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Archives of Plastic Surgery Jul 2014An abnormally obtuse cranial base angle, also known as platybasia, is a common finding in patients with 22q11.2 deletion syndrome (22q11DS). Platybasia increases the...
BACKGROUND
An abnormally obtuse cranial base angle, also known as platybasia, is a common finding in patients with 22q11.2 deletion syndrome (22q11DS). Platybasia increases the depth of the velopharynx and is therefore postulated to contribute to velopharyngeal dysfunction. Our objective was to determine the clinical significance of platybasia in 22q11DS by exploring the relationship between cranial base angles and speech resonance.
METHODS
In this retrospective chart review at a tertiary hospital, 24 children (age, 4.0-13.1 years) with 22q11.2DS underwent speech assessments and lateral cephalograms, which allowed for the measurement of the cranial base angles.
RESULTS
One patient (4%) had hyponasal resonance, 8 (33%) had normal resonance, 10 (42%) had hypernasal resonance on vowels only, and 5 (21%) had hypernasal resonance on both vowels and consonants. The mean cranial base angle was 136.5° (standard deviation, 5.3°; range, 122.3-144.8°). The Kruskal-Wallis test showed no significant relationship between the resonance ratings and cranial base angles (P=0.242). Cranial base angles and speech ratings were not correlated (Spearman correlation=0.321, P=0.126). The group with hypernasal resonance had a significantly more obtuse mean cranial base angle (138° vs. 134°, P=0.049) but did not have a greater prevalence of platybasia (73% vs. 56%, P=0.412).
CONCLUSIONS
In this retrospective chart review of patients with 22q11DS, cranial base angles were not correlated with speech resonance. The clinical significance of platybasia remains unknown.
PubMed: 25075355
DOI: 10.5999/aps.2014.41.4.344 -
Arquivos de Neuro-psiquiatria Sep 2013The prime objective in the surgical treatment of basilar impression (BI), Chiari malformation (CM), and/or syringomyelia (SM) is based on restoration of the normal...
The prime objective in the surgical treatment of basilar impression (BI), Chiari malformation (CM), and/or syringomyelia (SM) is based on restoration of the normal cerebrospinal fluid (CSF) dynamics at the craniovertebral junction and creation of a large artificial cisterna magna, avoiding the caudal migration of the hindbrain. It is observed that a large craniectomy might facilitate an upward migration of the posterior fossa structures. There are many surgical techniques to decompress the posterior fossa; however, a gold standard approach remains unclear. The authors present the results of 192 cases of BI, CM, and SM treated between 1975 and 2008 and whose surgical treatment was characterized by a large craniectomy without tonsillectomy with the patient in the sitting position, large opening of the fourth ventricle, and duraplasty.
Topics: Adolescent; Adult; Aged; Arnold-Chiari Malformation; Child; Child, Preschool; Craniotomy; Dura Mater; Female; Fourth Ventricle; Humans; Male; Middle Aged; Platybasia; Retrospective Studies; Syringomyelia; Treatment Outcome; Young Adult
PubMed: 24141441
DOI: 10.1590/0004-282X20130105 -
Neurology India 2013
Topics: Cerebral Ventricle Neoplasms; Dermoid Cyst; Fourth Ventricle; Humans; Klippel-Feil Syndrome; Male; Platybasia; Young Adult
PubMed: 23644332
DOI: 10.4103/0028-3886.111153 -
Neurosurgical Review Oct 2013The craniometric linear dimensions of the posterior fossa have been relatively well studied, but angular craniometry has been poorly studied and may reveal differences...
The craniometric linear dimensions of the posterior fossa have been relatively well studied, but angular craniometry has been poorly studied and may reveal differences in the several types of craniocervical junction malformation. The objectives of this study were to evaluate craniometric angles compared with normal subjects and elucidate the main angular differences among the types of craniocervical junction malformation and the correlation between craniocervical and cervical angles. Angular craniometries were studied using primary cranial angles (basal and Boogard's) and secondary craniocervical angles (clivus canal and cervical spine lordosis). Patients with basilar invagination had significantly wider basal angles, sharper clivus canal angles, larger Boogard's angles, and greater cervical lordosis than the Chiari malformation and control groups. The Chiari malformation group does not show significant differences when compared with normal controls. Platybasia occurred only in basilar invagination and is suggested to be more prevalent in type II than in type I. Platybasic patients have a more acute clivus canal angle and show greater cervical lordosis than non-platybasics. The Chiari group does not show significant differences when compared with the control, but the basilar invagination groups had craniometric variables significantly different from normal controls. Hyperlordosis observed in the basilar inavagination group was associated with craniocervical kyphosis conditioned by acute clivus canal angles.
Topics: Adult; Arnold-Chiari Malformation; Cephalometry; Cervical Vertebrae; Cranial Fossa, Posterior; Data Interpretation, Statistical; Female; Humans; Image Processing, Computer-Assisted; Lordosis; Magnetic Resonance Imaging; Male; Middle Aged; Platybasia; Radiography; Spine
PubMed: 23640096
DOI: 10.1007/s10143-013-0471-0 -
European Spine Journal : Official... May 2013Transoral resection of the odontoid has been accepted as a standard procedure to decompress the cervicomedullary junction during the past several decades. The endoscopic...
PURPOSE
Transoral resection of the odontoid has been accepted as a standard procedure to decompress the cervicomedullary junction during the past several decades. The endoscopic transnasal odontoidectomy is emerging as a feasible surgical alternative to conventional microscopic transoral approach. In this article, we describe several operative nuances and pearls from our experience about this approach, which provided successful decompression.
METHODS
From September 2009 to April 2010, three consecutive patients with basilar invagination, of which the etiology was congenital osseous malformations, underwent endoscopic transnasal odontoidectomy. All patients presented with myelopathy. The last two cases also received occipitocervical fixation and bone fusion during the same surgical episode to ensure stability.
RESULTS
All the patients were extubated after recovery from anesthesia and allowed oral food intake the next day. Cerebrospinal fluid rhinorrhea was found in the second case and cured by continuous lumber drainage of cerebrospinal fluid. No infection was noted. The average follow-up time was more than 24 months. Remarkable neurological recovery was observed postoperative in all patients.
CONCLUSION
The endoscopic transnasal odontoidectomy is a feasible approach for anterior decompression of pathology at the cervicomedullary junction. The advantages over the standard transoral odontoidectomy include elimination of risk of tongue swelling and teeth damaging, improvement of visualization, alleviation of prolonged intubation, reduction of need for enteral tube feeding and less risk of affecting phonation. The minimally invasive access and faster recovery associated with this technique make it a valid alternative for decompression of the ventral side of the cervicomedullary junction.
Topics: Adult; Decompression, Surgical; Female; Humans; Male; Middle Aged; Natural Orifice Endoscopic Surgery; Odontoid Process; Platybasia; Radiography; Treatment Outcome
PubMed: 23224062
DOI: 10.1007/s00586-012-2605-4 -
European Spine Journal : Official... May 2013Klippel-Feil syndrome (KFS) is a congenital cervical vertebral union caused by a failure of segmentation during abnormal development and frequently accompanies...
INTRODUCTION
Klippel-Feil syndrome (KFS) is a congenital cervical vertebral union caused by a failure of segmentation during abnormal development and frequently accompanies conditions such as basicranial malformation, atlas assimilation, or dens malformation. Especially in basilar invagination (BI), which is a dislocation of the dens in an upper direction, compression of the spinomedullary junction from the ventral side results in paralysis, and treatment is required.
CLINICAL PRESENTATION
We present the case of a 38-year-old male patient with KFS and severe BI. Plane radiographs and computed tomography (CT) images showed severe BI, and magnetic resonance image (MRI) revealed spinal cord compression caused by invagination of the dens into the foramen magnum and atlantoaxial subluxation. Reduction by halo vest and skeletal traction were not successful. Occipitocervical fusion along with decompression of the foramen magnum, C1 laminectomy, and reduction using instruments were performed. Paralysis was temporarily aggravated and then gradually improved. Unsupported walking was achieved 24 months after surgery, and activities of daily life could be independently performed at the same time. CT and MRI revealed that dramatic reduction of vertical subluxation and spinal cord decompression were achieved.
CONCLUSION
Reduction and internal fixation using instrumentation are effective techniques for KFS with BI; however, caution should be exercised because of the possibility of paralysis caused by intraoperative reduction.
Topics: Adult; Decompression, Surgical; Humans; Klippel-Feil Syndrome; Male; Odontoid Process; Orthopedic Procedures; Platybasia; Spinal Cord Compression
PubMed: 22926486
DOI: 10.1007/s00586-012-2489-3 -
BMJ Case Reports Jun 2012A teenage girl presented with progressively worsening neck and occipital pain since 8 months ago that was associated with restriction of neck movements, low to moderate...
A teenage girl presented with progressively worsening neck and occipital pain since 8 months ago that was associated with restriction of neck movements, low to moderate grade fever, anorexia and weight loss followed by gradually progressive quadriparesis since 3 months ago. Neurological examination revealed spastic quadriparesis without cranial nerve palsy. MRI of the cervical spine revealed prevertebral and paravertebral abscess from clivus to C2/C3 level, which extended into the anterior epidural space at C1-C3 level, causing atlantoaxial dislocation. There was evidence of basilar invagination. The dislocated dens caused compression over the cervicomedullary junction. On this basis, a diagnosis of craniovertebral junction Pott's disease was made. The patient was given neck immobilisation with cervical collar, and antitubercular treatment was started. She showed significant improvement in her neurological deficit during follow-up.
Topics: Adolescent; Atlanto-Occipital Joint; Cervical Vertebrae; Diagnosis, Differential; Female; Humans; Immobilization; Magnetic Resonance Imaging; Platybasia; Spinal Cord Compression
PubMed: 22669923
DOI: 10.1136/bcr.02.2012.5851 -
Journal of Bone and Mineral Research :... May 2012Pathology in the craniocervical junction is a serious complication of osteogenesis imperfecta (OI). Our aim was to analyze the prevalence and natural course of...
Pathology in the craniocervical junction is a serious complication of osteogenesis imperfecta (OI). Our aim was to analyze the prevalence and natural course of craniocervical junction anomalies in patients with OI during growth. In a one-center retrospective study, we analyzed lateral skull radiographs and midsagittal magnetic resonance images of 76 patients with either type I, III, or IV OI. The material included longitudinal series of 31 patients. In total, 150 patient images taken at ages 0 to 39 years were analyzed and compared with age-matched control data. Craniocervical anomalies were observed in 37% of patients and in all OI types studied. Of the three types of anomalies, basilar invagination was seen in 13%, basilar impression in 15%, and platybasia in 29% of the patients. From those with an abnormal finding, 44% displayed more than one type of anomaly. At a group level, we found no evidence of progression of craniocervical junction pathology with age. We provide longitudinal and cross-sectional data on craniocervical junction dimensions in growing patients with OI and, based on those, suggest a radiological management strategy for diagnosis of cranial base pathology. A higher risk of having any of the pathological conditions was associated with a lower height Z-score. Careful follow-up of cranial base anomalies particularly in subjects with OI and severe growth failure is warranted.
Topics: Adolescent; Adult; Case-Control Studies; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Longitudinal Studies; Male; Osteogenesis Imperfecta; Retrospective Studies; Skull Base
PubMed: 22258757
DOI: 10.1002/jbmr.1555 -
The Journal of Bone and Joint Surgery.... Nov 2011We present the case of a 15-year-old boy with symptoms due to Klippel-Feil syndrome. Radiographs and CT scans demonstrated basilar impression, occipitalisation of C1 and...
We present the case of a 15-year-old boy with symptoms due to Klippel-Feil syndrome. Radiographs and CT scans demonstrated basilar impression, occipitalisation of C1 and fusion of C2/C3. MRI showed ventral compression of the medullocervical junction. Skull traction was undertaken pre-operatively to determine whether the basilar impression could be safely reduced. During traction, the C3/C4 junction migrated 12 mm caudally and spasticity resolved. Peri-operative skull-femoral traction enabled posterior occipitocervical fixation without decompression. Following surgery, cervical alignment was restored and spasticity remained absent. One year after surgery he was not limited in his activities. The surgical strategy for patients with basilar impression and congenital anomalies remains controversial. The anterior approach with decompression is often recommended for patients with ventral compression of the medullocervical region, but such procedures are technically demanding and carry a significant risk of complications. Our surgical strategy was an alternative solution. Prior to a posterior cervical fixation, without decompression, skull traction was used to confirm that the deformity was reducible and effective in resolving associated myelopathy.
Topics: Adolescent; Cervical Vertebrae; Femur; Humans; Klippel-Feil Syndrome; Magnetic Resonance Imaging; Male; Medulla Oblongata; Occipital Bone; Platybasia; Spinal Cord Compression; Tomography, X-Ray Computed; Traction
PubMed: 22058314
DOI: 10.1302/0301-620X.93B11.26892 -
Arquivos de Neuro-psiquiatria Oct 2011The prime objective in the surgical treatment of basilar impression (BI), Chiari malformation (CM) and/or syringomyelia (SM) is based on the restoration of the normal...
The prime objective in the surgical treatment of basilar impression (BI), Chiari malformation (CM) and/or syringomyelia (SM) is based on the restoration of the normal cerebrospinal fluid (CSF) dynamics at the craniovertebral junction through the creation of a large artificial cisterna magna. A small suboccipital craniectomy has been emphasized to avoid caudal migration of the hindbrain structures into the vertebral canal. Nevertheless, the results showed downward migration of the hindbrain related to that type of craniectomy. The authors present, otherwise, the results of 104 cases of BI, CM and/or SM, whose surgical treatment was characterized by a large craniectomy with the patient in the sitting position, tonsillectomy, large opening of the fourth ventricle and duraplasty with creation of a large artificial cisterna magna. A significant upward migration of the posterior fossa structures was detected by postoperative magnetic resonance imaging.
Topics: Adolescent; Adult; Aged; Arnold-Chiari Malformation; Craniotomy; Decompression, Surgical; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Platybasia; Retrospective Studies; Syringomyelia; Tonsillectomy; Treatment Outcome; Young Adult
PubMed: 22042188
DOI: 10.1590/s0004-282x2011000600018