Upper-extremity spasticity and functionality after selective dorsal rhizotomy for cerebral palsy: a systematic review.

OBJECTIVE: Lumbosacral selective dorsal rhizotomy is a neurosurgical treatment option to reduce spasticity in the lower extremities in children with cerebral palsy. Surprisingly, concomitant improvement of spasticity in the upper extremities and functionality of the hands has been sporadically reported postoperatively. In this systematic review, the authors aimed to quantify the postoperative improvement in upper-extremity spasticity and functionality, identify predictors, and discuss underlying mechanisms. METHODS: The authors searched the MEDLINE and Embase databases for studies reporting upper-extremity outcomes in cerebral palsy patients after selective dorsal rhizotomy that reported one or more of the following clinical scales: the Ashworth Scale (AS), the Modified AS (MAS), the fine motor skills domain of the Peabody Developmental Motor Scales (PDMS), the Quality of Upper Extremity Skills Test (QUEST), the self-care domain of the Functional Independence Measure for Children (WeeFIM), or the self-care domain of the Pediatric Evaluation of Disability Inventory (PEDI). The authors arbitrarily divided postoperative follow-up into short-term (< 6 months), medium-term (6-24 months), and long-term (> 24 months) follow-up. A 1-point change in MAS score has been reported as clinically significant. To assess bias, the Cochrane Collaboration's tool and ROBINS-I tool were used. RESULTS: The authors included 24 articles describing 752 patients. Spasticity reduction of the upper extremities ranged from 0.30 to 0.55 (AS) and between 0 and 2.9 (MAS) at medium-term follow-up. This large variability may partially be attributed to a floor effect since patients with normal upper-extremity function would not be expected to have further improvement. QUEST improvement ranged from 2.7% to 4.5% at medium-term follow-up. The mean improvements in functional skills of the self-care domain of the PEDI were 4.3 at short-term and 7 at medium-term follow-ups and ranged from 10.8 to 34.7 at long-term follow-up. There are insufficient data to draw meaningful conclusions regarding the PDMS fine motor skills and the WeeFIM self-care domains. CONCLUSIONS: The literature suggests that a pronounced postoperative spasticity reduction in the lower extremities and a moderately severe preoperative upper-extremity spasticity may positively predict postoperative reduction in upper-extremity spasticity. There are at least 5 hypotheses that may explain the postoperative reduction in upper-extremity spasticity and functionality: 1) a somatosensory cortex reorganization favoring the hand region over the leg region, 2) a decrease in abnormal electrical transmission throughout the spinal cord, 3) an indirect result of improved posture due to improved truncal and leg stability, 4) an indirect consequence of occupational/physical therapy intensification, and 5) a maturation effect. However, all remain unproven to date.

Evolution of head-shaft angle and neck-shaft angle in childhood.

The current retrospective study investigates the natural evolution of head-shaft angle (HSA) and neck-shaft angle (NSA) in childhood. It is not known if a high HSA in early childhood leads to a high HSA in adulthood. This study aims to characterize the evolution of HSA and compares it with the more commonly known NSA in healthy children. We measured radiographs of 84 different healthy hips of children between 3 and 14.5 years old who underwent at least 2 radiographs of the pelvis, corresponding to 286 measurements. We used a linear mixed model to determine the covariance between random intercept and slope while allowing each individual hip to change over time. The covariance for HSA between random intercept and random slope was -4.262 (p < 0.001), corresponding to a high negative correlation of -0.717, for NSA -2.754 (p = 0.031) or a high negative correlation of -0.779. HSA and NSA were strongly correlated, a value of 0.736 (p < 0.001) was measured. The high negative correlation for random intercept and random slope means that the higher the initial value (intercept), the steeper the decline (slope). Therefore HSA decreases faster in hips with high HSA at an early age. Hips with high HSA in early childhood do not necessarily lead to hips with high HSA in adulthood. Our results may aid in future clinical decision making in patients with developmental dysplasia of the hip (DDH) with high HSA in particular.

Lowered dose full-spine radiography in pediatric patients with idiopathic scoliosis.

PURPOSE: To optimize our standard full-spine radiography with respect to diagnostic quality and dose. METHODS: A phantom study was performed to establish an optimal posterior-anterior view (PA) full spine protocol having the lowest dose with non-inferior quality compared to standard. We then applied this protocol in 40 pediatric patients (group B). The radiographs were scored on six criteria by a pediatric radiologist and orthopedist and compared to the scores of 40 PA full spine radiographs performed in 2013 with standard protocol (group A). Radiation dose was assessed by dose area product (DAP) and effective dose (E). Statistical analysis included independent samples t test, Mann-Whitney U test and intra-class correlation coefficient (ICC). RESULTS: An optimized protocol was defined (0.2 mm Cu filter, 0.87 relative exposure, with grid). Mean age was 13.3 ± 1.6 years for group A and 13.4 ± 1.7 years for group B. For group B, the mean DAP was 47.0 µGy m2 with an E of 0.13 mSv. For group A, the mean DAP was 85.3 µGy m2 with an E of 0.24 mSv. This represents a dose reduction of 45%. Mean image quality scores for group A (27.9 ± 2.4) and group B (28.1 ± 2.3) were similar (p = 0.612). Interobserver agreement was observed to be excellent (ICC 0.92). CONCLUSION: This study demonstrates that a low-dose full-spine radiograph can be performed in patients with idiopathic scoliosis without loss of image quality. These slides can be retrieved under Electronic Supplementary Material.

Prevalence of neurofibromatosis type 1 in congenital pseudarthrosis of the tibia.

UNLABELLED: A strong relationship between congenital pseudarthrosis of the tibia (CPT) and neurofibromatosis type 1 (NF1) has been suggested, but prevalence varies widely throughout the literature and the criteria used for diagnosis are very heterogeneous. Literature focus is mainly on treatment and no specific review on the prevalence of NF1 has been published. Based on our own observations, we hypothesized the prevalence of NF1 in patients with CPT to be higher than what is previously accepted. We conducted a comprehensive literature review on this topic and compared results with our study population. Twenty-one out of twenty-five patients in the study population matched the NIH diagnostic criteria for NF1 (84.0 %, CI95 = 69.6-98.4 %). These results are higher than the prevalence reported in the literature (55.4 %, CI95 = 50.4-60.4 %). CONCLUSIONS: The prevalence of NF1 in patients with CPT might be higher than what is reported until now because the criteria of NF1 generally appear only after the diagnosis of CPT. We propose a repeat meticulous examination and a multidisciplinary approach with a clinical genetic counseling in all CPT patients. WHAT IS KNOWN: • Congenital pseudarthrosis of the tibia and neurofibromatosis type 1 are closely related. • Literature focus is mainly on treatment and little epidemiologic research is available. What is New: • Prevalence of neurofibromatosis type 1 in patient with congenital pseudarthrosis of the tibia might be higher than what is reported until now. • A multidisciplinary approach with meticulous clinical examination and genetic counseling might lead to an earlier diagnosis of neurofibromatosis type 1 in patients with congenital pseudarthrosis of the tibia.

Ilizarov bone transport as a treatment of congenital pseudarthrosis of the tibia: a long-term follow-up study.

PURPOSE: Most studies on congenital pseudarthrosis of the tibia (CPT) report on the short-term union rate and refracture rate but do not take into account the long-term outcome. This review includes patients treated with an Ilizarov bone transport, who all reached skeletal maturity. It describes long-term results and highlights any prognostic factors that could predict the final outcome. METHODS: The records of patients with CPT treated with an Ilizarov bone transport in our institution were retrospectively evaluated. RESULTS: A total of 12 consecutive patients were studied. The mean follow-up was 24.5 years (range 6-39 years). Primary consolidation was seen in ten patients (83 %). Half of these patients had a refracture. At final follow-up, eight patients experienced union and four remained un-united, of whom one had an amputation. CONCLUSIONS: The present data confirm a good primary healing rate. However, tibial union at final follow-up was only seen in 67 %, indicating that refracture is the main issue. United bone is often of inferior biological and mechanical quality, so lifetime protection with intramedullary devices, braces or a combination of both is recommended.

The Pentaconcept in skeletal tissue engineering. A combined approach for the repair of bone defects.

Tissue engineering has become a hot topic in modern medicine. Its application in a surgical setting, such as for the treatment of skeletal defects, still has to tackle some problems that might look simple at first sight, but need a well-structured handling combining surgery and science, with in a central position the patient, who is both cell donor and receptor of the tissue engineered end product. To achieve this goal in a clinical setting, a five steps pathway is described and designated as the Pentaconcept, integrating all ingredients for successful reconstructive procedures.

Reduction of treatment time in external ring fixation using the monofix device.

One of the disadvantages of the Ilizarov method is the long treatment time needed for extensive lengthening or complex deformity correction. To minimize the discomfort of the circular frame the authors have introduced a 'frame reduction' technique in which the ring frame is converted towards a unilateral fixator, the 'Monofix'. Both its efficacy and patient satisfaction encouraged the authors to apply this method on a routine basis since 1998 for lower and upper limb corrections. The technique is demonstrated, its indications and potential problems are discussed.

Long-term outcome of 73 Zweymüller total hip prostheses with a screw cup in patients under 50 years of age.

Total hip arthroplasty in young patients is associated with high failure rates and the best option for this demanding group of patients remains controversial. We report the long-term results of 73 consecutive Zweymüller total hip arthroplasties with a titanium threaded cup and a polyethylene insert in 67 patients aged under 50 years at the time of surgery (mean 43 years, range 23-49). Independent assessment was performed clinically and radiographically, and Kaplan-Meier analysis was used to determine survival for different end-points. Three hips were revised for septic loosening, three cups for aseptic loosening and one hip because of a periprosthetic fracture. Three patients (3 hips) died and seven patients (8 hips) were lost to radiographic follow-up without any reoperation. The mean follow-up was 17.5 (15-21) years and the mean HHS was 90 (52-100). Survival with an end-point of revision for any reason was 89% (95% C.I. 85-93) and of revision for aseptic loosening was 94% (C.I. 95-99) at 17 years. Zweymüller total hip arthroplasty with a titanium threaded cup and a polyethylene insert showed good long-term results, even in this group of young patients.

A new digital preoperative planning method for total hip arthroplasties.

Preoperative templating is an important part of a THA. The ability to accurately determine magnification of the hip on the radiograph and apply identical magnification to the radiograph and template will improve accuracy of preoperative templating of THA. We designed a templating method using a new way of determining the hip magnification with a linear relationship between magnification of the hip and the reference object on top of the pubis symphysis; the relationship was determined on 50 radiographs. We then compared our method with two other templating methods: an analog method assuming an average hip magnification of 15% and a digital method determining the hip magnification with a one-to-one relationship between the reference object and the hip. All methods were reproducible. Uniform undersizing occurred when templating with the digital method based on the one-to-one relationship; the analog method best predicted the implanted prosthesis size, closely followed by our new digital templating method; the new method will be particularly applicable for preoperative THA when analog methods are replaced by digital methods.