There is no known correlation between Down syndrome and craniosynostosis. The authors report 2 infants with trisomy 21 and right unilateral coronal craniosynostosis. Both patients were clinically asymptomatic but displayed characteristic craniofacial features associated with each disorder. One patient underwent a bilateral fronto-orbital advancement and the other underwent an endoscopically assisted strip craniectomy with postoperative helmet therapy. Both patients demonstrated good cosmesis at follow-up.
Report of 2 cases
Alan Siu, Gary F. Rogers, John S. Myseros, Siri S. Khalsa, Robert F. Keating and Suresh N. Magge
Siri Sahib S. Khalsa, Alan Siu, Tiffani A. DeFreitas, Justin M. Cappuzzo, John S. Myseros, Suresh N. Magge, Chima O. Oluigbo and Robert F. Keating
Previous studies have indicated an association of Chiari malformation Type I (CM-I) and a small posterior fossa. Most of these studies have been limited by 2D quantitative methods, and more recent studies utilizing 3D methodologies are time-intensive with manual segmentation. The authors sought to develop a more automated tool to calculate the 3D posterior fossa volume, and correlate its changes after decompression with surgical outcomes.
A semiautomated segmentation program was developed, and used to compare the pre- and postoperative volumes of the posterior cranial fossa (PCF) and the CSF spaces (cisterna magna, prepontine cistern, and fourth ventricle) in a cohort of pediatric patients with CM-I. Volume changes were correlated with postoperative symptomatic improvements in headache, syrinx, tonsillar descent, cervicomedullary kinking, and overall surgical success.
Forty-two pediatric patients were included in this study. The mean percentage increase in PCF volume was significantly greater in patients who showed clinical improvement versus no improvement in headache (5.89% vs 1.54%, p < 0.05) and tonsillar descent (6.52% vs 2.57%, p < 0.05). Overall clinical success was associated with a larger postoperative PCF volume increase (p < 0.05). These clinical improvements were also significantly associated with a larger increase in the volume of the cisterna magna (p < 0.05). The increase in the caudal portion of the posterior fossa volume was also larger in patients who showed improvement in syrinx (6.63% vs 2.58%, p < 0.05) and cervicomedullary kinking (9.24% vs 3.79%, p < 0.05).
A greater increase in the postoperative PCF volume, and specifically an increase in the cisterna magna volume, was associated with a greater likelihood of clinical improvements in headache and tonsillar descent in patients with CM-I. Larger increases in the caudal portion of the posterior fossa volume were also associated with a greater likelihood of improvement in syrinx and cervicomedullary kinking.
Siri Sahib S. Khalsa, Ndi Geh, Bryn A. Martin, Philip A. Allen, Jennifer Strahle, Francis Loth, Desale Habtzghi, Aintzane Urbizu Serrano, Daniel McQuaide, Hugh J. L. Garton, Karin M. Muraszko and Cormac O. Maher
Chiari malformation Type I (CM-I) is typically defined on imaging by a cerebellar tonsil position ≥ 5 mm below the foramen magnum. Low cerebellar tonsil position is a frequent incidental finding on brain or cervical spine imaging, even in asymptomatic individuals. Nonspecific symptoms (e.g., headache and neck pain) are common in those with low tonsil position as well as in those with normal tonsil position, leading to uncertainty regarding appropriate management for many patients with low tonsil position and nonspecific symptoms. Because cerebellar tonsil position is not strictly correlated with the presence of typical CM-I symptoms, the authors sought to determine if other 2D morphometric or 3D volumetric measurements on MRI could distinguish between patients with asymptomatic and symptomatic CM-I.
The authors retrospectively analyzed records of 102 pediatric patients whose records were in the University of Michigan clinical CM-I database. All patients in this database had cerebellar tonsil position ≥ 5 mm below the foramen magnum. Fifty-one symptomatic and 51 asymptomatic patients were matched for age at diagnosis, sex, tonsil position, and tonsil morphology. National Institutes of Health ImageJ software was used to obtain six 2D anatomical MRI measurements, and a semiautomated segmentation tool was used to obtain four 3D volumetric measurements of the posterior fossa and CSF subvolumes on MRI.
No significant differences were observed between patients with symptomatic and asymptomatic CM-I related to tentorium length (50.3 vs 51.0 mm; p = 0.537), supraoccipital length (39.4 vs 42.6 mm; p = 0.055), clivus-tentorium distance (52.0 vs 52.1 mm; p = 0.964), clivus-torcula distance (81.5 vs 83.3 mm; p = 0.257), total posterior fossa volume (PFV; 183.4 vs 190.6 ml; p = 0.250), caudal PFV (152.5 vs 159.8 ml; p = 0.256), fourth ventricle volume to caudal PFV ratio (0.0140 vs 0.0136; p = 0.649), or CSF volume to caudal PFV ratio (0.071 vs 0.061; p = 0.138).
No clinically useful 2D or 3D measurements were identified that could reliably distinguish pediatric patients with symptoms attributable to CM-I from those with asymptomatic CM-I.
Timothy J. Yee, Brandon W. Smith, Jacob R. Joseph, Yamaan S. Saadeh, Jay K. Nathan, Elyne N. Kahn, Siri S. Khalsa, Kelsey J. Fearer, Michael J. Kirsch, David R. Nerenz, Victor Chang, Jason M. Schwalb, Muwaffak M. Abdulhak and Paul Park
The Oswestry Disability Index (ODI) is one of the most commonly used patient-reported outcome instruments, but completion of this 10-question survey can be cumbersome. Tools from the Patient-Reported Outcomes Measurement Information System (PROMIS) are an alternative, and potentially more efficient, means of assessing physical, mental, and social outcomes in spine surgery. Authors of this retrospective study assessed whether scores on the 4-item surveys of function and pain from the PROMIS initiative correlate with those on the ODI in lumbar spine surgery.
Patients evaluated in the adult neurosurgery spine clinic at a single institution completed the ODI, PROMIS Short Form v2.0 Physical Function 4a (PROMIS PF), and PROMIS Short Form v1.0 Pain Interference 4a (PROMIS PI) at various time points in their care. Score data were retrospectively analyzed using linear regressions with calculation of the Pearson correlation coefficient.
Three hundred forty-three sets of surveys (ODI, PROMIS PF, and PROMIS PI) were obtained from patients across initial visits (n = 147), 3-month follow-ups (n = 107), 12-month follow-ups (n = 52), and 24-month follow-ups (n = 37). ODI scores strongly correlated with PROMIS PF t-scores at baseline (r = −0.72, p < 0.0001), 3 months (r = −0.79, p < 0.0001), 12 months (r = −0.85, p < 0.0001), and 24 months (r = −0.89, p < 0.0001). ODI scores also correlated strongly with PROMIS PI t-scores at baseline (r = 0.71, p < 0.0001), at 3 months (r = 0.82, p < 0.0001), at 12 months (r = 0.86, p < 0.0001), and at 24 months (r = 0.88, p < 0.0001). Changes in ODI scores moderately correlated with changes in PROMIS PF t-scores (r = −0.68, p = 0.0003) and changes in PROMIS PI t-scores (r = 0.57, p = 0.0047) at 3 months postoperatively.
A strong correlation was found between the ODI and the 4-item PROMIS PF/PI at isolated time points for patients undergoing lumbar spine surgery. Large cohort studies are needed to determine longitudinal accuracy and precision and to assess possible benefits of time savings and improved rates of survey completion.
Clay M. Elswick, Siri Sahib S. Khalsa, Yamaan S. Saadeh, Aditya S. Pandey and Mark E. Oppenlander
Spinal dural arteriovenous fistulas are diagnostically challenging lesions, and they are not well described in patients with a history of a spinal deformity correction. The authors present the challenging case of a 74-year-old woman who had previously undergone correction of a spinal deformity with subsequent revision. Several years after the last deformity operation, she developed a progressive myelopathy with urinary incontinence over a 6-month period. After evaluation at the authors’ institution, an angiogram was obtained, demonstrating a fistula at the T12–L1 region. Surgical ligation of the fistula was performed with subsequent improvement of the neurological symptoms. This case is thought to represent the first fistula documented in an area of the spine that had previously been operated on, and to the authors’ knowledge, it is the first case report to be associated with spinal deformity surgery. A brief historical overview and review of the pathophysiology of spinal dural arteriovenous fistulas is also included.