·
The hypothesized pathogenesis of Swyer-James syndrome involves a viral
infection that arrests growth of an affected lung through an obliterative
bronchiolitis. This results in hyperlucency from air trapping and
diminished arterial flow. Traditionally, this was thought to be a unilateral
process, but CT examinations often demonstrate scattered areas of bilateral air
trapping.
·
hereditary
syndromes associated with renal cysts: Tuberous
sclerosis, von Hippel-Lindau
disease, Zellweger's
(cerebrohepatorenal) syndrome, Jeune's
syndrome (thoracic asphyxiating dystrophy), and Meckel-Gruber
syndrome.
·
Uterine and ovarian volumes are larger
than normal in true isosexual precocious puberty,
whereas enlargement of only the ovary has been reported with pseudosexual precocity. True isosexual
precocity is due to the premature activation of the hypothalamic pituitary
gonadal axis, whereas pseudosexual precocity refers to pubertal changes
occurring independent of these actions, as in the case of a functional ovarian
cyst or tumor. Multiple, small (less than 1 cm) cysts can be seen in the
ovaries of normal children, as well as in children who have precocious puberty.
However, the best predictor of true precocious
puberty is bilateral ovarian enlargement, but unilateral ovarian enlargement
associated with larger cysts is more related to pseudosexual precocity.
·
Normal neuronal
migration occurs during fetal brain development as neurons migrate
from the germinal matrix to the cortex along radial glial fibers. Partial or
total arrest of this process leads to a migrational anomaly. These anomalies
may include subependymal heterotopias (abnormal gray matter lining the
ventricles), band heterotopias (an extra band of gray matter exists
partially or completely underneath a normal-appearing cortex and a strip of
white matter), schizencephaly (a cleft lined by gray matter extending
from the outer cortex to the ventricles), polymicrogyria (too many
gyri), pachygyria (abnormally thickened gyri), or lissencephaly
(complete absence of gyri). Children typically present with developmental delay
or seizures.
·
GRADING OF
GERMINAL MATRIX HEMORRHAGE IN NEONATES
1.
Grade I: confined to the caudothalamic groove
2.
Grade II: extension into the lateral ventricle
3.
Grade III: extension into and dilation of the lateral
ventricle
4.
Grade IV: extension into the adjacent parenchyma
·
The corpus
callosum develops front to back, except for the rostrum (anterior
genu, body, splenium, and rostrum last). This is important because an in utero
insult may result in destruction of part of the corpus callosum. If posterior
portions of the corpus callosum are present and anterior portions are not, this
means that they were present at one point and were destroyed. If posterior
portions are absent instead, this may mean that they did not develop.
·
Holoprosencephaly
may follow an atypical pattern of corpus callosum development, in
which the posterior portions develop and the anterior portions do not. This is
termed atypical callosal dysgenesis.
·
The pituitary
stalk is considered thickened if it is greater than 2 mm at the
insertion of the gland. Close interval follow-up is warranted because this
thickening may be a manifestation of a germinoma
or Langerhans cell histiocytosis. Alternatively, the thickening may
be idiopathic.
·
Trilateral
retinoblastoma involves tumor in both eyes as well as in the
pineal gland. As in the retina, even a fleck of calcium in the pineal
gland of a child younger than 6 years old should be considered suggestive of
neoplasm.
·
Fibromatosis
colli is benign focal or fusiform enlargement of the
sternocleidomastoid muscle in infants that usually regresses by 6-8
months of age and is thought to be due to trauma to the sternocleidomastoid
muscle or to an in utero positioning abnormality. Ultrasound of the muscle
demonstrates either diffuse enlargement or a focal hyperechoic mass. If there
is question about the diagnosis, MR may be of use. MR should demonstrate low
signal on T2-weighted images, which represents fibrosis in the
sternocleidomastoid muscle.
·
CHARACTERISTIC AGES
OF PEDIATRIC HIP DISORDERS
1.
Developmental
dysplasia is a disorder of infants.
2.
Legg-Calvé-Perthes
disease occurs in children 3-12 years of age.
3.
Slipped capital
femoral epiphysis occurs in children 10-15 years old. This disorder
cannot occur once the physes have fused!
·
LESIONS THAT
SHOULD PROMPT A WORK-UP FOR CHILD ABUSE
- Metaphyseal corner fractures (highly specific and sensitive for abuse)
- Posterior rib fractures
- Fractures of spinous processes, scapula, or sternum or compression fractures of vertebral bodies
- Subdural hematomas, especially midline
- Duodenal hematoma and pancreatitis
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