Management of Chiari I deformity in Children and Adolescents: A report from the Consensus Taskforce of the Brazilian Society of Pediatric Neurosurgery

Abstract

Much controversy remains on the current management of Chiari I deformity (CID) in children, with many clinical, surgical and ethic-legal implications. The Brazilian Society of Pediatric Neurosurgery (SBNPed, in the Portuguese acronym) has put together a panel of experts to analyze updated published data on the medical literature about this matter and come up with several recommendations for pediatric neurosurgeons and allied health professionals when dealing with CID. Their conclusions are reported herein, along with the respective scientific background.

Research process, recap, and prediction of Chiari malformation based on bicentennial history of nomenclature and terms misuse

“…In 2017, Gadde et al 43 44 advocated the use of the Chiari Deformity to completely displace the "old terminology" of Malformation in a monograph. In 2020, the Brazilian Society of Pediatric Neurosurgery (BSPN) 45 was the rst to recommend the use of Chiari Deformity instead of Chiari Malformation.…”

“…In 2020, by consensus, the Brazilian Society of Pediatric Neurosurgery (BSPN) de ned Secondary Chiari as the herniation caused by abnormal brain expansion or reduction of the posterior cranial fossa volume. 45 Iatrogenic Chiari Malformation term Iatrogenic Chiari Malformation emerged later to describe cerebrospinal uid leaks form canalis spinalis 141 or secondary submandibular herniation due to lumboperitoneal shunt 142 . Although this term is still in common clinical usage today, the terminology Iatrogenic Chiari is not used as much because most scholars use "Iatrogenic tonsillar herniation" directly.…”

“…In 2018, Raybaud et al 155 suggested using the term Primary to describe cases with tonsillar dislocation due to a developmentally abnormal bony craniocervical junction (either enchondral growth abnormality or mis-segmentation). In 2020, the Brazilian Society of Pediatric Neurosurgery (BSPN) 45 de ned this term as Chiari cases caused by abnormal growth or missegmentation of craniocervical junction. In the same year, Faizutdinova et al 82 used the term to describe tonsillar herniation with a small posterior fossa.…”

Anatomoclinical correlation and main radiological findings in a patient affected by Arnold Chiari's malformation: a review

“…Type II is configured as the most common in the entire world population and happens when there is also herniation of the cerebellar tonsils, besides, there is herniation of the cerebellar worm and displacement of the ventricle IV; In type III, there is the herniation of the cerebellum or brainstem bringing in most of the times a high cervical meningocele; in type IV, there is little cerebellar development, being important to point out that there is no herniation in this case (Brock et al, 2017;Brito, 2019;Santos et al, 2020).…”

Papilledema and Chiari I in childhood: case report

“…Chiari malformation was initially described in the late 19th century by pathologists Julius Arnold and Hans Chiari (1851Chiari ( -1916 [1] and can be classified in grades from 0 to IV, with Chiari malformation type I (CM-I) being the most prevalent [2,3,4,5,6]. The main clinical manifestations described in CM-I are occipital headache associated with cough or Valsalva maneuver, respiratory disorders, truncal ataxia, corticospinal tract dysfunctions, and scoliosis [3,5]. Papilledema and visual complaints are rarely reported, especially in the pediatric group, and their presence is an indication for urgent posterior fossa decompression (PFD) [7,8,9,10,11].…”