Neurological Surgery 脳神経外科39巻4号

2011年04月発行

連載 先天奇形シリーズ

（5）脊髄髄膜瘤

著者： 長坂昌登¹

所属機関： ¹あいち小児保健医療総合センター脳神経外科

ページ範囲：P.394 - P.408

Ⅰ．疾患の概説

　脊髄髄膜瘤（myelomeningocele）は，一次神経管形成（primary neurulation）の閉鎖不全に由来する先天奇形（congenital malformation）と考えられている^12,17)．背部の体表に，neural placodeまたはneural plaqueと呼ばれる，開裂した脊髄が露出し，そこから脳脊髄液（以下，髄液）が漏れ出ている．Neural placodeが，その腹側のくも膜下腔に貯留した髄液により体表面よりドーム状に盛り上がっている状態（Fig. 1）を脊髄髄膜瘤，盛り上がらずに開裂した脊髄が体表に露出している状態（Fig. 2）を脊髄披裂（myeloschisis）と呼ぶが，本質的な差はないとされ¹²⁾，一般には脊髄披裂は病変の広がりが大きく，胸腰椎レベルに多く，下肢運動障害が重度である⁴⁷⁾．このような病態は総称として，脊髄髄膜瘤，脊髄披裂，顕在性二分脊椎（spina bifida aperta），開放性二分脊椎，囊胞性二分脊椎（spina bifida cystica），神経管閉鎖障害（neural tube defect）などと呼ばれる．

　脊髄髄膜瘤の発生頻度は，出生前スクリーニングおよび女性の葉酸サプリメント摂取や葉酸処方薬内服の普及などにより，欧米では減少しているが，日本では減少せず，現在は出生10,000人に4～5人程度とされる^22,65)．脊髄髄膜瘤の多くは単発例であるが²²⁾，他の臓器奇形を合併する例^40,51)や染色体異常を伴う例がある．

参考文献

1）Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA：Spina bifida outcome：a 25-year prospective. Pediatr Neurosurg 34：114-120, 2001

2）Caldarelli M, Di Rocco C, Colosimo C Jr, Fariello G, Di Gennaro M：Surgical treatment of late neurological deterioration in children with myelodysplasia. Acta Neurochir (Wien) 137：199-206, 1995

3）Callen AL, Filly RA：Supratentorial abnormalities in the Chiari Ⅱ malformation, Ⅰ：the ventricular “point”. J Ultrasound Med 27：33-38, 2008

4）Callen AL, Stengel JW, Filly RA：Supratentorial abnormalities in the Chiari Ⅱ malformation, Ⅱ：tectal morphologic changes. J Ultrasound Med 28：29-35, 2009

5）Cameron M, Moran P：Prenatal screening and diagnosis of neural tube defects. Prenat Diagn 29：402-411, 2009

6）Charney EB, Weller SC, Sutton LN, Bruce DA, Schut LB：Management of the newborn with myelomeningocele：time for a decision-making process. Pediatrics 75：58-64, 1985

7）Cheek WR, Laurent JP, Cech DA：Operative repair of lumbosacral myelomeningocele. Technical note. J Neurosurg 59：718-722, 1983

8）Cochrane D, Rassekh S, Thiessen P：Functional deterioration following placode untethering in myelomeningocele. Pediatric Neurosurg 28：57-62, 1998

9）Cohen AR, Robinson S：16. Early management of myelomeningocele. Section Ⅳ. Congenital Malformations of the Nervous System. McLone DG (ed)：Pediatric Neurosurgery, Surgery of the developing Nervous System, Fourth edition, W B Saunders Company, Philadelphia, pp241-259, 2001

10）Cuckle H, Murray J：Chapter 16. Antenatal screening for neurologic disorders. Levene MI, Chervenak FA, Whittle M (eds)：Fetal and Neonatal Neurology and Neurosurgery, Churchill Livingstone, London, pp267-273, 2001

11）D’Addario V, Rossi AC, Pinto V, Pintucci A, Di Cagno L：Comparison of six sonographic signs in the prenatal diagnosis of spina bifida. J Perinat Med 36：330-334, 2008

12）Dias MS, McLone DG：21. Myelomeningocele. Albright AL, Pollack IF, Adelson PD (eds)：Principles and Practice of Pediatric Neurosurgery, Second edition, Thieme, New York, pp338-366, 2008

13）Forest CP, Goodman D, Hahn RG：Meningomyelocele：early detection using 3-dimensional ultrasound imaging in the family medical center. J Am Board Fam Med 23：270-272, 2010

14）Hirose S, Farmer DL：Fetal surgery for myelomeningocele. Clin Perinatol 36：431-438, 2009

15）Hosseinpour M, Forghani S：Primary closure of large thoracolumbar myelomeningocele with bilateral latissimus dorsi flaps. J Neurosurg Pediatr 3：331-333, 2009

16）Hunt GM：Open spina bifida：outcome for a complete cohort treated unselectively and followed into adulthood. Dev Med Child Neurol 32：108-118, 1990

17）Hunt GM, Poulton A：Open spina bifida：a complete cohort reviewed 25 years after closure. Dev Med Child Neurol 37：19-29, 1995

18）稲垣隆介：二分脊椎の発生と予防対策．小児外科41：651-657, 2009

19）Kalache KD, Chitty LS：Chapter 17. Antenatal assessment of CNS anomalies, including neural tube defects using abdominal ultrasound, Levene MI, Chervenak FA, Whittle M (eds)：Fetal and Neonatal Neurology and Neurosurgery, 3rd edition, Churchill Livingstone, London, pp275-296, 2001

20）小室広昭：二分脊椎の胎内治療．小児外科41：665-669, 2009

21）近藤厚生，山崎麻美：2. 葉酸について．山崎麻美（編集）：胎児期水頭症．診断と治療ガイドライン，改訂2版，Ⅲ部資料，金芳堂，京都，pp203-210, 2010

22）古庄知己，福嶋義光：二分脊椎の遺伝カウンセリング．小児外科41：730-735, 2009

23）La Marca F, Herman M, Grant JA, McLone DG：Presentation and management of hydromyelia in children with Chiari Type-II malformation. Pediatr Neurosurg 26：57-67, 1997

24）Lee G, Paradiso G, Tator C, Gentili F, Massicotte E, Fehlings M：Surgical management of tethered cord syndrome in adults：indications, techniques, and long-term outcomes in 60 patients. J Neurosurg Spine 4：123-131, 2006

25）Lewis D, Tolosa JE, Kaufmann M, Goodman M, Farrell C, Berghella V：Elective cesarean delivery and long-term motor function or ambulation status in infants with meningomyelocele. Obstet Gynecol 103：469-473, 2004

26）Lien SC, Maher CO, Garton H J. L, Kasten SJ, Murazko KM, Buchman AR：Local and regional flap closure in myelomeningocele repair：a 15-year review. Childs Nerv Syst 26：1091-1095, 2010

27）Lorber J：Results of treatment of myelomeningocele：an analysis of 524 unselected cases, with special reference to possible selection for treatment. Dev Med Child Neurol 13：279-303, 1971

28）Luthy DA, Wardinsky T, Shurtleff DB, Hollenbach KA, Hickok DE, Nyberg DA, Benedetti TJ：Cesarean section before the onset of labor and subsequent motor function in infants with myelomeningocele diagnosed antenatally. N Engl J Med 324：652-666, 1991

29）Marlin AE：Management of hydrocephalus in the patient with myelomeningocele：an argument against third ventriculostomy. Neurosurg Focus 16 (2)：E4, 2004

30）Mataró M, Poca MA, Sahuquillo J, Cuxart A, Iborra J, de la Calzada MD, Junqué C：Cognitive changes after cerebrospinal fluid shunting in young adults with spina bifida and assumed arrested hydrocephalus. J Neurol Neurosurg Psychiatry 68：615-621, 2000

31）McLone DG：Technique for Closure of Myelomeningocele. Child's Brain 6：65-73, 1980

32）宮嶋雅一：二分脊椎に合併する水頭症の治療．小児外科41：682-687, 2009

33）森　惟明：脊髄髄膜瘤に伴う水頭症．伊丹康人，西尾篤人（編集）：二分脊椎症とその周辺疾患，整形外科MOOK No.49，金原出版，東京，pp94-100, 1987

34）森岡隆人，橋口公章，河村忠雄，三原　太，曳野俊治，永田秀昭，岩城　徹，佐々木富男：MRIによる中枢神経系形態異常の胎児期診断．小児の脳神経30：17-24, 2005

35）Morioka T, Hashiguchi K, Mukae N, Sayama T, Sasaki T：Neurosurgical Management of Patients with Lumbosacral Myeloschisis. Neurol Med Chir (Tokoyo) 50：870-876, 2010

36）長坂昌登，田仲　裕，山田博是，中村茂俊：脊髄髄膜瘤児の長期治療成績．小児の脳神経14：95-101, 1989

37）長坂昌登，田仲　裕：脊髄髄膜瘤児に合併した軽度水頭症の取り扱いについて．小児の脳神経18：449-454, 1993

38）長坂昌登：小児神経外科のABC，9. ChiariⅡ型奇形．ミニレクチャー．小児の脳神経25：121-126, 2000

39）長坂昌登：症候性ChiariⅡ型奇形の早期後頭下減圧術．小児の脳神経27：439-443, 2002

40）長坂昌登：囊胞性二分脊椎の診断と治療．小児外科41：675-681, 2009

41）Oakeshott P, Hunt GM, Poulton A, Reid F：Expectaion of life and unexpected death in open spina bifida：a 40-year complete, non-selective, longitudinal cohort study. Dev Med Child Neurol 52：749-753, 2010

42）Oi S, Yamada H, Matsumoto S：Tethered cord syndrome versus low-placed conus medullaris in an over-distended spinal cord following initial repair for myelodysplasia. Childs Nerv Syst 6：264-269, 1990

43）大井静雄：9. 癒合不全．松本　悟，大井静雄（編）：臨床小児脳神経外科学，医学書院，東京，pp234-265, 1992

44）Oi S, Honda Y, Hidaka M, Sato O, Matsumoto S：Intrauterine high-resolution magnetic resonance imaging in fetal hydrocephalus and prenatal estimation of postnatal outcomes with “perspective classification”. J Neurosurg 88：685-694, 1998

45）大井静雄：3.1顕在性二分脊椎．阿部　弘，菊池晴彦，田中隆一，坪川孝志，平川公義，松本　悟（編）：脳神経外科疾患の手術と適応─Ⅱ，朝倉書店，東京，pp521-542, 2003

46）沖　高司：二分脊椎のリハビリテーション．小児外科41：725-729, 2009

47）Park TS：16. Myelomeningocele. Albright AL, Pollack IF, Adelson PD (eds)：Principles and Practice of Pediatric Neurosurgery, Thieme, New York, pp291-320, 1999

48）Piatt JH：Syringomyelia complicating myelomeningoocele：review of the evidence. J Neurosurg (Pediatrics 2) 100：101-109, 2004

49）Pollack IF, Pang D, Albright AL, Krieger D：Outcome following hindbrain decompression of symptomatic Chiari malfolmation in children previously treated with myelomeningocele closure and shunts. J Neurosurg 77：881-888, 1992

50）Pollack IF, Kinnunen D, Albright AL：The effect of early craniocervical decompression on functional outcome in neonates and young infants with myelodysplasia and symptomatic ChiariⅡmalformatoins：Results from a prospective series. Neurosurgery 38：703-710, 1996

51）Reigel DH：2. Spina Bifida. Pediatric Neurosurgery, Surgery of the Developing Nervous System, Grune ＆ Stratton Inc, New York, pp23-47, 1982

52）Reigel DH：17. Myelomeningocele Repair. Section Ⅳ. Congenital Malformations of the Nervous System. McLone DG (ed)：Pediatric Neurosurgery, Surgery of the developing Nervous System, Fourth edition, WB Saunders Company, Philadelphia, pp261-265, 2001

53）坂本博昭：脊髄髄膜瘤．山崎麻美（編集）胎児期水頭症　診断と治療ガイドライン，改訂2版．金芳堂，京都，pp104-119, 2010

54）Saleem SN, Said AH, Abdel-Raouf M, El-Kattan EA, Zaki MS, Madkour N, Shokry M：Fetal MRI in the evaluation of fetus reffered for sonographically suspected neural tube defects (NTDs)：impact on diagnosis and management decision. Neuroradiology 51：761-772, 2009

55）Sarwark J, Weber D, Gabrieli A, McLone D, Dias L：Tethered cord syndrome in low motor level children with myelomeningocele. Pediatr Neurosurg 25：295-301, 1996

56）佐藤　潔，安本幸正，新井　一，久留　裕：11．脳形成不全─Chiari奇形，松本　悟，大井静雄（編集）：臨床小児脳神経外科学，医学書院，東京，pp284-309, 1992

57）Selber P, Dias L：Sacral-level myelomeningocele：Long-term outcome in adults. J Pediatr Orthop 18：423-427, 1998

58）Sharrard WJW, Zachary RB, Lober J, Bruce AM：A controlled trial of immediate and delayed closure of spina bifida cystica. Arch Dis Child 38：18-22, 1963

59）Soleau S, Tubbs RS, Oakes WJ：14. Chiari Malformations. Albright AL, Pollack IF, Adelson PD (eds)：Principles and Practice of Pediatric Neurosurgery, Second edition, Thieme, New York, Stuttgart, pp217-232, 2008

60）Talamonti G, D’Aliberti G, Collice M：Myelomeningocele：long-term neurosurgical treatment and follow-up in 202 patients. J Neurosurg (5 Suppl Pediatrics) 107：368-386, 2007

61）Tamaki N, Shirataki K, Kojima N, Shouse Y, Matsumoto S：Tethered cord syndrome of delayed onset following repair of myelomeningocele. J Neurosurg 69：393-398, 1988

62）田仲　裕，山田博是，中村茂俊，田島正孝，景山直樹：脊髄髄膜瘤の外科的治療．Neurol Med Chir (Tokyo) 22：983-988, 1982

63）山田博是：囊胞性二分脊椎の臨床．山田博是（編）：二分脊椎の臨床，医学書院，東京，pp19-37, 1985

64）山田博是：脊髄髄膜瘤に伴う水頭症．山田博是（編）：二分脊椎の臨床，医学書院，東京，pp59-73, 1985

65）山崎麻美，埜中正博，寺元千佳，伴　千秋，夫　律子：二分脊椎の胎児診断．小児外科41：658-664, 2009