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.

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.

The pathophysiology of oral pharyngeal apraxia and mutism following posterior fossa tumor resection in children

1995 ◽  
Vol 83 (3) ◽  
pp. 467-475 ◽  
Author(s):  
Andrew T. Dailey ◽  
Guy M. McKhann ◽  
Mitchel S. Berger
Keyword(s):  
Posterior Fossa ◽  
Pediatric Patients ◽  
Tumor Resection ◽  
Posterior Fossa Tumor ◽  
Posterior Fossa Tumors ◽  
Lower Cranial Nerve ◽  
Content Type ◽  
Cranial Nerve Function ◽  
Pharyngeal Musculature ◽  
Fine Print

✓ Mutism following posterior fossa tumor resection in pediatric patients has been previously recognized, although its pathophysiology remains unclear. A review of the available literature reveals 33 individuals with this condition, with only a few adults documented in the population. All of these patients had large midline posterior fossa tumors. To better understand the incidence and anatomical substrate of this syndrome, the authors reviewed a 7-year series of 110 children who underwent a posterior fossa tumor resection. During that time, nine (8.2%) of the 110 children exhibited mutism postoperatively. They ranged from 2.5 to 20 years of age (mean 8.1 years) and became mute within 12 to 48 hours of surgery. The period of mutism lasted from 1.5 to 12 weeks after onset: all children had difficulty coordinating their oral pharyngeal musculature as manifested by postoperative drooling and inability to swallow. Further analysis of these cases revealed that all children had splitting of the entire inferior vermis at surgery, as confirmed on postoperative magnetic resonance studies. Lower cranial nerve function was intact in all nine patients. Current concepts of cerebellar physiology emphasize the importance of the cerebellum in learning and language. The syndrome described resembles a loss of learned activities, or an apraxia, of the oral and pharyngeal musculature. To avoid the apraxia, therefore, the inferior vermis must be preserved. For large midline tumors that extend to the aqueduct, a combined approach through the fourth ventricle and a midvermis split may be used to avoid injuring the inferior vermis.

Posterior atlantooccipital membrane for duraplasty

2002 ◽  
Vol 97 (2) ◽  
pp. 266-268 ◽  
Author(s):  
R. Shane Tubbs ◽  
John C. Wellons ◽  
Jeffrey P. Blount ◽  
W. Jerry Oakes
Keyword(s):  
Foreign Body ◽  
Postoperative Complications ◽  
Pediatric Patients ◽  
Chiari I Malformation ◽  
Posterior Cranial Fossa ◽  
Content Type ◽  
Chiari I ◽  
Cranial Fossa ◽  
Fine Print

✓ The authors describe the use of autogenetic posterior atlantooccipital (PAO) membrane for duraplasty following after posterior cranial fossa surgery. The PAO membrane is routinely exposed for procedures of the posterior cranial fossa and merely needs to be dissected free of the underlying dura mater. Recently this membrane was obtained in several pediatric patients following procedures of the posterior cranial fossa such as duraplasty in case of Chiari I malformation. No postoperative complications were found at 6-month follow-up examination. The advantages of this intervention include less manipulation of muscle and fascia than that involved in other procedures and, therefore, seemingly less postoperative pain and the negation of issues inherent with foreign-body graft sources. The authors believe this structure to be of use as a dural substitute in small dural openings of the posterior cranial fossa.

scholarly journals Syringobulbia in pediatric patients with Chiari malformation type I

2018 ◽  
Vol 22 (1) ◽  
pp. 52-60 ◽  
Author(s):  
Arnold H. Menezes ◽  
Jeremy D. W. Greenlee ◽  
Brian J. Dlouhy
Keyword(s):  
Posterior Fossa ◽  
Cranial Nerve ◽  
Chiari Malformation ◽  
Fourth Ventricle ◽  
Pediatric Patients ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Presenting Symptoms ◽  
Cranial Nerve Palsies

OBJECTIVESyringobulbia (SB) is a rare entity, with few cases associated with Chiari malformation type I (CM-I) in the pediatric population. The authors reviewed all pediatric cases of CM-I–associated SB managed at their institution in order to better understand the presentation, treatment, and surgical outcomes of this condition.METHODSA prospectively maintained institutional database of craniovertebral junction abnormalities was analyzed to identify all cases of CM-I and SB from the MRI era (i.e., after 1984). The authors recorded presenting symptoms, physical examination findings, radiological findings, surgical treatment strategy, intraoperative findings, and outcomes. SB cases associated with tumors, infections, or type II Chiari malformations were excluded.RESULTSThe authors identified 326 pediatric patients with CM-I who were surgically treated. SB was identified in 13 (4%) of these 326 patients. Headache and neck pain were noted in all 13 cases. Cranial nerve abnormalities were common: vagus and glossopharyngeal nerve dysfunction was the most frequent observation. Other cranial nerves affected included the trigeminal, abducens, and hypoglossal nerves. Several patients exhibited multiple cranial nerve palsies at presentation. Central sleep apnea was present in 6 patients.Syringomyelia (SM) was present in all 13 patients. SB involved the medulla in all cases, and extended rostrally into the pons and midbrain in 2 patients; in 1 of these 2 cases the cavity extended further rostrally to the cerebrum (syringocephaly). SB communicated with the fourth ventricle in 7 of the 13 cases.All 13 patients were treated with posterior fossa decompression with intradural exploration to ensure CSF egress out of the fourth ventricle and through the foramen magnum. The foramen of Magendie was found to be occluded by an arachnoid veil in 9 cases. Follow-up evaluation revealed that SB improved before SM. Cranial nerve palsies regressed in 11 of the 13 patients, and SB improved in all 13.CONCLUSIONSThe incidence of SB in our surgical series of pediatric patients with CM-I was 4%, and all of these patients had accompanying SM. The SB cavity involved the medulla in all cases and was found to communicate with the fourth ventricle in 54% of cases. Posterior fossa decompression with intradural exploration and duraplasty is an effective treatment for these patients.

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.

Dural augmentation approaches and complication rates after posterior fossa decompression for Chiari I malformation and syringomyelia: a Park-Reeves Syringomyelia Research Consortium study

2021 ◽  
pp. 1-10
Author(s):  
Alexander T. Yahanda ◽  
P. David Adelson ◽  
S. Hassan A. Akbari ◽  
Gregory W. Albert ◽  
Philipp R. Aldana ◽  
...  
Keyword(s):  
Neck Pain ◽  
Pediatric Patients ◽  
Chiari I Malformation ◽  
Posterior Fossa Decompression ◽  
Complication Rates ◽  
Bovine Collagen ◽  
Postoperative Changes ◽  
Chiari I ◽  
Research Consortium ◽  
Chemical Meningitis

OBJECTIVEPosterior fossa decompression with duraplasty (PFDD) is commonly performed for Chiari I malformation (CM-I) with syringomyelia (SM). However, complication rates associated with various dural graft types are not well established. The objective of this study was to elucidate complication rates within 6 months of surgery among autograft and commonly used nonautologous grafts for pediatric patients who underwent PFDD for CM-I/SM.METHODSThe Park-Reeves Syringomyelia Research Consortium database was queried for pediatric patients who had undergone PFDD for CM-I with SM. All patients had tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and ≥ 6 months of postoperative follow-up after PFDD. Complications (e.g., pseudomeningocele, CSF leak, meningitis, and hydrocephalus) and postoperative changes in syrinx size, headaches, and neck pain were compared for autograft versus nonautologous graft.RESULTSA total of 781 PFDD cases were analyzed (359 autograft, 422 nonautologous graft). Nonautologous grafts included bovine pericardium (n = 63), bovine collagen (n = 225), synthetic (n = 99), and human cadaveric allograft (n = 35). Autograft (103/359, 28.7%) had a similar overall complication rate compared to nonautologous graft (143/422, 33.9%) (p = 0.12). However, nonautologous graft was associated with significantly higher rates of pseudomeningocele (p = 0.04) and meningitis (p < 0.001). The higher rate of meningitis was influenced particularly by the higher rate of chemical meningitis (p = 0.002) versus infectious meningitis (p = 0.132). Among 4 types of nonautologous grafts, there were differences in complication rates (p = 0.02), including chemical meningitis (p = 0.01) and postoperative nausea/vomiting (p = 0.03). Allograft demonstrated the lowest complication rates overall (14.3%) and yielded significantly fewer complications compared to bovine collagen (p = 0.02) and synthetic (p = 0.003) grafts. Synthetic graft yielded higher complication rates than autograft (p = 0.01). Autograft and nonautologous graft resulted in equal improvements in syrinx size (p < 0.0001). No differences were found for postoperative changes in headaches or neck pain.CONCLUSIONSIn the largest multicenter cohort to date, complication rates for dural autograft and nonautologous graft are similar after PFDD for CM-I/SM, although nonautologous graft results in higher rates of pseudomeningocele and meningitis. Rates of meningitis differ among nonautologous graft types. Autograft and nonautologous graft are equivalent for reducing syrinx size, headaches, and neck pain.

Rapid development of Chiari I malformation in an infant with Seckel syndrome and craniosynostosis

2003 ◽  
Vol 98 (5) ◽  
pp. 1113-1115 ◽  
Author(s):  
Timothy E. Hopkins ◽  
Stephen J. Haines
Keyword(s):  
Mr Imaging ◽  
Posterior Fossa ◽  
Rapid Development ◽  
Chiari I Malformation ◽  
Tonsillar Herniation ◽  
Seckel Syndrome ◽  
Content Type ◽  
Chiari I ◽  
Csf Diversion ◽  
Fine Print

✓ To illustrate the rapidity with which a child can develop a severe, symptomatic Chiari I malformation, the authors present the case of a 3-month-old infant with Seckel syndrome (microcephaly, micrognathia, craniosynostosis, and multiple other abnormalities) and posterior sagittal and bilateral lambdoid synostosis. The infant underwent magnetic resonance (MR) imaging shortly after birth; the initial image demonstrated the cerebellar tonsils in the posterior fossa, with no herniation. He subsequently developed severe apneic episodes and bradycardia; repeated MR imaging at 3 months demonstrated severe tonsillar herniation with compression of the brainstem. The child underwent posterior fossa remodeling surgery, including release of the posterior sagittal and lambdoid sutures and decompression of the Chiari I malformation. The patient's apnea gradually improved; however, he died of complications of pneumonia and sepsis several weeks later. The authors identified from the literature 21 patients in whom there was a documented MR image or other neuroimage that did not reveal evidence of a Chiari I malformation, followed by a subsequent study with clear documentation of the presence of Chiari I malformation. The interval between the initial study and the development of the tonsillar herniation ranged from 11 days to 18.5 years. In most cases, a lumbar cerebrospinal fluid (CSF) diversion had been performed. This patient developed a severely symptomatic Chiari I malformation during a 3-month period. These reports illustrate that the Chiari I malformation can develop rapidly in the face of increased intracranial pressure, craniosynostosis, and spinal CSF diversion.

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.

Atypical hemifacial microsomia associated with Chiari I malformation and syrinx: further evidence indicating that Chiari I malformation is a disorder of the paraaxial mesoderm

2001 ◽  
Vol 95 (6) ◽  
pp. 1034-1039 ◽  
Author(s):  
Ali H. Mesiwala ◽  
Christopher I. Shaffrey ◽  
Joseph S. Gruss ◽  
Richard G. Ellenbogen
Keyword(s):  
Posterior Fossa ◽  
Mandibular Condyle ◽  
Chiari I Malformation ◽  
Posterior Fossa Decompression ◽  
Hemifacial Microsomia ◽  
Middle Fossa ◽  
Content Type ◽  
Progressive Development ◽  
Chiari I ◽  
Left Middle

✓ The authors present the first known reported case of hemifacial microsomia associated with a Chiari I malformation and syrinx. A 14-year-old girl presented with progressive torticollis of 3 years' duration and headaches exacerbated by exercise. Computerized tomography scanning and magnetic resonance imaging revealed extensive craniofacial and vertebral abnormalities, including aplasia of the floor of the left middle fossa and posterior fossa cranium, articulation of the left mandibular condyle with the left temporal lobe, and progressive development of a Chiari I malformation with associated syringomyelia. The patient first underwent posterior fossa decompression, duraplasty, and occipitocervical fusion. This procedure was later followed by reconstruction of the floor of the left middle fossa and temporomandibular joint. The patient's outcome was excellent. In this case report the authors review the complex embryological development of craniofacial and craniovertebral structures, and emphasize the use of a staged approach to treat pathophysiological consequences of this congenital anomaly.

Factors associated with spinal fusion after posterior fossa decompression in pediatric patients with Chiari I malformation and scoliosis

2016 ◽  
Vol 18 (6) ◽  
pp. 737-743 ◽  
Author(s):  
Charles E. Mackel ◽  
Patrick J. Cahill ◽  
Marie Roguski ◽  
Amer F. Samdani ◽  
Patrick A. Sugrue ◽  
...  
Keyword(s):  
Cobb Angle ◽  
Spinal Fusion ◽  
Posterior Fossa ◽  
Pediatric Patients ◽  
Chiari I Malformation ◽  
Curve Progression ◽  
Posterior Fossa Decompression ◽  
Routine Monitoring ◽  
Chiari I ◽  
Curve Severity

OBJECTIVE The authors performed a study to identify clinical characteristics of pediatric patients diagnosed with Chiari I malformation and scoliosis associated with a need for spinal fusion after posterior fossa decompression when managing the scoliotic curve. METHODS The authors conducted a multicenter retrospective review of 44 patients, aged 18 years or younger, diagnosed with Chiari I malformation and scoliosis who underwent posterior fossa decompression from 2000 to 2010. The outcome of interest was the need for spinal fusion after decompression. RESULTS Overall, 18 patients (40%) underwent posterior fossa decompression alone, and 26 patients (60%) required a spinal fusion after the decompression. The mean Cobb angle at presentation and the proportion of patients with curves > 35° differed between the decompression-only and fusion cohorts (30.7° ± 11.8° vs 52.1° ± 26.3°, p = 0.002; 5 of 18 vs 17 of 26, p = 0.031). An odds ratio of 1.0625 favoring a need for fusion was established for each 1° of increase in Cobb angle (p = 0.012, OR 1.0625, 95% CI 1.0135–1.1138). Among the 14 patients older than 10 years of age with a primary Cobb angle exceeding 35°, 13 (93%) ultimately required fusion. Patients with at least 1 year of follow-up whose curves progressed more 10° after decompression were younger than those without curve progression (6.1 ± 3.0 years vs 13.7 ± 3.2 years, p = 0.001, Mann-Whitney U-test). Left apical thoracic curves constituted a higher proportion of curves in the decompression-only group (8 of 16 vs 1 of 21, p = 0.002). CONCLUSIONS The need for fusion after posterior fossa decompression reflected the curve severity at clinical presentation. Patients presenting with curves measuring > 35°, as well as those greater than 10 years of age, may be at greater risk for requiring fusion after posterior fossa decompression, while patients less than 10 years of age may require routine monitoring for curve progression. Left apical thoracic curves may have a better response to Chiari malformation decompression.

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