PEDIATRIA Flashcards
(26 cards)
STURGE WEBER CEREBRAL
La causa es un fallo en la regresión del plexo venoso primitivo craneal que resulta en angiomatosis venosa leotomeningea.
Sospechamos ante realce leptomeníngeo, calcificaciones corticales y atrofia principalmente posterior. Mirar si buftalmos (hemangioma coroideo que realza y aumenta tamaño del globo ocular).
Puede asociar hipertrofia compensadora del plexo coroideo homolateral.
ESCLEROSIS TUBEROSA
-Hamartomas/tubérculos corticales y subependimarios.
-Rabdomioma cardiaco.
-Angiomiolipomas renales.
-Descartar linfangioleiomiomatosis pulmonar
NIÑO CON ECTASIA CONGÉNITA DE LA VÍA BILIAR
Enfermedad de Caroli
Ectasia congénita de la vía + cálculos intrahepáticos + estructuras quísticas que comunican con el árbol biliar + realce dentro de los conductos intrabiliares (signo del punto central)
QUISTES ARACNOIDEOS BILATERALES EN PEDIATRIA
Raro, se asocia a nf1 y aciduria glutática tipo 1
ATRESIA DE COANAS
Oclusión congénita de la parte posterior de la cavidad nasal.
El 30% es óseo; el 1% membranoso y el 70% mixto.
Se asocia con síndrome de CHARGE
- coloboma
- heart deffects
- atresia of choanae
- retardation of development
- genitourinary anomalies
- ear anomalies
PYRIFORM APERTURE STENOSIS
Is a rare but potentially lethal cause of congenital upper airway obstruction, may mimic choanal atresia.
Caused by early hyperthrophy of the medial nasal processes. Piriform aperture less than 11 mm. Hypoplasia del paladar duro.
Associated to holoprosencephaly, central incisor tooth and pituitary dysfunction
JUVENILE NASOPHARYNGEAL ANGIOFIBROMA
Highly vascular, bening hamartomatous lesion in adolescent males. Originates in sphenopalatine foramen and spreada into the nasopharynx and pterygopalatine fossa causing bone remodeling.
Differential diagnosis with rhabdomyosarcoma (bone destruction)
¿Which is the most common soft tissue sarcoma in childhood?
Nasal rhabdomyosarcoma
Steeple sign
Croup or laryngotracheobronchitis
Loss of the normal shouldering of the subglottic trachea
Omega epiglottis
Apparently thickened normal epiglottis in an oblique angle image
Eppiglotitis
Haemophilus influenzae
Thickening of the epiglottis
Thumbprint sign
EXUDATIVE TRACHEITIS
Candle dripping sign
6-10 years
S aureus
Intrluminal membranes in subglotic and cervical trachea
Asymmetric narrowing of the subglottic trachea on frontal radiography
Subglottic hemangioma
(Simmetric narrowing in croup)
Subglottic hemangioma Association
PHACES
Posterior fossa malformation
Hemangioma
Arterial anomalies
Coarctation/cardiac defects
Eye abnormalities
Sternal cleft, supraumnilical raphes
LARYNGEAL PAPILLOMATOSIS
Multiole laryngeal nodules due to VPH infection, increasing laryngeal squamous cell carcinoma
TRACHEOBRONCOMALACIA
Excessive expiratory airway collapse from cartilage weakness (congenital, secondary to intubarion, infection, etc).
Reduction greater than 50% in expiratory CT or bronchoscopy
Association with atresia/tracheoesophageak fistula
The three most important vascular causes of stridor
- double aortic arch
- right arc with aberrant left subclavian artery
- pulmonary sing
INNOMINATE ARTERY SYNDROME
The large thymus can occasionally cause the normal innominate artery to preds against the anterior trachea, potentially producing innominate artery syndrome
ECN CAUSES
Pre-term infants
Term infants: heart disease, Hirschprung disease, umbilical venous catheter
Lissencephaly and types
Lissencephaly is characterized by absent or decressed cortical convolutions causing a smootj and thickened cortical surface.
Type I (classical): smooth cortex and an hour-glass configuration to the cerebral hemospheres
Type II (cobblestone): finely undulating cortex (serrated interface at the gray-white junction)
Polymicrogyria y más frecuente
Malformation of brain development by disturbance either late in neuronal migration or esrly lamellar organization.
The cortex appear thick with multiple small individual gyri and too few sulci.
Bilateral perisylvian polymicrogyria is the most common distribution
Secondsry abnormalities in agenesis of the corpus callosum
- colpocephaly (dilatation of the occipital horns of the lateral ventricles) and decressed white matter volume.
- parallel lateral ventricles.
- medial impressions on the lateral ventricles caused bu Probst bundles.
- Enlarged third ventricle
Schizencephaly
Full thickness cleft of the cerebral hemosphere lined by dysplastic gray matter (usually from polymicrogyria)
Hallazgos del Chiari tipo II
Herniation of the cerebellar vermis, cerebellar tonsils and medulla throug the foramen magnum with resultant beaking of the tectum.
Myelomeningocele universally present.
The fourth ventricle is low in position and elongated. 80-90% de los niños tienen hidrocephalus due to 4th ventricule obstruction
