Pediatric Surgery Department - Institute for Maternal and Child Health, IRCCS Burlo Garofolo, via dell'Istria, 65/1, Trieste, Italy.
Ital J Pediatr. 2021 Jun 29;47(1):141. doi: 10.1186/s13052-021-01089-2.
Sactosalpinx means a collection of fluid (serum, blood or pus) in the fallopian tube. CAH (Congenital Adrenal Hyperplasia) is a typical 46XX DSD (Disorder of Sex Development) due to a steroidogenic enzymatic defect. Both conditions are rare and can lead to reduced fertility rate.
We describe two post-menarche virgin girls with CAH who were hospitalized for acute abdomen due to laparoscopically confirmed sactosalpinx. Case 1 recovered after conservative management, case 2 after a second-look and bilateral salpingectomy. The first case consisted of right sactosalpinx and previous peritonitis reported; the second one of bilateral symptomatic pyosalpinx and previous vaginal stenosis. Recurrent abdominal pain persisted at follow-up in Case 1: post-operative MRI (Magnetic Resonance Imaging) showed bilateral hydrosapinx that disappeared at a following ultrasound scan control. Follow-up was uneventful 36 months after surgery in Case 2, except for the surgical revision of the vaginal introitus.
CAH-sactosalpinx association is a very rare but not negligible event. We suggest a conservative approach for sactosalpinx if tubal and/or ovary torsion can be excluded. Pyosalpinx is more challenging to treat, but during pediatric age we suggest starting with a conservative approach, especially in patients with CAH who have a potential low fertility rate. Careful gynecological follow-up after menarche is recommended to rule out any further causes of infertility.
输卵管积水是指输卵管内积聚的液体（血清、血液或脓液）。先天性肾上腺增生（CAH）是一种典型的 46XX 性发育障碍，由于类固醇生成酶缺陷所致。这两种情况都很少见，可能导致生育能力下降。
我们描述了两名患有 CAH 的初潮后处女，因腹腔镜证实的输卵管积水而住院治疗急性腹痛。病例 1 经保守治疗后痊愈，病例 2 经二次探查和双侧输卵管切除术治疗。第一例患者为右侧输卵管积水，并有既往腹膜炎病史；第二例患者为双侧症状性输卵管积脓，并有既往阴道狭窄病史。病例 1 随访时仍有持续性腹痛：术后 MRI 显示双侧输卵管积水，随后超声检查显示积水消失。病例 2 术后 36 个月除了阴道入口的手术修复外，无其他并发症。
CAH-输卵管积水的关联是一种非常罕见但不容忽视的情况。如果可以排除输卵管和/或卵巢扭转，我们建议对输卵管积水采取保守治疗方法。输卵管积脓更具挑战性，但在儿科时期，我们建议采用保守治疗方法，尤其是对于生育能力潜在较低的 CAH 患者。建议在初潮后进行仔细的妇科随访，以排除任何其他不孕原因。
