Adult Arnold-Chiari Malformation and Intrinsic Brain Stem Neoplasm: A Difficult Differential Diagnosis

Neurosurgery ◽  
1983 ◽  
Vol 13 (4) ◽  
pp. 345-350 ◽  
Author(s):  
Timothy W. Phillips ◽  
John E. McGillicuddy ◽  
Julian T. Hoff ◽  
Joseph Latack
Keyword(s):  
Differential Diagnosis ◽  
Brain Stem ◽  
Posterior Fossa ◽  
Chiari Malformation ◽  
Clinical Course ◽  
Posterior Fossa Decompression ◽  
Computed Tomographic ◽  
Arnold Chiari Malformation ◽  
Previously Treated

Abstract The occurrence of a brain stem neoplasm in three adults previously treated for an adult Arnold-Chiari malformation is reported. The diagnosis of adult Arnold-Chiari malformation had been confirmed by angiography and computed tomographic scanning. All patients were treated with a combination of ventricular shunts and posterior fossa decompression. The diagnosis of brain stem neoplasm was not made until late in the clinical course, and all patients died within 2 years of the onset of symptoms.

Absent Hypoxic and Hypercapneic Arousal Responses in Children With Myelomeningocele and Apnea

PEDIATRICS ◽  
1986 ◽  
Vol 78 (1) ◽  
pp. 44-50 ◽  
Author(s):  
Sally L. Davidson Ward ◽  
Bruce G. Nickerson ◽  
Andre van der Hal ◽  
Antonio M. Rodriguez ◽  
Robert A. Jacobs ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Posterior Fossa Decompression ◽  
Quiet Sleep ◽  
Arnold Chiari Malformation ◽  
Healthy Control ◽  
End Tidal

Hypoxic and hypercapneic arousal responses from quiet sleep were tested in seven infants with myelomeningocele and Arnold-Chiari malformation who were symptomatic with apnea and/or hypoventilation. All infants with myelomeningocele required tracheostomy and posterior fossa decompression. Responses were compared with those of nine healthy control infants. To assess hypoxic arousal, inspired Po2 was decreased until the end-tidal (alveolar) Po2 reached 45 mm Hg for a maximum of three minutes. Eleven studies were performed in seven infants with myelomeningocele, and arousal occurred in only two studies (18.2%). Eight of nine control infants aroused to hypoxia (89%). To test hypercapneic arousal, inspired Pco2 was increased until end-tidal Pco2 reached 60 mm Hg for a maximum of three minutes. Eight studies were performed on six infants with myelomeningocele, and arousal occurred in three studies (37.5%). All seven control infants studied aroused to hypercapnea (100%). Three infants with myelomeningocele subsequently died. Infants with myelomeningocele, Arnold-Chiari malformation, and apnea or hypoventilation have arousal deficits to respiratory stimuli.

Bilateral Internuclear Ophthalmoplegia Due to Hydrocephalus: A Case Report

Neurosurgery ◽  
1985 ◽  
Vol 17 (5) ◽  
pp. 822-825 ◽  
Author(s):  
Tomoyuki Nishizaki ◽  
Norihiko Tamaki ◽  
Yoshimitsu Nishida ◽  
Satoshi Matsumoto
Keyword(s):  
Case Report ◽  
Brain Stem ◽  
Chiari Malformation ◽  
Intraventricular Pressure ◽  
Internuclear Ophthalmoplegia ◽  
Pressure Monitoring ◽  
Computed Tomographic ◽  
Arnold Chiari Malformation ◽  
Ventriculoperitoneal Shunting ◽  
Arrested Hydrocephalus

Abstract We report a case of bilateral internuclear ophthalmoplegia due to hydrocephalus associated with Arnold-Chiari malformation. The bilateral internuclear ophthalmoplegia in this case was probably caused by brain stem displacement due to hydrocephalus. This patient was considered to have “arrested” hydrocephalus on the basis of the clinical and computed tomographic findings and the results of the continuous intraventricular pressure monitoring. However, the bilateral internuclear ophthalmoplegia gradually improved after ventriculoperitoneal shunting.

Arnold-Chiari malformation

1983 ◽  
Vol 58 (2) ◽  
pp. 183-187 ◽  
Author(s):  
Kamal S. Paul ◽  
Richard H. Lye ◽  
F. Alexander Strang ◽  
John Dutton
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Significant Proportion ◽  
Foramen Magnum ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Cerebellar Syndrome ◽  
Adult Type ◽  
Arnold Chiari Malformation ◽  
Central Cord Syndrome

✓ The natural history of symptomatic adult Type I Arnold-Chiari malformation (ACM) is variable, and the value of surgery in the management of this disease is difficult to assess. A series of 71 patients in whom a diagnosis of Type I ACM was confirmed at operation is presented, and the progress of the patients following posterior fossa decompression is analyzed. The length of history varied greatly. Pain was the commonest symptom (69% of patients); other symptoms included weakness (56%), numbness (52%), and unsteadiness (40%). The presenting physical signs consisted of a foramen magnum compression syndrome (22%), central cord syndrome (65%), or a cerebellar syndrome (11%). Myelography was performed in 69 patients, and was the most useful investigation. Only 23% of plain radiographs were abnormal. In addition to tonsillar descent, the operative findings included arachnoid adhesions (41%) and syringomyelia (32%). All patients underwent suboccipital craniectomy and C1–3 laminectomy. Respiratory depression was the most frequent postoperative complication (14%), and one patient died from sleep apnea. Early postoperative improvement of both symptoms (82%) and signs (70%) was followed by later relapse in 21% of patients, showing an initial benefit following surgery. None of the patients with a cerebellar syndrome deteriorated, whereas 56% of patients with evidence of foramen magnum compression and 66% of those with a central cord syndrome maintained their initial improvement. The authors conclude that posterior fossa decompression appears to benefit some patients, although a significant proportion might be expected to relapse within 2 to 3 years after operation, depending upon the presenting syndrome.

Absent Hypoxic and Hypercapnic Arousal Responses in Children With Myelomeningocele and Apnea

PEDIATRICS ◽  
1987 ◽  
Vol 79 (2) ◽  
pp. 313-313
Author(s):  
SALLY L. DAVIDSON WARD ◽  
THOMAS G. KEENS
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Decompression Surgery ◽  
Posterior Fossa Decompression ◽  
Mechanical Compression ◽  
Arnold Chiari Malformation ◽  
Before And After ◽  
Cns Anomalies

In Reply.— CNS anomalies associated with myelomeningocele and Arnold-Chiari malformation (Neurosurgery 1986;18:559-564) could account for the abnormal arousal responses we described (Pediatrics 1986;78:44-50). They may also explain the failure of posterior fossa decompression surgery to correct apnea in some patients with myelomeningocele. We were not able to study children before and after posterior fossa decompression surgery; nor did we examine postmortem specimens. Consequently, our paper does not determine whether the cause of abnormal arousal responses is due to mechanical compression or anatomical anomalies of the brainstem.

Radiological and clinical associations with scoliosis outcomes after posterior fossa decompression in patients with Chiari malformation and syrinx from the Park-Reeves Syringomyelia Research Consortium

2020 ◽  
Vol 26 (1) ◽  
pp. 53-59 ◽  
Author(s):  
Jennifer M. Strahle ◽  
Rukayat Taiwo ◽  
Christine Averill ◽  
James Torner ◽  
Jordan I. Gewirtz ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Craniocervical Junction ◽  
Curve Progression ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Younger Age ◽  
Curve Magnitude ◽  
The Mean

OBJECTIVEIn patients with Chiari malformation type I (CM-I) and a syrinx who also have scoliosis, clinical and radiological predictors of curve regression after posterior fossa decompression are not well known. Prior reports indicate that age younger than 10 years and a curve magnitude < 35° are favorable predictors of curve regression following surgery. The aim of this study was to determine baseline radiological factors, including craniocervical junction alignment, that might predict curve stability or improvement after posterior fossa decompression.METHODSA large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and a syrinx (≥ 3 mm in width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°) in patients who underwent posterior fossa decompression and who also had follow-up imaging.RESULTSOf 825 patients with CM-I and a syrinx, 251 (30.4%) were noted to have scoliosis present at the time of diagnosis. Forty-one (16.3%) of these patients underwent posterior fossa decompression and had follow-up imaging to assess for scoliosis. Twenty-three patients (56%) were female, the mean age at time of CM-I decompression was 10.0 years, and the mean follow-up duration was 1.3 years. Nine patients (22%) had stable curves, 16 (39%) showed improvement (> 5°), and 16 (39%) displayed curve progression (> 5°) during the follow-up period. Younger age at the time of decompression was associated with improvement in curve magnitude; for those with curves of ≤ 35°, 17% of patients younger than 10 years of age had curve progression compared with 64% of those 10 years of age or older (p = 0.008). There was no difference by age for those with curves > 35°. Tonsil position, baseline syrinx dimensions, and change in syrinx size were not associated with the change in curve magnitude. There was no difference in progression after surgery in patients who were also treated with a brace compared to those who were not treated with a brace for scoliosis.CONCLUSIONSIn this cohort of patients with CM-I, a syrinx, and scoliosis, younger age at the time of decompression was associated with improvement in curve magnitude following surgery, especially in patients younger than 10 years of age with curves of ≤ 35°. Baseline tonsil position, syrinx dimensions, frontooccipital horn ratio, and craniocervical junction morphology were not associated with changes in curve magnitude after surgery.

scholarly journals Syrinx resolution after posterior fossa decompression in patients with scoliosis secondary to Chiari malformation type I

2011 ◽  
Vol 21 (6) ◽  
pp. 1143-1150 ◽  
Author(s):  
Tao Wu ◽  
Zezhang Zhu ◽  
Jian Jiang ◽  
Xin Zheng ◽  
Xu Sun ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I

Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation Type I in pediatric patients: a meta-analysis

2008 ◽  
Vol 2 (1) ◽  
pp. 42-49 ◽  
Author(s):  
Susan R. Durham ◽  
Kristina Fjeld-Olenec
Keyword(s):  
Cerebrospinal Fluid ◽  
Surgical Treatment ◽  
Clinical Improvement ◽  
Posterior Fossa ◽  
Chiari Malformation ◽  
Meta Analysis ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Patient Age

Object Surgery for Chiari malformation Type I (CM-I) is one of the most common neurosurgical procedures performed in children, although there is clearly no consensus among practitioners about which surgical method is preferred. The objective of this meta-analysis was to compare the outcome of posterior fossa decompression with duraplasty (PFDD) and posterior fossa decompression without duraplasty (PFD) for the treatment of CM-I in children. Methods The authors searched Medline–Ovid, The Cochrane Library, and the conference proceedings of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons (2000–2007) for studies meeting the following inclusion criteria: 1) surgical treatment of CM-I; 2) surgical techniques of PFD and PFDD being reported in a single cohort; and 3) patient age < 18 years. Results Five retrospective and 2 prospective cohort studies involving a total of 582 patients met the criteria for inclusion in the meta-analysis. Of the 582 patients, 316 were treated with PFDD and 266 were treated with PFD alone. Patient age ranged from 6 months to 18 years. Patients undergoing PFDD had a significantly lower reoperation rate (2.1 vs 12.6%, risk ratio [RR] 0.23, 95% confidence interval [CI] 0.08–0.69) and a higher rate of cerebrospinal fluid–related complications (18.5 vs 1.8%, RR 7.64, 95% CI 2.53–23.09) than those undergoing PFD. No significant differences in either clinical improvement (78.6 vs 64.6%, RR 1.23, 95% CI 0.95–1.59) or syringomyelia decrease (87.0 vs 56.3%, RR 1.43, 95% CI 0.91–2.25) were noted between PFDD and PFD. Conclusions Posterior fossa decompression with duraplasty is associated with a lower risk of reoperation than PFD but a greater risk for cerebrospinal fluid–related complications. There was no significant difference between the 2 operative techniques with respect to clinical improvement or decrease in syringomyelia.

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