Progressive spasticity and scoliosis in children with myelomeningocele

1985 ◽  
Vol 62 (3) ◽  
pp. 367-375 ◽  
Author(s):  
T. S. Park ◽  
Wayne S. Cail ◽  
William M. Maggio ◽  
Diane C. Mitchell

✓ Seventeen myelodysplastic patients with progressive extremity spasticity and scoliosis underwent radiological evaluation and surgical treatment. All but one were under 18 years of age at the time of surgical treatment. Duration of the clinical presentation ranged from 1½ to 7 years. Metrizamide was instilled into the subarachnoid space in 12 patients, the lateral ventricle in two, and the hydromyelic cavity in three. Sequential computerized tomography scanning after intrathecal instillation of the contrast material clearly demonstrated hydromyelia in nine patients and compression of the brain stem in five. Posterior fossa decompression with plugging of the obex was performed in 12 patients, posterior fossa decompression alone in three, and ventriculoperitoneal (VP) shunting procedures in two. Of the 12 patients who underwent the obex plugging procedures, eight have shown partial or complete resolution of spasticity and an increase in motor strength with no significant postoperative complications. In contrast, posterior fossa decompression or VP shunting procedures alone have not led to a favorable neurological outcome. Hydromyelia may occur more commonly among myelodysplastic patients than previously recognized and may be treated most effectively by the obex plugging procedure.

1998 ◽  
Vol 88 (1) ◽  
pp. 116-121 ◽  
Author(s):  
David B. Clarke ◽  
Richard Leblanc ◽  
Gilles Bertrand ◽  
Gilbert R. C. Quartey ◽  
G. Jackson Snipes

✓ Meningeal melanocytomas are rare tumors of the central nervous system that are found almost exclusively in the posterior fossa and spinal cord and whose natural history is poorly defined. In this report, the authors review the clinical presentation, radiological appearance, operative findings, and histological features in two cases of meningeal melanocytoma: one cranial and one spinal. Two women, aged 21 and 30 years, were admitted to the hospital 60 years apart: the first because of progressive paraplegia and the second because of slowly progressive hearing loss. The first patient had an extradural tumor that was treated by laminectomy, subtotal resection, and postoperative radiotherapy in 1936. Her symptoms recurred 16 years later and she underwent reoperation of the residual tumor, which was found to have an intradural component. The authors' patient, who presented 60 years later, underwent plain and enhanced computerized tomography and magnetic resonance imaging that demonstrated a large posterior fossa lesion indicative of either an acoustic neuroma or a meningioma. She underwent posterior fossa decompression but only partial excision of the tumor could be accomplished because vigorous bleeding limited the extent of the resection. Surgery was followed by radiotherapy. The residual tumor enlarged despite these measures and required repeated resection 6 months later. At the second operation the tumor was much less vascular, perhaps reflecting the effects of radiotherapy, and was removed almost entirely. The patient died 6 months later from an anticoagulant-related cerebellar hemorrhage. In both cases the lesions were jet black, and histological examination revealed melanin-containing hypercellular tumors with rare mitotic figures. Meningeal melanocytomas are being diagnosed with increased frequency in parallel with improvements in neuroimaging and clarification of histological features. Clinical presentation of patients with these tumors typically occurs in their fifth decade and women are affected twice as often as men. The posterior fossa lesions can mimic acoustic neuromas and meningiomas in location and radiological appearance; however, the internal auditory canal is normal. In the spine, meningeal melanocytomas present with the clinical features of myeloradiculopathy. Diagnosis is made intraoperatively from the gross, jet-black appearance of the tumor and from histological examination. Vascularity, size, and location may render complete resection unfeasible. Because of the tumor's propensity to recur, radiotherapy has been recommended but its role remains to be elucidated.


2019 ◽  
Vol 23 (4) ◽  
pp. 498-506 ◽  
Author(s):  
Tofey J. Leon ◽  
Elizabeth N. Kuhn ◽  
Anastasia A. Arynchyna ◽  
Burkely P. Smith ◽  
R. Shane Tubbs ◽  
...  

OBJECTIVEThere are sparse published data on the natural history of “benign” Chiari I malformation (CM-I)—i.e., Chiari with minimal or no symptoms at presentation and no imaging evidence of syrinx, hydrocephalus, or spinal cord signal abnormality. The purpose of this study was to review a large cohort of children with benign CM-I and to determine whether these children become symptomatic and require surgical treatment.METHODSPatients were identified from institutional outpatient records using International Classification of Diseases, 9th Revision, diagnosis codes for CM-I from 1996 to 2016. After review of the medical records, patients were excluded if they 1) did not have a diagnosis of CM-I, 2) were not evaluated by a neurosurgeon, 3) had previously undergone posterior fossa decompression, or 4) had imaging evidence of syringomyelia at their first appointment. To include only patients with benign Chiari (without syrinx or classic Chiari symptoms that could prompt immediate intervention), any patient who underwent decompression within 9 months of initial evaluation was excluded. After a detailed chart review, patients were excluded if they had classical Chiari malformation symptoms at presentation. The authors then determined what changes in the clinical picture prompted surgical treatment. Patients were excluded from the multivariate logistic regression analysis if they had missing data such as race and insurance; however, these patients were included in the overall survival analysis.RESULTSA total of 427 patients were included for analysis with a median follow-up duration of 25.5 months (range 0.17–179.1 months) after initial evaluation. Fifteen patients had surgery at a median time of 21.0 months (range 11.3–139.3 months) after initial evaluation. The most common indications for surgery were tussive headache in 5 (33.3%), syringomyelia in 5 (33.3%), and nontussive headache in 5 (33.3%). Using the Kaplan-Meier method, rate of freedom from posterior fossa decompression was 95.8%, 94.1%, and 93.1% at 3, 5, and 10 years, respectively.CONCLUSIONSAmong a large cohort of patients with benign CM-I, progression of imaging abnormalities or symptoms that warrant surgical treatment is infrequent. Therefore, these patients should be managed conservatively. However, clinical follow-up of such individuals is justified, as there is a low, but nonzero, rate of new symptom or syringomyelia development. Future analyses will determine whether imaging or clinical features present at initial evaluation are associated with progression and future need for treatment.


1977 ◽  
Vol 47 (1) ◽  
pp. 50-56 ◽  
Author(s):  
Hiroshi Matsumura ◽  
Yasumasa Makita ◽  
Kuniyuki Someda ◽  
Akinori Kondo

✓ We have operated on 12 of 14 cases of arteriovenous malformation (AVM) in the posterior fossa since 1968, with one death. The lesions were in the cerebellum in 10 cases (three anteromedial, one central, three lateral, and three posteromedial), and in the cerebellopontine angle in two; in two cases the lesions were directly related to the brain stem. The AVM's in the anterior part of the cerebellum were operated on through a transtentorial occipital approach.


2008 ◽  
Vol 2 (1) ◽  
pp. 42-49 ◽  
Author(s):  
Susan R. Durham ◽  
Kristina Fjeld-Olenec

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.


1984 ◽  
Vol 61 (5) ◽  
pp. 972-974 ◽  
Author(s):  
José Hernansanz ◽  
Francisco Muñoz ◽  
Daniel Rodríguez ◽  
Concepción Soler ◽  
Carlos Principe

✓ Two cases are reported of subdural hematoma of the posterior fossa in normal-weight full-term newborn infants. The most salient factors in these cases were the lack of specific symptoms and signs indicating the nature and location of the lesion, the importance of computerized tomography for diagnosis, and the good results obtained with early surgical treatment.


2017 ◽  
Vol 63 (11) ◽  
pp. 946-949 ◽  
Author(s):  
Marcelo Ferreira Sabba ◽  
Beatriz Souza Renor ◽  
Enrico Ghizoni ◽  
Helder Tedeschi ◽  
Andrei Fernandes Joaquim

Summary Chiari malformation (CM) is the most common and prevalent symptomatic congenital craniocervical malformation. Radiological diagnosis is established when the cerebellar tonsils are located 5 mm or more below the level of the foramen magnum on magnetic resonance imaging (MRI). Surgical treatment is indicated whenever there is symptomatic tonsillar herniation or syringomyelia/hydrocephalus. The main surgical treatment for CM without craniocervical instability (such as atlantoaxial luxation) is posterior fossa decompression, with or without duraplasty. The authors describe in details and in a stepwise fashion the surgical approach of patients with CM as performed at the State University of Campinas, emphasizing technical nuances for minimizing the risks of the procedure and potentially improving patient outcome.


1981 ◽  
Vol 54 (3) ◽  
pp. 380-383 ◽  
Author(s):  
Josè Garcìa-Uria ◽  
Guillermo Leunda ◽  
Rafael Carrillo ◽  
Gonzalo Bravo

✓ A retrospective study of the surgical results in 31 adult patients with syringomyelia associated with Arnold-Chiari malformation was carried out. The standard surgical procedure consisted of a posterior fossa craniectomy. The vallecula was opened in 28 patients, with plugging of the central canal in 19. In three patients, severe arachnoidal scarring was present, and dissection was not attempted. The postoperative follow-up period ranged from 5 to 10 years. More than half of the patients considered themselves improved by surgery; however, only five cases showed objective improvement. Despite the operation, six patients experienced progressive neurological deterioration.


1990 ◽  
Vol 73 (1) ◽  
pp. 64-68 ◽  
Author(s):  
Jesús Vaquero ◽  
Roberto Martínez ◽  
Alicia Arias

✓ Thirty patients with syringomyelia-Chiari complex who underwent posterior fossa decompression or syringosubarachnoid shunting were studied clinically and by magnetic resonance (MR) imaging to assess the changes in the syrinx and in their clinical picture after surgery. When symptoms of posterior fossa compression were present, posterior fossa decompression was performed; however, when symptoms of posterior fossa compression were absent, the choice of posterior fossa decompression or syringosubarachnoid shunting depended, respectively, on whether the syrinx was narrow or wide on MR imaging. At least 1 year after surgery, subjective improvement or arrest of disease was recorded in 73% of the patients. The present study suggests that: 1) the symptoms attributed to spinal cord damage have no significant relationship to the size of the syrinx on MR images; 2) the surgical techniques employed in this series (posterior fossa decompression or syringosubarachnoid shunt) were equally useful in inducing syrinx collapse; and 3) when posterior fossa decompression is performed, plugging of the obex is not necessary for syrinx collapse.


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