Introduction
Adnexal lesions occur in infants, children, and adolescents with varying frequency and may represent ovarian or tubal pathology. Consideration of a patient’s age, pubertal status, clinical presentation, and physical examination serves to inform appropriate investigations and management. Through this chapter, we will outline an approach to adnexal masses in neonatal, pediatric, and adolescent patients and describe key diagnostic and management principles.
The fetus and neonate
Fetal ovarian cysts are often diagnosed incidentally during antenatal imaging. The vast majority of fetal cysts are functional and likely form secondary to stimulation from fetal follicle-stimulating hormone, maternal estrogen, and placental human chorionic gonadotropin (HCG). Diagnosis of an ovarian neoplasm in utero, such as a teratoma, cystadenoma, or malignancy, is uncommon. Approximately 30% to 70% of female fetuses will have a functional cyst identified on imaging. , , Incidence increases with increasing gestational age and some maternal complications of pregnancy, including hypertensive disorders of pregnancy, diabetes mellitus, and alloimmunization. Among live-born female neonates, the incidence of ovarian cysts is estimated to be 1 in 2500.
Diagnostic criteria for a fetal ovarian cyst include (1) female fetus, (2) nonmidline cystic structure, (3) normal-appearing urinary tract, and (4) normal-appearing gastrointestinal tract. The differential diagnosis for cystic lesions in the fetal abdomen is broad, with gastrointestinal and urinary anomalies considered ( Table 29.1 ). Most ovarian lesions are diagnosed at 28 weeks of gestational age or beyond. , Many antenatal cysts persist during the pregnancy with minimal change; some cases of complete resolution have been documented in utero. , , The most common complication of fetal ovarian cysts is antenatal adnexal torsion, which complicates 20% to 40% of cases.
| Gynecologic Origin |
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| Gastrointestinal Origin |
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| Urinary Origin |
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| Other |
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After delivery, there is rapid withdrawal of maternal estrogen and progesterone. As such, neonatal follicle-stimulating hormone levels rapidly increase, peak at 3 to 4 months of age, then fall to prepubertal levels by the second year of life. , After withdrawal of ovarian stimulation, the majority of functional cysts will resolve spontaneously. Predictors for cyst resolution include diameter less than 40 mm and simple characteristics on ultrasound imaging. In contrast, complex cysts measuring more than 40 mm in diameter are less likely to resolve spontaneously. Complex cystic characteristics on antenatal and postnatal imaging may predict loss of ipsilateral ovarian function secondary to antenatal torsion and autoamputation of the ovary.
Antenatal management
Management of fetal ovarian cysts remains an area of study. Serial ultrasounds are recommended to follow cyst size and characteristics. Evolution of a simple and anechoic cyst to a complex mass suggests intrauterine torsion with or without adnexal autoamputation. Risk factors for antenatal torsion include cyst diameter greater than 40 mm and complex appearance on ultrasound. Case reports have described rare antenatal complications secondary to large fetal ovarian cysts, including polyhydramnios likely secondary to cord compression, bowel obstruction, pulmonary hypoplasia, and hemorrhage.
Prenatal aspiration has been considered in an effort to reduce the risk of torsion and need for postnatal surgery. To date, studies have suggested prenatal aspiration increases rates of intrauterine resolution and reduces rates of postnatal oophorectomy; however, studies are limited by small sample sizes and low quality. Further study is required to inform evidence-based practice. The mode of delivery is based on obstetric indications.
Neonatal and infant management
Neonates with suspected ovarian cysts should undergo postnatal imaging to confirm the diagnosis. To date, there is significant variation in the management of neonatal cysts. Early surgical intervention was previously considered as a strategy to prevent loss of ovarian tissue secondary to ovarian torsion or hemorrhage. However, despite the goal of ovarian-sparing surgery, most surgical interventions resulted in oophorectomy. Older literature cited a concern about underlying malignancy as a rationale for early surgical management. However, epidemiologic studies have consistently demonstrated neonatal ovarian cysts to be functional with an exceedingly low rate of neoplasms; as such, concerns for malignancy should not inform management decisions. , , ,
Most neonates can be managed expectantly given high rates of resolution within the first year of life. Bascietto and colleagues describe spontaneous resolution of 70% of all simple cysts and 80% resolution of all cysts less than 40 mm in diameter. Even complex cysts suspicious for antenatal torsion will often resolve spontaneously within 6 to 18 months. , In the event of antenatal torsion with adnexal autoamputation, a wandering cystic lesion may be seen throughout the pelvis and abdomen; again, expectant management is considered, as several studies have documented resolution of these lesions over time. , Caregivers should be counseled about signs and symptoms of adnexal torsion, including pain or inconsolable crying, feeding intolerance, abdominal distention, and fevers.
Intervention with postnatal aspiration or surgical management is considered in the event of symptomatic or persistent, nonresolving lesions. Bryant and Laufer suggest the use of percutaneous aspiration for simple ovarian cysts greater than 60 mm to reduce the risk of ovarian torsion. Cyst recurrence was documented in up to 30% of patients. Predictors for nonresolution requiring surgical management include greater than 60 mm diameter at the time of diagnosis and complex characteristics. , , 
Whenever possible, ovarian cystectomy is preferred to salpingo-oophorectomy to preserve functional ovarian tissue.
Adnexal lesions in children and adolescents
Adnexal lesions can represent ovarian (neoplastic and nonneoplastic) and nonovarian lesions in children and adolescents. The incidence of each pathology varies based on age and pubertal status. As demonstrated by Hermans and colleagues, most ovarian lesions are benign in childhood and adolescence. A bimodal distribution is noted with a nadir in incidence in early childhood ( Fig. 29.1 ).
Next, we will review nonneoplastic ovarian lesions, neoplastic ovarian lesions, and nonovarian adnexal masses.
Nonneoplastic ovarian lesions
Functional ovarian cysts
Functional ovarian cysts typically develop secondary to stimulation from pituitary gonadotropins. Follicular cysts form when a dominant follicle fails to ovulate and involute, resulting in a thin-walled cystic structure. Similarly, fluid accumulation in a physiologic corpus luteum can result in a thicker-walled cystic structure within the ovary reaching up to 8 cm. Cyst walls become vascularized before regression; cysts can undergo hemorrhagic transformation with intracystic and/or intraabdominal hemorrhage.
Hemorrhagic cysts have been described as great imitators, as their clinical presentation with acute-onset pain may be confused for ovarian torsion. Imaging can also be difficult to interpret, with a broad differential diagnosis including ovarian torsion, neoplasm, or tubo-ovarian abscess. Careful review with radiology colleagues with consideration of the clinical context is important before planning intervention. In the event of cyst rupture, hemoperitoneum can develop with associated anemia and potential hemodynamic instability. Characteristic ultrasound findings of hemorrhagic cysts are highlighted in Fig. 29.2 .
As noted earlier, functional cysts account for nearly all antenatal and neonatal cysts secondary to stimulation from fetal/neonatal gonadotropins and maternal and placenta hormones. 
Functional cysts account for nearly 30% to 50% of ovarian lesions in adolescents with stimulation from pituitary gonadotropins. In contrast, functional ovarian lesions in childhood are uncommon, given low prepubertal gonadotropin levels. Functional cysts within childhood should prompt consideration of endocrinopathies such as precocious puberty, including McCune-Albright syndrome ( Table 29.2 ).
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Endometrioma
Endometriomas are benign ovarian masses of ectopic endometrium. Incidence within adolescents is not well defined but may occur in up to one-third of patients with surgically documented endometriosis. , In addition to an adnexal lesion on ultrasound characterized by a unilocular cyst with homogenous ground-glass echoes, patients will typically describe cyclic and acyclic pelvic pain ( Fig. 29.3 ). Moreover, patients often describe associated bladder and bowel symptoms such as dyschezia and dysuria and dyspareunia. ,
Ovarian neoplasms
Ovarian neoplasms—benign, borderline, and malignant—account for approximately 2% of all tumors in children and adolescents. Incidence is approximately 2.6 per 100,000 girls per year. Lesions can be classified based on cell line of origin, including germ cell tumor (GCT), epithelial tumor, or sex cord stromal tumor (SCST). Each tumor type is characterized by clinical presentations and imaging findings ( Table 29.3 ). Classic ultrasound findings for mature cystic teratomas are highlighted in Fig. 29.4 .
| Cell Line Origin | Clinical Features | Examples | Classic Imaging Characteristics |
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| Germ Cell Origin | |||
| Develop from primordial ovarian germ cells |
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| Epithelial Origin | |||
| Develop from epithelial cells of the ovary |
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| Sex Cord Stromal Tumors | |||
| Develop from the sex cord cells (Sertoli or granulosa cells) or stromal cells (fibroma, thecoma) |
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During childhood, GCTs account for more than 70% of ovarian neoplasms ( Fig. 29.5 ). In adolescents, germ cell and epithelial lesions occur at similar rates. Sex cord stromal lesions are most common in early childhood, accounting for approximately 20% of cases, and are rare in later childhood and adolescence.
Of all ovarian malignancies in children and adolescents, the majority are diagnosed in adolescents aged 15 to 19 years. Age-adjusted rates of ovarian malignancy in children ≤9 years old is 0.102 per 100,000 per year compared with 1.072 per 100,000 per year in children aged 10 to 19. Although the incidence of malignancy is 10-fold higher in adolescents, the proportion of malignant lesions is highest in childhood ( Fig. 29.6 ). This reflects low rates of functional, benign ovarian lesions in childhood, as the hypothalamic-pituitary-ovarian axis remains quiescent.
