The majority of antenatal ovarian cysts are unilateral, although both ovaries may be involved (16). Spontaneous regression occurs in both simple and complex fetal cysts both antenatally and postpartum. The first-line management of the patient with antenatally diagnosed ovarian cysts is observation, although the size and composition of the cyst must be considered as the treatment plan is formulated (9,10,11,12,18,19,20). The risks to the fetus of an ovarian cyst may include intracystic hemorrhage, rupture of the cyst with possible hemorrhage, gastrointestinal and urinary tract obstruction, ovarian torsion and necrosis, incarceration into a congenital inguinal hernia, difficulty with delivery due to abdominal dystocia, and respiratory distress at birth due to the mass effect (21,22). Antenatal aspiration of large (>4 cm) ovarian cysts has been advocated to reduce the potential complications (20,23,24,25); however, the role of antenatal cyst aspiration is controversial due to potential misdiagnosis and complications of the aspiration procedure (26). In some fetuses with large cysts (>6 cm) or masses that are not cystic, elective cesarean section may be the preferred route to prevent rupture and/or dystocia. The rate of malignancy is so low that it need not be considered in making therapeutic decisions. Long-term follow-up of children with ovarian cysts diagnosed prenatally has shown the importance of close monitoring of complex cysts in order to alert the clinician to the need for early intervention to avoid impending ovarian loss due to torsion (27).

Most neonatal cysts are asymptomatic and identified incidentally on antenatal neonatal ultrasound evaluation for another indication. As neonates have a shallow pelvis, the cysts are often displaced to the mid- or upper abdomen, at which time they are palpable abdominally. On abdominal examination, the ovary containing the cyst is generally freely mobile. Ultrasound reveals a cystic mass that may have a simple (Fig. 21-6) (clear, fluid-filled) or complex (Fig 21-7) (fluid, debris, septa, solid components, echogenic wall) sonographic pattern. The complex appearance on ultrasound may make a precise diagnosis more difficult (15).

Spontaneous regression occurs in these cysts postpartum usually by 3 to 4 months of age, but can take up to a year or longer to regress (30). They should be followed with serial ultrasound examinations to ensure regression. The greatest concern in the neonate with an ovarian cyst is the possibility of torsion with subsequent ovarian loss. If the cyst does not resolve, then either neoplasia is present (unlikely) or torsion with hemorrhage and/or necrosis (see Figs. 13-6 and 13-7) has occurred (11,31).

The traditional management of neonatal cysts that persist for longer than 4 months and are >5 cm or increasing in size is surgery (12,32,33,34,35). Every attempt should be made to salvage the ovary in cases of persistent ovarian cysts; in rare instances, such as cases of torsion with ovarian necrosis, oophorectomy is
necessary (14,25). Figures 21-8 to 21-10 demonstrate the preservation of some normal-appearing ovarian tissue in a case of neonatal ovarian torsion. Based on the rarity of malignancy, every effort should be made to preserve any normal ovarian tissue with an ovarian cystectomy as opposed to an oophorectomy.

It should be noted that infants and children may also have torsion of normal ovaries (see Chapter 13) (36,37,38). It is possible to untwist the vascular pedicle in an attempt to salvage the ovary (39) (see Ovarian and Tubal Torsion below). Neonatal ovarian cysts can be managed preferentially by a laparoscopic approach, but may require a mini-laparotomy (40,41,42,43,44).

Postpartum cyst aspiration has been advocated to reduce the likelihood of torsion (45). In contrast to antenatal cyst aspiration, the use of neonatal cyst aspiration has been clearly established, because the diagnosis is more certain and the risk of complications is low (46,47). Aspiration of persistent large simple cysts (>5 to 6 cm) is recommended (12). If the cysts recur, then the options include reaspiration or surgical removal.

In young children, an ovarian cyst is often discovered by a parent or clinician as an asymptomatic abdominal mass or as increasing abdominal girth. The embryologic ovary migrates from the level of 10th thoracic vertebra (in early life it is characteristically abdominal in location) and during maturation descends to the true pelvis by puberty. Thus, ovarian tumors may present as abdominal masses in the young child (3). Chronic abdominal aching pain, either periumbilical or located in one lower quadrant, may be present. Acute severe pain simulating appendicitis or peritonitis may develop secondary to torsion, perforation, infarction, or hemorrhage from or into a tumor or cyst (see Chapter 13). Patients may experience intermittent pain, presumably because of torsion without complete compromise of the vascular blood supply, that subsequently resolves without therapy. This may also be the warning sign of impending torsion with complete compromise of the blood supply and the need for emergency surgery (see Ovarian and Tubal Torsion below). Nonspecific symptoms including nausea, vomiting, a sense of abdominal fullness or bloating, and urinary frequency or retention may signal the presence of a tumor.

Ultrasound is the best method for evaluation of ovarian cysts in prepubertal girls. The mass should be measured, and its characteristics determined (simple, complex, or solid). As discussed in Chapters 13 and 23, Doppler flow ultrasound may or may not be helpful in determining cases of ovarian torsion (see discussion of torsion below); ultrasound determination of a discrepancy in ovarian volume can be helpful in determining ovarian torsion (57).

The management of an ovarian cyst in the prepubertal age group depends on the appearance of the cyst on ultrasound and the presence of significant symptomatology. Most likely these cysts will resolve spontaneously and require no intervention.

Rarely a simple ovarian cyst in a prepubertal child will rupture and may result in acute exacerbation of pain. This will most likely be self-limited and requires no surgical intervention.

If the prepubertal ovarian mass is purely cystic or has few internal echoes/debris suggestive of hemorrhage and no other complex features such as septation or calcification (Fig. 21-7), it is almost certainly benign and can be managed by observation (53,58). A follow-up ultrasound scan in 4 to 8 weeks should reveal a decrease in size. If the cyst has not resolved and the ultrasonic characteristics are still reassuring, then continued observation is still appropriate. Warner and associates (59) reported on the conservative management of 51 children with both simple and complex cysts, all >5 cm in diameter. Of these cysts, 90% spontaneously resolved, largely within 2 weeks. Of the 23 children treated surgically either because of initial sonographic appearance or for persistence, 10 were found to have neoplasms (6 teratomas, 2 cystadenomas, 1 granulosa cell tumor, 1 Sertoli-Leydig cell tumor) (59). Thind and colleagues (60) found that in 64 children with simple cysts <5.5 cm in diameter, all of the cysts resolved. These girls, however, tended to have persistent symptoms for a few months followed by spontaneous resolution. The cysts sometimes recurred, although new ovarian cysts also occurred on the contralateral side (60).

If a prepubertal simple ovarian cyst persists, is symptomatic, or is growing in size, then a surgical intervention is indicated. Laparoscopy can be safely performed in young girls and an ovarian cystectomy should be performed with preservation of normal ovarian cortex as opposed to an oophorectomy (see Figs. 21-11 to 21-14). Persistent simple cysts are most likely a mucinous or serous ovarian cyst. Although rare, these cysts can recur (56).

Ovarian masses in prepubertal girls associated with torsion are usually benign (61). In a series of 102 girls (aged 2 days to 20 years) who underwent 106 consecutive ovarian operations, the authors reported that 42% of those who presented with abdominal pain had ovarian torsion. In their series, the ovarian mass was malignant in only one patient. In comparison, 26% of those presenting with an asymptomatic mass were found to have an ovarian malignancy. The management of adnexal torsion involves detorsion of the torsed ovary and preservation of
the ovary unless it is necrotic and nonviable. Ovarian torsion management is more fully discussed below.

Functional cysts (20% to 50% of ovarian tumors) are not true neoplasms but rather should be considered a variation of a normal physiologic process (see Chapter 6). Functional cysts include follicular, corpus luteum, and theca-lutein types, all of which are benign and usually self-limited. The incidence of cysts and nonmalignant tumors in the community is probably even higher than indicated in most series, as percentages are based on referred cases and it is not possible to determine the underlying incidence of nonidentified or asymptomatic cysts or masses (8,62).

The development of simple cysts is quite common in postpubertal girls and adolescents. Most simple cysts result from the failure of the maturing follicle to ovulate and involute. Adolescent ovaries may contain multiple follicles in different stages of development (see Fig. 13-18).

Cysts in the postmenarchal adolescent may be asymptomatic or cause menstrual irregularities, pelvic pain, or, if large, urinary frequency, constipation, or pelvic heaviness. Cysts can rupture, causing intra-abdominal hemorrhage (see Fig. 13-12 and Management of Cysts section, discussed next). Torsion of a cyst causes acute pain, nausea, vomiting, and pallor, often followed by less severe localized pain (63). The white blood cell count may be elevated with a shift to the left, but this is usually a late sign of ischemic or necrotic changes. Examination reveals pain, peritoneal signs, and a tender mass.

As mentioned above, the embryologic ovary migrates from the level of 10th thoracic vertebra (in early life it is characteristically abdominal in location) and during maturation descends to the true pelvis by puberty (see Fig. 13-15). Based on the abdominal location of the ovary, and the long utero-ovarian ligament, the normal adnexa of prepubertal girls are at increased risk of torsion. Torsion can occur with a cyst of any size, just as it does with normal adnexa, particularly when long pedicles are present (46,68). When known cysts are followed conservatively, parents or other caregivers should be made aware of the signs and symptoms of torsion (sudden onset of pain, nausea, vomiting, and possible low-grade fever) (see Chapter 13) and urged to seek emergent care without delay.

As noted in Chapter 13, ultrasound may be helpful in determining the presence of ovarian torsion (57,69,70). The classic appearance of peripheral follicles (“string of pearls”) with stromal edema may be helpful in making a diagnosis of torsion (see Fig. 13-13) and a volume discrepancy is also helpful in making the diagnosis (57).

Adnexal torsion should be addressed as a surgical emergency (71,72,73). Patients usually experience sudden onset of pain, nausea, vomiting, and possible low-grade fevers and are found to have peritoneal signs with rebound and/or guarding and possibly a mild leukocytosis (63,74,75,76). Patients may also experience intermittent pain, presumably because of partial or incomplete torsion (without complete compromise of the vascular blood supply), that subsequently resolves without therapy or does not cause complete ischemia. This may also be the warning sign of impending complete torsion with ischemia and death of the ovary, and the need for emergency surgery. If there is a concern for torsion, then a surgical procedure should be carried out for diagnosis and appropriate management.

In cases of ovarian torsion, surgery is indicated at the time of the diagnosis. Ovarian masses in girls and young women (ages 2 days to 20 years) associated with torsion are usually benign (61). In an urgent situation a platelet (PLT) count is readily available and elevated PLT counts are associated with malignancy (77). Torsed ovaries should be “detorsed” laparoscopically with conservation of the ovary (39,71,73,78,79,80,81). The historical concerns regarding blood clots and emboli from detorsion appear to be unfounded. The anatomy should be identified, the contralateral tube and ovary should be visualized and confirmed to be present and normal (asynchronous ovarian torsion has been reported), and the affected tube/ovary should be detorsed and salvaged (82). Even in cases of a “black” tube and/or ovary, detorsion and preservation of the adnexa can be carried out (see Fig. 13-14A–D) (81,82,83). If the ovary appears “black,” then a “bivalve” procedure may help facilitate increased blood flow to the ovary by decreasing the arterial blood pulse pressure and improving blood flow (83). Detorsion and oophoropexy (see below) may be all that is indicated, but if an ovarian cyst is present, then an ovarian cystectomy is performed and not an oophorectomy. Once again, preservation of normal ovarian tissue is the rule. It is our feeling that it is better to leave an ovary in place, even if it looks “dusky” after detorsion and bivalve procedures, as it will most likely maintain function. It is always possible to return to the operating room at a later date to remove an ovary that did not survive (patient has persistence of pain, fevers, increased white blood cell (WBC) count); it is not possible to replace a potentially viable ovary once an oophorectomy has been performed.

Ovarian torsion may occur without diagnosis and/or intervention (84). In this case, the ovary will most likely resorb and/or calcify. It is also possible for the ovary to “autoamputate” and become a sessile complex mass within the abdominal cavity (see Fig. 13-7A,B).

Several weeks to years after adnexal torsion, these same individuals may experience torsion of the other adnexa, and if the second torsed ovary is not salvageable, the individual is sterile (37,38,82). Based on this occurrence of bilateral asynchronous ovarian torsion, oophoropexy should be considered in all cases of ovarian torsion of a normal (ovary without a large cyst that caused the torsion) ovary.

Prophylactic oophoropexy (of the contralateral normal ovary) should be considered at the time of the surgery for detorsion of a torsed adnexa. Whether oophoropexy of the contralateral ovary can be helpful in preventing a second episode of ovarian torsion of a normal ovary needs additional evaluation, but there is no downside to performing the procedure. We offer an oophoropexy to all girls and young women who have or have had torsion of a normal ovary. If the torsion was associated and most likely due to an ovarian cyst, then an ovarian cystectomy can be performed after the detorsion and oophoropexy is not routinely offered.

If oophoropexy is elected, it can be safely accomplished by operative laparoscopy (85,86). There are varying techniques to accomplish the stabilization of the ovary. The utero-ovarian ligament can be sewn to itself to shorten the ligament, as shown in Fig. 21-16A–D, or the ovary can be sutured to the uterosacral ligament in cases where the ovary is enlarged and shortening of the utero-ovarian ligament is felt not to decrease the risk of asynchronous torsion, as shown in Fig. 21-17A–D. We perform this procedure laparoscopically and utilize a permanent nonreactive suture such as 2-0 silk. In cases of asynchronous bilateral ovarian torsion patients may elect to have bilateral oophoropexies to decrease the risk of continued episodes of torsion (Fig. 21-18A–D).