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Kinder- und Jugendmedizin 2015; 15(02): 102-109
DOI: 10.1055/s-0038-1629263
Mund-, Kiefer- und Gesichtschirurgie
Schattauer GmbH

Diagnostik und Therapie isolierter Kraniosynostosen

Diagnosis and therapy of isolated craniosynostosis
C. Freudlsperger
1   Kraniofaziales Zentrum, Klinik und Poliklinik für Mund-, Kiefer- und Gesichtschirurgie, Universitätsklinikum Heidelberg
,
J. Hoffmann
1   Kraniofaziales Zentrum, Klinik und Poliklinik für Mund-, Kiefer- und Gesichtschirurgie, Universitätsklinikum Heidelberg
,
M. Engel
1   Kraniofaziales Zentrum, Klinik und Poliklinik für Mund-, Kiefer- und Gesichtschirurgie, Universitätsklinikum Heidelberg
› Author Affiliations
Further Information

Publication History

Eingereicht am: 01 December 2014

angenommen am: 04 December 2014

Publication Date:
31 January 2018 (online)

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Zusammenfassung

Kraniosynostosen gehören mit einer Ge -burtsprävalenz von 1 : 2500 Geburten zu den seltenen Fehlbildungen und bezeichnen den vorzeitigen Verschluss einer oder mehrerer Schädelnähte. Die überwiegende Mehrzahl der Kraniosynostosen stellen monosuturale, isolierte (= nicht syndromale) Formen dar, bei denen nur eine der sechs Schädelnähte betroffen ist, und die in der Regel mit keiner weiteren Fehlbildung assoziiert sind. Die frühzeitige Nahtfusion bedingt ein für die jeweils betroffene Schädelnaht charakteristisches klinisches Erscheinungsbild, welches wiederum Rückschlüsse auf die betroffene Schädelnaht zulässt.

Die Standarddiagnostik bei monosuturalen, isolierten Kraniosynostosen umfasst die eingehende klinische Untersuchung mit kephalometrischen Messungen und eine Sonografie der Schädelnähte. Der Einsatz ionisierender Strahlung (konventionelles Schädelröntgen, Computertomografie) ebenso wie die MRTUntersuchung in Sedierung sollten vermieden werden und nur in Ausnahmefällen und bei speziellen Fragestellungen in Betracht gezogen werden. Die operative Korrektur einer monosuturalen, isolierten Kraniosynostose erfolgt aufgrund funktioneller, psychosozialer, aber auch ästhetischer Indikationen. Die chirurgischen Techniken umfassen an das Alter der Patienten angepasste, standardisierte Operationsverfahren mit guten, vorhersagbaren Behandlungsergebnissen.

Summary

Craniosynostosis belongs to the rare diseases with a prevalence of approximately one in 2500 live births and derives from a premature fusion of one or more of the cranial sutures. The most common types of craniosynostosis are isolated (= non-syndromal) forms where only a single suture is involved and which are not associated with further malformation. The characteristic clinical manifestations of single-suture craniosynostosis are pathognomonic for the affected suture and usually lead the experienced craniofacial surgeon to an accurate diagnosis.

The standard diagnostics include a throughout clinical examination with cephalometric measurements and a sonography of the cranial sutures. With respect to the biological effects of ionizing radiation and risks of sedation especially in infants, preoperative imaging should be reduced to a minimum and should be reserved for complex craniofacial anomalies. The objective of the surgical treatment of isolated craniosynostosis using standardized surgical techniques, is to induce normal brain development, to prevent increased intracranial pressure (ICP) and to achieve an acceptable cranial morphology.

Schlüsselwörter

Kraniosynostosen - isoliert - monosutural - Ossifikationsstörung - Korrektur Neuro- und Viszerokranium

Keywords

- Craniosynostosis - scaphocephaly - trigonocephaly - cranial vault remodelling - fronto-orbital advancement
 

  • Literatur

  • 1 Virchow R. On dwarfism, particularly in Franconia, and on pathological skull shapes [in German]. Verh Phys Med Gesellsc 1851; 2: 230-270.
    Google Scholar
  • 2 Mühling J. Kraniofaziale Chirurgie. Kirschnerscher Operationslehre 1995; 403-427.
    Google Scholar
  • 3 Marchac D, Renier D. Craniosynostosis. World J Surg 1989; 13: 358-365.
    CrossrefPubMedGoogle Scholar
  • 4 Freudlsperger C, Castrillón-Oberndorfer G, Hoffmann J, Engel M. Isolierte, nichtsyndromale Kraniosynostosen. Der MKG-Chirurg 2013; 6: 301-313.
    Google Scholar
  • 5 van der Meulen J, van der Hulst R, van Adrichem L. et al. The increase of metopic synostosis: a pan-European observation. J Craniofac Surg 2009; 20: 283-286.
    CrossrefPubMedGoogle Scholar
  • 6 Alden TD, Lin KY, Jane JA. Mechanisms of premature closure of cranial sutures. Childs Nerv Syst 1999; 15: 670-675.
    CrossrefPubMedGoogle Scholar
  • 7 Park TS, Grady MS, Persing JA, Delashaw JB. One-stage reduction cranioplasty for macrocephaly associated with advanced hydrocephalus. Neurosurgery 1985; 17: 506-509.
    CrossrefPubMedGoogle Scholar
  • 8 Lajeunie E, Le Merrer M, Bonaiti-Pellie C. et al. Genetic study of scaphocephaly. Am J Med Genet 1996; 62: 282-285.
    CrossrefPubMedGoogle Scholar
  • 9 Lana-Elola E, Rice R, Grigoriadis AE, Rice DP. Cell fate specification during calvarial bone and suture development. Dev Biol 2007; 311: 335-346.
    CrossrefPubMedGoogle Scholar
  • 10 Coussens AK, Hughes IP, Wilkinson CR. et al. Identification of genes differentially expressed by prematurely fused human sutures using a novel in vivo - in vitro approach. Differentiation 2008; 76: 531-545.
    CrossrefPubMedGoogle Scholar
  • 11 Marchac D. Basic surgical principles in the treatment of craniostenosis, 31-38. Neurosurgical problems related to craniofacial surgery in children, 45 - 51. General surgical approach, 53-165. Complications, 175 -181. In. Marchac D, Renier D. (eds) Craniofacial surgery for craniosynostosis. 1 ed. Boston: Little Brown; 1982: 211
    Google Scholar
  • 12 Marchac D, Renier D. Craniofacial surgery for craniosynostosis. Boston: Little Brown & Co; 1986
    Google Scholar
  • 13 Mühling J. Zur operativen Behandlung der prämaturen Schädelnahtsynostosen. Würzburg: Julius-Maximilians-Universität; 1986
    Google Scholar
  • 14 Mühling J, Reuther J, Collmann H, Sörensen N. Principle of osteotomy for craniosynostoses. In. Pfeifer G. (ed) Craniofacial abnormities and clefts of lips, alveolus and palate. Stuttgart: Thieme; 1991: 52
    Google Scholar
  • 15 Whitaker LA, Bartlett SP, Schut L, Bruce D. Craniosynostosis: an analysis of the timing, treatment, and complications in 164 consecutive patients. Plast Reconstr Surg 1987; 80: 195-212.
    CrossrefPubMedGoogle Scholar
  • 16 Plooij JM, Verhamme Y, Bergé SJ. et al. Unilateral craniosynostosis of the frontosphenoidal suture: a case report and a review of literature. J Craniomaxillofac Surg 2009; 37: 162-166.
    CrossrefPubMedGoogle Scholar
  • 17 Mesa JM, Fang F, Muraszko KM, Buchman SR. Reconstruction of unicoronal plagiocephaly with a hypercorrection surgical technique. Neurosurg Focus 2011; 31: E4.
    CrossrefPubMedGoogle Scholar
  • 18 Ursitti F, Fadda T, Papetti L. et al. Evaluation and management of nonsyndromic craniosynostosis. Acta Paediatr 2011; 100: 1185-1194.
    CrossrefPubMedGoogle Scholar
  • 19 Smartt JM, Elliott RM, Reid RR, Bartlett SP. Analysis of differences in the cranial base and facial skeleton of patients with lambdoid synostosis and deformational plagiocephaly. Plast Reconstr Surg 2011; 127: 303-312.
    CrossrefPubMedGoogle Scholar
  • 20 Leboucq N, Montoya P, Martinez Y. et al. Lambdoid craniosynostosis. A 3D-computerized tomographic approach. J Neuroradiol 1993; 20: 24-33.
    Google Scholar
  • 21 Florisson JM, van Veelen ML, Bannink N. et al. Papilledema in isolated single-suture craniosynostosis: prevalence and predictive factors. J Craniofac Surg 2010; 21: 20-24.
    CrossrefPubMedGoogle Scholar
  • 22 Marchac D, Renier D. Treatment of craniosynostosis in infancy. Clin Plast Surg 1987; 14: 61-72.
    PubMedGoogle Scholar
  • 23 Cohen SR, Persing JA. Intracranial pressure in single-suture craniosynostosis. Cleft Palate Craniofac J 1998; 35: 194-196.
    CrossrefPubMedGoogle Scholar
  • 24 Heeckt P, Muhlbauer W, Anderl H. et al. Early radical treatment of pancraniofacial synostosis. Ann Plast Surg 1993; 30: 312-319.
    CrossrefPubMedGoogle Scholar
  • 25 Shillito Jr J, Matson DD. Craniosynostosis: a review of 519 surgical patients. Pediatrics 1968; 41: 829-853.
    CrossrefPubMedGoogle Scholar
  • 26 Tuite GF, Chong WK, Evanson J. et al. The effectiveness of papilledema as an indicator of raised intracranial pressure in children with craniosynostosis. Neurosurgery 1996; 38: 272-278.
    CrossrefPubMedGoogle Scholar
  • 27 Engel M, Thiele C, Mühling J. et al. Trigonocephaly: results after surgical correction of nonsyndromatic isolated metopic suture synostosis in 54 cases. J Craniomaxillofac Surg 2012; 40: 347-353.
    CrossrefPubMedGoogle Scholar
  • 28 Speltz ML, Kapp-Simon KA, Cunningham M. et al. Single-suture craniosynostosis: a review of neurobehavioral research and theory. J Pediatr Psychol 2004; 29: 651-668.
    CrossrefPubMedGoogle Scholar
  • 29 Becker PT, Engelhardt KF, Steinmann MF, Kane J. Infant age, context, and family system influences on the interactive behavior of mothers of infants with mental delay. Res Nurs Health 1997; 20: 39-50.
    CrossrefPubMedGoogle Scholar
  • 30 Sloan GM, Wells KC, Raffel C, McComb JG. Surgical treatment of craniosynostosis: outcome analysis of 250 consecutive patients. Pediatrics 1997; 100: E2.
    CrossrefPubMedGoogle Scholar
  • 31 Stavrou P, Sgouros S, Willshaw HE. et al. Visual failure caused by raised intracranial pressure in craniosynostosis. Childs Nerv Syst 1997; 13: 64-67.
    CrossrefPubMedGoogle Scholar
  • 32 Kapp-Simon KA, Figueroa A, Jocher CA, Schafer M. Longitudinal assessment of mental development in infants with nonsyndromic craniosynostosis with and without cranial release and reconstruction. Plast Reconstr Surg 1993; 92: 831-839. discussion 40-41.
    CrossrefPubMedGoogle Scholar
  • 33 Fearon JA, Singh DJ, Beals SP, Yu JC. The diagnosis and treatment of single-sutural synostoses: are computed tomographic scans necessary?. Plast Reconstr Surg 2007; 120: 1327-1331.
    CrossrefPubMedGoogle Scholar
  • 34 Engel M, Castrillon-Oberndorfer G, Hoffmann J, Freudlsperger C. Value of preoperative imaging in the diagnostics of isolated metopic suture synostosis: A risk-benefit analysis. J Plast Reconstr Aesthet Surg 2012; 65: 1246-1251.
    CrossrefPubMedGoogle Scholar
  • 35 Schweitzer T, Böhm H, Meyer-Marcotty P. et al. Avoiding CT scans in children with single-suture craniosynostosis. Childs Nerv Syst 2012; 28: 1077-1082.
    CrossrefPubMedGoogle Scholar
  • 36 Engel M, Hoffmann J, Mühling J. et al. Magnetic resonance imaging in isolated sagittal synostosis. J Craniofac Surg 2012; 23: e366-369.
    CrossrefPubMedGoogle Scholar
  • 37 Agrawal D, Steinbok P, Cochrane DD. Diagnosis of isolated sagittal synostosis: are radiographic studies necessary?. Childs Nerv Syst 2006; 22: 375-378.
    CrossrefPubMedGoogle Scholar
  • 38 Cerovac S, Neil-Dwyer JG, Rich P. et al. Are routine preoperative CT scans necessary in the management of single suture craniosynostosis?. Br J Neurosurg 2002; 16: 348-354.
    CrossrefPubMedGoogle Scholar
  • 39 Regelsberger J, Delling G, Helmke K. et al. Ultrasound in the diagnosis of craniosynostosis. J Craniofac Surg 2006; 17: 623-625. discussion 6-8.
    CrossrefPubMedGoogle Scholar
  • 40 Driessen C, Bannink N, Lequin M. et al. Are ultrasonography measurements of optic nerve sheath diameter an alternative to funduscopy in children with syndromic craniosynostosis?. J Neurosurg Pediatr 2011; 8: 329-334.
    CrossrefPubMedGoogle Scholar
  • 41 Wilbrand JF, Szczukowski A, Blecher JC. et al. Objectification of cranial vault correction for craniosynostosis by three-dimensional photography. J Craniomaxillofac Surg 2012; 40: 726-730.
    CrossrefPubMedGoogle Scholar
  • 42 McKay DR, Davidge KM, Williams SK. et al. Measuring cranial vault volume with three-dimensional photography: a method of measurement comparable to the gold standard. J Craniofac Surg 2010; 21: 1419-1422.
    CrossrefPubMedGoogle Scholar
  • 43 Mühling J. Osteotomies for treating developmental disorders of the neurocranium and visceral cranium. Fortschr Kieferorthop 1991; 52: 15-20.
    CrossrefPubMedGoogle Scholar
  • 44 Engel M, Thiele OC, Mühling J. et al. Trigonocephaly: Results after surgical correction of nonsyndromatic isolated metopic suture synostosis in 54 cases. J Craniomaxillofac Surg 2012; 40: 347-353.
    CrossrefPubMedGoogle Scholar
  • 45 Tessier P, Guiot G, Rougerie J. et al. Cranio-naso-orbito-facial osteotomies. Hypertelorism. Ann Chir Plast 1967; 12: 103-118.
    PubMedGoogle Scholar
  • 46 Zöller JE, Mischkowski RA, Speder B. Preliminary results of standardized occipital advancement in the treatment of lambdoid synostosis. J Craniomaxillofac Surg 2002; 30: 343-348.
    CrossrefPubMedGoogle Scholar
  • 47 Arnaud E, Renier D. Pediatric craniofacial osteosynthesis and distraction using an ultrasonic-assisted pinned resorbable system: a prospective report with a minimum 30 months’ follow-up. J Craniofac Surg 2009; 20: 2081-2086.
    CrossrefPubMedGoogle Scholar
  • 48 Jimenez DF, Barone CM. Endoscopic craniectomy for early surgical correction of sagittal craniosynostosis. J Neurosurg 1998; 88: 77-81.
    CrossrefPubMedGoogle Scholar
  • 49 Panchal J, Marsh JL, Park TS. et al. Sagittal craniosynostosis outcome assessment for two methods and timings of intervention. Plast Reconstr Surg 1999; 103: 1574-1584.
    CrossrefPubMedGoogle Scholar
  • 50 Sood S, Rozzelle A, Shaqiri B. et al. Effect of molding helmet on head shape in nonsurgically treated sagittal craniosynostosis. J Neurosurg Pediatr 2011; 7: 627-632.
    CrossrefPubMedGoogle Scholar
  • 51 Proctor MR, Rogers GF. Helmets and synostosis. J Neurosurg Pediatr 2012; 9: 680-681. author reply 1-2.
    CrossrefPubMedGoogle Scholar
  • 52 Di Rocco F, Marchac A, Duracher C. et al. Posterior remodeling flap for posterior plagiocephaly. Childs Nerv Syst 2012; 28: 1395-1397.
    CrossrefPubMedGoogle Scholar
  • 53 Jane JA, Lin KY. Sagittal synostosis. Neurosurg Focus 2000; 9: e3.
    CrossrefPubMedGoogle Scholar
  • 54 Engel M, Freudlsperger C, Hoffmann J. et al. Surgical outcome after using a modified technique of the pi-procedure for posterior sagittal suture closure. J Craniomaxillofac Surg 2012; 40: 363-368.
    CrossrefPubMedGoogle Scholar
  • 55 Lauritzen CG, Davis C, Ivarsson A. et al. The evolving role of springs in craniofacial surgery: the first 100 clinical cases. Plast Reconstr Surg 2008; 121: 545-554.
    CrossrefPubMedGoogle Scholar
  • 56 van Veelen ML, Mathijssen IM. Spring-assisted correction of sagittal suture synostosis. Childs Nerv Syst 2012; 28: 1347-1351.
    CrossrefPubMedGoogle Scholar