kraniosynostose (LH Indholdsside)

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    <Content>
      <HtmlField Name="PageContent"><![CDATA[<h2>Diagnose</h2>
<h3>Diagnostiske kriterier</h3>
<ul>
    <li>Diagnosen baserer sig p&aring; kliniske fund, CT og molekyl&aelig;rgenetiske unders&oslash;gelser</li>
</ul>
<h3>Sygehistorie</h3>
<ul>
    <li>Kraniosynostose er oftest til stede ved f&oslash;dslen, men tilstanden bliver ikke altid diagnosticeret, hvis den er mild</li>
    <li>Som regel diagnosticeres en kraniedeformitet i l&oslash;bet af de f&oslash;rste m&aring;neder af livet</li>
</ul>
<h4>Afklar f&oslash;lgende</h4>
<ul>
    <li>Hvilken stilling sover barnet i?
    <ul>
        <li>
        For at skelne kraniosynostose fra plagiocefali uden synostose</li>
    </ul>
    </li>
</ul>
<h3>Kliniske fund</h3>
<ul>
    <li>Formen p&aring; kalvariet er typisk for hver af de forskellige typer sutursynostoser</li>
    <li>M&aring;linger af hovedomfanget er afg&oslash;rende for at p&aring;vise ledsagende mikrocefali eller makrocefali (for&aring;rsaget af hydrocefalus)</li>
    <li>B&oslash;rn med kraniofaciale misdannelser b&oslash;r gennemg&aring; en grundig unders&oslash;gelse af nakke, ryg, fingre og t&aelig;er</li>
</ul>
<h3>Supplerende unders&oslash;gelser i almen praksis</h3>
<ul>
    <li>M&aring;ling af hovedomfang og palpation af kranium mhp. vurdering af fontaneller</li>
</ul>
<h3>Andre unders&oslash;gelser hos specialist</h3>
<ul>
    <li>Molekyl&aelig;rgenetisk unders&oslash;gelse ved mistanke om syndrom<a href="EditorPage.aspx?da=core&amp;id=%7BC7E1F5AB-D1E8-4BB5-A883-A36806B12A20%7D&amp;ed=FIELD5997447638&amp;vs&amp;la=da&amp;fld=%7B850D32D3-55A1-49E4-9CDF-F0C88D91563C%7D&amp;so=%2Fsitecore%2Fsystem%2FSettings%2FHtml%20Editor%20Profiles%2FRich%20Text%20LHPH&amp;di=0&amp;hdl=H5997447697&amp;us=sitecore%5Canvi&amp;mo&amp;pe=0&amp;fbd=1" data-type="journal-reference" data-value="Wilkie AO, Bochukova EG, Hansen RM, Taylor IB, Rannan-Eliya SV, Byren JC, Wall SA, Ramos L, Venâncio M, Hurst JA, O'rourke AW, Williams LJ, Seller A, Lester T, Clinical dividends from the molecular genetic diagnosis of craniosynostosis.. Am J Med Genet A 2007;143A: 1941-9" data-value-piped="Wilkie AO, Bochukova EG, Hansen RM, Taylor IB, Rannan-Eliya SV, Byren JC, Wall SA, Ramos L, Venâncio M, Hurst JA, O'rourke AW, Williams LJ, Seller A, Lester T|Clinical dividends from the molecular genetic diagnosis of craniosynostosis.|Am J Med Genet A|2007|143A|1941-9|17621648" data-url="reference-link" data-pubmedid="17621648" title="Wilkie AO, Bochukova EG, Hansen RM, Taylor IB, Rannan-Eliya SV, Byren JC, Wall SA, Ramos L, Venâncio M, Hurst JA, O'rourke AW, Williams LJ, Seller A, Lester T, Clinical dividends from the molecular genetic diagnosis of craniosynostosis.. Am J Med Genet A 2007;143A: 1941-9">1</a></li>
    <li>Klinisk unders&oslash;gelse er som regel tilstr&aelig;kkelig til at diagnosticere enkelt-sutur kraniosynostose<sup>,</sup></li>
    <li>CT giver et tydeligt billede af suturerne
    <ul>
        <li>Herudover bidrager CT til vurderingen af evt. strukturelle &aelig;ndringer i hjernen (f.eks. hydrocefalus, agenese af corpus callosum) og til at ekskludere andre &aring;rsager til asymmetrisk v&aelig;kst (f.eks. hjernehemiatrofi, kronisk subduralt h&aelig;matom)</li>
        <li>Tredimensionel rekonstruktion af CT-billeder er en hj&aelig;lp for kirurgen. som planl&aelig;gger rekonstruktion af kraniofacial deformitet</li>
    </ul>
    </li>
</ul>
<h3>Differentialdiagnoser</h3>
<ul>
    <li>Deformationsbetinget plagiocefali
    <ul>
        <li>Sk&aelig;vt kranium uden synostose, se&nbsp;<a href="~/link.aspx?_id=DAD9B22A3F0946FFB3F7AAB4DC3B3882&amp;_z=z">billede</a></li>
        <li>Er blevet hyppig, efter at man anbefaler, at sp&aelig;db&oslash;rn skal sove p&aring; ryggen, se&nbsp;<a href="~/link.aspx?_id=3D89F94295114370AD2EC02514CEDE52&amp;_z=z">positionel hovedsk&aelig;vhed</a></li>
        <li>Diagnosen stilles ved at inspicere barnets hoved ovenfra&nbsp;<a title="Governale LS, Craniosynostosis. Pediatr Neurol 2015;53: 394 - 401" data-url="reference-link" data-value-piped="Governale LS|Craniosynostosis|Pediatr Neurol|2015|53|394 - 401" data-value="Governale LS, Craniosynostosis. Pediatr Neurol 2015;53: 394 - 401" data-type="journal-reference" href="EditorPage.aspx?da=core&amp;id=%7BC7E1F5AB-D1E8-4BB5-A883-A36806B12A20%7D&amp;ed=FIELD5997447638&amp;vs&amp;la=da&amp;fld=%7B850D32D3-55A1-49E4-9CDF-F0C88D91563C%7D&amp;so=%2Fsitecore%2Fsystem%2FSettings%2FHtml%20Editor%20Profiles%2FRich%20Text%20LHPH&amp;di=0&amp;hdl=H5997447697&amp;us=sitecore%5Canvi&amp;mo&amp;pe=0&amp;fbd=1">2</a></li>
        <li>Hovedet er typisk rundt ved f&oslash;dslen, men bliver sk&aelig;vt i l&oslash;bet af uger til m&aring;neder</li>
        <li>Pr&aelig;mature b&oslash;rn har st&oslash;rre risiko for at f&aring; sk&aelig;vt hoved</li>
        <li>For at hindre udvikling af sk&aelig;vt kranium skal for&aelig;ldrene informeres om, at barnet b&oslash;r ligge p&aring; alternerende side af baghovedet</li>
    </ul>
    </li>
</ul>
<h2>Behandling</h2>
<h3>Behandlingsm&aring;l</h3>
<ul>
    <li>Forhindre hjerneskader</li>
    <li>Korrektion af misdannelser</li>
</ul>
<h3>Generelt om behandlingen&nbsp;<a href="EditorPage.aspx?da=core&amp;id=%7BC7E1F5AB-D1E8-4BB5-A883-A36806B12A20%7D&amp;ed=FIELD5997447638&amp;vs&amp;la=da&amp;fld=%7B850D32D3-55A1-49E4-9CDF-F0C88D91563C%7D&amp;so=%2Fsitecore%2Fsystem%2FSettings%2FHtml%20Editor%20Profiles%2FRich%20Text%20LHPH&amp;di=0&amp;hdl=H5997447697&amp;us=sitecore%5Canvi&amp;mo&amp;pe=0&amp;fbd=1" data-type="journal-reference" data-value="Nagy L, Demke JC, Craniofacial anomalies.. Facial Plast Surg Clin North Am 2014;22: 523-48" data-value-piped="Nagy L, Demke JC|Craniofacial anomalies.|Facial Plast Surg Clin North Am|2014|22|523-48|25444726" data-url="reference-link" data-pubmedid="25444726" title="Nagy L, Demke JC, Craniofacial anomalies.. Facial Plast Surg Clin North Am 2014;22: 523-48">3</a></h3>
<ul>
    <li>H&aring;ndteringen af et barn med kraniofaciale misdannelser kr&aelig;ver tv&aelig;rfagligt teamarbejde</li>
    <li>Barnet skal helst vurderes i l&oslash;bet af de f&oslash;rste uger af livet</li>
    <li>Behandlingen er kirurgisk korrektion</li>
</ul>
<h3>H&aring;ndtering i almen praksis</h3>
<ul>
    <li>Ved mistanke henvises elektivt til b&oslash;rneafdeling. Ved tegn p&aring; &oslash;get intrakranielt tryk akut</li>
</ul>
<h3>R&aring;d til patienten</h3>
<ul>
    <li>Ingen </li>
</ul>
<h3>Kirurgi</h3>
<ul>
    <li>Bedste tidspunkt for behandling er i 3-9-mdrs.alderen</li>
    <li>Ved &oslash;get intrakranielt tryk er hurtig dekompression p&aring;kr&aelig;vet</li>
</ul>
<h4>Kirurgisk intervention ved ikke-syndromal kraniosynostose&nbsp;</h4>
<ul>
    <li>Sagittal synostose
    <ul>
        <li>Indgrebet indeb&aelig;rer enten en stribeformet kraniektomi eller remodellering af kraniet med fjernelse af involverede knogler, som s&aring; trimmes, reformes og fikseres</li>
        <li>Der er rapporteret succes med anvendelse af en minimalt invasiv endoskopisk metode<a title="Jimenez DF, Barone CM, Endoscopic techniques for craniosynostosis. Atlas Oral Maxillofac Surg Clin North Am 2010;18: 93-107" data-url="reference-link" data-value-piped="Jimenez DF, Barone CM|Endoscopic techniques for craniosynostosis|Atlas Oral Maxillofac Surg Clin North Am|2010|18|93-107" data-value="Jimenez DF, Barone CM, Endoscopic techniques for craniosynostosis. Atlas Oral Maxillofac Surg Clin North Am 2010;18: 93-107" data-type="journal-reference" href="EditorPage.aspx?da=core&amp;id=%7BC7E1F5AB-D1E8-4BB5-A883-A36806B12A20%7D&amp;ed=FIELD5997447638&amp;vs&amp;la=da&amp;fld=%7B850D32D3-55A1-49E4-9CDF-F0C88D91563C%7D&amp;so=%2Fsitecore%2Fsystem%2FSettings%2FHtml%20Editor%20Profiles%2FRich%20Text%20LHPH&amp;di=0&amp;hdl=H5997447697&amp;us=sitecore%5Canvi&amp;mo&amp;pe=0&amp;fbd=1">4</a></li>
    </ul>
    </li>
    <li>Coronal synostose
    <ul>
        <li>Form&aring;let er at &oslash;ge den anteroposteriore l&aelig;ngde af kalvariet</li>
    </ul>
    </li>
    <li>Metopisk synostose
    <ul>
        <li>Form&aring;let er at &oslash;ge volumet af forreste kraniale fossa</li>
    </ul>
    </li>
</ul>
<h4>Kirurgisk intervention ved syndromal kraniosynostose<a title="Robin NH, Falk MJ, Haldeman-Enlert CR. FGFR-related craniosynostosis syndromes. In Pagon RA, Bird TD, Dolan, et al. GeneReviews, June 7, 2011" data-url="http://www.ncbi.nlm.nih.gov/books/NBK1455/" data-value="Robin NH, Falk MJ, Haldeman-Enlert CR. FGFR-related craniosynostosis syndromes. In Pagon RA, Bird TD, Dolan, et al. GeneReviews, June 7, 2011" data-type="other-reference" href="EditorPage.aspx?da=core&amp;id=%7BC7E1F5AB-D1E8-4BB5-A883-A36806B12A20%7D&amp;ed=FIELD5997447638&amp;vs&amp;la=da&amp;fld=%7B850D32D3-55A1-49E4-9CDF-F0C88D91563C%7D&amp;so=%2Fsitecore%2Fsystem%2FSettings%2FHtml%20Editor%20Profiles%2FRich%20Text%20LHPH&amp;di=0&amp;hdl=H5997447697&amp;us=sitecore%5Canvi&amp;mo&amp;pe=0&amp;fbd=1">5</a><sup>,</sup><a href="EditorPage.aspx?da=core&amp;id=%7BC7E1F5AB-D1E8-4BB5-A883-A36806B12A20%7D&amp;ed=FIELD5997447638&amp;vs&amp;la=da&amp;fld=%7B850D32D3-55A1-49E4-9CDF-F0C88D91563C%7D&amp;so=%2Fsitecore%2Fsystem%2FSettings%2FHtml%20Editor%20Profiles%2FRich%20Text%20LHPH&amp;di=0&amp;hdl=H5997447697&amp;us=sitecore%5Canvi&amp;mo&amp;pe=0&amp;fbd=1" data-type="journal-reference" data-value="Nagy L, Demke JC, Craniofacial anomalies.. Facial Plast Surg Clin North Am 2014;22: 523-48" data-value-piped="Nagy L, Demke JC|Craniofacial anomalies.|Facial Plast Surg Clin North Am|2014|22|523-48|25444726" data-url="reference-link" data-pubmedid="25444726" title="Nagy L, Demke JC, Craniofacial anomalies.. Facial Plast Surg Clin North Am 2014;22: 523-48">3</a></h4>
<ul>
    <li>Indeb&aelig;rer korrektion af kraniosynostosen i 3-6-mdrs.alder, og evt. korrektion af ekstremitetsdefekter i 1-2-&aring;rsalder</li>
    <li>Som ung voksen udf&oslash;res indgreb for at normalisere udseende og korrigere malokklusion, forudg&aring;et af ofte langvarig ortodontisk behandling</li>
    <li>T&aelig;t opf&oslash;lgning er vigtig efter kirurgi for at kontrollere, at suturerne ikke gror sammen igen</li>
</ul>
<h3>Forebyggende behandling</h3>
<ul>
    <li>Ingen</li>
</ul>
<h2>Henvisning</h2>
<ul>
    <li>Ved mistanke om kraniosynostose henvises til den lokale b&oslash;rneafdeling, som vil viderehenvise til det kraniofaciale team p&aring; Aarhus Universitet Hospital eller Rigshospitalet, s&aring;fremt diagnosen bekr&aelig;ftes</li>
</ul>
<h2>Opf&oslash;lgning</h2>
<ul>
    <li>Sker ved det kraniofasciale team</li>
</ul>
<h2>Sygdomsforl&oslash;b, komplikationer og prognose<a href=" " data-type="journal-reference" data-value="Blessing M, Gallagher ER, Epidemiology, Genetics, and Pathophysiology of Craniosynostosis. Oral Maxillofac Surg Clin North Am 2022;34: 341-352" data-value-piped="Blessing M, Gallagher ER|Epidemiology, Genetics, and Pathophysiology of Craniosynostosis.|Oral Maxillofac Surg Clin North Am|2022|34|341-352|35787827" data-url="reference-link" data-pubmedid="35787827" title="Blessing M, Gallagher ER, Epidemiology, Genetics, and Pathophysiology of Craniosynostosis. Oral Maxillofac Surg Clin North Am 2022;34: 341-352">6</a></h2>
<h3>Sygdomsforl&oslash;b</h3>
<ul>
    <li>Normalt ukompliceret </li>
</ul>
<h3>Komplikationer</h3>
<ul>
    <li>De vigtigste komplikationer forbundet med ukorrigeret kraniosynostose er &oslash;get intrakranielt tryk, asymmetri af ansigtet, forsinket udvikling, malokklusion og manglende samsyn</li>
    <li>Asymmetri af orbitae f&oslash;rer til skelen med risiko for unilateralt synstab</li>
</ul>
<h3>Prognose</h3>
<ul>
    <li>God</li>
</ul>
<h2>Baggrundsoplysninger</h2>
<h3>Definition&nbsp;<a href=" " data-type="journal-reference" data-value="Nagy L, Demke JC, Craniofacial anomalies.. Facial Plast Surg Clin North Am 2014;22: 523-48" data-value-piped="Nagy L, Demke JC|Craniofacial anomalies.|Facial Plast Surg Clin North Am|2014|22|523-48|25444726" data-url="reference-link" data-pubmedid="25444726" title="Nagy L, Demke JC, Craniofacial anomalies.. Facial Plast Surg Clin North Am 2014;22: 523-48">3</a><sup>,</sup><a title="Governale LS, Craniosynostosis. Pediatr Neurol 2015;53: 394 - 401" data-url="reference-link" data-value-piped="Governale LS|Craniosynostosis|Pediatr Neurol|2015|53|394 - 401" data-value="Governale LS, Craniosynostosis. Pediatr Neurol 2015;53: 394 - 401" data-type="journal-reference" href=" ">2</a></h3>
<ul>
    <li>Kraniosynostose er en kraniedeformitet hos b&oslash;rn, som skyldes for tidlig lukning af en eller flere kraniesuturer</li>
    <li>En kraniosynostose kan v&aelig;re en isoleret defekt&nbsp;eller en del af et syndrom</li>
</ul>
<h3>Forekomst</h3>
<ul>
    <li>Forekommer hos 1 af 2000 nyf&oslash;dte b&oslash;rn&nbsp;<a href=" " data-type="journal-reference" data-value="Nagy L, Demke JC, Craniofacial anomalies.. Facial Plast Surg Clin North Am 2014;22: 523-48" data-value-piped="Nagy L, Demke JC|Craniofacial anomalies.|Facial Plast Surg Clin North Am|2014|22|523-48|25444726" data-url="reference-link" data-pubmedid="25444726" title="Nagy L, Demke JC, Craniofacial anomalies.. Facial Plast Surg Clin North Am 2014;22: 523-48">3</a></li>
</ul>
<h3>&AElig;tiologi og patogenese</h3>
<ul>
    <li>Knoglerne i kraniet best&aring;r af kraniebasis og kalvariet - se illustrationer<a href=" " data-type="journal-reference" data-value="Kabbani H, Raghuveer TS, Craniosynostosis.. Am Fam Physician 2004;69: 2863-70" data-value-piped="Kabbani H, Raghuveer TS|Craniosynostosis.|Am Fam Physician|2004|69|2863-70|15222651" data-url="reference-link" data-pubmedid="15222651" title="Kabbani H, Raghuveer TS, Craniosynostosis.. Am Fam Physician 2004;69: 2863-70">7</a> </li>
    <li>V&aelig;ksten af kraniet stimuleres prim&aelig;rt af hjernens v&aelig;kst
    <ul>
        <li>Hjernen vokser hurtigt i fosterstadiet og de f&oslash;rste tre leve&aring;r </li>
        <li>Et barn f&oslash;dt til termin har n&aelig;sten 40 % af sit voksne hjernevolumen; ved 3-&aring;rsalderen er det 80 %</li>
    </ul>
    </li>
</ul>
<h4>Lukning af suturer og fontaneller </h4>
<p>
<img src="C0A62FD9DC754B48BF2352472AFE318B-480-276" alt="Tegning af kranium hos nyfødt." style="width: 480px; height: 276px;" title="Tegning af kranium hos nyfødt." height="276" width="480"></p>
<p>&nbsp;</p>
<ul>
    <li>Suturer og fontaneller lukker sig p&aring; forskellige tidspunkter </li>
    <li>Metopiske sutur
    <ul>
        <li>Lukker sig mellem 9 mdr. og 2 &aring;r, men kan best&aring; til voksenalder</li>
    </ul>
    </li>
    <li>Coronale, sagittale og lambdoide suturer
    <ul>
        <li>Lukker sig klinisk ved 6-12 mdr.</li>
        <li>Lukker sig anatomisk ved 30-40 &aring;r</li>
    </ul>
    </li>
    <li>Forreste fontanelle
    <ul>
        <li>Lukker sig i andet leve&aring;r</li>
    </ul>
    </li>
    <li>Bagerste fontanelle
    <ul>
        <li>Lukker sig mellem 3-6 m&aring;neder</li>
    </ul>
    </li>
</ul>
<h4>&AElig;tiologi</h4>
<ul>
    <li>&Aring;rsagen til ikke-syndromal kraniosynostose er ukendt, tilstanden optr&aelig;der sporadisk i de fleste tilf&aelig;lde</li>
</ul>
<h4>Kraniosynostoser</h4>
<ul>
    <li>Angives som enkle, n&aring;r kun &eacute;n sutur er involveret, og sammensatte, n&aring;r to eller flere suturer er involveret </li>
    <li>Sagittalsuturen er afficeret i 40-60 % af tilf&aelig;ldene </li>
    <li>Coronalsuturen er afficeret i 20-30 % </li>
    <li>Metopiske sutur er afficeret i f&aelig;rre end 10 % </li>
    <li>Ren lambdoid synostose er sj&aelig;lden </li>
    <li>Syndromisk kraniosynostose er mindre hyppig (20 %)</li>
</ul>
<h4>Kraniosynostose-syndromer&nbsp;<a href=" " data-type="journal-reference" data-value="Nagy L, Demke JC, Craniofacial anomalies.. Facial Plast Surg Clin North Am 2014;22: 523-48" data-value-piped="Nagy L, Demke JC|Craniofacial anomalies.|Facial Plast Surg Clin North Am|2014|22|523-48|25444726" data-url="reference-link" data-pubmedid="25444726" title="Nagy L, Demke JC, Craniofacial anomalies.. Facial Plast Surg Clin North Am 2014;22: 523-48">3</a><sup>,</sup><a title="Governale LS, Craniosynostosis. Pediatr Neurol 2015;53: 394 - 401" data-url="reference-link" data-value-piped="Governale LS|Craniosynostosis|Pediatr Neurol|2015|53|394 - 401" data-value="Governale LS, Craniosynostosis. Pediatr Neurol 2015;53: 394 - 401" data-type="journal-reference" href=" ">2</a><a href=" " data-type="journal-reference" data-value="Blessing M, Gallagher ER, Epidemiology, Genetics, and Pathophysiology of Craniosynostosis. Oral Maxillofac Surg Clin North Am 2022;34: 341-352" data-value-piped="Blessing M, Gallagher ER|Epidemiology, Genetics, and Pathophysiology of Craniosynostosis.|Oral Maxillofac Surg Clin North Am|2022|34|341-352|35787827" data-url="reference-link" data-pubmedid="35787827" title="Blessing M, Gallagher ER, Epidemiology, Genetics, and Pathophysiology of Craniosynostosis. Oral Maxillofac Surg Clin North Am 2022;34: 341-352">#</a></h4>
<ul>
    <li>Mere end 150 forskellige beskrevet&nbsp;</li>
    <li>&Oslash;get risiko for forh&oslash;jet intrakranielt tryk og hydrocephalus</li>
    <li>Involverer ofte andre organsystemer udover de kraniofaciale misdannelser </li>
    <li>Mange patienter har en familieanamnese med unormal hovedform </li>
    <li><a href="~/link.aspx?_id=B70DA53ADDD647F3BFDE7F17D883AF97&amp;_z=z">Muenkes syndrom</a><a href="http://www.ncbi.nlm.nih.gov/books/NBK1415" data-type="other-reference" data-value="Agochukwu NB, Doherty ES, Muenke M. Muenke Syndrome. In: Pagon RA, Bird TD, Dolan CR, Stephens K, Eds. GeneReviews May 10, 2006" data-url="http://www.ncbi.nlm.nih.gov/books/NBK1415" title="Agochukwu NB, Doherty ES, Muenke M. Muenke Syndrome. In: Pagon RA, Bird TD, Dolan CR, Stephens K, Eds. GeneReviews May 10, 2006">8</a>
    <ul>
        <li>Autosomal dominant arvelig tilstand </li>
        <li>Skyldes mutation i genet for fibroblast v&aelig;kst faktor receptor 3, FGFR3, i 75 % af tilf&aelig;ldene mutationen P250R </li>
        <li>Kliniske fund omfatter bl.a. coronal kraniosynostose (uni- eller bikoronal) </li>
        <li>Normal psykomotorisk udvikling </li>
        <li>Ofte normale h&aelig;nder og f&oslash;dder, men carpal-tarsal fusion er diagnostisk </li>
        <li>Evt. kirurgisk kranioplastik for at forhindre &oslash;get intrakranielt tryk eller for at korrigere kraniets form</li>
    </ul>
    </li>
    <li><a href="~/link.aspx?_id=AED5367183A14499BB43975765C5E183&amp;_z=z">Crouzons syndrom</a><a href="http://www.ncbi.nlm.nih.gov/books/NBK1455/" data-type="other-reference" data-value="Robin NH, Falk MJ, Haldeman-Enlert CR. FGFR-related craniosynostosis syndromes. In Pagon RA, Bird TD, Dolan, et al. GeneReviews, June 7, 2011" data-url="http://www.ncbi.nlm.nih.gov/books/NBK1455/" title="Robin NH, Falk MJ, Haldeman-Enlert CR. FGFR-related craniosynostosis syndromes. In Pagon RA, Bird TD, Dolan, et al. GeneReviews, June 7, 2011">5</a>
    <ul>
        <li>Autosomal dominant arvelig tilstand </li>
        <li>Skyldes mutation i genet for fibroblast v&aelig;kst faktor receptor 2, FGFR2 </li>
        <li>N&aelig;sten 60 % skyldes nymutationer, associeret med paternel alder over 35 &aring;r </li>
        <li>Forekommer hos 1 af 25.000 f&oslash;dte og udg&oslash;r 5 % af tilf&aelig;lde med kraniosynostose </li>
        <li>Kliniske fund inkluderer kraniosynostose, brakycefali, midfacial hypoplasi, hypertelorisme, proptose, opticusatrofi </li>
        <li>Normale h&aelig;nder og f&oslash;dder </li>
        <li>Evt. kirurgisk korrektion af kraniosynostose og/eller kraniofaciale deformiteter</li>
    </ul>
    </li>
    <li><a href="~/link.aspx?_id=CBC09582B20947479239450A77CCBAF6&amp;_z=z">Aperts syndrom</a><a href="http://www.ncbi.nlm.nih.gov/books/NBK1455/" data-type="other-reference" data-value="Robin NH, Falk MJ, Haldeman-Enlert CR. FGFR-related craniosynostosis syndromes. In Pagon RA, Bird TD, Dolan, et al. GeneReviews, June 7, 2011" data-url="http://www.ncbi.nlm.nih.gov/books/NBK1455/" title="Robin NH, Falk MJ, Haldeman-Enlert CR. FGFR-related craniosynostosis syndromes. In Pagon RA, Bird TD, Dolan, et al. GeneReviews, June 7, 2011">5</a>
    <ul>
        <li>Acrocephalosyndactyli type 1 </li>
        <li>Skyldes mutation i genet for fibroblast v&aelig;kst faktor receptor 2, FGFR2, oftest nymutation </li>
        <li>Autosomal dominant tilstand, som forekommer hos 1 af 160.000 nyf&oslash;dte </li>
        <li>Symmetrisk syndaktyli af alle ekstremiteter </li>
        <li>Andre kliniske fund inkluderer misdannet kranium, hypertelorisme, midfacial hypoplasi, choanalatresi- eller stenose og smalle orbitae </li>
        <li>Intrakranielle misdannelser forekommer med variabel mental retardering </li>
        <li>Kardiale og renale misdannelser kan ogs&aring; forekomme</li>
    </ul>
    </li>
</ul>
<h3>Disponerende faktorer</h3>
<ul>
    <li>Famili&aelig;r ikke-syndromal kraniosynostose hos 2-6 % af b&oslash;rn med sagittal synostose og 8-14 % af b&oslash;rn med coronal synostose. Overf&oslash;res som en autosomal dominant tilstand</li>
</ul>
<h3>ICPC-2</h3>
<p>[ICPC]</p>
<h3>ICD-10/SKS-koder</h3>
<p>[ICD10]</p>
<h2>Patientinformation</h2>
<h3>Hvad findes af skriftlig patientinformation</h3>
<ul>
    <li><a href="~/link.aspx?_id=FD678AEA69A64827BD4B73DBEC49A752&amp;_z=z">Kraniosynostose</a></li>
    <li><a href="~/link.aspx?_id=4FBADC55262A40E1AB5A915862BCBDB1&amp;_z=z">Sk&aelig;vt hoved hos sp&aelig;db&oslash;rn</a></li>
</ul>
<h2>Link til vejledninger</h2>
<ul>
    <li><a href="https://www.rigshospitalet.dk/afdelinger-og-klinikker/julianemarie/boern-og-unge/sjaeldne-sygdomme-center-5004/det-kraniofaciale-team/kraniofaciale-misdannelser/Sider/kraniosynostose.aspx">Juliane Marie Centret, Center for Sj&aelig;ldne Sygdomme, Det Kraniofaciale Team, Om Kraniofaciale misdannelser, Kraniosynostose</a></li>
    <li>Forl&oslash;bsbeskrivelser og pakkeforl&oslash;b [ICPC]</li>
</ul>
<h2>Illustrationer</h2>
<ul>
    <li><a href="~/link.aspx?_id=E2DCA46630894B9F8F89AE792DDF0FFD&amp;_z=z">Normalt kranium hos nyf&oslash;dt - tegning</a></li>
    <li><a href="~/link.aspx?_id=83C6FE3A6E6F49EB9770BDFEC2DB0CA6&amp;_z=z">Lukning af suturer og fontaneller - tegning</a></li>
    <li><a href="~/link.aspx?_id=1A340BD528744F82AEEB63B4A50C2A3A&amp;_z=z">Sk&aelig;vt kranium - tegning</a></li>
    <li><a href="~/link.aspx?_id=DAD9B22A3F0946FFB3F7AAB4DC3B3882&amp;_z=z">Sk&aelig;vt hoved, plagiocefali - foto</a></li>
</ul>]]></HtmlField>
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<h3>Diagnose</h3>
<ul>
    <li>Diagnosen baserer sig p&aring; kliniske fund, CT </li>
    <li>Ved mistanke om syndromal kraniosynostose foretages molekyl&aelig;rgenetiske unders&oslash;gelser</li>
</ul>
<h3>Behandling</h3>
<ul>
    <li>Operation i 3-9-mdrs.alderen</li>
</ul>
<h3>Henvisning</h3>
<ul>
    <li>Ved mistanke om kraniesynostose henvises til lokal b&oslash;rneafdeling, som tager stilling til viderehenvisning til det kraniofaciale team p&aring; Rigshospitalet eller Aarhus Universitet Hospital</li>
</ul>]]></HtmlField>
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      <TextField Name="ICD10">Q75, Q75.0, Q75.1, Q75.8, Q87, Q87.0, Q87.0B</TextField>
      <TextField Name="ICPC2">L82</TextField>
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      <TextField Name="Description">Kraniosynostose er en kraniedeformitet hos børn, som skyldes for tidlig lukning af en eller flere kraniesuturer. En kraniosynostose kan være en isoleret defekt eller en del af et syndrom.</TextField>
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      <TextField Name="MetaKeywords">apert, craniosynostosis, crouzons, fontanelle, Kraniosynostose, plagiocefali, plagiocephalia, skævt hoved, skævt kranium, sutur, synostose</TextField>
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