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At least 19 recordsLinked to original sources
PubMed2005

Spontaneous regression of syringomyelia in Hajdu-Cheney syndrome with severe platybasia. Case report.

Hadju-Cheney syndrome (HCS) is a rare autosomal-dominant disorder with variable expressivity. It is characterized by facial dysmorphism, premature tooth loss, osteolysis of distal phalanges, and skull abnormalities. In some cases, progressive platybasia can occur and can lead to Chiari malformation with an obstruction of cerebrospinal fluid flow. To the best of the authors' knowledge, only five cases of HCS-associated syringomyelia have been reported in the literature. Because of the rarity of this association, little is known about its natural history. The authors present the case of a 16-year-old boy affected by HCS. On initial magnetic resonance (MR) imaging, a severe basilar invagination with Chiari malformation and cervicothoracic syringomyelia was documented. The syringomyelia had no clinical manifestations. A repeated MR image demonstrated a spontaneous resolution of the syrinx with no changes in the tonsil or the platybasia. The regression of the syringomyelia was confirmed by a control MR imaging examination performed after a 2-year period. No changes in the patient's clinical conditions were found during the follow-up period. This is the first case of spontaneous regression of the syringomyelia despite a severe platybasia in HCS. It did not appear correlated to a modification of the tonsil's structure or position. This observation illustrates one possible evolution of syringomyelia in the natural history of HCS and raises the question of the potential mechanisms involved in the spontaneous drainage of the syringomyelic cavity.

Adolescent↗
PubMed2005

Evaluation of platybasia with MR imaging.

BACKGROUND AND PURPOSE: Platybasia, or abnormal obtuseness of the basal angle, was first measured on plain skull images. At present, evaluation of the brain and skull more commonly involves CT and MR imaging. We evaluated a new MR imaging method of evaluating platybasia. METHODS: We retrospectively evaluated midline sagittal MR images in 200 adults and 50 children. The basal angle of the skull base was measured by using two methods: The standard MR imaging technique measured the angle formed by two lines-one joining the nasion and the center of the pituitary fossa connected by a line joining the anterior border of the foramen magnum and center of the pituitary fossa. The modified technique measured the angle formed by a line across the anterior cranial fossa and dorsum sellae connecting a line along the clivus. RESULTS: With the standard MR imaging technique, we obtained mean angles of 129 degrees +/- 6 degrees for adults and 127 degrees +/- 5 degrees for children, compared with 135.3 degrees (composite mean) in previous series. The modified technique produced values of 117 degrees +/- 6 degrees for adults and 114 degrees +/- 5 degrees for children, which were significantly lower that those of standard MR imaging and traditional radiography (P <.05). CONCLUSION: Both the standard and modified MR imaging techniques produced basal angles lower than those previously reported with standard radiography. The modified technique uses clearly featured landmarks that can be reproduced consistently on midline sagittal T1 images. This technique and its corresponding values can be used as the new standard for evaluating the basal angle.

Adult↗
PubMed1982

Basilar impression and platybasia in osteogenesis imperfecta tarda.

Osteogenesis imperfecta, a rare, genetically transmitted disorder of bone, is known to be associated with the development of basilar impression and platybasia. These deformities of the base of the skull may cause neurosurgical abnormalities secondary to compression of the brainstem and hydrocephalus. The case is presented of a young boy with a family history of osteogenesis imperfecta tarda who suffered respiratory arrest during hospitalization. Cranial nerves and pyramidal tract signs were demonstrated. Roentgenograms showed severe basilar impression and hydrocephalus. Decompression of the brainstem and shunting were performed with improvement in the patient's neurological status. This case represents a rare by significant central nervous system complication of osteogenesis imperfecta. Early recognition and implementation of aggressive treatment are important if irreversible neurological deficits are to be avoided.

Adolescent↗
PubMed1996

[Platybasia, basilar groove, odontoid process and kinking of the brainstem: a common etiology with idiopathic syringomyelia, scoliosis and Chiari malformations].

INTRODUCTION: A common aetiopathogenic theory for basilar groove (IMB), platybasia (PTB), odontoid retrocession (RTO), kinking of the brainstem (KTC) applied to idiopathic syringomyelia (SMI), idiopathic scoliosis (ESCID) and Arnold-Chiari malformation (ARCH) is presented. Confirmation is based on an abnormally low position of the conus medullaris (CMB) in the patients with SMI. MATERIALS AND METHOD: 292 patients with syringomyelia (SM), 231 with SMI were selected. Of these, 55 were chosen who had SMI and in whom the level of the conus medullaris (NCM) could be determined, together with the figures for SMI, IMB, PTB, RTO, KTC, ESCID and ARCH on cervical and lumbar MR. The position of the conus medullaris in 50 patients who did not have SM, ESCID nor ARCH on cervical and lumbar RM was determined. RESULTS: 32 patients had an increased basal angle (58.18%). There was an IMB in a quarter of the patients (25.45%). RTO was observed in half of the patients (47.27%). Just over one third presented a KTC. 6% of the control group had CM at the level of the body of L1, whilst 84.21% of the patients with SMI presented a partial or complete CM image at this level. CONCLUSIONS: CMB in SMIU and its close relationship with IMB, PTB, RTO AND KTC and also with ESCID and ARCH make it likely that they share the same aetiopathogenic mechanism: an abnormal lack of synchronization of the growth of the neuro-axis and the neural canal (AACNN), causing a specific disorder which is seen as different syndromes.

Adult↗
PubMed1997

[A new surgical treatment for syringomyelia, scoliosis, Arnold-Chiari malformation, kinking of the brainstem, odontoid recess, idiopathic basilar impression and platybasia].

INTRODUCTION: Based on medullary traction as responsible for idiopathic syringomyelia (SMI), idiopathic scoliosis (ESCID), Arnold Chiari malformation (ARCH), platybasia (PTB), basilar impression (IMB), odontoid recess (RTO) kinking of the brain stem (KTC) and considering the medullary traction to be transmitted by the filum terminale (FT), a surgical technique for the section of FT (SFT) is described in three cases of SMI, one of ESCID, and one of ARCH with no lumbar dysraphia. MATERIAL AND METHODS: A 34-year-old woman with cervico-brachialgias, paresthesias, bilateral babinski and a centro-medullary cavity C3-C7. A 26-year-old male with cervico-brachialgias, hypoestesia in left hemybody, and cervicobulbar cavity. A 19-year-old female with ESCID since the age of 14th, with episodes of reacuting, and 38o of dorsolumbar curvature. A 67-year-old woman with intense headache, hypoesthesia of the hands, paraparesia and ARCH. A 23-year-old man with marked tetraparesia, bilateral babinski, anesthesia of both legs, SMI, ESCID, ARCH and hydrocephaly. RESULTS: After SFT: in the SMIs the thermo-algesic, disesthetic and algic dissociation disappeared. In ESCID there was a reduction to 31o in the curvature in nine months. On ARCH the headaches ceased and there was recovery of touch and paraparesia. CONCLUSIONS: SFT is a useful etiological treatment for SMI, ESCID, ARCH. Also, in ESCID it is possible to avoid stress on the medulla due to its surgical reduction.

Adult↗