Pediatrics Flashcards
Earliest indicator of shock in a child after trauma is?
tachycardia
In a pediatric trauma pt who is hypotensive and tachycardic what fluids do we give?
bolus of 20 cc/kg of crystalloid should be given x2
if that fails 10 cc/kg of pRBCs
How do we calculate maintenance fluids for an infant?
4 cc/kg for first 10 kg
2cc/kg for next 11-20 kg
1 cc/kg for each additional kg
(ex: 26 kg kid as maintenance fluids; (4 x 10) + (2x10) + (1x6) = 66
The most common cause of neck mass in children is?
lymphadenopathy
can be seen midline or laterally
What is a thyrglossal duct cyst?
thyroid descends down into normal anatomic position
residual thyroid tissue that remains after normal descent, usually found in neck midline is a thyroglossal duct cyst
In pts with thyrloglossal duct cysts, we usually need to a nuclear scan to confirm what?
if pt has normal thyroid tissue
sometimes a pts midline ectopic thyroid tissue can be mistaken for a thyroglossal duct cyst, and that’s all the thyroid tissue they have
Tx for thyrglossal duct cysts?
if cyst presents w/abscess; incision and drainage, abx
once inflammation resolved–> resection of cyst, with tract, and central portion of hyoid bone, and resection of foramen cecum
SISTRUNK procedure
What is a cystic hygroma?
lymphatic malformation
occurs due to obstructed lymph vessels
Cystic hygromas; lymphatic malforations, tend to occur where?
posterior neck triangle
axilla, groin, mediastinum
The presence of a lateral neck mass in infancy, in association with head rotation to opposite side indicates what?
congenital torticollis
What causes congenital torticollis?
fibrosis of SCM
Tx–> PT w/passive stretches
rarely–> surgery with SCM transection
What’s a Bochdalek hernia?
congenital diaphragmatic hernia
postero-lateral
This is a congenital diaphragmatic defect that is postero-lateral:
Bochdalek henria
Congenital diaphragmatic hernias are more common on Left or Right?
LEFT
Bochdaleks
What happens in pts with congenital diaphragmatic hernias?
bowel contents in chest shift mediastinum to opposite site, air exchange in contralateral lung is fucked
pulmonary htn develops; fetal circulation persists (you get R–>L shunting)
lung on affected side becomes hypoplastic; useless
Tx for CDH?
mechanical ventilation–>low, gentle settings (pCO2 in 50-60 range are accepted)
inhaled NO used to improve oxygenation
ECMO
How is a CDH repaired?
thoracic or abdominal approach
open vs laparoscopic
What is pulmonary sequestration?
a mass of lung tissue, usually in LLL
occurs without the usual connections to the tracheo-bronchial tree or pulmonary artery
but it has a systemic blood supply from aorta
***can be extralobar vs intralobar
Whats difference between extralobar vs intralobar sequestration?
extralobar–> small area of non-aerated lung separate from main lung, with a systemic blood supply, usually above left diaphragm
intrlaobar–> usually occurs within parenchyma of LLL, with no major connection to tracheobronchial tree
Most common object aspirated is?
peanuts
Most common anatomic location for a foreign body is?
R-mainstem bronchus or RLL
What type of bronchoscope should be used in trying to obtain aspirated foreign objects from airways?
rigid
Most common foreign body in the esophagus?
small coin
followed by small toy
In terms of objects getting stuck in the esophagus, what 3 locations do they usually get stuck at?
cricopharyngeus
aortic arch
GE junction
What three parts of the esophagus have a normal anatomic narrowing and objects can sometimes get stuck?
circopharyngeus
aortic arch area
GE junction
What are the 5 types of esophageal atresia and TEF?
A– > isolated esophageal atresia (10%)
B–> EA, with proximal TEF
C–> EA, with distal TEF (85%)
D–> EA, with TEF between distal and proximal parts of esophagus and trachea
E–> TEF without EA (H-type)
What is the most common TEF?
type C (85% of cases)
EA with distal TEF (distal esophagus connected to trachea)
Congenital anomalies assc with TEF?
VACTERRL; vertebral anomalies, anorectal, cardiac defects, TEF, renal
Most commonly used options for esophageal substitutions are?
R,L, T-colon
or stomach
What is a Morgnagni CHD?
antero-medial defect
Who gets pyloric stenosis more? M/F?
Males 5;1
**first born male infants at highest risk
Pyloric stenosis is most common during what age?
3-6 weeks
Metabolic derangement seen in pyloric stenosis?
hypochloremic, hypokalemic, metabolic alkalosis
US can diagnose pyloric stenosis, what do you need to see on US?
channel length >16 mm
pyloric thickness >4 mm
Tx for pyloric stenosis?
not a surgical emergency
correct electrolyte derangements
Fredet-Ramstedt pyloromyotomy
How is a pyloromyotomy done for pyloric stenosis?
open (RUQ or supraumbilical incision) vs laparoscopic
splitting of the pyloric muscle while leaving the submucosa intact
incision is from pyloric vein of mayo to gastric antrum (1-2 cm long)
Complications of pyloromyotomy?
perforation
incomplete myotomy
bleeding
Cardinal symptom of intestinal obstruction in the newborn is?
bilious vomiting
In all cases of suspected intestinal obstruction, what imaging do we get?
xrays to check for air fluid levels
infant bowel does not have haustra or plica circulares, difficult to differentiate large vs SB
In infants with duodenal obstruction, why is the emesis bilious?
usually obstruction is past the ampulla of vater in most cases
Classic xray seen in infants suspected of having duodenal atresia?
double bubble sign
1/3 of pts with duodenal obstruction/atresia have what assc syndrome?
down’s
Surgical tx of duodenal atresia/obstruction?
duodenoduodenostomy
During normal embryological development, the midgut expands out of abdominal cavity into the umbilical cord, returns back into the belly and does a 270 counterclockwise rotation around what structure?
SMA
Surgical procedure for malrotation?
Ladds procedure
What’s the Ladd band procedure for malrotation?
bands between cecum and abd wall removed
bands between duodenum and terminal ileum removed
appendix removed to avoid confusion later in life
Why is an appendectomy performed during a Ladds procedure of volvulus?
after detorsion and cutting of adhesive bands to free up the small intestine and cecum, the small intestine is on the RLQ, and the cecum is on the left side of abdomen
What is meconium ileus>
infants with CF (CFTR gene mutation **AR) have pancreatic enzyme deficiencies and abnormal Cl secretions
produce a viscous, water-poor meconium
this highly viscous meconium can become impacted in ileum –> high grade obstruction
What do we see on xray of baby with suspected meconium ileus?
ground-glass opacities
small bubbles of gas get trapped in meconium in terminal ileus
**air fluid levels don’t form, bc the meconium is so viscous, thick
How do pts with meconium ileus present?
abdominal distention
bilious emesis
failure to pass meconium
**these are seen in first 24-48 hrs of life
Meconium ileus can be simple vs complicated, whats the difference
complicated–> intestinal perforation
simple–> dilated SB loops with absent air fluid levels, ground-glass opacities seen
Initial diagnostic study of choice for meconium ileus?
contrast enema using gastrograffin
gastrograffin is hypertonic, can aid in meconium evacuation (***Just make to hydrate the kids)
What will a contrast enema study in suspected meconium ileus show?
micro-colon with soap bubble sign in RLQ
If contrast enema not successful for simple meconium ileus what do we do?
surgery–> enterotomy made in small bowel, N-acetylcysteine with warm saline injected into bowel lumen to help remove meconium from bowel mucosa *breaks up disulfide bonds)
enterotomy then closed
Most important risk factor for development of NEC?
prematurity
What are some hallmark features of NEC?
abdominal distention/tenderness
feeding intolerance
blood in stool
NEC commonly affects what part of colon?
terminal ileum
colon
What do we see on abdominal xray of kid with suspected NEC?
pneumatosis intestinalis
How do we manage NEC?
NGT decompression
IV abx
IVF
blood/plts transfusions
When do we operate on pts with NEC?
presence of intestinal perforation on xray
Surgical principles in tackling NEC?
resect all nonviable segments of bowel
create a stoma
Most common cause of short gut syndrome in kids?
NEC
In pts with NEC, who undergo surgical procedures, what is a common side effects?
20% develop strictures
contrast enema is mandatory before re-establishing GI continuity
Pts with NEC, who require intestinal resections are at risk of developing what?
short gut syndrome
Leading cause of intestinal obstruction in young kids?
intussusception; segment of intestine becomes drawn into the lumen of a proximal segment of bowel
Where does intussuception usually occur?
begins in terminal ileum and progresses through the colon
Cause of intussusception?
hypertrophied peyer’s patches in terminal ileum from a previous infection act as a lead point
What do we see with stool in suspected intussuspcetion?
currant jelly stools (blood stools)
Tx for intussusception?
if child does not have peritonitis; radiographic reduction is attempted
Preferred method for diagnosis/treatment of intussuseption?
air enema
**if unsuccessful the first time, repeat air enema in 2-3 hrs
Sx treatment for intussusception?
RLQ incision made
bowel is slowly milked out
appendectomy done due to vascular compromise of appendix
Whats the rate of recurrence of intussusception regardless if it was treated radiographyically or surgically?
5-10%
If you perform a diagnostic laparoscopy on a child who you suspect has appendicitis, but the appendix appears normal when you enter the abdomen, what do you do?
take the appendix
MOst common complication after an appendectomy is?
SSI
What is a Meckel’s?
remnant of ophalomesenteric (vitelline) duct
Where do we normally find Meckel’s diverticula
2 ft from ileo-cecal valve
on anti-mesenteric border of SB
Why do we see bleeding with Meckel’s?
can have ectopic gastric mucosa
can cause ileal ulcers
What two types of tissue do we see in a Meckels?
pancreatic and gastric mucosa
Rule of 2s for Meckels?
2% incidence
within 2 ft of ileo-cecal valve
2 inches long
symptomatic by age 2
Is a Meckel’s diverticulum a true diverticulum?
yes
encompasses all tissue layers
Most common symptom with a Meckels?
massive lower GI bleed, painless
Test used to diagnose a Meckels?
pertechnetate Meckels scan looking for gastric mucosa
Surgical tx for a Meckels?
if base is narrow and no mass at base noted, wedge resection with closure of ileum noted
if inflammation or perforation present, SB resection with end-to-end SB anastomosis
What causes Hirshchripungs dx?
abscence of ganglion cells in Auerbach’s plexus
and hypertrophy of associated nerve trunks
What causes Hirschurpgs dx?
failure of neural crest cells to migration to distal colon
How is definitive dx of Hirschprungs dx made?
rectal bx
What are the three surgical procedures used for Hirschrpungs dx?
Swenson procedure–>aganglionic rectum is dissected in the pelvis and removed down to the anus
ganglionic colon then anastomosed to anus via perineal approach
Duhamel procedure–> ganglionic colon is anastomosed to anus via posterior approach
Soave procedure–> rectal mucosa is stripped off from the muscular sleeve and ganglionic colon is brought down thru this channel to anus
What gene associated with Hirschprung dx?
ret proto-oncogene
What is omaphelocele?
congenital abdominal wall defect
herniation of abdominal contents contained within peritoneum and amniotic membrane
This is protrusion of abdominal contents thru the umbilical ring with a covering over the intestines:
omphalocele
With gastroschisis there is no covering over the abdominal organs as they protrude, do they protrude to the right or left of umbilicus?
almost always to the RIGHT (at site of obliterated umbilical vein)
Associated anomalies noted with omphalocele?
Beckwith-Wiedmann syndrome; macroglossia, gigantism, and umbilical defect
pentology of Cantrell; omphalocele, anterior diaphragmatic hernia, sternal cleft, ectopia cordis, cardiac defects
Incidence of inguinal hernias?
10;1 male
more common on right side
usually due to failure of processus vaginalis to close
What do we do with pediatric umbilical hernias?
most resolve on own
wait till 5 yrs
In terms of biliary atresia, who is affected more?
asian babies
winter months
higher in females
What is NEC-totalis?
fulminant, rapidly progressive form of NEC
causes gangrene and necrosis of 75% of bowel
you have 25% or less viable intestines
**occurs in about 20% of pts with NEC
Virtually all survivors of NEC-totalis develop short gut syndrome and will require lifelong TPN, and be pre-disposed to what?
liver problems, cholestasis
Jaundice in new born?
physiologic
presents 2-3 days after birth, resolves in 5-7 days
babies have a lot of blood, and immature conjugation machinery (glucuronyl transferase)
jaundice beyond 2 weeks is considered pathologic
What is biliary atresia?
fibroproliferative obliteration of biliary tree
progresses to hepatic fibrosis, cirrhosis and liver failure
How do infants with biliary atresia present?
jaundiced at birth
pale-gray appearing stools
progress to failure to thrive
liver failure, portal htn
How do we diagnose biliary atresia?
bilirubin levels
look for TORCHES infection
US; usually see absent GB, (10% of cases you have a GB present)
never see intra hepatic dilated ducts
What are the 5 types of choledochal cysts?
type 1–> fusiform dilation of CBD (most common)
type 2–> isolated diverticulum protruding from wall of CBD
type 3–> cysts arise from the intra-duodenal portion of CBD
Type 4A–> many dilations of intra-extra hepatic bile ducts
Type 4B–> many dilations of only extra-hepatic bile ducts
Type 5–> multiple dilations of intra-hepatic ducts
Cause of choledochal cysts?
long common biliary and pancreatic channel
reflux of enzymes into biliary channel and weakening of wall
Tx for choledochola cysts?
complete cyst removal; followed by biliary enteric reconstruction
What is prune belly syndrome?
lax abdominal musculature b/l undescended testes (remain sterile after orchiopexy due to retrograde ejac) distended bladder (dilated ureters)
*AKA eagle barrett syndrome
What do we worry about with undescended testis?
risk of malignancy
1st and 2nd leading causing of death in pediatric population?
1st–> trauma
2nd–> cancer
Most common primary malignant tumor of kidneys in children?
Wilms tumor
97% are sproadic, occur without any genetic or risk factor
What’s a Wilm’s tumor?
most common primary malignant tumor of kidneys in children
most diagnosed between 1-5 yrs (3 yrs avg)
How is Wilms tumor found?
usually as an asymptomatic mass in child’s flank
What syndrome is Wilms tumor associated with?
WAGR syndrome + Beckwith-Wiedmann
wilms
aniridia
GU probelms
mental retardation
What mutations cause WAGR syndrome?
abscence of WT1 gene and PAX6 gene
BEfore operating on Wilm’s tumor what is done first?
CT a/p/c to look for mets, identify mass better, and look at other kidney
Stages of Wilm’s tumor;
1–> confined to the kidney and completely excised
2–> extends beyond kidney but is completely excised , margins are clean, tumor was biopsied, local spillage of tumor confined to flank
3–> tumor confined to the abdomen, positive nodes, peritoneal implants, tumor not resectable because close to vital structures
4–> hematogenous mets
5—> b/l renal involvement
Do pts with Wilm’s tumor get chemo?
yes, for stage 1 they get a short course
for stage 4-5 they get a more intensive course
for stage 4 Wilm’s, cure rate 80%
Third most common pediatric malignancy?
neuroblastoma
Peak age of neuroblastoma?
2 yrs
Neuroblastomas arise from where?
neural crest cells
Where do neuroblastomas normally originate?
adrenals
posterior mediastinum
neck
pelvis
Some sxs of neuroblastomas?
proptosis and periorbital ecchymoses from retrobulbar mets
muscle weakness and sensory changes from spinal cord compression
diarrhea from VIPomas and paraneoplastic shit
WHat hormone markers help in diagnosis of neuroblastomas?
neuroblastomas derive from SNS
catecholamines and their metabolites will be produced in excess
you see increased levels of VMA and VHA, catecholamine metabolites
Most common liver lesion in kids?
hemangioma
Most common liver cancer in kids?
hepatoblastoma followed by HCC
Pectus excavatum is more common than carinatum and is associated with what syndromes?
congenital heart dx, MV prolapse
Ehlers-Danlos
marfans
Opsoclonus-myoclonus syndrome assc with what childhood can?
neuroblastoma
antibodies cross react with cerebellar tissue
Why do pts with neuroblastoma tend to have htn and tachycardia?
its a tumor from SNS
catecholamines are released
How much blood do we give a child in hypovolemic shock?
10 cc/kg
How much crystalloids do we give a child in trauma setting
20 cc/kg
Utility of FAST in pediatric trauma?
not routinely as reliable
Signs of non-accidental CNS trauma in kids?
retinal hemorrhages
intracranial hemorrhage without signs of external trauma
fractures of different stages of healing
After an appendectomy how long does it usually take for an abscess to form?
about 7 days
Primary reason for excision of thyroglossal duct cysts?
malignant potential
recurrent infections
In new borns why should choledochal cysts be removed?
risk of cholangitis
risk of malignant transformation
You suspect meconium ileus in a pt with abdominal distention, he is peritoneal and you take him to OR, why don’t you resect intestines?
bowel resection in a new born leaves them at risk for short gut syndrome
**surgery for meconium ileus–> make an enterotomy and use irrigation, there is no need for colostomy
MOst common abnormality associated with gastroschisis is?
intestinal atresia
chromosomal abnormalities and congenital heart dx more assc with omphalocele
In pts with congenital diaphragmatic hernias, what happens to their vessels?
can get pulmonary hypoplasia and pulmonary htn
You have a child with an undescended testis, and you can palpate it, what do you do?
laparoscopy
US not recommended due to poor sensitivity
Laproscopic vs open hernia repair in kids:
have similar recurrence rates
similar post-op length of stay
laparoscopic not less painful
**Laparoscopic can help visualize for femoral hernias while done routinely for inguinal hernias
When doing an open inguinal hernia repair, what nerve can be injured when opening the externl oblique aponeurosis?
ilioinguinal n
In trauma, handlebar injuries in kids assc with what injuries?
duodenal hematomas
pancreatic injuries
Male children with cystic fibrosis may have abscence of b/l what?
vas deferens
**usually seen during routine inguinal hernia repairs and should prompt looking for CF
Female children with sliding inguinal hernias have what organ trapped in hernia sac?
fallopian tube
Most H type EA fistulas are located at level of thoracic inlet, how do you access it?
right cervicotomy
Amyand hernia assc with?
appendix
Littre’s hernia assc with?
Meckel’s
4 different types of jejuno-ileal atresias?
1–> intact bowel wall and mesentery with a mucosal atresia
2-» blind ends separated by a fibrous cord
3a–> V-shaped mesenteric gap
3b–> large mesenteric gap
4–>multiple atresias
What is a poor prognostic factor for neuroblastoma?
MYCN amplification > 10 copies
Simple meconium ileus is treated with?
contrast enema as first line
aggressive fluids are given b/c gastrograffin will cause an osmotic diuresis
Most common cause of intussusception in a 2 year old is?
Meckels
followed by intestinal polyps and duplications
Pt has pyloric stenosis, underwent pyloromyotomy, is having persistent vomiting a week later, now what?
needs re-exploration
some nausea vomiting post-op is normal, but should resolve after 3-4 days
Gold standard for pectus excavatum?
Nuss bar
In someone suspected of hypertrophic pyloric stenosis, what do we see on US?
pyloric channel 16 mm or greater is usually seen
How long do you wait for an umbilical hernia to resolve on its own before you repair it?
> 4 yrs old
In EA, if we have an esophageal gap, how do we close the gap?
delayed primary repair is preferred if gap is less than 2 vertebral bodies long
if gap is larger, traction sutures are placed
for very large gaps, esophageal replacement with colon or jejunum can be done
Most common malignant neoplasm found in a Meckels?
carcinoid
0.5–5% chance of finding malignancy in Meckels
For choledochal cysts, first imaging modality ordered is?
US
then MRCP
Why dont we operate on pts with pyloric stenosis right away ?
need to correct their hypochloremic, hypokalemic, metabolic alkalosis first
they can develop apnea if not corrected and receive general anesthesia
How do we manage a type 1 choledochal cyst?
primary cyst excision
w/Roux-en-Y hepaticojejunostomy
What are type I and type IV branchial cleft cysts?
type 1–> superficial to SCM
type 4–> deep to carotid sheath
Things assc with jejunoileal atresia;
bilious emesis
distended abd
polyhydramnios
What is Poland syndrome?
congenital abscence of pec major m (sometimes pec minor, chest wall, ribs)
in females, assc w/abscence of breast tissue on affected side (amastia)
MOst common intra-abdominal solid tumor in children?
neuroblastoma
liver is most common site of mets
(histology: small round blue cells with rosette histology)
What is the kasai procedure used for?
for pt w/biliary atresia
roux-en-y hepatic protoenterostomy
**entire extrahepatic biliary tree is excised, roux limb is connected to liver plate
MOst common location for an undescended testis is?
superficial inguinal ring
next is superficial inguinal pouch
last is the inguinal canal
