Surgical treatment of Arnold-Chiari malformation type I in an adult patient

Abstract

Background. Herniation of the cerebellar tonsils through the foramen magnum into the cervical spinal canal with obliteration of the cerebellomedullary cistern is the primary feature of Arnold-Chiari type I malformation (ACM I). It is considered to be congenital malformation, although there have been reported cases of an acquired form. Case report. We presented a female patient, 45-year old, with ACM I without syringomyelia as a rare and unusual clinical image, as well as the effect of decompressive surgery in the treatment of this malformation. The patient was admitted to the Department of Neurosurgery with clinical signs of truncal ataxia worsening during the last six years. Moderate quadriparesis with predominant lower extremity involvement and the signs of the cranial nerves damages occured during the last seven months before admission, with progressive clinical course up to the date of admission. Neurosurgical treatment that included suboccipital medial craniectomy with resection of posterior arch C1 vertebrae and C2 laminectomy resulted in a significant clinical improvement and a much better quality of life. Conclusion. Posterior craniovertebral decompression with microsurgical reduction of the cerebellar tonsils and placement of an artificial dural graft is a treatment of choice in severe forms of ACM I without syringomyelia. .Uvod. Hernijacija tonzila cerebeluma kroz foramen magnum u cervikalni spinalni kanal uz obliteraciju cerebelomedularne cisterne glavna je karakteristika Arnold-Chiari malformacije tip I (ACM I). Radi se o malformaciji kongenitalnog porekla, mada su opisani i sporadicni, steceni oblici. Prikaz bolesnika. U radu je prikazana bolesnica stara 45 godina, sa ACM I bez siringomijelije koja se prezentovala retkom i neobicnom klinickom slikom i nuklearnom magnetnom rezonancijom (MR) mozga, kao i efekat dekompresivne hirurgije u terapiji ove malformacije. Bolesnica je primljena na Odeljenje neurohirurgije sa izrazenom trunkalnom ataksijom koja je nastala i pogorsavala se tokom sest godina. Kvadripareza umereno teskog stepena sa vecom zahvacenoscu donjih ekstremiteta i znaci ostecenja kranijalnih nerava pojavili su se sedam meseci pre prijema, sa postepenim pogorsanjem do dana prijema. Posle MR pregleda na kome je vidjena tonzilarna hernijacija teskog stepena, uradjena je subokcipitalna medijalna kraniektomija sa resekcijom luka atlasa i laminektomijom C2 sto je kod bolesnice dovelo do znacajnog klinickog poboljsanja i kvalitetnijeg zivota. Zakljucak. Dekompresivna hirurska intervencija u vidu subokcipitalne medijalne kraniektomije sa resekcijom luka atlasa i po potrebi laminektomijom C2, uz mikrohurursku resekciju vrha tonzila i plastiku dure graftom, predstavlja metodu izbora u lecenju teskih oblika ACM I bez siringomijelije. .