Djamila Kafoufi Al Galaa Military Hospital Cairo

Transcription

1 Djamila Kafoufi Al Galaa Military Hospital Cairo

2 Herniation cerebellar tonsils below the foramen magnum, Hans Chiari 4 types Chiari I less than 5mm,HDC rare,syringomyelia often present. Chiari II,protrusion cerebellar vermis, medulla and V4+supratentorial anomalies,almost always myelomeningocele(prenatal diagnosis)+hdc+spina Bifida Chiari III, very rare,prolaps cerebellum+cervical spina bifida Chiari IV, cerebella hypoplasia/aplasia,hindbrain malformation. Chiari 0,no herniation but crowded PF and Blocked CSF(3,7%) Chiari 1,5 recently introduce=chiari1+caudal displacement of the obex. Chiari 5 recently proposed,absence Cerebellar +herniation occipital brain bellow the FM

3 Tubbs and al.stated no single theory could explain all forms of the chiari and might be a hetegenous entity Symptoms are due to Traction/compression of neural structures/blood vessels and obstruction of normal CSF pulsation.

4 Prevalence of asymptomatic Chiari I in pediatric population is unknown, Pediatric population with MRI for symptoms related to Chiari/others diagnosis=1% Mixed pediatric and adult=0,77% Family cases of pediatric Chiari I=3%

5 Chiari 0,1and 1,5: headach, neck pain and dizziness.exagerated by valsalva maneuvers,cough,sneezing. Cerebellar signs as dysmetria,nystagmus,diplopia, in28% cerebellar fit as drop attacks Brainstem dysfonction:cranial nerves dysfunction. Scoliosis 30 to 60% Polysomnography:central apnea more than 3 with desaturation.

6 CT, McRae line(basion-opisthion) MRI,study of choice+csf Flow sequences More 5mm is a cutoff point for chiari

7 Symptomatic Chiari I Asymtomatic CI+Syringomyelia Asymptomatic CI+sleep apnea dysorder more than 3 central Scoliosis+CI>suboccipital decompression Ant.compression>ant.odontoidectomy followed post.decompression.

8 Suboccipital decompressiona+c1lamina +no dural opening+removal of the ligament. Bony decompression +duroplasty>lower rate reoperation>higher risk CSF leack (18 vs 2%) Preservation of the arachnoid

9 Crouzon 70% Pfeiffer 50% High incidence with sleep central apnea Non syndromic 5,6% Remodeling cranial vault improve tonsilar herniation.

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11 Present in up 28% Radiologic signs=flattening of the post sclera,distension perioptic subarachnoid space, partial empty sella TRT=Vpshunt+suboccipital decompression.

12 Radioth.to the skull base affects the growth of the PF and especially the development of the clivus, Aquillina and al.(2009)showed that the max herniation is around 20 months postrt. TRT=conservative management is emphasized.

13 Developped secondary to lumboperitoneal shunting(communicated HDC/benign ICH). Small cranial volum due to skull thickening or arrested post fossa growth. TRT=cranial vault enlargement rather than the occipital decompression.

14 HDC in 9,8%(Loukas and Al.) Both pathology caused by stenosis jugular foramina,pf croding obstructionof Lushka &Magendie or associated with craniosynostosis. TRT HDC 1 st by ETV

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16 Tethering is associated to Chiari in 14% Sphincter dysfunction,foot deformities,low back pain. Surgery of untethering 1 st Myelomeningocele+VP shunt>revision 1st

17 Approx.1/3 are symptomatic Lower nerves dysfunction: apnea, dysphagia, aspiration, sleep disorders. Spinal cord compression(spasticity,paresis). Syringomyelia, Scoliosis TRT of choice=cervical laminectomy to decompress the spinal cord without dura plasty>60% remain stable.

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19 10 th day post op and wound chek Return to school 2 to 3 weeks post op Physical activity after 6 weeks MRI 3 and 6 months(reduction of the herniation) No contact sport for non operated Chiari

20 Favorable clinical FU up to 91% Complete resolution of the symptoms in 70% Scoliosis might progress in 48% in post decompression.

21 Unpredictible natural history Lack of specific signs & symptoms Well identification of the symptomatology due to chiari Clinical research clarify in the future what we are enable to understand now.

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