Pediatrics Flashcards
Ddx long bone lesion in 2-year-old
Toddler’s fx, osteomyelitis, LCH, fibrous dysplasia, osteoid osteoma
What’s Legg-Calve-Perthes disease?
idiopathic AVN of femoral epiphysis
age 4-8 years
10% bilateral
asymmetry then fragmentation of epiphysis
Currarino triad:
anorectal malform (imperforate anus)
presacral mass: meningocele, teratoma, lipoma, dermoid, or enteric cyst
partial sacral agenesis
VACTERL =
V : vertebral anomalies : hemivertebrae congenital scoliosis caudal regression spina bifida A : anorectal anomalies : anal atresia C : cardiac anomalies / cleft lip T : tracheo- E : oesophageal fisula + / - oesophageal atresia R : radial ray anomalies / renal anomalies L : limb anomalies polydactyly oligodactyly
vessel between trachea and esophagus =
pulmonary sling (left PA arises from R PA and courses posterior to the trachea
Age of pyloric stenosis:
Numbers:
Age: 2-8 weeks
Male: female 4:1
Muscle thicker than 3mm
Length of pyloric channel >17mm
Ddx for microcolon:
Meconium ileus distal bowel atresia Hirshprung's (total colonic) Microcolon of prematurity Megacystitis-microcolon hypoperistalsis syndrome
What’s meconium ileus? What’s the difference between meconium ileus and meconium plug?
Meconium ileus is abnormally thick, impacted meconium in the ileum, usually related to CF
Meconium plug is a functional colonic obstruction in a newborn due to an obstructing meconium plug. It is usually transient and affects the left colon with meconium plugging the bowel distal to this segment. Small left colon syndrome is a subset of meconium plug syndrome (for most purposes is synonymous)
Enema on newborn shows colon small from splenic flexure distally
what’s it associated with?
Small left colon
Functional obstruction due to immaturity of myenteric plexus
Assoc. maternal DM, substance abuse
Ddx: hirschprung’s
ddx Abdominal cyst in a fetus or child
GI duplication cyst choledochal cyst ovarian cyst (on pedicle)
Ddx for SBO in a new born: … an infant: …in a child?
NB: intestinal atresia, Hirschprung’s disease, small left colon, meconium plug, meconium ileus, midgut volvulus
Infant-2 year old: Incarcerated hernia, Hirshprung’s, malrotation, intussusception
Older child: appendicitis, intussusception, malrotation, adhesions
Neuroblastoma - who gets it?
infants and very young children (mean age of presentation being ≈ 22 months) with 95% of cases <10 years. Occasionally identified antenatally or at birth Beckwith-Wiedemann syndrome DiGeorge syndrome Hirschsprung disease NF1
Beckwith-Wiedemann syndrome: what is it? Features?
congenital overgrowth disorder characterised by a unique set features which can consist of :
macroglossia (most common clinical finding)
omphalocoele
localised gigantism / macrosomia
hemihypertrophy
organomegalies (nephromegaly, hepatosplenomegaly)
Assoc. with *Neuroblastoma and *Wilm’s, hepatoblastoma
DiGeorge syndrome
cleft lip - / + palate
congenital heart disease (particularly conotruncal anomalies): often a major part of this syndrome- tetralogy of Fallot, interrupted aortic arch, truncus arteriosus
charcateristic facies : elongated face
Ddx for nasopharyngeal tumor in teenager
JNA
lymphoma
rhabdomyosarcoma
nasopharyngeal carcinoma
Newborn with liver mass
Hepatoblastoma or Congenital hemangioma
Hepatoblastoma: fat and osteoid elements, no AV shunting, peak age 18-24 mos. Get chest CT to look for mets
congenital hemangioma (aka Hemangioendothelioma): large endothelial-lined vascular channels (much AV shunting), fetus or neonate
What completes the ring in double aortic arch?
Ligamentum arteriosum
Sacrococcygeal teratoma - tell me about it
most common congenital tumor
80% in girls
70% benign, rest malignant
classified based on intrapelvic involvement
smooth brain with hourglass configuration
Lissencephaly - extreme form of migrational abnormality (do not confuse with normal appearance of preterm brain)
Pts usually present in status epilepticus
features of cleidocranial dysostosis
Skull: wormian bones, brachycephaly
Chest: absent/hypoplastic clavicles
Limbs: short / absent fibula, short / absent radius
Widened pubic symphysis
What are the common craniosynostoses? Describe each
- dolichocephaly/scaphocephaly (elongated): sagittal suture (most common)
-brachycephaly (round, wide): coronal and/or lamboid sutures
-plagiocephaly (asymmetric): unilateral coronal and lambdoid sutures
-trigonocephaly (pointy forehead, triangular head): metopic suture
(many more)
What solution do you use for an enema in an infant?
Cysto Conray, 400 mOsm
Congenital heart disease: increased pulmonary blood flow, not cyanotic ddx:
Shunts:
ASD (not enlarged)
heart enlarged: VSD, PDA, (ECD) endocardial cushion defect
Peripheral shunt (vein of Galen, congenital hemangioma of liver)
Congenital heart disease: increased pulmonary blood flow, cyanotic ddx:
The "Ts" Transposition TAPVR Truncus arteriosus Tricuspid Atresia w large VSD Single ventricle
Congenital heart disease: decreased pulmonary flow (cyanotic)
"right heart problems" TOF (not enlarged) Enlarged: Pulmonary atresia Tricuspid Atresia w small VSD Ebstein's anomaly (huge heart)
What’s usually the diagnosis for “snowman heart”
supracardiac TAPVR (dilated SVC and left vertical vein)
Pneumomediastinum in a child ddx
Foreign body aspiration
asthma
blunt trauma
Most common orbital mass in a child:
hemangioma
Ddx for aggressive lesion in retrobulbar space:
mets (neuroblastoma), LCH, rhabdomyosarcoma, lymphoma
vascular subcutaneous lesion over the skull that communicates with the dural venous sinuses
Sinus pericranei
What is PHACES syndrome?
•P : posterior fossa malformations •H : haemangiomas •A : arterial anomalies •C : coarctation of the aorta and cardiac anomalies •E : ocular anomalies S: sternal clefting
Ddx for VERY large heart in an infant or child
Ebstein’s, pulmonary atresia, cardiomyopathy
Pt with bilateral optic gliomas - what do they probably have?
NF1
pneumothorax in a child
asthma, pleural blebs, rib fractures, (infant: meconium aspiration)
Always look for tension!