Sactosalpinx is a rare cause of abdominal pain in pediatric age and the clinical picture range from asymptomatic form to acute abdomen.
It occurs when a distally blocked fallopian tube fills with fluid collection of serum (hydrosalpinx), blood (hematosalpinx) or pus (pyosalpinx).
Occlusion of the fimbriated end of the tube may lead to tubal dilatation, usually in the ampulla and infundibulum. As far as the etiology is concerned, primary causes involve the anatomy, like abnormalities of length, mobility and structure. Secondary causes are acquired. The most common cause of hydrosalpinx is pelvic inflammatory disease, other causes include endometriosis, peri-tubal adhesion from a previous operation, tubal cancer and tubal pregnancy [9]. These secondary causes are typical of the adult age. Hydrosalpinx is far less common in children and adolescent. In pediatric age the pathogenesis remain ill-defined [10]. It has been strongly suggested that in younger non-sexually active women, a hydrosalpinx occurs secondary to a developmental defect [11]. A systematic review in 2015 found 66 total cases described in literature and demonstrated comparable outcomes between surgical, medical and conservative management [12]. In this review an association was found with: congenital hydrocolpos/hydrometra, imperforate anus; congenital fallopian atresia; imperforate hymen; Herlyn-Werner-Wunderlich syndrome; Mayer-Rokitansky-Küster-Hauser syndrome; Hirshsprung’s disease; persistent urogenital sinus; bicornate uterus/septate vagina; previous abdomino-pelvic surgery; complex genitourinary malformation (Müllarian abnormality).
US and MRI guide for diagnosis. On MRI, hydrosalpinx appears as a fluid-filled C- or S-shaped tubular structure that arise from the upper lateral margin of the uterus [9], but often definite diagnosis requires an explorative laparoscopy or laparotomy.
CAH is the most common cause of 46XX-DSD due to fetal androgen exposure [13]. It is an autosomal recessive disorder involving synthesis of cortisol. The most common form is the classic form due to 21-hydroxylase deficiency: an excessive adrenal androgen biosynthesis results in masculinization of the genitalia in XX offspring [1] and lead to ambiguity of the external genitalia and salt wasting syndrome. Just virilizing forms without salt waste exist. Concerning female newborn, karyotype (46XX), increase of 17-OH-hydroxyprogesterone and genital inspection may lead the diagnosis. Replacement therapy with steroid and often mineralocorticoid drugs is required. To conform genital anatomy to the sex of rearing, FG is often required (timing is still controversial). Clitoris and UGS surgery consist of an appropriate reconstructive procedure that should be individualized according to patient genital anatomy. FG generally includes urethroplasty, vaginoplasty, clitoroplasty and labioplasty.
The most challenging part of this intervention is the separation of the vagina from the urethra, especially in cases with a high confluence (UGS length more than 3 cm). Besides the technical difficulty, the patient is at risk for post-operative urinary incontinence, urethra-vaginal fistula and vaginal or urethral stenosis [3].
To facilitate reconstruction and decrease the risk of complications, partial UGS mobilization was described [4, 5]. Rink et al. [4] applied this technique to children with CAH through a perineal prone approach. It also allowed en bloc mobilization of the urethrovaginal confluence, with repositioning of the urethral and vaginal orifices in a separate orthotopic location.
The goal of surgical reconstruction for girls with CAH and UGS are recreating normal appearing and functional external genitalia, preserving bladder function, and maximizing the potential for a normal sexual and reproductive adult life. This goal may be technically harder to achieve in children with a high urethra-vaginal confluence and higher degrees of virilization.
CAH and sactosalpinx are two rare in pediatric age and they both lead to reduced fertility rate.
In 2011 Boukaidi et al. [14] reported 6 cases of isolated tubal torsion associated with hydrosalpinx. Salpingectomy were required in 5/6 patients. The histopathologic assessment of the resected tubes showed remaining ciliated cells in 50% of cases suggesting the possibility for tubal conservation.
Both our cases had predisposing factors to sactosalpinx: Case 1 had peritoneal adhesions occluding the fallopian right tube due to previous surgery (laparotomy for peritonitis); Case 2 had a history of vaginal stenosis and recurrent urinary infections .
We recommend a conservative approach for asymptomatic hydrosalpinx and for symptomatic sactosalphinx in general, except for septic cases or no responder patients (worsening symptoms despite adequate antibiotic treatment). If symptoms are controlled by analgesic/antibiotic therapy and adnexal torsion or other associated conditions (neoplasm) are excluded at surgery, we encourage a conservative treatment and a strict clinical and radiological follow-up: ultrasound scan every 6–12 months until adult age and periodical genital visit under anesthesia to exclude predisposing factor like vaginal stenosis for CAH surgically treated patients.
In our opinion, especially in cases with reduced fertility potential, it is important not to neglect predisposing conditions like:
-
1)
Vaginal stenosis, particularly when associated with recurrent urinary infections
-
2)
Previous pelvic surgery or previous laparotomy/laparoscopy for peritonitis for example due to complicated appendicitis or other abdominal diseases.
