Comparing Percutaneous Physeal Epiphysiodesis and Eight-Plate Epiphysiodesis for the Treatment of Limb Length Discrepancy.

BACKGROUND: Either percutaneous or eight-plate epiphysiodesis have been shown to be effective growth modulation techniques for the treatment of limb length discrepancies (LLD). However, few studies compared the outcomes of both techniques with some confounding results. The aim of this study was to evaluate the outcomes of the both techniques in the treatment of the LLD. METHODS: Between 2004 and 2012, medical records of all cases that underwent either eight-plate or percutaneous epiphysiodesis (PE) were reviewed. Age at surgery, sex, diagnosis, surgical site (proximal tibia/distal femur), time of follow-up, complications, and additional procedures were noted. Correction of the LLD was evaluated to calculate the rate of correction and the percentage of improvement from the long-leg standing x-rays or scanograms, collected at each visit. RESULTS: There were 24 patients in the eight-plate (10 girls and 14 boys) and 48 patients in the PE group (28 girls and 20 boys). No statistically significant difference was found in the age, sex, preoperative LLD, or follow-up between groups. Both groups reached to an average LLD below 2 cm. The percentage of improvement was significantly higher in the PE group (P=0.031). The rate of individual femoral and tibial correction did not differ between the groups. CONCLUSIONS: Both methods are shown to be effective for LLD correction. However, PE led to greater improvement during the same follow-up time with fewer complications and less need for additional surgical procedures.

Sonographic Assessment of Hip Swaddling Techniques in Infants With and Without DDH.

BACKGROUND: The purpose of this single-examination pilot study was to confirm the ability to perform hip sonography while swaddled and to ascertain whether the various swaddling techniques influenced hip position and dynamics. METHODS: Dynamic sonography was used to evaluate 30 infants in both swaddled and unswaddled positions who were being seen in clinic for suspected or documented developmental dysplasia of the hip. A "treatment group" of 16 infants (32 hips) treated in a Pavlik harness and a "nontreatment group" of 14 untreated infants (28 hips) were studied.Criteria for comparing sonographic results between swaddled and unswaddled hip positions included femoral head position, instability, and range-of-motion restriction. RESULTS: Tight swaddling with a blanket was applied in 11 "nontreatment group" cases (20 hips; in 2 cases, only 1 hip studied) and produced limited flexion and abduction. One unstable left hip dislocated when tightly swaddled. Safe swaddling technique in 12 cases (24 hips) showed no limitation of flexion and abduction of the legs and no change in stability by sonography. Commercial swaddling products appeared to mildly restrict leg motion in 14 hips, but there was no change in hip position in the "nontreatment group." However, the commercial swaddling products changed the hip position in 3 Pavlik harness cases. CONCLUSIONS: Swaddling techniques that allow a free range of leg motion may not affect hip stability in normal infants or those being treated with Pavlik harness. Swaddling with restricted leg motion increases potential for hip instability. Tight swaddling dislocated 1 unstable hip, and commercial swaddling products judged to apply only mild restriction of leg motion negatively impacted 3 cases being treated for developmental dysplasia of the hip with Pavlik harness. On the basis of this pilot study, we advise caution when swaddling infants, especially with techniques that restrict leg motion. Further study of the long-term effects of swaddling is warranted. LEVEL OF EVIDENCE: Level II.

Comparison of Direct Pars Repair Techniques of Spondylolysis in Pediatric and Adolescent Patients: Pars Compression Screw Versus Pedicle Screw-Rod-Hook.

STUDY DESIGN: Retrospective clinical cohort study. OBJECTIVE: To compare the clinical and radiographic outcomes of patients who were treated with intrasegmental pars fixation by either laminar compression screw (LS) or a pedicle screw, rod, and laminar hook (PSRH) construct. SUMMARY OF BACKGROUND DATA: Spondylolysis is a nonunion defect of the pars interarticularis. In symptomatic spondylolysis, direct repair of the pars interarticularis defect can preserve motion and prevent abnormal stresses at the adjacent levels. METHODS: Sixteen patients who failed nonoperative treatment and underwent direct pars repair by using LS (n=9) or PSRH (n=7) constructs were included in the study. Clinical outcome was assessed by using the MacNab criteria. Radiologic fusion and complications were evaluated using plain radiographs or computed tomography images and patient charts. RESULTS: The healing rate was 100% after 6 months. The healing time was similar in both the groups: LS, 6.5 months; PSRH, 6.2 months. Patients with PSRH (5.9 mo) were more likely to return to sports earlier relative to patients with LS (7.7 mo). There were no complications in the LS group; in the PSRH group, 1 patient had mild sensory deficit and 2 had superficial wound infections. The MacNab criteria for pain assessment showed an excellent or good outcome in 8 of 9 patients in LS group and 6 of 7 patients in PSRH group. Relative to LS patients, there was a significant increase in surgical time and estimated blood loss among PSRH patients. CONCLUSIONS: Either of the mentioned 2 techniques appears to produce acceptable results. Biplanar fluoroscopy and navigation systems could minimize the risk of screw misplacement with LS construct. Familiarity with the various fixation techniques will allow the surgeon to select the most appropriate surgical technique.

The effect of serial growing rod lengthening on the sagittal profile and pelvic parameters in early-onset scoliosis.

STUDY DESIGN: Retrospective case series. OBJECTIVE: To report the effect of repeated growing rod (GR) lengthenings on the sagittal and pelvic profile in patients with early-onset scoliosis. SUMMARY OF BACKGROUND DATA: Posterior distraction-based GRs have gained popularity as a technique for the surgical management of early-onset scoliosis. However, there are no published studies on the effect of serial GR lengthenings on sagittal balance, thoracic kyphosis (TK), lumbar lordosis (LL), and pelvic parameters. METHODS: We retrospectively reviewed data from a multicenter early-onset scoliosis database. Forty-three patients who were able to walk with minimum 2-year follow-up who underwent single- or dual-GR surgery were included for review. Mean number of lengthenings was 6.4 (range, 3-16). Mean preoperative age was 5.6 years (standard deviation, 2.4 yr), and mean follow-up was 3.5 years. Maximum TK, LL, and sagittal balance were assessed preoperatively, after index surgery, and at the latest follow-up. RESULTS: There was a significant decrease both in TK and LL after index surgery, which then increased during the lengthening period. There was a significant increase in both proximal junctional kyphosis and distal junctional angle. Pelvic parameters (pelvic tilt, pelvic incidence, sacral slope) were unchanged during the treatment period. Significant improvement was observed in sagittal balance. There was a correlation between the change in TK and change in LL. CONCLUSION: TK decreased after index surgery and increased between the index surgery and the latest follow-up, which was accompanied by an increase in LL. All-screw proximal constructs had mean 9° more proximal junctional kyphosis than all-hook proximal constructs. An increase in proximal junctional kyphosis and distal junctional angle was found during the treatment period. Although there was an independent effect of number of lengthenings on TK, there was no significant detrimental effect on other sagittal spinopelvic parameters. GRs had a positive effect on sagittal vertical axis, which returned patients to a more neutral alignment through the course of treatment. LEVEL OF EVIDENCE: 4.

Hip pathology in Majewski osteodysplastic primordial dwarfism type II.

INTRODUCTION: Majewski osteodysplastic primordial dwarfism type II (MOPDII) is characterized by severe prenatal and postnatal growth failure with microcephaly, characteristic skeletal dysplasia, an increased risk for cerebrovascular disease, and insulin resistance. MOPDII is caused by mutations in the pericentrin (PCNT) gene and is inherited in an autosomal-recessive manner. This study aimed to determine the incidence of hip pathology in patients with molecularly confirmed MOPDII and to describe the functional outcomes of surgical treatment. METHODS: Thirty-three enrolled patients had a clinical diagnosis of MOPDII. Biallelic PCNT mutations or absent pericentrin protein was confirmed in 25 of these patients. Twelve patients (7 female) had appropriate clinical and radiographic records at this institution and were included in this study. The data collected included age at presentation, age at surgery, sex, body weight and height, weight-bearing status at diagnosis, and the clinical examination. RESULTS: Four patients (31%) had coxa vara: 3 unilateral and 1 bilateral. Three unilateral patients had in situ pinning at a mean age 4 years. The patient with bilateral coxa vara had valgus osteotomy at the age of 5 years. Two children had bilateral hip dysplasia and subluxation with no surgery. One patient had bilateral developmental hip dislocations. The patient was treated by open reduction-spica cast and 2 years after surgery, coxa valga was noted. Another patient was diagnosed at an age of 12 years with bilateral avascular necrosis of the hips. Four patients did not have hip pathology. CONCLUSIONS: Hip pathology is common among children with MOPDII; coxa vara is the most frequent diagnosis. Routine clinical and radiographic hip evaluation is important. The capital femoral epiphysis appears to slip down along the shaft, giving the appearance of a proximal femoral epiphysiolysis. A hip diagnosed with slipped capital femoral epiphysis in early life may progress to severe coxa vara. LEVEL OF EVIDENCE: Level IV.

Growth-sparing spinal instrumentation in skeletal dysplasia.

STUDY DESIGN: Retrospective case series. OBJECTIVE: To report the outcomes of distraction-based, growth-sparing spinal instrumentation in patients with skeletal dysplasia. SUMMARY OF BACKGROUND DATA: Patients with skeletal dysplasia with spinal deformity often undergo early fusion, further compromising an already small chest. Nonfusion techniques may provide a safe alternative and allow for thoracic growth. METHODS: Between 2004 and 2010, 12 children with a diagnosis of various types of skeletal dysplasia underwent growth-sparing spinal instrumentation for severe spinal deformities. The mean duration of treatment with growing rods was 57 months (42-84 mo). Nine patients were treated with growing rods (8 dual, 1 single), and 3 were treated with vertical expandable prosthetic titanium rib (VEPTR; Synthes). Preoperative, initial postoperative, and final follow-up anteroposterior and lateral spine radiographs were measured for magnitude of deformity, junctional kyphosis, and implant failure. RESULTS: The major curve Cobb angle improved from a mean of 79° preoperatively to a mean of 41° at the last follow-up (52%). There was a decrease in mean thoracic kyphosis from 77° preoperatively to 64° at final follow-up and an increase in mean lumbar lordosis from 58° preoperatively to 63° at final follow-up. The mean space available for the lungs increased by 26 mm on the concave and 24 mm on the convex side. Six patients required revision surgery for proximal junctional kyphosis. There were 4 rod failures and 6 hook and 8 screw dislodgements. One patient with vertical expandable prosthetic titanium rib had failed rib fixation that required revision. CONCLUSION: Growth-sparing spinal instrumentation in patients with skeletal dysplasia and severe spinal deformity has a high complication and revision rate, and surgeons should closely monitor these patients. The complication rate is comparable with previous reports on patients with other diagnoses. However, deformities were well controlled, some trunk growth was achieved, and fusion surgery was delayed in all cases. LEVEL OF EVIDENCE: 4.

Cerebral palsy patients discovered dead during sleep: experience from a comprehensive tertiary pediatric center.

OBJECTIVES: It is not uncommon for children with cerebral palsy (CP) to be discovered dead during sleep (DDDS); however, the factors associated with this pattern of mortality remain unknown. The current study aims to describe the mortality associated with children with CP from a single, tertiary care center who were DDDS. METHODS: A retrospective (case-only) design to examine proportionate mortality and patient characteristics and co-morbidities that may be related to children DDDS between 1993 and 2011. RESULTS: There were 177 patients with CP whose deaths were reported to our institution during the study period, of which 19 were DDDS at home. The period proportionate mortality (PPM) was 114.5 per 1000. The average age at time of death was 17 years and 6 months (minimum, 6 years; maximum, 25 years). All but one of the DDDS patients had gastrointestinal feeding tubes, seizure disorders, respiratory disorders, and were non-ambulatory. Very importantly, our DDDS patients manifested clusters of respiratory disorders, namely recurrent aspiration pneumonia (10/19), asthma pneumonitis (4/19), food/vomitius inhalation (6/19), reactive airway disease (16/19), respiratory failure (14/19), chronic bronchitis (7/19), chronic obstructive lung disease (9/19), and nocturnal respiratory insufficiency (16/19). CONCLUSIONS: Respiratory disorders, severe motor disability, seizures, and intellectual status are possible co-morbidities that may be associated with DDDS. There is a need for further study in order to understand what type of monitoring and care (if any) may help prevent DDDS related to these co-morbidities and sleep disorders/abnormalities.