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189 Cards in this Set
- Front
- Back
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Cyanotic
Normal pulmonary vascularity No cardiomegaly |
TOF
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Non-cyanotic
Increased pulmonary vascularity |
L->R shunt:
LA enlargement? No = ASD or PAPVR Yes => Aortic enlargement? No = VSD, endocardial cushion defect Yes = PDA |
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Acyanotic
Normal vascularity |
AS
PS PAPVR Coarctation Interrupted arch |
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Cyanotic
Normal/decreased pulmonary vascularity Cardiomegaly |
Ebsteins anomaly
Tricuspid atresia w/ restricted ASD Pulmonary stenosis with intact interventricular septum TR of the newborn |
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Cyanotic
Increased pulmonary vascularity |
"T-lesions"
TGA - narrow superior mediastinum - "egg on a string" Truncus arteriosus Tricuspid atresia TAPVR - widened superior mediastinum "Tingle" ventricle DORV DOLV |
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Cyanotic
Pulmonary edema Normal heart size |
Infradiaphragmatic TAPVR
Coarctation Anemia Asphyxia Hypocalcemia Hypoglycemia Arrhythmia Hyvervolemia Myocarditis |
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Hydronephrosis and hydroureter (Peds)
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Obstructed at UVJ:
- UVJ obstruction (primary) - UVJ stone - UVJ thrombus - Ureterocele - Extrinsic compression (mass) Non-obstructed UVJ - Prune belly - UV reflux Bladder outlet obstruction - neuropathic bladder - urethral valves - stone/clot Non-obstructed system - congenital mega ureter - post surgery w/o resolution - adynamic ureteral segment |
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Prune Belly
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AKA Eagle-Barrett syndrome
Rare congenital disorder characterized by Dramatic collecting system dilatation Deficiency of abdominal musculature Cryptorchidism Small, bell-shaped chest suggestive of pulmonary hypoplasia |
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Cystic renal disease (peds)
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Simple cyst
Cysti medullary disease - Medullary sponge kidney - Nephronophthisis Polycystic disease: AD polycystic KD AR polycystic KD Dysplasia: Multicystic dysplastic kidney Tumors: - multilocular cystic nephroma - cystic Wilms tumor Acquired - infectious - pyogenic - dialysis Nephroblastomatosis |
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Enlarged hyperechoic kidneys
Striated nephrogram |
ARPKD
Pyelo |
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Striated nephrogram (peds)
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ARPKD
Pyelo Medullary sponge kidney Acute ureteral obstruction Radiation nephritis Contusion RV thrombosis |
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ARPKD
Autosomal recessive polycystic kidney disease |
Tiny cysts in kidneys
- echogenic kidneys - striated nephrogram Periportal fibrosis - biliary dilitation Pancreatic fibrosis 4 forms: Antenatal - oligohydramnios - pulmonary hypoplasia - fatal Neonatal - renal failure in a month - death within a year Infantile - appears by 3-6 months - chronic renal failure - hepatic failure Juvenile form - portal hypertension |
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Von Hippel Lindau
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AD
Criteria: - fam hx + visceral lesion OR hemangioblastoma - 2 or more hemangioblastomas - 1 hemangio blastoma and 1 visceral lesion Renal cysts RCC hemangioblastomas - Posterior fossa/spine/retina - erythropoietin Adrenal Pheochromocytoma - type II Pancreatic cysts Islet cell tumors Serous cystadenoma 10x risk of pheochromocytoma |
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Tuberous sclerosis
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Multisystemic harmartomatous disease
triad - adenoma sebaceum - seizures - mental retardation Renal cysts Angiomyolipoma Cardiac rhabdomyoma Cortical tubers Subependymomas GCA Hepatic adenoma Pancreatic adenoma |
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Wilms tumor
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Ages 4 mo - 4 years
MC abdominal neoplasm in children 1-8 years 5-10% bilateral Claw sign Rarely calcifies Negative catecholamines Tumor thrombus IVC/renal vein pushes vessels away (as opposed to insinuating like neuroblastoma) Increased incidence in: Nephroblastomatosis (30-44%) sporadic aniridia hemihypertrophy WAGR - wilms - aniridia - genital anomalies - retardation Beckwith-Weideman - Omphalocele - Macroglossia - Gigantism - Pacreatic hyperplasia/hypoglycemia Horseshoe kidney/GU abnormalities Trisomy 18 Denys-Drash Staging: Surgical 1. confined to kidney 2. extends beyond kidney but excised 3. residual tumor in abdomen 4. hematogenous mets 5. bilateral involvement |
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Renal mass in a sickle cell child
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Renal Medullary CA
- mets to bone |
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Congenital Nasal Mass (Peds)
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Nasal Dermal Sinus
- pit along nasal dorsum - tract to formaen cecum traversing septum Frontoethmoidal encephalocele - herniation of brain and meninges through skull base defect Sinonasal hemangioma - vascular, high T2, well-defined Choanal atresia (bony or fibrous) Nasal Glioma - extranasal = SQ red/blue mass on nasal dorsum - intranasal = polypoid submucosal mass Pyriform aperature stenosis - midline central incisor - midline intracranial abnormalities |
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Nasal Mass (peds)
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Juvenile angiofibroma
- centered on sphenopalatine fossa - T2 hyperintense with salt and pepper appearance due to flow voids Rhinolith - Ca++ foreign body |
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Retropharyngeal rim enhancing lesion in a child
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Peritonsillar abscess
Necrotic LAD (lymphaoma) Mets - neuroblastoma Goiter |
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Vesicoureteral reflux
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Incompetent UVJ junction allows backflow due to short intramural course or angle of insertion
Incidence 1-2% F>>M Leads to recurrent UTI's in 35% Can lead to renal scarring leading to: - HTN - growth retardation - CRI Grading: International Reflux Study Committee grading system of vesicoureteral reflux I: Reflux into ureter not reaching renal pelvis II: Reflux reaching pelvis but no blunting of calyces III: Mild calyceal blunting IV: Progressive calyceal and ureteral dilation V: Very dilated and tortuous collecting system, intrarenal reflux Imaging VCUG Radionuclide cystography (more sensitive, less anatomic info) Tx: prophylactic abx Ureteral implantation |
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Associated disease with UC?
Complications? DDx |
Ankylosing spondylitis
Primary sclerosing cholangitis Episcleritis Reactive arthritis Cholangiocarcinoma Pyoderma gangrenosum Erythema nodosum Complications: - obstruction - Perforation - Toxic megacolon - Increased incidence of colorectal CA (10% incidence after 1 decade of UC. Can be multiple) DDx: Crohn's Pseudomembranous colitis Iscemic colitis Chronic cathartic use |
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UPJ obstruction
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MC congenital GU anomaly
Caused by a crossing fibrous band or vessel 20% bilateral Can lead to MCDK in utero P/W mass or hematuria Dx: - US - VCUG to r/o reflux - MAG3 to determine function Can be followed by US if mild. If severe or progressive pyeloplasty. |
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VACTERL
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Vertebral
Anal atresia Cardiac Tracheoesophageal fistula/Esophageal Renal Limb |
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Radial ray malformation
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Abnormalities in the radius, radial carpal bones and thumb
Often absence of radius, and aplasia, hypoplasia, or digitization (3 phalanges) in thumb. Often with radial deviation of hand position A/W: VACTERL Fanconi's anemia Holt-Oram (cardiac septal defects and UE abnormalities) Trisomy 13 Trisomy 18 Thrombocytopenia absent radius syndrome (TARS) (thumb present) Maternal valproic acid exposure Maternal DM |
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Unilateral hyperlucent lung in a child
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FB
Mucus plug Swyer-James Hypoplasia Bronchial stenosis Pulmonary sling (R > L or BL) CLO (LUL > RML > RUL) CPAM |
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Increased renal echogenicity in a child
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Pyelonephritis
Nephrotic syndrome ARPKD ADPKD GN Lymphoma/Leukemia Glycogen storage dz |
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Omphalocele
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Physiologic bowel herniation weeks 7-12
- 3 CCW 90° turns Omphalocele is normally covered by 2 layers of peritoneum (10-20% rate of rupture) US: Midline abdominal wall defect Herniated bowel sac Cord insertion at apex of sac Ascities A/W: 2/3 of patients have Cardiac defects Trisomy 13, 18, 21 Malrotation |
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Beckwith-Weidemann
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Omphalocele
Macroglossia Gigantism Pacreatic hyperplasia/hypoglycemia Increased incidence of Wilm's tumor |
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Gastroschisis
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Ischemic abdominal wall defect (usually to the right of the umbilicus)
Cord insertion to the right of sac No peritoneal covering |
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Neuroblastoma
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Age 2 mos-2 years
2/3 abdominal (adrenal) 1/3 chest/head/neck Mets to bone, marrow, skin, liver, LN Labs: elevated urine vanniylmandelic acid Imaging: 131-I MIBG Calcifies in 85% Vascular encasement is common Staging: Many Systems: Evan's system: 1 - confined to origin 2 - contiguous spread from origin but not across midline 3 - contiguous spread across midline 4 - disseminated disease 4S - primary stage 1 or 2 with mets (NON BONY) (liver, skin, marrow) |
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Primary megaureter
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Caused by fibrotic, aperistaltic ureteral segment within the distal ureter, leading to functional (not mechanical) distal outflow obstruction
95% isolated abnormality A/W: Calyceal diverticulum Ureterocele Papillary necrosis Ureteral duplication UPJ obstruction Renal ectopia Imaging - VCUG to r/o reflux - lasix renogram/Whitaker test to assess mechanical obstruction and function |
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Rickets findings?
Caused by? |
Metaphyseal fraying, cupping, irregularity
Widened physes Osteopenia Anterior rib splaying (rachitic rosary) Caused by Vitamin D deficiency: - lack of sun - malnutrition - genetic defect in synthesis/resistance - RTA - anticonvulsant meds - neonatal hepatitis |
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Renal agenesis
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Bilateral (incompatible with life) or unilateral
A/W gonadal anomalies Male: cryptorchidism hypospadias testicular agenesis Female hydrometrocolpos vaginal atresia/septum |
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Oligohydramnios
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Demise
Renal dysfunction IUGR Post dates PPROM Chromosomal abnormalities |
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Sites of osteochondritis dissecans
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Medial femoral condyle
Capitellum Talar dome Patella |
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Pediatric renal cortical calcification
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Chronic GN
Cortical ischemic insult Oxalosis Hypercalcemia Alport syndrome Ethylene glycol toxicity |
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Pediatric medullary nephrocalcinosis
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RTA (type I)
MSK/benign tubular ectasia Diuretics Hyperparathyroidism Milk-alkali Cushings Papillary necrosis Chronic pyelo |
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NEC
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Causes are controversial, may be ischemia due to hypotension, sepsis, stress, hypoxia
A/W NEC - prematurity - preeclampsia - PPROM - DM - early formula feeding - Multiparity Conservative management (bowel rest, abx) unless perforation and free air |
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Splenic trauma/laceration
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AAST (American Association For The Surgery Of Trauma)
Grade I - Subcapsular hematoma <10% of surface area. - Laceration: capsular tear <1 cm in depth into the parenchyma Grade II - Subcapsular hematoma 10-50% of surface area. - Laceration: capsular tear, 1 to 3 cm in depth, but not involving a trabecular vessel. Grade III - Subcapsular hematoma >50 percent of surface area OR expanding, ruptured subcapsular or parenchymal hematoma OR intraparenchymal hematoma >5 cm or expanding. - Laceration: >3 cm in depth or involving a trabecular vessel. Grade IV - Laceration involving segmental or hilar vessels with major devascularization (ie, >25 percent of spleen) Grade V - shattered spleen. - Laceration: hilar vascular injury which devascularizes spleen. |
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Scoliosis
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Idiopathic
Congenital - Chiari 2 - Hydromyelia - Segmentation anomaly - Diastematomyelia - Tethered cord - Lipoma - Meningocele - CP - NF |
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Idiopathic
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Infantile
- non-progressive vs. progressive - non-progressive = thoracic convex left < 30° that resolves Juvenile - MC girls 4-9 - thoracic right convex - progressive, poor prognosis Adolescent - MC form of idiopathic - presumed AD - F>M 8:1 - thoracic right convex > 30° usually resolves - risks for severity and progression include - female sex - severity at diagnosis - high thoracic level A/W - heart disease Imaging - standing frontal and lateral radiographs - bone age radiographs - measurement of angle using Cobb method - correction when Cobb angle is greater than 40° |
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Scimitar syndrome
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Hypoplasia of one or more pulmonary lobes
Subdiaphragmatic partial anomalous pulmonary venous return Pulmonary arterial hypoplasia Rib and vertebral anomalies NORMAL bronchial tree |
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Bilateral enlarged kidneys
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ADPKD/ARPKD
Nephrotic syndrome Nephroblastomatosis Lymphoma/Leukemia Glycogen storage disease |
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Wilms ddx
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Atypical rhabdoid
Clear cell sarcoma Multilocular cystic neproma (like cystic Wilms) Nephroblastomatosis Neuroblastoma Congenital mesoblastic nephroma (i.e. in an infant under 10 months of age) |
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Nephroblastomatosis
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BL kidneys contain rests of metanephric blastema
BL enlarged kidneys - iso/hyperechoic on US - low attenuation on CT May infiltrate in perilobar (subcapsularly) or intralobar pattern 30-44% incidence of Wilms A/W: Beckwith-Weidemann Denys-Drash Trisomy 18 Trisomy 13 |
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Germinal matrix hemorrhage
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Abnl echogenic material in the subependymal caudothalamic groove
Common in premature infants <32 wks GA Happens in first 3 days of life May be clinically silent (US SCREENING) Causes: - hypoxia/hypercapnia - hypertension - hyponatremia - hypervolemia - pneumothorax Serious complication include periventricular leukomalacia (PVL) Grading: I: subependymal II: intraventricular no hydrocephalus III: intraventricular with hydrocephalus IV: Intraparenchymal (echogenic -> cysts) |
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Bladder diverticula
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Outpouching of bladder mucosa nd submucosa through muscular wall
Congenital or due to increased pressure in the setting of obstruction Hutch diverticulum is adjacent to ureteral insertion 2% risk of maliganancy Imaging: - US jet during filling - VCUG |
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Feline esophagus
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Transient waves of no significance
If fixed, transverse esophageal folds are a sign of severe esophagitis |
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Neonatal acute respiratory disease
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TTN
- ground glass opacities bilaterally, small pleural effusions, normal to increased volume - C-section - prolonged delivery - maternal anaesthesia - resolution by 2 days DDx - CHD, meconium aspiration, neonatal pneumonia, hypervolemia Meconium aspiration - coarse airspace opacities, mixed lucencies and atx, increased lung volumes, pneumomediastinum or pneumothorax DDx - CHD, meconium aspiration, neonatal pneumonia, hypervolemia Neonatal pneumonia - instestitial infiltrates, c/b pneumatocele - GBS - Klebsiella, staph - DDx - CHD, meconium aspiration, neonatal pneumonia, hypervolemia |
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Ureterocele
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Orthotopic = normal insertion = us. adults
- "spring onion" or "cobra head" appearance - prolapsing into bladder with radiolucent halo - may be asymptomatic Ectopic - us. associated with duplicated collecting system - inferior pole a/w ureterocele and reflux - upper pole obstructs - may insert below external bladder sphincter in females (incontinence and UTIs) 10% BL |
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Vaginal mass in girls
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Proplasling ureterocele
rhabdomyosarcoma (cluster of grapes) Gardner dust cyst |
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Scurvy
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Vitamin C deficiency
Dense metaphyseal bands with adjacent lucent line Diffuse osteopenia and cortical thinning Periosteal rxn due to hemorrhage DDx leukemia |
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Dense metaphyseal bands
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Normal variant
Lead ingestion/Bisphosphonate therapy Treated Leukemia Healing rickets/hyperthyroidism Healing scurvy Hypervitaminosis D Trauma Radiation injury Anemia (SCA, thalassemia) Renal osteodystrophy |
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Hypertrophic pyloric stenosis
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Ages 3 months - 3 years (us. 1 week - 3 months)
4:1 M:F thickness >3 mm mucosa->serosa length > 17 mm |
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Cholelithiasis (peds)
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Sickle call
Thalassemia Idiopathic Poor feeding CF Lasix |
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CLO
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Hypoplastic or collapsed cartilage leading to impared drainage of fluid then air from distal lung
LUL > RML > RUL > LLs At first opaque then becomes air filled and hyperexpanded over time Surgical tx |
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Growing fx
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Depressed skull fx with dural rent
-> leptomeningeal cyst which may lead to mass-effect on fx and underlying parenchyma |
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Hydrometrocolpos
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Congenital:
vaginal atresia/web imperforate hymen Acquired endometrial tumors cervical/vaginal tumors post radiation fibrosis |
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Multilocular cystic nephroma
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Cystic mass arising from metanephric blastema
50% in boys < 3 years of age 2nd peak in young females Comprising multiple , non communicating, hypovascular cysts Herniate towards renal pelvis DDx: Multilocular cystic nephroma MCDK Cystic wilms |
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Holoprosencephaly
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lobar, semilobar and alobar
1:1000 A/W: trisomy 13 Chromosomal defects of 7 and 18 TORCH maternal DM maternal phenytoin, retinoic acid, EtOH Imaging: Partial to complete agenesis of CC Midline facial anomalies Rim of normal appearing cortex |
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Klippel-Feil
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Segmentation anomalies in the cervical spine
"wasp waist" appearance at the non segmented levels (as opposed to posterior fusion in JCA) Omovertebral bone or fibrous band A/W Renal agenesis cardiac anomalies low posterior hairline Chiari I neurenteric cyst |
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Craniosynostoses
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Scaphocephaly (aka Dolichocephaly):
↓ Transverse, ↑ AP ⇒ sagittal synostosis Trigonocephaly: "Ax-head" "pear-shaped" on axial "quizzical" orbits (elevation of medial orbital rim) ⇒ metopic syonstosis Plagiocephaly Asymmetry ⇒ unilateral single or asymmetric multiple synostoses Brachycephaly ↑ Transverse, ↓ AP "harlequin" orbits (elevtion of lateral orbital rim) ⇒ bicoronal or bilambdoid Turricephaly "Towering skull" ⇒ bicoronal or bilambdoid Kleebattschadel - clover leaf skull - universal synostosis Bulging temporal, shallow orbits ⇒ bicoronal and bilambdoid Craniofacial dysostoses: "Towering skull" shallow orbits |
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Craniosynostosis causes
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Primary
Secondary: rickets hypophosphotasia anemia decreased intracranial pressure Syndromic: Crouzon Aperts Carpenter |
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Tethered cord
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Conus inferior to L2
Primary: Tight filum terminal tehtered conus Secondary: Diastematomyelia Liomeningocele Meningocele Dermid Neurenteric cyst Sx: wekness gait disturbance LBP bowel/bladder incontinence |
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Peds spine tumors
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Intramedullary:
- astrocytoma - ependymoma - ganglioglioma Extramedullary intradural: - NST - NF - meningioma - drop mets - germ cell tumor - medulloblastoma - ependymoma - pinealcytoma/blastoma - glioma - CPP Epidural: - hematoma - abscess |
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Duplication cysts
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Mensenteric > antimesenteric
May communicate with lumen Ileum > esophagus Gut signature on US |
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Lissenencephaly
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Migrational abnormality
Smooth cortex without gyral/sulcal patterns "hourglass" or "figure of eight" configuration |
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Hepatoblastoma
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M>F
<5 years old, usually <2 years old Increased AFP Vascular invasion Lung mets A/W: Beckwith Weidemann hemihypertrophy precocious puberty fetal EtOH syndrome trisomy 18 Hyper or isoechoic Poor enhancement "spokewheel" angiographic appearance |
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AVMs
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Spetzler classification
- size - deep drainage - eloquence 2-3% annual risk of hemorrhage Responsible for 40% of spontaneous intracranial hemorrhage in peds |
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Legg-Calves-Pethes
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= idiopathic AVN of the femoral head in kids
5-8 years 80% asymmetric Risk factors: steroids sickle cell BMT autoimmune M>F DDx: - sickle cell anemia - hypothyroidism - Gaucher - Meyer dysplasia (asymptomatic, BL, 2-4yo, small underdeveloped epiphyses) |
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Erhlenmyer flask deformity
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Enlarged metaphyses due to marrow expansion
DDx: Thalassemia Gaucher (glycogen storage) mucopolysaccharidoses osteopetrosis |
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Rodent facies
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thalassemia
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Apophyseal equivalents
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Greater trochanter
Talus Anterior vertebral body Olacrenon |
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Epiphysis/Apophyseal equivalent lesions
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Limited to epiphysis:
Chondroblastoma EG Osteomyelitis Subchondral cyst Epiphysis extending to metaphysis: Enchondroma Osteoblastoma ABC |
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Cystic suprasellar mass
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Craniopharyngioma
Rathke's cleft cyst Pituitary macroademona Glioma Mets Aneurysm |
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Choledochal cysts
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Classic presentation:
intermittent abdominal pain (50%) jaundice (80%) abdominal mass (50%) Todani classification: Type I (MC): fusiform dilitation CBD Type 2: diverticulum of CBD Type 3: distal dilitation of CBD in duadenal wall (choledochocele) Type 4: intra and extrahepatic dilitation Type 5: Intrahepatic dilitation only (Caroli's) - 80% a/w medullary sponge kidney Increased risk of biliary tract carcinoma Tx: Surgical - delay -> cirrhosis, pancreatitis, cholangitis |
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Luckenschadel skull
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Chiari 2
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Biliary atresia
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Kasai portoenterostomy by 2 months of age
Normal GB in 10-20% |
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Lead point in peds intussusception
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Meckel's
Lymphoma (MC age > 5) polyp Hemorrhage (HSP) Inspissated stool (CF) Inflamed appendix |
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Hypoplastic left heart syndrome
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Atretic or hypoplastic LV, mitral and aortic valves
Requires ASD and PDA to sustain life When PDA closes -> hypoxia and hyperkalemia Mild cardiomegaly and pulmonary edema Norwood -> Glenn -> Fontan Norwood (2 or 3 stage correction for HLHS) Stage 1: - first days of life - Neo aorta from RVOT anastamosed to aortic root - BT (sublavian artery to PA) or Sano (RV to PA) shunt to PA (systemic return to PA) - PDA ligated - atrial septum resected Stage 2: - 3-6 months - pulmonary resistance decreased - excise BT or Sano shunt - place bidirectional Glenn shunt (end-to-side SVC to PA) Stage 3: - 18-36 months - lateral tunnel or extracardiac Fontan (IVC to PA) |
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TE fistula
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A/W
- Down's - prematurity - VACTERL - 47-75% have associated anomalies 1/3000 live births Two most common types have proximal EA (91%) 5 Types: 1 - 82% - esophageal atresial with distal fistula 2 - 9% - EA w/o TEF 3 - 6% - H-type - isolated TEF w/o EA 4 - 2% - proximal and distal TEF Imaging: - esophogram not necessary, clinically and radiographically apparent - esophagram for post-op leak check 5 - 1% - proximal TEF and distal TEF |
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Sinus masses in peds
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Mucoid retention cyst
Mucocele (CF) Benign Neoplasm: Juvenile angiofibroma Hemangioma Lymphatic malformation Odontogenic tumor Nasal glioma Malignant neoplasm: Rhabdomyosarcoma Lymphoma/leukemia Inflammation: Sinusitis Wegener's EG Encephalocele |
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Scheuermann's
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MC 13-17yo males
3 or more contiguous levels of endplate irregularity and anterior wedging Kyphosis > 40° abnormal DDx: Trauma Congenital Postural TB Spondyloepithelial dysplasia |
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Rheumatic heart disease
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GAS (group A strep) leads to autoantibody production with cross-reactivity to valvular tissue
Mitral > aortic > tricuspid > pulmonic Myocarditis in 50% |
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Hirschsprung disease
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Aganglionosis of the colon
Abnormal aganglionic segment cannot relax -> proximal normal bowel dilates Rectosigmoid > descending colon > transverse colon > ascending colon > TI Transition may be abrupt or gradual If inconclusive -> bx DDx: Anal atresia Hypoplastic left colon Meconium plug syndrome Ileal atresia Meconium ileus A/W: Down's Cardiac anomalies GU anolmalies Congenital neuroblastoma |
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Sequestration
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Intra (left atrial drainage) or extralobar (IVC or azygous drainage) depending upon pleural investment
Extralobar presents earlier and is A/W congenitl defects including diaphragmatic, pulmonary and cardiac Lung parenchyma not connected to bronchial tree or pulmonary artery P/W: recurrent infection hemoptysis bronchiectasis lung abscess MC lower lobes |
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Prenatal CMV infection
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P/W
seizures microcephaly DD chorioretinitis sensorineural hearing loss optic neuritis IUGR jaundice heptasplenomegaly pneumonitis US: curvilinear echogenicity in BG (leniculostriate vasculopathy) CT: periventricular subependymal Ca++ and cysts MR: polymicrogyria cerebellar agenesis lissencephaly |
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LCH
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= (Letterer-Siwe + Hand-Schuller-Christensen + EG)
Abnormal prolifertion of Langerhan's cells Affects (in order of decreasing incidence): Skull 25% Ribs Femur Pelvis Spine Mandible Humerus Flat bones > Long bones Radiography: lysis sclerosis (variable) +/- sequestrum "Beveled" skull lesion vetebra plana endosteal erosion |
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Acyanotic
Decreased or normal pulmonary vascularity |
AS
Pulmonic stenosis Interrupted aorta Coarctation |
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Acyanotic
Increased pulmonary vascularity |
ASD
VSD Endocardial cushion defect PDA |
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Cyanotic
Increased pulmonary vascularity |
T- lesions
Transposition Truncus Single ventricle DORV TAPVR |
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Cyanotic
Decreased or normal pulmonary vascularity |
TOF
Ebsteins Tricuspid atresia Pulmonic stenosis |
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Ebsteins anomaly
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A/W maternal lithium
Partial atrialization of RV Cyanotic, enlarged heart, normal/decreased vascularity Always has PFO or ASD Tx: Surgery or conservative depending on pulmonary flow |
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SCFE
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Boys 14 Girls 11
3:1 M:F Femoral epiphysis slips medial and posterior Frogleg lateral best Tx: Pin in place (no repositioning) C/B: AVN |
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Pulmonary sling
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True vascular ring
Left PA arises distally from right PA, passing anterior to esophagus and posterior to trachea P/W: stridor dyspnea A/W: tracheal cartilaginous ring C/B: tracheomalacia |
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Sturge Weber
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Encephalotrigeminal angiomatosis
A Phacomatosis Portwine stain in the V1 or V2 distribution Abnormal leptomeningeal and hemispheric drainage leads to ischemia, encephalomalacia, volume loss, and gyral "tram-tracking" calicifications Cortical atrophy leads to: Dyke-Davidoff-Masson syndrome = Calvarial expansion Enlarged diploic space Elevation of petrous ridge Increased pneumatization of mastoid air cellls P/W: DD seizures hemiplegia Tx: Seizure meds Hemispherectomy |
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Tricuspid atresia
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No direct connection between RA and RV
Relies on PFO/ASD and either VSD or PDA to get blood to PA Cyanosis/LVH/decreased or normal vascularity Tx: Fontan procedure Return of systemic venous blood to PA Usually extracardiac shunt between IVC and RPA, with fenestration to RA to decompress circuit |
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TS
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AD
Hamartomatous syndrome Classic (Vogt) triad: adenoma sebaceum MRDD seizures SGA retinal glial tumors angiomyolipomas rhabdomyomas pulmonary harmartomas Skin: Shagreen patches angiofibromas subungual fibromas adenoma sebaceum (=angiofibromas) |
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Multiple asynchronous fractures in a child
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Nonaccidental trauma
Osteogenesis imperfecta Hypophostasia Pain insensitivity |
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OI
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Brittle bones
Blue sclera Deafness Ligamentous laxity Poor dentition Collagen mutation Type 1: - AD - exuberant callus - bowing, epiphyseal enlargement - teenage presentation - blue sclera in 80% Type 2: - AR - detected in utero - poor calvarial mineralization - short femurs - blue sclera Type 3: - AR - FX and excessive callus at birth - normal sclera Type 4: - AD - Osteoporosis - tooth discoloration - normal sclera |
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Pediatric ovarian tumors
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Functional Cyst
Hemorrhagic cyst Endometrioma Torsion Abscess Mature cystic teratoma (15% BL) - 60% of ovarian neoplasms in F<40yo PCOS - follicles/ovary > 12 - volume/ovary > 10 ml Ectopic Dysgerminoma - 50% of female GCTs) - heterogeneous, Ca++, cysts - 5% b-HCG Yolk sac tumor (endodermal sinus tumor) - may secrete AFP - large heterogeneous Sertoli-Leydig tumor - sex cord tumor - virilization - precocious puberty Ovarian fibroma - Meigs syndrome (acsites + pleural effusions) Granulosa cell tumor - may secrete estrogen |
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Dandy Walker malformation
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Hypoplastic cerebellum
Agenesis or hypoplasia of vermis Partial or complete callosal agenesis Enlarged cisterna magna Cystic dilitation of the 4th ventricle Torcular-lambdoid inversion Hydrocephalus P/W Macrocephaly DD seizures A/W: Agenesis of the CC Grey matter heterotopia Polymicrogyria/agyria Schizencephaly Syndromic associations: PHACE - posterior fossa malformations - hemangiomas - arterial abnormalities - cardiac anomalies - coarctation - eye anomalies Meckel-Gruber - renal cystic dysplasia - cerebral abnormlities (classically posterior ocipital encephalocele) - post-axial polydactyly Walker-Warburg syndrome - cobblestome lissencephaly - hydrocephalus - agenesis/dysgenesis CC - cerebellar hypoplasia - brainstem abnormalities - eye abnormalities Aicardi syndrome - agenesis/dysgenesis of CC - cortical dysplasia - intracranial cysts - cerebellar abnormalities - microphthalmia |
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Duplex collecting system
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Kidney drained by 2 ureters
Intervening band of renal parenchyma divide upper and lower moieties Weigert-Meyer rule: - lower pole ureter inserts orthotopically (normal trigonal position) - upper pole ureter inserts ectopically (inferior and medial) (may insert on urethra, vagina, etc.) Upper pole: - obstructs - ureterocele - reflux rate is at baseline Lower pole - refluxes "Drooping lily" sign on VCUG |
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Mesenteric adenitis
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Abnormally enlarged mesenteric LN
- 3 or more > 0.5 cm in short axis - absence of associated bowel pathology Primary - w/o associated abdominal pathology Secondary - w/ inflammatory or infectious abdominal pathology P/W - abd pain - fever - leukocytosis - abd tenderness |
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Peds multiple large pulmonary masses/nodules
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Metastatic disease:
Osteosarcoma Ewing's Wilm's Septic emboli |
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Peds multiple small pulmonary nodules
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Mets:
Thyroid Granulomatous disease: Histo Coccidio Infection: Varicella Papillomatosus LIP |
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Peds small pulmonary nodule
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Granuloma
Hamartoma AVM Varix |
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Peds large pulmonary nodule/mass
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Round PNA
Infarct Abscess CPAM Sequestration Bronchogenic cyst |
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Peds primary pulmonary neoplasms
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Bronchogenic carcinoma
Leiomyosarcoma Rhabdomyosarcoma Hemngiopericytoma Askin tumor (peripheral PNET, Ewings) |
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NF-2
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Mutation on chromosome 22 in the schwannomin gene
= MISME syndrome Multiple Inherited Schwannomas, Meningiomas, and Ependymomas - BL vestibular schwannomas |
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Multiple meningiomas
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NF-2
Post radiation therapy |
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Cross fused renal ectopia
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A/W
Anorectal malformation Renal dysplasia VUR |
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Horseshoe kidney
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A/W
urinary stasis UTI scarring renal HTN Wilms Adenocarcinoma |
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Peds HCC
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10-15 yo
A/W Cirrhosis biliary atresia HBV glycogen storage disease |
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Fibrolamellar HCC
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Peak age 20 yo
Central calcified scar Ddx: FNH |
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Hepatic mass in a neonate
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Hemangioendothelioma
- MC neonatal hepatic mass - aka infantile hepatic hemangioma - p/w hepatic mass and high output HF - similar appearance to hemangioma with peripherl nodular enhancement - enlarged hepatic and celiac arteries Mets - neuroblastoma - leukemia - wilms Hepatoblastoma - <10% occur in the neonatal period - a/w Beckwith-Weidemann - large heterogeneous mass Mesenchymal hamartoma - large cystic and solid mass - benign - tx excision Unilocular hepatic cyst - no connection to biliary tree Choledochal cyst - connection to biliary tree - p/w jaundice, emesis, hepatomegaly - a/w biliary atresia - MRCP or US - risk of malignant degeneration - tx excision Abscess Angiosarcoma - similar appearance to hemangioendothelioma |
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Pediatric hepatic mass
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Hepatoblastoma
- Beckwith-Weidemann - increased AFP - Ca++ - 75% of all liver malignancies < 15yo HCC - 75% not a/w liver disease - rare before age 5 Hemangioendothelioma Abscess FNH Mets Choledochal cyst Mesenchymal hamartoma Embryonal sarcoma - aka undifferentiated sarcoma |
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ARPKD
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- medullary ductal ectasia
- enlarged echogenic kidneys -> HTN - hepatic fibrosis a/w: pulmonary hypoplasia |
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Agenesis of the CC a/w?
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SOD
Dandy-Walker Aicardi syndrome Encephalocele Gray matter heteroptopia CPP Fetal EtOH syndrome Polymicrogyria |
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Peds - GI Polyps
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Isolated juvenile polyps
- MC cause of pediatric painless BRBPR - 5 or fewer inflammatory polyps - low malignant potential Polyposis syndromes - 6 or more polyps - increased risk of malignant transformation Peutz-Jeghers - MC SB polyposis syndrome - mucocutaneous lesions - benign hamartomatous polyps - GI hamartomas - increased risk of gastric, duodenal and ovarian CA |
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Toxoplasmosis - congenital
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Cortical, periventricular, BG ca++
Encephalomalacia Cortical destruction Ependymitis Hydrocephalus |
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Hemophilic arthropathy
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Knee > elbow > ankle
Unilateral > bilateral Findings Enlargement of the intracondylar notch squaring of the patella Periarticular osteopenia Effusion Chronic -> Jont space narrowing subchondral cysts osteophytes MR: like PVNS with cartilage loss DDx: Septic arthritis JCA SCA |
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Double bubble
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Duodenal/jejunal atresia
Malrotation Duodenal web Annular pancreas Preduodenal portal vein A/W CHD TE fistula imperforate anus Down syndrome |
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Developmental hip dysplasia
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XR:
- Horizontal line through the triradiate cartilages (=Hilgenrener's line) - line from triradiate cartilage to acetabular roof. the shallow angle = acetabular angle - normal = 28° - decreases with age Perpendicular line (Perkin's line) along lateral ilium - femoral head should lie in lower inner quadrant Increased acetabular angle: - DDH - neurogenic hip Decreased acetabular angle: - Down's - achondroplasia US: coronal hip US (vertical) line drawn down the ilium margin (oblique) line drawn along the bony acetabulum alpha angle = intersection of these 2 lines and should be - should be > 50° beta angle = ngle between ilium line and line drawn to lateral femoral head margin - should be > 55° |
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Hypertelorism
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Eyes far apart
Crouzon Cleidocranial dysostosis Hunter's/Hurler's cephalocele thalassemia |
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Hypotelorism
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Eyes close together
Arhinencephaly Holoprosencephaly Microcephaly Trigonocephaly Sagittal craniosynostosis |
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Anencephaly
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MC CNS neural tube defect
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Branchial cleft anomaly
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Mesodermal remnant of branchial clefts
May be cyst, fistula, or tract Cyst has thin smooth wall, may be thick with rim enhancement if infected 1st BCC: - periauricular region, in or adjacent to parotid 2nd BCC: - MC - at mandibular angle - anterior to SCM - posterior to submandibular gland - lateral to carotid sheath 3rd BCC: - posterior triangle in upper neck - anterior triangle in lower neck 4th BCC: - often a tract extending from hypopharynx/pyriform sinus to anterior lower neck (adjacent to thyroid) - may present with thyroiditis Cervical thymic cyst - anywhen along thymic duct tract from hypo pharynx to mediastinum - may contain cystic and solid remnants (thymic tissue) |
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CPAM
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Congenital pulmonary airway malformation
90% present < 1yo with respiratory distress UL predominance Type 1 - (cysts > 2cm) - one dominant cyst - 50% Type 2 - (cysts 1-2 cm) - multiple cysts with variable solid component - 40% Type 3 - microcysts - solid mass - 10% |
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Mesoblastic nephroma
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MC neonatal mesenchymal neoplasm
Benign Arises from metanephric blastema Large solid, homogeneous mass replacing the kidney - rarely cystic degeneration - indistinguishable from Wilm's P/W: hematuria HTN anemia |
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MCDK
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2/2 in utero obstruction - us. UPJ
UPJ obstruction in the contralateral kidney in 15% DDx: cystic WIlm's Imaging: 6 month f/u to ensure no growth |
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Adrenal hemorrhage
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Birth trauma
Stress Anoxia Dehydration R>L (70%) BL (10%) US: heterogeneous cystic and solid mass - DDx Neuroblastoma (so F/U to RESOLUTION!) exophitic Wilm's |
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Osteopetrosis
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4 types:
- precocious (lethal due to medullary obliteration) - infantile (AR) - intermediate - tarda - adult (AD) - osteopetrosis with RTA Thickened cortex Undertubulation DDx: Any of a number of osteodysplasias Renal osteodystrophy Bisphosphonates |
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ADPKD
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AD
Enlarged kidneys Hyperechoic cysts "swiss cheese nephrogram" Cysts: Liver Pancreas Spleen Testes Ovary Berry aneuryms Imaging: MRA head screening |
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Lymphocytic interstitial pneumonia (peds)
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Perinatal HIV infection
Hypoxemia Non-productive cough Salivary gland enlargement Diffuse nodular or reticulonodular infiltrate Tx: Steroids |
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Acute disseminated encephalomyelitis
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ADEM
Post-viral or vaccination P/W: Sz fever HA neurologic deficits 90% resolve Patchy confluent areas of high T2W in WM and BG +enhancement DDx: MS Lyme SSPE (subacute sclerosing pan-encephalitis) |
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Madelung deformity
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2/2 premature closure of distal radial physis
Ulnar and volar tilt of distal radius Triangularization of the lunate Elongate dorsal subluxation of ulna Mesomelic dwarfism (dyschondrosteosis) Post traumatic MHE Turner syndrome |
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Neonatal herpes encephalitis
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HSV2
Birth canal or transplacental hematogenous CSF: gram - w/ mononuclear pleocytosis Diffuse brain destruction and hemorrhagic necrosis CT: Hypodensities in periventricular WM and cortex MR: Increased T2 gyral expansion leptomeningeal enhancement -> Necrosis Cystic degeneration "swiss cheese" Encephalomalacia DDx: TORCH Bacterial meningitis |
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Hurler
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A mucopolysaccharidosis
"bullet-shaped" thickening and tapering of the metacarpals Inferior beaking of the vertebral bodies (also in Hunter) Osteoporosis Macrocephaly MR: dysmyelination indistinct GW Atrophy Hydrocephalus Cystic changes in periventricular WM, CC, BG Tx: BMT |
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Juvenile nasopharyngeal angiofibroma
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Benign nasopharyngeal neoplasm
Teenage boys with epistaxis Centered on the sphenopalatine foramen Can grow along vasculature: pterygomaxillary fissure -> infratemporal fossa foramen rotundum -> middle cranial fossa inferior orbital fissure -> orbital apex |
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Cyanosis with right arch
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TOF
Truncus arteriosus |
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Meckel's
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Remnant of vitelline duct (midgut to extraembryonic sac)
2% of population 2 ft from ileocecal valve 2% present before age 2 A/W intussusception |
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GI hemorrhage in Peds
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Meckel's with gastric mucosa
Juvenile (inflammatory) polyps Anal fissure IBD PUD |
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Prune Belly syndrome
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= Eagle-Barrett syndrome
Abnormal laxity in ureteral and abdoniaml musculature Bladder enlargement Severe VUR -> renal dysplasia +- patent urachus a/w: pulmonary hypoplasia polydactyly/syndactyly CHD imperforate anus |
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Macrocephaly and abnormal increased T2W WM
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Canavan
Alexander Canavan: increased NAA |
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JCA
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Umbrella term including
juvenile onset ankylosing spondylitis juvenile onset RA juvenile onset seronegative chronic arthritis (Still) Age < 16yo F > M Ankylosis of the C-Spine Periarticular osteoporosis Intraarticular erosions Ulnar minus DDx |
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Diffuse polyostotic Periostitis (peds)
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Trauma (NAT)
Idiopathic/physiologic (<6 months) PGE Osteomyelitis Hypervitaminosis A Caffey disease - self limited diffuse periostitis - involves mandible - swelling, fever, anemia Scurvy Congenital Syphillis - Wimberger's sign (erosive notches at the medial proximal tibial metaphyses) |
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Kawasaki disease
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= mucocutaneous lymph node syndrome
postinfectious vasculitis Fever Cunjuctivitis LAD Coronary artery aneurysms in 30% c/b: MI pericarditis myocarditis arthritis Tx: IgG ASA |
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Krabbe disease
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b-galactosidase deficiency
-> inc. galactoceramide and psychosine Inc. T2W signal: POST LIMBS INT CAPSULE periventricular WM caudate heads dentate nucleii |
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MC congenital cardiac defect
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bicuspid aortic valve
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Septo-optic dysplasia
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Absent septum pellucidum
Atretic optic nerves A/W schizencephaly gray matter heterotopia polymicrogyria P/W nystagmus and blindness |
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Simple ovarian cyst (peds)
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CF
McCune-Albright Congenital juvenile hypothyroidism Sexual precocity DDx: Renal cyst/mass/hydronephrosis Urachal cyst Enteric duplication cyst |
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Peds fally liver
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CF
Steroids Hyperalimentation/Malnutrition Glycogen storage disease |
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Peds cirrhosis
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Hepatitis
Biliary atresia Cholestasis Glycogen storage disease Tyrosinemia a1 anti-trypsin deficiency |
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Blount disease
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Infantile
2-3 yo Bilateral More common No pain Adolescent 8-15 yo More often unilateral Painful May require surgical correction |
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Panner disease
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= AVN of the capitellum
- 5-10 yo - chance for resolution ≠ osteochondritis dissecans of the capitellum - 12-16 yo - OCD with chance of loose bodies |
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Ataxia telangectasia
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AR
Cutaneous telangectasias Cerebellar degeneration -> ataxia Lack of lymphoid tissue (spleen, thymus) Increased chance of lymphoma/leukemia Cerebellar and vermian degeneration |
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Rhabdomyosarcoma
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MC pediatric soft tissue malignancy
Children ages 2-6 SQ, intermuscular, intramuscular, periarticular H&N > GU > muscular Dark T1W Bright T2W Tx: surgery, chemo, XRT |
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Lethal skeletal dysplasias
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Osteogenesis imperfecta type 2 (MC)
Thanatophoric dysplasia (2nd MC) |
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Thanatophoric dysplasia
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AD lethal skeletal dysplasia
Death due to respiratory insufficiency Findings: polyhydramnios "telephone-receiver" femora "clover-leaf" skull small appendages, thorax large abdomen platyspondyly |
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Metachromatic leukodystrophy
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AR
arylsulfatase A deficiency MC dysmyelinating disease Can present as infants, child or adult Adults show FRONTAL LOBE PREDOMINANCE SPARING SUBCORTICAL U-FIBERS |
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Platyspondyly
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Congenital
Thanatophoric dwarfism Osteogenesis imperfecta type 2 Metatropic dwarfism Later in life Morquio's (mucopolysaccharidosis) spondoepithelial dysplasia Kniests dysplasia |
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Morquio's
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Tapered "bullet shaped" metacarpals
Playspondyly w/ vertebral body "beaking" valgus knees short stature atlanto-axial subluxation kyphosis dolichocephaly "canoe-paddle" ribs |
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Alexander disease
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Leukodystrophy
May occur infantile, juvenile or adult Symmetric increased WM signal on T2W with anterior to posterior progression MACROCEPHALY (along with Canavan disease) histologically, deposition of ROSENTHAL fibers (eosinophilic inclusion bodies) |
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Cleidocranial dysostosis
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AD
defect in intramembranous osteogenesis (skull, clavicles, pubic bone) Findings: ABSENT/HYPOPLASTIC CLAVICLES ELONGATE 2nd METACARPALS WORMIAN BONES basilar invagination |
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Pyknodysostosis
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Acromelic dwarfism
AR Delayed closure of anterior fontanelle parrot like nose bulging eyes recessed chin TOULOUSE-LATREC Findings: diffuse dense bones widened pubic symphysis acral osteolysis hypoplastic facial bones and mandible FX OF VARYING AGES |
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Chondroplasia punctata
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Multiple epiphyseal dysplasia
Abnl calcification of the epiphyses Short stature MRDD asymmetric limb shortening FINDINGS: punctate calcifications in: the ends of short tubular bones trachea pharyngeal cartilage VBs |
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Fibrous dysplasia
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Abnl proliferation of fibroblasts in the medullary cavity disrupting normal trabecular architecture
"ground glass" appearance 80% monoostotic 20% polyostotic A component of McCune-Albright - polyostotic FD - cafe-au-lait cutaneous spots - precocious puberty |
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Cardiac anomalies in Down's
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Endocardial cushion defects
ASD VSD PDA |
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Engelmann disease
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AD diaphyseal dysplasia
Symmetric sclerosis of the diaphyses, with irregular sclerosis and thickening May efface medullary canal DDx: melorheostosis |
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Dose of:
CXR? Lumbar radiograph? head CT? AP CT? |
0.02 mSv
1 mSv 2 mSv 5 mSv |
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Malrotation
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A/W
CDH Gastroschisis Omphalocele Heterotaxy Prune-belly syndrome |
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Common peds elbow fx
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Supracondylar (60%)
Lateral condylar (10-20%) Medial epicondyle (10%) Radial neck Olecranon |
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Tillaux fracture (peds)
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Salter Harris III through lateral tibial epiphysis
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Pentology of Cantrell
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Arising from error in the cephalad fold in the closing of the abdominal wall after physiologic bowel herniation
CHD Ventral hernia Absence of anterior diaphragm Pericardial defect Sternal defect |
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Cloacal extrophy
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Arising from error in the caudal fold in the closing of the abdominal wall after physiologic bowel herniation
Omphalocele Epispadias Bladder extrophy |
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Duodenal atresia
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A/W:
Down's (30%) CHD VACTERL Other intestinal atresias |
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Lower GI Obstruction in the newborn
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BE:
-> Is there a microcolon? If yes: No succus entericus got into colon -> ilieal atresia -> meconium ileus -> colonic atresia (vascular injury) -> functional immaturity (abnormal colonic motility) -> meconium plug ->small left colon -> Hirschsprung -> anal atresia |
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Meconium ileus
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Inspissated thick meconium
CF Seen in 5-10% of CF patients C/B volvulus perforation peritonitis Tx: BE surgery if still obstructed |
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Functional immaturity of the colon
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Abnormal colonic motility
A/W Maternal DM Maternal MgSO4 Meconium plug (no a/w CF) Small left colon Ddx; Hirschsprung |
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Filling defect within the renal pelvis
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Stone
Clot Fungus ball Neoplasm Sloughed papilla Pyeloureteritis cystica - multiple small subepithelial cysts in the wall of the ureter - may occur in renal pelvis (pyelitis cystica) or bladder (cystitis cystica) - reactive hyperplasia, not premalignant Leukoplakia - squamous metaplasia Malakoplakia - granulomatous inflammatory condition |
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Neonatal CHF
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Obstructed inflow:
TAPVR Obrstructed outflow: Coarctation/Interrupted aorta Muscle disease Non-cardiogenic: Hypovolemia Hypoxia Metabolic Arrythmia Hydrops AVF |
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Neonatal abdominal mass
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Usually Renal and benign
Renal: Hydronephosis (Obstructed kidney) MCDK Nephroblastomatosis Mesonephric blastoma PCK GI: Duplication cyst Mesenteric cyst GU: Ovarian cyst Hydrometrocolpos Adrenal: Adrenal hemorrhage Nephroblastomatosis Hepatic: Mets Hemangioendothelioma Hepatoblastoma Mesenchymal hamartoma Choledochal cyst |
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HUS
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Hemolytic uremic syndrome
- hemolytic anemia - renal failure - thrombocytopenia Unknown cause Also may have: - colitis - CNS manifestations - rhabdomyolysis - DM |
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Short 4th metacarpal
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Idiopathic
Turner syndrome Pseudo and pseudopseudohypopararthyroidism Acrodysostosis MED Acquired - trauma - JCA |
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Tarsal coalition
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Fibrous, cartilaginous, or osseus
1-2% BL in 20-50% May be painful (painful spastic pes planus) Calcaneonavicular Talocalcaneal - may have talar beak - may need CT for dx Talonavicular Calcaneocuboid |
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What's your reference for skeletal dysplasia's
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Taybi and Lachman
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Prostatic urethral dilitation
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Posterior urethral valves
Anterior urethral valves Urethral stricture Urethral diverticulum Prune Belly |
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Diffusely dense bones (peds)
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Osteopetrosis
Healing rickets Hypervitaminosis A or D Renal osteodystrophy Hypothyroid Pycnodysostosis |
