Written by Patricia Huguelet, MD
Ovarian cysts occur frequently in children and adolescent girls. Healthcare providers may discover these when patients have symptoms, during routine physical examination or incidentally through imaging studies. Historically, providers surgically removed ovarian cysts and masses when they discovered them in children and adolescents. Surgery often involved removal of the entire ovary.
During the last decade, however, the management of ovarian masses has shifted toward a more conservative approach with ovarian preservation. The reasons for this are likely multifactorial, including advances in radiologic imaging, the identification of tumor markers, and an increase in the availability and accessibility of pediatric and adolescent gynecologists within pediatric healthcare systems.
Benign ovarian masses
Benign ovarian masses are classified as either non-neoplastic or neoplastic. The majority of non-neoplastic cysts in this population are physiologic and can further be classified as simple or follicular, corpus luteum, hemorrhagic or paratubal cysts. Providers may diagnose these cysts as a result of acute pain, or they may discover them incidentally during routine physical examination or imaging. At the time of diagnosis, surgical exploration is required if there is high concern for torsion. However, if providers do not suspect torsion, the majority of non-neoplastic cysts can be managed expectantly with serial ultrasound imaging. Reimaging after 8 to 12 weeks often reveals resolution of the cyst. Some adolescents will then elect to start hormonal suppressive therapy to prevent future cysts, but this decision should be individualized and discussed with the treating gynecologist. Hormonal suppression does not cause regression of existing ovarian cysts.
The decision to proceed with surgery for cysts that do not resolve spontaneously is generally based on the patient's symptoms, physical examination and imaging findings. Although providers discuss size thresholds, the literature does not support single-size threshold as an indicator for mandatory surgical exploration.
Ovarian neoplasms are much less common than non-neoplastic cysts, accounting for approximately 1% of all tumors in children and teens. Most ovarian neoplasms are benign; fewer than 10% are malignant. In girls and adolescents, the majority of these benign ovarian neoplasms are mature cystic teratomas (dermoid cyst), serous and mucinous cystadenomas. The majority of malignant neoplasms in children and teens are germ cell in origin, compared with epithelial cell tumors, which account for most malignant neoplasms in adults.
Ultrasound is the imaging study of choice to distinguish between these masses, with assessment for cystic and solid features, as well as Doppler flow to look for increased vascularity, which frequently occurs with malignancy. Tumor markers for germ cell tumors (LDH, AFP, HCG and inhibin) are useful when imaging studies suggest malignancy. Surgery is always recommended in the setting of an ovarian neoplasm as it will not spontaneously regress. However, surgical intervention is still directed towards preservation of the ovary, with unilateral salpingo-oophorectomy reserved only for masses with a high suspicion for malignancy.
It is important to note that torsion can occur with a cyst of any size, particularly when long utero-ovarian pedicles are present. The embryologic ovary originates at the level of the 10th thoracic vertebrae and descends to the true pelvis by puberty. Prior to menarche, the ovary is an abdominal organ and therefore, the normal ovary is more susceptible to torsion on the naturally elongated utero-ovarian pedicle. After menarche, most cases of torsion occur in the setting of an ovarian cyst or mass that causes the enlarged ovary to twist on its smaller, vascular pedicle.
Signs and symptoms of torsion include the sudden onset of lower abdominal pain, nausea, vomiting and low-grade fever. Ultrasound evaluation most consistent with ovarian torsion includes size discrepancy in ovarian volumes, peripheralization of follicles and centralized edema. Providers can utilize Doppler flow to evaluate for blood flow to the ovary. However, providers should interpret it with extreme caution as 30% of cases of acute ovarian torsion in the adolescent patient will still demonstrate normal Doppler blood flow to the ovary.
Management of ovarian torsion
Providers should always make an attempt to salvage the torsed ovary by untwisting the vascular pedicle, thereby allowing reperfusion of the ovary. If a cyst is present, providers should remove it to prevent recurrence. Ovarian-sparing surgery is always preferred given the long-term risks of unilateral oophorectomy. The potential negative effects of oophorectomy include an increased risk for earlier menopause, premature ovarian failure, diminished ovarian reserve and fertility, and long-term adverse effects on bone health, sexual functioning and cardiovascular health.
Regardless of the necrotic appearance of the ovary at the time of surgery, detorsion with surveillance is the recommended management as most ovaries will show return of follicular activity. After detorsion, providers can perform reimaging of the ovary several weeks later to assess for return of ovarian function.
It's also important to recognize that sometimes the fallopian tube alone can be twisted on its pedicle. If providers do not recognize this in a timely manner, it can also adversely impact an adolescent's future fertility. In a case series published by our adolescent gynecologists at Children's Hospital Colorado, >90% of teenage girls with isolated tubal torsion had documented normal blood flow to the ovary, making the diagnosis particularly challenging. In this series, the triad of a simple paratubal cyst, normal blood flow and pain out of proportion to exam was highly predictive of isolated tubal torsion and something that we look for routinely to better assess and surgically manage our patients.
For consultation, referral of diagnostic dilemmas, contact our Pediatric and Adolescent Gynecology team through OneCall at 720-777-3999.