UNUSUAL ASSOCIATION OF MENINGIOMA AND PITUITARY HYPOPLASIA IN A PATIENT WITH OSTEOGENESIS IMPERFECTA

Osteogenesis imperfecta (O.I.) is a rare inherited disorder that alters the physiological structure of connective tissue. In some cases, several neurological features have been described. We report.the case.of.a.10years old boy with a knownfamilyhistory of type I OI who suffered from repeated bone fractures, dorsal kyphoscoliosis, bone fragilityand severe stature weight growthdelay. The Brain MRI revealed pituitary hypoplasia, with a fortuitous discovery of a parietal plane meningioma. The association of osteogenesis imperfecta and meningioma, as well as osteogenesis imperfecta and pituitary hypoplasia, have rarely been reported in the literature however, no accessible case of the association of the three entities has been described before within the limits of our research.The objective of our work is to demonstrate a possible pathophysiological link between these three entities.

成骨不全症（Osteogenesis imperfecta, O.I.）是一种罕见的遗传性疾病，可导致结缔组织的生理结构发生改变。部分病例可出现多种神经系统异常表现。本文报告1例存在I型成骨不全症家族史的10岁男性患儿，该患儿反复出现骨折、脊柱胸段后凸侧弯、骨脆性增加，且伴有严重的身高与体重发育迟缓。颅脑磁共振成像（Brain MRI）检查显示垂体发育不全，并偶然发现顶叶平面脑膜瘤。目前文献中关于成骨不全症合并脑膜瘤、成骨不全症合并垂体发育不全的报道较为少见；经我们检索，尚未发现同时累及上述三种病症的病例报告。

本研究旨在阐明这三种病症之间可能存在的病理生理联系。