C. Pienkowskia, C. Bauninb, M. Gayrarda, F. Lemassonc,
P. Vayssec, M. Taubera
aUnité d’Endocrinologie, Génétique et Gynécologie Médicale; bService d’Imagerie
Pédiatrique et cUnité de Chirurgie Viscérale, Hôpital des Enfants, Toulouse, France
Ovarian cysts can occur in early childhood (between 3 and 8 years of age) but are more common in the neonatal and adolescent periods: The frequency of functional cysts decreases in early childhood and then increases as puberty is approached. During childhood, most large banal cysts are derived from degenerated follicular cysts. These cysts are first formed by ovarian gonadotropin stimulation and result from failure of follicular apoptosis or interaction with other hormonal secretion. Besides functional cysts, cystic neoplasms may be encountered. Moreover, hormone-secreting cysts cause rapid pubertal development or precocious pseudopuberty. In these cases, the possibility of an autonomous ovarian cyst due to G-protein mutation in McCune-Albright syndrome should be considered. Ultrasonography has been an excellent tool for detecting the presence of banal cysts in young children and no malignancy has appeared as banal on ultrasonography or computed tomographic scan. Management of ovarian cysts depends on the clinical signs, the sonographic appearance and volume of the mass, and finally its persistence.
Two types of presenting signs may suggest an ovarian cyst, or a cyst may be asymptomatic and discovered incidentally on ultrasonography performed for other reasons. In the case of a painful syndrome, pelvic ultrasonography confirms the diagnosis. In the case of an endocrine syndrome, hormonal investigations are necessary and complete the ultrasound findings.
Sometimes when the patient is questioned, identical episodes are found to have occurred in the preceding weeks. In some cases, this syndrome is extremely painful and associated with vomiting. When an ovarian complication such as torsion or hemorrhage is feared, emergency surgical treatment is required because adult ovarian function is endangered. The family and physician should be attentive to repeated manifestations as they may signal recurrence; emergency ultrasonography should always be performed. The patient may also present vague pelvic pain associated with perceptible and visible development of an abdomino-pelvic mass.
The ‘endocrine’ syndrome is marked by precocious development of sexual characteristics, which may be associated with increased growth velocity and advanced bone maturation. Rapid breast development followed by metrorrhagia suggests precocious pseudopuberty due to ovarian cysts. Metrorrhagia often consists of recurrent red blood loss lasting 2–3 days. When looking for an etiology, skin pigmentation should be sought, as the association of precocious puberty, ‘café-au-lait’ skin spots and polyostotic fibrous dysplasia characterizes the McCune-Albright syndrome.
Revision date: June 21, 2011
Last revised: by Andrew G. Epstein, M.D.