an autossomic recessive disorder caused by impaired steroidogenesis. Patients with CAH may present adrenal insufficiency with or without salt-wasting, as well as various degrees of virilization and fertility impairment, carrying a high incidence of testicular adrenal rest tumors and increased incidence of adrenal tumors. The diagnosis of CAH is made based on the adrenocortical profile hormonal evaluation and genotyping, in selected cases. Follow-up is mainly based on hormonal and clinical evaluation. Utility of imaging in this clinical setting may be helpful for the diagnosis, management, and follow-up of the patients, although recommendations according to most guidelines are weak when present. Thus, the authors aimed to conduct a narrative synthesis of how imaging can help in the management of patients with CAH, especially focused on genitography, ultrasonography, computed tomography, and magnetic resonance imaging.

scholarly journals Sonographic features of the testicular adrenal rests tumors in patients with congenital adrenal hyperplasia: a single-center experience and literature review

2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Li Ma ◽  
Yu Xia ◽  
Linlin Wang ◽  
Ruifeng Liu ◽  
Xuepei Huang ◽  
...  
Keyword(s):  
Literature Review ◽  
Congenital Adrenal Hyperplasia ◽  
Medical Center ◽  
Semen Analysis ◽  
Steroid Treatment ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Deficiency ◽  
Scrotal Ultrasound ◽  
Testicular Lesions

Abstract Purpose Testicular adrenal rests tumor (TART) is a rare kind of benign tumor in the testis. It usually occurred secondary to congenital adrenal hyperplasia (CAH), a hormonal disorder caused by hydroxylase deficiency. As the first-line examination method, ultrasound provides crucial diagnostic information for TART, although misdiagnosis to malignancy is quite common because of its rare prevalence. We aimed to summarize the sonographic manifestations of TART to improve the diagnostic accuracy and specificity. Methods Eight CAH patients with TART identified by ultrasound in our medical center were retrospectively reviewed. Clinical and hormonal profile, semen analysis and treatment choices were collected. Sonographic examinations were performed at the first evaluation and interpreted by experienced radiologists individually. All patients received regular follow-up, and 5 patients undertook repeated scrotal ultrasound. A literature review of TART in CAH patients was conducted, with 123 patients from 23 articles since 1990 included. Results A total of 8 patients aged between 4 to 27 years old were enrolled. 7 of 8 (87.5%) patients exhibited bilateral testicular lesions. The sizes of the testicular lesions were between 0.18 ml to 5.68 ml, and all showed a clear boundary. 10/15 (66.7%) lesions were homogenously hypoechoic, 4/15 (26.7%) were heterogeneously iso-hypoechoic, and 1/15 (6.7%) were homogenously isoechoic. 10/15 (66.7%) lesions were hyper-vascular. The longitudinal follow-up of 5 patients showed testicular lesions changed in terms of size, echogenicity, and vascularity after steroid treatment. A potential correlation may exist between ACTH levels and tumor size (p = 0.066). From the literature review, 100/123 (81%) patients got bilateral lesions, and 95% of them were located near the mediastinum. 80/103 (78%) lesions exhibited a clear boundary, and predominant lesions (74%) were hypoechogenic. Vascularity was with great diversity. Seventy-nine lesions of 44 patients were followed-up by scrotal ultrasound, among which 29 (37%) remained unchanged, 29(37%) shrank, and 21(27%) disappeared. Conclusions Key sonographic characteristics of TART are: resembled lesions on both testes, located near the mediastinum, clear boundary, and changed in size or echogenicity after steroid treatment. These features can help radiologists to make an accurate diagnosis of TART.

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