Neurologic sequalae of Noonan syndrome have been postulated in the literature. A topic of significance is the role of RASopathy in the shared pathophysiology of Noonan Syndrome and Chiari I malformation. In this unique case report, we present a patient with concomitant Noonan Syndrome and Chiari I with 4th ventricular outflow obstruction. The case highlights the importance of close clinical suspicion in this patient population. We utilize the case to delve into intricacies of the known pathophysiology and encourage ongoing investigation. Keywords: Noonan syndrome; Chiari I malformation; RASopathy.
Paragangliomas are rare tumors that may present with cranial neuropathies when located along the skull base. Supratentorial paragangliomas are less likely to secrete catecholamines but should be worked up, nonetheless. We highlight a case of a female in her fourth decade found to have a petroclival lesion following initial presentation that included one month of tooth pain, dysphagia, diplopia, hoarseness and right hemifacial hypoesthesia. Magnetic resonance imaging of the brain demonstrated a T2 hyperintense lesion favored to be a petroclival meningioma. Pre-operative angiography demonstrated a hypervascular tumor. She underwent a combined presigmoid craniotomy with posterior petrosectomy performed by both neurosurgery and neuro-otology. Pathology demonstrated paraganglioma. She had small volume residual tumor and is planned for continued outpatient radiotherapy. Paragangliomas should be on the differential for skull base lesions. Management paradigm involves multidisciplinary care and a combination of surgical resection and post-operative radiation. In this paper, we discuss underlying pathophysiology as well as appropriate workup and management.
Transforaminal lumbar interbody fusion (TLIF) is a common approach and results in varying degrees of lordosis correction. The purpose of this study is to determine preoperative radiographic spinopelvic parameters that predict change in postoperative segmental and lumbar lordosis after TLIF.
Materials & MethodsThis study is a single surgeon retrospective review of one-level and two-level TLIFs from L3-S1. All patients underwent bilateral facetectomies, 10 mm TLIF cage (non-lordotic) insertions, and bilateral pedicle screwrod construct placements. Pre-and post-operative X-rays were assessed for preoperative segmental lordosis (SL), lumbar lordosis (LL), and pelvic incidence (PI). Univariate and multi-predictor linear regression analyses were performed to determine the relationships between preoperative radiographic findings and change in early postoperative segmental and lumbar lordosis.
ResultsNinety-seven patients contributing 128 intervertebral segments were examined. The mean change in SL after TLIF was 7.3 (range: 0.10-28.9°, SD 6.39°). The mean change in LL after TLIF was 5.5˚ (range: -14.8-39.2°, standard deviation (SD) 7.16°). Greater preoperative LL predicted less postoperative LL correction, while greater preoperative PI predicted more postoperative SL and LL correction. Greater anterior disk height was noted to be associated with a decreased change in SL (ΔSL). An annular tear on preoperative magnetic resonance imaging (MRI) predicted a 2.7° decrease in ΔSL. A Schmorl's node on preoperative MRI predicted a 4.0° decrease in change in LL (ΔLL).
ConclusionsA greater preoperative lordosis and a lower spinopelvic mismatch lessen the potential for an increase in the postoperative SL and LL after a TLIF, which is likely due to a 'ceiling' effect of an otherwise optimized spinal alignment. A greater anterior disk height and the presence of an annular tear are associated with decreased ΔSL.
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