Pediatric Surgery Department, Faculty of Medicine, Ain Shams University.
Department of Radiodiagnosis, Faculty of Medicine, Ain Shams University.
J Pediatr Surg. 2020 May;55(5):977-984. doi: 10.1016/j.jpedsurg.2020.01.002. Epub 2020 Jan 23.
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.
在本报告中，我们旨在利用 MRI 的多平面能力和高软组织分辨率来定义影响 CAH 女性生殖器官的不同程度的结构异常。
该研究纳入了 2016 年至 2019 年期间因生殖器重建而转诊至我们小儿外科机构的 CAH 病例。我们在参考临床和手术发现的同时，研究了 CAH 的盆腔 MRI 解剖结构。为了建立 CAH 中不同程度的男性化分级量表，我们纳入了另外两组正常男孩和女孩的对照组，代表了两个极端。
该研究纳入了 23 例接受术前盆腔 MRI 检查的 CAH 病例。所有病例均具有正常的染色体分析（46XX）。他们在接受 MRI 检查时的年龄从 1 至 156 个月不等（平均 42.4；中位数 25）。阴道下端的水平在矢状 T2WI 中确定，并在连续的轴位切片中得到证实。根据阴道下端与耻骨联合的关系，我们将 CAH 病例分为低型或高型。此外，我们还可以观察到阴道下降程度与两性之间勃起组织结构变化之间的相关性。
MRI 可以通过显示内部异常的严重程度在 CAH 的评估中发挥重要作用，这对于适当的术前咨询至关重要。
病例对照研究。
III 级。
