Vigabatrin

The new antiepileptic drugs introduced over the last decade provide a welcome increase in the therapeutic options available for the 25% of children with epilepsy who remain refractory to traditional therapy.¹ In the mid 1980s, vigabatrin (VGB) was the first of these to be made available in clinical practice. In 1997, the visual field deficits reported in association with VGB prompted a review of its place in the management of epilepsy. We provide an overview of the uses of VGB and a discussion of the difficulties which currently affect our clinical practice.     

Presumed perinatal stroke in a child with Down syndrome and moyamoya disease

Moyamoya disease describes a cerebral arteriopathy characterized by stenosis or occlusion of the terminal internal carotid and/or the proximal middle cerebral arteries. We report a female child with trisomy 21 and bilateral moyamoya disease who presented, unusually, with a presumed perinatal cerebral infarct. The clinical, radiological, and angiographic features of moyamoya disease in children with Down syndrome are similar to those with other aetiologies or idiopathic cases. Early recognition is important as moyamoya disease presenting in childhood is associated with a high rate of recurrent stroke and there is evidence that surgical revascularization can prevent further events. An important practical lesson arising from this case is that although the evaluation of children with presumed perinatal stroke tends to be limited relative to the evaluation of arterial ischaemic stroke in older children, this may need to be more comprehensive in infants at high risk of arteriopathy. The parents of the child gave informed consent to the publication of this report.     

Degos disease in a child presenting with acute renal failure

Degos disease, also termed malignant atrophic papulosis, is a rare systemic vaso-occlusive disorder, seldom reported in the pediatric population. The pathognomonic skin lesion in Degos disease is a papule with an atrophic porcelain-white center with an erythematous, telangiectatic rim. The benign form of the disease remains limited to the skin, whereas, in others, it progresses to thrombotic vasculopathy in multiple organs including the gastrointestinal, cardiorespiratory, and central nervous systems, with a high mortality rate. We present a rare case of Degos disease in an adolescent female, presenting as acute renal failure secondary to thrombotic vasculopathy, with the characteristic skin lesion distinctively seen on dermoscopy.     

Self-induced panniculitis in adolescence: Three cases and review of the literature

Self-induced trauma is a rare cause of panniculitis in adolescents and may be associated with undiagnosed underlying psychological conditions. The condition often poses a diagnostic challenge as patients usually present with non-specific signs and symptoms, and often a concealed history of self-induced trauma. Here, we present three adolescent patients with self-induced traumatic panniculitis. All three patients were repeatedly admitted with non-specific cutaneous lesions as well as multiple somatic complaints. After extensive evaluation to exclude organic causes, psychiatric assessment eventually revealed underlying psychosomatic disorders as a cause of the self-induced trauma.     

Skeletal Muscle Ultrasonography in Nutrition and Functional Outcome Assessment of Critically Ill Children: Experience and Insights From Pediatric Disease and Adult Critical Care Studies

Evidence suggests that critically ill children develop muscle wasting, which could affect outcomes. Muscle ultrasound has been used to track muscle wasting and association with outcomes in critically ill adults but not children. This review aims to summarize methodological considerations of muscle ultrasound, structural findings, and possibilities for its application in the assessment of nutrition and functional outcomes in critically ill children. Medline, Embase, and CINAHL databases were searched up until April 2016. Articles describing skeletal muscle ultrasound in children and critically ill adults were analyzed qualitatively for details on techniques and findings. Thickness and cross‐sectional area of various upper and lower body muscles have been studied to quantify muscle mass and detect muscle changes. The quadriceps femoris muscle is one of the most commonly measured muscles due to its relation to mobility and is sensitive to changes over time. However, the margin of error for quadriceps thickness is too wide to reliably detect muscle changes in critically ill children. Muscle size and its correlation with strength and function also have not yet been studied in critically ill children. Echogenicity, used to detect compromised muscle structure in neuromuscular disease, may be another property worth studying in critically ill children. Muscle ultrasound may be useful in detecting muscle wasting in critically ill children but has not been shown to be sufficiently reliable in this population. Further study of the reliability and correlation with functional outcomes and nutrition intake is required before muscle ultrasound is routinely employed in critically ill children.     

Outcomes of Infants With Home Tube Feeding: Comparing Nasogastric vs Gastrostomy Tubes

Background: The aim of this study was to determine the tube‐related complications and feeding outcomes of infants discharged home from the neonatal intensive care unit (NICU) with nasogastric (NG) tube feeding or gastrostomy (G‐tube) feeding. Materials and Methods: We performed a chart review of 335 infants discharged from our NICU with home NG tube or G‐tube feeding between January 2009 and December 2013. The primary outcome was the incidence of feeding tube–related complications requiring emergency department (ED) visits, hospitalizations, or deaths. Secondary outcome was feeding status at 6 months postdischarge. Univariate and multivariate analyses were conducted. Results: There were 322 infants discharged with home enteral tube feeding (NG tube, n = 84; G‐tube, n = 238), with available outpatient data for the 6‐month postdischarge period. A total of 115 ED visits, 28 hospitalizations, and 2 deaths were due to a tube‐related complication. The incidence of tube‐related complications requiring an ED visit was significantly higher in the G‐tube group compared with the NG tube group (33.6% vs 9.5%, P < .001). Two patients died due to a G‐tube–related complication. By 6 months postdischarge, full oral feeding was achieved in 71.4% of infants in the NG tube group compared with 19.3% in the G‐tube group (P < .001). Type of feeding tube and percentage of oral feeding at discharge were significantly associated with continued tube feeding at 6 months postdischarge. Conclusion: Home NG tube feeding is associated with fewer ED visits for tube‐related complications compared with home G‐tube feeding. Some infants could benefit from a trial home NG tube feeding.     

Transcatheter tricuspid valve replacement With the Edwards SAPIEN valve

We describe a case of percutaneous tricuspid valve implantation in a 20‐year‐old man with previous tricuspid valve replacement in the setting of pulmonary atresia with intact ventricular septum. He developed symptomatic endocarditis‐induced tricuspid regurgitation of the tricuspid bioprosthesis. Tricuspid valvar competence was restored with implantation of a 26‐mm Edwards SAPIEN valve. © 2011 Wiley‐Liss, Inc.     

A biodegradable device (BioSTAR™) for atrial septal defect closure in children

Background : Percutaneous closure of atrial defects (ASD) has evolved as the treatment of choice for the majority of defects and patent oval foramens. The BioSTAR? biodegradable implant avoids many issues associated with devices containing substantial amounts of metal. Methods : Reviewed was a consecutive series of 10 ASD occlusions in a pediatric population with the BioSTAR biodegradable device. All implantations were performed by one operator. The inclusion criterion was a balloon stretched ASD diameter of ≤16 mm. Procedural data and acute and early‐term closure rates were retrospectively matched to a cohort of children having defect closure using the Amplatzer Septal Occluder? (ASO?). Results : Acute and 6 month follow up closure rates for the BioSTAR were 90% and 100% vs. 100% and 100% closure with the ASO implants. There was a statistically significant difference in the median procedure time (52 min: BioSTAR; 39.5 min: ASO device, P < 0.05), with fluoroscopy times slightly longer for the BioSTAR group (6.7 min vs. 6.1 min, P = ns). There were no significant complications in either group. Conclusions : The BioSTAR implant can achieve comparable closure rates to the ASO in small‐ to moderate‐atrial septal defects with only a minimal skeleton of foreign material remaining after 6 months. Longer fluoroscopy and procedure times were a drawback; however, these should improve with familiarity with the implant and deployment system. © 2010 Wiley‐Liss, Inc.