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Pedi Neuro

:pediatric_astrocytomas.jpg :pediatric_misc_supratentorial.jpg :pediatric_extraaxial.jpg

External Hydrocephalus

  • Benign extraaxial collections of infancy

Cholesteatoma Types

  • Primary/Congenital
    • Epitympanum
  • Secondary/Acquired
    • Mesotympanum

Mineralizing Vasculopathy

  • Branched-linear pattern of echogenicity in the region of the basal ganglia and thalami on echoencephalogram
  • Follow the course of the lenticulostriate branches of the middle cerebral arteries
  • Secondary to a cerebrovasculitic response to congenital infection (most often ascribed to rubella, CMV, and syphilis), anoxia, or rarely trisomy 13

DDx Scalp Fluid Collection

  • Cephalohematoma
  • Caput succedaneum
  • Leptomeningeal cyst
    • related to dural tear
  • Subgaleal hematoma

DDx Stenosis @ Aqueduct Level

  • Aqueductal stenosis
  • Tectal glioma

Craniosynostosis

  • Scaphocephaly - Early fusion of the sagittal suture
  • Anterior plagiocephaly - Early fusion of 1 coronal suture
  • Brachycephaly - Early bilateral coronal suture fusion
  • Posterior plagiocephaly - Early closure of 1 lambdoid suture
  • Trigonocephaly - Early fusion of the metopic suture (metopic suture fuses between 6-8 months and sometimes as early as 3 months)

Pedi Cardiac

  • Proper location for umbilical artery catheter = T8 - T12; alternative at L3 - L4
  • Umbilical vein catheter tip should be at the right atrium

Pedi Cardiac DDx

Acyanotic, normal vascularity Acyanotic, increased vascularity Cyanotic, decreased vascularity Cyanotic, increased vascularity
Aortic Stenosis ASD (no LAE) Tets (normal heart size, boot shaped)TAPVR (Type 3 has nl heart size)
Pulmonic Stenosis ECD (no LAE) Ebstein Transpostion
Coarctation VSD Pulmonic Atresia
(w/ intact ventricular septum)
Truncus Arteriosis
Interrupted Aortic Arch PDA Tricuspid Atresia w/ PS Tricuspid Atresia w/o PS
Cardiomyopathy/Pericardial Eff. Single Ventricle/Double Outlet RV
  • I classify Pulmonic Atresia w/ VSD as a severe form of tetralogy
  • Right Arch common w/ TGV, Truncus, Tets, Tricuspid Atresia (basically the cyanotic, increased vascularity ddx plus tets and minus TAPVR)
  • Increased pulmonary flow, a right arch and cardiomegaly (biventricular) is classic for truncus arteriosus
  • Hypoplastic Left Heart Syndrome has cardiomegaly, pulmonary edema, a small aorta, and is cyanotic

Cardiac Tumors in Children

  • Rhabdomyoma (small, multiple)
  • Fibroma
  • Myxoma

Dilitation of Aortic Root

  • Connective tissue disorders
  • Takayasu's
  • Syphilitic aortitis
  • Neurofibromatosis
  • FMD
  • Giant cell arteritis
  • Williams Syndrome

Tetralogy of Fallot

  1. Overriding Aorta
  2. VSD
  3. Pulmonary Stenosis
  4. Right Ventricular Hypertrophy

Types of TAPVR

  • Type 1
    • cardiomegaly and increased pulmonary vascularity
    • widened mediastinum (“snowman heart”) due to vertical vein
  • Type 2
    • cardiomegaly and increased pulmonary vascularity
    • simulates shunt physiology
    • blood returns via pulmonary veins → coronary sinus → RA
  • Type 3
    • normal heart size, increased pulmonary vascularity
    • blood returns via infradiaphragmatic systemic venous connection (IVC, hepatic vein, portal vein, etc.)

Newborn w/ CHF

  • Aortic Anomalies
    • aortic stenosis
    • coarctation
  • LV dysfxn
    • aberrant L coronary artery
    • myocarditis
    • hypoplastic L heart syndrome
  • Glycogen Storage Disease (Pompe)
  • High Flow States
    • vein of galen
    • hemangioendothelioma
    • anemia
    • polycythemia

Most Common Vascular Rings

Double Aortic Arch Right Arch w/ Aberrant L Subclavian Right Arch w/ Mirror Image Branching
(Not a vascular ring)
Double Aortic Arch Right Arch w/ Aberrant L Subclavian Right Arch w/ Mirror Image Branching

L Arch w/ Aberrant R Subclavian

  • dysphagia lusoria
  • low association w/ congenital heart disease
  • not a true ring

Pedi Chest

Lung Lesions

Lucent

  • CCAM (Type 1)
  • CLE
  • CDH
  • PIE

Solid

  • CCAM (Type 3)
  • sequestration
  • bronchgenic cyst

Complications of Cystic Fibrosis

  • Recurrent Infections → Pseudomonas and Staph
  • Lung Fibrosis
  • Progressive Respiratory Failure
  • Clubbing
  • Pancreatic Insufficiency
  • Liver Cirrhosis
  • Rectal Prolapse
  • Meconium Ileus, Meconium Peritonitis, Intussusception
  • Sinusitis
  • Infertility in Males

Hyperlucent Lung

  • foreign body
  • PTX
  • congenital lobar emphysema
    • most common LUL > RML > RUL
  • pulmonary artery hypoplasia
  • compensatory hyperinflation b/c of bad c/l lung

Neonatal Lung Infiltrates

Surfactant Deficiency TTN Meconium Aspiration Neonatal Pneumonia
Typical Patient Preemie Term/C-section Post-term PROM
Time Course < 6 hrs 24-48 hrs 12-24 hrs <6 hrs
Lung Volume Decreased Increased Increased Increased
Imaging Ground-glass Interstitial Edema Coarse, Nodular, Asymmetric Streaky, Perihilar

TTN

  • early (2-6 hr) prominent vascular markings with patchy round alveolar opacities and pleural fluid
  • normal to large lung volumes, mild CM, rapid clearance by 42-78hrs, starting by 10-12hrs

RDS/HMD/Surfactant Deficiency

  • Low lung volumes
  • Ground-glass opacities

Meconium Aspiration

  • patchy, bilateral, asymmetric coarse opacities, representing areas of SSA
  • marked hyperaeration - 25% get PTX and pneumomediastinum
  • CXR clearing takes several weeks & lags clinical improvement
  • treatment is ECMO if severe

Neonatal PNA

  • GBS looks like RDS, with bilateral granular, symmetric opacities and low lung volumes
    • GBS usually has effusions and RDS rarely does
  • other pneumonias resemble meconium aspiration, w/ patchy, asymmetric opacities and hyperaeration
  • organisms - Group B Strep, CMV (viral pneumonitis), Chlamydia

Low Volume Pulmonary Opacification

  • Pneumonia
  • Hyaline Membrane Disease
  • Neurogenic (Brain Damage)

DDx Retrocardiac Mass

  • Neuroblastoma
  • Sequestration
  • Pneumonia
  • Nerve Sheath Tumors

Tracheoesophageal Fistulas

tefistulas.jpg

Pedi GI/GU

Normal Spleen Size

Age Spleen Length (cm)
0-3 mo <6 cm
3-6 mo <6.5 cm
6-12 mo <7 cm
1-2 yr <8 cm
2-4 yr <9 cm
4-6 yr <9.5 cm
6-8 yr <10 cm
8-10 yr <11 cm
10-12 yr <11.5 cm
12-15 yr <12 cm
15-20 yr <12 cm (female)
15-20 yr <13 cm (male)

* Rosenberg, et.al. AJR 1991

Pediatric Liver Masses

<5 yr old

  • hepatoblastoma
    • most common primary malignant liver tumor of childhood, M > F, elevated AFP
    • inhomogeneous appearance
    • associated with Beckwith-Wiedemann syndrome
  • hemangioendothelioma
    • same CT appearance as adult hemangiomas; 50% calcify
    • MR - low T1, very high T2, large flow voids, Gd enhancement like CT
    • often presents w/ CHF or Kasabach-Meritt
  • mesenchymal hamartoma
    • large multilocular cystic mass with thin internal septations
    • septa and any solid portions will enhance
  • neuroblastoma mets

>5 yr old

  • HCC
  • mesenchymal sarcomas
  • mets
  • lymphoma
  • adenoma

Abdominal Cyst

  • Mesenteric Cyst
  • Urachal Cyst
  • Duplication Cyst
  • Choledochal Cyst
  • Abscess
  • Hydronephrosis
  • Multicystic Dysplastic Kidney
  • Cystic Ovarian Mass

Bilateral Dilated Ureters

  • Posterior Urethral Valves
  • Prune-Belly Syndrome
  • Bilateral Megaureter

Wilms vs Neuroblastoma

Mnemonics:

  • Neuroblastoma encases and calcifies (NEC)
  • Wilms invades and lung mets (WILM)
Wilms Neuroblastoma
Origin Kidney Adrenal or Sympathetic chain
Calcification Uncommon (<15%) Common (85%)
Appearance Cystic/Solid Solid
Vessels Displaces & Invades Encases
Mets Lung Early (bone, neural foramina, nodes)
Peak Age 3 years <2 years

* Drash syndrome - Wilms, pseudohermaphroditism, glomerulonephritis

Neuroblastoma Staging

  • 1 - confined to the organ of origin
  • 2 - extension beyond the organ but not crossing midline
  • 3 - extension across midline
  • 4 - distal mets
  • 4S - age <1 year, mets confined to skin, liver, and bone marrow (SLiM)

Wilms Staging

  • 1 - confined to kidney, completely excised
  • 2 - local extension, completely resected
  • 3 - incomplete resection, no distant mets
  • 4 - distant mets to lung, liver, bone, or brain
  • 5 - bilateral synchronous tumors

Hypoperfusion Complex

  • decreased calibur of aorta and IVC
  • intense enhancement of bowel wall, aorta, IVC, pancreas, and kidneys
  • dilated, fluid-filled bowel
  • suggests tenuous hemodynamic state

Eagle-Barrett Syndrome

  • abdominal wall muscle deficiency (“prune belly”)
  • nonobstructed, but markedly dilated redundant ureters ± hydronephrosis ± renal dysplasia
  • bilateral cryptorchidism

Vesicoureteral Reflux

Reflux Grades

  • Grade I: Urine backs up into the ureter only, and the renal pelvis appears healthy, with sharp calyces.
  • Grade II: Urine backs up into the ureter, renal pelvis, and calyces. The renal pelvis appears healthy and has sharp calyces.
  • Grade III: Urine backs up into the ureter and collecting system. The ureter and pelvis appear mildly dilated, and the calyces are mildly blunted.
  • Grade IV: Urine backs up into the ureter and collecting system. The ureter and pelvis appear moderately dilated, and the calyces are moderately blunted.
  • Grade V: Urine backs up into the ureter and collecting system. The pelvis severely dilates, the ureter appears tortuous, and the calyces are severely blunted.

DDx Bladder Thickening

  • hemorrhagic cystitis
  • rhabdomyosarcoma

Pedi MSK

  • Kohler's disease, if asymptomatic, is normal variant called Karp Dysplasia
  • Blount's Disease = Tibia Vara
  • The tarda form of hypophosphatasia mimics rickets radiographically
  • Toddler's Fx - nondisplaced spiral fx of distal tibia

Scoliosis

Risser Grades

:risser.gif

  • Ossification of the iliac apophysis starts at the anterior superior iliac spine and progresses posteromedially.
  • The iliac crest is divided into quadrants, and the stage of maturity is designated as the number of ossified quadrants. For example, 50 percent ossified is a Risser grade 2. On the anatomic left (right side of the figure), all quadrants are ossified and the apophysis is fused to the iliac crest, for a Risser grade 5.

DDx

  • Idiopathic (adolescent)
    • usually females
    • R thoracic ± L lumbar curve
  • Juvenile (3-10 years)
    • idiopathic
    • 2:1 female predominance
  • Infantile
    • convex to L
    • boys

Developmental Dysplasia of the Hip

Risk Factors

  • left hip is more often involved (? due to fetal positioning)
  • females: 80% of cases
  • breech birth (may account for 25-45% of DDH cases)
  • first born
  • fixed positions of extension and adduction
  • Down's syndrome

Plain Film

AP pelvis demonstrating superior and lateral displacement of the femoral heads. Note Hilgenreiner's line, Perkin's line, and acetabular angle.

  • Hilgenreiner's line is drawn thru the triradiate cartilages
  • Perkins line is perpendicular to that, drawn from the anterior inferior iliac spine
  • Femoral head should be in the lower inner quadrant fromed by the lines

Ultrasound

  • Alpha Angle
    • measurement of acetabular concavity
    • angle between the baseline and the roofline
  • Beta Angle
    • measured between the baseline and the inclination line
    • indicates the acetabular cartilaginous roof coverage
  • Type I hips
    • alpha angle > 60°
    • normal
    • require no treatment and no follow-up
  • Type II hips
    • alpha angle = 50-59 degrees
    • hip is normally located, but the bony acetabulum is immature
    • if < 3mos → physiologic immaturity
    • if > 3mos → mild dysplasia
    • no treatment but should be closely observed clinically and with US until they meet type I criteria
    • small risk of delayed displacement or acetabular dysplasia
  • Type III hips (low displacement) and type IV hips (high displacement)
    • beta angle < 55 degrees
    • usually very apparent clinically
    • both require immediate treatment

AVM of Hip

  • causes coxa magna & coxa plana

Dense Metaphyseal Bands

  • Lead Poisoning
  • Heavy Metals
  • Healing Phase of Rickets

Lucent Metaphyseal Bands

  • Blue Cell Tumor (Ewings/Neuroblastoma)
  • Leukemia/Lymphoma
  • Ricketts
  • Rubella
  • Congenital Syphillis → Wimberger Sign

DDx Fragmented Epiphyses (HAMS)

  • hypothyroidism
    • delayed skeletal maturity (bone age more than 2 SD below the mean)
    • small stature
    • wormian bones
    • enlarged sella
    • fragmented epiphyses
    • delayed closure of cranial sutures
  • AVN (few epiphyses involved)
  • multiple epiphyseal dysaplsia
  • spondyloepiphyseal dysplasia

DDx Metaphyseal Fraying (CHARMS)

  • Congenital infections (Syphilis, CMV, Rubella)
  • Hypophosphatasia
  • Achondroplasia
  • Ricketts
  • Metaphyseal dysostosis
  • Scurvy

DDx Erlenmeyer Flask (TOP DOG)

  • Thalassemia (or other severe anemias)
  • Osteopetrosis
  • Pyle's disease (metaphyseal dysplasia)
  • Diaphyseal aclasis (multiple osteochondromas)
  • Ollier disease (multiple enchondromas)
  • Gaucher disease

Mucopolysaccharidoses (Hunter/Hurler/Morquio)

  • J-shaped Sella
  • Dense Skull/Dense Bones
  • Vertebral Body Beaking
  • Canoe Paddle Ribs
  • Basilar Invagination
  • Dural Ectasia
  • Proximal Metacarpal Tapering

Dwarfism

General

  • Rhizomelic → proximal shortening (humerus or femur)
  • Mesomelic → middle shortening (tibia/fibula or radius/ulna)
  • Acromelic → distal shortening (hand)
  • Micromelic → entire limb shortened

Achondroplasia

  • Rhizomelic
  • Increased craniofacial ratio
  • Small skull base/foramen magnum
  • Short vertebral bodies and large disc space
  • Interpediculate distance decreases at more inferior levels in the L spine
  • Pedicles are short and there is posterior vertebral body scalloping
  • Metaphyses of long bones are flared
  • Trident hand due to increased space between the middle fingers
  • Iliac bones are short and rounded
  • Decreased acetabular angles
  • Champagne glass shaped true pelvis
  • Lower extremities are short and thick
  • Growth plates are “V” shaped

Thanatophoric

  • Rhizomelic
  • Severe platyspondyly, but with enlarged disc space, such that the overall thorax is nl in length
  • Bowing and widening of femurs which look like telephone recievers
  • Metaphyses are flared
  • Small iliac wings and decreased acetabular angles
  • May be a trident acetabulum
  • Ribs are very short
  • Tubular bones of the hands are feet and short and broad
  • Interpediculate distance may be narrowed
  • Cloverleaf skull

Jeune syndrome (asphyxiating thoracic dystrophy) and Ellis van Creveld

  • Both are acromelic
  • Very short ribs (especially Jeune) resulting in a very narrow chest, with a large appearing heart
  • Trident acetabulum and decreased acetabular angle
  • Premature ossification of the prox femoral epiphysis
  • Skull and spine are normal, which helps distinguish from other dwarfisms
  • Carpal bone fusions
  • Polydactyly and an extra carpal bone are found much more commonly in ellis van creveld
  • Could ask if the pt is Amish (ellis van creveld)

Camptomelic

  • Main feature is bowing of the long bones of the lower extremity, with the apex pointing toward a skin dimple
  • Also hypoplastic scapulae, small face vs skull, and dysplastic pelvic bones

Child Abuse

Suspicious Fractures for Child Abuse (MR SSS)

  • Metaphyseal
  • Rib
  • Scapula
  • Sternum
  • Spinous process

Other Causes of Multiple Fxs

  • osteogenesis imperfecta (look for osteopenia and wormian bones)
  • neuromuscular disorders
  • Menkes syndrome (also causes osteopenia and wormian bones plus WM hypomyelination, cerebral atrophy and subdural collections due to decreased copper absorption in GI tract)
  • congenital insensitivity to pain

Periosteal Reaction

  • physiologic/nl variant - should be only diaphyseal, smooth, symmetric, mostly along medial prox femur
  • prostaglnadin txt
  • Caffey’s
  • neuroblastoma mets
  • TORCH infections
  • syphilis

Miscellaneous

  • Lap belt injury complex = lap-belt ecchymosis, distraction fx of L-spine, bowel injury
Hemangioma Lymphatic Malformations Vascular Malformations
discrete masses cystic, fluid-fluid levels AV fistula, AVM
prominent enhancement rim, septal, or no enhancement capillary malformation
grow 1-2 years then involutegrows as child grows venous malformation (may have calcification)

Prostaglandin

  • Keeps PDA Open
  • Causes Mucosal Pyloric Hypertrophy
  • Causes Periostitis

DDx for Thanatophoric Dwarf

  • Jeune Syndrome (asphyxiating thoracic dysplasia)
  • Homozygous Achondroplasia

DDx Wormian Bones (CHOP DIK)

  • Cleidocranial Dysostosis
  • Hypothyroidism/Hypophosphatasia
  • Osteogenesis Imperfecta
  • Pyknodysostosis/Progeria
  • Down Syndrome
  • Idiopathic
  • Kinky Hair Syndrome
pedi.txt · Last modified: 2017/07/07 18:30 (external edit)