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Journal articles on the topic 'Foramen magnum stenosis'

Author: Grafiati

Published: 4 June 2021

Last updated: 1 February 2022

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1

Hecht, J. T., W. A. Horton, I. J. Butler, et al. "Foramen magnum stenosis in homozygous achondroplasia." European Journal of Pediatrics 145, no. 6 (1986): 545–47. http://dx.doi.org/10.1007/bf02429060.

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Nadel, Jeffrey L., D. Andrew Wilkinson, Hugh J. L. Garton, Karin M. Muraszko, and Cormac O. Maher. "Screening and surgery for foramen magnum stenosis in children with achondroplasia: a large, national database analysis." Journal of Neurosurgery: Pediatrics 23, no. 3 (2019): 374–80. http://dx.doi.org/10.3171/2018.9.peds18410.

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OBJECTIVEThe goal of this study was to determine the rates of screening and surgery for foramen magnum stenosis in children with achondroplasia in a large, privately insured healthcare network.METHODSRates of screening and surgery for foramen magnum stenosis in children with achondroplasia were determined using de-identified insurance claims data from a large, privately insured healthcare network of over 58 million beneficiaries across the United States between 2001 and 2014. Cases of achondroplasia and screening and surgery claims were identified using a combination of International Classific

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Bagley, Carlos A., Jonathan A. Pindrik, Markus J. Bookland, Joaquin Q. Camara-Quintana, and Benjamin S. Carson. "Cervicomedullary decompression for foramen magnum stenosis in achondroplasia." Journal of Neurosurgery: Pediatrics 104, no. 3 (2006): 166–72. http://dx.doi.org/10.3171/ped.2006.104.3.166.

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Jung, Ileok, Jin-Man Jung, and Moon Ho Park. "Rotatory Vertebral Artery Syndrome in Foramen Magnum Stenosis." Research in Vestibular Science 17, no. 4 (2018): 167–69. http://dx.doi.org/10.21790/rvs.2018.17.4.167.

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Jha, Ruchira M., Paul Klimo, and Edward R. Smith. "Foramen magnum stenosis from overgrowth of the opisthion in a child with achondroplasia." Journal of Neurosurgery: Pediatrics 2, no. 2 (2008): 136–38. http://dx.doi.org/10.3171/ped/2008/2/8/136.

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Achondroplasia has a known association with foramen magnum stenosis that can result in cervicomedullary compression, which is most often due to a hypertrophied posterior occipital rim and an undersized transverse diameter. The authors present a unique case of a child with achondroplasia with symptomatic craniocervical compression from marked overgrowth of his opisthion anterior to the posterior arch of the atlas. This 22-month-old child with achondroplasia presented with severe respiratory and motor disabilities, including progressive quadriparesis and apneic episodes requiring continuous posi

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Ok, Michale S., Usha D. Nagaraj, and Mohamed Mahmoud. "Foramen Magnum Stenosis and Spinal Cord Compression in Achondroplasia." Anesthesiology 131, no. 2 (2019): 396. http://dx.doi.org/10.1097/aln.0000000000002757.

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Shoji, Hidehiko, Yasunobu Ito, Kazuo Mizoi, Atushi Sugawara, and Tadahiko Ito. "Achondroplasia associated with Foramen Magnum Stenosis : Report of Two Cases." Japanese Journal of Neurosurgery 12, no. 10 (2003): 707–11. http://dx.doi.org/10.7887/jcns.12.707.

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Savarirayan, Ravi, Melita Irving, Wirginia Maixner, et al. "Rationale, design, and methods of a randomized, controlled, open-label clinical trial with open-label extension to investigate the safety of vosoritide in infants, and young children with achondroplasia at risk of requiring cervicomedullary decompression surgery." Science Progress 104, no. 1 (2021): 003685042110037. http://dx.doi.org/10.1177/00368504211003782.

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Achondroplasia causes narrowing of the foramen magnum and the spinal canal leading to increased mortality due to cervicomedullary compression in infants and significant morbidity due to spinal stenosis later in adulthood. Vosoritide is a C-natriuretic peptide analogue that has been shown to improve endochondral ossification in children with achondroplasia. The objective of this trial is to evaluate the safety of vosoritide and whether vosoritide can improve the growth of the foramen magnum and spinal canal in children that may require decompression surgery. An Achondroplasia Foramen Magnum Sco

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Vender, John R., Steven J. Harrison, and Dennis E. McDonnell. "Fusion and instrumentation at C1–3 via the high anterior cervical approach." Journal of Neurosurgery: Spine 92, no. 1 (2000): 24–29. http://dx.doi.org/10.3171/spi.2000.92.1.0024.

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Object. The high anterior cervical, retropharyngeal approach to the anterior foramen magnum and upper cervical spine is a favorable alternative to the transoral and posterolateral approaches, which both cause instability of the craniovertebral junction. Previously, such instability was corrected via an occipitocervical fusion during a separate surgical procedure. Methods. Seven patients requiring C-2 corpectomy (foramen magnum meningioma [two patients], critical stenosis secondary to rheumatoid arthritis [two patients], C-2 fracture [two patients], and stenosis secondary to Rickets [one patien

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Barone, Constance M., Sidney Eisig, David F. Jimenez, Ravelo V. Argamaso, and Robert J. Shprintzen. "Achondroplasia: Pre- and Postsurgical Considerations for Midface Advancement." Cleft Palate-Craniofacial Journal 31, no. 1 (1994): 74–77. http://dx.doi.org/10.1597/1545-1569_1994_031_0074_apapcf_2.3.co_2.

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Achondroplasia, the most common type of dwarfism, Involves several physical characteristics that can cause pre- and intraoperative respiratory problems. The characteristics Include rib-cage deformities, midface hypoplasia, choanal stenosis, muscular hypotonia, foramen magnum compression, and cervical abnormalities. Specific pre- and postoperative strategies for dealing with achondroplastic dwarfs are discussed.

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Gadot, Ron, Sricharan Gopakumar, Kathryn Wagner, et al. "Foramen Magnum Dural Arteriovenous Fistula Presenting With Thoracic Myelopathy: Technical Case Report With 2-Dimensional Operative Video." Operative Neurosurgery 21, no. 1 (2021): E55—E59. http://dx.doi.org/10.1093/ons/opab077.

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Abstract BACKGROUND AND IMPORTANCE Dural arteriovenous fistulas (dAVFs) are vascular abnormalities of the central nervous system that can cause a wide array of neurological dysfunction depending on their location, flow, and propensity to rupture. Symptomatic dAVFs at the cranio-cervical junction usually result in hemorrhage or cervical myelopathy. Distantly located dAVFs of the foramen magnum are a rare cause of thoracic intrinsic myelopathy. CLINICAL PRESENTATION An 83-yr-old man presented with progressive lower extremity weakness, numbness, and difficulty walking along with episodes of bowel

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Mukherjee, Debraj, Barry D. Pressman, Deborah Krakow, David L. Rimoin, and Moise Danielpour. "Dynamic cervicomedullary cord compression and alterations in cerebrospinal fluid dynamics in children with achondroplasia: review of an 11-year surgical case series." Journal of Neurosurgery: Pediatrics 14, no. 3 (2014): 238–44. http://dx.doi.org/10.3171/2014.5.peds12614.

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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 decompr

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Swift, Dale, Laszlo Nagy, and Brian Robertson. "Endoscopic third ventriculostomy in hydrocephalus associated with achondroplasia." Journal of Neurosurgery: Pediatrics 9, no. 1 (2012): 73–81. http://dx.doi.org/10.3171/2011.10.peds1169.

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Hydrocephalus in patients with achondroplasia is thought to be due to increased dural sinus venous pressure resulting from narrowing of the jugular foramen. In this setting, where hydrocephalus is presumed to be “vascular” in origin and therefore communicating, endoscopic third ventriculostomy (ETV) would seem contraindicated. The authors describe 3 patients in whom ETV was successfully performed, resulting in MR imaging–documented decreases in ventricle size. The patients were 11 months, 33 months, and 13 years at the time of surgery. All patients had serial preoperative MR images demonstrati

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Cheshier, Samuel Henry, Mohammad Yashar Sorena Kalani, Arjun Pendakaur, et al. "Two-year-old girl with cervicomedullary junction stenosis and an unknown type of skeletal dysplasia." Journal of Neurosurgery: Pediatrics 2, no. 3 (2008): 200–202. http://dx.doi.org/10.3171/ped/2008/2/9/200.

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The authors present a novel case of skeletal dysplasia in a 2.8-year-old girl. The patient presented with progressive lower cranial nerve palsy and myelopathy due to constriction at the cervicomedullary junction caused by overgrowth of the occipital bone of the foramen magnum and the C-1. She also had prominent bone overgrowth of the superior orbital ridges, resulting in excessive stretching of periorbital skin and an inability to fully close her eyes.

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Matsushita, Masaki, Kenichi Mishima, Ryusaku Esaki, Naoki Ishiguro, Kinji Ohno, and Hiroshi Kitoh. "Maternal administration of meclozine for the treatment of foramen magnum stenosis in transgenic mice with achondroplasia." Journal of Neurosurgery: Pediatrics 19, no. 1 (2017): 91–95. http://dx.doi.org/10.3171/2016.7.peds16199.

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OBJECTIVE Achondroplasia (ACH) is the most common short-limbed skeletal dysplasia caused by gain-of-function mutations in the fibroblast growth factor receptor 3 (FGFR3) gene. Foramen magnum stenosis (FMS) is one of the serious neurological complications in ACH. Through comprehensive drug screening, the authors identified that meclozine, an over-the-counter drug for motion sickness, inhibited activation of FGFR3 signaling. Oral administration of meclozine to the growing ACH mice promoted longitudinal bone growth, but it did not prevent FMS. In the current study, the authors evaluated the effec

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Vukovic, Milos, Jelena Vukovic, Milos Maletin, and Radenko Vukovic. "Analysis of sudden death caused by the unusual shape of the foramen magnum stenosis." Vojnosanitetski pregled, no. 00 (2020): 22. http://dx.doi.org/10.2298/vsp190522022v.

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Sano, Masakazu, Nao Takahashi, Keisuke Nagasaki, Makoto Oishi, Junichi Yoshimura, and Yukihiko Fujii. "Polysomnography as an indicator for cervicomedullary decompression to treat foramen magnum stenosis in achondroplasia." Child's Nervous System 34, no. 11 (2018): 2275–81. http://dx.doi.org/10.1007/s00381-018-3880-0.

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White, Klane K., Ravi Savarirayan, Michael J. Goldberg, et al. "Response: “Best practices in the evaluation and treatment of foramen magnum stenosis in achondroplasia during infancy” and “is there a correlation between sleep disordered breathing and foramen magnum stenosis in children with achondroplasia?”." American Journal of Medical Genetics Part A 170, no. 4 (2016): 1101–3. http://dx.doi.org/10.1002/ajmg.a.37546.

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Giordano, Flavio, Matteo Lenge, Pierarturo Donati, et al. "Exclusive Neurogenic Bladder and Fecal Incontinency in an Achondroplasic Child Successfully Treated with Lumbar Foraminal Decompression." Pediatric Neurosurgery 56, no. 5 (2021): 471–76. http://dx.doi.org/10.1159/000517652.

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<b><i>Introduction:</i></b> Achondroplasia is a genetic disorder characterized by defects in the development of endochondral bone resulting in skeletal abnormalities like stenosis of the foramen magnum and of the spine, shortened limb bones, and macrocephaly. Congenital spinal stenosis is frequent and due to premature fusion of the pedicles to the laminae. <b><i>Case Presentation:</i></b> We report a case of neurogenic bladder and fecal incontinence due to lumbar stenosis successfully treated with L1–L5 partial laminectomy and foraminotomy in a 7

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ARISHIMA, Hidetaka, Kenzo TSUNETOSHI, Toshiaki KODERA, Ryuhei KITAI, Hiroaki TAKEUCHI, and Ken-ichiro KIKUTA. "Intraoperative Computed Tomography for Cervicomedullary Decompression of Foramen Magnum Stenosis in Achondroplasia: Two Case Reports." Neurologia medico-chirurgica 53, no. 12 (2013): 902–6. http://dx.doi.org/10.2176/nmc.cr2012-0421.

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Kashanian, Alon, Julie Chan, Debraj Mukherjee, Barry D. Pressman, Deborah Krakow, and Moise Danielpour. "Improvement in ventriculomegaly following cervicomedullary decompressive surgery in children with achondroplasia and foramen magnum stenosis." American Journal of Medical Genetics Part A 182, no. 8 (2020): 1896–905. http://dx.doi.org/10.1002/ajmg.a.61640.

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White, Klane K., Viviana Bompadre, Michael J. Goldberg, et al. "Best practices in the evaluation and treatment of foramen magnum stenosis in achondroplasia during infancy." American Journal of Medical Genetics Part A 170, no. 1 (2015): 42–51. http://dx.doi.org/10.1002/ajmg.a.37394.

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Senter, Howard J., Sami M. Bittar, and Edwin T. Long. "Revascularization of the extracranial vertebral artery at any level without cross-clamping." Journal of Neurosurgery 62, no. 3 (1985): 334–39. http://dx.doi.org/10.3171/jns.1985.62.3.0334.

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✓ Hemodynamic insufficiency resulting from extracranial vertebral artery stenosis or occlusion is believed to be the major cause of vertebrobasilar transient ischemic attacks. The major difficulties in treating this disorder have been exposure of the vertebral artery distal to the stenosis and the risk of vertebral artery cross-clamping for vein grafting or carotid artery transposition. The authors describe a new technique for vertebral artery reconstruction at any level by the use of an intraluminal shunt, thus avoiding the necessity to cross-clamp the artery. This procedure was successfully

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Fornarino, Stefania, Daniela Paola Rossi, Mariasavina Severino, et al. "Early impairment of somatosensory evoked potentials in very young children with achondroplasia with foramen magnum stenosis." Developmental Medicine & Child Neurology 59, no. 2 (2016): 192–98. http://dx.doi.org/10.1111/dmcn.13243.

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White, Klane K., Shawn E. Parnell, Yemiserach Kifle, Marcella Blackledge, and Viviana Bompadre. "Is there a correlation between sleep disordered breathing and foramen magnum stenosis in children with achondroplasia?" American Journal of Medical Genetics Part A 170, no. 1 (2015): 32–41. http://dx.doi.org/10.1002/ajmg.a.37385.

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Aryanpur, John, Orest Hurko, Clair Francomano, Henry Wang, and Benjamin Carson. "Craniocervical decompression for cervicomedullary compression in pediatric patients with achondroplasia." Journal of Neurosurgery 73, no. 3 (1990): 375–82. http://dx.doi.org/10.3171/jns.1990.73.3.0375.

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✓ The congenital osseous abnormalities associated with achondroplasia include stenosis of the foramen magnum and the upper cervical spinal canal. In the pediatric achondroplastic patient, such stenosis may lead to cervicomedullary compression with serious sequelae, including paresis, hypertonia, delayed motor milestones, and respiratory compromise. Using a standardized protocol the authors have treated 15 young achondroplastic patients with documented cervicomedullary compression by craniocervical decompression and duroplasty. Following this procedure, significant improvement in presenting neu

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Knisely, A. S., and Don B. Singer. "A technique for necropsy evaluation of stenosis of the foramen magnum and rostral spinal canal in osteochondrodysplasia." Human Pathology 19, no. 12 (1988): 1372–75. http://dx.doi.org/10.1016/s0046-8177(88)80228-4.

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Boulos, Alan S., Eric M. Deshaies, Jiang Qian, and A. John Popp. "Preoperative stent placement for intradural vertebral artery stenosis from a rare xanthogranuloma." Journal of Neurosurgery 101, no. 5 (2004): 864–68. http://dx.doi.org/10.3171/jns.2004.101.5.0864.

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✓ In this report, the authors discuss a novel use of intradural vertebral artery (VA) stent placement to protect a tumor-encased vessel from injury during lesion resection. The tumor was a rare foramen magnum region xanthogranuloma and a component of Erdheim—Chester disease (ECD). This 64-year-old man presented with large masses encasing and compressing the intracranial segments of each VA. Preoperatively, a left VA stent was placed to protect the arterial wall during resection of the tumor. Histopathological study results on the subtotally resected mass were consistent with xanthogranuloma, a

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Knisely, A. S., and Carmen K. Steigman. "Stenosis of the Foramen Magnum and Rostral Spinal Canal, With Spinal Cord Deformity, In Jeune's Asphyxiating Thoracic Dystrophy." Pediatric Pathology 9, no. 3 (1989): 299–305. http://dx.doi.org/10.3109/15513818909037734.

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Teresa, De Luca, Pietravalle Andrea, Castellano Chiara, Boccanera Francesca, Ferro Rosalia, and Colarizi Patrizia. "Resolution of apnea spells after sub occipital decompression in a 2-month-old infant with foramen magnum stenosis." Early Human Development 84 (November 2008): S10. http://dx.doi.org/10.1016/j.earlhumdev.2008.09.025.

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Ryken, Timothy C., and Arnold H. Menezes. "Cervicomedullary compression in achondroplasia." Journal of Neurosurgery 81, no. 1 (1994): 43–48. http://dx.doi.org/10.3171/jns.1994.81.1.0043.

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✓ 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

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Kahraman, Sinan, Meriç Enercan, Özkan Demirhan, Türker Şengül, Levent Dalar, and Azmi Hamzaoğlu. "Pneumomediastinum, Subcutaneous Emphysema, and Tracheal Tear in the Early Postoperative Period of Spinal Surgery in a Paraplegic Achondroplastic Dwarf." Case Reports in Orthopedics 2013 (2013): 1–4. http://dx.doi.org/10.1155/2013/987578.

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Achondroplasia was first described in 1878 and is the most common form of human skeletal dysplasia. Spinal manifestations include thoracolumbar kyphosis, foramen magnum, and spinal stenosis. Progressive kyphosis can result in spinal cord compression and paraplegia due to the reduced size of spinal canal. The deficits are typically progressive, presenting as an insidious onset of paresthesia, followed by the inability to walk and then by urinary incontinence. Paraplegia can be the result of direct pressure on the cord by bone or the injury to the anterior spinal vessels by a protruding bone. Su

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Khalid, Kirran, and Asif Saifuddin. "Pictorial review: imaging of the spinal manifestations of achondroplasia." British Journal of Radiology 94, no. 1123 (2021): 20210223. http://dx.doi.org/10.1259/bjr.20210223.

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Achondroplasia is the commonest hereditary skeletal dysplasia exhibiting dwarfism with characteristic rhizomelic (proximal) shortening of the limbs. It is predominantly linked with an autosomal dominant inheritance, but sporadic mutations can occur which are associated with advanced maternal age. Approximately 1 in every 25 000–30 000 live births are affected, and the overall life expectancy is marginally reduced by ~10 years. Mutations in the fibroblast growth factor receptor causes a decrease in endochondral ossification, which results in stunted growth of cartilaginous bones. A resultant na

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Wrobel, Wiktoria, Emilia Pach, and Iwona Ben-Skowronek. "Advantages and Disadvantages of Different Treatment Methods in Achondroplasia: A Review." International Journal of Molecular Sciences 22, no. 11 (2021): 5573. http://dx.doi.org/10.3390/ijms22115573.

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Achondroplasia (ACH) is a disease caused by a missense mutation in the FGFR3 (fibroblast growth factor receptor 3) gene, which is the most common cause of short stature in humans. The treatment of ACH is necessary and urgent because untreated achondroplasia has many complications, both orthopedic and neurological, which ultimately lead to disability. This review presents the current and potential pharmacological treatments for achondroplasia, highlighting the advantages and disadvantages of all the drugs that have been demonstrated in human and animal studies in different stages of clinical tr

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Yotsumoto, Takafumi, Naomi Morozumi, Mayumi Furuya, et al. "Foramen magnum stenosis and midface hypoplasia in C-type natriuretic peptide-deficient rats and restoration by the administration of human C-type natriuretic peptide with 53 amino acids." PLOS ONE 14, no. 5 (2019): e0216340. http://dx.doi.org/10.1371/journal.pone.0216340.

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Noudel, Rémy, Nicolas Jovenin, Cristophe Eap, Bernard Scherpereel, Laurent Pierot, and Pascal Rousseaux. "Incidence of basioccipital hypoplasia in Chiari malformation Type I: comparative morphometric study of the posterior cranial fossa." Journal of Neurosurgery 111, no. 5 (2009): 1046–52. http://dx.doi.org/10.3171/2009.2.jns08284.

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Object The chronic tonsillar herniation defining Chiari malformation Type I (CMI) is thought to result from overcrowding of a normally developing hindbrain within a congenitally small posterior cranial fossa (PCF) due to occipital hypoplasia. The goals in the present study were to authenticate the cranioencephalic disproportion in a group of patients with CMI and to discuss new developmental aspects according to which part of the occipital bone was underdeveloped. Methods The authors retrospectively examined a group of 17 patients with CMI. Measurements of osteotentorial and neural structures

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Matys, Tomasz, Avril Horsburgh, Ramez W. Kirollos, and Tarik F. Massoud. "The Aqueduct of Sylvius: Applied 3-T Magnetic Resonance Imaging Anatomy and Morphometry With Neuroendoscopic Relevance." Operative Neurosurgery 73, no. 2 (2013): ons132—ons140. http://dx.doi.org/10.1227/01.neu.0000430286.08552.ca.

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Abstract BACKGROUND: The aqueduct of Sylvius (AqSylv) is a structure of increasing importance in neuroendoscopic procedures. However, there is currently no clear and adequate description of the normal anatomy of the AqSylv. OBJECTIVE: To study in detail hitherto unavailable normal magnetic resonance imaging morphometry and anatomic variants of the AqSylv. METHODS: We retrospectively studied normal midsagittal T1-weighted 3-T magnetic resonance images in 100 patients. We measured widths of the AqSylv pars anterior, ampulla, and pars posterior; its narrowest point; and its length. We recorded an

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Sharma, Namita A., and Rajendra S. Garud. "FORAMINA OF THE POSTERIOR CRANIAL BASE: A STUDY OF ADULT INDIAN SKULLS. 89 Las foraminas de la base posterior del cráneo: Un estudio en cráneos de indios adultos." Revista Argentina de Anatomía Clínica 3, no. 2 (2016): 89–98. http://dx.doi.org/10.31051/1852.8023.v3.n2.13925.

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Introducción: Las foraminas craneales son los únicos puntos de entrada a un cráneo que, de otra manera, permanecería cerrado. La evaluación de estas foraminas es una parte muy importante para el diagnóstico médico y debería ayudar al clínico en su enfoque quirúrgico a esta delicada región. El presente estudio se centra en las foraminas de la base posterior del cráneo incluyendo los pares de fosas yugulares, el agujero estilomastoideo, el canal hipogloso; el impar agujero magno y otras foraminas auxiliares tales como el agujero mastoideo y el canal condíleo posterior. Material y Método: El estu

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Ward, Leanne M., Josep Maria de Bergua, Marie-Eve Robinson, et al. "Design and Baseline Demographics of a Five-Year, Multi-National Observational Cohort Study of Children With Achondroplasia (ACHieve Study)." Journal of the Endocrine Society 5, Supplement_1 (2021): A712. http://dx.doi.org/10.1210/jendso/bvab048.1449.

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Abstract Background: Achondroplasia (ACH) is the most common form of dwarfism occurring in 1 in 22,000 births (95% CI 18,500 to 26,000). This skeletal dysplasia is caused by a gain-of-function mutation in the fibroblast growth factor receptor 3 (FGFR3) gene located on chromosome 4p16.3 and results in sustained activation of the FGFR3 pathway leading to impaired chondrogenesis and endochondral bone formation. Both typical childhood growth patterns and the impaired linear growth of ACH are modulated by the delicate balance between growth-inhibiting FGFR3/mitogen-activated phosphokinase and growt

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Elliott, Robert, Stephen Kalhorn, Donato Pacione, Howard Weiner, Jeffrey Wisoff, and David Harter. "Shunt malfunction causing acute neurological deterioration in 2 patients with previously asymptomatic Chiari malformation Type I." Journal of Neurosurgery: Pediatrics 4, no. 2 (2009): 170–75. http://dx.doi.org/10.3171/2009.4.peds0936.

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Patients with symptomatic Chiari malformation Type I (CM-I) typically exhibit a chronic, slowly progressive disease course with evolution of symptoms. However, some authors have reported acute neurological deterioration in the setting of CM-I and acquired Chiari malformations. Although brainstem dysfunction has been documented in patients with CM-II and hydrocephalus or shunt malfunction, to the authors' knowledge only 1 report describing ventriculoperitoneal (VP) shunt malfunction causing neurological deterioration in a patient with CM-I exists. The authors report on their experience with the

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S, Choayb, Adil H, and El Haddad S. "Foramen Magnum Stenosis in Achondroplasia." Austin Journal of Radiology 8, no. 4 (2021). http://dx.doi.org/10.26420/austinjradiol.2021.1136.

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Achondroplasia is the most common hereditary skeletal dysplasia and is characterized by disproportionately short stature with rhizomelic short extremities [1]. The skull features include a narrowed foramen magnum, short skull base, and clivus [2]. Foramen magnum stenosis is a characteristic funding, secondary to an abnormal placement and premature fusion of the posterior synchondroses [1]. The second factor responsible for stenosis is a defect in endochondral ossification in the basiocciput that may result in an extension of the squamous occipital bone [2]. It can cause hydrocephalus and promi

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Asilioglu, Nazik, Alper Ates, Ayse Ayzit Kilinc, and Adnan Dagcinar. "Hypochondroplasia With Foramen Magnum Stenosis : A Case Report." Medical Journal of Trakya University, 2009. http://dx.doi.org/10.5174/tutfd.2009.02633.2.

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Cheung, Moira S., Melita Irving, Alessandra Cocca, et al. "Achondroplasia Foramen Magnum Score: screening infants for stenosis." Archives of Disease in Childhood, September 3, 2020, archdischild—2020–319625. http://dx.doi.org/10.1136/archdischild-2020-319625.

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BackgroundAchondroplasia is associated with foramen magnum stenosis (FMS) and significant risk of morbidity and sudden death in infants. A sensitive and reliable method of detecting infants who require decompressive surgery is required. This study aims to describe the incidence and severity of FMS in an unselected, sequential series of infants using a novel MRI score and retrospectively correlate severity with clinical examination and cardiorespiratory sleep (CRS) studies.MethodsThe Achondroplasia Foramen Magnum Score (AFMS) was developed and scores were retrospectively correlated with clinica

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Almeida, Timoteo, William Singleton, Fergal Monsell, Sarah Smithson, Richard Edwards, and Christine Burren. "Management of foramen magnum stenosis in patients with achondroplasia: relative merit of clinical and radiological indications for foramen magnum decompression." Bone Abstracts, July 31, 2019. http://dx.doi.org/10.1530/boneabs.7.p148.

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Cristiano, Antunes, Pereira Paulo, Alves Daniela, Vaz Rui, and Pereira Josué. "Decompressive Surgery for Craniovertebral Foramen Magnum Stenosis with Medullary Compression in Paediatric Skeletal Dysplasia Syndromes." Neurosurgery Cases and Reviews 4, no. 3 (2021). http://dx.doi.org/10.23937/2643-4474/1710073.

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Matsushita, Masaki, Hiroshi Kitoh, Kenichi Mishima, et al. "Meclozine has a potential effects on short stature and foramen magnum stenosis in transgenic mice with achondroplasia." Bone Abstracts, June 9, 2015. http://dx.doi.org/10.1530/boneabs.4.oc13.

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Allegri, Anna Elsa Maria, Iorgi Natascia Di, Mariasavina Severino, et al. "Foramen magnum stenosis (FMS): neuroradiological aspects before and after cervical decompression in paediatric patients with achondroplasia (ACH)." Bone Abstracts, July 31, 2019. http://dx.doi.org/10.1530/boneabs.7.lb10.

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Taylor, Davis G., Ajay Chatrath, Panagiotis Mastorakos, et al. "Cerebrospinal fluid area and syringogenesis in Chiari malformation type I." Journal of Neurosurgery, February 2020, 1–6. http://dx.doi.org/10.3171/2019.11.jns191439.

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OBJECTIVESyringogenesis in Chiari malformation type I (CM-I) is thought to occur secondary to impaction of the cerebellar tonsils within the foramen magnum (FM). However, the correlation between the CSF area and syringogenesis has yet to be elucidated. The authors sought to determine whether the diminution in subarachnoid space is associated with syringogenesis. Further, the authors sought to determine if syrinx resolution was associated with the degree of expansion of subarachnoid spaces after surgery.METHODSThe authors performed a retrospective review of all patients undergoing posterior fos

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Savarirayan, Ravi, Peter Kannu, Carl L. Dambkowski, Daniela Rogoff, and Melita Irving. "SUN-093 Prospective Clinical Assessment Study in Children with Achondroplasia: The PROPEL Trial." Journal of the Endocrine Society 4, Supplement_1 (2020). http://dx.doi.org/10.1210/jendso/bvaa046.557.

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Abstract BACKGROUND: Achondroplasia (ACH) is the most common non-lethal form of skeletal dysplasia, affecting between 1 in 15,000 to 1 in 30,000 live births [Horton et al. 2007; Waller et al. 2008]. Children and adults with ACH have disproportionate short stature, with a final height of approximately 131 cm for males and 124 cm for females. They are prone to significant co-morbidities, including obstructive sleep apnea, chronic otitis media with conductive hearing loss, spinal stenosis and a propensity towards obesity. In some infants, narrowing of the foramen magnum may result in compression

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