Introduction: Ovarian stimulation syndrome (OSS) in an infrequent disorder, in preterm infants under 30 weeks gestation. The are very few cases described in the literature. The immaturity, lack of gonadal axis feed-back or mutations in the FSH receptor genes, may explain its physiopathology. We present the case of a 26-week neonate with this syndrome.
Case summary: 26-week newborn with extremely low weight (460g) born by emergency cesarean section due to severe maternal preeclampsia with loss of fetal wellbeing. She had an Apgar 4/6. She needed to be admitted to NICU for 3 months, where she was hemodinamically stable and presented pathologies due to her immaturity, such as: bronchopulmonary dysplasia, necrotizing enterocolitis grade 1, and cerebral haemorrhage grade IV. At 2 months of age genital swelling began to appeared, including clitoris, minor and major labia, reaching upper part of inferior extremities and hypogastrium. An ultrasound was performed to rule out vascular and lymphatic pathology. Some investigations were performed: kidney and liver function, electrolytes and proteins, and all of them were normal. Hormonal investigations shown elevated serum oestradiol levels (232 pg/mL), LH (14UI/L), and FSH (25.2UI/L), 17. OH-progesterone levels were normal. An ovarian ultrasound was performed finding a normal uterus size and morphology (20x10x10mm), normal right ovary and a simple cyst (18 mm) in the left ovary. We decided to maintain an expectant attitude. When she was discharged the edema was limited to genital area. She had monthly follow-up observing a progressive disappearance of the edema.
Conclusions: A pathognomonic sign characterizes OSS, which is edema. Ovarian cysts are observed in most of the cases described in the literature. It is convenient to identify this disorder by clinical signs to avoid both performing useless investigations and misdiagnosis. It is also important to know that it does not need treatment.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology