Neurological Surgery 脳神経外科39巻5号

2011年05月発行

連載 先天奇形シリーズ

（6）潜在性二分脊椎（脊髄脂肪腫を除く）--先天性皮膚洞，緊張終糸，神経腸管囊胞，分離脊髄奇形，尾部退行症候群など

著者： 重田裕明¹

所属機関： ¹長野県立こども病院脳神経外科

ページ範囲：P.513 - P.527

Ⅰ．二分脊椎の分類と発生機序

1．二分脊椎の分類

　二分脊椎spina bifidaはその外表所見から，顕在性と潜在性，あるいは囊胞性などに分類される．顕在性には神経組織が皮膚に覆われずに露出している脊髄髄膜瘤（脊髄裂）と皮膚で覆われている髄膜瘤が含まれ，囊胞性とほぼ同義で使用されることも多い．それ以外の瘤を形成しないものは潜在性に分類され，皮膚に覆われる脊髄脂肪腫，緊張終糸，先天性皮膚洞などが含まれる．また潜在性という用語は，閉鎖性（神経組織が皮膚に覆われて露出していない）と同義に使用される場合もある．潜在性二分脊椎spina bifida occultaは，本来は椎骨の癒合不全を指しており，神経系の異常も認める場合には潜在性脊椎閉鎖不全症occult spinal dysraphismの用語が適切である．本稿では，occult spinal dysraphismに分類される疾患のうち脊髄脂肪腫以外の病変について概説する．

参考文献

1）Agnoli AL, Laun A, Schonmayr R：Enterogenous intraspinal cysts. J Neurosurg 61：834-840, 1984

2）Barkovich AJ：Congenital anomalies of the spine. pp704-772 (Barkovich AJ：Pediatric Neuroimaging 4th ed, Chapter 9, Lippincott Williams ＆Wilkins, Philadelphia, 2005)

3）Barson AJ：The vertebral level of termination of the spinal cord during normal and abnormal development. J Anat 106：489-497, 1970

4）Choi SH, McComb JG：Long-term outcome of terminal myelocystocele patients. Pediatr Neurosurg 32：86-91, 2000

5）de Oliveira RS, Cinalli G, Roujeau T, Saint-Rose C, Pierre-Kahn, Zerah M：Neurenteric cysts in children：16 consecutive cases and review of the literature. J Neurosurg (6 Supll Pediatrics) 103：512-523, 2005

6）Dias MS, McLone DG：Normal and abnormal early development of the nervous system. pp31-71 (McLone DG：Pediatric Neurosurgery. Surgery of the Developing, Chapter 4, Nervous System 4th ed, WB Saunders, Philadelphia, 2001)

7）Ersahin Y, Mutluer S, Kocaman S, Demirtas E：Split spinal cord malformations in children. J Neurosurg 88：57-65, 1998

8）Fabiano AJ, Khan MF, Rozzelle CJ, Li V：Preoperative predictors for improvement after surgical untethering in occult tight filum terminale syndrome. Pediatr Neurosurg 45：256-261, 2009

9）Gaskill SJ, Marlin AE：The Currariono triad：Its importance in pediatric neurosurgery. Pediatr Neurosurg 25：143-146, 1996

10）Gupta DK, Mahapatra AK：Terminal myelocystoceles：a series of 17 cases. J Neurosurg (Pediatrics 4) 103：344-352, 2005

11）浜田秀雄，林　央周，梅村公子，黒崎邦和，栗本昌紀，Alain Pierre-Kahn，遠藤俊郎：割髄症の治療転帰に関する検討．小児の脳神経32：281-284, 2007

12）Iskandar BJ, Oakes WJ, McLaughlin C, Osumi AK, Rien RD：Terminal syringohydromyelia and occult spinal dysraphism. J Neurosurg 81：513-519, 1994

13）Iskandar BJ, Oakes WJ：Anomalies of the spine and spinal cord. pp307-324 (McLone DG：Pediatric Neurosurgery. Surgery of the Developing Nervous System 4th ed, Chapter 23, W B Saunders, Philadelphia, 2001)

14）James HE, Lubinsky G：Terminal myelocystocele. J Neurosurg (5 Suppl Pediatrics) 103：443-445, 2005

15）Keating RF, Multani J, Cogen PH：Occult spinal dysraphism and the tethered spinal cord. pp3257-3283 (Winn RH：Youmans Neurological Surgery 5th ed, Chapter 206, WB Saunders, Philadelphia, 2004)

16）Kennedy PR：New data on diastematomyelia. J Neurosurg 51：355-261, 1979

17）Kriss VM, Kriss TC, Warf BC：Dorsal tethering bands of the meningocele manqué. AJR Am J Roentgenol 167：1293-1295, 1996

18）Martinez-Lage JF, Pérez-Espejo MA, Tortosa JG, de San Pedro JR, Ruiz-Espejo AM：Hydrocephalus in intraspinal dermoids and dermal sinuses：the spectrum of an uncommon association in children. Childs Nerv Syst 22：698-703, 2006

19）西本　博，栗原　淳，岸本宏志，竹澤陽子：meningocele manquéの手術ならびに病理学的所見．小児の脳神経31：298-303, 2006

20）Pang D：Split cord malformation：PartⅠ：A unified theory of embryogenesis for double spinal cord malformations. Neurosurgery 31：451-480, 1992

21）Pang D：Split cord malformation：PartⅡ：Clinical syndrome. Neurosurgery 31：481-500, 1992

22）Pang D：Sacral agenesis and caudal spinal cord malformations. Neurosurgery 32：755-779, 1993

23）Rajpal S, Salamat MS, Tubbs RS, Kelly DR, Oakes WJ, Iskandar BJ：Tethered tracts in spina bifida occulta：revisiting an established nomenclature. J Neurosurg Spine 7：315-322, 2007

24）Ramnarayan R, Dominic A, Alapatt J, Buxton N：Congenital spinal dermal sinuses：poor awareness leads to delayed treatment. Childs Nerv Syst 22：1220-1224, 2006

25）Reimann AF, Anson BJ：Vertebral level of termination of the spinal cord with a report of a case of sacral cord. Anat Rec 88：127-138, 1944

26）Selden NR：Occult tethered cord syndrome：the case for surgery. J Neurosurg 104：302-304, 2006

27）Selden NR Nixon RR, Skoog SR, Lashley DB：Minimal tethered cord syndrome associated with thickening of the terminal filum. J Neurosurg (3 Suppl Pediatrics) 105：214-218, 2006

28）Steinbok P, Garton HJL, Gupta N：Occult tethered cord syndrome：a survey of practice patterns. J Neurosurg (5 Suppl Pediatrics) 104：309-313, 2006

29）Steinbok P, Kariyattil R, MacNeily AE：Comparison of section of filum terminale and non-neurosurgical management for urinary incontinence in patients with normal conus position and possible occult tethered cord syndrome. Neurosurgery 61：550-556, 2007

30）Tani S, Yamada S, Kninghton RS：Extensibility of the lumbar and sacal cord. J Neurosurg 66：116-123, 1987

31）Tani S, Okuda Y, Abe T：Surgical strategy for anterior sacral meningocele. Two case reports. Neurol Med Chir (Tokyo) 43：204-209, 2003

32）Warder DE, Oakes WJ：Tethered cord syndrome and the conus in a normal position. Neurosurgery 33：374-378, 1993

33）Yamada S, Zinke DE, Sanders D：Pathophysiology of tethered cord syndrome. J Neurosurg 54：494-503, 1981

34）Yamada S, Won DJ：Occult tethered cord. J Neurosurg (Pediatrics) 106：411-413, 2007

35）Zuccaro G：Split spinal cord malformation. Childs Nerv Syst 19：104-105, 2003