British Columbia Children Research Articles

Pediatric lumbar Chance fractures in British Columbia: Chart review and analysis of the use of shoulder restraints in MVAs

Chance fractures of the skeletally immature spine classically occur in frontal motor vehicle accidents (MVAs) when the occupants are restrained by a lap belt only and undergo traumatic hyperflexion of the torso during the impact. We retrospectively examined all MVA-related Chance fractures at British Columbia's Children's Hospital since 1986, by collecting injury and seat-belt use information from chart data and imaging studies. Twenty-six patients were included in the study, 14 wore a lap belt only, seven wore a three-point restraint properly, and five were reportedly misusing the shoulder portion of a three-point restraint. The subjects ranged in age from 3 to 16 with a mean age of 10.6 years. Eleven of the 26 (42%) patients sustained abdominal viscera injuries, seven of the 26 patients suffered neurologic injury (spinal cord and/or spinal nerve injury) associated with their spinal fracture, with two cases of complete paralysis, and there was a 38% incidence of head injury. Concomitant injuries (i.e. to the head, abdomen and abdominal contents) tended to be mitigated by the presence of a properly worn shoulder restraint. This leads to the conclusion that Chance fractures can be sustained even when the occupant is using a shoulder belt to restrain their torso. The mechanism responsible for this is unknown. This may indicate that Chance fractures can be caused by a lesser degree of torso hyperflexion than previously thought. Alternatively, we also speculate that Chance fractures can occur while the torso is restrained by the shoulder belt if the hips submarine beneath the lap belt and the torso experiences hyperflexion secondary to forward excursion of the pelvis and legs during the collision. Future work is necessary to confirm these mechanisms and to find ways to prevent them. These studies will need to use computational or experimental child surrogates that can sit in a slouched posture and submarine during a collision.

Severe retinopathy of prematurity and visual outcomes in British Columbia: a 10-year analysis

To describe the incidence trend and long-term visual outcomes of infants diagnosed with stages 3 to 4 retinopathy of prematurity (ROP) or laser-treated ROP born in British Columbia (Canada). Data from all (n=1384) neonates with birth weight (BW) <1250 g, admitted to British Columbia Children's Hospital between period 1 (January 1992 to December 1996) and period 2 (January 1997 to December 2001) were analyzed. Ophthalmologic records of infants with stages 3 to 4 ROP or laser-treated ROP were abstracted. chi(2)- and t-test were used to compare neonatal characteristics between periods. Logistic regression was used to identify risk factors associated with visual impairment (defined as visual acuity <or=20/60 or visual field restriction of 20 degrees binocularly). Of 1159 surviving infants, 887 were examined for acute ROP (473 in period 1, 414 in period 2). Stages 3 to 4 ROP or laser-treated ROP were present in 35 infants in period 1 (7%) and 59 in period 2 (14%), P<or=0001. Infants born in period 2 had lower mean BW and gestational age. Among infants who developed severe ROP or laser-treated ROP, binocular visual impairment was present in eight children in period 1 and seven in period 2. Refractive errors, including myopia and astigmatism, were increased in period 2. Children who developed periventricular leucomalacia had the highest risk of visual impairment at 4 to 6 years of age. During the 10-year study period, a significant increase in rates of stages 3 to 4 or laser-treated ROP was not associated with increases in visual impairment rates.

Aerobic and anaerobic capacity in Juvenile Idiopathic Arthritis: evaluation of the cardiorespiratory response

Objectives: To compare the cardiac response during maximal aerobic exercise in JIA with healthy children. Methods: Thirteen children aged 10 to 17 years with JIA and 9 controls (CON) participated. All performed maximal VO2 and Wingate tests on a cycle ergometer. Cardiac output (CO) was measured during exercise with Doppler echocardiography. Arterial – mixed venous oxygen index (A-VO2) and systemic vascular resistance (SVR) were calculated. Results: JIA subjects had decreased aerobic fitness (VO2 31.3, CON 47.9 ml/kg/min p = 0.013]. There was no significant difference in CO, A-VO2 or SVR but we observed trends towards lower CO and higher SVR in JIA. During anaerobic exercise JIA subjects completed less total work. Conclusions: Children with JIA have moderate impairment in aerobic fitness. CO and A-VO2 during aerobic exercise did not differ between JIA subjects and CON. Further research with is required to determine factors contributing to limited fitness in JIA. CONTRIBUTING INSTITUTIONS Divisions of Rheumatology and Cardiology, British Columbia Children's Hospital, Department of Pediatrics, University of British Columbia, Vancouver, Canada. School of Human Kinetics, University of British Columbia, Vancouver, Canada.

An analysis of sport-related injuries: British Columbia children's hospital Emergency Department 1999 – 2003

An analysis of sport-related injuries: British Columbia children's hospital Emergency Department 1999 – 2003

Erythema Nodosum, Mycobacterium avium Lymphadenitis, and a 15-mm Mantoux Test

To the Editors: We report the case of a girl with erythema nodosum (EN)1 that was triggered by a Mycobacterium avium intracellulare (MAC) cervical lymphadenitis. The association of EN with nontuberculous mycobacteria (NTM) is very rare in pediatrics.2 A 2-year-old healthy girl was admitted to our children's hospital with a 3-week illness that began with an upper respiratory infection with fever. She then developed left cervical lymphadenitis followed by erythematous maculopapular lesions in her skin, 2 weeks before admission. Outpatient amoxicillin and cefixime courses were given for presumed bacterial lymphadenitis, with no improvement. On admission, she had a solitary 5 cm indurated, warm, nonfluctuant, painful, left cervical mass and multiple erythematous and violaceous demarcated, shiny, painful raised lesions on her legs, arms, and shoulders. They erupted in patches consistent with EN. She was Canadian born and had no prior BCG vaccination, contacts with or family history of tuberculosis. Ultrasound examination confirmed extensive left-sided cervical lymphadenopathy without abscesses. Tuberculin skin test induration was 15 mm. Chest radiograph was normal and gastric aspirates were negative for M. tuberculosis. Serologic tests were negative for cytomegalovirus, Lyme disease, Epstein-Barr virus, Mycoplasma pneumoniae, and Bartonella henselae. Throat, blood, and stool cultures revealed no pathogens. Screening tests for connective tissue disorders and antistreptolysin O titer were negative. Intravenous clindamycin was administered for 6 days but the patient did not improve. Surgical drainage of the mass revealed pus that contained no organisms on Gram stain. She was discharged without antibiotics, pending culture results. Eight days later, her EN rash had resolved, but drainage was again required. Initial investigations for bacteria and fungi were negative on both specimens. The second specimen contained acid-fast bacilli. MAC was grown after 10 days of incubation from both specimens. Clarithromycin was prescribed but stopped because of oral intolerance. She required a complete excision after which rifampin was given for 6 months. No further recurrence occurred. EN is an acute panniculitis occuring more commonly in adults, associated with infectious and noninfectious etiologies.1 It presents as tender, erythematous nodules predominantly in the lower limbs, with associated symptoms including fever. It usually resolves in weeks and the etiology remains undetermined in up to half of the cases. In one of the largest EN series ever published,1 no case of NTM infection was found among 129 patients seen during 35 years. Among the infectious etiologies, Streptococcus pyogenes and tuberculosis have been the most common triggers. BCG vaccination has been occasionally implicated, and reports of pediatric EN triggered by NTM as in our patient are extremely rare in the literature.2 This case illustrates that a positive Mantoux skin test, independently of the induration size, can be seen in NTM infections.2–4 In this girl, it seems likely that a massive MAC antigen release might have contributed to the EN lesions. In children, NTM should be considered among the infectious etiologies of EN. Cervical lymphadenitis in presence of a positive Mantoux test should include suspicion of NTM infection. Presented in part as an abstract at the 41st Annual Meeting of the Infectious Diseases Society of America (IDSA)—San Diego, CA. October 9–12, 2003. Abstract No. 810.5 Heidi Budden, MD Department of Pediatrics British Columbia's Children's Hospital and The University of British Columbia Vancouver, British Columbia Canada Rolando Ulloa-Gutierrez, MD Simon Dobson, MD, FRCPC David Scheifele, MD Infectious and Immunological Diseases Division

Effects of adherence to treatment on short‐term outcomes in children with juvenile idiopathic arthritis

To determine the impact of adherence to treatment (medication and prescribed exercise) on outcomes in children with juvenile idiopathic arthritis (JIA). In this longitudinal study, we studied parents of patients with JIA at the Montreal Children's Hospital and British Columbia Children's Hospital in Vancouver. Adherence was evaluated on a visual analog scale in the Parent Adherence Report Questionnaire. Outcomes of interest were active joint count, pain, child functional score on the Child Health Assessment Questionnaire, quality of life score on the Juvenile Arthritis Quality of Life Questionnaire, and parental global impression of overall well-being. The association between adherence to treatment and subsequent outcomes was evaluated using generalized estimating equations and logistic regression. Mean age and disease duration of our sample of 175 children were 10.2 and 4.1 years, respectively. Moderate adherence to medication was associated with lower active joint count (odds ratio [OR] 0.47, 95% confidence interval [95% CI] 0.22-0.99). Moderate adherence to exercise was associated with better functional score (OR 0.13, 95% CI 0.03-0.54), and lower pain during the last week (OR 0.14, 95% CI 0.04-0.50). Both high and moderate adherence to exercise were associated with parental perception of global improvement. Improved outcomes in patients who adhered to treatment underscores the need for clinicians to address adherence issues with their patients. Sustaining adherence, particularly to the more time-consuming treatment of exercise, is a challenge.

ADHD and Epilepsy Comorbidity

The prevalence of ADHD, its neurological correlates, and the effect of ADHD on health-related quality of life in 203 children (mean age 11.8 years, SD 3.8) with severe epilepsy were evaluated at British Columbia Children's Hospital, Vancouver, Canada.

Research, policy and practice related to survivors of childhood, adolescent and young adult cancers in British Columbia (BC), Canada: A population-based approach

9539 Background and Purpose: Prevalence of long term survivors of pediatric, adolescent and young adult cancers is increasing. In BC we are using a multidimensional population approach to address research and care for these survivors. Information on trends in prevalence of survivors and use of physician services are presented. Methods: We have identified a cohort of patients under 25, diagnosed from population registries since 1970, and linked their records with person-based longitudinal records of health utilization. Results: Prevalence rate of cancer survivors aged 0–59 years, originally diagnosed under age 25, increased 5 fold between 1975 and 2005, from 26 per 100,000 to 147 per 100,000; 78% were aged 20 or older. A total subgroup of 3,787 individuals surviving five years or more, diagnosed between 1970 and 1995, were identified; 2,590 (68%) of these were linked to physician visit records (excluding visits within BC's Children's Hospital) from 1986 to 2000. In year 2000, 77% of survivors had at least one physician visit; 75% of survivors saw a family physician (FP), and 44% of survivors visited a specialist physician with a specialty that could be related to a late effect. Between 1986 and 2000 survivor prevalence increased 3.3% per year; total visits by survivors to FPs increased by 11.6% per year and by 10.6% per year to specialists. In 1986, 66% of physician visits were for patients older than 20 which increased to 79% by 2000. A provincial pediatric oncology network consisting of oncologists and community care representatives has been established to implement changes to long-term follow up policy. Conclusion: The prevalence rate and utilization of health services among survivors of childhood, adolescent and young adult cancers continues to escalate. Research results, along with other published literature, will inform the development of policy and practice within the BC Network. No significant financial relationships to disclose.

How often do anesthesiologists really check their monitors?

Simon Ford, British Columbia Childrens' Hospital, Vancouver, BC, Canada; Simon Ford, British Columbia Childrens' Hospital; Elina Birmingham, Department of psychology, University of British Columbia, British; G Dumont, Department of Electrical and Computer Engineering; J Lim, British Columbia Childrens' hospital, British Columbia; J Daniels, British Columbia Children's Hospital, British Columbia; JM Ansermino, British Columbia Children's Hospital, British Columbia;

Intracystic bleomycin therapy for craniopharyngioma in children

Surgical removal and radiation therapy are associated with significant risk for morbidity in the pediatric population with craniopharyngioma. Intracystic therapies have been utilized in some centers to potentially decrease morbidity associated with cystic craniopharyngioma. The aim of the study was to review the Canadian experience with intracystic bleomycin therapy (ICB). All centers in the Canadian Pediatric Brain Tumor Consortium (CPBTC) were invited to participate in a retrospective review of this treatment. A questionnaire was sent to each center. The data were analyzed at British Columbia's Children's Hospital. In all, 6 of 17 centers utilized ICB and submitted data. A total of 17 of 19 patients with the intention to treat received bleomycin. Twelve were treated at the time of diagnosis, and 5 at the time of recurrence. Five patients achieved a complete response, 6 achieved a partial response, and 5 achieved a minor response to bleomycin. One patient was stable for 2.8 years. At the time of last follow-up, 8 patients have not required further intervention. Complications included transient symptomatic peritumoral edema (2 patients), precocious puberty (1 patient) and panhypopituitarism (2 patients). The median follow-up was 4 years (range, 0.5-10.2 years). The median progression-free survival was 1.8 years (range, 0.3-6.1 years). One patient died of a massive infarct secondary to radiation-induced moyamoya syndrome. ICB was found to be well tolerated in this group of children. ICB may be a feasible and effective therapy for certain children with craniopharyngioma. Bleomycin may delay the need for aggressive surgery or radiation therapy for several years. Prospective multiinstitutional clinical trials are required to further evaluate the feasibility, effectiveness, and dose schedules of this treatment.

EARLY HYPODENSITY ON COMPUTED TOMOGRAPHIC SCAN OF THE BRAIN IN AN ACCIDENTAL PEDIATRIC HEAD INJURY

Hypodensities on computed tomographic (CT) brain scans are thought to take at least 6 hours to become apparent after blunt head trauma. This finding, in conjunction with the later evolution of the hypodensities, is used in timing the injury in children with suspected non-accidental brain injury, in whom the history may be inaccurate. The purpose of this study is to report the occurrence of diffuse cerebral parenchymal hypodensities on CT scans performed within 5 hours of a well-defined accidental head injury. A retrospective review was performed of five patients admitted to British Columbia Children's Hospital who had accidental head injury and who were identified as having diffuse cerebral hemispheric hypodensities on early CT scans. We present five patients (age range, 4 mo-14 yr) with well-documented accidental head injuries who demonstrated obvious and extensive CT brain scan cerebral hemispheric hypodensity from 60 minutes to 4.5 hours after trauma. All five patients presented with severe head injuries and immediate, unremitting coma, and all five progressed rapidly to brain death within 48 hours. It is unusual, but possible, to develop CT hypodensities as early as 1 hour after accidental head injury. In our small series of cerebral hemispheric hypodensity occurring less than 5 hours after trauma, all five patients had a uniformly fatal outcome. These observations may be important medicolegally in the assessment of the timing of head injury when the history of the trauma is not clear, as in children with suspected non-accidentally inflicted injury. It is inappropriate to generalize these findings to patients who are not unconscious immediately after a head injury, who regain consciousness after an injury before deteriorating, or who do not progress rapidly to brain death.

Perceived adherence to prescribed treatment in juvenile idiopathic arthritis over a one‐year period

To document perceived adherence to treatment (taking medications and performing exercises) in patients with juvenile idiopathic arthritis (JIA) over a 1-year period and to identify related factors. We surveyed parents of patients with JIA at the Montreal Children's Hospital and British Columbia's Children's Hospital in Vancouver. Parents were asked to respond to a series of questionnaires every 3 months over a 12-month period. Perceived adherence was evaluated on a 100-mm visual analog scale (VAS) in the Parent Adherence Report Questionnaire (PARQ). Parental coping, distress, child function, disease severity and duration, perceived helpfulness of treatment, problems encountered, and sociodemographic data were also assessed. The mean age of our sample of 175 children was 10.2 years; mean age at diagnosis was 6.1 years and mean disease duration was 4.1 years. Perceived adherence to medications was consistently high, with average adherence at baseline, 3, 6, 9, and 12 months being 86.1, 91.7, 90.4, 92.0, and 88.8, respectively, on the PARQ VAS. Perceived adherence to exercise was lower but remained steady, with corresponding means of 54.5, 64.1, 61.2, 63.0, and 54.3, respectively. Using generalized estimating equation analysis, factors associated with higher perceived adherence to medications included perceived helpfulness of medications and lower disease severity; those associated with higher perceived adherence to exercise were younger age of the child, child involvement in responsibility for treatment, and higher perceived helpfulness of the treatment. Belief in helpfulness of treatment is associated with higher parental perceived adherence to treatment.

Surgical intervention in children with Crohn’s disease

Crohn's disease (CD) causes chronic inflammation of the gastrointestinal tract leading to extensive medical treatments and surgery with two thirds of patients having surgery over their lifetime. In this study, we reviewed the pediatric population at the British Columbia Children's Hospital diagnosed with CD and examined their demographics and treatments, in particular assessing those who ultimately underwent surgery. Two hundred and eighty children (median age 11.9 years [CI 11.5-12.28]) diagnosed with CD from January 1994 to December 2003 were included. Demographic data were documented including age, ethnicity, duration of symptoms before diagnosis, treatment to date and surgical parameters. Comparison was made between operative and non-operative patients including involvement of disease, medical treatment, complications and recurrence of disease leading to repeat operations. Fifty-five (19.6%) children had surgical procedures. There was a significant increase in surgery in those patients who had not received immunomodulator therapy before surgery (odds ratio 1.95 [CI 1.02-3.73]). We also observed that those CD patients with extensive small intestinal involvement had lower likelihood of having surgery (odds ratio 0.386 [CI 0.145-1.033]). No significant difference was found between the two groups with regard to age of diagnosis (p = 0.41), duration of symptoms (p = 0.22), gender (p = 0.50) or ethnicity (p = 0.451). There was an increased incidence of surgery in those patients who were not treated with immunomodulator therapy. In addition, children with extensive as opposed to isolated small intestinal disease were less likely to have surgery in childhood.

The effect of recent changes in oxygen regime on retinopathy of prematurity incidence at British Columbia Children’s Hospital

The effect of recent changes in oxygen regime on retinopathy of prematurity incidence at British Columbia Children’s Hospital

Pediatric Spine Trauma

Corresponding author: Christopher W. Reilly, MD, FRCSC , Department of Pediatric Orthopaedic Surgery, British Columbia Children's Hospital, Room A234, 4480 Oak Street, Vancouver, British Columbia V6H 3V4, Canada. , E-mail address: [email protected]

Accuracy of the Weber and Rinne Tuning Fork Tests in Evaluation of Children with Otitis Media with Effusion

The purpose of this study was to determine the accuracy of the Rinne and Weber tuning fork tests in predicting the degree of hearing loss secondary to otitis media with effusion (OME) in children compared with pure-tone audiometry. A blinded prospective study was performed on cases of unilateral and bilateral OME in children between ages 2 and 11 years. Patients were recruited from the tertiary Pediatric Otolaryngology Clinic at British Columbia's Children's Hospital in Vancouver. The subjects consisted of 58 children with unilateral or bilateral OME. The Rinne and Weber tests were performed using a 256 Hz and a 512 Hz tuning fork. Pure-tone audiometry was performed to establish the presence and degree of an air-bone gap (ABG). The tuning fork responses were correlated with the ABG to determine their accuracy. The Weber test did not show an association between the ABG and the lateralization response using either the 256 Hz (p = .484) or the 512 Hz tuning fork (p = .120). For the Rinne test, an increase in ABG was significantly associated with a higher rate of response air conduction < bone conduction using either the 256 Hz or the 512 Hz fork (p < .04). Age was not associated with outcome for any of the tuning fork tests. The overall accuracy of the Rinne and Weber tuning fork tests in predicting conductive hearing loss associated with OME in children is poor.

The assessment of management morbidity in children with myelomeningocoele

Address: 1Division of Paediatric Neurosurgery, British Columbia's Children's Hospital, 4480 Oak St, #K3-159, Vancouver, BC, Canada, 2Myelomeningocoele Program, Department of Paediatric Neurosciences, British Columbia's Children's Hospital, 4480 Oak St, #K3-159, Vancouver, BC, Canada and 3Department of Paediatric Neurosciences, British Columbia's Children's Hospital, 4480 Oak St, #K3-159, Vancouver, BC, Canada

Cutaneous changes in fibrous hamartoma of infancy

Fibrous hamartoma of infancy (FHI) is a fast growing soft tissue tumor that usually arises in the first 2 years of life. The histology of the lesion has been well described. Few studies, however, have looked at changes in the overlying skin and its appendages. A database search performed at British Columbia Children's Hospital yielded 15 cases of unequivocal FHI occurring in 12 patients (three were recurrences). Of these, we were able to retrieve 13. Five of 13 cases had sections including epidermis. These slides were reviewed with specific emphasis on skin adnexae. Of the cases with excised epidermis in continuity with the lesion, 5/5 had eccrine changes, including hyperplasia, duct dilatation, intraluminal papillary formations, and squamous syringometaplasia. One case showed epidermal basaloid follicular hyperplasia. This study shows that eccrine changes are frequently seen in cases of FHI when overlying skin is sampled. This may be a useful clue to consider this diagnosis, especially when the biopsy is superficial.

Cerebrospinal fluid (CSF) leak and pseudomeningocele formation after posterior fossa tumor resection in children: a retrospective analysis

The purpose of this study was to determine the incidence of pseudomeningocele and cerebrospinal fluid (CSF) leak after posterior fossa tumor surgery and to analyze factors that may be associated with these conditions. In particular, we wished to determine if there was evidence to support the hypothesis that the use of tissue glue, dural grafts, or external ventricular drainage (EVD) prevented CSF from leaking outside the closed dura. A retrospective chart review was carried out of posterior fossa tumor resections at British Columbia's Children's Hospital. Information was collected regarding tumor location, surgical approach, CSF diversion, dural grafting, and use of tissue glue. Multiple univariate analyses and step-wise logistic regression were performed to identify factors associated with pseudomeningocele formation or CSF leak. A pseudomeningocele was said to be present if it was noted in the clinical records or if a fluid collection was present superficial to the craniotomy flap on a postoperative CT or MR scan. Out of 174 posterior fossa operations, 53 pseudomeningoceles with or without CSF leak were identified along with five CSF leaks in the absence of pseudomeningocele (33%). None of the factors examined reached statistical significance, although there was a trend towards higher rates in patients with external CSF drainage (P=0.06631), dural graft usage (p=0.06492), and patients in whom tissue glue was used (p=0.06181). On logistic regression, only tissue glue use and external CSF drainage were associated with increased incidence of pseudomeningocele and/or CSF leak. In this retrospective study, the use of tissue glue, dural grafts, and external ventricular drainage was not associated with a reduced rate of clinically or radiologically diagnosed pseudomeningocele formation or postoperative CSF leak. The results of this study provide a basis for planning a randomized controlled trial to determine the effectiveness of tissue glue and/or dural grafting in preventing these complications.

Electroclinical Features of Absence Epilepsy

Clinical and EEG features of absence seizures in 47 children with newly diagnosed, untreated childhood absence epilepsy (CAE) were analyzed using video-EEG recordings, in a study at University of Otago, Wellington, New Zealand; British Columbia Children’s Hospital, Vancouver, BC, Canada; and Royal Children’s Hospital, Melbourne, Australia.