Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
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The surgical management of craniovertebral junction instability in pediatric patients has unique challenges. While the indications for internal fixation in children are similar to those of adults, the data concerning techniques, complications, and outcomes of spinal instrumentation comes from experience with adult patients. Diminutive osseous and ligamentous structures and anatomical variations associated with syndromic craniovertebral abnormalities frequently complicates the approaches and limits the use of internal fixation in children. Cervical arthrodesis in the pediatric age group has the potential for limiting growth potential and causing secondary deformity. Recent advances in image analysis have enabled preoperative planning which is critical to evaluate the size of instrumentation and its relation to the patient's anatomy. Newer techniques have recently evolved and have been incorporated in the management of pediatric patients with requirement for craniocervical stabilization. ⋯ Abnormal cervical spine growth was not seen in children who underwent craniocervical stabilization below the age of 5. The authors have reserved rigid instrumentation for children above the age of 10 years and dependent on the anatomy.
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The incidence of tumors at the craniovertebral junction in the pediatric population is low. Because of the variable pathology and the rarity of these tumors, ideal therapies are only now being defined. ⋯ Chordomas of the clivus and foramen magnum were seen in eight, fibrous dysplasia in four, aneurysmal bone cysts in four, eosinophilic granuloma affecting the atlas and axis vertebra in four, Ewing's sarcoma involving the atlas in two, osteoblastoma in two, neurenteric cysts in four, meningioma in five, schwannoma in two, and plexiform neurofibromas in three. The location of these tumors was predominantly ventral, and a very small number had a lateral or dorsal location. The ventral tumors included chordoma, meningioma, fibrous dysplasia, aneurysmal bone cyst, and osteoblastoma. Plexiform neurofibroma affecting the craniocervical junction was ventral to the clivus and upper cervical spine causing severe kyphosis of the craniocervical region. Pain in the head and neck occurred in 70%. Paresthesias and dysesthesias in the hands were seen in 40% and spastic weakness of extremities in 22%. Cranial nerve palsies were seen in 33%. Twenty-eight percent of children showed dysphagia or dysarthria. The cranial nerves affected were the vagus followed by hypoglossal and glossopharyngeal nerves. This led to dysphagia, slurred speech, repeated aspiration pneumonia, and weight loss. The most common findings for chordomas at the craniocervical junction were isolated hypoglossal nerve palsy. All individuals underwent magnetic resonance imaging, computed tomography, and 3D computed tomography and angiography. Vertebral angiography was used to understand the dynamics of collateral circulation and tumor vascularity. Tumor embolization was performed in chordoma and aneurysmal bone cysts. Our experience and results are presented here.
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Lesions that affect the lower clivus, foramen magnum, the craniocervical junction, and the upper cervical spinal canal that are anterolateral and at times intradural require access ventral to the cerebellum and spinal cord. The posterolateral transcondylar approach provides such a route. In addition, posterior craniocervical stabilization can be accomplished at the same time. The author has reviewed the technique as well as the surgical results here. ⋯ The posterolateral transcondylar route is a versatile avenue to approach a variety of lesions ventrolateral to the brain stem and upper cervical cord. Exposure is quite satisfactory with minimal or no retraction of important neurovascular structures in the region. Modifications of this theme can be applied as the lesions require.
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Congenital and developmental osseous abnormalities and anomalies that affect the craniocervical junction complex can result in neural compression and vascular compromise and can manifest itself with abnormal cerebrospinal fluid dynamics. An understanding of the development of the craniocervical junction is essential to recognize the pathological abnormalities. ⋯ The conclusion was that os odontoideum was associated with an unrecognized fracture in children below the age of 5 with a previously normal odontoid structure as observed in our series. Atlas and axis abnormalities were reviewed in this series. This large database has provided an understanding of the natural history of many entities and allowed treatment protocols to be established that have stood the test of time.