Study to evaluate the optimal dose of remifentanil required to ensure apnea during magnetic resonance imaging of the heart under general anesthesia.

BACKGROUND: Magnetic resonance (MRI) scanning of the heart is an established part of the investigation of cardiovascular conditions in children. In young children, sedation is likely to be needed, and multiple controlled periods of apnea are often required to allow image acquisition. Suppression of spontaneous ventilation is possible with remifentanil; however, the dose required is uncertain. AIMS: To establish the dose of remifentanil, by infusion, required to suppress ventilation sufficiently to allow a 30-s apnea during MRI imaging of the heart. METHOD: Patients aged 1-6 years were exposed to different doses of remifentanil, and the success in achieving a 30-s apnea was recorded. A dose recommendation was made for each patient, informed by responses of previous patients using an adaptive Bayesian dose-escalation design. Other aspects of anesthesia were standardized. A final estimate of the dose needed to achieve a successful outcome in 80% of patients (ED80) was made using logistic regression. RESULTS: 38 patients were recruited, and apnea achieved in 31 patients. The estimate of the ED80 was 0.184 µg/kg/min (95% CI 0.178-0.190). Post hoc analysis revealed that higher doses were required in younger patients. CONCLUSION: The ED80 for this indication was 0.184 µg/kg/min (95% CI 0.178-0.190). This is different from optimal dosing identified for other indications and dosing of remifentanil should be specific to the clinical context in which it is used.

Imaging of torticollis in children.

The normal anatomy of the cervical spine and various imaging techniques for the evaluation of torticollis are reviewed, and possible causes of torticollis in infants and children are discussed, with an emphasis on relevant imaging findings. Torticollis is a congenital or acquired deformity characterized by rotational deformity of the cervical spine with secondary tilting of the head. Although torticollis is a sign of an underlying disease process, its presence does not imply a specific diagnosis, and the cause should be sought if torticollis persists or is associated with other symptoms. Congenital torticollis, seen in neonates and infants, usually results from craniocervical vertebral anomalies or muscular causes, although ocular abnormalities such as congenital paralytic squint (strabismus) and congenital nystagmus should also be considered. Acquired torticollis, seen in older children and adolescents, is often secondary to trauma, infection, or tumors. Imaging should be used as a general screening tool only after a complete medical history and clinical findings have been obtained. In newborns or infants with congenital torticollis, ultrasonography (US) is the modality of choice. In cases of acquired torticollis resulting from trauma, conventional radiography (lateral and anteroposterior views) should be the first-line imaging modality. In nontraumatic acquired torticollis, computed tomography (CT) of the neck or cervical spine is the initial imaging study. If CT findings are negative, magnetic resonance (MR) imaging of the brain and cervical spine should be performed. The use of multiple imaging modalities (conventional radiography, US, CT, and MR imaging) is common in the radiologic work-up of torticollis, and radiologists must understand the role of each imaging modality in patients of various ages.

Vitamin D for Growth and Rickets in Stunted Children: A Randomized Trial.

BACKGROUND AND OBJECTIVES: Vitamin D is essential for healthy development of bones, but little is known about the effects of supplementation in young stunted children. Our objective was to assess the effect of vitamin D supplementation on risk of rickets and linear growth among Afghan children. METHODS: In this double-blind, placebo-controlled trial, 3046 children ages 1 to 11 months from inner-city Kabul were randomly assigned to receive oral vitamin D3 (100 000 IU) or placebo every 3 months for 18 months. Rickets Severity Score was calculated by using wrist and knee radiographs for 631 randomly selected infants at 18 months, and rickets was defined as a score >1.5. Weight and length were measured at baseline and 18 months by using standard techniques, and z scores were calculated. RESULTS: Mean (95% confidence interval [CI]) serum 25-hydroxyvitamin D (seasonally corrected) and dietary calcium intake were insufficient at 37 (35-39) nmol/L and 372 (327-418) mg/day, respectively. Prevalence of rickets was 5.5% (placebo) and 5.3% (vitamin D): odds ratio 0.96 (95% CI: 0.48 to 1.92); P = .9. The mean difference in height-for-age z score was 0.05 (95% CI: -0.05 to 0.15), P = .3, although the effect of vitamin D was greater for those consuming >300 mg/day of dietary calcium (0.14 [95% CI: 0 to 0.29]; P = .05). There were no between-group differences in weight-for-age or weight-for-height z scores. CONCLUSIONS: Except in those with higher calcium intake, vitamin D supplementation had no effect on rickets or growth.

A national consensus management pathway for paediatric inflammatory multisystem syndrome temporally associated with COVID-19 (PIMS-TS): results of a national Delphi process.

Paediatric inflammatory multisystem syndrome temporally associated with COVID-19 (PIMS-TS) is a novel condition that was first reported in April, 2020. We aimed to develop a national consensus management pathway for the UK to provide guidance for clinicians caring for children with PIMS-TS. A three-phase online Delphi process and virtual consensus meeting sought consensus over the investigation, management, and research priorities from multidisciplinary clinicians caring for children with PIMS-TS. We used 140 consensus statements to derive a consensus management pathway that describes the initial investigation of children with suspected PIMS-TS, including blood markers to help determine the severity of disease, an echocardiogram, and a viral and septic screen to exclude other infectious causes of illness. The importance of a multidisciplinary team in decision making for children with PIMS-TS is highlighted throughout the guidance, along with the recommended treatment options, including supportive care, intravenous immunoglobulin, methylprednisolone, and biological therapies. These include IL-1 antagonists (eg, anakinra), IL-6 receptor blockers (eg, tocilizumab), and anti-TNF agents (eg, infliximab) for children with Kawasaki disease-like phenotype and non-specific presentations. Use of a rapid online Delphi process has made it possible to generate a national consensus pathway in a timely and cost-efficient manner in the middle of a global pandemic. The consensus statements represent the views of UK clinicians and are applicable to children in the UK suspected of having PIMS-TS. Future evidence will inform updates to this guidance, which in the interim provides a solid framework to support clinicians caring for children with PIMS-TS. This process has directly informed new PIMS-TS specific treatment groups as part of the adaptive UK RECOVERY trial protocol, which is the first formal randomised controlled trial of therapies for PIMS-TS globally.

Skeletal abnormalities secondary to antenatal etidronate treatment for suspected generalised arterial calcification of infancy.

BACKGROUND: Generalised arterial calcification of infancy (GACI) is a rare disorder characterised by the deposition of hydroxyapatite crystals within the vessel walls. It is associated with a high mortality rate. Bisphosphonates have been used with some success in the treatment of GACI. However, there is a paucity of data on the antenatal use of bisphosphonates for GACI. In this paper, we report development of the skeletal changes suggestive of hypophosphatasia (HPP) in an infant with GACI, whose mother was treated with etidronate during pregnancy. CASE REPORT: A Caucasian infant boy had a suspected antenatal diagnosis of GACI based on the findings suggestive of calcification of the annulus of the tricuspid valve and wall of the right ventricular (RV) outflow tract and main pulmonary artery on foetal echocardiography and the genetic analysis which showed a pathogenic heterozygous mutation in ABCC6. Based on these findings, mother was started on etidronate treatment from 26 weeks of gestation. A healthy male baby was delivered at 38 weeks of gestation. Initial postnatal echocardiogram on day 1 of life was normal with good biventricular function; subtle changes suggestive of microcalcifications were detected on the CT angiography. Serum calcium, phosphate, alkaline phosphatase and renal profile were normal. Further, the serum inorganic pyrophosphate (PPi) level was significantly low. Skeletal changes suggestive of HPP were seen on the radiographs. The baby developed cardiac dysfunction on day 4 of life with evidence of ischaemic changes on electrocardiogram (ECG).Treatment with etidronate was started in view of probable evolving coronary calcifications. Despite treatment with cardiac supportive measures and bisphosphonate, he succumbed to death in the third week of life. DISCUSSION: We believe, this is the first report of skeletal changes suggestive of HPP, arising secondary to antenatal etidronate (first generation bisphosphonate) used for the treatment of suspected GACI due to a heterozygous ABCC6 mutation.

Primary pleural precursor B-Cell lymphoblastic lymphoma.

Intrathoracic lymphoblastic lymphoma (LBL) is classically of T-cell lineage, but these cases of pleural B-cell LBL suggest that this is not always the case. Despite the clinical challenges involved every attempt should be made to secure a biopsy and histological diagnosis, as we move into an era of lineage-directed therapies.

Posttraumatic nonunion of the clavicle in a 13-year-old boy causing an arteriovenous fistula.

CONTEXT: Fractures of the clavicle are one of the most common injuries to the bone in childhood, but posttraumatic nonunion of pediatric clavicle fractures are extremely rare, with only isolated reports in literature. CASE REPORT: We report a case of a posttraumatic painful nonunion of a clavicle fracture in a 13-year-old boy that caused symptomatic compression of the external jugular vein (EJV) and the formation of an arteriovenous fistula. The fracture was treated successfully with open reduction and internal fixation with a contoured recon plate 6 months following the injury. The fistula was treated by ligation and closure. CONCLUSION: The patient made a full recovery 6 months following surgery and was asymptomatic with full range of shoulder movement. Fracture union was confirmed by computed tomography (CT) scanning and no residual fistula was found.

Unsuspected periventricular leukomalacia in children with strabismus: a case series.

PURPOSE: We report a case series of seven patients who presented with strabismus and no apparent known neurological deficit. METHODS: A retrospective review of notes was performed on all patients presenting at the Royal Liverpool Children's Hospital (Alder Hey) with strabismus, in whom subsequent investigation revealed the presence of periventricular leukomalacia (PVL). RESULTS: All seven patients presented with esotropia. One patient also had dissociated vertical deviation. A history of premature birth was elicited in six cases; however, one patient had been born at 42 weeks. Apparently uncomplicated routine surgery produced unexpectedly poor results. CONCLUSIONS: Patients with undiagnosed PVL may present to the ophthalmologist with strabismus and no other apparent neurological abnormality. Where it is suspected that the patient may have suffered a significant hypoxic-ischaemic event, the ophthalmologist should have a low threshold for investigating with magnetic resonance imaging, particularly if the patient shows unexpected outcomes following appropriate treatment. Patients and parents should be counselled on possible suboptimal results prior to the offer of surgical correction of strabismus.