engleski

Central Precocious Puberty in a Girl with Duplication of Hypophysis Treated with LHRH Agonists

Duplication of hypophysis is an extremely rare malformation with only eighteen previous case reports in the literature. Until now, only four patients have lived to the age of adolescence. The developmental disorder leading to pituitary duplication and diffuse thickening of the hypothalamus may alter secretion of luteinizing-hormone-releasing hormone as a result of derangement and a failure of regulation. We present a 12-year-old girl with duplication of hypophysis and the associated cleft palate and tongue, hypertelorism, dysmorphic facial features, delayed developmental milestones and central precociuos puberty. MRI revealed paired infundibula extending to two small pituitary glands, a midline hypothalamic mass and anomallies of the basilar artery (duplication of M1 segment of the left a. cerebri media and fenestration of ultrashort segment of basilar arthery). At the age of five she presented with breast development (Tanner 2). At the age of eight she achieved menarche. Examination revealed a height of 136 cm (95th percentile) and a weight of 34 kg (95th percentile), a growth velocity above 95th percentile, bone age of a 10-year- old, breasts Tanner 3 and pubic hair Tanner 2. Repeated measurements of folicle-stimulating (FSH) and luteinizing hormones (LH) and estradiol (E2) did not reveal significantly elevated levels (LH 0.12 – 1.68 IU/l, FSH 1.88 – 3.41 IU/l, E2 30 – 70 pmol/l, normal values for girls, age 5 – 9 years: LH < 2 IU/l, FSH < 4 IU/ l, E2 5-60 pmol/l). Laboratory assessment of the function of adenohypophysis and its target glands and the neurohypophysis were within the normal range. The treatment with LHRH agonists (Decapeptyl CR, 3.75 mg of triptorelin, once a month intramusculary) was initiated. The function of hypothalamo- pituitary-gonadal axis was completely supressed after 3 months of therapy and the progression of breast development and menstrual bleeding successfully ceased. Having received the treatment for 3.5 years, the patient has a normal growth velocity and a normal bone maturation now, and only pubic hair development progressed to Tanner stage 3. Among four previously reported patients with duplication of hypophysis who have reached the age of puberty, two patients had delayed pubertal development, one had precocious puberty and in one patient the data regarding puberty were not reported. Our patient is the second one reported in the literature with precociuos puberty and duplication of hypophysis and the first one successfully treated with LHRH agonists.

hypophysis; duplication; LHRH; precocious puberty

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