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Background: The association of scoliosis and congenital limb deficiency has been well described. However, the incidence of neural axis abnormalities in this population is not known. The ability to assess the neural axis by physical examination may be limited in patients with a limb deficiency. Although mobility of the spine is important for all children, it can be especially so in children with a limb deficiency. As spinal fusion in children with limb deficiency potentially has more functional impact, detecting reversible forms of scoliosis seems particularly important., Methods: Retrospective review of children treated at 1 institution between 1990 and 2017 with both a diagnosis of a congenital limb deficiency, upper or lower, and scoliosis. Children were excluded if they had any neurological difference on history or physical examination, if they had sacral agenesis or spina bifida, or if their limb deficiency was related to trauma or early amniotic rupture sequence., Results: Twenty-four children were identified, 11 with lower extremity deficiency, 14 with upper extremity deficiency with 1 having both. Fifteen children demonstrated neural axis abnormalities, 6 (40%) required neurosurgery. Five (45%) of 11 lower extremity deficiency children had MRI findings, 3 of these needing neurosurgery. Of the 14 upper extremity deficiency children, 10 had MRI changes, and 3 required neurosurgery. Eight children with congenital scoliosis, 5 had MRI findings, with 4 children requiring neurosurgery. The other 16 children had scoliosis without vertebral abnormalities, 10 had MRI findings, and 2 required neurosurgery., Conclusions: There is a high incidence of neural axis abnormalities (63%) in children with congenital limb deficiencies and scoliosis. A large portion of these require neurosurgical intervention. MRI should be considered soon after presentation in this population of children., Level of Evidence: Level IV., Design: Retrospective cohort.

Background: Early-onset scoliosis is a spine deformity that presents before the age of 3 years. When compared with age-matched controls, children with the condition are known to be at risk for significant morbidity and mortality. Although many works support the use of casting for this condition, the key technical factors to optimize the outcomes of casting are not clear. This work was designed to evaluate the role of frequency of radiographic imaging and over the shoulder straps in the outcomes of casting., Methods: Two surgeons at 2 centers followed nearly identical protocols for applying casts for early-onset scoliosis. At center A, the surgeon hoped to improve outcomes by obtaining radiographs after each cast and by reinforcing the cast with shoulder straps. At center B, the surgeon did not use shoulder straps and limited radiographs to once every 6 months. Children were included if they were 3 years or below of age and had a curve of ≥50 degrees at the time of the first cast and had a minimum of 3 years of follow-up. Center was used as a variable in a multivariable regression that also included: age at first cast, initial curve magnitude, and presence of a syrinx or genetic syndrome with the outcome of curve resolution., Results: There were 40 children at center A, 9 of whom experienced resolution of their scoliosis. There were 36 children at center B, and 11 demonstrated scoliosis resolution. At center A, 2 of 10 children with a syrinx or genetic syndrome demonstrated curve resolution while 7 of 30 without these comorbidities did. At center B 3 of 10 children with a syrinx or genetic syndrome had curve resolution while 8 of 26 children without these comorbidities did., Conclusions: The children in group A demonstrated results very similar to the children of group B. Thus, the extra burden of shoulder straps and frequent radiographs are unnecessary., Level of Evidence: Level III.

Background: The purpose of this study is to examine the frequency of complications in children with myelodysplasia (MD) undergoing tibial rotational osteotomies with a matched cohort of children with cerebral palsy (CP). It was postulated that because of the unique health issues facing children with MD more complications would be observed., Methods: A retrospective chart review was performed to identify children with MD who underwent primary tibial rotational osteotomy between 1997 and 2012 and had a minimum 2-year follow-up. The 15 children thus identified were matched for age, body mass index, and functional ability with 15 children with CP. Outcome measures were complications that occurred within a year of osteotomy or hardware removal. Major complications were defined as nonunions or malunions, hardware failures, deep infections, fractures, and stage III or IV decubiti. Recurrence of rotational deformity requiring revision osteotomy at any time was also defined as a major complication. Minor wound problems healing within 6 weeks with only local care were considered minor complications., Results: Fifteen children with MD, who underwent 21 tibial derotational osteotomies, were available for review with a mean 7-year follow-up. The 15 children with CP underwent 22 tibial derotational osteotomies with a mean of 6 years of follow-up. In each cohort there were 3 children classified as GMFCS I, 3 children as GMFCS II, 4 children as GMFCS III, and 5 as GMFCS IV. Three (20%) of the children with MD experienced major complications (1 infected nonunion and 2 children who experienced bilateral malunions requiring revisions). One child with a major complication was classified as GMFCS II and the other 2 as GMFCS IV. None of the children with CP experienced a major complication., Conclusions: The majority of children in both groups experienced good results, but children with MD have more frequent major complications. More frequent complications were seen in children with less functional ability., Level of Evidence: Level III-prognostic study, case-control study.

Background: Previous work has demonstrated best results for casting in infantile scoliosis when the curves are small and the child begins casting under 2 years of age. This study examines if casting can delay the need for growth friendly instrumentation in severe curves (50 to 106 degrees) and how the comorbidities of syrinx or genetic syndromes affected outcomes., Methods: All children undergoing casting for scoliosis at a single institution over an 8-year period were examined. Inclusion criteria included initial curve at first casting of ≥50 degrees, age ≤3 years at the start of casting, and a minimum follow-up of 3 years. Of 148 children undergoing casting during this period, 44 met our inclusion criteria. All children underwent magnetic resonance imaging. Ten children with a syrinx were identified. Ten children had known genetic syndromes (2 who also had a syrinx). The 26 children without these comorbidities were considered idiopathic. Curve magnitude ranged from 50 to 106 degrees., Results: Nine of the 26 (35%) children in the children with idiopathic curves demonstrated resolution of their curves, while only 3 of the remaining 18 (17%) did. Of the children that did not have resolution of their curves, 14 were maintained over the entire follow-up period to within 15 degrees of their initial curve and 13 were improved 15 degrees or more. Only 5 children had an increase of 15 degrees or more over the follow-up period and 4 of these have undergone growth friendly instrumentation after a mean delay from initial cast of 71 months (range, 18 to 100 mo)., Conclusion: This study demonstrates that even in severe curves, casting was effective in delaying instrumentation in all cases, and led to curve resolution of the curves in 12 of 44 children., Level of Evidence: Level III-case control study.

Background: Percutaneous epiphysiodesis can be achieved using a single-portal or a double-portal technique. This study was performed to demonstrate any differences in outcomes, especially complications, between the 2 techniques., Methods: This was a retrospective review of cases at a single institution from 1983 to 2002 that yielded 336 children, in which 63 qualified for the study with at least 3 years of clinical follow-up. A comparison was performed through clinic chart review and radiographic measurement outcomes, searching for patient satisfaction, surgical time, and complications., Results: Minor complications included superficial infections, hematomas and effusions, whereas major complications included failure to arrest growth, partial arrest with angular deformity, fracture, and joint penetration. The single-portal group had an overall complication rate of 33.3%, with a major complication rate of 20% per patient. The double-portal group had a similar overall complication rate but only a 5.3% major complication rate per patient. There was no significant difference in patient demographics, operative times, or subjective complaints., Conclusions: Many methods of percutaneous epiphysiodesis exist in the literature that report low complication rates that are comparable with the complication rate of the original open procedure. Our study demonstrated a significantly higher rate of complications (both minor and major) compared with results previously reported. Moreover, the use of a single-portal approach increased the possibility of major complication by nearly 4-fold as compared with the use of a double-portal approach that avoids crossing the midline of the physis.

Background: Children with congenital spine disorders are known to have a high prevalence of congenital heart disease. The purpose of this work is to examine the presentation of these cardiac defects. It was our hypothesis that the anomalies are clinically obvious; thus, screening with electrocardiogram (ECG) or echocardiogram for occult disease would prove unnecessary after orthopaedic referral., Methods: The records and radiographs of 190 consecutive children known to have a congenital vertebral abnormality (excluding myelodysplasia and sacral agenesis) were retrospectively reviewed. Twenty-four children presented to the orthopaedist with a known cardiac anomaly. Of the remaining children, 64 underwent cardiac screening with echocardiogram, 27 underwent screening ECG and pediatric or cardiology evaluation, and 75 underwent evaluation by the orthopaedist only. Level of evidence was case series, level IV., Results: Twenty-four children presented to the orthopaedist with an established diagnosis of congenital heart disease. One child, who underwent echocardiogram, was detected to have a bicuspid aortic valve. This prevalence is consistent with what would be expected in screening the general population with echocardiogram. None of the 27 children screened with ECG and pediatric or cardiology consultation, and none of the 75 who underwent evaluation only by the orthopaedist were detected to have a cardiac abnormality. Also, no additional cardiac abnormalities have been found with follow-up of 2 to 17 years., Conclusions: The presentation of congenital heart disease associated with congenital spine disorders is general clinically evident and made before the referral to the orthopaedist. Routine referral by the orthopaedist for ECG or echocardiogram is not supported by this work.

This is a retrospective study of the functional status of children who underwent a lower extremity amputation for complications of myelodysplasia. With a computerized surgical database, 12 children with myelodysplasia who underwent an amputation at the Boyd level or above at a single children's referral hospital between 1983 and 2001 were identified. Four patients could not be contacted, but the remaining 8 patients were evaluated through chart review and interview to assess the impact of the amputation on their function. With a mean follow-up time of 9 years (range, 5-15 years), all 6 of the patients with a below-knee or Boyd amputation continued to ambulate using a prosthesis. Most patients occasionally reported having ulcers on their residual limb, but these cases were easily managed and did not result in amputation revisions.The only patient in this series with an above-knee amputation and the only patient with a knee disarticulation were exclusively wheelchair ambulators and no longer owned a prosthesis. This study supports the notion that children with myelodysplasia can have amputations and successfully wear a prosthesis to maintain their ambulation.

Twelve boys, aged 11-17 years, who underwent percutaneus proximal tibial osteotomy with acute angular correction and application of external fixator for unilateral Blount's disease were retrospectively reviewed. Preoperative radiographs were compared with radiographs at healing to evaluate changes in tibial length and overall limb length. Angular correction increased overall limb length by a mean of 1.4 cm (range -0.4 cm to 3.2 cm). This increase was a mean 0.7 cm less than was predicted by adding the preoperative tibial and femoral lengths. This failure to achieve the predicted limb length occurs due to shortening in the tibia and should be considered when planning an osteotomy.

This is a study of the time required to return to preoperative functional levels after proximal femoral osteotomy in children with cerebral palsy. Seventy-one consecutive children who underwent proximal femoral osteotomy to treat an unstable hip secondary to cerebral palsy are retrospectively reviewed. All children returned to their preoperative ambulatory function within 30 months of the procedure. Children who were community or household ambulators returned to their preoperative function at a mean of 7 months after osteotomy, while wheelchair and therapeutic ambulators required a mean of 10 months. Children who had regular visits with a licensed therapist tended to return to function more quickly than those who had exercises preformed by their parents or care-takers after instruction by a therapist. The authors conclude that families should be advised that rehabilitation after osteotomy requires on average 7-10 months, but times up to 30 months are possible.

This retrospective review of children surgically treated for King Type II or IV curvature of the spine required a minimum lumbar Cobb angle of 40 degrees and a minimum lumbar inclination (the angle formed between a line through the spinous processes of the three most caudal lumbar vertebrae and a line perpendicular to the floor) of 10 degrees. Twenty children had combined anterior thoracolumbar and posterior instrumentations whereas 20 had only posterior instrumentation. Children who had combined surgery had significantly better corrections of their lumbar Cobb angles. They had a mean correction of 43.3 degrees compared with 26.7 degrees in children with posterior instrumentation only. These superior corrections of the lumbar Cobb angles did not result in significantly better improvements in the lumbar inclinations. Patients who had the combined procedures had a mean improvement of 10.1 degrees, whereas patients who had posterior instrumentation only had a mean improvement of 8.0 degrees in lumbar inclination. Instead of having superior corrections of the lumbar inclinations, the combined surgeries resulted in a significant worsening of the angle between the end plates of the last instrumented vertebra and the next most caudal end plate. In patients who had combined surgery this angle averaged 8.4 degrees, whereas in patients who had posterior instrumentation only this angle averaged 4.1 degrees.

This study was conducted to evaluate the risk factors for epiphyseal changes suggestive of osteonecrosis after proximal femoral osteotomy for hip subluxation associated with cerebral palsy. Forty-eight children with 94 hips were reviewed. Two observers rated the radiographs using a written protocol on two occasions each so that reproducibility of these observations could be assured. Concomitant pelvic osteotomy proved to have the greatest association with risk of epiphyseal changes. These findings, suggestive of osteonecrosis, were present in 7 of 68 (10%) hips that had isolated femoral osteotomy, and in 12 of 26 (46%) hips that had concomitant pelvic osteotomy.

The predictive value of intraoperative radiographic assessment and its correlation with short term revision rates is presented for surgical correction of resistant clubfoot. Seventy-three children (115 feet) who underwent a primary procedure at one institution for idiopathic clubfoot between January 1991 and December 1994 were reviewed. A strategy using sequential release guided by intraoperative radiographs was employed in all cases. The intraoperative radiographic findings correlated with the need for early revision surgery, with residual radiographic evidence of cavus deformity associated with the greatest risk for revision. The sequential release strategy resulted in fewer overcorrections compared with historical controls of more complete subtalar release.

This is a 2-year prospective observational study of fractures occurring in residents of a live in center for developmentally disabled adults that is designed to identify risk factors that predispose to injury in this group. Fifty-eight individuals sustained 67 fractures during the study period. Only 18 of the 67 (27%) fractures were witnessed by the healthcare staff. Most fractures were diagnosed by subtle findings of erythema or swelling or by a change in the patient's behavior. Thirty-three of 332 (10%) community ambulators compared with 25 of 103 (24%) less functional residents incurred fractures. This difference is highly significant. Thirty-one of the 58 (53%) patients who sustained fractures during the study had a history of fracture before the study period. Thus, programs to reduce fractures should focus on residents with previous fractures and those with more severe disabilities.

Seventy-nine consecutive children with cerebral palsy who underwent osteotomies about the hip for subluxation or dislocation were studied retrospectively to determine risk factors that would correlate with postoperative complications of death, fracture, or decubitus ulcer. Except for the three patients who died, all of the children had > or = 1 year of follow-up. Twenty (25%) patients had at least one complication. Three children died; one at 1 week, one at 2 weeks, and one at 5 months after surgery. Sixteen patients sustained 25 fractures. All were managed with cast or splint immobilization in the clinic. Five patients developed decubitus ulcers requiring > or = 2 weeks of local care, but none required skin grafts or flaps. Complications occurred in 13 (68%) of 19 children with gastrostomies or tracheostomies but in only seven (12%) of the remaining 60 children. Only one (8%) of 13 ambulatory patients had a complication compared with 19 (29%) of 66 nonambulatory patients. In conclusion, ambulatory function correlates well with the risk of complications after osteotomies. A nonambulatory patient with a gastrostomy or tracheostomy is at even greater risk. Fortunately the fractures and ulcers observed in this series healed uneventfully with no operative intervention.

Post-Harrington techniques for adolescent idiopathic scoliosis have increased the complexity of scoliosis surgery while contributing only biomechanically more secure fixation and sagittal contour preservation. On the the basis of principles defined by Harrington, the authors developed a simplified technique that accomplishes equivalent outcomes.

A meta-analysis of the acute correction outcomes in adolescent idiopathic scoliosis is reported. Posterior instrumentation systems generally gave similar coronal plane corrections with average corrections for differing systems ranging from 48% to 67%. There was significant overlap of corrections reported for each of the systems. Anterior instrumentation gave better results with average corrections ranging from 71% to 93%. No instrumentation demonstrated a consistent ability to restore a normal thoracic kyphosis, but all seemed to maintain preoperative kyphosis measures. All systems, including newer multihook systems, showed a small loss of lumbar lordosis postoperatively. The available data demonstrates that no posterior implant produces vertebral derotation.

The short-term outcomes of a new distraction technique using a small rod spinal instrumentation system (WRIGHTLOCK) are reported for adolescent idiopathic scoliosis. No caudally directed thoracic hooks were used. Seventy-four children aged 11.7 to 17.5 years who underwent consecutive posterior instrumentations are reviewed. Their curves improved from a mean of 55.6 degrees to 22.7 degrees (average of 59.2% correction). At 2.7 years mean follow-up, coronal curves lost an average of 4.2 degrees correction. Overall, there was a mean increase of 4.4 degrees in kyphosis. Children with the most preoperative hypokyphosis were afforded the greatest gains in kyphosis. Instrumentation resulted in a mean loss of 8.2 degrees lumbar lordosis. These results compare favorably with published results for other multihook systems.

We performed a retrospective review of the medical records and radiographs of fifty children who had had unilateral slipped capital femoral epiphysis. Our purpose was to determine if there were any epidemiological parameters that were associated with the later development of a contralateral slip. The mean duration of follow-up was thirty-four months (range, twenty-four to sixty-eight months). Physiological maturity, determined with a modified form of the Oxford method for assessment of bone age, strongly correlated with the risk of development of a contralateral slip in patients who were initially seen with a unilateral slip (r = 0.59, p < 0.0005). The scores were determined by four raters; the variation in the predictive value of the scores among the raters was not significant (p = 0.5). The relationship between the score as determined with the modified Oxford method and the risk of contralateral involvement had a linear distribution. The four raters performed a total of 442 observations to determine the modified Oxford scores for the fifty hips. The prevalence of a subsequent slip for the hips that had been assigned a score of 16 points was 85 +/- 15 per cent (95 per cent confidence limit) (seventeen of twenty observations). The risk of contralateral involvement when the score was 21 points was 11 +/- 9 per cent (six of fifty-four observations). A slip did not develop in any hip with a score of 22 points or more (sixty-nine observations). For boys, the age at the time of the initial slip was predictive of a contralateral slip. A contralateral slip developed in all four boys who had been eleven years and seven months old or less at the time of the initial presentation; however, a contralateral slip developed in only nine of the twenty-two boys who had been eleven years and eight months to fourteen years and eleven months old. A contralateral slip did not develop in the three boys who had been fifteen years old or more. There was no association between age and the risk of a contralateral slip in girls.

We managed thirty-three patients who had open injuries of the foot related to the use of a lawn mower from 1985 through 1992. Twenty-eight of the patients were male and five were female. They ranged in age from four to seventy-three years old. The injuries were associated with the use of push lawn mowers (twenty-two patients), riding lawn mowers (nine patients), and self-propelled lawn mowers (two patients). The injuries included forty open fractures, twenty amputations, eighteen lacerations of the skin and nail beds, nine lacerations of tendons, two closed fractures, segmental loss of bone in two patients, and segmental loss of the Achilles tendon in one patient. The findings on culture of intraoperative specimens revealed a mean of 3.1 organisms (range, one to nine organisms) per patient. All of the patients were managed with at least one operative procedure (mean, 2.4 operations; range, one to five operations), and all were treated with parenteral antibiotic therapy (mean, 2.3 antibiotics; range, one to six antibiotics) except for one patient who had oral antibiotic therapy. The mechanism of injury was documented for twenty of the twenty-two patients who had been injured by a push lawn mower. Seventeen patients were injured while pulling the push lawn mower backward, and eight of those patients had been pulling the lawn mower up a slope.(ABSTRACT TRUNCATED AT 250 WORDS)

Six consecutive patients with infected intra-articular fractures of the distal tibia were studied. They were treated with resection of all infected or necrotic bone, systemic antibiotics, and instrumentation with a small pin fixator (Ilizarov external fixator). The fixator was used to perform an ankle arthrodesis and to fill the defect created by bone resection with distraction osteogenesis. In all cases, the infections were eradicated, and a solid arthrodesis was attained. The patients required from zero to two revision procedures (average 1.3), and their time in the fixator varied from 3 to 13 months (average 8 months). All of the patients experienced at least minor complications during treatment (superficial pin tract infections). At final follow-up, no patient demonstrated shortening of more than 1.5 cm. One patient has an internal rotation deformity of 15 degrees; a second has a varus deformity of 10 degrees and occasionally uses lateral support (a cane) secondary to unsteadiness on uneven ground; and one patient uses aspirin occasionally for subtalar pain. All are pleased with their results and would undergo the same procedure again without reservation.