Robert Adderley

Long-term ventilation in children: longitudinal trends and outcomes

Background Cross-sectional studies have suggested a rapid expansion in paediatric long-term ventilation (LTV) over the last 20 years but information on longitudinal trends is limited. Methods Data were collected prospectively on all patients receiving LTV over a 15-year period (1.1.95–31.12.09) in a single regional referral centre. Results 144 children commenced LTV during the 15-year period. The incidence of LTV increased significantly over time, with an accompanying 10-fold increase in prevalence due to a significant increase in institution of non-invasive ventilation (NIV). There was no significant increase in invasive ventilation. 5-year survival was 94% overall and was significantly higher for patients on NIV (97%) than invasively ventilated patients (84%). 10-year survival was 91% overall. Although some children were able to discontinue respiratory support (21% at 5 years and 42% at 10 years), the number of patients transitioned to adult services increased significantly over time (26% of total cohort). Patients with neuromuscular disease were less likely to discontinue support than other patients. Conclusions The paediatric LTV population has expanded significantly over 15 years. Future planning of paediatric hospital and community services, as well as adult services, must take into account the needs of this growing population.

Changes in pediatric tracheostomy 1982–2011: a Canadian tertiary children's hospital review

BACKGROUND Pediatric tracheostomy has undergone notable changes in frequency and indication over the past 30 years. This study investigates pediatric tracheostomy at British Columbia Childrens Hospital (BCCH) over a 30-year period. METHODS A retrospective chart review of tracheostomy cases at BCCH from 1982 to 2011 was conducted. Charts were reviewed for demographics, date of tracheostomy, indication, complications, mortality and date of decannulation. Data from three 10-year time periods were compared using Fishers Exact test to examine changes over time. RESULTS 251 procedures (154 males) performed on 231 patients were reviewed. Mean age at tracheostomy was 3.74 years with 48% of procedures undertaken before the age of one year. Frequency of procedure by year has generally declined into the early 2000s. Upper airway obstruction was the most common indication accounting for 33% of procedures. The rate of complication across the entire cohort was 22% with 63% of patients being decannulated. Tracheostomy related mortality occurred in 2.0% of cases reviewed. CONCLUSIONS Changes occurred in primary indications with infections indicating less procedures and neurological impairments indicating more procedures over time. Complications increased and the decannulation rate decreased over this 30-year review. Pediatric tracheostomy is considered a safe and effective procedure at BCCH.

Branhamella catarrhalis bacteremia in children.

ABSTRACT. Two cases of Branhamella catarrhalis bacteremia in childhood are presented. The literature is reviewed for this unusual cause of bacteremia.

Supraventricular tachycardia associated with continuous furosemide infusion

Three cases of supraventricular tachycardia (SVT) associated with the use of furosemide infusion (F1) in children following cardiac surgery are reported. The SVT occurred three to seven hours after starting an infusion at 1.0 mg · kg−1 · hr−1. All three patients had a diuresis of 8–10 ml · kg−1 · hr−1 compared with a mean average of 2.5 ml · kg−1 · hr−1 in 22 other patients who had received a similar infusion. A rapid fluid shift was the most likely mechanism of the tachycardia. Sotalol was effective in controlling the tachycardia in the two patients in whom it was tried. We now recommend a starting dose of 0.3 mg · kg−1 · hr−1 in using furosemide as a continuous infusion, with hourly increments of 0.1 mg · kg−1 · hr−1 until the desired diuresis is obtained.RésuméTrois cas de tachycardie supraventriculaire (SVT) associée à l’utilisation de perfusion de furosémide postchirurgie cardiaque (F1) chez les enfants sont rapportés. Les SVT sont survenus trois à sept heures après le debut de la perfusion à 1,0 mg · kg−1 · hre−1. Tous les trois patients avaient une diurese de 8–10 ml · kg−1 · hre−1 compare une moyenne de 2,5 ml· kg−1 · hre−1 chez 22 autres patients qui out reçu une perfusion identique. Une translocation rapide des liquides fut le mecanisme le plus probable de la tachycardie. Le sotalol fut ejficace pour controler la tachycardie chez les deux patients à qui il fut administré. On recommande actuellement une dose initiate de 0,3 mg · kg−1 · hre−1 lorsqu’on utilise le furosémide en perfusion continue avec line augmentation horaire de 0,1 mg · kg−1 · hre−1 jusqu’à ce que la diurese désirée soil obtenue.

Pediatric home mechanical ventilation: A Canadian Thoracic Society clinical practice guideline executive summary

ABSTRACT Over the last 30 to 40 years, improvements in technology, as well as changing clinical practice regarding the appropriateness of long-term ventilation in patients with “non-curable” disorders, have resulted in increasing numbers of children surviving what were previously considered fatal conditions. This has come but at the expense of requiring ongoing, long-term prolonged mechanical ventilation (both invasive and noninvasive). Although there are many publications pertaining to specific aspects of home mechanical ventilation (HMV) in children, there are few comprehensive guidelines that bring together all of the current literature. In 2011 the Canadian Thoracic Society HMV Guideline Committee published a review of the available English literature on topics related to HMV in adults, and completed a detailed guideline that will help standardize and improve the assessment and management of individuals requiring noninvasive or invasive HMV. This current document is intended to be a companion to the 2011 guidelines, concentrating on the issues that are either unique to children on HMV (individuals under 18 years of age), or where common pediatric practice diverges significantly from that employed in adults on long-term home ventilation. As with the adult guidelines,1 this document provides a disease-specific review of illnesses associated with the necessity for long-term ventilation in children, including children with chronic lung disease, spinal muscle atrophy, muscular dystrophies, kyphoscoliosis, obesity hypoventilation syndrome, and central hypoventilation syndromes. It also covers important common themes such as airway clearance, the ethics of initiation of long-term ventilation in individuals unable to give consent, the process of transition to home and to adult centers, and the impact, both financial, as well as social, that this may have on the childs families and caregivers. The guidelines have been extensively reviewed by international experts, allied health professionals and target audiences. They will be updated on a regular basis to incorporate any new information.

Acute respiratory failure following shunt disconnection

Background A sixteen year old boy with a Meningomyocele/Arnold Chiari Malformation was admitted to the Emergency Department of a large community regional hospital, with acute respiratory failure. His back defect had been closed shortly after birth and a ventriculoperitoneal (VP) shunt inserted. He had had only one shunt revision, when he was thirteen months old, and he underwent an uneventful scoliosis repair at twelve years of age for a significant scoliosis.

Section 9: Home mechanical ventilation in children with kyphoscoliosis

Kyphoscoliosis is a well-recognized cause of respiratory failure in adults. In pediatrics, there is a growing body of literature on respiratory failure secondary to early onset scoliosis associated with other bony abnormalities of the thorax. Most children with scoliosis who require long-term assisted ventilation have neuromuscular scoliosis, in which case, although scoliosis may contribute to respiratory compromise, the requirement for assisted ventilation is usually mandated by the underlying condition. Rotation of the thoracic vertebrae impairs the upward displacement of the ribs during inspiration and places the respiratory muscles at a mechanical disadvantage. In addition, there is displacement and crowding of the organs of the thoracic cavity on the concave side with resulting decreases in both chest wall compliance and lung compliance. Surgical spinal stabilization is an integral component of the treatment of scoliosis, and is regularly performed prophylactically to preserve respiratory function, prevent the future development of pulmonary hypertension, improve quality of life by facilitating seating and for cosmetic reasons (to improve symmetry on standing). Historically, spinal surgery was denied to children at risk of not being able to be weaned from supportive ventilation postoperatively. For example, based on the Duchenne’s muscular dystrophy (DMD) literature, many patients with scoliosis were deemed ineligible for surgery if the forced vital capacity (FVC) was less than 30–40% of predicted values. Some surgeons in the past advocated mandatory preoperative tracheostomy in patients with severe restrictive disease. With the introduction of noninvasive ventilation (NIV), both for immediate postoperative as well as long-term ventilatory support, in addition to improved anesthetic and operative techniques, such limitations have been reexamined. It is therefore important that a guideline not only address the question of providing home mechanical ventilation (HMV) to pediatric patients with respiratory failure and scoliosis, but also the eligibility for corrective scoliosis surgery, the consequences of surgery on pulmonary function and discussion of techniques to reduce morbidity following surgery. Literature review: Methodology

Temporary tracheostomy required as an infant may be a risk factor for future centrally mediated disordered sleep ventilation

Background Children with spina bifida experience lifelong complex medical issues. The problems of locomotion, and neurogenic bowel and bladder are well appreciated, and the necessity of shunting obstructive hydrocephalus is equally well known. There are however, serious, less common problems associated with control of ventilation. Most common is vocal cord dysfunction, with unilateral or bilateral vocal cord paresis, and as a consequence, upper airway obstruction. Rarer still, are patients with life threatening breath holding spells, central apnea, or mixed central and obstructive apnea. In many cases, surgical decompression of the posterior fossa can result in return of vocal cord function, and relief of obstructive apnea. In a few cases tracheostomy is required. With a Chairi II malformation, the upper medulla, where the nuclei of cranial nerves IX and X lie close to the rostral tracts of the respiratory centre, may have an abnormal and tenuous blood supply. The medulla may be compromised by bony pressure or by compromise of the blood supply (herniation or chronic arachnoiditis).

HIGH RISK PAEDIATRIC INTER-HOSIPTAL TRANSPORT

While neonatal transport is widespread and relatively standardized, paediatric transport is newer with more varied requirements for optimal management, equipment and personnel. Over a recent 14 month period (ending 30/9/86), our center coordinated inter-hospital transfer of 400 children.Thirty percent were neonates, with a total of 59% less than 1 year, 19% between 1-4 years, 12% between 4-8 years, and 10% older than 8 years. Major diagnoses were trauma, infection, seizures, hypoxic-ischemic injury and congenital anomalies. Eighty-two percent of the calls were emergent and 60% of children required admission to the Intensive Care Unit. Six (1.5%) died during resuscitation prior to transfer, and no children died in transit. Sixty-three percent were transported by air.Problems occurred on 20% of transports, of which 8% were predicted (due to the severity of illness, equipment limitation or poor weather). Significant problems occurred more frequently when patients were escorted by adult paramedics as compared to infant trained crews. Thirty percent of patients required major interventions by the transport team for stabilization prior to transfer and 5% required major interventions enroute.The logistics of coordinating paediatric emergency transport are complex because of the diversity of diagnoses, physical size, medication and equipment, requiring dedicated physician staff with a transport team trained to manage paediatric patients.

NEAR-DROWNING IN BRITISH COLUMBIA

Near-drowning is a cause of major neurological damage in children. Following reports of unexpected survival, vigorous measures to control intracranial pressure and minimize cerebral metabolic requirements became popular (the “HYPER” regimen). Our approach has been supportive (no barbituate coma or hypothermia) to achieve optimal oxygenation and cerebral perfusion while minimizing potential insults which raise intracranial pressure. We stress prompt resuscitation with re-warming to normothermia, fluid restriction, and diuresis. Head elevation and judicious sedation are used to avoid adverse effects on intracranial pressure from noxious stimuli.Our results are comparable to other centers. Sixty-one percent drowned in baths, hot-tubs or swimming pools, 36% in lakes or streams, and one child in the sea. The age range was from 5 months to 12 years (mean 3.2 years). Of 31 patients admitted over 46 months, 71% survived. Using Conns classification, 10 Group A (awake) patients had a 100% intact survival. Of 5 Group B (blunted) patients, 4 survived and 1 died. From 16 Group C (comatose) patients, 4 survived (all C-1) with good neurological function, 4 were handicapped and 8 died.Important prognostic factors include cold water submersion, early resuscitation at the scene, short duration of resuscitation, transport which avoids secondary insult and meticulous intensive care.