The Clinical Utility of Flexion-extension Cervical Spine MRI in 22q11.2 Deletion Syndrome.

BACKGROUND: Our goal is to correlate the findings on flexion and extension radiographs with dynamic magnetic resonance imaging (MRI), and the clinical history, in a nonrandomly selected cohort of patients with 22q11.2 deletion syndrome (22q). METHODS: All patients with the 22q who had a dynamic MRI from January 2004 to March 2015 were included. We analyzed multiple radiographic measurements on both the dynamic plain films and the MRIs, and correlated these findings with a review of each patient's medical record. RESULTS: Multiple congenital anomalies were identified as noted in previous studies, and 61% of the patients had a failure of fusion of the anterior (n=2, 9%), posterior (n=2, 9%), or anterior and posterior arches (n=10, 43%). Quantitative measurements were impossible to report with certainty because of the upper cervical anomalies, and no cases of instability were identified using a qualitative assessment. We identified spinal cord encroachment (30%) and impingement (18%); however, none of the patients had any signal change in their spinal cord. None of these findings could be definitively correlated with any clinical symptoms. A single patient was diagnosed with a Chiari I malformation, while another had cerebellar ectopia. CONCLUSIONS: Although the upper cervical anomalies are extremely common in 22q, we did not identify cases of instability on dynamic plain radiographs and MRI. Although our findings do not support routine screening with flexion and extension MRI, this study may be required in patients with neurological symptoms and/or findings or abnormalities on dynamic plain radiographs. LEVEL OF EVIDENCE: Level III.

Salvage Options in the Cerebral Palsy Hip: A Systematic Review.

BACKGROUND: No preferred procedure exists for the chronically painful, unreconstructable subluxated or dislocated hip in cerebral palsy. The purpose of this study was to compare pain relief and complication rates of salvage procedures in cerebral palsy for ambulatory and nonambulatory populations. METHODS: We searched Medline, Embase, and Cochrane databases using the search terms "cerebral palsy" and "hip dislocation." Inclusion and exclusion criteria were established to maintain data quality for analysis. A systematic review yielded 28 studies. Relevant information for postoperative pain and complications were extracted from each study and described. Our initial search identified 721 articles. Two hundred twenty duplications were excluded. Five hundred one were screened by title and abstract. One hundred articles underwent further full text and reference evaluation, yielding 25 studies. An additional 3 studies were then identified from the list of 25, yielding a total of 28 studies, which met our inclusion criteria. RESULTS: Among nonambulators, femoral head resection (FHR), valgus osteotomy (VO), and total hip arthroplasty (THA) were found to relieve pain better than arthrodesis [odds ratio (OR) 7.3, 95% confidence interval (CI), 2.2-24.8; OR 5.9, 95% CI, 1.6-22.8; OR 11.7, 95% CI, 1.1-297.5, respectively]. Arthrodesis had a significantly higher complication rate than FHR, VO, THA, and shoulder prosthetic interposition. No significant differences in complication rate were found between FHR and VO. Pain relief rates among nonambulators for FHR, VO, THA, shoulder prosthetic interposition, and arthrodesis were 90.4%, 88.4%, 93.8%, 90.9%, and 56.3%, respectively. Complication rates among nonambulators were 24.0%, 33.3%, 35.3%, 28.6%, and 106.3%, respectively. Comparison of pain relief and complication rates among ambulatory cerebral palsy patients in all procedures except THA was not possible because the populations could not be separated from nonambulators in numbers sufficient to perform statistical analysis. Data were available for 32 confirmed cases of THA in ambulators and was associated with a 93.3% pain relief rate and a 38.2% complication rate. CONCLUSIONS: Among nonambulators, the available literature suggests that FHR, VO, and THA may be superior at relieving pain than arthrodesis. FHR had the lowest absolute percentage of complications; however, no significant differences in complication rate or pain relief were found in nonambulators undergoing FHR or VO. Most of the complications for VO were implant related, and potentially amenable to hardware removal versus complications in FHR, which were related to the procedure itself such as proximal migration and heterotopic bone formation. THA in nonambulators was associated with complications such as dislocation and revision. Arthrodesis in nonambulators was associated with >100% complication rate and inferior pain relief compared with other procedures. Ambulatory patients had excellent pain relief with THA; however, the complication rate is higher than can be expected with non-neurological populations. Insufficient data exist to support use of other salvage procedures in ambulators. These conclusions should be interpreted with caution as all studies involved level IV evidence. LEVEL OF EVIDENCE: IV (systematic review of level IV studies).

Factors that Predict Overall Health and Quality of Life in Non-Ambulatory Individuals with Cerebral Palsy.

Background: It is unknown what role specific tasks associated with personal care, positioning, communication and social interaction, and comfort and emotions play in predicting the overall health and quality of life of individuals with non-ambulatory cerebral palsy (CP). In this study, we prospectively evaluated which of these factors were significant predictors of overall health and quality of life. Methods: Parents and guardians of non-ambulatory children, adolescents and young adults with CP were prospectively recruited from the Cerebral Palsy Clinic of a large pediatric academic hospital. Caregivers completed the CP Child Questionnaire®. Univariate analyses were used to identify relationships between overall health, overall quality of life (QOL), and responses in the following categories: personal care and activities of daily living, positioning and transfer mobility, comfort and emotions, and communication and social interaction. Significant predictors of overall health and QOL were then determined via logistic regression. Results: 64 patients ages 0-20 years and Gross Motor Function Classification System levels IV and V were included in our study (mean age 9.16 ±4.96 years). Overall QOL (OR 194.2, 95% CI, 9.5-3964.9) and comfort while sitting (OR 15.9, 95% CI, 1.2-205.3) were significant predictors of overall health. Feelings of unhappiness or sadness (OR 59.9, 95% CI, 1.6-2209.8), difficulty understanding the parent or guardian (OR 29.8, 95% CI, 1.6-543.7), and not attending school (OR 57.2, 95% CI, 2.6-1274.4) were significant predictors of lower overall quality of life. Conclusions: Overall QOL appears to predict overall health. Factors associated with comfort and emotions and communication and social interaction appear to predict overall QOL to a greater extent than personal care and transfer mobility.Level of Evidence: Prognostic II.