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Ovarian cysts in infants, children, and adolescents
Author: Marc R Laufer, MD Section Editors: Charles J Lockwood, MD, MHCM, Amy B Middleman, MD, MPH, MS Ed Deputy Editor: Mary M Torchia, MD All topics are updated as new evidence becomes available and our peer review process is complete. Literature review current through: Sep 2022. | This topic last updated: Jun 01, 2022.
INTRODUCTION
Cystic ovarian masses occur in female infants, children, and adolescents. They may present with associated symptoms or signs (eg, abdominal pain or distension, palpable mass) or be identified through imaging studies. Cystic ovarian lesions may be due to enlargement of a cystic follicle (ie, physiologic cyst, also called a functional cyst) or benign or malignant ovarian tumors. The differential diagnosis varies with age. Although most cystic masses in children are physiologic ovarian cysts or benign tumors, early diagnosis is necessary to reduce the risk of ovarian torsion and to improve the prognosis for children with malignant neoplasms.
The epidemiology, clinical features, and management of ovarian cysts in infants, children, and adolescents will be discussed here. The approach to the evaluation and management of ovarian masses in children and adolescents is discussed separately. (See "Evaluation of ovarian masses in infants, children, and adolescents".)
OVARIAN CYSTS IN THE FETUS AND NEONATE
Epidemiology and pathogenesis — Follicular ovarian cysts are common in fetuses and neonates [ 1 ]. They increase in frequency with advancing gestational age and some maternal complications (eg, diabetes mellitus, preeclampsia, rhesus isoimmunization) [2,3]. Although the true incidence of fetal ovarian cysts is unknown, they have been detected in approximately 30 percent of female fetuses on necropsy [ 2 ] and 30 to 70 percent of fetuses on routine prenatal ultrasonography [3-6]. Among live-born female infants, incidence of clinically important ovarian
®
cysts is estimated to be 1 in 2500 [ 7 ]. There is no increased risk of recurrence in subsequent pregnancies [ 3 ].
Fetal and neonatal ovarian cysts most likely arise from ovarian stimulation by maternal and fetal gonadotropins, although the pathogenesis is uncertain [ 3 ].
Clinical features and diagnosis — The majority of fetal ovarian cysts are unilateral [ 6 ].
Differential diagnosis — The differential diagnosis of a fetal or neonatal cystic intra-abdominal mass includes [ 9 ]:
Fetal ovarian cysts – Fetal follicular cysts are detected incidentally on prenatal ultrasonography ( image 1) [ 4 ]. Diagnosis is established with four sonographic criteria: female sex, nonmidline regular cystic structure, normal-appearing urinary tract, and normal-appearing gastrointestinal tract [ 3 ]. Associated anomalies are rare [ 3 ].
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Size and appearance are used to characterize cysts as probably physiologic or probably pathologic (eg, ovarian torsion, intracystic hemorrhage) [1,4,8]:
- Probably physiologic – Simple (clear, fluid filled) cysts <2 cm in diameter
Probably pathologic – Complex (containing debris, septae, or solid components; echogenic wall) cysts or cysts that are ≥2 cm in diameter
•
Neonatal ovarian cysts – In neonates, physiologic ovarian cysts often present as asymptomatic pelvic or abdominal cystic masses; given the shallowness of the pelvis in the neonate, the cyst may be displaced to the mid- or upper abdomen [4-6]. The ovary containing the cyst can be mobile.
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Ultrasonography may demonstrate a simple or complex pattern. A simple pattern usually indicates a physiologic cyst. A complex pattern may indicate ovarian torsion or hemorrhage [ 1 ].
Genitourinary tract disorders (eg, reproductive tract anomalies, urinary tract obstruction, urachal cyst)
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Gastrointestinal tract disorders (eg, mesenteric or omental cyst, volvulus, colonic atresia, intestinal duplication, biliary cyst, pancreatic cyst)
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● Other disorders (eg, splenic cyst, lymphangioma)
and risk of complications [ 3 ].
Complex cysts are usually observed [ 19 ].
Simple cysts that are <4 cm in diameter are also usually observed; they are likely to resolve spontaneously [18,20].
We suggest observation for simple prenatal cysts that are <6 cm in diameter. After birth, the diagnosis is more certain, and although most cases are managed with continued observation, aspiration (if elected) has a low risk of complications [7,21].
Although prenatal ultrasound-guided aspiration of large simple cysts may reduce the risk of cyst-related complications ( table 1) and the rate of postnatal oophorectomy [13,19,22-26], prenatal aspiration is controversial because of the potential for misdiagnosis [ 11 ], spillage, and other procedure-related complications [ 27 ]. Prenatal aspiration is generally reserved for the rare cases in which there is concern for compromise to the fetus (eg, interference with vaginal delivery, adverse effects on lung function).
The vast majority of fetuses with ovarian cysts can be delivered vaginally, with cesarean delivery reserved for the usual obstetric indications [ 3 ]. Cesarean delivery may be preferred for fetuses with an extremely large or complex cyst (although this occurrence is very rare) to prevent rupture and/or abdominal dystocia. Prenatal cyst aspiration is an alternative approach.
● Postnatal management
Counseling – Parents and other caregivers of neonates with ovarian cysts should be counseled regarding the signs and symptoms of ovarian torsion so they can seek emergency care without delay. (See 'Ovarian torsion' below.)
•
Monitoring and management – Primary care providers can monitor ovarian cysts in neonates if they are comfortable doing so; if they are not, the cysts can be monitored by a pediatric and adolescent gynecologist or pediatric surgeon.
•
We suggest initial observation for ovarian cysts in the neonate. We perform ultrasonography at birth (if the cyst was detected prenatally) and every four to six weeks thereafter until the cyst resolves, enlarges, is associated with signs or symptoms of torsion, or has persisted for four months. Failure to resolve after four months may indicate malignancy (very rare), ovarian torsion with subsequent loss, or nonovarian
OVARIAN CYSTS IN INFANTS AND PREPUBERTAL CHILDREN
Epidemiology and pathogenesis — The incidence of ovarian cysts in infants and prepubertal children is lower than in neonates and perimenarchal/menarcheal girls because gonadotropin stimulation of the ovary decreases after the neonatal period and generally remains low until puberty [ 31 ].
Most simple ovarian cysts in infants and children are related to failure of an ovarian follicle to involute [4-6]. Some ovarian cysts in prepubertal girls are hormonally active and result in peripheral precocious puberty (also called pseudopuberty) [ 32 ]. (See "Definition, etiology, and evaluation of precocious puberty", section on 'Causes of peripheral precocity'.)
pathology. (See "Evaluation of ovarian masses in infants, children, and adolescents", section on 'Causes of ovarian mass'.)
For cysts that enlarge or persist for ≥4 months, continued observation, aspiration, or surgical removal (cystectomy) are all reasonable options.
Aspiration is an option for simple neonatal ovarian cysts that are ≥6 cm in diameter if they have not regressed spontaneously after four months [ 3 ]. Aspiration reduces the risk of ovarian torsion and has a low risk of complications, including recurrence [ 21 ]. Observation, reaspiration, or surgical removal can be undertaken if the cyst recurs.
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Surgical exploration, with every attempt to salvage the ovary, is another option for simple neonatal ovarian cysts that persist for more than four months.
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Surgery is generally indicated for neonatal ovarian cysts that are complex, symptomatic (particularly symptoms of ovarian/tubal torsion), or increasing in size [28-30]. Laparoscopic surgery is feasible and safe in neonates with ovarian cysts and is the recommended surgical approach.
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We do not provide long-term follow-up and monitoring for patients whose neonatal cysts resolve spontaneously, are aspirated, or undergo cystectomy because the vast majority are benign. For the rare patient whose pathology reveals a nonfunctional cyst, we perform follow-up ultrasonography and surveillance.
● Other causes of ovarian cysts in prepubertal girls include:
Additional evaluation depends upon the ultrasonographic appearance and associated clinical findings.
Differential diagnosis — Other cystic adnexal masses in prepubertal children include paraovarian, mesothelial, and broad ligament cysts. These can usually be differentiated from ovarian cyst with ultrasonography.
Management — The management of ovarian cysts in prepubertal children depends upon the ultrasonographic appearance of the cyst and associated clinical findings. Ovarian cysts in infants and prepubertal children are typically managed by a specialist in pediatric and adolescent gynecology or a pediatric surgeon.
Parents and other caregivers of infants and prepubertal children with ovarian cysts should be counseled regarding the signs and symptoms of ovarian torsion so they can seek emergency care without delay. (See 'Ovarian torsion' below.)
Children with acute severe abdominal pain or intermittent severe pain accompanied by nausea and/or vomiting that resolves spontaneously should be evaluated for ovarian torsion and other causes of acute abdominal pain. (See 'Ovarian torsion' below and "Emergency evaluation of the child with acute abdominal pain".)
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Children with recurrent, large, or multicystic ovarian masses and signs of early sexual development (eg, breast buds before age 8 years, growth spurt) should be evaluated for precocious puberty, including the possibility of primary hypothyroidism and ovarian stromal or germ cell tumors. (See "Definition, etiology, and evaluation of precocious puberty", section on 'Evaluation'.)
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Those with prepubertal vaginal bleeding and ovarian enlargement should be evaluated for features of McCune-Albright syndrome ( picture 1) to avoid unnecessary oophorectomy [ 39 ]. (See "Definition, etiology, and evaluation of precocious puberty", section on 'McCune- Albright syndrome'.)
Monitoring – For patients without symptoms, we suggest initial observation of cysts that appear purely cystic on ultrasonography or have findings suggestive of intracystic hemorrhage (eg, few internal echoes or debris and no other complex features such as septation, increased solid tissue, or calcification). These cysts are almost certainly benign. Parents and other caregivers should be counseled regarding the signs and symptoms of ovarian torsion so they can seek emergency care without delay. (See 'Ovarian torsion' below.)
●
OVARIAN CYSTS IN ADOLESCENTS
Epidemiology and pathogenesis — Simple and complex ovarian cysts are common in postmenarchal adolescents. Adolescent ovaries may contain multiple follicles in different stages of development ( figure 1). Most ovarian cysts in adolescents are follicular cysts, which result from failure of the maturing follicle to ovulate and involute; a normal mature follicle can be 2 to 3 cm in diameter [ 5 ]. Corpus luteum cysts result from fluid accumulation in the corpus luteum, which forms after ovulation; corpus luteum cysts can reach 5 to 12 cm in diameter if there is bleeding into the corpus luteum.
Clinical features — Ovarian cysts in the postmenarchal adolescent may be asymptomatic (and found incidentally) or associated with menstrual irregularities or pelvic pain [ 5 ]. Large cysts may cause urinary frequency, constipation, or feelings of pressure in the lower abdomen (also described as pelvic heaviness).
Acute severe pain mimicking appendicitis or peritonitis may result from torsion, rupture, or hemorrhage (intracystic or intra-abdominal). (See 'Complications of ovarian cysts' below.)
Evaluation and diagnosis
If the cyst has not resolved and ultrasonography remains reassuring (eg, no increase in size, septation, solid tissue, or calcification), continued observation is appropriate, provided there are no associated symptoms (of torsion, rupture, precocious puberty, or malignancy). In observational studies, most simple and complex ovarian cysts <9 cm in diameter in prepubertal children and adolescents resolve spontaneously [40,41]. Those that do not resolve spontaneously are rarely, if ever, malignant [42,43].
● Indications for surgical exploration – Indications for surgical exploration include:
- Ovarian cysts ≥9 cm in diameter, which are at increased risk of being malignant [42,43]
Ultrasonographic features concerning for benign or malignant tumor (eg, septation, increased solid tissue, calcification)
•
- Ovarian torsion (to salvage the ovary) [ 44 ] (see 'Ovarian torsion' below)
Acute rupture with hemorrhage and hemodynamic instability (after hemodynamic status is stabilized) (see 'Ruptured and/or hemorrhagic cyst' below)
•
Management — The management of ovarian cysts in adolescents depends upon the ultrasonographic appearance of the cyst and associated clinical findings. Ovarian cysts in adolescents are typically managed by a pediatric and adolescent gynecologist, adult gynecologist, or pediatric surgeon.
Adolescent patients with ovarian cysts and their parents/caregivers should be counseled regarding the signs and symptoms of ovarian torsion so they can seek emergency care without delay. (See 'Ovarian torsion' below.)
history")
Gastrointestinal conditions (eg, appendiceal abscess) (see "Acute appendicitis in children: Clinical manifestations and diagnosis")
●
Follicular cysts — Most follicular cysts found on routine examination in adolescents resolve spontaneously in two to eight weeks. Asymptomatic simple cysts <6 cm in diameter on ultrasonography can be observed. We prescribe monophasic combination estrogen/progestin oral contraceptive pills (OCPs) with ≥35 mcg ethinyl estradiol to adolescent patients after discussing the benefits and risks with the patient and caregivers and obtaining informed consent. (See "Contraception: Issues specific to adolescents", section on 'Initiation and increasing adherence'.)
●
Although OCPs do not decrease the size of the existing cyst [ 45 ], they suppress the ovarian-hypothalamic axis and may prevent ovulation and the formation of a new functional cyst [46,47], which can make it challenging to know whether the first cyst has resolved. When used for this purpose, OCPs with ≥35 mcg of ethinyl estradiol appear to be more effective than formulations with <20 mcg ethinyl estradiol [48-50].
The patient should be re-evaluated usually within two to four weeks with bimanual examination (if possible, or other evaluation appropriate for the age of the patient) or ultrasonography.
Indications for laparoscopic cystectomy or aspiration (laparoscopic or ultrasound-guided) include one or more of the following:
- Persistence for ≥3 months
Increase in size is a relative indication for cystectomy or aspiration – We discuss the pros and cons of continued observation and surgical intervention and use a shared- decision making process
•
Size ≥6 cm in diameter is a relative indication for cystectomy or aspiration; asymptomatic simple cysts between 6 and 12 cm may resolve spontaneously and can be safely observed in some patients [4-6]. We use a shared decision-making process to determine the management approach for cysts ≥6 cm in diameter, considering associated symptoms in the benefit-risk analysis.
If the cyst recurs after cystectomy or aspiration or if operative intervention is needed, the procedure should be conservative and preserve as much ovarian tissue as possible.
- Symptoms (eg, pelvic pain, urinary frequency)
When cystectomy or aspiration is undertaken, we prefer laparoscopic cystectomy to laparoscopic or ultrasound-guided aspiration because of the high rate of recurrence with aspiration [ 51 ]. Ultrasound-guided aspiration is generally reserved for patients with contraindications to surgery or preference for a nonsurgical approach. Ovarian surgery may be complicated by ovarian and peritubal adhesions, which may be associated with infertility and/or pelvic pain.
If cystectomy is performed, the cyst wall should be removed completely and sent for pathologic examination.
Small follicular cysts found incidentally at the time of surgery will resolve spontaneously and should not be aspirated or excised.
If cyst aspiration is performed (laparoscopic or ultrasound-guided), cyst fluid should be sent for cytology.
Corpus luteum cysts — For adolescent patients with corpus luteum cysts who are without pain or intraperitoneal bleeding, we suggest observation for three months. We prescribe monophasic combination estrogen/progestin OCPs with ≥35 mcg ethinyl estradiol to adolescent patients after discussing the benefits and risks with the patient and caregivers and obtaining informed consent. (See "Contraception: Issues specific to adolescents", section on 'Initiation and increasing adherence'.)
●
Most asymptomatic corpus luteal cysts involute during the observation period. Although OCPs do not decrease the size of the existing cyst [ 45 ], they suppress the ovarian- hypothalamic axis and may prevent the formation of new functional cysts [46,47], which can make it challenging to know whether the first cyst has resolved. When used for this purpose, OCPs with ≥35 mcg of ethinyl estradiol appear to be more effective than formulations with <35 mcg ethinyl estradiol [48-50].
Isolated tubal torsion can be more difficult to identify as the ovary may appear normal on imaging.
Ruptured and/or hemorrhagic cyst — Rupture of ovarian cyst is usually accompanied by acute onset of focal unilateral lower abdominal pain, classically in the midmenstrual cycle, often immediately following sexual intercourse. Rupture may be accompanied by intraperitoneal hemorrhage.
Complete blood count with differential may demonstrate leukocytosis with a left shift. (See "Ovarian and fallopian tube torsion", section on 'Clinical presentation'.)
Evaluation – Evaluation for ovarian torsion includes ultrasonography to evaluate findings consistent with ovarian torsion and to exclude other causes of acute severe abdominal pain (eg, appendicitis, tubo-ovarian abscess, ectopic pregnancy). Ultrasonography demonstrating a size discrepancy in ovarian volumes with classic peripheralization of follicles supports the diagnosis [ 57 ].
●
The addition of color flow Doppler ultrasonography may be helpful, although it is not always conclusive. Doppler flow can be appreciated in a torsed ovary without complete cessation of blood flow because the ovary has a dual blood supply. Magnetic resonance imaging is not routinely performed in the evaluation for ovarian torsion but may be helpful if sonographic findings are equivocal. (See "Ovarian and fallopian tube torsion", section on 'Imaging studies'.)
Management – Ovarian and/or tubal torsion is a surgical emergency. Surgery is always indicated at the time of diagnosis of adnexal torsion because a torsed ovary and/or tube can usually be salvaged by untwisting the vascular pedicle [ 44 ]. Untwisting can be accomplished laparoscopically [58-61]. Almost all torsed ovaries and tubes can be salvaged, even those that have a dark coloration ( picture 2). In rare instances, oophorectomy is necessary because of severe extensive necrosis, or nonviable gelatinous appearance [ 62 ]. Surgical techniques are discussed separately. (See "Ovarian and fallopian tube torsion", section on 'Management'.)
●
It is unclear whether oophoropexy of the contralateral ovary should be performed to prevent torsion of the normal ovary. Oophoropexy can also be safely performed laparoscopically [ 63 ]. (See "Oophorectomy and ovarian cystectomy".)
Outcome – Ovarian masses associated with torsion are usually benign [53,64]. In a national cohort of 1232 females age 1 to 20 years hospitalized for ovarian torsion, only 0. percent had a malignant neoplasm [ 53 ].
●
An ovarian cyst also may become hemorrhagic without rupture. Hemorrhagic cysts may be asymptomatic or may cause pelvic pain. The bleeding may be self-limiting or associated with hemodynamic instability (eg, altered vital signs).
Patients with self-limiting bleeding are observed with serial examination and hemoglobin/hematocrit [4-6]. Patients who are hemodynamically unstable should be stabilized before surgery, which often can be performed laparoscopically [58,65]. Free blood and clots are aspirated and hemostasis assured by fulguration of the areas of bleeding. Hemoperitoneum alone is not an indication for open laparotomy. Laparotomy is indicated if the surgeon is not experienced in laparoscopic procedures on children or if the patient is hypotensive and cannot be promptly stabilized.
SUMMARY AND RECOMMENDATIONS
● Ovarian cysts in the fetus and neonate
Clinical features – Follicular ovarian cysts are a common incidental finding on prenatal ultrasonography. In the neonate, follicular ovarian cysts often present as an asymptomatic freely mobile pelvic or abdominal mass and are confirmed with ultrasonography. Most fetal and neonatal ovarian cysts regress spontaneously; complications are rare ( table 1). (See 'Clinical features and diagnosis' above and 'Complications' above and 'Natural history' above.)
•
Management – We suggest initial observation rather than aspiration for simple ovarian cysts in the fetus and neonate (Grade 2C). For cysts that enlarge or persist for ≥ 4 months after birth, continued observation, aspiration, and cystectomy are reasonable options. Failure to resolve after four months may indicate malignancy (very rare), ovarian torsion with subsequent loss, or nonovarian pathology. (See 'Management' above.)
•
Counseling – Caregivers of neonates with ovarian cysts should be counseled regarding the signs and symptoms of ovarian torsion so they can seek emergency care without delay. (See 'Ovarian torsion' above.)
•
● Ovarian cysts in infants and prepubertal children
Clinical features – In infants and prepubertal children, ovarian cysts often present as an asymptomatic abdominal mass, increasing abdominal girth, or an incidental imaging finding. When present, symptoms include abdominal pain, bloating, and urinary
•
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For adolescent patients with corpus luteum cysts who are without pain or intraperitoneal bleeding, we suggest initial observation (Grade 2C). Most asymptomatic corpus luteum cysts involute within three months.
-
For adolescent patients with either follicular or corpus luteum cysts, after discussing the benefits and risks with the patient and caregivers and obtaining informed consent, we prescribe monophasic combination estrogen/progestin oral contraceptive pills with ≥35 mcg of ethinyl estradiol to potentially prevent the formation of new functional cysts, which can make it difficult to know whether the first cyst has resolved.
-
Complications of ovarian cysts – Ovarian torsion, rupture, and hemorrhage are important complications of ovarian cysts. These complications may present with acute severe unilateral lower abdominal pain and require urgent evaluation. (See 'Complications of ovarian cysts' above and "Ovarian and fallopian tube torsion".)
●
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