CASE DESCRIPTION
A 10-year-old Chinese girl with increased volume of vaginal discharge
was hospitalized at the Department of pediatric gynecology in June 2020.
By the time of hospitalization, the patient’s medical history was 4
years. Initially, parents noticed the patient had vaginal secretions,
like milky, thin discharge, odor,and the amount varies. Vaginal
bleeding, pruritus, requent micturition, fever were not detected. With
suspected vulvovaginitis, hygiene measures were suggested.Vaginal
culture and testing for pathogen were administered with no positive
findings.Then empiric antimicrobial treatment (oral amoxicillin) was
given while symptoms were not relieved. Based on that, hysteroscopy was
administered while no special findings was discovered.
Due to persistent discharge, the patient was referred to our department.
At admission, vital signs as blood pressure, heart rate, respiration and
pulse were within normal range.The patient’s weight was 41 kg, height
was 143.5cm.Physical examination showed breast development staging
Tanner III and pubic hair staging Tanner I.No vulvar rashes, vulvar
pigmented/nonpigmented lesions,masses,lichen sclerosis or labial
adhesions were seen. Numerous white granular projections could be seen
in the hymen,and milky, thin discharge in the vaginal opening(Figure
1A).During admission,vaginal secretions were about 150ml per day.No
surgery/trauma history or estrogen exposure was reported by the patient.
Multiple diseases could lead to increased vaginal secretions in pubertal
females.The most common were vulvovaginitis and vaginal foreign
body.Furthermore,vaginal and vulvar tumors, vulvar skin
conditions(eg,Hemangiomas,LMs),systematic illness(eg,Crohn
disease),urinary tract abnormalities should be considered.
Based on that, further examinations were administered.Lab examinations
showed that blood routine test, urine routine test, liver and kidney
function, thyroid function were normal.Follicle stimulating hormone was
7.100 mIU/mL, and luteinizing hormone 5.980 mIU/mL, pituitary prolactin
35.870 ng/mL, estradiol 44.510 pg/mL, testosterone 0.150 ng/mL,
progesterone 0.637 ng/mL.Tumor markers such as alpha fetoprotein,
carcinoembryonic antigen and human chorionic gonadotropin were
negative.Pelvic ultrasonography indicated that uterine and ovarian
volumes increased to the thresholds for puberty.Hence, pelvic MRI were
administered,which indicated vascular malformations,as multiple lamellar
and corp-like abnormal signals can be seen around bilateral iliac
vessels in the pelvic cavity, uterus, vagina and rectum, bilateral
inguinal lymph nodes, and bilateral lateral iliac wings(Figure 2A).
With the consent of the guardian and the patient, ”direct vaginal and
pelvic lymphatic malformation angiography through lymph nodes and
lesions guided by ultrasound and DSA ” was performed under general
anesthesia. A large amount of light yellow clear liquid was
extracted.Then puncturing through the swollen lymph nodes in the groin
and the hyperplasia mucous membrane inside the vaginal opening, and
injecting contrast agent with high-tension injection.Pelvic and vaginal
lesions were completely revealed in an hour–microcystic lymphatic
malformation with partial abnormal lymphatic dilatation was
confirmed,which was consistent with preoperative MRI outcomes.Until
then, primary vaginal and pelvic LMs was clinically confirmed.
Sclerotherapy with Bleomycin was given during the operation.The vaginal
discharge was about 1ml 24 hours after the operation, and was
disappeared 3 days after.Sirolimus (1mg qd) was administered orally one
week after surgery to prevent the recurrence of some microcystic lesios,
which might not be achieved by sclerotherapy. In 1 month follow-up,the
granular protrusions in hymen disappeared, as well as the vaginal
discharge.In 8 month follow-up,no vaginal discharge was observed,while
sporadic granular projections could be seen in the hymen(Figure 1B).
Pelvic MRI was performed again which showed lesions were reduced than
before, and no vaginal involvement was observed(Figure 2B).
Sclerotherapy with Bleomycin was recommended, but was refused by the
parents,so we adjusted the the oral dose of Sirolimus to 1mg bid. In 14
month follow-up, there was no recurrence of clinical symptoms, and
pelvic MRI display was stable(Figure 2C).