Usefulness of Phase Contrast Cine Mode Magnetic Resonance Imaging for Surgical Decision Making in Patients With Hydrocephalus Combined With Achondroplasia  -Case Report-

Miyamoto, Junichi; Tatsuzawa, Kazunori; Sasajima, Hiroyasu; Mineura, Katsuyoshi

doi:10.2176/nmc.50.1116

Cited by 10 publications

(7 citation statements)

References 8 publications

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“…Although this is likely the most important mechanism, there is also evidence that obliteration of cerebrospinal fluid flow at the craniocervical junction may be a factor in development of hydrocephalus, too [128, 129, 201, 202]. It is not unreasonable to posit that there are two distinct processes that can give rise to increased intracranial pressure in those with achondroplasia.…”

Section: Natural History and Managementmentioning

confidence: 99%

Achondroplasia: a comprehensive clinical review

Pauli¹

2019

Orphanet J Rare Dis

323

374

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Achondroplasia is the most common of the skeletal dysplasias that result in marked short stature (dwarfism). Although its clinical and radiologic phenotype has been described for more than 50 years, there is still a great deal to be learned about the medical issues that arise secondary to this diagnosis, the manner in which these are best diagnosed and addressed, and whether preventive strategies can ameliorate the problems that can compromise the health and well being of affected individuals. This review provides both an updated discussion of the care needs of those with achondroplasia and an exploration of the limits of evidence that is available regarding care recommendations, controversies that are currently present, and the many areas of ignorance that remain.

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Section: Natural History and Managementmentioning

confidence: 99%

Achondroplasia: a comprehensive clinical review

Pauli¹

2019

Orphanet J Rare Dis

323

374

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“…Interestingly, none of these patients were found to have any obstruction of CSF or delay in transit of contrast materials from the ventricular system to the basal cisterns, spinal canal, and into the subarachnoid space over the hemispheres. However, in a prior study by our group 6 and a case report by Miyamoto et al, 14 several patients were noted to have decreased or obliterated CSF flow at the cervicomedullary junction as demonstrated using MR CSF flow studies, presenting the possibility that at least in some patients with foramen magnum stenosis there may be additional factors leading to ventriculomegaly or hydrocephalus. Additionally, in both the study by Steinbok et al and Miyamoto et al, there was a compensatory increase in occipital and marginal sinus flow as well as condylar emissary vein dilation in the presence of jugular foraminal stenosis.…”

Section: Discussionmentioning

confidence: 72%

Dynamic cervicomedullary cord compression and alterations in cerebrospinal fluid dynamics in children with achondroplasia: review of an 11-year surgical case series

Mukherjee

Pressman

Krakow

et al. 2014

PED

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Object Achondroplasia may be associated with compression at the cervicomedullary junction. Determining which patients are at greatest risk for neurological complications of cervicomedullary compression can be difficult. In the current study the authors reviewed their records to determine the incidence and clinical significance of dynamic cervicomedullary stenosis and obstruction of CSF flow along with surgical outcomes following posterior fossa decompression. Methods The authors reviewed 34 consecutive cases involving symptomatic children with achondroplasia undergoing cervicomedullary decompression performed by a single surgeon over 11 years. Of these patients, 29 had undergone preoperative dynamic MRI of the cervicomedullary junction with cine (cinema) CSF flow studies; 13 of these patients underwent postoperative dynamic MRI studies. Clinical outcomes included changes in polysomnography, head circumference percentile, and fontanel characteristics. Radiographic outcomes included changes in dynamic spinal cord diameter, improvement in CSF flow at the foramen magnum, and change in the Evans ratio. Results Patients were predominantly female, with a mean age at presentation of 6.6 years and mean follow-up of 3.7 years (range 1–10 years). All patients had moderate to excellent improvement in postoperative polysomnography, slight decrease in average head circumference percentile (from 46.9th percentile to 45.7th percentile), and no subjective worsening of fontanel characteristics. The Evans ratio decreased by 2%, spinal cord diameter increased an average of 3.1 mm, 5.2 mm, and 0.2 mm in the neutral, flexed, and extended positions, respectively, and CSF flow improved qualitatively in all 3 positions. There were no postoperative infections, CSF leaks, or other major complications. None of the patients undergoing initial foramen magnum decompression performed at our medical center required reoperation. Conclusions Patients with achondroplasia and symptomatic cervicomedullary compression have increased risk of dynamic stenosis at the foramen magnum evident upon dynamic cine MRI. Operative decompression may be offered with low risk of complications or need for reoperation.

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“…1b). [3] CSF studies also provide a better understanding of the pathophysiology of Chiari I malformation regarding the spatial and temporal foramen magnum CSF flow patterns (Figs 1c -e …”

Section: Indications For Csf Flow Studies In Paediatricsmentioning

confidence: 99%

“…• achondroplasia [3] • Chiari I malformation [6] • patency of third ventriculostomy. [5] REVIEW Line graphs demonstrating different time courses for CSF flow use the Y-axis to show the magnitude of flow in positive and negative directions in ml/s; the X-axis shows time as a decimal fraction of the cardiac cycle.…”

Section: Table 1 Indications For Performing Csf Flow Studies In Chilmentioning

confidence: 99%