Cervicomedullary compression in achondroplasia

1994 ◽  
Vol 81 (1) ◽  
pp. 43-48 ◽  
Author(s):  
Timothy C. Ryken ◽  
Arnold H. Menezes
Keyword(s):  
Posterior Fossa ◽  
Foramen Magnum ◽  
Posterior Arch ◽  
Posterior Fossa Decompression ◽  
Duration Of Symptoms ◽  
Content Type ◽  
Cervicomedullary Junction ◽  
Plain Films ◽  
Cervicomedullary Compression ◽  
Fine Print

✓ Six patients with achondroplasia and symptoms suggestive of cervicomedullary junction compression are reviewed; these included three females and three males, with an average age of 8 years (range 7 months to 30 years). The mean duration of symptoms prior to intervention was 1.9 years. Symptoms included occipitocervical pain, ataxia, incontinence, apnea, and respiratory arrest. Radiological investigations consisted of plain films with flexion and extension views, pluridirectional tomography, thin-section computerized tomography, and magnetic resonance imaging. Typical findings included marked foramen magnum stenosis, ventrolateral cervicomedullary junction compression secondary to central and paramesial basilar invagination, and dorsal cervicomedullary junction compression secondary to ligamentous hypertrophy and invagination of the posterior atlantal arch. All patients underwent posterior fossa decompression and atlantal laminectomy. Surgery consistently revealed marked dorsal and paramesial overgrowth of the rim of the foramen magnum, with thickening and invagination of the atlantal posterior arch and a dense fibrotic epidural band resulting in dorsal cervicomedullary compression. Intraoperative ultrasonography was used to determine the extent of decompression required. Three patients required duraplasty. Three patients had concurrent hydrocephalus, two of whom had undergone ventriculoperitoneal shunting prior to surgical decompression of the posterior fossa. One patient developed a pseudomeningocele postoperatively, requiring serial lumbar punctures before it resolved. No patient developed craniovertebral instability following decompression. Improvement or resolution of symptoms was noted in all patients, with an average follow-up period of 4.8 years. Thus, cervicomedullary compression in patients with achondroplasia can be successfully treated with dorsal decompression of the craniovertebral junction. Dense epidural fibrotic bands are frequently noted in these cases and must be aggressively released to ensure satisfactory decompression.

Meningeal melanocytoma

1998 ◽  
Vol 88 (1) ◽  
pp. 116-121 ◽  
Author(s):  
David B. Clarke ◽  
Richard Leblanc ◽  
Gilles Bertrand ◽  
Gilbert R. C. Quartey ◽  
G. Jackson Snipes
Keyword(s):  
Histological Examination ◽  
Posterior Fossa ◽  
Clinical Presentation ◽  
Residual Tumor ◽  
Posterior Fossa Decompression ◽  
Content Type ◽  
Meningeal Melanocytoma ◽  
Radiological Appearance ◽  
Histological Features ◽  
Fine Print

✓ 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.

Long-term outcome after operation for trigeminal neuralgia in patients with posterior fossa tumors

1996 ◽  
Vol 84 (5) ◽  
pp. 818-825 ◽  
Author(s):  
Fred G. Barker ◽  
Peter J. Jannetta ◽  
Ramesh P. Babu ◽  
Spiros Pomonis ◽  
David J. Bissonette ◽  
...  
Keyword(s):  
Trigeminal Neuralgia ◽  
Posterior Fossa ◽  
Trigeminal Nerve ◽  
Posterior Fossa Tumors ◽  
Duration Of Symptoms ◽  
Long Term Outcome ◽  
Entry Zone ◽  
Content Type ◽  
Root Entry Zone ◽  
Fine Print

✓ During a 20-year period, 26 patients with typical symptoms of trigeminal neuralgia were found to have posterior fossa tumors at operation. These cases included 14 meningiomas, eight acoustic neurinomas, two epidermoid tumors, one angiolipoma, and one ependymoma. The median patient age was 60 years and 69% of the patients were women. Sixty-five percent of the symptoms were left sided. The median preoperative duration of symptoms was 5 years. The distribution of pain among the three divisions of the trigeminal nerve was similar to that found in patients with trigeminal neuralgia who did not have tumors; however, more divisions tended to be involved in the tumor patients. The mean postoperative follow-up period was 9 years. At operation, the root entry zone of the trigeminal nerve was examined for vascular cross-compression in 21 patients. Vessels compressing the nerve at the root entry zone were observed in all patients examined. Postoperative pain relief was frequent and long lasting. Using Kaplan—Meier methods the authors estimated excellent relief in 81% of the patients 10 years postoperatively, with partial relief in an additional 4%.

Odontoid upward migration in rheumatoid arthritis

1985 ◽  
Vol 63 (4) ◽  
pp. 500-509 ◽  
Author(s):  
Arnold H. Menezes ◽  
John C. VanGilder ◽  
Charles R. Clark ◽  
George El-Khoury
Keyword(s):  
Rheumatoid Arthritis ◽  
Cervical Vertebrae ◽  
Posterior Arch ◽  
Upward Migration ◽  
Lower Cranial Nerve ◽  
Content Type ◽  
Cervicomedullary Junction ◽  
Asymptomatic Patients ◽  
Cranial Nerve Palsies ◽  
Fine Print

✓ Lack of correlation between the severity of rheumatoid subluxation of the upper cervical vertebrae and supposed absence of neurological damage has led to the erroneous supposition that this finding is innocuous. Incomplete autopsy studies in rheumatoid arthritis have failed to recognize the cause of death, despite previously proven dramatic occipito-atlanto-axial dislocations. The most feared entity of rheumatoid basilar invagination, namely “cranial settling,” is poorly understood. Between 1978 and 1984, the authors treated 45 rheumatoid arthritis patients who were symptomatic with “cranial settling.” This consisted of vertical odontoid penetration through the foramen magnum (9 to 33 mm), occipito-atlanto-axial dislocation, lateral atlantal mass erosion, downward telescoping of the anterior arch of C-1 on the axis, and rostral rotation of the posterior arch of C-1 producing ventral and dorsal cervicomedullary junction compromise. Cervicomedullary junction dysfunction has mistakenly been called “entrapment neuropathy,” “progression of disease,” or “vasculitis.” Occipital pain occurred in all 45 patients, myelopathy in 36, blackout spells in 24, brain-stem signs in 17, and lower cranial nerve palsies in 10. Four patients had prior tracheostomies. Four previously asymptomatic patients with “cranial settling” presented acutely quadriplegic. The factors governing treatment were reducibility and direction of encroachment determined by skeletal traction and myelotomography. Transoral odontoidectomy was performed in seven patients with irreducible pathology. All patients underwent occipitocervical bone fusion (with C-1 decompression if needed) and acrylic fixation. Improvement occurred during traction, implying that compression might be the etiology for the neurological signs. There were no complications. Thus, “cranial settling” is a frequent complication of rheumatoid arthritis; although it is poorly recognized, it has serious implications and is treatable.

Foramen magnum decompression in an infant with homozygous achondroplasia

1989 ◽  
Vol 70 (1) ◽  
pp. 126-128 ◽  
Author(s):  
Nathan Moskowitz ◽  
Benjamin Carson ◽  
Steven Kopits ◽  
Roy Levitt ◽  
Graeme Hart
Keyword(s):  
Upper Airway ◽  
Early Death ◽  
Foramen Magnum ◽  
Foramen Magnum Decompression ◽  
Thoracic Cage ◽  
Respiratory Compromise ◽  
Content Type ◽  
Surgical Measures ◽  
Cervicomedullary Compression ◽  
Fine Print

✓ Homozygous achondroplasia is a rare yet distinct clinical entity. Most infants succumb to an early death as a result of respiratory compromise due to upper airway obstruction, thoracic cage deformity, and/or cervicomedullary compression. The successful cervicomedullary decompression of a 16-week-old infant with homozygous achondroplasia is described. This report suggests that homozygous achondroplasia is not universally fatal and that these infants are potentially viable if managed by aggressive respiratory and surgical measures.

An angiographic study of meningiomas of the posterior fossa

1971 ◽  
Vol 35 (6) ◽  
pp. 731-741 ◽  
Author(s):  
Georges M. Salamon ◽  
André Combalbert ◽  
Charles Raybaud ◽  
Jorge Gonzalez
Keyword(s):  
Vertebral Artery ◽  
Posterior Fossa ◽  
Foramen Magnum ◽  
Posterior Cranial Fossa ◽  
Occipital Artery ◽  
Angiographic Study ◽  
Content Type ◽  
Meningeal Arteries ◽  
Cranial Fossa ◽  
Fine Print

✓ The meningeal vasculature of the posterior cranial fossa was studied on injected normal anatomical specimens and in angiograms of patients with posterior fossa meningiomas. The correlated results indicate that the dura anterior to the foramen magnum is supplied by meningeal vessels from the carotid siphon, the ascending pharyngeal and middle meningeal arteries. The dura posterior to the foramen magnum is supplied primarily by the occipital artery and secondarily by the vertebral artery.

Basilar invagination in osteogenesis imperfecta and related osteochondrodysplasias: medical and surgical management

1997 ◽  
Vol 86 (6) ◽  
pp. 950-960 ◽  
Author(s):  
Paul D. Sawin ◽  
Arnold H. Menezes
Keyword(s):  
Osteogenesis Imperfecta ◽  
Posterior Fossa ◽  
Basilar Invagination ◽  
Posterior Fossa Decompression ◽  
Occipitocervical Fusion ◽  
Female Ratio ◽  
Content Type ◽  
Brainstem Compression ◽  
The Mean ◽  
Fine Print

✓ Osteogenesis imperfecta (OI) is a heritable disorder of bone development caused by defective collagen synthesis. Basilar invagination is an uncommon but devastating complication of this disease. The authors present a comprehensive strategy for management of craniovertebral anomalies associated with OI and related osteochondrodysplasias. Twenty-five patients with congenital osteochondrodysplasias (18 OI, four Hajdu—Cheney syndrome, and three spondyloepiphyseal dysplasia) and basilar invagination were evaluated between 1985 and 1995. The male/female ratio in this cohort was 1:1. The mean age at presentation was 11.9 years (range 13 months–20 years). Fourteen patients (56%) presented during adolescence (11–15 years of age). Symptoms and signs included headache (76%), lower cranial nerve dysfunction (68%), hyperreflexia (56%), quadriparesis (48%), ataxia (32%), nystagmus (28%), and scoliosis (20%). Four patients (16%) were asymptomatic. Seven (28%) had undergone previous posterior fossa decompression; one had also undergone ventral decompression. Imaging findings included basilar invagination (100%), ventral brainstem compression (84%), hydrocephalus (32%), hindbrain herniation (28%), and syringomyelia/syringobulbia (16%). Patients with hydrocephalus underwent ventricular shunt placement. Reducible basilar invagination (40%) was treated with posterior fossa decompression and occipitocervical fusion. Those with irreducible ventral compression (60%) underwent transoral—transpalatopharyngeal decompression followed by occipitocervical fusion. All patients improved initially. However, basilar invagination progressed radiographically in 80% (symptomatic in 24%) despite successful fusion. Prolonged external orthotic immobilization with the modified Minerva brace afforded symptomatic improvement and arrested progression of the deformity. The mean follow-up period was 5.9 years (range 1.1–10.5 years). Ventral brainstem compression in OI should be treated with ventral decompression, followed by occipitocervical fusion with contoured loop instrumentation to prevent further squamooccipital infolding. Despite fusion, however, basilar invagination tends to progress. Prolonged immobilization (particularly during adolescence) may stabilize symptoms and halt further invagination. This study represents the largest series to date addressing craniovertebral anomalies in OI and related congenital bone softening disorders.

Surgical experience in 130 pediatric patients with Chiari I malformations

2003 ◽  
Vol 99 (2) ◽  
pp. 291-296 ◽  
Author(s):  
R. Shane Tubbs ◽  
Matthew J. McGirt ◽  
W. Jerry Oakes
Keyword(s):  
Neck Pain ◽  
Posterior Fossa ◽  
Pediatric Patients ◽  
Neurofibromatosis Type ◽  
Hormone Deficiency ◽  
Posterior Fossa Decompression ◽  
Content Type ◽  
Presenting Symptoms ◽  
Chiari I ◽  
Fine Print

Object. The aim of this study was to present the long-term findings of a surgical series of pediatric patients with Chiari I malformations. Methods. One hundred thirty symptomatic pediatric patients with Chiari I malformations underwent posterior fossa decompression. The age in this group of patients ranged from 2 months to 20 years (mean 11 years). The length of the hospital stay extended from 2 to 7 days (mean 2.7 days), and follow up was from 3 months to 15 years (mean 4.2 years). Patients most often presented with headache/neck pain (38%) and scoliosis (18%). Examples of associated diagnoses included neurofibromatosis Type 1 (5.5%), hydrocephalus (11%), idiopathic growth hormone deficiency (5.5%), and Klippel—Feil anomaly (5%). Syringes were present in 58% of patients. Seventeen percent of patients had caudal displacement of the brainstem and fourth ventricle. Postoperative relief of preoperative pathologies was experienced in 83% of patients. Of the most common presenting symptoms—headache/neck pain and scoliosis—12 and 17%, respectively, were not alleviated postoperatively. Complications occurred in 2.3% of this group and included the development of acute hydrocephalus postoperatively and severe life-threatening signs of brainstem compression that necessitated a transoral odontoidectomy. Nine patients have had to undergo repeated operations for continued symptoms or persistent large syringes. During surgery 10 patients (7.7%) were found to have arachnoid veils occluding the fourth ventricular outlet, and nine of these had syringomyelia. In our experience almost all syringes will stabilize or improve with posterior fossa decompression and duraplasty. Conclusions. The authors believe this to be the largest reported series of pediatric patients who have undergone posterior fossa decompression for Chiari I malformations.

Basilar impression in osteogenesis imperfecta tarda

1991 ◽  
Vol 74 (1) ◽  
pp. 136-138 ◽  
Author(s):  
Masanori Kurimoto ◽  
Susumu Ohara ◽  
Akira Takaku
Keyword(s):  
Osteogenesis Imperfecta ◽  
Brain Stem ◽  
Posterior Fossa ◽  
Hemifacial Spasm ◽  
Posterior Fusion ◽  
Posterior Fossa Decompression ◽  
Basilar Impression ◽  
Content Type ◽  
Compression Syndrome ◽  
Fine Print

✓ A case is presented of basilar impression secondary to osteogenesis imperfecta tarda, associated with hemifacial spasm and brain-stem compression syndrome. The symptoms improved with posterior fossa decompression and posterior fusion.

Arteriovenous malformations in the posterior fossa

1977 ◽  
Vol 47 (1) ◽  
pp. 50-56 ◽  
Author(s):  
Hiroshi Matsumura ◽  
Yasumasa Makita ◽  
Kuniyuki Someda ◽  
Akinori Kondo
Keyword(s):  
Arteriovenous Malformation ◽  
Brain Stem ◽  
Posterior Fossa ◽  
Arteriovenous Malformations ◽  
Cerebellopontine Angle ◽  
Anterior Part ◽  
Content Type ◽  
The Brain ◽  
Fine Print

✓ 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.

Astrocytomas of the spinal cord in children and adolescents

1985 ◽  
Vol 63 (5) ◽  
pp. 669-675 ◽  
Author(s):  
Ronald Reimer ◽  
Burton M. Onofrio
Keyword(s):  
Spinal Cord ◽  
Histological Grade ◽  
Extended Period ◽  
Spinal Deformities ◽  
Duration Of Symptoms ◽  
Female Ratio ◽  
Content Type ◽  
Orthopedic Procedure ◽  
Male To Female ◽  
Fine Print

✓ The authors review 32 cases of spinal cord astrocytoma in patients under 20 years of age who were treated at the Mayo Clinic between 1955 and 1980. There was a 1.3:1 male to female ratio. Twenty patients were between 6 and 15 years of age at the time of diagnosis. The duration of symptoms prior to definitive diagnosis varied from 5 days to 9 years, with an average of 24 months. The most common symptoms were pain (62.5%), gait disturbance (43.7%), numbness (18.8%), and sphincteric dysfunction (18.8%). The most common neurological findings were a Babinski response (50.0%), posterior column sensory dysfunction (40.6%), and paraparesis (37.5%). A median follow-up period of 8.6 years (range 0.8 to 25.5 years) revealed that the survival time diminished with increased histological grade of the astrocytoma (p < 0.001). The development of postlaminectomy spinal deformities represented a serious postoperative complication. This occurred in 13 patients and was first recognized between 8 and 90 months postoperatively. Six deformities occurred following cervical laminectomy, and eight patients required at least one orthopedic procedure. It is crucial to follow these patients for an extended period of time to watch for postoperative spinal deformities.

Sign in / Sign up

Export Citation Format

Share Document