43 Pediatrics Flashcards
foregut
lungs, esophagus, stomach, pancreas, liver, gallbladder, bile duct, duodenum proximal to ampulla
midgut
duodenum distal to ampulla, small bowel, large bowel to distal 1/3 of transverse colon
hindgut
distal 1/3 of t colon to anal canal
rotation of gut during embryonic development
midgut rotation 270 degrees counterclockwise normally
define low birth weight
<2500 g
define premature
< 37 weeks
immunity at birth
IgA from mom’s milk … IgG crosses placenta
1 cause of childhood death
trauma
trauma bolus in kids
20cc/kg x 2, then give blood 10cc/kg
best indicator of shock
tachycardia …. neonat >150, <1yr >120, rest >100
goal UOP in trauma
> 2-4 cc/kg/hr
renal function in kids
kids <6mo only have 25% GFR cpacity of adults, poor concentrating ability
mechanism of bone growth in kids
inc alk phos in kids relative to adults
umbilical vessels
2 arteries, 1 vein
mIVF amounts
4cc/kg/hr for 1st 10kg … 2 for 2nd 10kg … 1 for everything after that
congenital cystic disease of the lung: list types
pulmonary sequestration, congenital lobar overinflation (emphysema), congenital cystic adenoid malformation, bronchiogenic cyst
congenital cystic disease of the lung: pulmonary sequestration - describe
lung tissue has anomalous systemic arterial supply (via thoracic aorta or abdominal aorta through inferior pulmonary ligament), have either systemic venous or pulmonary vein drainage
congenital cystic disease of the lung: pulmonary sequestration - locations
extra-lobar = more likely to have systemic venous drainage (azygous system) …. vs … intra-lobar = more likely to have pulmonary vein drainage
congenital cystic disease of the lung: pulmonary sequestration - px
does NOT communicate with tracheobronchial tree … MC p/w infection, can also have resp compromise or abnormal CXR … tx w lobectomy
congenital cystic disease of the lung: congenital lobar overinflation (emphysema) - pathophys
cartilage fails to develop in bronchus —> air trapping with expiration … vascular supply and other lobes are normal (except to the extent to which they are compressed by the hyperinflated lung) … can develop hemodynamic instability (same mech as tension PTX) or respiratory compromise
congenital cystic disease of the lung: congenital lobar overinflation (emphysema) - MC lobe affected
LUL
congenital cystic disease of the lung: congenital lobar overinflation (emphysema) - tx
lobectomy
congenital cystic disease of the lung: congenital cystic adenoid malformation - pathophys
communicates with airway, alveolar structure is poorly developed, although lung tissue is present
congenital cystic disease of the lung: congenital cystic adenoid malformation - sx
respiratory compromise or recurrent infection
congenital cystic disease of the lung: congenital cystic adenoid malformation - tx
lobectomy
congenital cystic disease of the lung: bronchiogenic cyst - describe
MC cysts of the mediastinum, usually posterior to the carina, extra-pulmonary cysts formed from bronchial tissue and cartilage wall
congenital cystic disease of the lung: bronchiogenic cyst - px
usually p/w mediastinal mass filled with milky liquid, can compress adjacent structures or become infected, have malignant potential, occasionally are intra-pulmonary
congenital cystic disease of the lung: bronchiogenic cyst - tx
resect cyst
mediastinal masses in children: MC
neurogenic tumors (neurofibroma, neuroganglioma, neurobalstoma) are the MC mediastinal tumors in kids, usually located posteriorly
mediastinal masses in children: px
respiratory sx, dysphagia - common to all mediastinal masses, regardless of location
mediastinal masses in children: list regions
anterior, middle, posterior
mediastinal masses in children: anterior masses
T cell lymphoma, teratoma, other germ cell tumors (MC anterior mediastinal mass in kids), thyroid CA
mediastinal masses in children: middle masses
T cell lymphoma, teratoma, cyst (cardiogenic or bronchiogenic)
mediastinal masses in children: posterior
T cell lymphoma, neuroblastoma, neurogenic tumor
mediastinal masses in children: thymoma
RARE in kids
choledochal cyst: casued by what?
reflux of pancreatic enzymes int the biliary system
choledochal cyst: inc risk for what?
cholangiocarcinoma, pancreatitis, cholangitis, obstructive jaundice
choledochal cyst: mgmt
need to resect
choledochal cyst: list types and frequencies
1 = 85%, 2 = 3%, 3 = 1%, 4 = 10%, 5 = 1%
choledochal cyst: rate, description, tx - type 1
85% …. fusiform dilation of entire CBD, mildly dilated common hepatic duct, normal intrahepatic ducts … tx w resection, hepaticojejunostomy
choledochal cyst: rate, description, tx - type 2
3% …. true diverticulum that hangs off CBD … resection off CBD, may be able to preserve CBD and avoid hepaticojejunostomy
choledochal cyst: rate, description, tx - type 3
1% …. dilation of distal intramural CBD, invovled sphincter of Oddi … resection, choledochojejunostomy
choledochal cyst: rate, description, tx - type 4
10% … multiple cysts, both intrahepatic and extrahepatic … resection, may need liver lobectomy, possible TXP
choledochal cyst: rate, description, tx - type 5
1% … caroli’s disease - intrahepatic cysts, get hepatic fibrosis, may be assoc with congenital hepatic fibrosis and medullary sponge kidney … resection, may need lobectomy, possible liver txp
lymphadenopathy: MC cause
acute suppurative adenitis assoc with URI or pharyngitis
lymphadenopathy: mgmt if fluctuant
FNA, culture and sensitivity, and abx … may need I&D if fails to resolve
lymphadenopathy: chronic causes
cat scratch fever, atypical mycoplasma (often fluctuant)
lymphadenopathy: mgmt if a-sx
abx x10d —> excisional bx if not improvement … dx is lymphoma until proven otherwise
lymphadenopathy: cystic hygroma
aka lymphangioma, usually found in lateral cervical regions in neck, gets infected, is usually lateral to SCM muscle, tx w resection
diaphragmatic hernia and chest wall: overall survival rate
50%
diaphragmatic hernia and chest wall: px
80% on L side … can have severe pulm HTN …. 80% have assoc anomalies (cardiac and neural tube defects mostly, malrotation)
diaphragmatic hernia and chest wall: dx
can be made with prenatal U/S
diaphragmatic hernia and chest wall: sx
respiratory distress
diaphragmatic hernia and chest wall: CXR
bowel in chest
diaphragmatic hernia and chest wall: tx
high frequency ventilation, inhaled nitric oxide, may need ECMO … stabilize before OR …. need to reduce bowel and repair defect +/- mesh (abdominal approach) … look for visceral anomalies (run the bowel)
Bochdalek’s vs Morgagni’s hernia
Bochdalek’s = MC, located posteriorly …. Morgagni’s = rare, located anteriorly
pectus excavatum vs carinatum
excavatum = sinks in, sternal osteotomy, need strut, performed if causing respiratory sx or emotional stree … carinatum = pigeon chest, strut not necessary, repair for emotional stress
branchial cleft cysts: lead to what
cysts, sinuses, fistulas
branchial cleft cysts: types, MC
1st, 2nd, 3rd branchial cleft cysts … 2nd = MC
1st branchial cleft cysts
angle of the mandible, may connect with external auditory canal, often assoc with facial nerve
2nd branchial cleft cysts
MC, on anterior border of mid-SCM muscle, goes through carotid bifurcation into tonsillar pillar
3rd branchial cleft cysts
lower neck, medial to or through the lower SCM
branchial cleft cysts: tx
resection
branchial cleft cysts: px
sinuses and fistulas usually in infants and young kids … cysts usually appear at later age
thyroglossal duct cyst: pathophys, px, tx
from the descent of the thyroid gland from the foramen cecum, may be only thyroid tissue pt has, presents as a midline cervical mass, goes through the hyoid bone, tx w excision of cyst, tract, and hyoid bone (at least central portion)
hemangioma: px, tx
appears at birth or shortly after, rapid growth during first 6-12 months of life, then begins to involute … tx = observation, most resolve by age 7-8 … if lesion has uncontrollable growth, impairs function (eyelid or ear canal) or is persistent after age 8 - can tx w oral steroids –> laser or resection if steroids are not successful
1 solid abdominal malignancy in kids
neuroblastoma
CT of neuroblastoma vs nephroblastoma
displacement vs replacement
neuroblastoma: px
a-sx abd mass in kids (MC) … can also have secretory diarrhea, raccoon eyes (orbital mets), HTN, and opsomyoclonus syndrome (unsteady gait)
neuroblastoma: location
MC is adrenal, can occur anywhere along sympathetic chain
neuroblastoma: age
MC in 1st 2 years of life
neuroblastoma: prognosis
best in kids <1yr
neuroblastoma: labs
inc catecholamines, VMA, HVA, metanephrines (HTN)
neuroblastoma: cell of origin
neural crest cells
neuroblastoma: vascular involvement
encases vasculature rather than invading
neuroblastoma: mets
rare, goes to lung and bone
neuroblastoma: abd xray findings
may show stippled calcifications in the tumor
neuroblastoma: worse prognosis
NSE, LDH, HVA, diploid tumors, N-myc amplification (>3 copies)
neuroblastoma: seen in pts with mets
NSE increase
neuroblastoma: tx
resection (adrenal gland and kidney), 40% cure rate … intially unresectable tumors may be resectable after doxorubicin-based chemo
neuroblastoma: staging
1 = localized, complete excision …. 2 = incomplete excision but does not cross midline … 3 = crosses midline +/- regional nodes …. 4 = distant mets (nodes or solid organ) … 4s = localized tumor with distant mets
Wilms tumor: aka
nephroblastoma
Wilms tumor aka nephroblastoma: px
usually px as a=sx mass, can have hematuria or HTN, 10% b/l
Wilms tumor aka nephroblastoma: mean age at dx
3yo
Wilms tumor aka nephroblastoma: prognosis
based on tumor grade (anaplastic and sarcomatous variation have worse prognosis)
Wilms tumor aka nephroblastoma: mets
frequent, to bone and lung
Wilms tumor aka nephroblastoma: mgmt of pulm mets
resect if resectable
Wilms tumor aka nephroblastoma: abd CT findings
replacement of renal parenchyma and NOT displacement (differentiates from neuroblastoma)
Wilms tumor aka nephroblastoma: tx
nephrectomy (90% cure rate) … if venous extension occurs in the renal vein, the tumor can be extracted from the vein … need to examine the contralateral kidney and look for peritoneal implants … avoid rupture of tumor with resection (this would increase stage) …. actinomycin and vincristine based chemo in all unless stage 1 and <500g tumor
Wilms tumor aka nephroblastoma: staging
1 = limited to kidney, complete excised … 2 = beyond kidney but completely excised … 3 = residual nonhematogenous tumor … 4 = hematogenous mets … 5 = b/l renal involvement
MC malignant tumor in kids
hepatoblastoma
hepatoblastoma: labs
inc AFP in 90%
hepatoblastoma: px
fractures, precocious puberty (from beta-hcg release)
hepatoblastoma: prognosis
better than HCC
hepatoblastoma: describe mass
can be pedunculated, vascular invasion is common
hepatoblastoma: tx
resection optimal, o/w doxorubicin and cisplatin based chemo –> may downstage tumors and make them resectable
hepatoblastoma: survival
primarily related to resectability
hepatoblastoma: best prognosis
fetal histology
1 kids malignancy overall
leukemia (ALL)
1 solid tumor class
CNS tumors
1 general surgery tumor in kids … in kids <2yo … in kids >2yo
neuroblastoma …. neuroblastoma … Wilms tumor
1 cause of duodenal obstruction in newborns (<1week) vs after newborn period (>1week) and overall
duodenal atresia … malrotation
1 cause of colon obstruction
Hirschsprung’s disease
1 liver tumor in kids
hepatoblastoma … 2/3 of liver tumors in kids are malignant
1 lung tumor in kids
carcinoid
painful vs painless lower GI bleeding
painful = anorectal lesions (i.e. fissues, etc) … painless = Meckel’s
upper GI bleeding 0-1yo vs 1yo-adult
gastritis, esophagitis … esophageal varcies, esophagitis
meckel’s diverticulum: location
antimesenteric border of small bowel
meckel’s diverticulum: embyrology
persistent vitelline duct
meckel’s diverticulum: rule of 2s
2 feet from ileocecal valve, 2% of population, 2% symptomatic, 2 tissue types (pancreatic MC, gastric most liklely to be sympomatic), and 2 presentations (diverticulitis and bleeding)
meckel’s diverticulum: #1 cause of
painless lower GI bleeding in kids
meckel’s diverticulum: workup
can get Meckel’s scan with pertechnetate if suspicious of diverticulum but having hard time locating
meckel’s diverticulum: tx
resection with sx, suspicion of gastric mucosa, narrow neck … diverticulitis involving base or if the base if >1/3 the size of the bowel, need to perform segmental resection
pyloric stenosis: typical pt
firstborn M, 3-12 weeks
pyloric stenosis: px
projectile vomiting … get hypoCl, hypoK metabolic alkalosis … feel olive mass in stomach
pyloric stenosis: U/S findings
pylorus >=4mm thick, >=14mm long
pyloric stenosis: fluid mgmt - boluses, what to avoid, infants
severe dehydration —> resuscitate with NS boluses until making urine —> switch to D5 NS with 10mEq K for maintenance …. AVOID fluid resus with K containing fluids in kids with severe dehydration b/c hyperK develops quickly, AVOID non-alt containing solutions in infants b/c hypoNa can quickly develop …. infants should always have a maintenance fluid with glucose b/c of their limited reserves for gluconeogenesis and vulnerability for hypoglycemia
pyloric stenosis: tx
pyloromyotomy = RUQ incision, proximal extent should be the circular muscles of the stomach
intussusception: typical age
3mo to 3yrs
intussusception: px
currant jelly stools (from vascular congestion, NOT an indication for resection), sausage mass, abdominal distention, RUQ pain, vomiting
intussusception: pathophys
invagination of one loop of intestine into another … lead points in kids = enlarged peyer’s patches (#1), lymphoma, Meckel’s diverticulum
intussusception: recurrence rate
15% after reduction
intussusception: tx
reduce with air contrast enema, 80% successful and no surgery needed if reduced … if fails, peritonitis present, or free air present, then go to OR
intussusception: enema characeristics
max P with air contrast enema is 120mmHg … max column height with barium enema is 1meter (3ft) … high perforation risk beyond
intussusception: surgical indications
peritonitis, free air, unable to reduce
intussusception: surgical technique
when reducing in OR, do NOT place traction on proximal limb of bowel - need to apply P to distal limb …. usually NO resection required unless associated with lead point (Meckel’s)
intussusception: mgmt if px in adults
most likely malignant lead point (i.e. colon CA in cecum) —> OR for resection
intestinal atresia: caused by what, sx, location, mgmt and tx
develop 2/2 intrauterine vascular accidents … sx = bilious emesis, distention, most do NOT pass meconium … MC in jejunum, can be multiple … get rectal bx to r/o Hirschsprung’s preop … tx w resection
1 cause of duodenal obstruction in newborns
duodenal atresia
duodenal atresia: location, px
usually distal to ampulla of vater … p/w bilious emesis, feeding intolerance
duodenal atresia: associated with what
polyhydramnios in mom … cardiac, renal, other GI anomalies … 20% with Down’s syndrome (check chromosomal studies)
duodenal atresia: xray
abd xr shows double-bubble sign
duodenal atresia: tx
resuscitation, duodenoduodenostomy or duodenojejunostomy
tracheoesophageal fistulas (TEF): MC type, 2nd MC type
type C = 80% = proximal esophageal atresia (blind pouch) and distal TE fistula … type A = 5% = esophageal atresia and no fistula
tracheoesophageal fistulas (TEF): type C - describe, sx, imaging
MC type (80%), proximal esophageal atresia (blind pouch) and distal TE fistula … sx = newborn spits up feed, excessive drooling, respiratory sx with feeding, cannot place NGT in stomach … abd xray = distended, gas-filled stomach
tracheoesophageal fistulas (TEF): type A - describe, sx, imaging
2nd MC type (5%), esophageal atresia and no fistula … sx similar to type C = newborn spits up, XS drooling, resp sx w feeds, can’t place NGT in stomach … abd xray = gasless abdomen
tracheoesophageal fistulas (TEF): tx
R extrapleural thoracotomy for most, perform primary repair, placed G tube, azygous vein often needs to be divided
tracheoesophageal fistulas (TEF): mgmt of infants that are premature, <2500g, or sick
replogle tube, treat resp sx, place G tube, delay repair
A Replogle tube is a medical device used in the treatment of babies with oesophageal atresia. It is a double-lumen tube which is inserted through the baby’s nostril into the blind-ending oesophageal pouch and used to drain saliva
tracheoesophageal fistulas (TEF): complications of repair
GERD, leak, empyema, stricture, fistula
tracheoesophageal fistulas (TEF): survival related to what
birth weight, assoc anomalies
describe VACTERL syndrome
Vertebral, Anorectal (imperforate anus), Cardiac, TE fistula, Radius/renal, Limb anomalies
malrotation: px
sudden onset bilious vomiting (Ladd’s bands cause duod obstruction, coming out from the R retroperitoneum)
malrotation: pathophys
assoc with compromise of SMA –> infarct of the intestine …. failure of normal COUNTERclockwise rotation (270 degrees) … Ladd’s bands cause duo obstruction, coming out from the R retroperitoneum
malrotation: age at presentation
75% in the first 1 month, 90% present by 1 year old
malrotation: dx
UGI - duo does NOT cross midline, duo-jej junction displaced to the R
malrotation: tx
resect Ladd’s bands, counterclockwise rotation (may require multiple turns), place cecum in LLQ (cecopexy), place duo in RUQ, and appendectomy
meconium ileus: leads to what
distal ileal obstruction, abd distention, bilious vomiting, distended loops of bowel
meconium ileus: assoc with what
CF - occurs in 10% of children with CF
meconium ileus: workup
need sweat chloride test or PCR for Cl channel defect (b/c of frequency of occurrence with CF)
meconium ileus: imaging
abd xr: dilated loops of small bowel without air-fluid levels - b/c meconium is too thick to separate from the bowel wall, can have ground glass or soapsuds appearance
meconium ileus: complications
can cause perforation, leading to meconium pseudocyst or free perforation —> requires laparotomy
meconium ileus: tx
gastrografin enema (effective in 80%), can also make the dx and potentially treat the pt … can also use N-acetylcysteine enema … if surgery required, manueal decompression and create a vent for N-acetylcysteine antegrade enemas
necrotizing enterocolitis (NEC): px
bloody stools after 1st feeding in premature neonate
necrotizing enterocolitis (NEC): risk factors
prematurity, hypoxia, sepsis
necrotizing enterocolitis (NEC): sx
lethargy, resp decompensation, abd distention, vomiting, blood per rectum
necrotizing enterocolitis (NEC): imaging
abd xr may show pneumatosis intestinalis, free air, or portal vein air … need serial lateral decubitus films to look for perforation
necrotizing enterocolitis (NEC): initial tx
resuscitation, NPO, abx, TPN, orogastric tube
necrotizing enterocolitis (NEC): surgical indications
free air, peritonitis, clinical deterioration
necrotizing enterocolitis (NEC): surgical approach
resect dead bowel and bring up ostomies … need Ba contrast enema before taking down ostomy to r/o distal obstruction from stenosis
necrotizing enterocolitis (NEC): mortality rate
10%
congenital vascular malformation: surgical indications, tx
surgery for hemorrhage, ischemia, CHF, nonbleeding ulcers, functional impairment, limb-length discrepancy … tx w embolization (may be sufficient on its own) and/or resection
imperforate anus: MC patients
M
imperforate anus: workup
check for associated VACTERL anomalies (vertebral, cardiac, TE fistula, Radius/Renal, Limb)
imperforate anus: types
high (above levators) … low (below levators)
imperforate anus: high type - px, tx
high = above levators … meconium in urine or vagina (fistula to bladder/vagina/prostatic urethra) … tx w colostomy, later anal reconstruction with posterior sagittal anoplasty (pull anus down into sphincter mechanism) … need postop anal dilatation to void structure, these pts are prone to constipation
imperforate anus: low type - px, tx
low = below levators … meconium to perineal skin … perform posterior sagittal anoplasty (pull anus down into sphincter mechanism), NO colostomy needed … need postop anal dilatation to void structure, these pts are prone to constipation
imperforate anus: px, tx - high vs low
high = above levators, low = below … high = mec in urine or vagina (fistula to bladder/vag/prostatic urethra), low = mec to perineal skin … BOTH = tx w posterior sagittal anoplasty (pull anus into sphincter mechanism), but high = colostomy first and later recon, low = no colostomy … BOTH = need postop anal dilatation to void structure, these pts are prone to constipation
gastrochisis vs omphalocele
gastro = intrauterine rupture of umbilical vein, does NOT have peritoneal sac, 10% assoc congenital anomalies, to R of midline … omphalo = failure of emb development, WITH peritoneal sac, 50% with assoc abnL, midline defect … malrotation may occur with both … omphalo has worse prognosis 2/2 assoc abnormalities
gastroschisis: pathophys
intrauterine rupture of umbilical vein, does NOT have a peritoneal sac
gastroschisis: describe appearance
to R of midline, NO peritoneal sac, stiff bowel from exposure to amniotic fluid
gastroschisis: assoc congential anomalies
only about 10%, malrotation
gastroschisis: tx
initially place saline-soaked gauze and resuscitate pt (can lose a lot of fluid from the exposed bowel), TPN, NPO … repair when pt is stable … at operation may try to place bowel in abdomen, +/- vicryl mesh silo - primary closure at a later date if use silo
Cantrell pentalogy
(1) cardiac defect, (2) pericardium defects (usually at disphragmatic pericardium), (3) sternal cleft or absence of lower sternum, (4) diaphragmatic septum transversum absence, (5) omphalocele
omphalocele: pathophys
failure of embryoneal development, HAS peritoneal sac with cord attached
omphalocele: describe appearance
midline defect, WITH peritoneal sac with cord attached, ac can contain structures other than bowel (i.e. liver, spleen, etc)
omphalocele: assoc congenital abnormalities
50%, midline defect
omphalocele: tx
initially place saline-soaked gauze and resuscitation pt, can lose a lot of fluid from exposed bowel, TPN, NPO … repair with pt is stable … at operration, try to placed bowel back in abdomen, may need Vicryl mesh silo, primary closure at a later date if mesh used
omphalocele: prognosis
worse than gastroschisis 2/2 congenital anomalies
1 cause of colonic obstruction in infants
Hirschsprung’s disease
Hirschsprung’s disease: gender
MC in M
Hirschsprung’s disease: MC sign
(#1 cause of colonic obstruction in infants), MC sign = infants fail to pass meconium in 1st 24 hours … can also p/w chronic constipation in ages 2-3
Hirschsprung’s disease: px
infant fails to pass meconium in 1st 24hours, chronic constipation in ages 2-3 …. distention, occasionally colitis … explosive release of watery stool with anorectal exam
Hirschsprung’s disease: dx
rectal bx = absence of ganglion cells in myenteric plexus
Hirschsprung’s disease: pathophys
failure of neural crest cells (ganglion cells) to progress in caudad direction (which is why you see the absence of ganglion cells in myenteric plexus on rectal bx)
Hirschsprung’s disease: surgery
resect colon proximally to the point where ganglion cells appear … tx = may need to bring up initial colostomy, then eventually connect the colon to the anus (Soave or Duhamel procedures)
Hirschsprung’s colitis: describe, px, tx
may be rapidly progressive …abd distention, foul-smelling diarrhea, +/- lethargy and signs of sepsis …. tx = rectal irrigation and try to empty colon, may need emergency colectomy
umbilical hernia: describe, MC pts, tx
failure of closure of linea alba, most close by age 3, rare incarceration … inc in Af American and premature infants … tx = surgery if not closed by age 5, incarceration, pt w VP shunt
inguinal hernia: pathophys
persistent processus vaginalis
The vaginal process (or processus vaginalis) is an embryonic developmental outpouching of the parietal peritoneum. It is present from around the 12th week of gestation, and commences as a peritoneal outpouching.
In males, it precedes the testes in their descent down within the gubernaculum, and closes. This closure (also called fusion) occurs at any point from a few weeks before birth, to a few weeks after birth. The remaining portion around the testes becomes the tunica vaginalis. If it does not close in females, it forms the canal of Nuck.
inguinal hernia: rate in infants, MC gender
3% of infants, M > F
inguinal hernia: laterality
60% on R, 30% on L, 10% b/l
inguinal hernia vs hydrocele
extension of hernia into the internal ring
inguinal hernia: tx
non reducible —> emergent operation … o/w elective repair with high ligation … consider exploring the contralateral side IF L sided, F, chidl <1yo …. need operation within 24 hours of reduction
hydrocele: describe, tx
most disappear by 1yo, noncommunicating will resolve, shoudl transilluminate … tx = surgery at 1 year if not resolved or if through to be communicating (waxing and waning size), resect hydrocele and ligate processus vaginalis
cyst duplication: MC site, tx
MC in ileum, often on mesenteric border, tx = resect cyst
MC cause of neonatal jaundice requiring surgery
biliary atresia
biliary atresia: px
progressive jaundice persisting >2weeks after birth is suggestive
biliary atresia: pathophys
can involve the extrahepatic, intrahepatic, or both biliary trees
biliary atresia: dx
liver bx –> periportal fibrosis, bile plugging, eventual cirrhosis
biliary atresia: imaging
U/S and cholangiography can reveal atretic biliary tree
biliary atresia: progression
get cholangitis, continued cirrhosis, eventual liver failure
biliary atresia: surgical tx
Kasai procedure (hepaticoportojejunostomy, resect atretic extrahepatic bile duct segment) … BEFORE 3mo old, o/w get irreversible liver damage
biliary atresia: surgical outcomes
1/3 get better, 1/3 need liver txp, 1/3 die
teratoma: labs
inc AFP and b-HCG
teratoma: MC site
neonates = sacrococcygeal … adolescents = ovarian
teratoma: tx
excision
sacrococcygeal teratomas: malignancy, marker, tx
90% benign at birth (almost all have exophytic component), but HIGH malignant potential … 2 months is an important transition point: <2mo usually benign, >2mo usually malignant … AFP = good marker … tx = coccygectomy and long-term follow up
undescended testicles: treatment age
wait until 2yo to treat
undescended testicles: inc risk for what
testicular cancer … inc cancer risk stay the same even after the testicles are brought into the scrotum … get seminomas
undescended testicles: mgmt if b/l
get chromosomal studies
undescended testicles: imaging needed
if you cannot feel the testes in the inguinal canal, then get MRI to confirm their presence
undescended testicles: tx
orchiopexy through inguinal incision … if unable to get testicles down –> close and wait 6mo before trying again … if they will not come down then perform division of spermatic vessels
tracheomalacia: describe
elliptical, fragmented tracheal rings instead of C shape
tracheomalacia: px
wheezing, usually improves after 1-2 years
tracheomalacia: surgical indications
dying spell, failure to wean from vent, recurrent infections
tracheomalacia: surgical approach
aortopexy (aorta suture to the back of the sternum, open up trachea)
MC cause of airway obstruction in infants
laryngomalacia
laryngomalacia: pathophys
immature epiglottis cartilage with intermittent collapse of the epiglottis airway
laryngomalacia: sx
intermittent respiratory distress and stridor exacerbation in the supine position
laryngomalacia: natural history
most children outgrow this by 12months
laryngomalacia: surgical mgmt
surgical tracheostomy for very few patients
choanal atresia: describe
obstruction of choanal opening (nasal passage) by either bone or mucus membrane, usually unilateral
choanal atresia: sx
intermittent resp distress, poor suckling
choanal atresia: tx
surgical correction
MC tumor of the pediatric larynx
laryngeal papillomatosis
laryngeal papillomatosis: natural hx
frequently involutes after puberty
laryngeal papillomatosis: tx
can tx w endoscopic removal or laser, but frequently recurs
laryngeal papillomatosis: caused by what
thought to be 2/2 HPV in the mom during passage through the birth canal
GI issue assoc with cerebral palsy
GERD
dx studies and likely dx with following hx and PE: bilious emesis, abd distention
plain abd film … dx = intestinal atresia or stenosis
dx studies and likely dx with following hx and PE: failure to pass meconium, bilious emesis, acholic meconium, gastric distention, trisomy 21
contrast enema, plain abd film, upper GI contrast study … dx = duodenal atresia or stenosis
dx studies and likely dx with following hx and PE: failure to pass meconium, bilious emesis (late), absent anus or visible fistula, abd distention
plain chest and abd films, U/S of kidneys, sacrum, rectum … dx = imperforate anus
dx studies and likely dx with following hx and PE: high-risk premature infant, bilious emesis, abd distention, hematochezia, guaiac positive stool
plain abd film … dx = necrotizing enterocolitis
dx studies and likely dx with following hx and PE: CF (10%), bilious emesis, acholic meconium, abd distention
plain abd film, contrast enema … dx = meconium ileus
dx studies and likely dx with following hx and PE: bilious emesis, term healthy infant, no abd distention
plain abd film … dx = malrotation
dx studies and likely dx with following hx and PE: delayed passage of meconium, bilious emesis, abd distention, trisomy 21
plain abd film, contrast enema … dx = Hirschsprung’s disease
GI anomalies assoc with trisomy 21
Hirschsprung’s, duodenal atresia
ddx of bilious emesis in infant
intestinal atresia (also w abd distention) … duodenal atresia (also w failure to pass or acholic mec, gastric distention, trisomy 21) … imperforate anus (late bilious emesis, also w failure to pass mec) … NEC (in high risk, premature infant w abd distention and hematochezia) … mec ileus (assoc w CF) … malrotation (no abd distention, healthy term infant) … Hirschprung’s (with delayed mec, abd distention, trisomy 21)
ddx of failure to pass meconium in infant
duodenal atresia or stenosis, imperforate anus, meconium ileus (10% of CF patients), Hirschsprung’s (delayed passage, >24hr)
