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PEDIATRICS
TURNERS SYDROME:
20% have cardiac abnormalities: bicuspid aortic valve and coarction
TORCH:
CMV is the most common cause of in utero infection
PENTALOGY OF CANTRELL:
deficiency of diaphragmatic pericardium, ectopia cordis, deficiency of anterior diaphragm, cardiac (vsd, asd, tof), sternal cleft, gastroschisis (check this). Associated with trisomy.
GASTROSHISIS:
abnormal involution of umbilical vein results in area of abdominal wall weakness. The result is a full thickness abdominal fusion defect on R side of cord. There are thickened freely floating loops of bowel. There is associated non rotation or malrotation of bowel
OMPHALOCELE:
midline defect in anterior abdominal wall due to failure to form umbilical ring during 3rd to 4th week of gestation with herniation of intraabdominal contents into the base of the cord. Can contain liver in 27%. High incidence of associated anomalies (45-88%).
OEIS COMPLEX:
omphalocele, extrophy, imperforate anus, spinal abnormality
LIMB BODY COMPLEX:
limb body wall complex: results from failure of closure of ventral abdominal wall. Can be due to early vascular insult or less likely, amniotic bands. The cord is absent or short, and the amniotic bands are an association.
PRUNE BELLY:
Triad of (1) absent or hypoplastic abdominal wall muscle, (2) non obstructed markedly distended ureters plus or minus hydronephrosis and variable degree of renal dysplasia , (3) undescended testes due to bladder distension interfering with their descent. The urethra is elongated with dilated prostatic urethra and absence of the prostate
MOST COMMON CYSTIC LUNG MASS IN PEDS:
ccam. Types 1-3. Types 2 and 3 have the worst prognosis due to 2ary abnormalities, while 1 has good prognosis after resection. M=F
INHALED FB:
this happens 50% in <3 yo. 85% of the inhaled FB are vegetable matter. R>L (2:1). The main complication is bronciectasis
HYPOPHOSPATASIA:
3 forms: first 2 are AR (infantile lethal in >50% and childhood form whose first syptom is loss of teeth). The third form is the adult form which is AD. Alk phos deficiency leads to hypercalcemia and ethanolamine phospatemia and ethanolamine phosphaturia. The skull is lucent due to defective mineralization. It can look like OI, and likewise, there are blue schlera
CAFFEY DZ:
mandible>clavicle>tubular bone diaphysis and ribs
CHILDHOOD MEDIASTINAL MASSES:
the most common ones are: neuroblastoma, and lymphoma
NEUROBLASTOMA:
two third of all cases are in the abdomen and 2/3 of these are in the adrenal. The most common extraadrenal location is in the organ of zuckerkandl
ATLANTOAXIAL SUBLUXATION IN KIDS:
Downs, JRA, trauma, morquio syndrome
GROWTH PLATE PRESENT AT BIRTH:
proximal femur
CDH:
the barlow test is to see if the hip can be dislocated. It is performed by adducting and flexing the hip while pushing it posteriorly. The Ortolani test is extension of the hip which should lead to a click as the hip relocates. Ultrasound is not useful after 1 year of age due to shadowing from increased ossification of the hip. In terms of echogenicity, the acetabulum is more echogenic than the femoral head
IN MALROTATION:
the sma and smv can be normally related in one third of cases
NON TRAUMATIC LIMP IN A CHILD:
most common cause is toxic synovitis
CRITOE
1,5,7,10,10,11 years old
INTUSSUSCEPTION IN CHILDREN:
95% are idiopathic. Most common is ileocolic. 75% occur before age 3. 90% have no pathologic lead point. Lead points include meckels, lyphoma, polyp, HSP, inspissated meconium
SUTURE CLOSURE:
divided into primary and secondary causes main secondary causes are due to hematological dz, metabolic dz, bone dysplasias, other sydromes
scaphocephaly=dolicocephaly: premature closure of the sagital suture
brachi: coronal
trigonal: metopic
occicephaly: lambdoid
clover leaf: coronal, lambdoid
MECKELS:
remember rule of
2's and also that meckels frequently present as bleeding in kids and as
intussusception or inflammation in adults
VACTERAL:
in pts with TEF,
cardiac (39%) > msk ( 24%) > gi (20%) > renal agenesis ( 12%). A better way to remember it is CLAR
INFANT OF DIABETIC MOTHER:
Most specific sign in infant of diabetic mother is sacral
agenesis. Also see bilateral adrenal hemorrage, renal vein thrombosis and small
left colon
SMALL LEFT COLON:
meconium plug sydrome: occurs in full term babies in the spectrum with small left colon. It is most associated with infants of diabetic mothers
However, according to other sources: meconium plug sydrome is seen in the newborn in the first 24 hours and is caused by CF (24%), Hirshprungs, prematurity, and maternal MgSO4 administration
MECKELS DIVERTICULUM:
meckels diverticulum is an omphalomesenteric duct (vitelline) anomaly: there are 4 types-umbilicoenteric fistula, umbilical sinus, meckels diverticulum, and vitelline cyst. The urachus is the median umbilical ligament which is a thick fibrous cord as the remnant of the allantois (endodermal outgrowth of yolk sac into stalk). It regresses by the 5th month of development. A patent urachus is a fistula between the bladder and the umbilicus. A urachal sinus is where the urachus is patent only at the umbilicus. A urachal diverticulum is where the urachus communicates only with the bladder dome and a urachal cyst is a gradually enlarging cyst due to closure of both ends of the urachus. Complications of a urachal cyst include infection (23%), intestinal obstruction, hemorrage into the cyst, and malignant degeneration (adenoca 84% and tcc 3%). The prognosis is poor with a 7-16% 5 year survival
BILIARY ATRESIA VS NEONATAL HEPATITIS IN NEONATE WITH HEPATITIS:
biliary atresia is correctable in 12% and non correctable in 88%. This is what Dr. Cindy Miller at Yale gave us in her lecture: the gall bladder is norml in 10%. Liver echogenicity is normal or increased based on the appearance of the periportal triads. It is not possible to exclude or rule out neonatal hepatis by US exam. If you do a HIDA, first priming with phenobarbital, (5mg/kg for 5d prior) and if no excretion is seen into the small bowel than the diagnosis is biliary atresia. Can carry out the study to 24h to rule out hepatitis because biliary excretion would be slower even with phenobarbital induction.
In neonatal hepatitis, the GB can be large or small. Liver echogenicity can be normal, increased, or decreased. Delayed excretion into the bowel and excretion into the bladder is seen.
choledochal cyst, can also cause jaundice during the neonatal period. There is a cyst in the GB fossa.
PEDIATRIC LIVER TUMORS:
hemangioendothelioma is the most common benign hepatic neoplasm in children
hepatoblastoma is the most common malig hepatic neoplasm and is associated with ca++ in 50%;
<5yo: hepatroblastoma; >5yo: hcc, mesenchymal sarcoma (can get chf); <2yo: mesechymal hamartoma
note that hcc is the second most common hepatic malig after hepatoblastoma. also note that hepatoblastoma is associated with precocious puberty, and in Rliver lobe > L lobe. There is also increased AFP and renal failure. Also associated with BW, hemihypertrophy
HEMANGIOENDOTHELIOMA:
most common in first 6 mo of life. Have chf and cutaneous hemangiomas
DR MILLER NOTES ON PEDIATRIC LIVER TUMORS:
hemangioendotheliomas occur at < 6 mo. They can be single or multiple. Occasionally Ca++. The aorta is decreased in caliber after the takeoff of the celiac axis. There are typically multiple hypoechoic lesions in the liver. These children also present with CHF. About 50% of them have hemangiomas of the skin. Hemangioendotheliomas are not malignant. If you support the child through the CHF, the lesion will go away by itself
Mesenchymal hamartoma is a frequently pedunculated lesion hanging off the liver. It is a benign lesion. Occurs from 3 mo to 2 y typically.
Hepatoblastoma can be seen in BW syndrome. It is less common than Wilms in Beckwith Weidmann. The lesions can be single or multiple and mixed in echogenicity. They can invade the hepatic, portal veins and IVC. There are Ca++ in up to 50% of them. Venous invasion cannot be assessed on CT. However, resectability can be assessed by CT
CYSTIC FIBROSIS:
Tracheobronchial mucus, the Na and Cl is slightly elevated. In saliva, its normal. In sweat, the Na and Cl is elevated. Urinary excretion of PABA is decreased.
MORE ON CF:
97% of pts with mec ileus have CF and 10-20% of pts with CF have mec ileus.
PEDIATRIC TUMORS:
wilms (15% ca++ on CT) is most common abdominal malig in children while neuroblastoma (85% ca++) is the most common intraabdominal malignancy in infancy
PULMONARY SEEQUESTRATION:
PULMONARY SEQUESTRATION
pulmonary sequestration is the triad of nonfuntioning lung segment, no communication with the TE tree, and systemic arterial supply. They are usually 6 cm is size and smooth, round or oval. This is usually near the diaphragm. Occasionally, a finger like projection is seen posteriorly and medially which usually represents the anomalous vessel. It is sometimes surrounded by recurrent pulmonary consolidation in a lower lobe that never really clears completely. It may communicate with the esophagus or bronchus. If it communicates with the GI tract, then it is called bronchopulmonary foregut malformation. The ddx is brochiectasis, lung absess, empyema, CCAM, intrapulmonary bronchogenic cyst, Swyer James, pneumonia, met, hernia of Bochdalek. There are 2 main types as follows:
Intralobar:
prevalence 75%, surrounded by visceral pleura, drained by the pulmonary veins, become symptomatic in adulthood, and are usually acquired and are associated with congenital anomalies in 15%. They have a L to L shunt. The main complication is massive spontaneous non traumatic pleural hemorrage, chronic inflammation, and fibrosis. M=F. Can get recurrent pneumonia
Extralobar:
they represent 25%. They have their own pleura, and are drained by systemic veins. They become symptomatic during the first 6 months of life and are considered developmental lesions and have a higher incidence of congenital anomalies (50%) compared with the extralobar variety. This has a L to R shunt. Can get infected in cases of communication with a bronchus or the GI tract. M>F
CCAM TYPES:
1: single or multiple air filled cysts
2: cysts < 2 cm with solid
3: solid, associated with maternal polyhydramnios
2 and 3 have the worse prognosis
MORGAGNI HERNIA:
they are anteriomedially situated more commonly on the right. They often contain liver, fat, or transverse colon. The presentation is usually in older children as compared with bochdalek hernia
INTUSSUSCEPTION:
perforation due to intuscusseption ocurrs at the proximal end
DUODENAL ATRESIA VS STENOSIS:
DA is much more common than DS. 30% of DA have Downs. 20% of DA are preampullary so they dont have bilious vomiting
BIRTH ANOXIA:
causes thalamic infarcts bilaterally
CONGENITAL RUBELLA:
retinopathy, deafness, catarracts, mental deficiency, microcephaly, celery stalk sign, hepatosplenomegaly, congen HD (PDA), punctate Ca++ in the CNS
CHOANAL ATRESIA:
90% bony, 10% membranous, and children are obligate mouth breathers
RDS or HMD (older term):
the peak for this is 3-5 d. The lungs have a ground glass appearance classically. There is hypoxemia and low lung volumes. Typical is the absence of pleural effusions.
TTN: Normal lung vols. Pulm edema pattern. Fluid in fissure. Pleural effusion
THYMUS:
it is an expected finding up to the age of 2. It becomes progressively less visible up to the age of 8. After the age of 8, it is rarely seen. It presence is not necessarity abnormal
MOST COMMON CAUSE OF PNEUMATOCELES IN CHILDHOOD:
staph is the most common. E coli and klebsiella are common causes in the older patient, along with trauma
LIP:
this indicates the presence of AIDS in a young child and it can be mimicked by TB
SUBGLOTTIC EDEMA IN EPIGLOTTITIS:
can be seen in up to 25% of pts. Note that epiglottitis occurs at an older age than croup
PANCREATIC INSUFFIENCY IN CF:
only 2% of pts with CF experience endocrine insuffiency requiring insulin
SMALL CELL TUMORS IN KIDS:
ewings, nhl, neuroblastoma, embryonal rhabdomyosarcoma. Note that Ewings metastasizes to lungs (85%)>bones (69%)> pleura,>CNS
ELBOW DISLOCATION:
90% are posterior or posterolateral. Dislocation is associated with hyperextension. #1 is injury of medial condyl and #2 is separation of the radial head. Fx of the coronoid process is common in adults with posterior dislocation
NURSE MAID ELBOW:
children: 2-5 y. Pull on the elbow results in dislocation with radial head slipping out of the annular ligament. This results in elbow held in pronation and it usually gets reduced be the tech during supine positioning for the X ray
CHROMOSOMAL ANOMALIES AND THEIR RELATION TO CHD:
TOF: Downs
peripheral pulmonary stenosis: Williams, congenital rubella
septal defect: Ellis van Creveld
PDA: isolated Tris 18 ( in addition to numerous other things has rocker bottom feet and choroid plexus cysts)
Coarctation, VSD: Turners, NF, Sturge Weber
ASD, VSD: Holt Oram
TURNER:
short 4th MC, V shaped carpal row, osteopenia
CONGITAL RUBELLA:
80% cardiac, 20% osseous
MARFAN:
aortic root abnormality, prolapsed MV, MR, 90% die of cardiovascular complications
CARPAL COALITION:
LT is the most common
CH is the 2nd most common and may be associated with Ellis Van Creveld
RETROPHARYNGEAL CELLULITIS IN CHILDREN:
tonsillitis is the most common antecedent illness prededing cellulitis in the retropharngeal space. While we are talking about the RPS, note that the node of Rouvier is in the lateral RPS. Some RPS tumors include: lipoma, hemangioma, vascular malformations, plexiform NF
TUBULAR REABSORPTION IN CHILDREN:
Na++ reabsorption in children is in DCT unlike in adults due to poor development of the PCT
XGP DIFFERENCES IN CHILDREN:
in kids it is focal. In adults, it usually involves the whole kidney
URETHRAL INJURY:
instrumentation usually injures the external sphincter and long term indwelling catheter causes erosions and structures at the penoscrotal junction
WATERHOUSE FRIEDRICHSON SYNDROME:
this is caused by meningococcicemia which results in bilateral adrenal hemorrage leading to adreanal insufficiency known as WF syndrome
MOST COMMON RENAL TUMOR IN A NEONATE:
mlcn
ESOPHAGEAL AND DUODENAL ATRESIA ASSOCIATION:
EA is associated with DA and imperforate anus. DA is associated with Downs
CAUSES OF A LIMP IN A CHILD:
1-3Y: #1 is infection, #2 is tumor, #3 is trauma
3-10y: #1 is infection and toxic synovitis, #2 is perthes, and #3 is tarsal coalition
>11y: #1 is SCFE, #2 is rheumatological condition, and #3 is trauma, #4 is tarsal coalition, #5 is neoplasia
ANORECTAL MALFORMATION:
if high, ureteral reflux is more common, so there will be increased incidence of UTI
VASCULAR RINGS:
double aortic arch is the most common one and also the most symptomatic. A pulm sling is also symptomatic, except that it is a sling and not a ring. You get ring-sling sydrome due to associated complete tracheal cartilage ring. The second most common vasc ring would be a R arch with an aberrant L subclavian and a ductus
OLIGOHYDRAMNIOS:
DRIPP C or demise, renal anomalies, infection, post dates, PROM, choramnionitis
POLYHYDRAMNIOS:
TARDI (twins, anomalies, rh incompatability, diabetes, idiopathic)
INTUSSUSCEPTION:
per Dr. Miller: 3 mo to 3 y. Crampy abdominal pain. Currant Jelly stool. Lethargy. Plain film shows SBO. There is presence of a palpable RLQ mass. Characteristic mass can be seen on US with concentric rings and a "pseudokidney sign." With pneumatic treatment, the success rate is 80% using about 80-120 mm of Hg. With hydraulic, there is a 70% success rate. Use the rule of 3's. Bag 3 feet above table, try 3 x, and wait 3 min to find it. This procedure can only be done in a facility with pediatric surgical backup. Using the pneumatic method, 18F foley catheter is inserted in the rectum. After taping it in place, 120 mmHg max, 3 min, make sure that you do not inflate the balloon. Try for 3 attempts
APPENDICITIS:
per Dr. Miller: US of the appendix is sensitive in > 90% of cases. It is also >90% specific.Diameter of the appendix of >6mm from outer to outer, appendicolith, free fluid, color flow all help to improve specificity
MECKELS:
15-50% contain ectopic mucosa and of these, 50% contain gastric mucosa. Then use the rule of 2's ie 2 y, 2% of the population, 2 feet from the terminal ileum, 2 inches long etc
BONE MARROW DEVELOPMENT:
conversion of red to yellow marrow takes place during growth and development. It begins immediately post natal in the diaphysis and progresses toward the metaphysis and axial skeleton. The epiphysis is not yet formed; however, once they ossify, rapid conversion to yellow marrow is the rule
TOO MUCH AIR IN THE STOMACH:
baby crying, attempted NG intubation, antral web, pyloric stenosis, duodenal atresia, midgut volvulus. Other causes include: pylorospasm, antropyloric inflammation
TOO LITTLE AIR IN THE STOMACH:
esophageal atresia, microgastria
DDX OF A HIGH OBSTRUCTION:
malrotation, annular pancreas, duodenal or jejunal atresia, duodenal web, preduodenal portal vein. Note that atresia is much more common than stenosis. Ladd bands can obstruct the duodenum descending portion. Bile stained vomitus within the first 24 h of life is the hallmark of duodenal atresia
DDX OF LOW INTESTINAL OBSTRUCTION:
imperforate anus, meconium plug syndrome, meconium plug sydrome, hirshprungs, meconium ileus, ilieal atresia, colonic atresia
NEONATAL BOWEL OBSTRUCTION:
atresia>malrotation> meconium ileus
BLOUNTS:
congenital tibia vara. 3 age groups: infantile (1-3y), juvenile (4-10), adolescent
CAFFEY DZ:
cortical periostitis at multiple sites of unknown etiology. Could be viral. It is self limited, benign, and occurs before 6 mo of age. It affects the tibia, ulna and mandible as well as digits
DEFINITION OF DWARFISM:
height 4 SD below the mean
EPIGLOTTITIS:
supraglottic inflammatory process with thumb like epiglottis, thickened aryepiglottic folds, and subglottic edema in up to 25% of patients
COMMON PLACES FOR AN INGESTED F.B TO LODGE:
thoracic inlet, aortic arch, L mainstem brochus, GEJ
RETROPHARYNGEAL CELLULITIS:
increased ap diameter of the prevertebral ST's and straigtening of the normal cervical lordosis. Plain film is limited in its ability to distinguish retropharyngeal and parapharyngeal abscess
ORIGIN OF MECKELS DIVERTICULUM:
vitelline duct. Male>female for symptomatic ones, and M=F for assymtomatic ones
MOST COMMON MEDIASTINAL MASS IN A CHILD:
neuroblastoma>teratoma