The Association of Chiari Type I Malformation and Neurofibromatosis Type 1

1993 ◽  
Vol 32 (3) ◽  
pp. 189-190 ◽  
Author(s):  
Joseph Dooley ◽  
Daniel Vaughan ◽  
Michael Riding ◽  
Peter Camfield
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Chiari Malformation ◽  
Neurofibromatosis Type ◽  
Foramen Magnum ◽  
Type I ◽  
Chiari Type ◽  
Chiari Type I Malformation ◽  
Chiari Type I ◽  
Chiari Malformations

The association of neurofibromatosis type 1 (NF1) with Chiari malformations of the cerebellum and brain stem has been reported on only two previous occasions.1,2 The pathogenesis of both conditions has remained unclear, although the Chiari type I malformation is most likely due to hypoplasia of the posterior fossa with subsequent extension of the cerebellum through the foramen magnum.3 NF1 is also associated with a variety of cerebral dysplasias.4 We present a patient with both of these dysplastic lesions whose Chiari malformation was asymptomatic.

Trzyipółletni chłopiec z malformacją Chiariego typu I – opis przypadku

2017 ◽  
Vol 21 (3) ◽  
Author(s):  
Anna Worch ◽  
Małgorzata Wielopolska
Keyword(s):  
Chiari Malformation ◽  
Foramen Magnum ◽  
Common Type ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Chiari Type I Malformation ◽  
Large Head ◽  
Exacerbation Of Symptoms ◽  
Chiari Type I ◽  
Cerebellar Tonsils

Chiari malformation is the most common structural defect of the posterior fossa and cerebellum, consisting of displacements of hindbrain inclination through the foramen magnum, often associated with syringomyelia. In the most common type I of this disease, only the cerebellar tonsils are descended. Chiari type I malformation may remain asymptomatic in early childhood, however a number of unspecific symptoms, such as: neck pains and headaches, vertigos, balance and sight disorders, may strengthen with age. Because of unspecificity and various exacerbation of symptoms the disease is often misdiagnosed or undiagnosed. We describe a case of a 3.5-year old boy with whom his mother visited a pediatrician because of a chronic cough. During the examination mother of the patient informed that all infections start with vomiting and the patient is not able to stand up from horizontal position without turning first to the side and then to the abdomen. During the examination, a large head circumference was noticed (56 cm, > 97 percentile). MRI showed elongated cerebellar tonsils descended below the foramen magnum (left 14 mm, right 11 mm). Chiari malformation type I with associated syringomyelia was diagnosed. The patient was reffered to neurosurgery consultation.

scholarly journals Traumatic Transient Herniation Concomitant with Tonsillar Hemorrhagic Contusion in a Child

2017 ◽  
Vol 5 (6) ◽  
pp. 771-773 ◽  
Author(s):  
Ahmet Öğrenci ◽  
Orkun Koban ◽  
Murat Ekşi ◽  
Onur Yaman ◽  
Sedat Dalbayrak
Keyword(s):  
Foramen Magnum ◽  
Type I ◽  
Tonsillar Ectopia ◽  
Chiari Type ◽  
Chiari Type I Malformation ◽  
Pediatric Age Group ◽  
Downward Displacement ◽  
The Relationship

Downward displacement of cerebellar tonsils more than 5 mm below the foramen magnum is named as Chiari type I malformation and named benign tonsillar ectopia if herniation is less than 3 mm. It does not just depend on congenital causes. There are also some reasons for acquired Chiari Type 1 and benign tonsillar ectopia/herniation. Trauma is one of them. Trauma may increase tonsillar ectopia or may be the cause of new-onset Chiari type 1. The relationship between the tonsil contusion and its position is unclear. We present a case of pediatric age group with tonsillar herniation with a hemorrhagic contusion. Only 1 case has been presented so far in the literature. A case with unilateral tonsil contusion has not been presented to date. We will discuss the possible reasons for taking the place of the tonsils to the above level of the foramen magnum in the follow-up period, by looking at the literature.

scholarly journals Improvement of syrinx resolution after tonsillar cautery in pediatric patients with Chiari Type I malformation

2016 ◽  
Vol 17 (2) ◽  
pp. 174-181 ◽  
Author(s):  
Kevin M. Stanko ◽  
Young M. Lee ◽  
Jennifer Rios ◽  
Adela Wu ◽  
Giovanna W. Sobrinho ◽  
...  
Keyword(s):  
Perioperative Complications ◽  
Binary Logistic Regression ◽  
Foramen Magnum ◽  
Type I ◽  
Binary Logistic Regression Model ◽  
Cerebrospinal Fluid Flow ◽  
Chiari Type ◽  
Chiari Type I Malformation ◽  
Surgical History ◽  
Chiari Type I

OBJECT Chiari Type I malformation involves caudal displacement of the cerebellar tonsils below the foramen magnum, which obstructs normal cerebrospinal fluid flow and increases intracranial pressure. Certain aspects of its surgical treatment remain controversial. A retrospective study was conducted to assess the efficacy of tonsillar cautery on syrinx resolution among pediatric Chiari patients undergoing cervicomedullary decompression. METHODS A retrospective cohort study was performed for patients 0–18 years of age who underwent surgical correction for Chiari Type I malformation with syrinx between 1995 and 2013. Basic demographic information was collected as well as data for preoperative symptoms, prior surgical history, perioperative characteristics, and postsurgical outcomes. Descriptive statistics were performed in addition to bivariate analyses. Candidate predictor variables were identified based on an association with tonsillar cautery with p < 0.10. Forward stepwise likelihood ratio was used to select candidate predictors in a binary logistic regression model (Pin = 0.05, Pout = 0.10) most strongly associated with the outcome. RESULTS A total of 171 patients with Chiari Type I malformation with syrinx were identified, and 43 underwent tonsillar cautery. Patients who underwent tonsillar cautery had 6.11 times greater odds of improvement in their syrinx (95% CI 2.57–14.49, p < 0.001). There was no effect of tonsillar cautery on increased perioperative complications as well as the need for repeat decompressions. CONCLUSIONS Tonsillar cautery is safe and effective in the treatment of Chiari Type I malformation with syrinx and may decrease time to syrinx resolution after cervicomedullary decompression. Tonsillar cautery does not increase postoperative complications in pediatric Chiari Type I malformation patients.

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