Transformation of the female genitalia in congenital adrenal hyperplasia: MRI study

• Published in: Journal of pediatric surgery 2020-05, Vol.55 (5), p.977-984
• Main Authors: AbouZeid, Amr Abdelhamid, Mohammad, Shaimaa Abdelsattar
• Format: Article
• Language: English
• Subjects: Classification; Disorders of sexual development; MRI; Urethra; Urogenital sinus; Vagina
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/j.jpedsurg.2020.01.002

In this report, we aim to define the different degrees of structural abnormality affecting the female genitalia in cases of CAH by using the multiplanar capabilities and high soft tissue resolution of MRI. The study included cases of CAH who were referred to our pediatric surgical facility for genital reconstruction during the period 2016 through 2019. We studied the pelvic MRI anatomy in cases of CAH while referring to clinical and operative findings. To set up a grading scale for the degree of virilization in cases of CAH, we included another two control groups of normal boys and girls representing the two ends of the spectrum. The study included 23 cases of CAH who underwent preoperative pelvic MRI examination. All cases had normal chromosomal analysis (46 XX). Their age ranged from 1 to 156 months at time of MRI examination (mean 42.4; median 25). The level of the lower end of the vagina was identified in midsagittal T2WI and confirmed in sequential axial cuts. Based on the level of the lower end of the vagina in relation to the pubic symphysis, we classified cases of CAH into either low or high types. Moreover, we could observe a correlation between the degree of vaginal descent and structural transformation of erectile tissue between both genders. MRI can have an important role in the evaluation of cases of CAH by displaying the severity of internal anomaly which is crucial for proper preoperative counseling. Case control study. Level III.