Any age
Antenatal and newborns (cf Chap. 5)
Postmenarchal period
• Adnexal torsion
• Infection of the pelvis
• Hydrocolpos
• Hematocoplpos
• Torsion or hemorrhage of an ovarian cyst
• Extrauterine pregnancy
• Hematocolpos
• Endometriosis
• Hemorrhagic and rupture of functionnal cyst
US is the imaging modality of choice and is often sufficient for an accurate diagnosis when coupled with the clinical and biologicals data. Before starting the examinations, the radiologist should rapidly discuss with the young patient and her parents; it should be rapidly clarified whether the parents will remain in the room during the examination. Indeed, some intimate questions could be asked, particularly if a vaginal way is possible and necessary. The radiologist performing the US examination should be reassuring as much as possible especially when examining adolescent girls. He/She should explain the way the examination will be performed.
The transabdominal way is the only possibility in the young virgin girls. Transabdominal US has to be performed with a full bladder (1/2 L of any liquid 1 h before the examination) in order to obtain an acoustic window that would facilitate the visualization of the adnexa. The use of perfusion or diuretic medications may be discussed to accelerate the process, allowing keeping the stomach empty in case that surgery is an option. Vaginal US if performed, has to be decided in agreement with the patient and only if the abdominal way is insufficient to clarify the diagnosis. MR imaging can be a good alternative examination in such case.
24.2 Adnexal Torsion (AT)
24.2.1 Epidemiology—Physiopathology—Clinical Data
Pediatric isolated ovarian torsion or entire adnexal torsion accounts for approximately 15% of all cases of ovarian torsion. In children, up to 52% of cases of adnexal torsion (AT) occur between 9 and 14 years, with a median age of 11 years. Noteworthy, there are two peaks: one during the first year of life and the second around 12 years. Pediatric AT represents about 3% of patients with acute abdominal pain referred to an emergency department [1, 2].
Whenever an AT progresses unrecognized, the ovarian blood supply becomes compromised, leading to tissue necrosis and potentially to a reduction of the reproductive capabilities of the patient. Therefore, timely diagnosis is crucial. Because the clinical presentation of AT may be nonspecific, preoperative diagnosis will potentially challenging.
AT is caused by partial or complete twisting of the ovary around its pedicle. True isolated ovarian torsion corresponds to a twisting of the ovary alone around the mesovarium. This form of torsion is rare. Global AT, including all adnexal components—ovary, fallopian tube and vascular pedicle – around the suspensory ligaments and the broad ligament is by far more common (Fig. 24.1). Rarely, an isolated torsion of the fallopian tube is also possible.
Fig. 24.1
Diagram defining the axis of torsion in true isolated ovarian (small arrow) and adnexal torsion (large arrow)
Torsion of the ovarian pedicle will first affect the lymphatic flow and only thereafter the venous flow, with subsequent stasis and thrombosis. This will result in progressive ovarian edema and enlargement. An interval of more than 10 h between initial symptoms and surgery is associated with high percentage of adnexal necrosis; still the actual duration of ischemia beyond which the damage is irreversible remains unknown [3].
AT occurs either in normal ovaries or in ovaries with an associated ovarian or para-ovarian mass. The mechanism of torsion in case of an associated ovarian mass is probably related to the increased size and weight of the ovary that becomes pivotal to the torsion. The etiology of a torsion of an apparently normal ovary is less obvious. Possible explanations include excess meso-ovarium mobility, congenitally long pelvic ligaments, tubal spasm, or acute modifications in the intraabdominal pressure. For these reasons, the twist on healthy ovaries and their recurrence is more common in children than in adults as confirmed by Ashwal et al. who reported a higher rate of recurrent torsion in pre-menarchal patients [3, 8].
Neonatal (in girls younger than 1 month) AT is classical but rare, and its incidence is unknown. All aged considered, approximately 16% of pediatric cases occur in girls younger than 1 year. There are two potential mechanisms for adnexal torsion in neonates and infants. The prenatal migration of the ovaries from the abdomen to the pelvis indicates an increase in gonadal mobility. Alternatively, maternal-hormone-induced stimulation with resultant ovarian enlargement and cyst development could serve as a fulcrum for the torsion [8].
Pre-menarchal and post-menarchal girls with AT may or may not have an underlying anomaly. In large pediatric series, the incidence of an underlying ovarian pathology ranged from 51% to 84%. Whenever an underlying anomaly is present, it is more probably a benign tumor like a benign cystic teratoma, a follicular hemorrhagic cyst or a serous cystadenoma [4–7]. Malignant masses associated with torsion are extremely rare, accounting for 2% of cases only; this is possibly related to an invasion of the adjacent structures by the malignancy, with adhesions that limit the mobility of the adnexal structures.
Many studies indicate that the right ovary is more frequently involved [1, 9]. The lower rate of left torsion may be explained by the partial adhesion of the left adnexa to the mesosigmoid [4, 10, 11].
Abdominal pain, accompanied by nausea and vomiting, is the main clinical symptom [3, 9]. In case of classic ovarian torsion, pain is sudden and acute. The most common sign on physical examination is abdominal tenderness. The presence of fever is more common in children than in adults. Other symptoms or signs such as restlessness and a palpable pelvic mass are more frequently reported in childhood. Raised white cells count is associated with ovarian necrosis. Raised temperature is associated with more advanced cases and a higher risk of tissue necrosis [3, 9, 12, 13].
24.2.2 Imaging
As mentioned, transabdominal ultrasound is the preferred imaging modality in girls with pelvic pain and (clinically) suspected AT. CT and MR imaging are infrequently used, except in cases with misleading presentation or when sonography is inconclusive. A fairly filled bladder, serving as an acoustic window, is ideal in order to obtain diagnostic US images (Table 24.2).
Table 24.2
Imaging findings in typical form of adnexal torsion
All modality signs, but especially US findings | • Unilaterally enlarged ovary • Peripherally displaced follicles • Central stromal edema • Complex adnexal mass • Twist of the pedicle vessels (“whirlpool sign” or “nipple sign”) • Medialization of the ovary • Displacement of the uterus of the midline • Free pelvic fluid • Thickened or enlarged fallopian tube. |
Additional CT and MRI findings | • Lack of enhancement of the ovary after contrast injection • Twisted lombo-ovary pedicle |
Specific addition of CT (if performed for another clinical suspicion) | • Differential diagnoses • Calcifications/fat in an adnexal germinal mass |
Specific addition of MRI | • Anatomical characterization • Underlying adnexal mass • Evidence of necrosis and hemorrhagic infarction |
24.2.2.1 Ultrasound Findings
On US, the demonstration of an unilaterally enlarged ovary is the most common finding in case of AT [1, 11, 14] (Fig. 24.2). Oltmann et al. [15] has reported that, in a pediatric population older than 1 year, the presence of a pelvic mass larger than 5 cm diameter has the highest diagnostic sensitivity for the diagnosis of ovarian torsion. Servaes et al. have reported the interest of a volume ratio: the size of the twisted ovary was compared with that of the contralateral ovary; the median ratio between the twisted side and the normal side was 12. The median volume ratio was significantly greater (>20) when an adnexal mass was present [1]. The torsed adnexa determines the visualization (in up to 70% of cases) of a complex adnexal or abdomino-pelvic mass, that can predominantly be cystic, solid/echogenic or both [11, 16].
Fig. 24.2
Adnexal torsion in a 6 year-old girl. (a) Ultrasound Axial view: Significant increase in size of the left ovary which is medialized. The follicles are displaced peripherally. (b) Color Doppler US Axial view: Persisting vascularization on color Doppler (despite torsion). (c) MR imaging: Coronal T2-Weighted sequence: similar findings as in US. Significant asymmetry of size of the ovaries and medialization of the left adnexa. Note the central stromal edema visualized as a central hypersignal. (d) MR imaging: Coronal T1-Weighted sequence post Gd. Central lack of enhancement related to necrosis. US had shown persisting flow on Doppler. Whirl pool sign on the left side (e) Laparoscopic view showing the torsed adnexa (arrow)
In addition to the ovarian enlargement, a peripheral displacement of the follicles secondary to stromal edema and venous congestion can be visualized in up to 70% of patients with AT [3, 17].
A “whirlpool” sign or the “nipple” sign has been reported in case of AT; it appears as a pseudo-mass of concentric stripes with a beaked center. This can also be visualized as an ellipsoid or tubular mass with internal heterogeneous echoes, depending on the plane of scanning. This sign corresponds to the torsion of the vascular pedicle, tubal structures and supporting ligaments. In a study by Lee et al., a twisted vascular pedicle was detected in 88% of cases of AT. When present, this finding is pathognomonic of AT [18].
Another potentially helpful sonographic sign is the medialization of the adnexal structures with sometimes a complete controlateral position of the twisted ovarian. As torsion occurs, the twisting of the adnexal structures adjacent to the broad ligament creates a centrifugal force that attracts the adnexal structures closer to the uterus [19].
The rates of the various US findings such as adnexal enlargement, adnexal edema, or ovarian or paraovarian cysts are reported similar between the pre- and the post-menarchal girls [13].
Ipsilateral deviation of the uterus (IDU) was found to be frequent in AT. This results from the displacement of the abnormal adnexa toward the midline with the broad ligament acting as a fulcrum pulling the uterus towards the affected side. The contralateral fallopian tube is straightened as well [20]. This sign is insufficient by itself. In the absence of supporting clinical and imaging findings of torsion, the identification of IDU in the routine clinical setting of an adnexal mass is not a strong predictor of AT.
Thickened or enlarged fallopian tube: fallopian tube torsion associated with a hydrosalpinx has been reported in approximately 9% of AT. Additional findings such as a thickened, echogenic fallopian wall and the presence of internal tubal debris or hemorrhage may render difficult the differentiaton between a fallopian tube torsion and hydro/pyosalpinx in relation with pelvic inflammatory disease. The clinical context and the search of a pelvis surgical history are essential for the differentiation.
In addition, free pelvic fluid in the cul-de-sac has been detected with US in up to 87% of cases of ovarian torsion [11, 16].
There are conflicting data regarding the utility of Doppler evaluation for the demonstration of vascular compromise in the setting of suspected AT. Evidence for ovarian torsion on color Doppler flow are highly variable and depend partially on the degree of vascular compromise.
The classic color Doppler sonographic sequence in adnexal torsion is first, the absence of venous flow followed by the absence of arterial flow. Noteworthy, the ovaries have a dual supply from both ovarian and uterine arteries, which can result in detection of arterial flow even when the ovary is found to be partially necrotic at surgery. Some authors have reported arterial or venous blood flow abnormalities in two-thirds of patients [4] while others report much higher rates [5, 21]. Conversely, in another study, 60% of patients with AT had normal color Doppler flow findings [11, 22]. Absence of Doppler flow does not occur in every case of AT and may occur only as a late finding [23]. So, the sensitivity of absent arterial flow can be as low as 40% [17]. However, some authors insist on a high negative predictive value [24].
Therefore, neither the presence nor the absence of Doppler flow can be used to definitively confirm or exclude torsion; still the degree of vascularization and the presence of flow might be useful for predicting the viability of the ovary following detorsion. Fleischer et al. [25] have reported that the presence of central venous flow in a twisted ovary was associated with increased likelihood of ovarian viability post detorsion.
Noteworthy, in post-pubertal girls, hemorrhagic cysts can have a similar appearance to twisted ovaries [26]. Both entities may have a complex US appearance and a reduced or absent arterial flow. Large hemorrhagic cysts may be responsible of a mass effect on the adjacent ovarian tissue and on the blood flow.
If a AT on tumor is diagnosed by US, detorsion must be made in emergency by laparascopy after dosage of tumoral markers. MR imaging will be performed after surgery, to fully assess the mass.
24.2.2.2 MR Imaging and CT
In an acute clinical setting, after confirmatory US, no complementary examination should delay exploring laparoscopy.
When the diagnosis of AT is unclear, as in case of subacute or intermittent ovarian torsion, MR imaging can be useful to further delineate the anatomy, particularly in a preoperative setting. MR imaging can also characterize an underlying mass.
MR imaging protocol includes T2-Weighted sequences, T1-weighted fat-suppressed sequences to detect hemorrhage and fat in germinal masses, as well as T1-Weighted fat-suppressed post-contrast sequences. The latter are aimed to detect compromized vascular supply, which would suggest infarction or necrosis (Fig. 24.3).
Fig. 24.3
Adnexal torsion in a 13 year-old girl with underlying benign cystic teratoma. (a) US Sagittal view: Large parauterine mass with follicles. (b) US Axial view: Central cystic component with a hyperechogenic crown. (c) MR imaging: Axial T2-Weighted sequence. Significant asymmetry of size of the ovaries and medialization of the left adnexa. Note the pelvic effusion and the displacement of the uterus off the midline. (d) MR imaging: Axial T1-Weighted post Gd. Lack of enhancement of the large twisted ovary with a central cystic area limited by a “fat component” (arrow) corresponding to a teratoma. (e) MR imaging: Axial T1-Weighted fat-saturated sequence. The fat component disappears confirming the diagnosis of teratoma
As with US, an enlarged ovary is the most common finding on MR imaging [14]. It is more specific when it is associated with an edematous central stromal area and peripherally displaced follicles. This finding is best seen on T2-weighted sequences, appearing as a central hypersignal.
Performing CT is not recommended in the evaluation of suspected ovarian torsion in children, mainly because of ionizing radiation hazards and poor cost-efficiency when compared to US. It could be performed when the clinical presentation is unclear. Whenever performed, only a venous acquisition after contrast injection should be obtained. Noteworthy, a CT is useful as it can identify other causes of the acute pelvic symptoms such as appendicitis or urolithiasis, both may clinically mimic AT.
A further contribution of CT is its ability to demonstrate calcifications within germinal tumors.
CT and MR imaging were also found to be useful in delineating the twisted vascular pedicle, similarly to the sonographic whirpool sign (Fig. 24.2). The twisted lombo-ovarian pedicle can itself be visualized as inflamed and thickened.
A decreased or absent enhancement of the ovarian parenchyma after contrast injection confirms ovarian necrosis.
Further CT and MR imaging features include ascites, fallopian tube thickening, displacement of the uterus towards the twisted ovary, and change in position of the involved adnexa toward either the midline (medialization) or the contralateral side of the pelvis.
Finally, hemorrhagic infarct will appear as a T1 hypersignal and lack of enhancement.
24.2.3 Special Cases and Imaging Pitfalls
24.2.3.1 Isolated Torsion of a Fallopian Tube
Rarely, torsion can be limited to the Fallopian tube itself, leaving the ovary undamaged. The etiologies of fallopian tube torsion includes both intrinsic fallopian tube abnormalities and extrinsic causes like pelvic post-surgical or infectious adhesions, para-adnexal—para-tubal or para-ovarian—cysts, the latter are the most common cause for fallopian tube torsion in the pediatric population [27–29].
The clinical picture is the same as for a complete AT but it may be more subtle. Imaging may show tubal changes, such as dilated thick-walled tube or a pseudomass between the uterus and the ovary, whereas the ovary appears normal (Fig. 24.4). Tubal dilatation can be global or segmental with an appearance of a cyst. The visibility of plicae tubariae is useful and confirms the diagnosis of tubal dilatation. A paratubal or paraovarian mass is often associated as reported in the literature [30, 31]. The whirpool sign is difficult to detect.
Fig. 24.4
Isolated left fallopian tube torsion in a 12 year-old girl, confirmed at laparoscopy. (a) US Axial view: Isolated tubal dilatation with presence of plicae tubariae ant thickening of tubal walls. (b) MR imaging: Axial T2-Weighted sequence. Two normal ovaries (two arrows) with isolated hydrosalpinx
24.2.3.2 Recurrent or Asynchronous Adnexal Torsion
Asynchronous bilateral torsion is defined as torsion of both ovaries but at different periods; asynchronous torsion is rare, but can be devastating in terms of fertility especially if repeated oophorectomy has to be performed. The risk of recurrent ipsilateral or asynchronous contralateral AT is unknown, though estimated figures range from 2% to 5% [32] to as high as 10% in the absence of apparent ovarian disease [5]. It is likely that these patients have underlying anatomic variations that render them at increased risk of AT.
Though paratubal and paraovarian cysts are rare in adolescent females, the influence of post-menarchal hormonal stimulation on these tubal derivates could favor asynchronous AT [33].
24.2.3.3 Association with an Incarcerated Inguinal Hernia
The inclusion of an ovary in an inguinal hernia is “classic” and the diagnosis can be confirmed easily by US [34]. It is usually not painful; when uncomplicated, the treatment should not an emergent but a programmed surgery.
AT may occur within an indirect inguinal hernia that will become incarcerated. It occurs more often in pre-menarchal girls [13]. Merriman reviewed 71 cases of irreducible hernias in girls and report that 82% the hernias contained an ovary; 11 ovaries had twisted [35]. Very rarely the hernia may contain the fallopian tube and the uterus (Fig. 24.5). All these conditions require emergency treatment, usually surgery.
Fig. 24.5
Incarcerated ovary and uterus in an inguinal hernia. (a) US Axial view: Incarcerated ovary (arrow) with fluid in a inguinal hernia. (b) US Sagittal view: Uterus gets into the hernia (arrow)
24.2.3.4 Context of Primary Hypothyroidism
Precocious puberty, large bilateral polycystic ovaries and premature menarche can develop in a context of severe hypothyroidism, facilitating a twist of the adnexa [36].
24.2.3.5 Uterine Torsion
Uterine torsion is considered exceedingly rare in children and very rare reports have been published. The diagnosis is rarely made preoperatively and necrosis of the uterus is a dramatic complication [37].
24.2.4 Treatment
The exact duration of torsion that would lead to permanent ovarian necrosis is unknown. Surgery has to be performed as early as possible.
The question of a malignant tumor is rapidly raised when a mass is present. Surgery in emergency is mandatory to treat the torsion, but the tumor is not always removed at that time, allowing secondary biological and imaging work-up before deciding on the optimal treatment.
The best treatment of a confirmed AT is controversial. Over the past decade, multiple studies have demonstrated a partial preservation of ovarian function after a conservative approach, just untwisting the vascular pedicle; this even despite the necrotic appearance of the twisted ovary at surgery [38]. As the risk and clinical significance of secondary emboli is unproven and the risk of malignancy is low, ovarian conservation is currently the accepted management in girls with ovarian torsion.
Another debate is whether patients should undergo ovariopexy to prevent recurrent torsion. There are multiple case reports of adolescents with recurrent ipsilateral and contralateral torsion in normal-appearing ovaries that have been successfully treated via ovariopexy to the pelvic sidewall [39].
24.3 Hemorrhagic Cysts and Rupture of Functional Cysts
In the post-menarchal period, functional cysts are very common. There are two types of functional cysts: the follicular cyst and the luteal cyst. Both can get complicated by hemorrhage and rupture that can induce acute pain, most often in the second part of the cycle.
Noteworthy, a rupture or a hemorrhage of a functional cyst can be encountered in the perinatal period as well (see Chap. 5).
24.3.1 Physiopathology
A follicular cyst is the result from a dominant pre-ovulatory follicle whose size is increasing due to a continuous stimulation by gonadotropic hormones. The rate of discovery of “pure fluid containing cysts” on US examinations ranges between 13.7–20% [40, 41].
The corpus luteum operates like a cyclic physiological endocrine gland. Its normal size ranges between 15 and 25 mm. A cyst of the corpus luteum results from excessive intracystic bleeding. The US presentation of a corpus luteum and of a cyst of the corpus luteum is similar, only their size varies [42] (Fig. 24.6).
Fig. 24.6
Diagrammatic representation showing the relationship between the different physiological and pathological changes of the dominant follicle
24.3.2 Imaging
Functional cysts and their complications are clearly best imaged by US.
On US, a follicular cyst corresponds to an intra-ovarian unilocular cystic lesion larger than 3 cm but less than 8 cm diameter. This criterion of 3 cm allows to differentiate it from a simple physiological dominant follicle that would measure between 15 and 28 mm. The follicular cyst is usually anechoic containing pure fluid leading to posterior acoustic enhancement. The cyst wall is thin and can sometimes appear splitted due to the detachment of the granulosa cells layer. No atypical sign such as calcification or intracystic proliferation should be observed. A subtle vascularization of the wall of the cyst is typical of its functional character.
There is no absolute ultrasound criteria to differentiate an uncomplicated follicular cyst from some pure cystic ovarian tumors. Only US follow-up with a spontaneous regression of the cyst allows a retrospective confirmation of the functional nature of the cyst. A follicular cyst usually regresses spontaneously in 1–3 months. However, up to 35% of follicular cysts do persist (“persisting follicular cyst”) over the 3 months period.
A cystic but heterogeneous structure measuring less than 25 mm, surrounded by a crown of peripheral hypervascularisation without internal vascularization is characteristic of a simple corpus luteum. It is unilocular and may present carved outlines. There is a typical peripheral vascularization [41]. A cyst of the corpus luteum is defined by a diameter larger than 30 mm with the same US characteristics.
Number of events may complicate the course of functional cysts: namely intracystic hemorrhage, cracking or breaking and AT; all conditions associated with acute abdominal pain. Intracystic bleeding may be responsible for the acute enlargement of these cysts with a very heterogeneous content. Multiple patterns exist; the most common being an aspect in so called “fishing net” corresponding to tracts of fibrin [43] (Figs. 24.7 and 24.8).
Fig. 24.7
Functional hemorrhagic cyst in a 14 year-old girl. (a) US Axial view: Hyperechoic cyst within the ovary. The echogenicity raises a doubt about a possible endometrioma justifying the realization of a MRI. (b) MR imaging: Coronal T2-Weighted sequence. Cyst with homogeneous high T2-W intensity (arrow). (c) MR imaging: Axial T1-Weighted fat-saturated sequence. Hypersignal of the cyst due to hemorrhagic content. (d) MR imaging: Sagittal T1-weighted fat-saturated post Gd sequence. Thin peripherical vascularization of the walls in connection with the functional character. (e) US Axial view: Normal size of the ovaries 3 months later
Fig. 24.8
Endocavitary US of a functional hemorrhagic cyst in a 16 year-old girl. Heterogeneus content resembling a “fishing net” with peripherical hypervacularization
In cases of rupture, intraperitoneal effusion will appear usually limited to the pelvis but sometimes diffusing into the entire abdomen in case of vascular rupture. The cyst would then have disappeared or present a slumped appearance.
MR imaging can provide additional information in case of complex adnexal mass whenever there is a doubt on the presence of a solid tumoral component. MR imaging can also be more useful in evaluating atypical forms, especially in the absence of significant changes during follow-up ultrasound examinations.
24.4 Hematocolpos
There are two circumstances beyond the neonatal period where a hematocolpos can be discovered:
Painful primary amenorrhea without pubertal delay, frequently associated with a painful retention of the first menstruations
Primary dysmenorrhea caused by the retention of menstruation in a one-eyed hemivagina in a context of uterine malformation
The role of imaging is to characterize the uterine malformation and its complications, to determine the optimal treatment and to check for associated urologic complications, which are present in 30–50% of cases. Other malformations can be discovered on this occasion like spinal or heart abnormalities [44].