Type I Arnold-Chiari malformation (ACM) usually presents in adulthood and consists of a downward displacement of the cerebellar tonsils through the foramen magnum. A 25-year-old woman presented with a 5-month history ...Type I Arnold-Chiari malformation (ACM) usually presents in adulthood and consists of a downward displacement of the cerebellar tonsils through the foramen magnum. A 25-year-old woman presented with a 5-month history of headache associated with blurred vision, tinnitus and sickness. Imaging recognised the need for surgical intervention, but whilst awaiting for surgery she fell pregnant. Considering the risks of neurological deterioration, the woman underwent surgical decompression of type I ACM at 15 weeks gestation. She subsequently presented with progressively worsening headaches during late pregnancy from 35 weeks. The obstetric plan was initially induction of labour at term but since the onset of worsening symptoms, this date was brought forward to 39 + 1 weeks gestation. She proceeded to have a normal delivery with no neonatal complications and an uneventful puerperium followed. Since the delivery, the patient reported fewer symptoms, showed no signs of neurological deficit and a repeat magnetic resonance imaging of the head showed good relief of neural compression. This case illustrates how judicious selection of the appropriate mode of delivery of women following surgically corrected ACM and a multidisciplinary approach is critical in the successful management of the antepartum period and labour.展开更多
Objective: To verify the efficacy of cochlear implantation in a patient with Arnold-Chiari syndrome. Design: Review of a medical chart from a patient with Arnold-Chiari syndrome that underwent to cochlear implant surg...Objective: To verify the efficacy of cochlear implantation in a patient with Arnold-Chiari syndrome. Design: Review of a medical chart from a patient with Arnold-Chiari syndrome that underwent to cochlear implant surgery. Case report: A 6 year-old female, with meningomyelocele and congenital hydrocephalus, deaf in the past 3 years, after meningitis. At that time, she used oral language with adequate speech development. Tone audiometry revealed bilateral profound sensorineural hearing loss. The MRI was compatible with the Arnold-Chiari syndrome. Cochlear implantation was performed on the left ear with MED-EL? Implant (model SONATATi100). Currently, the child is 12 year-old, performs speech therapy three times a week, with good hearing and language development. Conclusion: Despite the presence of Arnold-Chiari syndrome (Chiari type II) with neurological disorders and with ventriculoperitoneal shunt, patient had good hearing results after cochlear implant surgery.展开更多
Background About 50%--70% of patients with Chiad malformation I （CMI） presented with syringomyelia （SM）, which is supposed to be related to abnormal cerebrospinal fluid （CSF） flow around the foramen magnum. The ...Background About 50%--70% of patients with Chiad malformation I （CMI） presented with syringomyelia （SM）, which is supposed to be related to abnormal cerebrospinal fluid （CSF） flow around the foramen magnum. The aim of this study was to investigate the cerebrospinal fluid dynamics at levels of the aqueduct and upper cervical spine in patients with CMI associated with SM, and to discuss the possible mechanism of formation of SM. Methods From January to Apdl 2004, we examined 10 adult patients with symptomatic CMI associated with SM and 10 healthy volunteers by phase-contrast MRI. CSF flow patterns were evaluated at seven regions of interest （ROI）： the aqueduct and ventral and dorsal subarachnoid spaces of the spine at levels of the cerebellar tonsil, C2-3, and C5-6. The CSF flow waveforms were analyzed by measuring CSF circulation time, durations and maximum velocities of cranial- and caudal-directed flows, and the ratio between the two maximum velocities. Data were analyzed by ttest using SPSS 11.5. Results We found no definite communication between the fourth ventricle and syringomyelia by MRI in the 10 patients. In both the groups, we observed cranial-directed flow of CSF in the early cardiac systolic phase, which changed the direction from cranial to caudal from the middle systolic phase to the early diastolic phase, and then turned back in cranial direction in the late diastolic phase. The CSF flow disappeared at the dorsal ROI at the level of C2-3 in 3 patients and 1 volunteer, and at the level of C5-6 in 6 patients and 3 volunteers. The durations of CSF circulation at all the ROIs were significantly shorter in the patients than those in the healthy volunteers （P=-0.014 at the midbrain aqueduct, P=-0.019 at the inferior margin of the cerebellar tonsil, P=-0.014 at the level of C2-3, and P=-0.022 at the level of C5-6）. No significant difference existed between the two groups in the initial point and duration of the caudal-directed CSF flow during a cardiac cycle at all the ROIs. The maximum velocities of both cranial- and caudal-directed CSF flows were significantly higher in the patients than those in the volunteers at the aqueduct （P=-0.018 and P=-0.007） and ventral ROI at the inferior margin of the cerebellar tonsil （P〈0.001 and P=-0.002）, as so did the maximum velocities of the caudal-directed flow in the ventral and dorsal ROIs at the level of C2-3 （P=-0.004; P=-0.007）. Conclusions The direction of CSF flow changes in accordance with cardiac cycle. The syringomyelia in patients with CMI may be due to the decreased circulation time and abnormal dynamics of the CSF in the upper cervical segment. The decompression of the foramen magnum with dural plasty is an alternative for patients with CMI associated with SM.展开更多
文摘Type I Arnold-Chiari malformation (ACM) usually presents in adulthood and consists of a downward displacement of the cerebellar tonsils through the foramen magnum. A 25-year-old woman presented with a 5-month history of headache associated with blurred vision, tinnitus and sickness. Imaging recognised the need for surgical intervention, but whilst awaiting for surgery she fell pregnant. Considering the risks of neurological deterioration, the woman underwent surgical decompression of type I ACM at 15 weeks gestation. She subsequently presented with progressively worsening headaches during late pregnancy from 35 weeks. The obstetric plan was initially induction of labour at term but since the onset of worsening symptoms, this date was brought forward to 39 + 1 weeks gestation. She proceeded to have a normal delivery with no neonatal complications and an uneventful puerperium followed. Since the delivery, the patient reported fewer symptoms, showed no signs of neurological deficit and a repeat magnetic resonance imaging of the head showed good relief of neural compression. This case illustrates how judicious selection of the appropriate mode of delivery of women following surgically corrected ACM and a multidisciplinary approach is critical in the successful management of the antepartum period and labour.
文摘Objective: To verify the efficacy of cochlear implantation in a patient with Arnold-Chiari syndrome. Design: Review of a medical chart from a patient with Arnold-Chiari syndrome that underwent to cochlear implant surgery. Case report: A 6 year-old female, with meningomyelocele and congenital hydrocephalus, deaf in the past 3 years, after meningitis. At that time, she used oral language with adequate speech development. Tone audiometry revealed bilateral profound sensorineural hearing loss. The MRI was compatible with the Arnold-Chiari syndrome. Cochlear implantation was performed on the left ear with MED-EL? Implant (model SONATATi100). Currently, the child is 12 year-old, performs speech therapy three times a week, with good hearing and language development. Conclusion: Despite the presence of Arnold-Chiari syndrome (Chiari type II) with neurological disorders and with ventriculoperitoneal shunt, patient had good hearing results after cochlear implant surgery.
基金the Foundation of Peking University Third Hospital(YZZ05-17-07)
文摘Background About 50%--70% of patients with Chiad malformation I （CMI） presented with syringomyelia （SM）, which is supposed to be related to abnormal cerebrospinal fluid （CSF） flow around the foramen magnum. The aim of this study was to investigate the cerebrospinal fluid dynamics at levels of the aqueduct and upper cervical spine in patients with CMI associated with SM, and to discuss the possible mechanism of formation of SM. Methods From January to Apdl 2004, we examined 10 adult patients with symptomatic CMI associated with SM and 10 healthy volunteers by phase-contrast MRI. CSF flow patterns were evaluated at seven regions of interest （ROI）： the aqueduct and ventral and dorsal subarachnoid spaces of the spine at levels of the cerebellar tonsil, C2-3, and C5-6. The CSF flow waveforms were analyzed by measuring CSF circulation time, durations and maximum velocities of cranial- and caudal-directed flows, and the ratio between the two maximum velocities. Data were analyzed by ttest using SPSS 11.5. Results We found no definite communication between the fourth ventricle and syringomyelia by MRI in the 10 patients. In both the groups, we observed cranial-directed flow of CSF in the early cardiac systolic phase, which changed the direction from cranial to caudal from the middle systolic phase to the early diastolic phase, and then turned back in cranial direction in the late diastolic phase. The CSF flow disappeared at the dorsal ROI at the level of C2-3 in 3 patients and 1 volunteer, and at the level of C5-6 in 6 patients and 3 volunteers. The durations of CSF circulation at all the ROIs were significantly shorter in the patients than those in the healthy volunteers （P=-0.014 at the midbrain aqueduct, P=-0.019 at the inferior margin of the cerebellar tonsil, P=-0.014 at the level of C2-3, and P=-0.022 at the level of C5-6）. No significant difference existed between the two groups in the initial point and duration of the caudal-directed CSF flow during a cardiac cycle at all the ROIs. The maximum velocities of both cranial- and caudal-directed CSF flows were significantly higher in the patients than those in the volunteers at the aqueduct （P=-0.018 and P=-0.007） and ventral ROI at the inferior margin of the cerebellar tonsil （P〈0.001 and P=-0.002）, as so did the maximum velocities of the caudal-directed flow in the ventral and dorsal ROIs at the level of C2-3 （P=-0.004; P=-0.007）. Conclusions The direction of CSF flow changes in accordance with cardiac cycle. The syringomyelia in patients with CMI may be due to the decreased circulation time and abnormal dynamics of the CSF in the upper cervical segment. The decompression of the foramen magnum with dural plasty is an alternative for patients with CMI associated with SM.
