Protocol-driven early tracheal extubation in patients with flaccid neuromuscular scoliosis and pre-existing lung disease.

Purpose: To review the results of a postoperative respiratory pathway for patients with muscular dystrophy (MD) and spinal muscular atrophy (SMA) undergoing spinal surgery.

Methods: With IRB approval, a retrospective review was done on all patients with SMA and MD undergoing spinal surgery on a neuromuscular protocol. Baseline demographics, perioperative results, and long-term outcomes were collected.

Evolving Surgical Management for Early-Onset Scoliosis in Spinal Muscular Atrophy Type 1 Given Improvements in Survival.

Case: We present a case of an 18-month-old child with early-onset scoliosis in the setting of spinal muscular atrophy (SMA) type 1 whose rapidly progressive scoliosis is successfully managed with magnetic growing rods, the youngest age of implantation in a patient with SMA we are currently aware of. Technical challenges, complications, and outcome are described in this case presentation.

Conclusion: Patients with SMA type 1 and early-onset scoliosis can be managed with growing-rod constructs given dramatic improvements in medical care that have expanded life expectancy.

Customised hybrid CT-MRI 3D-printed model for grade V spondylolisthesis in an adolescent.

3D-printed patient-specific models provide added value for initial clinical diagnosis, preoperative surgical and implant planning and patient and trainee education. 3D spine models are usually designed using CT data, due to the ability to rapidly image osseous structures with high spatial resolution. Combining CT and MRI to derive a composite model of bony and neurological anatomy can potentially provide even more useful information for complex cases.

Immune Function following Major Spinal Surgery and General Anesthesia.

There are reported differences in the effects that general anesthetics may have on immune function after minor surgery. To date, there are no prospective trials comparing total intravenous anesthesia (TIVA) with a volatile agent-based technique and its effects on immune function after major spinal surgery in adolescents. Twenty-six adolescents undergoing spinal fusion were randomized to receive TIVA with propofol-remifentanil or a volatile agent-based technique with desflurane-remifentanil.

Impact of Intraoperative Fluid Management on Electrolyte and Acid-Base Variables During Posterior Spinal Fusion in Adolescents.

Introduction: Various isotonic fluids may be used to maintain intravascular homeostasis during major surgical procedures. Variations in the electrolyte and buffer concentrations between these solutions may result in differential changes in electrolyte and acid-base status during fluid resuscitation. This study evaluates these changes during posterior spinal fusion in adolescents.

Defining the volume of consultations for musculoskeletal infection encountered by pediatric orthopaedic services in the United States.

Objective: Adequate resources are required to rapidly diagnose and treat pediatric musculoskeletal infection (MSKI). The workload MSKI consults contribute to pediatric orthopaedic services is unknown as prior epidemiologic studies are variable and negative work-ups are not included in national discharge databases. The hypothesis was tested that MSKI consults constitute a substantial volume of total consultations for pediatric orthopaedic services across the United States.

Etiology and Treatment of Intraoperative Hyperkalemia During Posterior Spinal Fusion in an Adolescent.

Hyperkalemia, defined as a serum or plasma potassium greater than 5.5 mEq/L, while an uncommon occurrence in children, is a serious medical problem that warrants immediate attention as it can result in serious cardiac arrhythmias and death. Although hyperkalemia may occur in the critically ill patient or in the setting of renal failure and insufficiency, there are limited reports of its occurrence during intraoperative care.

Anesthetic Care During Posterior Spinal Fusion in an Adolescent With Ebstein's Anomaly.

Ebstein's anomaly is a rare form of cyanotic congenital heart disease (CHD) that involves malformation and dysfunction of the tricuspid valve and right ventricle (RV). The severity of the defect impacts clinical presentation, survival, and treatment options. Presentation during the neonatal period with hypoxemia and cyanosis is noted in patients with severe tricuspid valve malformation, a hypoplastic RV, or RV outflow tract obstruction.

The Utility of Preoperative Neuromonitoring for Adolescent Idiopathic Scoliosis.

Background: Intraoperative neuromonitoring is well established and widely used to assist in completing corrective surgery for adolescent idiopathic scoliosis (AIS) safely. The role of preoperative measurement of somatosensory evoked potentials (SSEPs) and/or transcranial magnetic stimulation (TMS) to determine if there is transpinal pathology, however, is not clear. We sought to determine if preoperative SSEP and/or TMS measurement provided clinical benefit to patients with AIS.

Changes in cerebral oxygenation based on intraoperative ventilation strategy.

Introduction: Cerebral oxygenation can be monitored clinically by cerebral oximetry (regional oxygen saturation, rSO) using near-infrared spectroscopy (NIRS). Changes in rSO have been shown to precede changes in pulse oximetry, providing an early detection of clinical deterioration. Cerebral oximetry values may be affected by various factors, including changes in ventilation.

Analgesic effects of methadone and magnesium following posterior spinal fusion for idiopathic scoliosis in adolescents: a randomized controlled trial.

Purpose: To provide optimal conditions for neurophysiological monitoring and rapid awakening, remifentanil is commonly used during pediatric spinal surgery. However, remifentanil may induce hyperalgesia and increase postoperative opioid requirements. We evaluated the potential of methadone or magnesium to prevent remifentanil-induced hyperalgesia.

Spinal anesthesia instead of general anesthesia for infants undergoing tendon Achilles lengthening.

Spinal anesthesia (SA) has been used relatively sparingly in the pediatric population, as it is typically reserved for patients in whom the perceived risk of general anesthesia is high due to comorbid conditions. Recently, concern has been expressed regarding the potential long-term neurocognitive effects of general anesthesia during the early stages of life. In view of this, our center has developed a program in which SA may be used as the sole agent for applicable surgical procedures.

Changes in Cerebral Oxygenation during Transfusion Therapy.

This study assesses the effects of transfusion of autologous or allogeneic blood on cerebral and tissue oxygenation during spinal surgery. Packed red blood cell transfusions are indicated to improve oxygen delivery to tissues. There are limited data demonstrating changes in tissue oxygenation with blood administration.

Intraoperative Anaphylactic Reaction: Is it the Floseal?

When hemodynamic or respiratory instability occurs intraoperatively, the inciting event must be determined so that a therapeutic plan can be provided to ensure patient safety. Although generally uncommon, one cause of cardiorespiratory instability is anaphylactic reactions. During anesthetic care, these most commonly involve neuromuscular blocking agents, antibiotics, or latex.

Pediatric Diaphyseal Femur Fractures: Submuscular Plating Compared With Intramedullary Nailing.

This study compared the radiographic and clinical outcomes of pediatric diaphyseal femur fractures treated by submuscular plating, flexible retrograde intramedullary nailing, or rigid antegrade intramedullary nailing with a trochanteric entry point in skeletally immature patients who were 8 years and older. A retrospective review was conducted of skeletally immature patients 8 years and older who were treated for femur fracture with submuscular plating, flexible intramedullary nailing, or rigid intramedullary nailing from 2001 to 2014 with a minimum 12-week follow-up. Treatment outcomes were compared for statistical significance, including time to union, malunion, nonunion, heterotopic ossification, avascular necrosis, time to full weight bearing, limb length discrepancy, residual limp, painful hardware, and infection.

Bridge Plating Length-Unstable Pediatric Femoral Shaft Fractures.

The treatment of pediatric diaphyseal femur fractures, particularly length-unstable fractures, continues to be an area of controversy in patients from age 6 to skeletal maturity. Submuscular bridge plating is an alternative that allows for stable internal fixation while minimizing soft tissue disruption. We describe a surgical technique that has simplified both implantation and removal.

Utilization of Orthopaedic Trauma Surgical Time: An Evaluation of Three Different Models at a Level I Pediatric Trauma Center.

Objective: Over the past decade, our institution has instituted three different scheduling models in an attempt to care for pediatric trauma at our Level I Trauma Center. This has been in response to a number of factors, including a limited number of physicians covering the call schedule, increasing competition for operating room (OR) time after hours (pediatric surgery, urology, neurosurgery), an attempt to fully utilize OR time during the daytime, fully staffed hours, and optimizing patients' timeliness to surgery. We examined the three on-call systems in place at our institution to determine whether a more flexible approach to pediatric trauma call resulted in delays in treatment.

Percutaneous Doxycycline Treatment of Juxtaphyseal Aneurysmal Bone Cysts.

Background: A recurrence rate of 19% to 23% has been reported in juxtaphyseal aneurysmal bone cysts (ABC) without en bloc resection or amputation. No percutaneous surgical techniques or drug treatments have demonstrated consistent bone healing with normal physeal growth and a recurrence rate of <19%. Doxycycline has properties that may make it an appropriate agent for percutaneous treatment of juxtaphyseal ABC in skeletally immature patients.

Traumatic, Posterior Pediatric Hip Dislocations With Associated Posterior Labrum Osteochondral Avulsion: Recognizing the Acetabular "Fleck" Sign.

Background: Traumatic dislocation of the hip is uncommon in the pediatric population. Concentric reduction is usually achieved by closed means. Open reduction may be needed if there is femoral head fracture, incarcerated fragment, or incomplete reduction due to soft tissue entrapment.

A prospective, open-label trial of clevidipine for controlled hypotension during posterior spinal fusion.

Objectives: Controlled hypotension is one means to limit or avoid the need for allogeneic blood products. Clevidipine is a short-acting, intravenous calcium channel antagonist with a half-life of 1 to 3 minutes due to rapid metabolism by non-specific blood and tissue esterases. To date, there are no prospective evaluations with clevidipine in the pediatric population.

Phenylephrine to Treat Hypoxemia during One-Lung Ventilation in a Pediatric Patient.

To improve surgical visualization and facilitate the procedure, one-lung ventilation (OLV) is frequently used during thoracic surgery. Although generally well tolerated, the ventilation-perfusion inequality induced by OLV may lead to a decrease in oxygenation and, at times, hypoxemia. Effective treatment algorithms and strategies are necessary for the treatment of hypoxemia during OLV to ensure that the technique can be continued without interruption and allow for completion of the surgical procedure.

A preliminary study of volatile agents or total intravenous anesthesia for neurophysiological monitoring during posterior spinal fusion in adolescents with idiopathic scoliosis.

Study Design: A prospective randomized controlled trial.

Objective: The purpose of this study was to prospectively compare the efficacy of neurophysiological monitoring during general anesthesia with either a total intravenous technique or with the volatile anesthetic agent, desflurane.

Summary Of Background Data: A total intravenous anesthetic technique is generally chosen when neurophysiological monitoring is used as it has been shown to facilitate such monitoring.

Successful Medical Therapy for Hypophosphatemic Rickets due to Mitochondrial Complex I Deficiency Induced de Toni-Debré-Fanconi Syndrome.

Primary de Toni-Debré-Fanconi syndrome is a non-FGF23-mediated hypophosphatemic disorder due to a primary defect in renal proximal tubule cell function resulting in hyperphosphaturia, renal tubular acidosis, glycosuria, and generalized aminoaciduria. The orthopaedic sequela and response to treatment of this rare disorder are limited in the literature. Herein we report a long term followup of a 10-year-old female presenting at 1 year of age with rickets initially misdiagnosed as vitamin D deficiency rickets.

Is there still a place for cast wedging in pediatric forearm fractures?

Background: Forearm fractures are common skeletal injuries in childhood and can usually be treated nonoperatively with closed reduction and casting. Trends toward increasing operative treatment of these fractures have emerged. We aim to demonstrate the safety and efficacy of cast wedging for treatment of pediatric forearm fractures.