Mitsuhara Takafumi, Yamaguchi Satoshi, Takeda Masaaki, Kurisu Kaoru
Department of Neurosurgery, Graduate School of Biomedical Sciences, Hiroshima University, 1-2-3 Kasumi, Minami.ku, Hiroshima 734-8551, Japan.
Surg Neurol Int. 2014 Jan 20;5:6. doi: 10.4103/2152-7806.125546. eCollection 2014.
Idiopathic hemorrhage in a syrinx is a rare entity known as Gowers' intrasyringeal hemorrhage. Bleeding confined to the syrinx cavity causes severe, sometimes acute, neurological deficits. We report a case of intrasyringeal hemorrhage into a preexisting lumbosacral syrinx associated with Chiari type I malformation.
A 39-year-old female with Noonan syndrome underwent foramen magnum decompression and a cervical syrinx-subarachnoid shunt for Chiari type I malformation-associated syringomyelia 7 years ago. She presented progressive gait deterioration and acute urinary dysfunction, indicating conus medullaris syndrome. Initial magnetic resonance imaging revealed massive hemorrhage in the intrasyringeal cavity of the conus medullaris. The patient underwent surgical removal of the intrasyringeal hematoma and her neurological symptoms improved postoperatively.
Although Gowers' intrasyringeal hemorrhage is rare, this entity should be taken into consideration in patients with syringomyelia showing acute neurological deterioration.
脊髓空洞症特发性出血是一种罕见的情况，称为高尔斯脊髓内出血。局限于脊髓空洞腔内的出血会导致严重的，有时是急性的神经功能缺损。我们报告一例先前存在的腰骶部脊髓空洞症伴I型Chiari畸形并发脊髓内出血的病例。
一名39岁患有努南综合征的女性7年前因I型Chiari畸形相关的脊髓空洞症接受了枕骨大孔减压术和颈段脊髓空洞-蛛网膜下腔分流术。她出现进行性步态恶化和急性排尿功能障碍，提示圆锥马尾综合征。最初的磁共振成像显示圆锥马尾脊髓空洞腔内大量出血。患者接受了脊髓内血肿手术清除，术后神经症状改善。
尽管高尔斯脊髓内出血罕见，但在表现为急性神经功能恶化的脊髓空洞症患者中应考虑到这种情况。
