In practice, the clinician is confronted by two very different situations: (1) a pelvic mass or an endocrine syndrome is discovered and complementary investigations are required for better definition of the nature of the tumor, and (2) acute abdominal pain and vomiting signal an emergency situation, with the danger of adnexal torsion, and surgery is indicated without further delay.
Ovarian Mass Discovered on Subacute Pain or an Endocrine Syndrome
Once pregnancy and PID have been eliminated, the initial diagnosis is based on the association of plain abdominal radiographs and ultrasonography, which (1) confirms the presence of the mass (differential diagnosis with pseudo-masses such as fecalomas, obstructed bladder, pregnancy, urinary disorder or ascites); (2) identifies its location in the upper intervesico-rectal space, thus excluding pelvic tumors of the other spaces and directing attention to the genital apparatus; (3) analyzes its structure: fluid, solid or mixed, and (4) assesses tumor spread.
In most cases, these simple investigations will suggest the diagnosis of an ovarian cyst or teratoma. Viewed in an overall context of suspected leukemia or a lymphoma, they will be sufficient to identify a tissue mass localized in an ovary.
Diagnosis may sometimes be more difficult: a complex cyst with a pseudo-solid appearance or teratomas with a marked calcified component. Here tumor marker measurement, sectional imaging and above all MRI come into their own. MRI is more specific in the analysis of the various components and provides indispensable information on the relationships and spread of the mass.
Torsion is the most common complication of ovarian masses, with a frequency ranging from 3 to 33%. When the ovarian mass manifests with acute pain, torsion is highly probable, ranging from 42% to 80%, depending on the series.
Several publications have suggested that ovarian tumors with torsion are usually benign. A possible explanation is that the rapid growth of a malignant tumor leads to peritumoral inflammation and the formation of adherences. The size of the mass does not appear to be significantly correlated with the presence or absence of torsion. Although torsion has not been shown to correlate with cyst size, larger cysts, and thus heavier ovaries, appear to be less prone to torsion. Moreover, in several publications of acute ovarian torsion, the frequency of associated tumors varies from 32% to 84%.
The clinical symptoms of tumoral adnexal torsion are in no way specific.
Usually, acute subumbilical abdominal pain is associated with vomiting. The diagnosis can be difficult and the differential diagnosis may include appendicitis, kidney stone, PID, ectopic pregnancy and ruptured ovarian cyst.
Ultrasonography reveals an echogenic pelvic mass that is usually complex (solid and cystic) and the homolateral ovary is not visualized. Effusion in Douglas’ pouch is generally a late manifestation. If there are abnormal calcifications on ultrasound study or on the plain radiograph, a teratoma may be suspected. Lack of blood flow on Doppler ultrasonography suggests arterial obstruction. CT scan may be useful but treatment must not be delayed too long just to obtain complementary imaging or biological tests (tumor markers).
Surgical intervention by laparoscopy or laparotomy is in fact the most reliable method of diagnosis and first-stage treatment.
Numerous series of torsions of tumoral or healthy adnexae show that in many cases wide excision with oophorectomy or salpingo-oophorectomy was performed. Many reasons were given for excision, such as fear of Pulmonary embolism after detorsion or of missing a malignant ovarian tumor on an enlarged adnexa (any ovarian torsion, whether on tumoral or healthy adnexae, presents as a mass), as well as the generally necrotic, bluish-black appearance of the adnexa which is then considered unsalvageable.
In the light of numerous publications, all these reasons are debatable and should lead to a much more conservative attitude with the aim of preserving later fertility: (1) Ovarian detorsion was considered unadvisable because of the theoretical risk of Pulmonary embolism from the gonadal veins. There has been no published case confirming this possibility in the child. To the contrary, detorsion as reported by several authors did not lead to any thromboembolic incident. (2) Adnexal torsion is often associated with an ovarian tumor but as a rule the latter is benign. As far as we are aware, in children there have been no cases of torsion associated with a malignant tumor. (3) Ovarian viability after detorsion is very difficult to evaluate.
Numerous studies in both adults and children have demonstrated the amazing capacity of the ovary not only to revascularize but also to recover satisfactory function after simple detorsion (on condition that only detorsion is performed, together with aspiration of a cystic lesion if necessary, but avoiding cyst excision during the acute phase).
Because of the lack of proof of thromboembolic risk after detorsion, the low incidence of malignancy and the difficulty of evaluating ovarian viability, in cases of adnexal torsion detorsion is perfectly justified whether by laparoscopy or conventional surgery; if necessary it may be associated with aspiration of a cystic lesion if the latter appears benign.
Excision of a cyst is probably unadvisable during the acute phase.
The child can then be followed by ultrasonography and tumor marker measurement. If an ovarian lesion persists with normal tumor markers, the tumor should be excised at a distance from the acute episode.
Ovarian fixation is usually advisable but this has recently been debated as it may produce adherences and modify the relation between the ovary and the fallopian tube and thus in itself adversely affect later fertility; also, it does not totally exclude the risk of later torsion.
Revision date: June 20, 2011
Last revised: by Sebastian Scheller, MD, ScD