DVOSTRUKA HIPOFIZA

Nevena Krnić, Katja Dumić, Marko Radoš, Nataša Rojnić Putarek, Andrija Stanimirović

Sažetak. Dvostruka hipofiza rijetka je malformacija. Do sada je u svijetu opisana u četrdesetak bolesnika, a zbog pridruženih anomalija često je povezana s letalnim ishodom. Samo 7 bolesnica doživjelo je pubertet, koji je u tri od njih započeo preuranjeno. Prikazujemo djevojčicu s dvostrukom hipofizom, pravim preuranjenim pubertetom i pridruženim malformacijama središnje linije. Uz već opisanu fenestraciju bazilarne arterije imala je i duplikaciju M1-segmenta lijeve medijalne cerebralne arterije koja do sada nije nađena u ovih bolesnika. Naša je bolesnica jedna od četiri u kojih je započeta terapija preuranjenog puberteta agonistima »releasing hormona« za gonadotropine (GnRH), no jedina u koje je ta terapija do sada i uspješno završena.

Summary. Duplication of the pituitary gland is a rare malformation. To the best of our knowledge, only 40 cases throughout the world have been reported previously. Due to associated severe developmental anomalies lots of these cases were diagnosed at autopsy. Only 7 patients survived beyond puberty, however, it is important to stress that none of them had major brain anomalies. We present the case of a girl with duplication of the pituitary gland (DPG), central precocious puberty and associated midline defects. Among fenestration of basilar artery, previously described in the literature, we also found, for the first time, a duplication of the M1-segment of the left median cerebral artery. Our patient is one of the 4 DPG patients with associated precocious puberty treated with gonadotropine releasing hormone agonists (GnRH), but the only one in whom this treatment was finished successfully.