Introduction

Peripheral precocious puberty is defined as the appearance of secondary sexual characteristics not dependent on the hypothalamic-pituitary-gonadal axis ⁽¹⁾. Its most common cause is ovarian cysts ⁽²⁾. On the other hand, ovarian cysts have been associated with an increased risk of ovarian torsion. Ovarian torsion is a pathology considered a surgical emergency ⁽³⁾. Early diagnosis and treatment are required to avoid loss of adnexa and other complications. After the intervention, recurrence is rare and management options include oophoropexy after detorsion ⁽⁴⁾. We describe an exceptional case of a girl with precocious puberty who suffered from intermittent vaginal bleeding and recurrent episodes of ovarian torsion.

Case Description 

A girl aged 6 years and 8 months was referred to the paediatric endocrinology clinic for left unilateral thelarche. Advanced bone age: 8 years and 5 months old (Greulich & Pyle). Abdomino-pelvic ultrasound: both ovaries enlarged (right ovary 13 cc, left 15 cc) with follicular cysts (<9 mm). At 7 years and 5 months, a pubertal development in TANNER stage III (S3, P1, A1) was observed with a prepubertal response to the requested GnRH stimulation test. No cafe au lait patches on the skin or cystic changes in bones were found. At 8 years and 2 months old, she presented self-limited vaginal bleeding with a new episode at 8 years and 6 months old, this time accompanied by abdominal pain and vomiting. A new abdominal ultrasound was requested, showing an enlarged right ovary with a volume of 185.5 cc (10 x 5 x 6 cm) with a solid, rounded appearance and multiple="multiple" cysts of up to 15 mm, as well as a uterus with an infantile morphology and left ovary 27.22 cc (2.5 x 4.5 x 3 cm), oval with follicular cysts (<10 mm) (Figure 1). Abdominal magnetic resonance imaging was performed confirming a right ovarian tumour with an image consistent with thickened angulated vascular pedicle (gonadal vein), indicative of torsion. She presented an oedematous ovary with multiple="multiple" cysts in its periphery (the largest measuring 15 mm) and free fluid in the pelvis (Figure 2). Blood test: FSH 0.7 mU/mL, LH <0.3 mU/mL, estradiol <5 pg/mL. Tumour markers were negative. The operation was performed laparoscopically. During the operation, a very enlarged right ovary, with pedicle torsion, was observed. Throughout the procedure, an improvement in the colouration of the adnexa was observed. The operation involved detorsion of the ovary (2 turns) and right oophoropexy. No cystectomy was performed. Histopathological examination of peritoneal fluid was negative for malignant cells and showed acute and chronic inflammation and reactive mesothelial hyperplasia. A control ultrasound was performed 15 days post-intervention with ovarian reduction (60 cc). A month and a half later, she presented a new episode of abdominal pain and vomiting.  Abdominal-pelvic ultrasound was repeated, showing a new increase in right ovarian volume (85 cc) with asymmetric arterial flow compared to the contralateral and ascites. A new intervention was carried out with detorsion (2 turns) and attachment of the adnexa. A control ultrasound was performed at one month without any alterations (right ovary 23.4 cc, left 22.6 cc).                         

Discussion 

Ovarian torsion in girls is a rare pathology that represents a surgical emergency, the most frequent associated symptoms of which are abdominal pain, nausea and vomiting ^(5,6). The most frequent radiological findings are asymmetric enlargement of the ovary, peripheral location of the follicles and sometimes coexistence of an ovarian mass inside ^(6,7). All these findings were present in our patient.

Most cases of paediatric ovarian torsion occur in ovaries with an adnexal pathology, such as teratomas or cysts. The risk of torsion increases when the mass is benign and large, with a size of 5 centimetres or more ^(3,8). Our patient's case is peculiar since she did not present any large mass, only follicular cysts smaller than 1.5 cm.

Surgical treatment options include isolated detorsion, detorsion with oophoropexy, and oophorectomy. In the paediatric population, laparoscopic ovarian detorsion and preservation should be the procedure of choice ^(3,4,10). However, abdominal magnetic resonance showed ovarian oedema and free fluid in pelvis. Some authors suggest that oedema can present as a result of partial intermittent torsion of the ovarian pedicle and may present as a solid, adnexal mass ⁽⁹⁾. Idiopathic recurrence of ovarian torsion is a phenomenon rarely found in the literature, accounting for around 5-18% of the cases described in different reviews ⁽⁴⁾. After idiopathic recurrence, oophoropexy by permanent suture or by plication of the utero-ovarian ligament are the recommended treatments, although this does not completely rule out the possibility of an adnexal torsion in the future ⁽¹¹⁾. In the case of our patient, her initial oophoropexy did not prevent a subsequent adnexal torsion. Our case also represents a diagnostic challenge regarding the characteristics of precocious puberty present prior to the appearance of the ovarian torsion. In the clinical evaluation, the patient presented symptoms compatible with peripheral precocious puberty with bilateral thelarche and advanced bone age, not mediated by the hypothalamic-pituitary-gonadal axis ⁽¹⁾. The ultrasound scan revealed the presence of enlarged ovaries and multiple cysts, which may lead to the suspected presence of functional cysts, the most frequent cause of peripheral precocious puberty ^(1,2). However, hormonal studies are not conclusive of peripheral precocious puberty since we did not find an increase in sex steroids as might be expected ^(1,2).

Taking into account the presence of intermittent vaginal bleeding and recurrent ovarian torsion, in our patient we can assume the presence of functional ovarian cysts with oestrogen production that may have returned due to associated ischaemic-haemorrhagic phenomena, as has been proposed by other authors ⁽²⁾. This would also explain why we obtain low estradiol levels when making analytical determinations. 

Conclusion

In conclusion, precocious puberty in girls may be due to the presence of follicular cysts with autonomous oestrogen production. On the other hand, the presence of several of these cysts favours the development of ovarian torsion, with some cases of recurrent episodes. These episodes of ovarian torsion can cause ischaemic-haemorrhagic phenomena in the cysts, leading to their autoregression or functional involution. We must be aware of the existence of this phenomenon when studying girls with clear signs of precocious puberty in whom a central origin has been ruled out and who present ovarian cysts and low estradiol levels in blood tests.

Conflicts of interest

The authors declare no conflicts of interest related to this article.

^©Sociedad Española de Endocrinología Pediátrica (https://www.seep.es). Published by Pulso ediciones, S.L. (https://www.pulso.com).

Open Access article under the CCBY-NC-ND licence (http://creativecommons.org/licenses/by-nc-nd/4.0/).

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