Faculty Bibliography

Cervical spinal deformity (CSD) in adult patients is a relatively uncommon yet debilitating condition with diverse etiologies and clinical manifestations. Similar to thoracolumbar deformity, CSD can be broadly divided into scoliosis and kyphosis. Severe forms of CSD can lead to pain; neurologic deterioration, including myelopathy; and cervical spine-specific symptoms such as difficulty with horizontal gaze, dysphagia, and dyspnea. Recently, an increased interest is shown in systematically studying CSD with introduction of classification schemes and treatment algorithms. Both major and minor complications after surgical intervention have been analyzed and juxtaposed to patient-reported outcomes. An ongoing effort exists to better understand the relationship between cervical and thoracolumbar spinal alignment, most importantly in the sagittal plane.

BACKGROUND:Amyloidosis encompasses a group of disorders sharing the common feature of intercellular deposition of amyloid protein by several different pathogenetic mechanisms. Primary solitary amyloidosis, or amyloidoma, is a rare subset of amyloidosis in which amyloid deposition is focal and not secondary to a systemic process or plasma cell dyscrasia. CASE DESCRIPTION/METHODS:This 84-year-old female presented with history of multiple syncopal episodes, dysphagia, and ataxia. Motor strength was 3+/5 in the right upper extremity. Rheumatoid factor, cyclic citrullinated peptide (CCP), and anti-nuclear antibody (ANA) were normal. Serum and urine immune-electrophoresis detected no abnormal bands. Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated a non-enhancing soft-tissue mass extending from the retro-clivus to C2 posteriorly, eccentric to the right with severe mass effect on the upper cervical medullary junction. Endoscopic trans-nasal debulking of the retro-clival mass was performed with occiput to C5 posterior instrumentation for spinal stabilization. CONCLUSIONS:Primary solitary amyloidosis, unlike other forms of amyloidosis, has an excellent prognosis with local resection. Diagnosis requires special stains and a degree of suspicion for the disease. This is the first report to document an endoscopic trans-nasal approach for removal of a primary solitary amyloidosis of the retro-clivus. Management of vertebral amyloidoma involves aggressive local resection of the tumor when feasible and spine stabilization as the degree of tumor involvement mandates. Complete evaluation for the diagnosis of systemic amyloidosis is essential for the management and prognostication. Surgeons encountering such lesions must maintain high suspicion for this rare disease and advise pathologists accordingly to establish the correct diagnosis.

BACKGROUND:Cervical spinal injury encompasses up to 1.5% of all pediatric injuries. Children, and more specifically infants, are a difficult subset of patients to obtain neurological exam in the setting of trauma, thus necessitating the use of cervical X-rays, CT scans, and MRI imaging. CASE DESCRIPTION/METHODS:A healthy, 15-month-old boy had an unwitnessed fall down a flight of stairs and received a CT scan of the head and cervical spine in the emergency department due to cephalohematoma and mechanism of injury. The patient was initially diagnosed with a unilateral facet dislocation but after additional imaging and rigorous interdisciplinary discussions, the patient was correctly diagnosed with a congenitally absent left C5 pedicle. Surgical intervention was not pursued and the patient was discharged home with close follow up. CONCLUSION/CONCLUSIONS:In the acute trauma setting, congenital absent cervical pedicle can be difficult to differentiate from unilateral facet dislocation and may require the use of advanced imaging and close communication between the neurosurgery and radiology departments. Given the high morbidity and mortality involved in the repair of facet dislocation in a child, it is crucial to maintain high degree of clinical suspicion for absent spinal pedicle. In this case, the patient nearly underwent surgical intervention, but was ultimately able to be discharged home with no symptoms or deficits after correct diagnosis.

The endovascular realm has steadily increased its footing in the treatment of the aorta and all of its territories since the foundational case in 1990 by Parodi. The aortic arch, however, continues to be one of the last bastions for treatment via open surgery, which remains the gold standard. Significant comorbidity and prior cardiac surgery prevent open surgery from being the only preferred option, allowing novel endovascular procedures to be considered. Since 1999, more advanced endovascular systems have been created by companies such as Cook Medical, Bolton Medical, Medtronic, Endospan, Gore Medical, and, recently, Kawasumi. The unique shape and angulation of the aortic arch often require the use of custom-made grafts, though arch reconstruction may also include in situ or back-table physician alterations to off-the-shelf devices. The goal of branched endografts is to exclude the aneurysm, while maintaining flow to supra-aortic trunk vessels. Technical success and device durability are limited by the physical constraints of the aortic arch, though greater experience may yield better patient outcomes. Typically, the initial stent-graft (SG) is introduced and deployed into the arch first. Bridging SG are then inserted via axillary or carotid access. Most often, the bridging SG extends from the innominate branch to the distal innominate, and from the left carotid branch to the left common carotid. The major concern is that manipulation of catheters and wires, both within the carotid arteries and aortic arch, create the potential for emboli leading to stroke and paraplegia. The development of endovascular-only techniques for aortic arch pathology will only increase with the aging population of the United States and associated accumulation of comorbidities, making open surgery too grave of a risk.

Aortic aneurysms involving the ascending aorta, aortic arch, and descending thoracic aorta have been a challenging entity to surgically treat for over 60 years. Despite the mortality of the disease, early open surgical procedures also had significant morbidity and mortality. The inherent risk in treating multiple anatomic segments simultaneously led to the innovation of the staged elephant trunk (ET) approach by Borst in 1983. To avoid the thoracotomy and associated complications related to the second stage of the procedure, an endovascular completion paradigm was begun by Volodos in 1991. This theoretical hybrid technique combinined shorter and less elaborate open supra-aortic trunk debranching with less invasive endovascular exclusion and has grown since then in terms of different approaches and case volume. The rise of thoracic endovascular aortic repair (TEVAR) combined with debranching bypass has allowed certain lesions to be treated without a large scale intrathoracic open surgical procedure. The complexity and extensiveness of certain lesions, however, has necessitated a hybrid approach such as the frozen elephant trunk (FET) and the standard ET with second stage TEVAR. The former has been used to treat multifocal degenerative aneurysms, chronic dissections with aneurysm, and acute extensive dissections. After conventional proximal aortic replacement, a stent-graft (SG) is delivered antegrade through the transected arch where it is sutured proximally and then "frozen" distally via endovascular means. The FET has the advantage of avoiding a second stage, but potentially introduces a greater rate of spinal cord ischemia compared to the standard elephant trunk. Improvements on the FET procedure have included the development of more advanced hybrid SG such as the Vascutek® Thoraflex™ Hybrid graft (Vascutek Ltd, Scotland, UK), which consists of a distal en,dograft sealed to a proximal four-branched Vascutek Gelweave™ Vascutek Ltd, Scotland, UK) and incorporated sewing collar. While open surgery continues to be a component of complex aortic arch aneurysms, the development of hybrid devices that can bridge the gap between open and endovascular surgery will continue to flourish.