Symptomatic Arnold-Chiari malformation: review of experience with 22 cases (original) (raw)
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Surgical decompression for symptomatic Chiari II malformation in neonates with myelomeningocele
Journal of Neurosurgery, 1992
✓ Between January, 1981, and July, 1991, 17 infants under 1 month of age were admitted to The Hospital for Sick Children with the signs and symptoms of a Chiari II malformation. These patients' presentation included swallowing difficulty (71%), stridor (59%), apneic spells (29%), aspiration (12%), weakness of cry (18%), and arm weakness (53%). Decompression of the Chiari II malformation was performed in all patients, with a time interval between onset of symptoms and surgery ranging from 1 to 121 days. Fifteen patients (88%) remain alive, all of whom have shown a complete recovery. The mean follow-up period in this group of patients was 65 months. Two patients died, one due to respiratory arrest 8 months after decompression and the other because of shunt infection and peritonitis 7 years after decompression. These results support the concept that compressive forces, rather than a primary intrinsic disorder of the brain-stem nuclei, play a crucial etiological role in the developm...
Journal of Neurosurgery, 1992
✓ Between 1975and 1989, 25 children treated with myelomeningocele closure and shunting for hydrocephalus at the Children's Hospital of Pittsburgh developed progressive lower brain-stem dysfunction from their Chiari malformation. Retrospective univariate and multivariate analyses of these cases were undertaken to assess the relationship between preoperative clinical factors and postoperative outcome. Since earlier reports have suggested that neonates with symptomatic Chiari malformations show a less favorable response than older children to craniocervical decompression, particular attention was directed at examining the effect of age on preoperative symptoms and postoperative outcome. Patients were subdivided by age into two groups, namely: 13 patients who became symptomatic before 2 months of age (neonatal group) and 12 older infants and children who developed initial symptoms between 6 months and 10 years of age. Once symptoms developed, patients in both groups deteriorated pro...
Clinical and radiological findings in Arnold Chiari malformation
Journal of Ayub Medical College Abbottabad Jamc, 2010
BACKGROUND: The Chiari Malformation I (CMI) is a disorder of uncertain origin that has been traditionally defined as downward herniation of the cerebellar tonsils through the foramen magnum. The anomaly is a leading cause of syringomyelia and occurs inassociation with osseus abnormalities at the craniovertebral junction. In contrast to other Chiari malformations, CMI tends to present in the second or third decade of life and is sometimes referred to as the 'adult-type' Chiari malformation. The objective was to document clinical and radiological findings in Arnold Chiari Malformation-I.METHOD: This was a descriptive study carried out in Ayub Teaching Hospital Abbottabad at Neurosurgery Department during July 2008-July 2010. We examined a prospective cohort of 60 symptomatic patients. All patients underwent magnetic resonance imaging of the head and spine.RESULTS: There were 40 female and 20 male patients. The age of onset was 24.9 +/- 15.8 years. Common associated radiological problems included syringomyelia (60%), scoliosis (25%), and basilarinvagination (12%), increased cervical lordosis 5 (8.5%), and Klippel Feil syndrome 2 (3.3%). The most consistent magnetic resonance imaging findings were obliteration of the retrocerebellar cerebrospinal fluid spaces (70% patients), tonsillar herniation of at least 5 mm (100% patients), and varying degrees of post fossa anomalies. Linical manifestations were headaches, pseudotumor-like episodes, a Meniere's disease-like syndrome, lower cranial nerve signs, and spinal cord disturbances in the absence of syringomyelia.CONCLUSION: These data support accumulating evidence that CMI is a disorder of the para-axial mesoderm that is characterised by underdevelopment of the posterior cranial fossa and overcrowding of the normally developed hindbrain. Tonsillar herniation of less than 5 mm does not exclude the diagnosis. Clinical manifestations of CMI seem to be related to cerebrospinal fluid disturbances (which are responsible for headaches, pseudotumor-like episodes, endolymphatic hydrops, syringomyelia, and hydrocephalus) and direct compression of nervous tissue.
Surgical management of Chiari malformation type II
Child's Nervous System
Background Chiari malformation type II is present in almost all patients with myelomeningocele but usually remains asymptomatic. Symptoms are generally more severe in neonates, who have the worst prognosis. The association symptoms/ hydrocephalus is well known, and first treatment usually consists of ensuring adequate ventricular drainage. Craniovertebral decompression may be required in patients who do not improve after drainage. However, mechanisms of symptom development are not yet completely understood, timing and techniques of surgery are not codified, long-term evolution is poorly reported, and there are few paper reporting clinical onset and treatment in older patients. Methods We reviewed our personal series of 42 consecutive symptomatic patients that required surgical treatment. Age at surgery ranged from 1 week to 44 years (mean 6.6 years). Surgical timing strictly depended on clinical conditions: urgent management in the more compromised patients (usually infants) and elective treatment before severe deterioration in patients with less severe conditions. All patients first underwent external ventricular drainage, which resolved the symptomatology in 17 cases (40%). Craniocervical decompression was required by 25 patients (60%) who received no benefit from the ventricular drainage. Results Early mortality (2 cases = 4.7%) occurred only in neonates. Clinical improvement was achieved in 37 of 40 survivors (92%). During a follow-up ranging from 2 to 20 years (mean 10.3 years), late mortality consisted of 4 cases (10%), mainly due to cardio-respiratory arrest. Twenty-two patients (55%) required surgery for shunt malfunction and 4 for cord detethering. Six patients (15%) required reoperation owing to symptom recurrence. Conclusion Early treatment of symptomatic Chiari II malformations may warrant satisfactory results in a significant number of patients, even in neonates. Nevertheless, overall mortality remains relatively high, throughout the patient life. Formal transition programs and adult spina bifida care processes have become crucial. Keywords Chiari II malformation. Craniocervical decompression. Myelomeningocele. Ventriculoperitoneal shunt. Spina bifida Chiari 2 malformation (CM-II) is a myelomeningocele (MMC) associated anomaly consisting of downward displace
Surgical management of the Chiari malformation Type I: the way forward
Journal of neurosurgery. Pediatrics, 2008
Background Chiari malformation type II is present in almost all patients with myelomeningocele but usually remains asymptomatic. Symptoms are generally more severe in neonates, who have the worst prognosis. The association symptoms/ hydrocephalus is well known, and first treatment usually consists of ensuring adequate ventricular drainage. Craniovertebral decompression may be required in patients who do not improve after drainage. However, mechanisms of symptom development are not yet completely understood, timing and techniques of surgery are not codified, long-term evolution is poorly reported, and there are few paper reporting clinical onset and treatment in older patients. Methods We reviewed our personal series of 42 consecutive symptomatic patients that required surgical treatment. Age at surgery ranged from 1 week to 44 years (mean 6.6 years). Surgical timing strictly depended on clinical conditions: urgent management in the more compromised patients (usually infants) and elective treatment before severe deterioration in patients with less severe conditions. All patients first underwent external ventricular drainage, which resolved the symptomatology in 17 cases (40%). Craniocervical decompression was required by 25 patients (60%) who received no benefit from the ventricular drainage. Results Early mortality (2 cases = 4.7%) occurred only in neonates. Clinical improvement was achieved in 37 of 40 survivors (92%). During a follow-up ranging from 2 to 20 years (mean 10.3 years), late mortality consisted of 4 cases (10%), mainly due to cardio-respiratory arrest. Twenty-two patients (55%) required surgery for shunt malfunction and 4 for cord detethering. Six patients (15%) required reoperation owing to symptom recurrence. Conclusion Early treatment of symptomatic Chiari II malformations may warrant satisfactory results in a significant number of patients, even in neonates. Nevertheless, overall mortality remains relatively high, throughout the patient life. Formal transition programs and adult spina bifida care processes have become crucial. Keywords Chiari II malformation. Craniocervical decompression. Myelomeningocele. Ventriculoperitoneal shunt. Spina bifida Chiari 2 malformation (CM-II) is a myelomeningocele (MMC) associated anomaly consisting of downward displace
Asian journal of neurosurgery, 2023
Background Chiari malformation type II (CM-II) may not always present as an asymptomatic disorder but prove to be difficult in managing. This is especially true for neonates who show the worst prognosis. There is confounding data over whether shunting or craniocervical junction (CVJ) decompression should be employed. This retrospective analysis summarizes the results of 100 patients diagnosed and treated for CM-II along with hydrocephalus and myelomeningocele. Methods We reviewed all the children who were diagnosed and surgically treated for CM-II at the Moscow Regional Hospital. Surgical timing was decided on the clinical conditions of each patient. Urgent surgery in the more compromised patients (usually infants) and elective surgery for patients with less severe conditions was performed. All patients first underwent CVJ decompression. Results The retrospective review yielded 100 patients operated on for CM-II with concomitant hydrocephalus and myelomeningocele. The average herniation was 11.2 AE 5.1 mm. However, herniation level did not correlate with clinical findings. Concomitant syringomyelia was observed in 60% of patients. More severe spinal deformity was observed in patients with widespread syringomyelia (p ¼ 0.04). In children of the younger age group, cerebellar symptoms and bulbar disorders were more frequently observed (p ¼ 0.03), and cephalic syndrome was noted much less frequently (p ¼ 0.005). The severity of scoliotic deformity correlated with the prevalence of syringomyelia (p ¼ 0.03). Satisfactory results were significantly more often observed in patients of the older age group (p ¼ 0.02). Patients with unsatisfactory results at the time of treatment were significantly younger (p ¼ 0.02). Conclusion If CM-II is asymptomatic, then no specific treatment is prescribed. If the patient develops pain in the occiput and neck, then pain relievers are prescribed. If a
Two Cases of Arnold-Chiari Malformation with Respiratory Failure
European Journal of Case Reports in Internal Medicine
Arnold-Chiari malformation is defined as downward displacement of the brainstem and cerebellum through the foramen magnum. It has different clinical presentations and four subtypes. It is known that downward migration of posterior fossa components through the foramen magnum and associated lower cranial nerve palsy and brainstem compression can cause respiratory failure. Acute respiratory failure could mark the onset of the disease. Posterior fossa decompression performed to treat primary disease can improve the central sleep abnormalities. As respiratory failure is rarely seen, this paper presents two cases of Arnold-Chiari malformation with respiratory failure.
The International Journal of Frontier Sciences, 2020
Objective: to investigate the role of CT and MRI in diagnosis of Arnold chiari malformation of hydrocephalus and its treatment with VP shunt along with complications. Methodology: Fifty patients of congenital hydrocephalus who were not treated previously were selected. Detailed history about disease and clinical examination of patients was performed. Follow ups were done at neurosurgery OPD. SPSS software for data analysis was used and mean ± SD, frequency and percentages were calculated for variables. P value ≤0.05 was considered as significant. Results: Treatment in case of congenital hydrocephalus as VP shunt, intra-aneurysmal coiling, excision of meningomyelocele with VP shunt and suboccipital craniectomy + upper cervical laminectomy (scucl) were observed as 10%, 6%, 8% and 8% respectively. Twelve percent of patients were not treated. Conclusion: CT and MRI are the main diagnostic tools for diagnosis of Arnold chiari malformation and VP shunt is the treatment of choice. Among complications of VP shunt infection of shunt and shunt block are the main complications.