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!
Retropharyngeal abscess - what do you look for?
Jugular vein thrombosis, septic emboli (Lemierre syndrome), ICA spasm
Ddx for no radius:
TAR (thrombocytopenia with absent radius) VACTERL Holt Oram cleidocranial dysostosis fanconi anemia (hypoplasia) Trisomy 18
Lucent skull lesion in a child
LCH, fibrous dysplasia, osteomyelitis, lymphoma, mets
Normal pattern/order of myelination:
bottom to top
back to front
central to peripheral
Complete around age 2 or so
Chest xray featuers of surfactant deficiency aka hyaline membrane disease
Low lung volumes (can be high IF intubated), premature infant, ground glass/granular opacities, air-bronchograms, NO pleural effusion, bilateral and symmetrical
complications of treating HMD / surfactant deficiency:
Acute persistent patent ductus arteriosus (PDA) due to reduced oxygen stimulus barotrauma (from treatment) oxygen toxicity (from treatment) pulmonary hemorrhage (can also included in the differential diagnosis) Chronic bronchopulmonary dysplasia pulmonary interstital emphysema (PIE) recurrent pulmonary infection subglottic stenosis (from intubation)
Features of meconium aspiration on neonatal chest xray
coarse, asymmetric, patchy densities full-term or post-term high lung volumes \+/- pleural effusions up to 40% have pneumothorax
chest xray features of TTN
interstitial edema, small pleural effusions
more common with C-section delivery
always resolves in 1-2 days
What are the pulmonary manifestations of CF?
bronchiectasis
pneumothorax
recurrent bacterial infection
pulmonary arterial hypertension
What are the abdominal manifestations of CF?
distal intestinal obstruction syndrome (DIOS) - sort of the meconium ileus of older children/young adults meconium ileus: 10 - 20% rectal prolapse cirrhosis and hepatic steatosis esophageal dysfunction / GERD pancreatic insufficiency fatty replacement of pancreas
Head and neck manifestations of CF:
chronic sinusitis
nasal polyposis
Germinal matrix hemorrhage - grades:
1: GM hemorrhage
2. GM hem. + blood in vents
3. GM hem with dilated vents
4. Hemorrhage in brain (+/- GM hem)
What abnormality(ies) are associated with multicystic dysplastic kidney?
Contra lateral ureteral reflux or UPJ obstruction
Sometimes is bilateral = incompatible with life
thin-walled lung cyst in a child
Post-infectious pneumatocele, CPAM
enlarged unilateral SCM - called? Treatment?
Fibromatosis coli
Rx is physiotherapy (not surgery)
Ddx frayed metaphysis
Rickets!!
hypophosphatasia, stress eg. gymnast, copper deficiency
Ddx sclerotic bones in an infant
osteopetrosis (fatal 2/2 anemia, bone-in-bone) physiologic up to 2 mos old TORCH inxn pyknodysostosis skeletal dysplasia
beaking of the medial tibial physis, lateral knee subluxation and varus alignment:
Blount’s dz - can be in toddlers or adolescents. Thought maybe related to repatative stress. Toddlers - conservative Rx
Teenagers: may need osteotomy
Baby has 11 pairs of ribs, heart disease =
Trisomy 21 / Down’s syndrome
Radiographic features that differentiate Wilms from Neuroblastoma
Wilms: claw sign from kidney, only 10% calcifiy, invades vasculature, mets to IVC, lungs, liver
Neuroblastoma: punctate calcification 90%, encases vessels, mets to bone
Hip joint effusion in child - Ddx:
infection vs toxic synovitis (self-limited synovitis)
Ddx for gibbus deformity of vertebra in a child
Trauma, mets/LCH, congenital (segmentation), TB, achondroplasia, mucopolysacharidosis (hunters, hurlers), scheuerman’s (thoracic)
Ddx widened intercondylar notch in a child:
hemophilia, JIA (look for widened epiphyses and osteopenia from hyperemia)
Ddx expanded bones (child)
Mucopolysacharidosis (hunter, hurler, Morquio), sickle cell, thalassemia, gaucher’s
Solid, well-circumscribed, highly vascular mass in an infant:
Hemangioma
How do you differentiate Coat’s dz from PHPV?
PHPV - small globe
Coat’s - dense, normal sized globe. Usually in boys under 10 (retinal vascular abnl)
What renal tumor is most likely in an infant?
Mesoblastic nephroma - a benign hamartomatous lesion - resect.
proper course and positioning of an umbilical artery catheter
Course: umbilical artery, hypogastric, iliac, aorta
Should terminate tebween T6 and T12
OR low: below the IMA (below L3)
Proper course and positioning of an umbilical vein catheter
umbilical vein, left portal vein, ductus venosus, middle or left hepatic vein, and into the inferior vena cava
Proper position is at jctn IVC and RA
Child neck film: symmetric subglottic stenosis
asymmetric subglottic stenosis
What about a thick “thumb” appearance?
-Croup infants (Viral -H. Flu, paraflu) - airway emergency! Look for overdistended hypopharynx
-Membranous Croup - bacterial (staph) inflammation of larynx, trachea and bronchi
asymmetric - hemangioma, papilloma, or granuloma (prior intubation)
Thumb = epiglottitis (3-6y, emergency)
Radiologic signs of child abuse:
Intracranial bleeds of different ages, DAI, retinal hemorrhage
corner/bucket handle fracture
posterior and lateral rib fx
fx sternum, scapula, spiral fx long bones in non-weight-bearing infant
Retroperitoneal hematoma
What is Potter sequence/syndrome?
The consequence of oligohydramnios in utero
pulmonary hypoplasia
growth restriction (IUGR)
abnormal facies (Potter facies
Caused by bilateral agenesis, MCDK, UPJ, or ARPCKD
Ddx large liver in a neonate
hepatoblastoma, neuroblastoma, hepatitis, TAPVR
Rasmussen encephalitis:
a chronic inflammatory disease of unknown origin, usually affecting one brain hemisphere
Late: cortical atrophy
kids under 10, usually intractable epilepsy
What do you do next when you suspect osteosarcoma or Ewings?
MRI of whole bone
Bone scan for mets/synchronous
Chest CT for lung mets
Ddx for fragmentation of proximal femoral epiphysis
AVN (e.g. Legg-Calve-Perthes) Meyer dysplasia (normal variant, not painful)
Flat, sclerotic navicular bone in foot =
What should you do next?
AVN - Kohler disease
Usually self-limited and heals, but may get a cast for a few weeks
Posteromedial bowing of the tibia
idiopathic (don’t worry)
Oar-shaped ribs are seen in:
mucopolysaccharidoses
Causes of rickets:
dietary deficiency, dilantin, breast feeding, liver, renal, GI, Primary hypophosphatemia
Ddx cortical thickening in a child
Osteoid osteoma, chronic osteomyelitis
What problems are associated with a R aortic arch?
TGA, TOF, truncus
Rarely isolated
Staging of neuroblastoma
Stage 1: local, completely excised
Stage 2: local, incompletely excised (2B ipsilateral nodes)
Stage 3: crosses midline or contralateral nodes
Stage 4S: < 1 year of age
localised tumour (stage 1, 2A or 2B)
distant metastases confined to skin, liver and/or bone marrow
Stage 4: Distant mets (and not 4S)
Staging of Wilm’s tumor
Stage 1: in kidney
Stage 2: kidney + renal vein (can be resected)
Stage 3: nodes or peritoneal spread (can’t be resected)
Stage 4: mets
Stage 5: bilateral (then stage each separately)
Fetal or neonatal head ultrasound - bright spots around ventricles
Calcification - TORCH, esp CMV or toxo
Prior hemorrahge
Coronary aneurysm in a child
Kawasaki disease
