Rachel Moor

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.

Objective 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 & Methods This 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 screw-rod 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. Results Ninety-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 (∆SL). An annular tear on preoperative magnetic resonance imaging (MRI) predicted a 2.7° decrease in ∆SL. A Schmorl's node on preoperative MRI predicted a 4.0° decrease in change in LL (∆LL). Conclusions A 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 ∆SL.