SPINALNI DISRAFIZAM – NEUROHIRURŠKI PROBLEM DEČIJEG UZRASTA

Autori

  • Mirjana Raičević Univerzitetska dečja klinika, Beograd, Srbija
  • Milan Mrdak Univerzitetska dečja klinika, Beograd, Srbija
  • Maša Radovanović Univerzitetska dečja klinika, Beograd, Srbija
  • Srđan Nikolovski Univerzitetska dečja klinika, Beograd, Srbija

DOI:

https://doi.org/10.46793/PP170305009R

Ključne reči:

spina bifida, defekt neuroalne tube, spinalni dizrafizam, tethered cord sindrom

Apstrakt

Spina bifida je jedna od najkompleksnijih i najtežih urođenih anomalija. Raniji naziv spina bifida danas je potisnut terminima „defekt neuralne tube“ (NTDs) i „spinalni dizrafizam“ koji mnogo bolje  determinišu njenu suštinu. U osnovi ove anomalije je neadekvatno „zatvaranje  –  srastanje srednje linije„  kičmenog stuba te je termin spinalni dizradfizam najadekvatnji. Defekt srastanja srednje linije može nastati u različitim nivoima kičmenog kanala te govorimo o  cervikalnom, torakalnom, lumbalnom i sakralnom spinalnom disrafizmu. Spinalni disrafizam se javlja u dva osnovna entiteta – otvoreni i zatvoreni, koji se suštinski razlikuju. Incidenca  ove anomalije u svetu pokazuje geografsku i etničku osobenost – najčešća je kod keltskih naroda (12,5 na 10.000 ž.r.), a retka je kod azijata i u crnačkoj populaciji. Devojčice češće obolevaju nego dečaci. Etiologija je još uvek nepoznata. Dijagnoza otvorenog spinalnog dizrafizma  se mora postaviti prenatalno. Otvorenu spinu treba operisati  u prvih 48h po rođenju, pogotovu ako je rupturirana. U svetu postoje medicinski centri gde se primenjuje prenatalni – fetalni  hirurški zahvat. Benefit ovakvih hirurških zahvata još uvek nije  dokazan. Zatvoreni spinalni dizrafizam je veoma  podmukla, prikrivena anomalija, te stoga i naziv okultni spinalni dizrafizam (OSD). Incidenca ove anomalije je nepoznata, zna se samo da je češća kod ženske dece. Etiologija je takođe nepoznata. Dijagnozu OSD je praktično nemoguće postaviti prenatalno. Izuzetno važan problem u sklopu spinalnog disrafizma je „tethered cord sy“ koji označava stanje u kome je kičmena moždina pričvršćena za neku nepokretnu strukturu kao što je dura, pršljen, lipom, koža. Lečenje spinalnog disrafizma je izuzetno kompleksno i zahteva multidisciplinarni pristup.

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