Neurological Surgery 脳神経外科39巻10号

2011年10月発行

連載 先天奇形シリーズ

（11）非症候群性頭蓋骨縫合早期癒合症

著者： 坂本博昭¹

所属機関： ¹大阪市立総合医療センター小児医療センター，小児脳神経外科

ページ範囲：P.1005 - P.1020

Ⅰ．はじめに

　頭囲の標準発育曲線で示されるように，頭蓋骨は生まれてから2，3歳までの脳容積の増大に対応して急速に成長する．この骨の成長部が頭蓋骨同士を結合する頭蓋縫合にあたる．前頭縫合は神経堤に由来し生後2歳頃に閉鎖するが，中胚葉由来の冠状縫合，矢状縫合，人字縫合，鱗状縫合は成人期まで開存する．頭蓋縫合が早期に癒合する頭蓋骨縫合早期癒合症（頭蓋骨癒合症，craniosynostosis）では，①早期癒合を来した縫合部を中心に頭蓋の成長が妨げられて頭蓋の変形を来す，②出生後の脳の急速な容積増加につれて頭蓋容積が増加せず，相対的な狭小化による慢性の頭蓋内圧亢進を起こして，脳や視神経の障害を起こす． 同義語として狭頭症（craniostenosis）があるが，これは早期癒合の結果生じる現象をさす．

　頭蓋骨縫合早期癒合症は症候群性（syndromic craniosynostosis）と非症候群性（non-syndromic（isolated）craniosynostosis）とに分けられる³¹⁾．前者は頭蓋に加え，上顎骨の低形成や指などの骨の形成異常を伴い，遺伝子変異が原因とされ家族性に発生する例がある．また，早期癒合は複数の縫合で来しやすいため頭蓋の変形や狭小化が強く，年少時期での治療が必要となる．ここで述べる非症候群性の病変は頭蓋縫合にとどまり，頭蓋骨以外には先天性の骨病変を伴わないが，症候群性よりも発生頻度が高い．

参考文献

1）Adamo MA, Pollack IF：A single-center experience with symptomatic postoperative calvarial growth restriction after extended strip craniectomy for sagittal craniosynostosis. J Neurosurg Pediatr 5：131-135, 2010

2）Agrawal D, Steinbok P, Cochrane DD：Reformation of the sagittal suture following surgery for isolated sagittal craniosynostosis. J Neurosurg (2 Suppl) 105：115-117, 2006

3）新井　一，佐藤　潔：Ⅲ Modified techniques. 先天奇形．頭蓋顔面奇形．Sagittal synostosisに対するtotal cranial reconstruction. pp314-321 （山浦　晶，森竹浩三（編）：小児脳神経の外科，医学書院，東京，1998）

4）Arnaud E, Meneses P, Lajeunie E, Thorne JA, Marchac D, Renier D：Postoperative mental and morphological outcome for nonsyndromic brachycephaly. Plast Reconstr Surg 110：6-13, 2002

5）Becker DB, Petersen JD, Kane AA, Cradock MM, Pilgram TK, Marsh JL：Speech, cognitive, and behavioral outcomes in nonsyndromic craniosynostosis. Plast Reconstr Surg 116：400-407, 2005

6）Bellew M, Liddington M, Chumas P, Russell J：Preoperative and postoperative developmental attainment in patients with sagittal synostosis：5-year follow-up. J Neurosurg Pediatr 7：121-126, 2011

7）Chieffo D, Tamburrini G, Massimi L, Di Giovanni S, Giansanti C, Caldarelli M, Di Rocco C：Long-term neuropsychological development in single-suture craniosynostosis treated early. J Neurosurg Pediatr 5：232-237, 2010

8）Cohen MM Jr：Epidemiology of craniosynostosis. pp112-118 (Cohen MM Jr, MacLean RE：Craniosynsotosis 2nd ed, Chapter 10, Oxford University Press, New York, 2000)

9）Da Costa AC, Walters I, Savarirayan R, Anderson VA, Wrennall JA, Meara JG：Intellectual outcomes in children and adolescents with syndromic and nonsyndromic craniosynostosis. Plast Reconstr Surg 118：175-183, 2006

10）Eppley BL, Morales L, Wood R, Pensler J, Goldstein J, Havlik RJ, Habal M, Losken A, Williams JK, Burstein F, Rozzelle AA, Sadove AM：Resorbable PLLA-PGA plate and screw fixation in pediatric craniofacial surgery：clinical experience in 1883 patients. Plast Reconstr Surg 114：850-857, 2004

11）Florisson JM, van Veelen ML, Bannink N, van Adrichem LN, van der Meulen JJ, Bartels MC, Mathijssen IM：Papilledema in isolated single-suture craniosynostosis：prevalence and predictive factors. J Craniofac Surg 21：20-24, 2010

12）Foster KA, Frim DM, McKinnon M：Recurrence of synostosis following surgical repair of craniosynostosis. Plast Reconstr Surg 121：70e-76e, 2008

13）Gault DT, Renier D, Marchac D, Jones BM：Intracranial pressure and intracranial volume in children with craniosynostosis. Plast Reconstr Surg 90：377-381, 1992

14）Hankinson TC, Fontana EJ, Anderson RC, Feldstein NA：Surgical treatment of single-suture craniosynostosis：an argument for quantitative methods to evaluate cosmetic outcomes. J Neurosurg Pediatr 6：193-197, 2010

15）Imai K, Komune H, Toda C, Nomachi T, Enoki E, Sakamoto H, Kitano S, Hatoko M, Fujimoto T：Cranial remodeling to treat craniosynostosis by gradual distraction using a new device. J Neurosurg 96：654-659, 2002

16）Jane JA, Edgerton MT, Futrell JW, Park TS：Immediate correction of sagittal synostosis. J Neurosurg 49：705-710, 1978

17）Jentink J, Loane MA, Dolk H, Barisic I, Garne E, Morris JK, de Jong-van den Berg LT；EUROCAT Antiepileptic Study Working Group：Valproic acid monotherapy in pregnancy and major congenital malformations. N Engl J Med 362：2185-2193, 2010

18）Jimenez DF, Barone CM：Multiple-suture nonsyndromic craniosynostosis：early and effective management using endoscopic techniques. J Neurosurg Pediatrics 5：223-231, 2010

19）Kapp-Simon KA, Speltz ML, Cunningham ML, Patel PK, Tomita T：Neurodevelopment of children with single suture craniosynostosis：a review. Childs Nerv Syst 23：269-281, 2007

20）Koizumi T, Komuro Y, Hashizume K, Yanai A：Cephalic index of Japanese children with normal brain development. J Craniofac Surg 21：1434-1437, 2010

21）Lauritzen CG, Davis C, Ivarsson A, Sanger C, Hewitt TD：The evolving role of springs in craniofacial surgery：the first 100 clinical cases. Plast Reconstr Surg 121：545-554, 2008

22）Marchac D, Renier D, Broumand S：Timing of treatment for craniosynostosis and facio-craniosynostosis：a 20-year experience. Br J Plast Surg 47：211-222, 1994

23）Marucci DD, Johnston CP, Anslow P, Jayamohan J, Richards PG, Wilkie AO, Wall SA：Implications of a vertex bulge following modified strip craniectomy for sagittal synostosis. Plast Reconstr Surg 122：217-224, 2008

24）Mathijssen I, Arnaud E, Lajeunie E, Marchac D, Renier D：Postoperative cognitive outcome for synostotic frontal plagiocephaly. J Neurosurg (1 Suppl Pediatrics) 105：16-20, 2006

25）Mendonca DA, White N, West E, Dover S, Solanki G, Nishikawa H：Is there a relationship between the severity of metopic synostosis and speech and language impairments? J Craniofac Surg 20：85-89, 2009

26）Paige KT, Cohen SR, Simms C, Burstein FD, Hudgins R, Boydston W：Predicting the risk of reoperation in metopic synostosis：a quantitative CT scan analysis. Ann Plast Surg 59：6-13, 2007

27）Pearson GD, Havlik RJ, Eppley B, Nykiel M, Sadove AM：Craniosynostosis：a single institution's outcome assessment from surgical reconstruction. J Craniofac Surg 19：65-71, 2008

28）Persing JA, Jane JA：Craniosynostosis. pp995-1011 (Youmans J：Neurological Surgery 4th ed. WB Saunders, Philadelphia, 1996)

29）Renier D, Lajeunie E, Arnaud E, Marchac D：Management of craniosynostoses. Childs Nerv Syst 16：645-658, 2000

30）坂本博昭，北野昌平：頭蓋縫合早期癒合症に対する手術．脳外速報12：1165-1172, 2004

31）坂本博昭：第5章 先天性疾患，§7 頭蓋骨縫合早期癒合症．pp405-431（横田　晃（監修），山崎麻美，坂本博昭（編）：小児脳神経外科学，金芳堂，京都，2009）

32）坂本博昭，松阪康弘：Ⅴ．小児，7．頭蓋骨縫合早期癒合症の手術．骨延長法による頭蓋骨形成の適応と限界は？．pp375-382（宮本　亨，新井　一，鈴木倫保，渋井壮一郎，中瀬裕之（編）：EBM脳神経外科疾患の治療，中外医学社，東京，2010）

33）Selber JC, Brooks C, Kurichi JE, Temmen T, Sonnad SS, Whitaker LA：Long-term results following fronto-orbital reconstruction in nonsyndromic unicoronal synostosis. Plast Reconstr Surg 121：251e-260e, 2008

34）Seruya M, Oh AK, Boyajian MJ, Posnick JC, Myseros JS, Yaun AL, Keating RF：Long-term outcomes of primary craniofacial reconstruction for craniosynostosis：a 12-year experience. Plast Reconstr Surg 127：2397-2406, 2011

35）Sgouros S, Hockley AD, Goldin JH, Wake MJ, Natarajan K：Intracranial volume change in craniosynostosis. J Neurosurg 91：617-625, 1999

36）Shimoji T, Shimabukuro S, Sugama S, Ochiai Y：Mild trigonocephaly with clinical symptoms：analysis of surgical results in 65 patients. Childs Nerv Syst 18：215-224, 2002

37）Starr JR, Kapp-Simon KA, Cloonan YK, Collett BR, Cradock MM, Buono L, Cunningham ML, Speltz ML：Presurgical and postsurgical assessment of the neurodevelopment of infants with single-suture craniosynostosis：comparison with controls. J Neurosurg (2 Suppl) 107：103-110, 2007

38）Starr JR, Lin HJ, Ruiz-Correa S, Cunningham ML, Ellenbogen RG, Collett BR, Kapp-Simon KA, Speltz ML：Little evidence of association between severity of trigonocephaly and cognitive development in infants with single-suture metopic synostosis. Neurosurgery 67：408-416, 2010

39）Tamburrini G, Caldarelli M, Massimi L, Santini P, Di Rocco C：Intracranial pressure monitoring in children with single suture and complex craniosynostosis：a review. Childs Nerv Syst 21：913-921, 2005

40）van der Meulen J, van der Vlugt J, Okkerse J, Hofman B：Early beaten-copper pattern：its long-term effect on intelligence quotients in 95 children with craniosynostosis. J Neurosurg Pediatr 1：25-30, 2008

41）Wagner JD, Cohen SR, Maher H, Dauser RC, Newman MH：Critical analysis of results of craniofacial surgery for nonsyndromic bicoronal synostosis. J Craniofac Surg 6：32-39, 1995

42）Whitaker LA, Bartlett SP, Schut L, Bruce D：Craniosynostosis：an analysis of the timing, treatment, and complications in 164 consecutive patients. Plast Reconstr Surg 80：195-212, 1987

43）Williams JK, Cohen SR, Burstein FD, Hudgins R, Boydston W, Simms C：A longitudinal, statistical study of reoperation rates in craniosynostosis. Plast Reconstr Surg 100：305-310, 1997

44）Winston KR, Ketch LL, Dowlati D：Cranial vault expansion by distraction osteogenesis. J Neurosurg Pediatr 7：351-361, 2011

45）Yamada A, Imai K, Nomachi T, Fujimoto T, Sakamoto H, Kitano S：Cranial distraction for plagiocephaly：quantitative morphologic analyses of cranium using three-dimensional computed tomography and a life-size model. J Craniofac Surg 16：688-693, 2005