Epidemiology and Pathophysiology
Ovarian torsion is the partial or complete twisting of an ovary around its ligamental support, often leading to an occlusion of its blood supply and associated with the sudden onset of abdominal pain and, if untreated, ovarian necrosis. Ovarian torsion accounts for 2.7% of females presenting with acute abdominal pain, and is a "must not miss" diagnosis due to the possibility of salvaging that ovary. Torsion commonly associated with ovarian cysts, which can act as a catalyst to the torsion process. The larger the mass, the more likely ovarian torsion will occur.
Ovarian cysts can be physiologic or malignant and are known to occur in children of all ages.
- Neonates: Commonly physiologic due to maternal hormones and resolve spontaneously.
- Pre-pubertal Children: Uncommon due to lack of hormones. Cysts can occur in cases of premature puberty, such as McCune Albright or Idiopathic Central Precocious Puberty.
- Pubertal Children: Cysts can be relatively common, mostly asymptomatic, and the result of a follicle which has failed to ovulate. The cysts can lead to pain, itermittent in the case of recurrent partial torsion or constant in the case of full torsion or rupture. Rupture is also associated with bleeding, which can be minor or severe.
Although rare, ovarian malignancies do make up 1 percent of all malignancies found in children and adolescents, and must be considered.
Presentation
Ovarian torsion is a must-not-miss diagnosis. It can be itermittent or constant, and typically presents with:
- Acute Pelvic Pain
- Adenexal Mass
- Nausea/Vomiting
- Low grade fevers
- Orthostasis or Light-headedness if hemorrhaging
Ovarian cysts or masses can present with a wide spectrum of symptoms based on the age of the patient, their pubertal status, and/or any functional properties of the cyst or mass. Ovarian cysts and masses should be suspected in the cases of:
- Precocious Puberty
- Dull, aching pelvic pain or "heaviness"
- Abdominal fullness or bloating
- Adenexal Mass
- Unexplained constipation
Differential Diagnosis
- Torsion
- Pregnancy
- Ectopic Pregnancy!!!
- Molar Pregnancy
- PID
- Tubo-ovarian Abscess
- Ovarian Cysts
- Follicular Cyst
- Corpus Luteum Cyst
- Polycystic Ovarian Syndrome
- Obstructive Genital Lesions
- Imperforate Hymen
- Noncommunicating Uterine Horn
- Endometriosis
- Associated Chocolate Cyst of Endometriosis
- Ovarian Malignancies
- Endodermal Sinus Tumor
- Dysgerminoma
- Granulosa Cell Tumor
- Sertoli-Leydig Cell Tumor
- Teratoma
- Other Malignancies
- Wilm's tumor
- Neuroblastoma
- Appendicitis
- Appendiceal Abscess
- Pyelonephritis
- Renal Abscess
- Lymphadenitis
- Gastroenteritis
- Constipation
Management
A thorough history should be the first part of the work-up. A thorough menstrual history and sexual history should be performed. Important additional history items will include onset of the pain, quality, location, timing, associated symptoms such as fever, nausea, vomiting, precoscious puberty, hirsutism, abdominal girth, bowel habits, and signs of orthostasis.
Physical exam should pay careful attention to the patient's tanner stage, the abdominal exam for palpable masses, tenderness and/or rebound, the skin exam for cafe-au-lait spots, and, if the patient is old enough and the exam is tolerated, a pelvic exam for palpable masses.
Every patient should receive a pregnancy test immediately upon presentation.
If clinical suspicion for torsion is high, the patient must be rushed to the OR immediately to try and salvage the torsed ovary. During the surgery, if there is no evidence of a treatable ovarian mass, the surgeon may perform an oophoropexy in order to prevent a repeat torsion.
If the suspicion for torsion is low, imaging may be considered for further work-up of the pelvic pain and/or mass.
Ultrasound is the gold standard to assess ovarian cysts and masses. It will help evaluate size of the mass (simple cysts <2cm are considered physiologic), the character (thick walls, calcifications, and septations are suspicious for malignancy), and the presence of free fluid and/or blood in the peritoneum. Dopper flow can be added on to evaluate for ovarian torsion, but is not always conclusive.
CT/MRI are only indicated if ultrasound is strongly suspicious for malignancy. A solid ovarian mass in childhood is a malignancy until proven otherwise by histology.
Serum Markers may be additionally considered if ultrasound is suspicious for malignancy. Markers include AFP (endodermal sinus tumors), LDH (dysgerminomas), hCG (molar pregnancy, ectopic pregnancy), Estrogen (Granulosa cell tumor), Testosterone (Sertoli-Leydig Cell). CA-125 is associated with epithelial ovarian cancers, which are excedingly rare in pediatric patients.
Treatment of Ovarian Cysts without Torsion
Fetal/Neonatal: Expectant management and serial ultrasounds as cysts resolve once maternal hormones cleared. Aspiration may be considered for cysts >4-5cm.
Infants/Prepubertal: If the cyst is simple and asymptomatic, follow-up ultrasounds can watch the cyst. If complex or suspicious for malignancy, surgery for histological evaluation and/or excision must be performed.
Adolescent: Observation, NSAIDs for pain. Oral contraceptive pills can be considered for cysts <6cm. If >6cm, laparoscopic evaluation and cystectomy may be warranted.
References
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2. deSa DJ. Follicular ovarian cysts in stillbirths and neonates. Arch Dis Child 1975; 50:45.
3. Bryant AE, Laufer MR. Fetal ovarian cysts: incidence, diagnosis and management. J Reprod Med 2004; 49:329.
4. Sultan C. Pediatric and Adolescent Gynecology. Evidence‐Based Clinical Practice. Endocr Dev. Basel, Karger, 2004, vol 7, pp 66‐76.
5. Porcu E et al. Frequency and treatment of ovarian cysts in adolescence. Arch Gynecol Obstet 1994; 255(2): 69‐72.
6. Roe A et al. The Diagnosis of Polycystic Ovary Syndrome in Adolescents. Rev Obstet Gynecol. 2011 Summer; 4(2): 45‐ 51.