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Cradle to College
Vol. 19 No 3 | Spring 2017
Feature
Ovarian complications in adolescents

Adolescence is defined as the time between puberty and adulthood. Ovarian complications in adolescents have a similar underlying pathology to their paediatric and/or adult counterparts. Ovarian problems in this age group may be associated with congenital disorders, benign or neoplastic transformation, infection, inflammatory or normal physiological changes. This article aims to review the presentation, investigation and pathology of ovarian complications in adolescents. Detailed management is beyond the scope of this article.

Presentation

The majority of patients identified with an ovarian pathology present with lower abdominal or pelvic pain. This commonly occurs as chronic pain, but acute presentations contribute significantly to emergency surgeries for a pelvic mass or acute abdominal pain.1 In a study of 130 cases of children and adolescents treated surgically for ovarian lesions, approximately 52 per cent presented with chronic abdominal pain and a further 25 per cent presented with acute abdominal pain.2 Patients may complain of intermittent or cyclical pain. Chronic pain with increasing abdominal girth may be associated with large adnexal cysts or neoplasms;3 the pain may be unilateral or bilateral. The pain associated with malignant tumours tends towards a chronic unilateral pain on presentation.4 Right iliac fossa pain is more common than left, which overlaps with the presentation of common gastrointestinal conditions, such as appendicitis.5 A small, but significant, number of ovarian pathologies are identified incidentally on ultrasound scan (USS).6 7

Other presenting symptoms may include nausea, vomiting and/or fever.8 Premenarchal girls may first be seen with precocious puberty, a feature of underlying functional cysts and hormone-producing neoplasms.9 Girls may also present with abnormal uterine bleeding or dysmenorrhea.10

Diagnosis

It is important to obtain a thorough clinical history of the presenting complaint. The gynaecological history should be ascertained alongside a confidential sexual history. The physical examination should be tailored to the differential diagnosis for an adolescent presenting with symptoms associated with a suspected ovarian lesion. This could dissociate between gastrointestinal and ovarian pathology, but also guide subsequent investigations. Clinical examination should identify signs of precocious puberty such as breast, axillary and/or pubic hair development. Sensitivity is required, particularly when the adolescent is not sexually active. Large adnexal lesions may be palpable by abdominal examination, but a vaginal examination or, for the non-sexually active, a per rectum (PR) may be required for assessment.11

Common investigations in the adolescent presenting with lower abdominal pain include a full blood count, C-reactive protein and urinalysis, including for pregnancy. Tumour markers (AFP, beta HCG, LDH, CA-125, CEA, oestradiol, testosterone) may be required after clinical or radiological assessment. As with adults, transabdominal USS is useful in identifying lesions, but complex or solid lesions require further imaging such as CT or MRI.12 MRI is more accurate (97 per cent) in identifying ovarian pathology than CT (87 per cent) or USS (77 per cent).13 The latter is preferable as it provides an economical, low-risk (no sedation required) and non-ionising radiation modality. Although an initial vascular assessment with Doppler can be performed, USS has low specificity and sensitivity to detect ovarian torsion.14 This is due to the intermittent nature of ovarian torsion and the presence of a collateral ovarian blood supply.15

Serum tumour markers can be increased in benign (non-neoplastic lesions and benign tumours) and malignant ovarian lesions.16 17 18 Despite the high false-positive and false-negative results, tumour markers are a useful tool in identifying high-risk patients and planning their subsequent management.19 One study identified the probability of malignancy to be 0.25 per cent post-testing in the presence of negative tumour markers and a tumour less than 10cm diameter with no solid components.20

Ovarian cyst rupture and mittelshmerz

Simple cysts are the commonest adnexal findings in paediatric patients.21 In comparison to premenstrual girls, menstruating females have a higher incidence of ovarian cysts, which are also larger due to endogenous hormone production. Ovarian cysts in this group usually grow to 2–5cm and then resolve spontaneously, allowing for conservative management.22 Mittelschmerz syndrome is pain on follicular rupture at ovulation. It may be due to a small haemorrhage at this time causing peritoneal irritation. Pain is typically mild, mid-cycle and unilateral. It may occur as a sporadic event or less commonly, chronically.

Benign cystic lesions are largely asymptomatic, but carry the risk of rupture, haemorrhage or torsion, particularly if large.23 They may present with pain requiring surgical intervention, particularly if the cyst persists or grows. They occur due to the lack of involution of ovarian follicles, which subsequently develop into corpus luteal or functional cysts. Prepubertal girls may develop ovarian cysts as a result of intermittent gonadotrophin production from the maturing pituitary. It is uncommon for these cysts to grow over 2cm and most spontaneously resolve requiring no follow up.24 Haemorrhagic corpus luteal cysts are difficult to differentiate on USS from complex ovarian masses or tubo-ovarian abscesses (TOA).25 26

Polycystic ovary syndrome

Polycystic ovary syndrome (PCOS) is a complex multifactorial disorder that may first present in adolescence.27 It is diagnosed using the Rotterdam criteria by the presence of two of the three signs: clinical and/or biochemical signs of hyperandrogenaemia, anovulation and/or polycystic ovaries.28 Its aetiology is related to the disorder of hypothalamic-pituitary-ovarian axis and, although benign, is related to extensive physical and mental morbidity.29 In particular, the associated symptoms of obesity, hirsutism and acne are psychologically difficult. The biochemical sequelae of PCOS are now known to be related to an increased risk of endometrial cancer and cardiovascular disease in later life.

Endometrioma

Endometriomas are associated with adult females with a history of chronic pelvic pain and endometriosis. They may, however, first be diagnosed in adolescence, with one report of an 11-year-old premenstrual girl with an ovarian endometrioma.30 Endometriomas are identified on USS and treatment is usually laparoscopic ovarian-preserving surgery.

Ovarian torsion

Ovarian torsion has an incidence of approximately 4.9 per 100,000 girls aged 1–20 years old31 and accounts for 3 per cent of acute cases of lower abdominal pain in this group.32 It is uncommon, but requires early diagnosis and prompt treatment to preserve the ovary. Ovarian torsion is associated with acute pelvic pain,33 but chronic cyclical pain may indicate an intermittent mass torsion. It occurs more frequently on the right than left (3:2), possibly due the stabilising effect of the sigmoid on the left or the mobile caecum on the right allowing greater movement of the right ovary.34

Ovarian torsion may occur in normal ovaries as well as with large ovarian cysts.35 Up to half of children with ovarian torsion will have normal ovaries.36 37 Benign teratomas, tubal cysts, follicular or corpus luteum cysts and serous or mucinous cystadenomas are the most common lesions associated with torsion. Malignant lesions are a less common cause for torsion (2–4 per cent), possibly due to the stabilising effect of inflammatory adhesions.38 39 40 41

Ovarian neoplasms

Neoplastic lesions of the ovary can be either benign or malignant. Benign neoplastic ovarian masses are the second commonest ovarian lesions found in paediatric age groups. They are usually cystic, although solid lesions do occur. The incidence of ovarian neoplasms in children and adolescents is approximately 2.6 per 100,000 girls.42 Ovarian tumours contribute to 1 per cent of childhood cancers, but account for approximately 10 per cent of malignancies.43 The main ovarian tumour cell types are germ cell, stromal and epithelial cell tumours.

In children and adolescents, germ cell tumours are the most common neoplasm and 70 per cent are mature cystic teratomas (dermoid cyst).44 Mature teratomas require surgical intervention and up to 15 per cent may be bilateral.45 Unfortunately, they may recur in the ipsilateral ovary after cystectomy, especially in the presence of a few tumours or rupture.46 Benign neoplastic lesions recur more frequently than non-neoplastic lesions.47 In paediatric patients with recurrent, multiple or bilateral mature cystic teratomas, there is a 2–3 per cent incidence of the development of germ cell tumours.48 There is also a risk of malignant transformation in later life.

Malignant germ cell tumours in adolescents include immature teratomas, dysgerminoma and yolk sac tumours. They may present with precocious puberty, as a result of oestrogen and βHCG production.49 Dysgerminomas represent approximately 1 per cent of all ovarian cancers and up to 30 per cent of malignant ovarian germ cell tumours. A small, but significant, number (5 per cent) may undergo trophoblastic change and secrete βHCG and LDH.50 Yolk sac tumours may be associated with increased βHCG and AFP. Gonadal dysgenesis is associated with a rare germ cell tumour known as gonadoblastoma. Due to the high risk of malignancy in such patients, gonadectomy is recommended, particularly in pure gonadal dysgenesis (46XY) or girls with mosaic Turner’s syndrome (46X/46XY).51

Benign stromal tumours (for example, thecomas and fibromas) are uncommon in children and adolescents, representing only 1.5 per cent of ovarian neoplasms. Malignant stromal tumours, such as juvenile granulosa cell tumours, are associated with precocious puberty and 80 per cent are diagnosed before the age of 20.52 Sertoli-Leydig tumours are rare and present before the age of 30, with symptoms of hyperandrogenism such as hirsutism, amenorrhoea, hoarse voice and clitoromegaly.

Benign epithelial tumours are uncommon in adolescents. They may be an incidental finding or present with abdominal pain or menstrual disorders.53 These tumours may be serous, mucinous or mixed with endometroid. The risk of malignancy is up to 16 per cent and, therefore, requires close surveillance post-surgery. Up to 40 per cent are considered borderline epithelial tumours and these may present with similar symptoms as well as abdominal distension. Surgical treatment is much like the adult female, with emphasis on preservation of fertility. FIGO stage I borderline tumours have a good prognosis, but risk of recurrence may be up to 60 per cent with stage II-IV disease.54

Ovarian ectopic pregnancy

Primary ovarian ectopic pregnancy contributes to 0.5–3 per cent of all ectopic pregnancies.55 It is rare in adolescence, but must be included in the differential diagnosis of a pubertal girl who presents with lower abdominal pain and/or vaginal bleeding. Risk factors, such as pelvic inflammatory disease (PID) and smoking, are more prevalent in this age group.56 Management is similar to adults and attempts should be made towards ovarian-tissue-sparing techniques to preserve fertility.

PID and tubo-ovarian abscesses

Approximately half of adolescent females are sexually active and 20 per cent of PID cases occur in girls less than 19 years old.57 Therefore, the age-related risk of developing PID in adolescence is much higher than for older women. Unfortunately, there are inadequate rates of testing for STIs in this vulnerable group, which may lead to further complications, such as tubo-ovarian abscesses, chronic pelvic pain and infertility. One study showed that approximately one fifth of adolescents with PID presented to hospital with tubo-ovarian abscess with an acute abdomen.58

Ovarian complications in adolescents are uncommon, but they have an important impact on the physical and mental health of the developing girl. This is further confounded by issues surrounding body image and sexuality. Ovarian disorders in adolescents require prompt diagnosis and management to prevent future complications, including consideration of fertility-sparing treatments.

References

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