1/37. klippel-feil syndrome in association with posterior fossa suboccipital dermoid cyst.We present the second case of klippel-feil syndrome in association with a posterior fossa dermoid cyst extending through the occipital bone and presenting as a suboccipital subcutaneous mass. We describe its radiographic, CT, and MRI appearances as well as on MRI diffusion-weighted images. Posterior cranial fossa dermoid cysts and sinuses should be added to the list of congenital abnormalities which must be sought in patients with klippel-feil syndrome. diffusion-weighted images of brain may differentiate these masses from cerebral spinal fluid collections.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/37. Posterior cranial fossa dermoid in association with craniovertebral and cervical spinal anomaly: report of two cases.Two patients, a 12-year-old girl and an 8-year-old boy, with congenital craniovertebral anomaly and klippel-feil syndrome also had a posterior cranial fossa dermoid. The association of these two discrete pathological lesions in the same individual is extremely rare. As both lesions are related to an embryological disorder, issues regarding the possible stage of dysgenesis are analyzed. The treatment options in such cases are discussed.- - - - - - - - - - ranking = 4keywords = spinal (Clic here for more details about this article) |
3/37. klippel-feil syndrome - the risk of cervical spinal cord injury: a case report.BACKGROUND: klippel-feil syndrome is defined as congenital fusion of two or more cervical vertebrae and is believed to result from faulty segmentation along the embryo's developing axis during weeks 3-8 of gestation. persons with klippel-feil syndrome and cervical stenosis may be at increased risk for spinal cord injury after minor trauma as a result of hypermobility of the various cervical segments. persons with klippel-feil syndrome often have congenital anomalies of the urinary tract as well. CASE PRESENTATION: A 51-year male developed incomplete tetraplegia in 1997 when he slipped and fell backwards hitting his head on the floor. x-rays of cervical spine showed fusion at two levels: C2 and C3 vertebrae, and C4 and C5 vertebrae. Intravenous urography (IVU) revealed no kidneys in the renal fossa on both sides, but the presence of crossed, fused renal ectopia in the left ilio-lumbar region. This patient had a similar cervical spinal cord injury about 15 years ago, when he developed transient numbness and paresis of the lower limbs following a fall. DISCUSSION AND CONCLUSION: 1) persons with klippel-feil syndrome should be made aware of the increased risk of sustaining transient neurologic deterioration after minor trauma if there is associated radiographic evidence of spinal stenosis.2) patients with klippel-feil syndrome often have congenital anomalies of the urinary tract. Our patient had crossed, fused, ectopia of kidney.3) When patients with klippel-feil syndrome sustain tetraplegia they have increased chances of developing urinary tract calculi. Treatment of kidney stones may pose a challenge because of associated renal anomalies.4) health professionals caring for cervical spinal cord injury patients with klippel-feil syndrome and renal anomalies should place emphasis on prevention of kidney stones. A large fluid intake is recommended for these patients, as a high intake of fluids is still the most powerful and certainly the most economical means of prevention of nephrolithiasis.- - - - - - - - - - ranking = 8keywords = spinal (Clic here for more details about this article) |
4/37. De novo inv(2)(p12q34) associated with Klippel-Feil anomaly and hypodontia.The present case report describes a patient with Klippel-Feil anomaly (KFA) and oligodontia, carrying a de novo pericentric inversion of chromosome 2 (p12q34). KFA is characterised by congenital vertebral fusion of the cervical spine and a wide spectrum of associated anomalies. It therefore constitutes a heterogenous group of clinical conditions and has been classified morphologically, although its aetiology remains unclear. We present an 18-year-old female with KFA, associated with congenital impairment of hearing, psychomotor retardation, speech limitation, short stature, spinal scoliosis, facial asymmetry and latent hypothyroidism. No renal anomaly or heart disease was present. In addition, she exhibited oligodontia of both the deciduous and permanent dentition, a unique characteristic that has not yet been reported in any non-cleft palate KFA case. CONCLUSION: The current report of a patient with oligodontia and an inversion on chromosome 2 may aid in the identification of novel genes for oligodontia.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
5/37. A case of basilar impression complicated with left frontal meningioma.Case report: A 75-year-old baikarian woman was admitted to our hospital for treatment of seizures. From the results of neurological and radiological examination, a left frontal meningioma was suspected and the patient was referred to our department for neurosurgical intervention. At admission, the MRI showed a basilar impression accompanied by klippel-feil syndrome of C4/5/6/7, but neurological symptoms of basilar impression were absent. Subsequently, the tumor was resected via the left frontal approach using microsurgical technique. Histological examination disclosed fibroblastic meningioma. DISCUSSION: The coincidence of basilar impression with a brain tumor is a relatively rare occurrence. There are a few reports about craniovertebral junction anomaly including basilar impression associated with spinal or cerebral tumor. This time, we present an interesting combined case of BI and klippel-feil syndrome associated with left frontal meningioma.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
6/37. Removal of an anterior spinal dermoid cyst with fenestra corpectomy in klippel-feil syndrome: technical case report.OBJECTIVE AND IMPORTANCE: A spinal cord tumor occurring in association with klippel-feil syndrome is quite rare. The removal of an anteriorly located spinal cord tumor at the level of block vertebrae creates a surgical challenge. CLINICAL PRESENTATION: A case of an intradural extramedullary dermoid cyst located anterior to the spinal cord and a syringomyelic cavity at the level of block vertebrae in a 43-year-old woman with klippel-feil syndrome is presented. She experienced pain and numbness in both shoulders and in her neck, and she had a slight weakness in both arms before the operation. Her weakness and the clinical symptoms completely disappeared after the operation, and the resolution of the syringomyelic cavity was observed at control magnetic resonance imaging. INTERVENTION: An anterior approach creating a fenestra corpectomy to the block vertebrae was performed, and the tumor was removed totally. No fusion or fixation was performed. CONCLUSION: To our knowledge, this is the first report of an anteriorly located intradural extramedullary cervical spine tumor in association with klippel-feil syndrome treated with this surgical technique. A three-dimensional computed tomographic control scan obtained 1 year after the operation did not show any instability.- - - - - - - - - - ranking = 7keywords = spinal (Clic here for more details about this article) |
7/37. Lumbar split cord malformation and klippel-feil syndrome.The authors report a patient with type II split cord malformation (SCM) and klippel-feil syndrome (KFS). Considering our review of the medical literature and present case report, we believe lumbar SCM and KFS in the same patient not to be coincidental findings but rather embryologically related entities. We propose that KFS in at least one of its phenotypes represents a forme fruste of occult spinal dysraphism.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
8/37. klippel-feil syndrome associated with persistent trigeminal artery.STUDY DESIGN: In the present report, radiologic findings of the patient with the klippel-feil syndrome associated with persistent trigeminal artery are reviewed. In same case, there was also spina bifida occulta, cervical spondylosis, atlanto-occipital assimilation, bilateral cervical ribs, and lack of flow signal in the C4 segment of contralateral vertebral artery. The persistent trigeminal artery and klippel-feil syndrome can be seen together and with the other anomalies. It should be considered that they might be related with the insufficiency or dysfunction of the embryologic developmental process. OBJECTIVES: To draw attention to this rare presentation of klippel-feil syndrome associated with persistent trigeminal artery. SUMMARY OF BACKGROUND DATA: klippel-feil syndrome is a congenital spinal malformation characterized by the failure in segmentation of 2 or more cervical vertebrae. Although the anomaly is defined by its skeletal component, klippel-feil syndrome may also be associated with developmental defects in many other organ systems including the inner ear, spinal cord, heart, and genitourinary tract. The primitive trigeminal, otic, hypoglossal, and proatlantal intersegmental arteries are persistent fetal anastomoses between the carotid and vertebrobasilar circulations. The authors report the association of a persistent trigeminal artery with the klippel-feil syndrome. methods: Radiologic findings of a case of klippel-feil syndrome associated with persistent trigeminal artery are described. RESULTS: There was vertebral fusion, spina bifida occulta, bilateral cervical ribs, atlanto-occipital assimilation, and cervical spondylosis. magnetic resonance angiography showed the persistent trigeminal artery between the left internal carotid and basilar artery, but there was no sign of flow in the C4 segment of right vertebral artery. CONCLUSIONS: It should be kept in mind that a persistent trigeminal artery and klippel-feil syndrome can be seen together and with the other anomalies. Because of this reason, this type of case should prompt a search for the other related anomalies, and magnetic resonance angiography can be used as a noninvasive diagnostic technique in the persistent trigeminal artery determination.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
9/37. Isolated atlantal stenosis in a patient with idiopathic growth hormone deficiency, and Klippel-Feil and Duane's syndromes.OBJECTIVE AND IMPORTANCE: Isolated atlantal stenosis is very rare with less than ten cases reported, mostly in adult Asians; however, this pathology should be recognized by the neurosurgeon. An unusual case of this rare clinical entity is reported in conjunction with multiple other anomalies. CLINICAL PRESENTATION: We report a Caucasian girl with symptomatic stenosis of the first cervical vertebrae who presented with episodes of loss of tone with subsequent falling, facial cyanosis, urinary incontinence, hand weakness, and difficulties with swallowing. This patient also had the diagnoses of Duane's and Klippel-Feil syndromes, and idiopathic growth hormone deficiency. In addition, this patient was found to have retroflexion of the odontoid process, which further compromised the spinal canal. Flexion-extension radiographs failed to identify cervical spine instability. INTERVENTION: Following suboccipital craniectomy and the removal of the posterior arch of the atlas, the patient's symptoms were resolved and her urinary incontinence improved. CONCLUSIONS: We theorize that our case represents a variant of Wildervanck syndrome. Further case reports are necessary to verify that isolated atlantal stenosis is a component of this constellation of findings. Furthermore, our case demonstrates that degenerative changes of the spine are not necessary for the production of symptoms in cases of isolated stenosis of the atlas.- - - - - - - - - - ranking = 4.4972963859481keywords = spinal canal, spinal, canal (Clic here for more details about this article) |
10/37. Spinal neurenteric cyst in association with syringomyelia: case report.A case of spinal neurenteric cyst in association with Klippel-Feil deformity, spinal lipoma, and syringomyelia is presented. Neuroradiological evaluation was performed by means of magnetic resonance imaging of the spine and a computed tomography myelograph. The diagnosis of neurenteric cyst was established by histological and immunohistochemical studies. The pathogenesis of syringomyelia associated with an extramedullary mass lesion is discussed.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
| | Next -> |