Charcot spinal disease is a destructive degenerative process involving the vertebrae and surrounding discs, resulting from repetitive microtrauma in patients who have decreased joint protective mechanisms due to loss of deep pain and proprioceptive sensation. The typical presentation of the disease is back pain and progressive spinal instability and deformity. The authors report an unusual case of massive Charcot spinal disease deformity in a patient presenting with increasing abdominal girth and discomfort.
Frank S. Bishop, Andrew T. Dailey and Meic H. Schmidt
Case report and review of the literature
Frank S. Bishop, James K. Liu, Todd D. McCall and Douglas L. Brockmeyer
✓Glutaric aciduria type 1 (GA1) is a rare neurometabolic disorder with characteristic neuroimaging and clinicopathological features. The authors describe a case of GA1 in a 7-month-old girl presenting with macrocephaly and bilateral subdural hematomas (SDHs) who was initially evaluated for nonaccidental trauma (NAT). Bilateral subdural drains were placed because of significant mass effect from the chronic SDHs, with subsequent neurological and neuroimaging-documented improvement. Clinical and neuroimaging findings led to further laboratory investigation to confirm the diagnosis of GA1, after which a specialized low-protein diet was initiated. After a thorough investigation, NAT was ruled out. At the follow-up examination, the patient experienced improvement in her symptoms and resolution of the bilateral subdural collections. The presence of bilateral SDHs in an infant raises the suspicion of NAT and presents a difficult diagnostic challenge because of the legal and social implications. Glutaric aciduria type 1 should be considered in the differential diagnosis of bilateral SDHs, and an evaluation should be performed. The authors review the clinical manifestations, diagnosis, medical and surgical management, and specific considerations regarding GA1, including misdiagnosis of NAT.
Frank S. Bishop, Michael A. Finn, Mical Samuelson and Richard H. Schmidt
In severe cases, posttraumatic cerebral sinus thrombosis can result in venous congestion and persistent intracranial hypertension refractory to both conventional medical therapy and surgical decompression. The authors report a unique case of a patient successfully treated with endovascular mechanical thrombolysis using balloon angioplasty for clinically significant posttraumatic venous sinus thrombosis and review the reported treatments for cerebral venous sinus occlusive disease.
This 18-year-old man suffered severe closed head injury from a fall while skateboarding. A head CT scan demonstrated basilar skull fractures involving the left jugular foramen. A CT angiogram revealed thrombosis of the left transverse sinus and occlusion of the sigmoid sinus and internal jugular vein. Despite treatment with anticoagulation therapy and decompressive hemi- and suboccipital craniectomies, intracranial hypertension remained refractory. Serial angiography demonstrated progressive sinus occlusion. Endovascular balloon thrombolysis of the left transverse and sigmoid sinuses resulted in immediate reduction of intracranial pressures and improved sinus patency. Intracranial pressure measurements remained low after the procedure. The patient eventually improved neurologically, was able to follow commands and walk, and was discharged to a rehabilitation facility for further recovery.
Anticoagulation therapy, surgical decompression, and endovascular thrombolysis have been reported as treatment modalities for clinically significant posttraumatic venous sinus thrombosis. In this case, endovascular mechanical thrombolysis with balloon angioplasty resulted in resolution of thrombus and successful immediate reduction of intracranial pressure. This treatment may be considered in patients with critically elevated intracranial pressure from posttraumatic venous sinus occlusion refractory to other treatment measures.
Sarah T. Garber, Jay Riva-Cambrin, Frank S. Bishop and Douglas L. Brockmeyer
Fourth ventricle hydrocephalus, or a “trapped” fourth ventricle, presents a treatment challenge in pediatric neurosurgery. Fourth ventricle hydrocephalus develops most commonly as a result of congenital anomalies, intraventricular hemorrhage, or infection. Standard management of loculated fourth ventricle hydrocephalus consists of fourth ventricle shunt placement via a suboccipital approach. An alternative approach is stereotactic-guided transtentorial fourth ventricle shunt placement via the nondominant superior parietal lobule. In this report, the authors compare shunt survival after placement via the suboccipital and stereotactic parietal transtentorial (SPT) approaches.
A retrospective chart review was performed to find all patients with a fourth ventricle shunt placed between January 1, 1998, and December 31, 2011. Time to shunt failure was quantified as the number of days from shunt placement to first shunt revision or removal. Other variables studied included patient age and sex, origin of hydrocephalus, comorbidities, number of existing supratentorial catheters at the time of fourth ventricle shunt placement (as a proxy for complexity), operating surgeon, and number of previous shunt revisions. The crossover rate from one technique to the other after shunt failure from the original approach was also investigated.
In the 29 fourth ventricle shunts placed during the study period, 18 were placed via the suboccipital approach (62.1%) and 11 via the SPT approach (37.9%). There was a statistically significant difference in time to shunt failure, with the SPT shunts lasting an average of 901 days and suboccipital shunts lasting 122 days (p = 0.04). In addition, there was a significant difference in the rate of crossover from one technique to another, with 1 SPT shunt changed to a suboccipital shunt (5.6%) and 5 suboccipital shunts changed to SPT shunts (45.5%).
Fourth ventricle shunt placement using an SPT approach resulted in significantly longer shunt survival times and lower rates of revision than the traditional suboccipital approach, despite a higher rate of crossover from previously failed shunting procedures. Stereotactic parietal transtentorial shunt placement may be considered for patients with loculated fourth ventricle hydrocephalus, especially when shunt placement via the standard suboccipital approach fails. It is therefore reasonable to offer this procedure either as a first option for the treatment of fourth ventricle hydrocephalus or when the need for fourth ventricle shunt revision arises.
Frank S. Bishop, Mical M. Samuelson, Michael A. Finn, Kent N. Bachus, Darrel S. Brodke and Meic H. Schmidt
Thoracolumbar corpectomy is a procedure commonly required for the treatment of various pathologies involving the vertebral body. Although the biomechanical stability of anterior reconstruction with plating has been studied, the biomechanical contribution of posterior instrumentation to anterior constructs remains unknown. The purpose of this study was to evaluate biomechanical stability after anterior thoracolumbar corpectomy and reconstruction with varying posterior constructs by measuring bending stiffness for the axes of flexion/extension, lateral bending, and axial rotation.
Seven fresh human cadaveric thoracolumbar spine specimens were tested intact and after L-1 corpectomy and strut grafting with 4 different fixation techniques: anterior plating with bilateral, ipsilateral, contralateral, or no posterior pedicle screw fixation. Bending stiffness was measured under pure moments of ± 5 Nm in flexion/extension, lateral bending, and axial rotation, while maintaining an axial preload of 100 N with a follower load. Results for each configuration were normalized to the intact condition and were compared using ANOVA.
Spinal constructs with anterior-posterior spinal reconstruction and bilateral posterior pedicle screws were significantly stiffer in flexion/extension than intact spines or spines with anterior plating alone. Anterior plating without pedicle screw fixation was no different from the intact spine in flexion/extension and lateral bending. All constructs had reduced stiffness in axial rotation compared with intact spines.
The addition of bilateral posterior instrumentation provided significantly greater stability at the thoracolumbar junction after total corpectomy than anterior plating and should be considered in cases in which anterior column reconstruction alone may be insufficient. In cases precluding bilateral posterior fixation, unilateral posterior instrumentation may provide some additional stability.