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<article article-type="review-article" dtd-version="1.0" xml:lang="hr" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:mml="http://www.w3.org/1998/Math/MathML">
<front>
<journal-meta>
<journal-id journal-id-type="publisher-id">LV</journal-id>
<journal-id journal-id-type="nlm-ta">Lijec Vjesn</journal-id>
<journal-title-group>
<journal-title>Lijecnicki Vjesnik</journal-title>
<abbrev-journal-title abbrev-type="pubmed">Lijec. Vjesn.</abbrev-journal-title>
</journal-title-group>
<issn pub-type="ppub">0024-3477</issn>
<issn pub-type="epub">1849-2177</issn>
<publisher><publisher-name>Croatian Medical Association</publisher-name></publisher>
</journal-meta>
<article-meta>
<article-id pub-id-type="publisher-id">LV-147-223</article-id>
<article-id pub-id-type="doi">10.26800/LV-147-5-6-7</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>Review</subject></subj-group>
</article-categories>
<title-group>
<article-title>Uro&#x0111;ena pseudoartroza klju&#x010D;ne kosti</article-title>
<trans-title-group xml:lang="en">
<trans-title>Congenital pseudoarthrosis of the clavicle</trans-title>
</trans-title-group>
</title-group>
<contrib-group>
<contrib contrib-type="author"><name><surname>Antabak</surname><given-names>Anko</given-names></name><xref ref-type="aff" rid="aff1"><sup>1</sup></xref><xref ref-type="aff" rid="aff2"><sup>2</sup></xref></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9880-1185</contrib-id><name><surname>Juri&#x0107;</surname><given-names>Filip</given-names></name><xref ref-type="aff" rid="aff2"><sup>2</sup></xref><xref ref-type="aff" rid="aff3"><sup>3</sup></xref></contrib><contrib contrib-type="author"><name><surname>Vlahek</surname><given-names>Tomislav</given-names></name><xref ref-type="aff" rid="aff1"><sup>1</sup></xref></contrib><contrib contrib-type="author"><name><surname>Pape&#x0161;</surname><given-names>Dino</given-names></name><xref ref-type="aff" rid="aff1"><sup>1</sup></xref><xref ref-type="aff" rid="aff2"><sup>2</sup></xref></contrib><contrib contrib-type="author"><name><surname>Bukvi&#x0107;</surname><given-names>Nado</given-names></name><xref ref-type="aff" rid="aff3"><sup>3</sup></xref><xref ref-type="aff" rid="aff4"><sup>4</sup></xref></contrib>
<aff id="aff1"><label>1</label><institution content-type="dept">Zavod za dje&#x010D;ju kirurgiju, Klinika za kirurgiju</institution>, <institution>KBC Zagreb</institution>, <addr-line>Zagreb</addr-line></aff>
<aff id="aff2"><label>2</label><institution>Medicinski fakultet Sveu&#x010D;ili&#x0161;ta u Zagrebu</institution>, <addr-line>Zagreb</addr-line></aff>
<aff id="aff3"><label>3</label><institution content-type="dept">Klinika za dje&#x010D;ju kirurgiju</institution>, <institution>KBC Rijeka</institution>, <addr-line>Rijeka</addr-line></aff>
<aff id="aff4"><label>4</label><institution>Medicinski fakultet Sveu&#x010D;ili&#x0161;ta u Rijeci</institution>, <addr-line>Rijeka</addr-line></aff>
</contrib-group>
<author-notes>
<corresp id="cor1">Adresa za dopisivanje: Filip Juri&#x0107;, dr. med., <ext-link ext-link-type="uri" xlink:href="https://orcid.org/0000-0002-9880-1185">https://orcid.org/0000-0002-9880-1185</ext-link>, Klinika za dje&#x010D;ju kirurgiju, KBC Rijeka, V. Duki&#x0107;a 7, 51000 Rijeka, e-po&#x0161;ta: <email xlink:href="fjuric@gmail.com">fjuric@gmail.com</email></corresp>
<fn fn-type="con">
<p content-type="fn-title">DOPRINOS AUTORA</p>
<p>K<sc>oncepcija</sc> <sc>ili</sc> <sc>nacrt</sc> <sc>rada</sc>: AA</p>
<p>P<sc>rikupljanje</sc>, <sc>analiza</sc> <sc>i</sc> <sc>interpretacija</sc> <sc>podataka</sc>: AA, FJ, TV, DP, NB</p>
<p>P<sc>isanje</sc> <sc>prve</sc> <sc>verzije</sc> <sc>rada</sc>: AA, FJ, TV, DP, NB</p>
<p>K<sc>riti&#x010D;ka</sc> <sc>revizija</sc>: AA, FJ</p>
</fn>
</author-notes>
<pub-date date-type="pub" publication-format="electronic"><month>07</month><year>2025</year></pub-date>
<pub-date date-type="pub" publication-format="print"><month>07</month><year>2025</year></pub-date>
<volume>147</volume>
<issue>5-6</issue>
<fpage>223</fpage>
<lpage>228</lpage>
<permissions>
<copyright-statement>Croatian Medical Association</copyright-statement>
<copyright-year>2025</copyright-year>
<copyright-holder>Croatian Medical Association</copyright-holder>
<license xlink:href="https://creativecommons.org/licenses/by-nc-nd/4.0/" specific-use="CC BY-NC-ND 4.0"><license-p>This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivatives (CC BY-NC-ND) 4.0 License.</license-p></license>
</permissions>
<abstract>
<title>SA&#x017D;ETAK</title>
<p>Uro&#x0111;ena pseudoatroza klju&#x010D;ne kosti iznimno je rijetka anomalija obilje&#x017E;ena nesrastanjem medijalne i lateralne osifikacijske jezgre. Prvi put je opisana 1910. godine i od tada je zabilje&#x017E;eno tek tristotinjak slu&#x010D;ajeva. Pojavnost ove anomalije je oko 1:150.000, a dijagnoza se ve&#x0107;inom postavi u adolescentskoj dobi. U ranijoj dobi &#x010D;esto se previdi jer se zadebljanje klju&#x010D;ne kosti protuma&#x010D;i kao posttraumatski kalus. Klju&#x010D;na kost je prva kost koja osificira intrauterino s razvijenim osifikacijskim centrima (medijalni i latealni) ve&#x0107; sredinom &#x010D;etvrtog tjedna gestacije, koji se me&#x0111;usobno spoje krajem sedmog tjedna. Ve&#x0107;i dio klju&#x010D;ne kosti razvija se iz medijalnog dijela (oko 80%). Prvi radiolo&#x0161;ki znakovi oko&#x0161;tavanja medijalnog kraja vide se izme&#x0111;u 13. i 14. godine &#x017E;ivota. Etiologija jo&#x0161; uvijek nije razja&#x0161;njena. Jedna teorija povezuje pojavu povi&#x0161;enog pritiska potklju&#x010D;ne arterije, druge ju povezuju s intrauternim polo&#x017E;ajem fetusa ili kao mogu&#x0107;i nasljedni poreme&#x0107;aj. Lije&#x010D;enje priro&#x0111;ene pseudoartroze klju&#x010D;ne kosti kontraverzno je. Opisani slu&#x010D;ajevi ve&#x0107;inom su lije&#x010D;eni konzervativno, osobito ako su bili asimptomatski. Kirur&#x0161;ki su pak lije&#x010D;eni oni s funkcionalnim ograni&#x010D;enjima, iz estetskih razloga, te djeca koja su osje&#x0107;ala progresivnu bol. Kirur&#x0161;ko lije&#x010D;enje se preporu&#x010D;uje i kod djece kojima su ulomci razmaknuti vi&#x0161;e od 1 cm. Predlo&#x017E;ene tehnike za stabilizaciju su razli&#x010D;ite: intramedularna Kirschnerova &#x017E;ica, plo&#x010D;ica i vijci, Steinmannov &#x010D;avao te vanjska fiksacija. U ve&#x0107;ini slu&#x010D;ajeva potreban je ko&#x0161;tani transplantat, osobito u starije djece, a naj&#x010D;e&#x0161;&#x0107;e mjesto donora je krista ilijaka. Optimalna dob za operativno lije&#x010D;enje jest izme&#x0111;u tre&#x0107;e i &#x0161;este godine &#x017E;ivota, kada je mogu&#x0107;nost komplikacija najmanja. Prijelomi klju&#x010D;ne kosti i uro&#x0111;ena pseudartroza te&#x0161;ko se razlikuju kod male djece, osobito pri prvom pregledu. Iako je uro&#x0111;ena pseudartroza rijetka, treba na nju misliti jer nelije&#x010D;ena u pred&#x0161;kolskoj dobi mo&#x017E;e imati lo&#x0161;ije ishode u kasnijoj dobi. Autori u ovom radu daju pregled dosada&#x0161;njih spoznaja o uro&#x0111;enoj pseudoartrozi klju&#x010D;ne kosti te razmatraju terapijske postupke.</p>
</abstract>
<trans-abstract xml:lang="en">
<title>SUMMARY</title>
<p>Congenital pseudarthrosis of the clavicle is an extremely rare anomaly characterized by the failure of fusion between the medial and lateral ossification centers. First described in 1910, only about 300 cases have been documented since. The incidence of this anomaly is approximately 1 in 150,000, with diagnosis mostly occurring in adolescence. In early childhood, it is often overlooked as clavicular thickening may be misinterpreted as a post-traumatic callus. The clavicle is the first bone to ossify in utero, with developed ossification centers (medial and lateral) by the middle of the fourth week of gestation, which fuse by the end of the seventh week. Most of the clavicle develops from the medial part (around 80%). The initial radiographic signs of ossification at the medial end become visible between the ages of 13 and 14. The precise etiology of this malformation remains unclear. One theory links it to increased pressure from the subclavian artery, while others associate it with fetal intrauterine positioning or a possible hereditary disorder. The treatment of congenital pseudarthrosis of the clavicle is controversial. Most documented cases have been treated conservatively, especially if asymptomatic. Surgical intervention is reserved for those with functional limitations, esthetic concerns, or progressive pain. Surgery is also recommended in children with a gap between fragments exceeding 1 cm. Various stabilization techniques have been proposed, including intramedullary Kirschner wire, plates and screws, Steinmann pins, and external fixation. Bone grafting is often required, especially in older children, with the iliac crest being the most common donor site. The optimal age for surgical treatment is between three and six years, as complication rates are lower. Clavicle fractures and congenital pseudarthrosis are challenging to distinguish in young children, especially during initial examination. Although congenital pseudarthrosis is rare, it should be considered as untreated cases in preschool years may result in poorer outcomes later. The authors of this paper provide a review of current knowledge on congenital pseudarthrosis of the clavicle and discuss therapeutic approaches.</p>
</trans-abstract>
<kwd-group kwd-group-type="author"><kwd>Deskriptori PSEUDOARTROZA &#x2013; dijagnoza, kirurgija, uro&#x0111;ena</kwd><kwd>KLJU&#x010C;NA KOST &#x2013; anomalije, kirurgija</kwd><kwd>ORTOPEDSKI ZAHVATI &#x2013; metode</kwd></kwd-group>
<kwd-group kwd-group-type="translator" xml:lang="en"><title>Descriptors </title><kwd>PSEUDARTHROSIS &#x2013; congenital, diagnosis, surgery</kwd><kwd>CLAVICLE &#x2013; abnormalities, surgery</kwd><kwd>ORTHOPEDIC PROCEDURES &#x2013; methods</kwd></kwd-group>
</article-meta>
</front>
<body>
<p>Pseudoartroza ili la&#x017E;ni zglob patolo&#x0161;ka je gibljivost ko&#x0161;tanoga sustava na mjestu gdje normalno ne postoji. Mo&#x017E;e biti ste&#x010D;ena i uro&#x0111;ena. Ste&#x010D;ena nastaje obi&#x010D;no nakon nesrastanja ulomaka prelomljene duge kosti, kada izme&#x0111;u ulomaka kosti proraste vezivno tkivo. Uro&#x0111;ena pseudoartroza rijetka je anomalija (pojavnost im je oko 1:150.000 novoro&#x0111;en&#x010D;adi). Dijagnoza se postavi ve&#x0107;inom u adolescenata. (<xref ref-type="bibr" rid="r1"><italic>1</italic></xref>, <xref ref-type="bibr" rid="r2"><italic>2</italic></xref>) Uro&#x0111;ena pseudoartroza klju&#x010D;ne kosti izuzetno je rijetka anomalija i &#x010D;esto se previdi u prvim godinama &#x017E;ivota. Prvi put je opisana 1910. godine i od tada je opisano tek tristotinjak slu&#x010D;ajeva. (<xref ref-type="bibr" rid="r3"><italic>3</italic></xref>) U pravilu su jednostrane, rijetko i obostrane. (<xref ref-type="bibr" rid="r4"><italic>4</italic></xref>, <xref ref-type="bibr" rid="r5"><italic>5</italic></xref>) S obzirom na raritetnu pojavnost, priro&#x0111;ena pseudoartroza klju&#x010D;ne kosti u prvim godinama &#x017E;ivota u pravilu se pogre&#x0161;no smatra samo prolaznim zadebljanjem periostalnog kalusa nakon traume. Pseudoartroze previ&#x0111;ene u mladosti u odrasloj dobi nije uvijek jednostavno razlikovati od posttraumatskih. (<xref ref-type="bibr" rid="r6"><italic>6</italic></xref>) Takvom razvoju promi&#x0161;ljanja pogoduje i &#x010D;injenica kako je srednja tre&#x0107;ina klju&#x010D;ne kosti jedno od &#x010D;estih mjesta razvoja postraumatske pseudoartroze (pored dijafize humerusa, distalne tibije i vrata talusa). U dje&#x010D;joj dobi prijelomi klavikule su &#x010D;este ozljede, nastaju u razli&#x010D;itim aktivnostima djece svih uzrasta, a odabir metode lije&#x010D;enja i ishodi lije&#x010D;enja uglavnom su dobri. (<xref ref-type="bibr" rid="r7"><italic>7</italic></xref>-<xref ref-type="bibr" rid="r9"><italic>9</italic></xref>) Posttraumatske ste&#x010D;ene pesudoartroze u djece nakon neuspje&#x0161;nog lije&#x010D;enja vidimo sporadi&#x010D;no u adolescenata (<xref ref-type="fig" rid="f1">Figure 1</xref>). Neprepoznate priro&#x0111;ene pseudoartroze ve&#x0107;inom se lije&#x010D;e kao odgo&#x0111;eno cijeljenje nakon prijeloma (imobilizacijom kao traumatske) bez uspjeha. (<xref ref-type="bibr" rid="r10"><italic>10</italic></xref>) A kada se postavi dijagnoza priro&#x0111;ene pseudoartroze nema standardnog pristupa u lije&#x010D;enju. (<xref ref-type="bibr" rid="r11"><italic>11</italic></xref>-<xref ref-type="bibr" rid="r13"><italic>13</italic></xref>)</p>
<fig id="f1" position="float" fig-type="figure"><label>Figure 1</label><caption><p>Post-traumatic pseudoarthrosis in a 13-year-old girl; X-ray one year after left clavicle fracture; extensive periosteal callus is visible, with no bony healing of the fracture.</p></caption><graphic xlink:href="LV-147-223-f1"></graphic></fig>
<p>Autori u ovom radu daju pregled dosada&#x0161;njih spoznaja o uro&#x0111;enoj pseudoartrozi klju&#x010D;ne kosti te razmatraju terapijske postupke.</p>
<sec sec-type="other1">
<title>Razvoj klju&#x010D;ne kosti</title>
<p>Tijekom embriolo&#x0161;kog razvoja klju&#x010D;na kost nastaje i intramembranskim i endohondralnim procesom oko&#x0161;tavanja, &#x0161;to je razli&#x010D;ito od bilo koje druge duge kosti. (<xref ref-type="bibr" rid="r14"><italic>14</italic></xref>) Klju&#x010D;na kost je prva kost koja osificira intrauterino, ujedno i zadnja kojoj se zatvore epifize u &#x017E;ivotu &#x010D;ovjeka. Specifi&#x010D;na je jer se centralni dio klju&#x010D;ne kosti razvija iz dvaju primarnih osifikacijskih centara. Naznake osifikacijskih centara po&#x010D;inju se pojavljivati sredinom &#x010D;etvrtog tjedna gestacije, a dva osifikacijska centra spoje se me&#x0111;usobno krajem sedmog tjedna gestacije. (<xref ref-type="bibr" rid="r15"><italic>15</italic></xref>, <xref ref-type="bibr" rid="r16"><italic>16</italic></xref>) Kasnije tijekom &#x017E;ivota pojavljuju se sekundarni centri oko&#x0161;tavanja u medijalnom i lateralnom dijelu klju&#x010D;ne kosti. Medijalni dio je odgovoran za rast oko 80% ukupne duljine klju&#x010D;ne kosti. U mu&#x0161;ke djece prvi radiolo&#x0161;ki znakovi po&#x010D;etka oko&#x0161;tavanja medijalnog kraja vide se s 14 godina, dok se kod djevoj&#x010D;ica to isto vidi malo ranije, u prosjeku s oko 13 godina. Medijalna epifiza je posljednja u tijelu koja potpuno osificira i spoji se s dijafizom, u prosjeku oko 25. godine &#x017E;ivota (u oba spola). (<xref ref-type="bibr" rid="r17"><italic>17</italic></xref>) Lateralna epifiza potpuno se spoji s dijafizom u prosjeku s oko 20 godina &#x017E;ivota u oba spola. (<xref ref-type="bibr" rid="r18"><italic>18</italic></xref>)</p>
</sec>
<sec sec-type="other2">
<title>Etiologija</title>
<p>Patofiziologija i etiologija nije jasna i postoje mnoge teorije za&#x0161;to dolazi do izostanka spajanja dvaju dijafiznih osifikacijskih centara izme&#x0111;u &#x0161;estog i sedmog tjedna intrauterinog razvoja. (<xref ref-type="bibr" rid="r19"><italic>19</italic></xref>) Jedna teorija govori da nagla&#x0161;ene pulsacije potklju&#x010D;ne arterije mogu uzrokovati povi&#x0161;eni pritisak na klju&#x010D;nu kost te tako dovesti do smetnje razvoja, pseudoartroze. Odre&#x0111;ene anatomske abnormalnosti kao &#x0161;to su vratna rebra ili podignuto prvo rebro dodatno mogu uzrokovati kompresiju arterije izme&#x0111;u prvog rebra i klju&#x010D;ne kosti, &#x0161;to dodatno pridonosi pove&#x0107;anju pritiska potkju&#x010D;ne arterije. (<xref ref-type="bibr" rid="r20"><italic>20</italic></xref>) Ovo tako&#x0111;er obja&#x0161;njava za&#x0161;to su desne &#x010D;e&#x0161;&#x0107;e te za&#x0161;to su ljevostrane &#x010D;esto povezane s dekstrokardijom. Druga teorija govori kako promijenjen intrauterini polo&#x017E;aj fetusa koji kranijalno odi&#x017E;e desnu potklju&#x010D;nu arteriju dovodi do izostanka spajanja osifikacijskih centara. (<xref ref-type="bibr" rid="r21"><italic>21</italic></xref>, <xref ref-type="bibr" rid="r22"><italic>22</italic></xref>) Opisivane su i obiteljske povezanosti, &#x0161;to ukazuje na potencijalnu nasljednost. (<xref ref-type="bibr" rid="r23"><italic>23</italic></xref>)</p>
</sec>
<sec sec-type="other3">
<title>Udru&#x017E;ene anomalije</title>
<p>U oko &#x010D;etiri posto djece s uro&#x0111;enom pseudoatrozom klavikule postoje pridru&#x017E;ene anomalije. Tako je opisano postojanje dodatnoga vratnog rebra, vertikalnih gornjih rebara, visoko polo&#x017E;ena potklju&#x010D;na arterija na strani pseudoartroze te hipertrofirani popre&#x010D;ni nastavci sedmog kralje&#x0161;ka. (<xref ref-type="bibr" rid="r24"><italic>24</italic></xref>) Neka djeca imaju uro&#x0111;eni poreme&#x0107;aj generalnog oko&#x0161;tavanja te su ove anomalije pridru&#x017E;ene genskim sindromima kao &#x0161;to su Ehlers-Danlos, Al-Awadi/Ras-Rothschild, Kabuki i Prader-Willi. (<xref ref-type="bibr" rid="r25"><italic>25</italic></xref>)</p>
</sec>
<sec sec-type="other4">
<title>Klini&#x010D;ka slika</title>
<p>Karakteristi&#x010D;na klini&#x010D;ka slika uro&#x0111;ene pseudoartroze u djece jest postojanost jednostrane otekline iznad srednjeg dijela klju&#x010D;ne kosti koja postoji od ro&#x0111;enja i starenjem se pove&#x0107;ava. U vi&#x0161;e od 90% slu&#x010D;ajeva nalazi se na desnoj strani, iako postoje i ekstremno rijetki slu&#x010D;ajevi kada je oteklina obostrana. Ranija istra&#x017E;ivanja su povezivala ve&#x0107;u u&#x010D;estalost u &#x017E;enskog spola. (<xref ref-type="bibr" rid="r19"><italic>19</italic></xref>) Subjektivno i klini&#x010D;ki nema simptoma. Pokreti u ramenu naj&#x010D;e&#x0161;&#x0107;e su normalni i bezbolni. Zapravo je glavni razlog za&#x0161;to roditelji dovode dijete na pregled estetski, &#x201E;oteklina koja ne prolazi, i pove&#x0107;ava se&#x201C;. Djeca &#x0161;kolske dobi opisuju neugodnosti no&#x0161;enja naramenice &#x0161;kolske torbe i smetnje funkcija ramenog zgloba. (<xref ref-type="bibr" rid="r26"><italic>26</italic></xref>) Osim toga, opisani su slu&#x010D;ajevi progresivne kompresije brahijalnog pleksusa te sindrom gornjeg otvora prsnog ko&#x0161;a, koji su bili indikacija za operacijsko lije&#x010D;enje bilo u dje&#x010D;joj ili kasnije u odrasloj dobi. (<xref ref-type="bibr" rid="r27"><italic>27</italic></xref>) Mogu se javiti parestezije i slabiji osjet dijelova &#x0161;ake i ruke te atrofija ko&#x017E;e iznad otekline. Zbog prevelikog pokreta u samoj pseudoartrozi mo&#x017E;e se prezentirati kao slabost ruke koja zamjetno ometa dijete tijekom puzanja. Udaljenost izme&#x0111;u sternuma i akromiona smanjena je, &#x0161;to je preduvjet razvoja &#x201E;krilaste lopatice&#x201C; (lopatica str&#x0161;i iz le&#x0111;a te stvara privid krila). (<xref ref-type="bibr" rid="r28"><italic>28</italic></xref>) Ograni&#x010D;enja pokreta ramena najizra&#x017E;enija su kod odizanja (abdukcije) ruke. (<xref ref-type="bibr" rid="r29"><italic>29</italic></xref>) S vremenom kompenzatorna promjena biomehanike dovodi do degenerativnih zglobnih promjena, me&#x0111;utim nema radova koji opisuju dugoro&#x010D;no pra&#x0107;enje ovoga klini&#x010D;kog entiteta.</p>
</sec>
<sec sec-type="other5">
<title>Dijagnoza</title>
<p>Rendgenogram klavikule pokazuje o&#x010D;evidnu razdvojenost dvaju dijelova klju&#x010D;ne kosti na srednjem dijelu, naj&#x010D;e&#x0161;&#x0107;e na granici izme&#x0111;u lateralne i srednje tre&#x0107;ine klju&#x010D;ne kosti. Oba dijela imaju vlastiti, ravan i neprekinut korteks. Nema znakova formiranja kalusa ili reaktivnosti. Medijalni dio je gotovo uvijek kranijalnije polo&#x017E;en zbog vlaka mi&#x0161;i&#x0107;a vrata i te&#x017E;ine ruke. Nazo&#x010D;na je deformacija u smislu kutne deformacije, i defekt kosti (<xref ref-type="fig" rid="f2">Figure 2</xref>). Kraj jednog dijela mo&#x017E;e izgledati kao &#x201E;slonovo stopalo&#x201C; &#x2013; gdje je fragment &#x0161;iri na kraju u usporedbi s tijelom, a drugi ima izgled &#x201E;<italic>pencil point</italic>&#x201C; &#x2013; fragment je poja&#x010D;ano tanji prema kraju fragmenta (<xref ref-type="fig" rid="f3">Figure 3</xref>). Medularni kanal je zatvoren i skleroti&#x010D;an. (<xref ref-type="bibr" rid="r30"><italic>30</italic></xref>) Nema ko&#x0161;tanog kalusa. Klju&#x010D;na kost u pseudoartrozi ekstremno rijetko mo&#x017E;e biti i u obliku bifurkacije. (<xref ref-type="bibr" rid="r28"><italic>28</italic></xref>)</p>
<fig id="f2" position="float" fig-type="figure"><label>Figure 2</label><caption><p>Congenital pseudoarthrosis of the right clavicle, a 3-year-old girl; deformation is present in terms of angular deformation (75.6 degrees), and a bone defect of 6 mm in length.</p></caption><graphic xlink:href="LV-147-223-f2"></graphic></fig>
<fig id="f3" position="float" fig-type="figure"><label>Figure 3</label><caption><p>Congenital pseudoarthrosis of the clavicle, a 3-year-old girl; bone defect in the middle third of the clavicle; A &#x2013; lateral end has a &#x201C;pencil point&#x201D; appearance, B &#x2013; medial end has an &#x201C;elephant&#x2019;s foot&#x201D; appearance; closed medullary canals at both ends.</p></caption><graphic xlink:href="LV-147-223-f3"></graphic></fig>
</sec>
<sec sec-type="other6">
<title>Diferencijalna dijagnoza</title>
<p>Kongenitalnu pseudartrozu treba razlikovati od prijeloma klju&#x010D;ne kosti. Nadalje, nekoliko drugih dijagnoza mo&#x017E;e imati sli&#x010D;nu prezentaciju i treba ih uzeti u obzir u diferencijalnoj dijagnozi. Od sistemskih sindroma treba isklju&#x010D;iti neurofibromatozu. Ve&#x0107;ina takve djece ima hiperpigmentirane &#x201E;mrlje od kave&#x201C; na ko&#x017E;i, patognomoni&#x010D;ne za neurofibromatozu. (<xref ref-type="bibr" rid="r21"><italic>21</italic></xref>) Prezentacije prijeloma i kongenitalne pseudoartroze nisu iste iako imaju sli&#x010D;no prikazivanje, pogotovo u neonatusa. Kod njih je ovo pogotovo te&#x0161;ko razlikovati zbog nesuradljivosti i nesigurnosti oko boli. U starije djece lagano se isklju&#x010D;i time &#x0161;to akutna fraktura, za razliku od pseudoartroze, boli na mjestu prijeloma te prilikom pokreta u ramenu. Tako&#x0111;er, rubovi fragmenata kod pseudoartroze bulozni su i skleroti&#x010D;ni, za razliku od frakture gdje su rubovi o&#x0161;tri i nisu skleroti&#x010D;ni. &#x010C;esto kod pseudoartroza postoje i hipoplasti&#x010D;ne promjene uz samu pseudoartrozu, dok kod frakture na kontrolnim snimkama postoji formiranje kalusa. U diferencijaloj dijagnozi va&#x017E;na je dobra anamneza i suradnja roditelja. Izostanak cijeljenja prijeloma, nejasna trauma, pogor&#x0161;anje lokalnog nalaza odnosno previd uro&#x0111;ene pseudoratroze mo&#x017E;e dovesti do neosnovane sumnje na obiteljsko nasilje. Zbog toga je va&#x017E;no za dje&#x010D;jeg kirurga da razumije klini&#x010D;ku sliku i prognozu bolesti, radi ispravne dijagnoze i terapije.</p>
</sec>
<sec sec-type="other7">
<title>Lije&#x010D;enje</title>
<p>Terapija priro&#x0111;ene pseudoartroze klju&#x010D;ne kosti sporna je, dvojbena, osobito jer je opisano i spontano zacijeljenje. (<xref ref-type="bibr" rid="r4"><italic>4</italic></xref>) U ve&#x0107;ini slu&#x010D;ajeva dijete je bez tegoba te je prognoza dobra. Pokretljivost u ramenu nije ograni&#x010D;ena i nema drugih simptoma. U manjeg broja djece postoji bol i funkcionalna ograni&#x010D;enost, a opisani su i sindrom gornjega torakalnog otvora i simptomi kompresije &#x017E;ivaca brahijalnog pleksusa. Kod takve djece indicirano je operacijsko lije&#x010D;enje, no ve&#x0107;inom se zahvati izvode iz estetskih razloga, uvjetovani nelagodom i strahom roditelja. Ve&#x0107;ina studija ishoda lije&#x010D;enja uro&#x0111;ene pseudoartroze klavikule ra&#x0111;ena je na malim uzorcima te je heterogenost lije&#x010D;enja velika. (<xref ref-type="bibr" rid="r23"><italic>23</italic></xref>)</p>
<sec>
<title>Neoperacijsko lije&#x010D;enje</title>
<p>Ve&#x0107;ina djece lije&#x010D;i se samo promatranjem, bez intervencija, osobito ako nemaju simptome niti pate od estetskih tegoba zbog izbo&#x010D;ine. Ishodi nakon neoperativnog lije&#x010D;enja djece op&#x0107;enito su izvrsni; ve&#x0107;ina tijekom adolescencije i kasnije ne osje&#x0107;a bol, nelagodu ili ograni&#x010D;enje raspona pokreta. (<xref ref-type="bibr" rid="r21"><italic>21</italic></xref>)</p>
</sec>
<sec>
<title>Operacijsko lije&#x010D;enje</title>
<p>Indikacije za kirur&#x0161;ko lije&#x010D;enje jesu progresivna bol, funkcionalna ograni&#x010D;enja i kasno nastali sindrom gornjeg otvora prsnog ko&#x0161;a. (<xref ref-type="bibr" rid="r31"><italic>31</italic></xref>) Ipak, ve&#x0107;ina operacija izvodi se iz estetskih razloga, s obzirom na izbo&#x010D;inu koja s vremenom raste. Kirurgija se razmatra i u bolesnika gdje su fragmenti udaljeni vi&#x0161;e od jednog centimetra te postoji zna&#x010D;ajan nesrazmjer sternoakromijalne udaljenosti (<xref ref-type="fig" rid="f4">Figure 4</xref>). Klavikula u pseudoartrozi kra&#x0107;a je od zdrave. Resekcijom ko&#x0161;tanog defekta i krajeva kosti, kako bi se dobili ravni rubovi i otvorio medularni kanal, dolazi do daljnjeg skra&#x0107;enja ukupne du&#x017E;ine klavikule. Poznato je kako pseudoartroze defekta ve&#x0107;eg od jednog centimetra imaju ve&#x0107;u u&#x010D;estalost odgo&#x0111;enog cijeljenja i komplikacija nakon operacije. (<xref ref-type="bibr" rid="r32"><italic>32</italic></xref>) Primjenjuje se nekoliko mogu&#x0107;nosti kirur&#x0161;kog lije&#x010D;enja: resekcija pseudartroze uz kori&#x0161;tenje ko&#x0161;tanog grafta, osteosinteze ili oboje. (<xref ref-type="bibr" rid="r33"><italic>33</italic></xref>) Jednostavna resekcija krajeva pseudoartroze bez stabilizacije dovest &#x0107;e do razvoja boli, nestabilnosti, asimetrije lijevog i desnog ramena te nagr&#x0111;enosti deformacijama kostiju tijekom pokreta ramena. Za stabilizaciju se koriste razli&#x010D;ite tehnike: intramedularna Kirschnerova &#x017E;ica, plo&#x010D;a i vijci, samo vijci, Steinmannov intramedularni &#x010D;avao ili vanjska fiksacija. Naj&#x010D;e&#x0161;&#x0107;e kori&#x0161;teno donorsko mjesto za transplantaciju kosti jest krista ilijake, ali i tibija, rebra i vaskularizirani fibularni transplantati. Postoperativno lije&#x010D;enje uklju&#x010D;uje imobilizaciju Velpeau remenom ili Desaultovim zavojem tijekom &#x010D;etiri do &#x0161;est tjedana, ovisno o &#x017E;ivotnoj dobi djeteta odnosno adolescenta. Ishodi nakon kirur&#x0161;kog lije&#x010D;enja op&#x0107;enito su uspje&#x0161;ni. Bolji su ishodi u slu&#x010D;ajevima s minimalnim ili nikakvim pomakom ulomaka i intaktnim periostom te s upotrebom ko&#x0161;tanog presatka. Komplikacije su vrlo rijetke, ali se ipak doga&#x0111;aju. O&#x017E;iljno tkivo mo&#x017E;e postati hipertrofi&#x010D;no, bolno ili formirati keloid. Naj&#x010D;e&#x0161;&#x0107;a komplikacija je nezarastanje, &#x0161;to je &#x010D;esto indikacija za revizijski kirur&#x0161;ki zahvat. U radu Di Gennara i suradnika 75% operiranih ovom metodom postiglo je spajanje ulomaka klju&#x010D;ne kosti. (<xref ref-type="bibr" rid="r26"><italic>26</italic></xref>) Svi neuspje&#x0161;ni bili su stariji od osam godina i u 75% njih kori&#x0161;ten je alograft. Stoga autori ovog rada ne preporu&#x010D;uju kori&#x0161;tenje alografta. (<xref ref-type="bibr" rid="r26"><italic>26</italic></xref>) Ekstenzivno kori&#x0161;tenje ko&#x0161;tanog transplantata nije potrebno u operaciji djece mla&#x0111;e od &#x010D;etiri godine. (<xref ref-type="bibr" rid="r34"><italic>34</italic></xref>) U djece starije od &#x010D;etiri godine kori&#x0161;tenje ko&#x0161;tanog transplantata se preporu&#x010D;uje, a u onih starijih od osam godina i odraslih &#x010D;ak se predstavlja kao nu&#x017E;no za uspje&#x0161;nost postizanja spajanja krajeva resecirane pseudoartroze. (<xref ref-type="bibr" rid="r35"><italic>35</italic></xref>) Kim i suradnici su opisali kori&#x0161;tenje neresorptivnih &#x0161;avi za fiksaciju ulomaka u operacijama mla&#x0111;ih od 18 mjeseci. (<xref ref-type="bibr" rid="r31"><italic>31</italic></xref>) Iako su opisane komplikacije za sve vrste fiksacijskih metoda, danas najvi&#x0161;e studija favorizira rekonstrukcijske plo&#x010D;ice. Ve&#x0107;ina radova s Kirschnerovim &#x017E;icama datira iz doba prije razvoja zaklju&#x010D;avaju&#x0107;ih plo&#x010D;ica i plo&#x010D;ica za specifi&#x010D;ne anatomske dijelove pojedinih kostiju. (<xref ref-type="bibr" rid="r36"><italic>36</italic></xref>) Noviji radovi preferiraju kori&#x0161;tenje zaklju&#x010D;avaju&#x0107;ih plo&#x010D;ica jer su pacijenti lije&#x010D;eni s rekonstrukcijskom plo&#x010D;icom prije postigli spajanje rubova resecirane pseudoartoze, u prosjeku za tri mjeseca, u usporedbi sa &#x0161;est mjeseci u onih gdje su kori&#x0161;tene Kirschnerove &#x017E;ice. (<xref ref-type="bibr" rid="r37"><italic>37</italic></xref>) Suprotno navedenom, druga skupina autora preferira Kirschnerove &#x017E;ice jer su u jednom slu&#x010D;aju zabilje&#x017E;ili te&#x0161;ku komplikaciju infekcijom kod kori&#x0161;tenja plo&#x010D;ica. Uspje&#x0161;no spajanje ko&#x0161;tanih ulomaka nakon operacije posti&#x017E;e se u otprilike 88% operirane djece, bez obzira na dob. (<xref ref-type="bibr" rid="r37"><italic>37</italic></xref>)</p>
<fig id="f4" position="float" fig-type="figure"><label>Figure 4</label><caption><p>Congenital pseudoarthrosis of the right clavicle in a 9-year-old girl, medial part in ventral position; absence of periosteal callus, sclerotic edges of falsely articulated parts of the bone; right clavicle shorter by 6 mm; the bone defect of congenital pseudoarthrosis is 6 mm.</p></caption><graphic xlink:href="LV-147-223-f4"></graphic></fig>
</sec>
<sec>
<title>Komplikacije operacijskog lije&#x010D;enja</title>
<p>Generalno, komplikacije ove operacije su rijetke, a naj&#x010D;e&#x0161;&#x0107;e su prolazna postoperativna bol i slabost ruke operirane strane. Mo&#x017E;e do&#x0107;i do pucanja osteosintetskog materijala, nespajanja ulomaka, infekcije ili o&#x0161;te&#x0107;enja &#x017E;ivaca brahijalnog spleta. U ve&#x0107;ini tih te&#x017E;ih komplikacija bit &#x0107;e potrebna nova operacija. Razmak izme&#x0111;u fragmenata prije operacije ve&#x0107;i od jednog centimetra ima ve&#x0107;u incidenciju nespajanja i komplikacija nakon operacije. Pacijent i roditelji trebaju biti upozoreni da &#x0107;e na mjestu deformacije odnosno operacije nastati o&#x017E;iljak, kao i na mjestu uzimanja ko&#x0161;tanog transplantata.</p>
</sec>
<sec>
<title>Vrijeme operacije / <italic>timing</italic></title>
<p>Postoje razli&#x010D;ita mi&#x0161;ljenja o idealnom vremenu operacije. Mi&#x0161;ljenje je da je bolje napraviti zahvat u ranijoj dobi, najidealnije izme&#x0111;u tre&#x0107;e i &#x0161;este godine &#x017E;ivota (zbog najmanje u&#x010D;estalosti komplikacija). (<xref ref-type="bibr" rid="r38"><italic>38</italic></xref>, <xref ref-type="bibr" rid="r39"><italic>39</italic></xref>) Naime, nespajanje ulomaka &#x010D;e&#x0161;&#x0107;e se javljalo u djece koja su operirana u dobi prije 18 mjeseci te u starijih od osam godina. U starije djece u pravilu je razmak izme&#x0111;u kostiju ve&#x0107;i, a cijeljenje du&#x017E;e traje. Transplantacija kosti mo&#x017E;e se napraviti u bilo kojoj dobi, me&#x0111;utim, &#x0161;to je pacijent stariji to je potreban ve&#x0107;i transplantat te je time zahvat kompleksniji. (<xref ref-type="bibr" rid="r40"><italic>40</italic></xref>)</p>
</sec>
</sec>
<sec sec-type="other8">
<title>Zaklju&#x010D;ak</title>
<p>Prijelome klju&#x010D;ne kosti i uro&#x0111;enu pseudartrozu te&#x0161;ko je razlikovati pri prvom pregledu, posebno u djece tijekom prvih godina &#x017E;ivota. Iako je uro&#x0111;ena pseudartroza klju&#x010D;ne kosti rijetka, valja ju imati na umu. Ako nisu lije&#x010D;ene u pred&#x0161;kolskoj dobi, lije&#x010D;enje u kasnijoj dobi ima ve&#x0107;u pojavnost lo&#x0161;ih ishoda. Unato&#x010D; ote&#x017E;anoj dijagnozi, treba razlikovati uro&#x0111;ene od ste&#x010D;enih pseudoartoza. Ste&#x010D;ene nastaju nakon traume, prvotno je deformacija osjetljiva na palpaciju. Kretnje ramena su ograni&#x010D;ene bolovima. Ako se kroz vi&#x0161;emjese&#x010D;ni period izbo&#x010D;ina ne smanjuje, a radiolo&#x0161;ki nalaz ukazuje na izostanak cijeljenja kosti, mo&#x017E;emo sumnjati u nastanak ste&#x010D;ene pseudoartroze. Kod uro&#x0111;ene pseudartroze klju&#x010D;ne kosti izbo&#x010D;ina je bezbolna i vidljiva je po ro&#x0111;enju, s vremenom se pove&#x0107;ava, a kretnje su bezbolne. U ve&#x0107;ini slu&#x010D;ajeva posve je asimptomatska. Ste&#x010D;ene i priro&#x0111;ene pseudoartroze mogu se lije&#x010D;iti operativno ili neoperativno. Za uro&#x0111;enu pseudartrozu kirur&#x0161;ko lije&#x010D;enje dolazi u obzir u slu&#x010D;ajevima progresivne boli, funkcionalnog ograni&#x010D;enja i kasnog nastupa sindroma gornjeg otvora prsnog ko&#x0161;a. Me&#x0111;utim, ve&#x0107;ina operacija priro&#x0111;ene pseudoartroze klju&#x010D;ne kosti izvodi se iz estetskih razloga. Mogu&#x0107;e rizike i koristi operativnog i neoperativnog lije&#x010D;enja treba razmotriti s djetetom i roditeljima.</p>
</sec>
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<p>Autori nisu deklarirali sukob interesa relevantan za ovaj rad.</p>
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<p content-type="fn-title">INFORMACIJA O FINANCIRANJU</p>
<p>Za ovaj &#x010D;lanak nisu primljena financijska sredstva.</p>
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