Del Maestro Mattia, De Paulis Danilo, Ricci Alessandro, Di Cola Francesco, Galzio Renato
Department of Life, Health & Environmental Sciences (Me.S.V.A.), University of L'Aquila, L'Aquila, Italy.
Childs Nerv Syst. 2014 Oct;30(10):1749-52. doi: 10.1007/s00381-014-2457-9. Epub 2014 Jun 5.
Syringomyelia can be defined as a degenerative, progressive and chronic spinal cord disease. Its association with tumors of the posterior cranial fossa (PCF) is a rare condition.
The authors report a rare case of syringobulbia consequent to a meningioma originating from PCF in a 17-year-old female, discussing the pathogenetic mechanism of development and the resolution of the syrinx cavity after surgical procedure.
The postoperative period was uneventful without complications. At 6-month follow-up, MRI revealed complete tumor removal with resolution of the syrinx cavity.
In cases of syringomyelia and tonsillar herniation associated with PCF meningioma, the tumor resection allows to eliminate the mass effect and increases the size of the posterior fossa with the progressive ascent of the cerebellar tonsils and the consequent reduction of their downward movement with systolic pulsation. The re-establishment of a normal anatomical condition led to the gradual disappearance of syrinx and hydrocephalus.
脊髓空洞症可被定义为一种退行性、进行性慢性脊髓疾病。它与后颅窝（PCF）肿瘤的关联是一种罕见情况。
作者报告了一例17岁女性因起源于PCF的脑膜瘤导致延髓空洞症的罕见病例，讨论了其发病机制以及手术后脊髓空洞腔的消退情况。
术后过程顺利，无并发症。在6个月的随访中，MRI显示肿瘤完全切除，脊髓空洞腔消失。
在与PCF脑膜瘤相关的脊髓空洞症和扁桃体疝病例中，肿瘤切除可消除占位效应，并随着小脑扁桃体的逐渐上升以及其随收缩期搏动向下运动的减少而增加后颅窝的大小。正常解剖状态的重建导致脊髓空洞症和脑积水逐渐消失。
