Clinical and Imaging Characteristics of Arteriopathy Subtypes in Children with Arterial Ischemic Stroke: Results of the VIPS Study.

BACKGROUND AND PURPOSE: Childhood arteriopathies are rare but heterogenous, and difficult to diagnose and classify, especially by nonexperts. We quantified clinical and imaging characteristics associated with childhood arteriopathy subtypes to facilitate their diagnosis and classification in research and clinical settings. MATERIALS AND METHODS: The Vascular Effects of Infection in Pediatric Stroke (VIPS) study prospectively enrolled 355 children with arterial ischemic stroke (2010-2014). A central team of experts reviewed all data to diagnose childhood arteriopathy and classify subtypes, including arterial dissection and focal cerebral arteriopathy-inflammatory type, which includes transient cerebral arteriopathy, Moyamoya disease, and diffuse/multifocal vasculitis. Only children whose stroke etiology could be conclusively diagnosed were included in these analyses. We constructed logistic regression models to identify characteristics associated with each arteriopathy subtype. RESULTS: Among 127 children with definite arteriopathy, the arteriopathy subtype could not be classified in 18 (14%). Moyamoya disease (n = 34) occurred mostly in children younger than 8 years of age; focal cerebral arteriopathy-inflammatory type (n = 25), in children 8-15 years of age; and dissection (n = 26), at all ages. Vertigo at stroke presentation was common in dissection. Dissection affected the cervical arteries, while Moyamoya disease involved the supraclinoid internal carotid arteries. A banded appearance of the M1 segment of the middle cerebral artery was pathognomonic of focal cerebral arteriopathy-inflammatory type but was present in <25% of patients with focal cerebral arteriopathy-inflammatory type; a small lenticulostriate distribution infarct was a more common predictor of focal cerebral arteriopathy-inflammatory type, present in 76%. It remained difficult to distinguish focal cerebral arteriopathy-inflammatory type from intracranial dissection of the anterior circulation. We observed only secondary forms of diffuse/multifocal vasculitis, mostly due to meningitis. CONCLUSIONS: Childhood arteriopathy subtypes have some typical features that aid diagnosis. Better imaging methods, including vessel wall imaging, are needed for improved classification of focal cerebral arteriopathy of childhood.

Rapid evolution of elaborate male coloration is driven by visual system in Australian fairy-wrens (Maluridae).

The interplay between colour vision and animal signalling is of keen interest to behavioural ecologists and evolutionary biologists alike, but is difficult to address in terrestrial animals. Unlike most avian lineages, in which colour vision is relatively invariant among species, the fairy-wrens and allies (Maluridae) show a recent gain of ultraviolet sensitivity (UVS). Here, we compare the rates of colour evolution on 11 patches for males and females across Maluridae in the context of their visual system. We measured reflectance spectra for 24 species, estimating five vision-independent colour metrics as well as metrics of colour contrast among patches and sexual dichromatism in a receiver-neutral colour space. We fit Brownian motion (BM) and Ornstein-Uhlenbeck (OU) models to estimate evolutionary rates for these metrics and to test whether male coloration, female coloration or dichromatism was driven by selective regimes defined by visual system or geography. We found that in general male coloration evolved rapidly in comparison with females. Male colour contrast was strongly correlated with visual system and expanded greatly in UVS lineages, whereas female coloration was weakly associated with geography (Australia vs. Papua New Guinea). These results suggest that dichromatism has evolved in Maluridae as males and females evolve at different rates, and are driven by different selection pressures.

Pituitary stalk duplication in association with moya moya disease and bilateral morning glory disc anomaly - broadening the clinical spectrum of midline defects.

BACKGROUND: Duplication of the pituitary stalk, morning glory disc anomaly and moya moya are rare malformations. The combination of these findings may be syndromic and may have an underlying genetic etiology. METHODS: Case report and review of the literature of neurological, ophthalmological, and neuroradiological findings including ophthalmic examination, MRI and MRA. CASE REPORT: A 2 year-old girl presented with reduced visual acuity and roving eye movements since birth. Ophthalmological workup revealed bilateral morning glory disc anomaly. MRI showed duplication of the pituitary stalk and caudal displacement of the floor of the third ventricle. MRA showed narrowing of the supraclinoid internal carotid arteries with focal narrowing of the proximal middle cerebral arteries consistent with early moya moya disease. CONCLUSIONS: Review of the literature of pituitary gland duplication and of the combination of morning glory disc anomaly and moya moya disease revealed only one previously reported case. However, the spectrum of this possibly syndromic presentation may be much broader and include various types of anterior midline defects and may have a common underlying genetic cause.

An update on inflammatory disorders of the pediatric airway: epiglottitis, croup, and tracheitis.

Acute infections of the respiratory tract are common in pediatric patients. Respiratory disease is the leading cause of hospitalization in children less than 4 years of age and is responsible for many physicians' office and emergency department visits.(1) The severity of upper respiratory tract infection ranges from mild, self-limited disease to potentially life-threatening airway obstruction. The prepared clinician can often make a diagnosis based solely on the history and physical examination, using radiographs and laboratory examinations to aid in diagnosis when the clinical picture is unclear. At times, airway collapse is imminent, and the clinician must proceed directly to endoscopy for definitive diagnosis and airway protection. This article will discuss the pathogenesis, clinical presentation, and management of epiglottitis, croup, and bacterial tracheitis in the pediatric population.

Optimal technique to diagnose primary ciliary dyskinesia.

OBJECTIVE: To develop a cost-effective protocol for diagnosing primary ciliary dyskinesia (PCD). STUDY DESIGN: Retrospective chart review in a tertiary care academic medical center. METHODS: A review of the electron microscopy logbook identified all patients who had a suspected diagnosis of PCD. Biopsy of respiratory tract mucosa was performed using a cytology brush or a cup forceps in the outpatient clinic or operating room (OR). Outcome measures were to determine the diagnostic adequacy of cytological evaluation compared with tissue biopsy and to determine whether an adequate nasal mucosa sample can be collected in the outpatient clinic setting as compared with the OR and the use of general anesthesia RESULTS: Twenty-seven patients underwent 31 biopsies. Fifteen specimens were collected with a cup forceps, and 16 with a cytology brush. The sampling sites were nasal mucosa in 28 cases and trachea in the rest. Twelve specimens (39%) were collected in the clinic; the rest were obtained in the OR in conjunction with another procedure. Neither method of specimen collection nor mode of anesthesia made a significant difference in the probability of obtaining an adequate specimen. Ten samples were nondiagnostic: cytological evaluation, 31% (n = 16); tissue biopsy, 27% (n = 15); clinic, 42% (n = 12); and OR, 31% (n = 16). The cost of evaluating ciliary motion at our institution was $150, with an additional charge of $1,297 for electron microscopic evaluation. The nonprofessional fee for an outpatient nasal biopsy was $98; in the OR the cost of anesthesia supplies, surgical suite, recovery room, and day-surgery bed was at least $1,860. CONCLUSION: Our results suggest that the optimal method for diagnosis of PCD is in the outpatient clinic with specimen collection by means of either a cup forceps or a cytology brush.

Metabolic myopathies: a clinical approach; part I.

Children and adults with metabolic myopathies have underlying deficiencies of energy production, which may result in dysfunction of muscle or other energy-dependent tissues, or both. Patients with disorders of glycogen, lipid, or mitochondrial metabolism in muscle may present with dynamic findings (i.e., exercise intolerance, reversible weakness, and myoglobinuria) or progressive muscle weakness, or both. In this first part of the review, we present a brief description of energy metabolism in muscle, a simplified overview of the clinical and laboratory evaluation of the patient with suspected metabolic myopathy, and a diagnostic algorithm aimed at predicting the nature of the underlying biochemical abnormality. The goal is to simplify a complex field of neuromuscular disease and thus lead to early recognition and treatment of these disorders.

Metabolic myopathies: a clinical approach; part II.

Major recent advances in the field of metabolic myopathies have helped delineate the genetic and biochemical basis of these disorders. This progress has also resulted in the development of new diagnostic and therapeutic methodologies. In this second part, we present an updated review of the main nonlysosomal and lysosomal glycogenoses and lipid metabolism defects that manifest with signs of transient or permanent muscle dysfunction. Our intent is to increase the pediatric neurologist's familiarity with these conditions and thus improve decision making in the areas of diagnosis and treatment.

Management and outcome of choanal atresia correction.

OBJECTIVES: Outcome analyses of factors that may either maximize success or predict a better outcome following choanal atresia correction. METHODS: A retrospective review of children undergoing choanal atresia correction at Great Ormond Street Hospital for Children, London between January 1990 and April 1998. Children with unilateral or bilateral choanal atresia were studied. In all cases, correction was by a transnasal approach under endoscopic control. A 120 degrees Hopkins rod telescope was used to visualize the atretic plate from the nasopharynx. Straight urethral sounds were used to perforate the plate followed by use of an air drill to remove the bony component. Portex endotracheal tubes were subsequently inserted as nasal stents. RESULTS: Sixty-five children (19 M, 46 F: age range 1 day to 17 years) presented with choanal atresia and the outcomes for 46 were included in the study. Twenty-six patients (40%) had other major anomalies. In children with unilateral atresia neither duration of stenting nor presence of facial anomalies had an impact on outcome. Of those children with bilateral choanal atresia and associated facial anomalies (n=9), 56% were asymptomatic following correction. In children with isolated bilateral choanal atresia (n=19), 74% were asymptomatic following correction; 29% (n2.3 kg (n3. 5 mm (n=6) had an 83% chance of a good outcome. Those patients stented with a tube

Effect of basic fibroblast growth factor on perforated chinchilla tympanic membranes.

Basic fibroblast growth factor (bFGF) is a polypeptide mitogen which stimulates proliferation of epidermal and connective tissue cells. When applied to tympanic membrane perforations it has been reported to enhance healing and produce connective tissue hyperplasia. Previous work with animal models has shown that hyperplastic alterations of the tympanic membrane play an essential role in cholesteatoma development. This study was designed to further investigate the hyperplastic effects of bFGF and to determine if it might induce cholesteatoma formation during the healing process. Ten chinchillas received bilateral tympanic membrane perforations. In each animal, three doses of bFGF (400 nanograms per dose) were applied to the perforated tympanic membrane on one side; the opposite (control) ear received saline alone. The animals were terminated at either two or four weeks and studied histologically. Although the dosage and administration schedule used were consistent with previous studies utilizing other rodent species, there was little evidence that bFGF affected tympanic membrane healing in chinchillas. In both control and bFGF-treated ears, dense connective tissue occupied the lamina propria of the tympanic membrane, providing an effective barrier against ingrowth of skin toward the middle ear. No cholesteatomas developed in any animals included in the study. The results of this work indicate that the risk of cholesteatoma formation following administration of bFGF is minimal when it is applied short-term to acute perforations.

Outpatient adenotonsillectomy. Is it safe in children younger than 3 years?

OBJECTIVE: To evaluate the safety of ambulatory adenotonsillectomy in children younger than 3 years. MATERIALS AND METHODS: The records of 102 children younger than 3 years who underwent adenotonsillectomy as an outpatient procedure were reviewed during a 3-year period. RESULTS: Ten patients (10%) required overnight hospital admission for an average of 1.4 days. Nine patients were admitted directly from the day-stay unit and 1 patient was admitted 48 hours after surgery. The reason for hospital admission was poor oral intake. None of the patients had postoperative bleeding or respiratory problems or required intensive care unit admission. CONCLUSION: The safety of ambulatory adenotonsillectomy depends on judicious selection criteria and can be performed in children younger than 3 years.

Peritonsillar abscess in early childhood. Presentation and management.

OBJECTIVE: To highlight the modes of presentation and management of a peritonsillar abscess in children younger than 5 years. DESIGN: Retrospective case series. SETTING: Tertiary referral pediatric otolaryngology practice. PATIENTS: Seven children younger than 5 years. RESULTS: The mean age of the children studied was 27 months (age range, 7-41 months). Five (71%) of the 7 patients underwent computed tomographic scanning to confirm the diagnosis. Pus was cultured at surgery in every case. The most common organism detected was Streptococcus viridans. The average hospital stay was 72 hours (range, 22 hours to 12 days). After diagnosis of an abscess, all patients underwent an electrocautery tonsillectomy and had an uneventful recovery. CONCLUSIONS: Children younger than 5 years who present with poor oral intake, high fever, drooling, and trismus should be suspected of having a peritonsillar abscess. A computed tomographic scan of the neck is usually required to confirm a suspected diagnosis. Prompt diagnosis and treatment will lead to a considerable decrease in morbidity. Immediate tonsillectomy is a safe and effective means of abscess drainage.

Safety parameters for avoiding acute ocular damage from the reflected CO2 (10.6 microns) laser beam.

Reflections from instruments in the surgical field involving the CO2 laser beam present a serious ocular hazard. In addition to the use of plastic or glass protective eyewear, this hazard can be minimized by utilizing anodized instruments and recognizing the specific distances at which various reflections are no longer hazardous depending upon certain variables, including laser output wattage, emergent beam lengths, and surface characteristics of the reflecting instruments.