Nakasu S, Nakasu Y, Matsuda I, Handa J
No To Shinkei. 1981 Feb;33(2):181-5.
Calvarial bone defect associated with a hugh neurofibroma in the region of the lambdoid suture was reported in a 42-year-old man with v. Recklinghausen's neurofibromatosis. An enlarging occipital tumor was first noted at age 4 similar to or approximately 5, and an operation was performed at age 12. Lambda defect was noted at that time, but the operative diagnosis was, apparently, "meningocele". Available literature was reviewed, and the significance of calvarial bone defect in the region of the lambdoid suture as a manifestation of v. Recklinghausen's disease was stressed. Lambda defect witn no sclerosing margin, particularly if associated with hypoplasia of the ipsilateral mastoid cells, has been known to represent the primary nature of the bone defect due to mesodermal dysplasia. In the present case, plain radiographs of the skull revealed abnormal sclerotic change around the bone defect. It is suggested that such an osteoblastic change may well indicate the secondary nature of the bone defect due to the presence of overlying neurogenic tumor.
一名患有冯·雷克林霍增氏神经纤维瘤病的42岁男性，被报道存在与枕骨粗隆缝区域巨大神经纤维瘤相关的颅骨缺损。一个逐渐增大的枕部肿瘤最初在4岁时被发现，类似或大约5岁时，12岁时进行了手术。当时发现了枕骨粗隆缺损，但手术诊断显然是“脑脊膜膨出”。查阅了现有文献，并强调了枕骨粗隆缝区域颅骨缺损作为冯·雷克林霍增氏病表现的意义。已知没有硬化边缘的枕骨粗隆缺损，特别是如果与同侧乳突细胞发育不全相关，代表了由于中胚层发育异常导致的骨缺损的原发性。在本病例中，颅骨平片显示骨缺损周围有异常的硬化改变。提示这种成骨细胞改变很可能表明由于上方神经源性肿瘤的存在导致骨缺损的继发性。
