Jonathan De Lima

Use of the ASA Physical Status Grading System in pediatric practice

Background:  The American Society of Anesthesiologists (ASA) Grading System is widely used to describe preoperative physical status. Inconsistency of grading between anesthetists has been demonstrated in studies using hypothetical adult patient scenarios. We aimed to investigate the use and interrater reliability of the ASA Grading System in pediatric anesthesia practice.

Anesthesia for pediatric renal transplantation with and without epidural analgesia – a review of 7 years experience

Background : Few objective data exist describing current anesthesia practice for pediatric renal transplantation. We describe here, the experience from an Australian tertiary pediatric center that has continued an active pediatric renal transplantation program after relocation in 1995. Areas of interest include preoperative status, fluid management, hemodynamic stability, perioperative complications, and the use of epidural analgesia. In particular, the influence of perioperative epidural analgesia on hemodynamic stability is addressed.

Cortisol response to general anaesthesia for medical imaging in children

Objective  The cortisol response to surgical stress has been frequently studied, and recommendations developed for steroid replacement in adrenally insufficient patients. There are currently no guidelines, however, for adrenal hormone replacement during anaesthesia alone. The objective of this study was to characterize the normal cortisol response to general anaesthesia in the absence of a surgical procedure in children.

Effect of general anesthesia on pulmonary function and clinical status on children with cystic fibrosis

Children with cystic fibrosis (CF) receive general anesthesia (GA) for a variety of different procedures. Historical studies assessing risk of GA report a high risk of morbidity. There is a paucity of data evaluating the risk of currently available anesthetic agents. The aim of this study was to assess the effect of GA on clinical status and lung function on children with CF.

Exploratory study of sleeping patterns in children admitted to hospital

Sleep is considered an important time of healing and restoration during illness. The primary aim of this study was to determine the prevalence of self‐reported sleep disturbance in children admitted to a tertiary childrens hospital with a variety of medical diagnoses.

Anesthesia for children with hyperleukocytosis a retrospective review

Background:  Hyperleukocytosis (a white cell count in peripheral blood >100 × 109 l−1) is a well‐recognized medical emergency. Rates of morbidity associated with anesthesia in hyperleukocytotic patients have not been previously described. This retrospective study describes the perioperative morbidity and mortality of children who present acutely with hyperleukocytosis.

Profile of children with developmental disabilities attending a complex pain clinic of a children's hospital in Australia

To document the profile and management of children with developmental disabilities (DD) attending an outpatient complex pain clinic at a Childrens Hospital in Sydney, Australia.

A case series of general anesthesia in children with laminin alpha2 (merosin)-deficient congenital muscular dystrophy.

SIR—Serious morbidity associated with general anesthesia has been reported in patients with a variety of muscular dystrophies (1). Malignant hyperthermia (MH) is an often-quoted concern when such patients are exposed to volatile anesthetic agents or depolarizing muscle relaxants (2). These concerns often translate into recommendations to use a ‘trigger-free’ anesthetic technique in children ‘at risk’. There is, however, debate about the relationship of these disorders to MH. In 2008, the Society for Paediatric Anaesthesia met to discuss this matter, and no absolute consensus was reached (2). More recently, there has been a growing awareness of the risk of anesthesia-induced hyperkalemia in children with muscle disease. This life-threatening complication has been best documented in children with Duchenne muscular dystrophy (DMD) but remains a poorly defined risk in children with other muscular dystrophies. Managing these risks, especially in the context of undiagnosed muscle disorders (as in anesthesia for muscle biopsy), requires a good understanding of the documented risks in well-defined samples of patients with congenital muscular dystrophy (CMD). Laminin a2 (merosin)-deficient CMD (LAMA2 CMD) is a primary congenital myopathy with a population prevalence of approximately 0.7 per 100 000. Clinical features of patients with LAMA2 CMD include generalized hypotonia, weakness, and delayed motor milestones (3). Serum CK concentration is elevated up to fourfold, and Magnetic Resonance Imaging (MRI) frequently reveals diffuse white matter changes which usually become evident by the age of 6–12 months (3). Definitive diagnosis is made on muscle or skin biopsy wherein altered laminin expression can be detected by immunocytochemical testing or by detecting mutations in the LAMA2 gene (4). Patients with LAMA2 CMD were not traditionally thought to be susceptible to MH; however, a single case report appeared in 2006 describing a suspected MH episode in a child with LAMA2 CMD undergoing ‘triggerfree’ anesthesia (5). The report concluded that patients with laminin a2 deficiency might be susceptible to MH. To provide further guidance, we reviewed our institutional experience of anesthesia in children with LAMA2 CMD. The Children’s Hospital at Westmead has a welldefined cohort of children with LAMA2 CMD. With ethics committee approval, a retrospective case note review of our cohort of patients was undertaken, looking specifically at morbidity associated with general anesthesia. Over a period of 20 years, from 1991 to 2011, 10 children had a biopsy-confirmed diagnosis of LAMA2 CMD. These 10 underwent a total of 16 general anesthetics (to complete 21 individual procedures). Thirteen of these included a volatile anesthetic agent. Three children received total intravenous anesthesia (TIVA). The average age at the time of anesthesia was 3.1 years (range: 42 days to 11 years). Procedures included muscle and liver biopsies (11), MRI (6), orthopedic procedures (3), and dental extraction (1). Standard monitoring for anesthesia was applied (ECG, pulse oximetry, endtidal CO2, noninvasive blood pressure, and postoperative temperature monitoring). The mean duration of anesthesia was 75 min (range: 30–150 min). Induction with volatile-based anesthetics included sevoflurane (7), halothane (5) in a nitrous oxide–oxygen mixture and was not specified in one case. Anesthesia was maintained with halothane (6), isoflurane (5), and sevoflurane (2). In the procedures carried out using TIVA, propofol was used for both induction and maintenance. All perioperative records were systematically searched for adverse events, complications, and signs suggestive of MH (tachycardia, hypercarbia, hyperthermia, muscle stiffness, and rigidity). No serious morbidity was detected during or following any of the anesthetics studied. For all 16 anesthetics, no evidence to suggest an episode of malignant hyperpyrexia was detected. All of the recorded temperatures were 37.0°C or below. One infant, aged 6 months, was transiently tachycardic (150 bpm) and tachypneic (35 bpm) for approximately 2 h postanesthesia. This infant had received TIVA for a muscle and liver biopsy. No muscle stiffness was detected and his temperature remained normal for the 36 h of observation that followed. Thus, no patients in our cohort of 10 children with laminin a2 (merosin)-deficient CMD exposed to volatile anesthetic agents developed features consistent with MH. Estimating the risk of anesthesia-induced hyperkalemia in children with LAMA2 CMD remains a theoretical exercise. Current understanding of the pathophysiology suggests that anesthetic agents stress an already susceptible sarcolemma and impair muscle membrane stability. The increased membrane Correspondence