PEDIATRIA

Question 1

STURGE WEBER CEREBRAL

Answer 1

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.

Question 2

ESCLEROSIS TUBEROSA

Answer 2

-Hamartomas/tubérculos corticales y subependimarios.
-Rabdomioma cardiaco.
-Angiomiolipomas renales.
-Descartar linfangioleiomiomatosis pulmonar

Question 3

NIÑO CON ECTASIA CONGÉNITA DE LA VÍA BILIAR

Answer 3

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)

Question 4

QUISTES ARACNOIDEOS BILATERALES EN PEDIATRIA

Answer 4

Raro, se asocia a nf1 y aciduria glutática tipo 1

Question 5

ATRESIA DE COANAS

Answer 5

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

Question 6

PYRIFORM APERTURE STENOSIS

Answer 6

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

Question 7

JUVENILE NASOPHARYNGEAL ANGIOFIBROMA

Answer 7

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)

Question 8

¿Which is the most common soft tissue sarcoma in childhood?

Answer 8

Nasal rhabdomyosarcoma

Question 9

Steeple sign

Answer 9

Croup or laryngotracheobronchitis

Loss of the normal shouldering of the subglottic trachea

Question 10

Omega epiglottis

Answer 10

Apparently thickened normal epiglottis in an oblique angle image

Question 11

Eppiglotitis

Answer 11

Haemophilus influenzae

Thickening of the epiglottis

Thumbprint sign

Question 12

EXUDATIVE TRACHEITIS

Answer 12

Candle dripping sign

6-10 years

S aureus

Intrluminal membranes in subglotic and cervical trachea

Question 13

Asymmetric narrowing of the subglottic trachea on frontal radiography

Answer 13

Subglottic hemangioma

(Simmetric narrowing in croup)

Question 14

Subglottic hemangioma Association

Answer 14

PHACES

Posterior fossa malformation
Hemangioma
Arterial anomalies
Coarctation/cardiac defects
Eye abnormalities
Sternal cleft, supraumnilical raphes

Question 15

LARYNGEAL PAPILLOMATOSIS

Answer 15

Multiole laryngeal nodules due to VPH infection, increasing laryngeal squamous cell carcinoma

Question 16

TRACHEOBRONCOMALACIA

Answer 16

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

Question 17

The three most important vascular causes of stridor

Answer 17

• double aortic arch
• right arc with aberrant left subclavian artery
• pulmonary sing

Question 18

INNOMINATE ARTERY SYNDROME

Answer 18

The large thymus can occasionally cause the normal innominate artery to preds against the anterior trachea, potentially producing innominate artery syndrome

Question 19

ECN CAUSES

Answer 19

Pre-term infants
Term infants: heart disease, Hirschprung disease, umbilical venous catheter

Question 20

Lissencephaly and types

Answer 20

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)

Question 21

Polymicrogyria y más frecuente

Answer 21

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

Question 22

Secondsry abnormalities in agenesis of the corpus callosum

Answer 22

• 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

Question 23

Schizencephaly

Answer 23

Full thickness cleft of the cerebral hemosphere lined by dysplastic gray matter (usually from polymicrogyria)

Question 24

Hallazgos del Chiari tipo II

Answer 24

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

Question 25

Regla pnemotécnica de NF2

Answer 25

No es lo MISME que la NF1

Multiple
Schwannomas
Meningiomas
Ependimomas

Question 26

Síndrome de Joubert

Answer 26

Apariencia “‘Molar tooth” del troncoencéfalo, con aplasia/hipoplasiq del vermis y a veces tejido cerebeloso displásico. Los niños presentan ataxia, retraso del desarrollo e hipotonía.

Buscar anomalías oftalmológicas.