The orthopaedic manifestations and management of children with Stuve-Wiedemann syndrome.

Stüve-Wiedemann syndrome is an autosomal-recessive disorder characterised by bowing of the long bones, progressive scoliosis, episodic hyperthermia and respiratory distress, usually resulting in death in infancy. We reviewed five children with the condition who had been followed since birth and who survived into childhood with a mean age at operation of 7.8 years (5 to 14). There was marked functional impairment with dysplasia of the long bones and scoliosis. Treatment of the triplanar deformities of the femora involved the use of the Ilizarov technique with the Taylor Spatial Frame. Walking was preserved and improved in three children along with considerable enhancement of the appearance. Early insertion of a growing rod to control the progressive juvenile scoliosis was beneficial. The use of the Taylor Spatial Frame is strongly recommended to address the major complex deformities of the lower limbs which are encountered in this condition and to prevent their progression.

Resolution of severe, adolescent-onset hypophosphatemic rickets following resection of an FGF-23-producing tumour of the distal ulna.

Oncogenic hypophosphatemic osteomalacia (OHO) is an uncommon hypophosphatemic syndrome characterized by bone pain, proximal muscle weakness and rickets. It has been postulated that OHO results from overproduction of a humoral phosphaturic factor by an occult tumour. Recently, some OHO tumours have been shown to elaborate fibroblast growth factor-23 (FGF-23), which causes renal phosphate wasting when administered to mice. The purpose of this study was to undertake detailed investigations to confirm the diagnosis of OHO in a pediatric patient and to document the biochemical, radiographic and bone histological phenotype before and after tumour removal. We describe an 11-year-old, previously healthy girl with significant pain and functional disability associated with hypophosphatemic rickets. Circulating 1,25-(OH)(2) vitamin D was very low (14 pM; N: 40-140) while the FGF-23 serum level was markedly elevated [359.5 reference units (RU)/ml, N: 33-105]. An iliac bone biopsy revealed severe osteomalacia, but periosteocytic lesions, as are typical for X-linked hypophosphatemic rickets, were not seen. Sequence analyses of the PHEX and FGF23 genes were normal. A radiographic skeletal survey revealed a small exostosis of the left, distal ulnar metaphysis. A tumour was subsequently removed from this site and the pathology was consistent with benign, fibro-osseous tissue. Serum FGF-23 was normal when measured at 7 h post-operatively, while serum phosphate reached the low-normal range at 16 days following surgery. An iliac bone biopsy taken 5 months after the operation showed improvement, but not yet resolution, of the osteomalacia. Biochemical parameters of bone and mineral metabolism suggested that complete resolution of the osteomalacia was not achieved until 12 months following surgery. One year after tumour removal, the patient was pain-free and had resumed a normal level of activity. The rapid normalization of FGF-23 levels following removal of a benign tumour and the subsequent improvement in the biochemical and histological parameters of bone and mineral metabolism suggest that FGF-23 played a key role in this girl's disease.

Differentiation of psoas muscle abscess from septic arthritis of the hip in children.

A 20-year review was conducted of children presenting with psoas abscess at two major pediatric hospitals. Eleven children with psoas abscesses were identified. The extreme variability in the clinical presentation of this condition is shown. Psoas abscess was most difficult to differentiate from septic arthritis of the hip in pediatric patients. This study also shows the often circuitous investigative route traversed before arriving at the diagnosis of psoas abscess. Atypical features, such as femoral nerve neurapraxia or bladder irritability in association with hip pain, should alert the clinician to consider psoas abscess. Based on this study, a diagnostic algorithm to differentiate between psoas abscess and septic hip was formulated.

Effects of a tensioned tendon graft in a bone tunnel across the rabbit physis.

Children who sustain anterior cruciate disruption often are denied the standard reconstructive procedures because of the concern that drilling across the physis of the tibia and femur and compression from a tensioned graft will result in growth plate arrest. To test this concept and to assess whether a tendon placed in the tunnel would function in a manner similar to a fat graft after the resection of a physeal bar, tunnels were made across the proximal tibial physis and distal femoral physis in a group of immature rabbits. Four tunnel diameters were used from 1.95 to 3.97 mm, in three rabbits at each diameter, with patellar tendon autografts being used as the reconstruction of the anterior cruciate ligament in two of the animals. The knees were radiographed every 4 weeks, and the animals were euthanized 4 months after surgery. The surgically treated and control knees were salvaged, and each knee was examined grossly, radiographically, and histologically. Eight of the 11 animals had growth arrest of one or both physes. The larger the drill hole diameter the more marked was the deformity. The proximal tibial physis seemed to be the most vulnerable for growth arrest, occurring in eight of the knees. The insertion of a tendon did not seem to offer any protection to physeal arrest. Because of these findings, it is not recommended that tunnels involving 1% or more of the area of the physis be placed across the tibial and femoral physis to reconstruct the anterior cruciate in very skeletally immature children.

Trauma to the thoracic and lumbar spine in the adolescent.

During adolescence the spine undergoes rapid growth and changes in anatomy and biomechanical properties. Fractures of the adolescent spine are relatively uncommon but may give rise to serious problems. This review describes thoracolumbar fractures in adolescents with respect to epidemiology, anatomy, mechanisms of injury, clinical and radiologic assessment, and nonoperative and operative treatment. The treatment of these injuries follows many of the same principles as spinal fractures in adults but nonoperative treatment is used more frequently because there is less spinal instability and better tolerance of bed rest and spinal immobilization in this young population.

In-line skating injuries in children: a 10-year review.

In-line skating is an increasingly popular recreational activity for both children and adults. As with any high-speed sport, traumatic injury is an associated hazard. It was the purpose of this study to focus on the injuries in children associated specifically with in-line skating with an emphasis on education and prevention. During a 9-year period, 331 injuries were identified, with 105 families completing an in-depth epidemiologic questionnaire. Sixty-one percent of the victims were boys, with an overall average age of 12 years. The upper extremity was most commonly injured (64%); the head and neck area was injured in 16% and the lower extremity in 20%. Fractures represented 38% of all injuries, with inexperience appearing to be the most common cause. An analysis of preventive strategies for childhood in-line skating was undertaken using Haddon's 10 basic preventive principles. Educational programs regarding instruction in in-line skating and the value of protective equipment need to be part of the school curriculum.

Patent foramen ovale and brain microembolization during scoliosis surgery in adolescents.

STUDY DESIGN: A case series is reported. OBJECTIVE: To improve understanding of the potential mechanisms associated with cerebral microemboli during scoliosis surgery in adolescents. SUMMARY OF BACKGROUND DATA: Paradoxical cerebral fat microembolization during scoliosis surgery has been associated with right-to-left shunting through an undetected patent foramen ovale. The prevalence of this cardiac defect in the adult population may be as high as 15% to 25%. Although the clinical relevance of this embolic phenomenon during scoliosis surgery has not been investigated, a few reports have documented its fatal consequences. It has been suspected in some patients with postoperative reduced visual function, particularly after complex instrumented fusions. METHODS: Bilateral transcranial Doppler (2 MHz) was used to monitor cerebral microemboli in the right and left middle cerebral arteries during spinal instrumentation and fusion in four patients. Additionally, transthoracic or transesophageal echocardiography incorporating pulse and color Doppler (3.5-5 MHz) and a contrast test was used in these patients to detect atrial shunts. RESULTS: Intraoperative transcranial Doppler monitoring detected high rates of cerebral microemboli associated with the presence of an atrial communication in two adolescents. In two additional patients with no detected brain microembolization, echocardiographic examination indicated the absence of an atrial shunt. CONCLUSION: The study findings suggest that unrecognized atrial communications in adolescents undergoing scoliosis surgery contribute to higher rates of Doppler-detected cerebral microemboli than those found in adolescents without this congenital defect.

Cerebral microembolization during pediatric scoliosis surgery: a transcranial doppler study.

SUMMARY: The goal of this study was to identify cerebral microemboli during scoliosis surgery and their potential relationship with visual alterations. Transcranial Doppler identified high-intensity transient signals (HITS) during surgery in both middle cerebral arteries, and ophthalmologic examination assessed their potential effects on the visual system. Thirteen children (age 13-17 years) undergoing surgery for scoliosis or kyphosis with spine curvature >45 degrees were studied. HITS were identified in 92%. Eleven patients had a total count of <15 HITS, but in the remaining two the count was unexpectedly high (63 and 265 HITS). Echocardiography in these two patients indicated the presence of an atrial right-to-left shunt. Uneventful preoperative and postoperative visual function was found in 11 patients. One patient had preoperative blindness and in another ophthalmologic complications developed not related to microembolization. Scoliosis surgery is frequently associated with low counts of cerebral microemboli. It appears that such low embolic counts have no effects on postoperative visual function as determined clinically. Some patients may show high rates of microemboli, which may be related to the presence of right-to-left cardiac shunts. The impact of these signals on brain function remains to be investigated.

Analysis of the efficacy of pediatric day surgery.

OBJECTIVES: To assess the efficacy and safety of a pediatric day surgery program and its benefits to the child and family. DESIGN: A review and analysis of prospectively gathered data. SETTING: The Children's Hospital of Eastern Ontario (CHEO), a university-affiliated pediatric centre with a referral base of 2.5 million people. PATIENTS: All children undergoing day surgery at CHEO during the 5 years between 1992 and 1997. INTERVENTIONS: All surgical procedures performed on a day surgery basis. MAIN OUTCOME MEASURES: Procedures performed, complications and unexpected admissions. RESULTS: An average of 4,899 children per year underwent surgical procedures in the ambulatory day surgery program. The 4 commonest procedures performed were myringotomy, tonsillectomy and adenoidectomy, dental procedures, and inguinal hernia repair. The majority of children were between 2 and 7 years of age. Complications were few, averaging 1.6% per year, with postoperative bleeding, primarily secondary to tonsillectomy and adenoidectomy, being the most common. None resulted in permanent disability and there were no deaths. CONCLUSIONS: Pediatric day surgery is a safe and cost-effective program that benefits the child, the family and the pediatric surgical program.

An analysis of burst fractures of the spine in adolescents.

Burst fractures are less common in children than in adults because of the greater mobility and elasticity of the pediatric spine. Because of these spine characteristics, these fractures may behave differently in childhood than in adulthood. To try to address these differences, we reviewed our experience with 11 children (5 boys, 6 girls) treated for burst fractures. Average age at time of injury was 14.4 years. Follow-up averaged 9 years. All fractures were categorized using the Denis classification system for burst fractures. Three children had associated spinal cord injury. Five children were treated with nonoperative bedrest and casting; the other 6 children, who had the most severe burst fractures, were treated with posterior spinal fusion and instrumentation. Satisfactory functional results were found in 90% of the children at follow-up. Radiological evaluation at initial and follow-up examination showed that children treated operatively improved or maintained their fracture kyphosis (range, 12 degrees - 19 degrees). Anterior vertebral compression improved an average of 15% (range, 24%-39%). In the children treated nonoperatively, kyphosis progressed an average of 9 degrees (range, 15 degrees - 24 degrees), and anterior vertebral compression increased a further 8% (range, 36%-44%). Our results showed that (a) the children who sustained burst fractures tended to develop mild progressive angular deformity at the site of the fracture, (b) operative stabilization prevented further kyphotic deformity as well as decreased the length of hospitalization without contributing to further cord compromise, and (c) nonoperative treatment of burst fracture was a viable option in neurologically intact children, but progressive angular deformity occurred during the first year after the fracture.

The adolescent pilon fracture: management and outcome.

Pilon fractures in the adolescent are complicated by the presence of the adjacent physis. These fractures usually result from high-energy trauma, frequently associated with soft-tissue trauma, further potentiating treatment difficulties. Although rare, such fractures are associated with a high complication rate, including physeal arrest. It was the objective of this review to increase awareness of this fracture pattern in the adolescent, to determine the types of complications in this difficult group, and to develop a treatment plan to improve the outcome of treatment. Seven children, with a total of eight pilon fractures were treated at a major pediatric tertiary referral center over the past 10 years. The average age of the children was 15 years 10 months (range, 13 years 6 months to 17 years 7 months). The average length of follow-up was 16 months (range, 3 months and 3 years 10 months). There were three Reudi type II equivalent fractures and two Reudi type III equivalent injuries. Three fractures did not fit the Reudi classification system as there was an associated ankle dislocation. All fractures were treated with open reduction and internal fixation. There were two cases of posttraumatic osteoarthritis and one physeal arrest. Results were good to excellent in 63% of cases. A new classification system for pediatric pilon fractures has been proposed.

Snowboarding injuries in children.

OBJECTIVE: To study injury patterns of snowboarding trauma in children. DESIGN: A retrospective case series. SETTING: A major pediatric trauma centre. PATIENTS: A cohort of 113 children (97 boys, 16 girls), mean age 13.9 years, who sustained 118 injuries secondary to snowboarding. INTERVENTIONS: All fractures were treated by immobilization in a cast; 19 children required a closed reduction. OUTCOME MEASURES: Sex and age of patients, mechanism of injury, injury sustained, treatments and complications. RESULTS: Falls on the outstretched hand from a loss of balance accounted for 66 (57%) of the injuries, uncontrolled falls for 42 (36%) and collisions for 5 (4%). Seventy-nine percent of the injuries were to the upper extremity, whereas 7% were to the lower extremity. CONCLUSIONS: The predominance of snowboarding injuries of the upper extremity seen in children differs significantly from those in adults in whom lower extremity injuries are more common.

Children in crashes: mechanisms of injury and restraint systems.

OBJECTIVES: To explore the levels of protection offered to children involved in motor vehicle collisions. DESIGN: A joint study by the Children's Hospital of Eastern Ontario (CHEO) and Transport Canada, Ottawa, conducted in 2 phases: retrospective from 1990 to 1997 and prospective from 1998 to 2000. SETTING: CHEO, a university affiliated tertiary care centre. PATIENTS: Children admitted to CHEO between 1990 and 2000 with spinal trauma due to motor vehical crashes (MVCs). Phase I of the study involved analysis, in a series of 45 children after MVAs, by location of spinal injury versus belt type. Phase 2 was a prospective study of 22 children injured in 15 MVAs. INTERVENTIONS: A biomechanical assessment of the vehicle and its influence on the injuries sustained. MAIN OUTCOME MEASURES: The nature and extent of the injuries sustained, and the vehicle dynamics and associated occupant kinematics. RESULTS: The odds ratio of sustaining a spinal injury while wearing a 2-point belt versus a 3-point belt was 24 (95% confidence interval 2.0-2.45, p < 0.1), indicating a much higher incidence with a lap belt than a shoulder strap. CONCLUSIONS: Proper seat-belt restraint reduces the morbidity in children involved in MVCs. Children under the age of 12 years should not be front-seat passengers until the sensitivity of air bags has been improved. Three-point pediatric seat belts should be available for family automobiles to reduce childhood trauma in MVCs.

Subcutaneous granuloma annulare of the extremities in children.

OBJECTIVE: To familiarize surgeons to the natural history and treatment of granuloma annulare of the extremities in children. DESIGN: Case review and follow-up by appointment and questionnaire. SETTING: The Children's Hospital of Eastern Ontario, a tertiary referral centre. PATIENTS: Twenty-seven children (23 girls, 4 boys) seen between 1983 and 1998 with subcutaneous granuloma annulare, proven pathologically. The mean age at initial presentation was 8.3 years (range from 1.8 years to 16.7 years). The mean follow-up was 45 weeks (range from 1 week to 5 years). INTERVENTION: Biopsy excision of the lesions. OUTCOME MEASURES: The incidence multiple lesions, the commonest site of occurrence and the incidence of recurrence. RESULTS: Six children presented with multiple lesions for a total of 46 lesions. The anterior pretibial area was the most frequently affected site (16 lesions), followed by the ankle (6 lesions) and the long finger (4 lesions). Five patients suffered recurrence of the lesion, with a total of 7 lesions recurring. CONCLUSIONS: Subcutaneous granuloma annulare is a benign inflammatory skin lesion occurring most frequently in the anterior pretibial area in children, predominantly girls. The incidence of recurrence and of multiple lesions is high. Biopsy is required for definitive diagnosis. The lesion resolves spontaneously without treatment.

An analysis of a preoperative pediatric autologous blood donation program.

OBJECTIVE: To determine the efficacy of a pediatric autologous blood donation program. DESIGN: A retrospective study of patient charts and blood-bank records. SETTING: The Children's Hospital of Eastern Ontario, Ottawa, a tertiary care, pediatric centre. PATIENTS: One hundred and seventy-three children who received blood transfusions for a total of 182 procedures between June 1987 and June 1997. INTERVENTIONS: Autologous and homologous blood transfusion required for major surgical intervention, primarily spinal fusion. MAIN OUTCOME MEASURES: Surgeons' accuracy in predicting the number of autologous blood units required for a given procedure, compliance rate (children's ability to donate the requested volume of blood), utilization rate of autologous units and rate of allogeneic transfusion. RESULTS: The surgeons' accuracy in predicting the number of autologous units required for a given procedure was 53.8%. The compliance rate of children to donate the requested amount of blood was 80.3%. In children below the standard age and weight criteria for blood donation the compliance rate was 75.5%. The utilization rate of autologous units obtained was 84.4% and the incidence of allogeneic transfusion was 26.6%. CONCLUSIONS: There was a high rate of compliance and utilization of predonated autologous blood in the children in the study. Preoperative blood donation programs are safe and effective in children, even in those below the standard age and weight criteria of 10 years and 40 kg.

Avascular necrosis of the radial head in children.

Avascular necrosis of the radial head is uncommon. A 20-year review of records at a Pediatric Trauma Center revealed eight cases of avascular necrosis of the radial head in children with an average of 10 years (range, 7-16 years). Symptoms of pain or restriction of elbow motion began at an average of 23 months after trauma in seven cases, and in one case, there was no history of trauma. Of the seven secondary to trauma, six resulted from a fall, and one from a torsional injury. The types of injuries associated with avascular necrosis of the radial head were two supracondylar fractures, a fracture of the lateral condyle, two fractures of the radius, a radial head dislocation that underwent a proximal radial osteotomy, and one with only an elbow contusion. The long-term results of radial head necrosis were poor, with three ultimately requiring radial head resection, and the remainder having considerable limitation of elbow motion. Loss of vascularity to the radial head is very analogous to avascular necrosis of the femoral head, with the late onset of incongruity, osteoarthritis, and hypertrophy of the head. It should be considered in any child with late-onset loss of elbow motion after trauma.

Childhood rhabdomyosarcoma of the trunk and extremities.

Since 1979, 15 children with rhabdomyosarcoma have been treated at our institution. Included in this group are six children who presented with rhabdomyosarcoma of an extremity or trunk, requiring the use of combined multimodality therapy. The patients were clinically grouped and treated in accordance with the Intergroup Rhabdomyosarcoma Study protocol. All patients received combination chemotherapy based on their respective stage of disease at diagnosis. When feasible, the primary tumor was resected en bloc before chemotherapy was begun. After surgery, patients with unclear postoperative surgical margins and an initial good response to chemotherapy received radiotherapy to the primary site and at the regional lymphatics. Three of six patients developed or maintained a complete tumor response to induction chemotherapy. Radiotherapy maintained control of local disease in both groups. Overall, four patients, including one with disseminated disease at diagnosis, are alive, with a median survival time from diagnosis of 39 months. In children, treatment must be individualized, but complete local excision of the tumor with a tumor-free margin should be the goal. Major ablative amputation surgery was not performed.

Congenital synspondylism: diagnostic features.

Congenital synspondylism is a recently described condition characterized by short stature, an unusual constellation of vertebral fusions without rib anomalies and carpal coalition. A new case is described to illustrate the diagnostic features and the orthopaedic problems associated with this rare syndrome. Review of available literature shows that patients with congenital synspondylism commonly develop spinal deformity and mild respiratory compromise.

Sickle cell disease of the spine in children.

OBJECTIVE: To determine the incidence of back pain in children admitted with sickle cell disease so as to increase awareness of this disease in black children presenting with back pain or discomfort. DESIGN: A retrospective review. SETTING: The Children's Hospital of Eastern Ontario in Ottawa, a tertiary care, university affiliated centre. PATIENTS: Thirty children were treated for active sickle cell disease between 1990 and 1996. Eleven (5 boys, 6 girls) suffered vascular-occlusive phenomena occurring in bone, referred to as "bony crises," requiring a total of 49 admissions. MAIN OUTCOME MEASURES: Clinical manifestations of spinal involvement by sickle cell disease. MAIN RESULTS: The spine represented the second most common area of bone involvement (26%) exceeded only by the knee (35%). The vertebral level affected was lumbosacral in 66% of cases, followed by thoracic in 22% and cervical in only 12%. Eighty-six percent of the children with spinal pain were anemic upon presentation, 71% had an elevated leukocyte count, 15% were hyponatremic and 15% were hyperkalemic. Minimal physical signs in the spine were noted, other than a local tenderness over the spinous process in 71% and a decreased range of back motion in 17%. CONCLUSIONS: Sickle cell disease is becoming more common in Canada as a result of increasing immigration from African countries and should always be considered as a possible cause of back pain in a black child.