Anesthetic Management for a Child With a Newly Identified Mitochondrial Disease SLC25A46 Mutation: A Case Report.

SLC25A46 mutation is a newly recognized mitochondrial mutation causing neurological and muscular abnormalities. We describe a first-ever report of the anesthetic management of a seven-year-old boy with an SLC25A46 mutation during a major orthopedic procedure. The patient was nonverbal and presented with cerebral visual impairment, torticollis, and lower extremity contractures. Because of his new diagnosis of mitochondrial disease and history of delayed awakening after anesthesia, we performed general anesthesia with sevoflurane, a low-dose ketamine infusion, and small doses of fentanyl while avoiding propofol and maintaining normoglycemia and normothermia. No postoperative complications were noted during the recovery period.

Intrailiac Osteotomy With Superior Lateral Outcropping Bone: A Previously Undescribed Procedure for Hip Subluxation in Cerebral Palsy.

BACKGROUND: Surgical treatment of hip subluxation in cerebral palsy typically involves proximal femoral osteotomy with or without concurrent supra-acetabular pelvic osteotomy. The literature lacks data on isolated pelvic osteotomy for this condition. We present superior lateral outcropping bone as a novel procedure for augmenting pelvic osteotomies for additional femoral coverage. METHODS: In this retrospective case series, all patients were included for whom a single surgeon at a single institution performed pelvic osteotomy with adjunctive superior lateral outcropping bone for the treatment of hip subluxation in cerebral palsy over a 12-year period. Patients with less than two years of X-ray follow-up were excluded, as were patients with frank dislocation preoperatively. Regarding each case, multiple variables were collected, including X-ray measurements of migration percentage and acetabular index preoperatively, immediately postoperatively, and at last available X-ray. Paired t tests were performed to confirm a significant difference between preoperative and postoperative measurements. Surgical failure was defined as either any subsequent hip or pelvic procedure other than myotendinous lengthening or alcohol nerve blocks, or final migration percentage of greater than 50%. RESULTS: Thirty-three hips (23 patients, 13 males) were included. Mean age at surgery was seven years. Mean time to follow-up was 49 months. Migration percentage of the hips improved from an average 44% preoperatively to 25% at first postoperative measurement and 22% at final follow-up (p < 0.001). Acetabular index improved from an average 27 degrees preoperatively to 15 degrees at first postoperative measurement and 17 degrees at final follow-up (p < 0.001). No hips met failure criteria of repeat surgery other than myotendinous lengthening or nerve blocks, but two presented with a migration percentage of greater than 50% at final follow-up, giving us a failure rate of 6%. CONCLUSIONS: We present a novel procedure that appears to provide safe and successful outcomes for hip subluxation in cerebral palsy. Our clinical results compare favorably to those in the literature for isolated proximal femoral osteotomy for similar patient populations, yet there is no need for implanted hardware.

Minimally Invasive Adductor Release With Obturator Block for Hip Subluxation in Cerebral Palsy: A Report of Two Cases.

Cerebral palsy (CP) is the most common motor disability in childhood and presents with spasticity, increased tone, decreased range of motion, and difficulty with ambulation. Abnormal communication between the cerebrum and the motor fibers leads to functional deficits and long-term adverse sequelae. This case report focuses on hip dysplasia. Two children with CP who were 4.4 and 3.8 years at initial surgery had substantial hip dysplasia with migration percentages (MPs) by X-ray of 60 and 55 and Gross Motor Functional Classification System (GMFCS) levels of 4 and 5. Each patient underwent minimally invasive selective percutaneous myofascial lengthening (SPML) of the hip adductors and ethanol block of the obturator nerves, along with other indicated procedures. Follow-ups were four and six years for the two cases. Indications for surgery included adductor spasticity with contracture, brisk adductor reflexes, scissoring, and hip dysplasia. The goals were to relieve symptoms and to serve as temporizing measures prior to possible later hip reconstruction. Results showed that, in each case, the MP improved substantially. Case 1 was a child who initially took steps with assistance and became independent by age six, with GMFCS scores improving from 4 to 2. The MP improved from 60 to 35 over four years. Case 2 was a child of GMFCS 5 who could not stand or take steps. The MP improved from 55 to 25 over six years. In addition to the initial SPML surgery, he had a second SPML surgery 31 months later at age six. This case is noteworthy in that the child consistently used a hip abduction orthosis and an abducted wheelchair through the entire six-year follow-up period. In conclusion, some young children with a significant hip subluxation can achieve improvement following minimally invasive surgery at medium-term follow-up. Our two children each had special circumstances. One was more highly functioning and became an independent walker. The other had consistent use of a hip abduction orthosis and an abducted wheelchair.

Recurvatum of the Knee in Cerebral Palsy: A Review.

Recurvatum is defined as hyperextension of the knee in the stance phase of gait. Recurvatum knee is a naturally occurring common gait deviation in those with cerebral palsy, along with crouch knee, jump knee, and stiff knee gaits. Early and late recurvatum occur in the first and second halves of stance. Early recurvatum is associated with dynamic calf contraction that raises the heel and pushes the knee into hyperextension as the forefoot contacts the floor. Late recurvatum occurs after the foot is already flat on the floor. As the body weight comes forward over the foot, the tibia stops its forward motion too early as the ankle comes to its range-of-motion limit. The advancing body then moves forward over a hyperextending knee. Surgical hamstring lengthening can have recurvatum as a side effect. There are several strategies to decrease this risk. Medial hamstring lengthening may be safer than combined medial and lateral lengthening. The concept here is that less lengthening or less aggressive lengthening means less recurvatum risk. However, combined medial and lateral lengthening can be reasonably safe from the risk of causing recurvatum if the knee is showing enough preoperative flexion in stance to warrant the increased surgery. More flexion in stance relates to less risk, while less flexion in stance relates to more risk. Knee flexion in stance can be measured. This is done by measuring knee flexion at initial contact and knee flexion in stance in a gait lab or with stop-action video. If there is minimal knee flexion in stance, hamstring lengthening might not be advisable, even if the hamstrings are tight on popliteal angle testing. There are other factors that contribute to recurvatum risk, such as knee hyperextension on static exam, equinus contracture, and jump knee gait. For treatment of recurvatum, the mainstay is the use of ankle foot orthoses set in dorsiflexion. Surgical equinus correction in those with early stance recurvatum can be effective but it is not likely to be effective in those with late stance recurvatum.

Minimally Invasive SPML Surgery for Children with Cerebral Palsy: Program Development.

Improvements in surgical and rehabilitation care are critical to lessen the burden of cerebral palsy (CP), the most common cause of severe physical disability in childhood. The selective percutaneous myofascial lengthening (SPML) surgical procedure is a minimally invasive method designed to improve ambulation by lengthening contracted musculoskeletal tissues. Information on surgical procedures, efficacy, and safety of SPML for children with CP is lacking. Phase 1 of our research is a "proof-of-principle" study for multisite SPML to improve functional mobility of children with CP, and Phase 2 assesses safety, reoperation rates, and efficacy over time in subsequent patient series. Phase 1 was a repeated measurement case series study of 17 children (mean age 7.6 years). One physical therapist, blinded to the surgeon's measurements, measured bilateral knee and ankle motion before and after SPML procedures, using video recordings of a standardized gait path. Functional Mobility Scale (FMS) 5, 50, and 500 outcomes were taken pre- and postoperatively and via telephone follow-up. In Phase 2, multisite SPLM surgeries were implemented in larger successive cohorts from 2006 to 2017. Complications, reoperation rates, and efficacy were retrospectively analyzed. Phase 1 results showed improvement in the children's knee and ankle motion while ambulating and improved FMS 5, 50, and 500 outcomes postoperatively (mean, 6.3 months). At second follow-up (mean 33.3 months), FMS 500 scores continued improvement, while FMS 5 and FMS 50 scores maintained. During Phase 2, the complication rate was 2.4%, and reoperation rates (including reoperations due to maturation) were between 8% and 13%. Improvements to correct ankle equinus were recorded in 498 cases. In conclusion, in a specialized center, single-event, multilevel SPML surgeries of children with CP safely improved ambulatory knee and ankle angle motion and daily mobility outcomes. Future educational studies of training needs for surgeons new to the approach are needed.

The Effect of Cerebral Palsy Disease Severity, Socioeconomic Status, and Educational Background on Selective Percutaneous Myofascial Lengthening Reoperation Rates.

Cerebral palsy (CP) is a neuro-developmental disorder. Spastic CP is the most common type of CP and is characterized by contractures of the extremities. Selective Percutaneous Myofascial Lengthening (SPML) is a minimally invasive procedure practiced by a handful of physicians in the US, and it decreases contractures and increases the range of motion in individuals with spastic CP. This study wanted to examine if there was an association between CP severity, socioeconomic status, and reoperation rates. This study used electronic medical records (EMR) to include 626 patients with spastic CP who had surgeries between January 2006 and December 2012. The zip codes from the EMR were used to determine the inflation-adjusted mean income and educational qualification (a high school education or higher) of the community via the US Census Bureau. Disease severity before the initial surgery was determined by using the functional mobility scale in the EMR to compute the Gross Motor Function Classification System (GMFCS) level. Then the data was graphed and averages were taken for the reoperation versus the no-reoperation populations, and Student's t-tests were run to determine statistical significance. The data showed that communities with higher education and income tended to reoperate more often. The higher number of reoperations in affluent communities could mean that either more affluent communities are better educated and know the benefits of bringing their children back for reoperation or that they require further education about physical therapy after the initial surgery to decrease the incidence of reoperation. This retrospective study is a level 2 study looking at the socioeconomic and educational backgrounds and disease severity and their association with reoperation rates.

Elastomeric Pain Pumps for Scoliosis Surgery.

Objective  Postoperative pain management following scoliosis surgery has traditionally relied on intravenous opioids. The objective of this study was to evaluate the effect of elastomeric pain pumps. Methods A retrospective chart review of 81 adolescent patients who underwent scoliosis surgery in a seven-year period was performed. Patients were divided into three groups as the practice changed: (1) patient-controlled analgesia group (12 patients) who used intravenous (IV) opioids with oral opioids; (2) elastomeric pain pump group (28 patients) with the added use of bupivacaine; and (3) multimodal pain pump group (41 patients) with the added use of gabapentin and methocarbamol. Endpoints were analyzed for length of stay in the hospital, infection rate, and gastrointestinal retention. Results The length of stay in the elastomeric pain pump group was 3.1 days shorter than in the patient-controlled analgesia group (P = 0.004). The length of stay in the multimodal group was 3.9 days shorter than in the patient-controlled analgesia group (P = 0.001). The incidence of prolonged postoperative bowel retention decreased significantly from 25% to 18% to 2% (P = 0.03). Conclusions To our knowledge, this is the first study on the use of elastomeric pain pumps in conjunction with multimodal pain medication following scoliosis surgery. The use of elastomeric pain pumps was associated with clinically and statistically significant improvements in the postoperative course. The addition of methocarbamol and gabapentin was associated with a trend toward further improvements.

Acute Appendicitis After Spine Fusion for Adolescent Idiopathic Scoliosis: A Case Report.

Appendicitis is a common cause of pediatric abdominal pain, largely occurring in the second decade of life. We present the case of a 14-year-old girl who underwent an uncomplicated posterior spinal fusion with instrumentation for scoliosis, who later developed abdominal pain, nausea, and emesis secondary to acute appendicitis. Her hospital course was significant for prolonged intravenous use of narcotics for pain control and subsequent constipation but negative for abdominal pain or tenderness during her admission. While gastrointestinal complications are a common cause of unplanned 30-day readmissions in the pediatric population, appendicitis has yet to be reported. To our knowledge, this is the first case report of acute appendicitis after posterior spinal fusion, likely resulting from postoperative pain management.

Pre-surgery evaluations by telephone decrease travel and cost for families of children with cerebral palsy.

INTRODUCTION: Children with cerebral palsy need highly specialized care. This can be very burdensome for families, particularly in large rural states, due to the need for long-distance travel to appointments. In this study, children undergoing the selective percutaneous myofascial lengthening surgery utilized a telephone-based telemedicine evaluation to assess for surgical eligibility. The goal was to avoid a separate preoperative clinic visit weeks before the surgery. If possible, eligibility was determined by telephone, and then, the patient could be scheduled for a clinic visit and possible surgery the next day, saving the family a trip. The purposes of the study were to calculate estimated reductions in miles traveled, in travel expenses, and in carbon emissions and to determine whether the telephone assessment was accurate and effective in determining eligibility for surgery. METHODS: From 2010 to 2012, 279 patients were retrospectively reviewed, and of those, 161 mailed four-page questionnaire and anteroposterior pelvis X-ray followed by a telephone conference. Geographic information system methods were used to geocode patients by location. Savings in mileage and travel costs were calculated. From 2014 to 2015, 195 patients were additionally studied to determine accuracy and effectiveness. RESULTS: The telephone prescreening method saved 106,070 miles in transportation over 3 years, a 38% reduction with US$55,326 in savings. Each family saved an average of 658 (standard deviation = 340) miles of travel and US$343.64 (standard deviation = US$178) in travel expenses. For each increase of 10 miles in distance from the health center, the odds of a patient utilizing telephone screening increased by 10% (odds ratio: 1.101, 95% confidence interval: 1.073-1.129, p < 0.001). After the telephone prescreening, 86% were determined to be likely candidates for the procedure. For 14%, a clinic visit only was scheduled, and they were not scheduled for surgery. CONCLUSION: Families seeking specialized surgical care for their disabled children particularly benefited from this approach.

Spinal Deformities in Pediatric Burn Patients.

INTRODUCTION: Extensive third-degree burns in pediatric patients can be associated with the development of spinal deformities. METHODS: Forty pediatric burn patients with scoliosis and one with kyphosis were identified based on a retrospective review of patient records at the Shriners Hospital for Children in Galveston, Texas. RESULTS: Average age at time of burn was 7 years 4 months. Average age at follow-up was 11 years 10 months. Twenty-one patients had a thoracolumbar curve, with an average of 20 degrees. Sixteen patients had a thoracic curve, with an average of 16 degrees. Three patients had a lumbar curve, with an average of 9 degrees. Five patients had scoliosis greater than 40 degrees. The kyphosis was 90 degrees. All patients had contractile burn scar on the trunk on the concavity of the curve. DISCUSSION: Mild scoliosis curves are common following third-degree burns in the pediatric population, but extensive burns at a young age can cause severe curves. Risk factors include burn scar traversing the entire length of the trunk, young age, greater total body surface area burn, and greater percentage of third-degree burn. Soft tissue procedures and bracing may be used to treat flexible curves. Posterior stabilization may be needed for more severe curves.

The use of ultrasound to determine timing of Pavlik harness discontinuation in treatment of developmental dysplasia of the hip.

The timing of Pavlik harness removal in patients with developmental dysplasia of the hip is typically determined by clinical examination. Ultrasound is considered more sensitive than clinical examination in diagnosis of instability of the hip, but it is not routinely used in cases of developmental dysplasia of the hip, especially when determining the timing of Pavlik harness removal. The purpose of this study is to investigate if ultrasound examination is more effective than clinical examination alone in determining completion of Pavlik harness treatment. Fifty consecutive infants with developmental dysplasia of the hip were given both a clinical examination and dynamic ultrasound examination to determine if Pavlik harness treatment could be discontinued. A pediatric orthopedist conducted the physical examinations. Both a radiologist and a pediatric orthopedist interpreted the ultrasounds. Cost estimates for ultrasound and operative procedures were obtained at our institution and compared. The average age at the time of Pavlik harness placement was 5.3 days, and the average age at the time of ultrasound was 54.3 days. There were 35 females and 15 males and 31 left hips, 4 right hips, and 15 bilateral hips. All 50 patient hips were deemed clinically stable prior to obtaining an ultrasound. The ultrasound interpretations by a radiologist and pediatric orthopedist were in agreement in all cases. In one case, the ultrasound results did not correlate with results of the clinical examination. In that one case, the hip was clinically stable, but dynamic ultrasound revealed that the hip was located, but dislocatable. The Pavlik harness was reapplied to the patient for an additional 42 days. At 1-year follow-up the hip is stable and developing normally as determined by radiographs. The cost comparison revealed that the cost of 50 ultrasounds is less than the cost of a single operative procedure. The use of ultrasound to determine the timing of Pavlik harness cessation is justified from both a financial and a patient outcome perspective in this small study. Larger studies are needed before ultrasound examination used to help determine Pavlik cessation is considered standard of care.

Proximal humerus fractures sustained during the use of restraints in adolescents.

Injuries to skeletally immature individuals sustained during restraints have been rarely documented. The authors report a series of five proximal humerus fractures in skeletally immature patients (average age 15.3 years) that occurred during restraint with handcuffs. A similar mechanism of injury was identified in all of the cases. All five injuries were Salter-Harris type I or II, and three of the five required operative intervention. At short-term follow-up, all patients are doing well without evidence of growth disturbances. To the authors' knowledge, this is a unique mechanism of injury that could be avoided with proper education.

Valgus slipped capital femoral epiphysis.

Abnormally high stresses involving the capital femoral physis were found in a series of valgus slipped capital femoral epiphysis cases. Using a single leg stance model we studied 10 hips in seven patients in which the epiphysis was lateral and posterior in relation to the femoral neck and calculated that shear stresses were high enough to be associated with failure of the physis. Valgus neck shaft angles and lateral tilt of the physes were seen in all cases.

The angled buckle fracture in pediatrics: a frequently missed fracture.

Buckle (torus) fractures in childhood are very common, and most assume a typical configuration wherein the trabeculae across the fracture line are compressed and the corresponding cortex bulges outward (unilateral or bilateral). In other cases the fracture merely shows cortical angulation along one side of the bone and classical buckling is not present. These latter fractures frequently are subtle and easily overlooked. However, if one knows what they look like and where they are likely to occur, one is able to detect them with greater frequency and confidence. The purpose of this communication is to bring attention to this fracture, describe the mechanism by which it occurs, and indicate its most common sites.

Lateral femoral condylar shearing fractures.

Lateral femoral condylar shearing fractures occur as the aftermath of acute patellar dislocation. The fracture fragment may be larger than originally appreciated on plain films. Diagnosis depends on identifying the fracture fragment somewhere within the knee joint, and the "donor site" on the lateral femoral condyle. Therefore, MRI or arthroscopy should be performed to identify the true size of the fracture fragment. The larger of these fragments may require surgical intervention with fixing of the fracture fragment onto the lateral condyle with screws. We present the radiographic and clinical findings in seven patients.

Rectus and hamstring surgery in cerebral palsy: a gait analysis study of results by functional ambulation level.

The purpose of this study is to determine if children more severely involved with cerebral palsy respond as well to rectus transfer and hamstring surgery as those with less severe involvement. Ninety-nine children were classified as independent community ambulators, crutch/walker-dependent community ambulators, or household/exercise ambulators. Maximum knee extension in stance and total range of knee motion in gait increased following surgery in all groups. Peak knee flexion in swing was maintained in the independent group only, but timing of knee flexion in swing improved in all groups. All groups showed increases in stride length, and the household/exercise group also showed an increase in walking speed. Four of 39 crutch/walker-dependent community ambulators and 13 of 21 household/exercise ambulators progressed to the next higher functional ambulation group.

Foot progression angle after distal tibial physeal fractures.

Twenty-three patients with Salter 1 or 2 fractures of the distal tibia had follow-up with footprints to assess rotational deformities. Fourteen had external rotation deformity >+2 SD of the normal mean of 5 degrees to 6 degrees. Eight of the 14 had the finding of physeal widening of 2 mm or more. The widening was most commonly wedge-shaped and based anteriorly. The mean foot progression angle of the fractured sides was externally rotated 14 degrees, and the mean foot progression angle of the uninjured sides was externally rotated 10 degrees; both exceeded the normal mean. The fractured side mean was significantly more externally rotated than the uninjured side mean. Preexisting external rotation deformity may be a risk factor for these fractures. Closed reduction with long leg bent-knee casting is justified in more than half of these patients.