chapter 43: pediatric surgery Flashcards
embryology: lungs, esophagus, stomach, pancreas liver, gallbladder, bile duct, and duodenum proximal to ampulla
foregut
embryology: lungs, esophagus, stomach, pancreas liver, gallbladder, bile duct, and duodenum proximal to ampulla
foregut
embryology: duodenum distal to ampulla, small bowel, and large bowel to distal 1/3 of transverse colon
midgut
distal 1/3 of transverse colon to anal canal
hindgut
how does the midgut develop?
midgut rotates 270 degrees counterclockwise normally
low birth weight
premature
immunity at birth
IgA from mother’s milk
IgG crosses the placenta
1 cause of childhood death
trauma
trauma bolus
20cc/kg x 2, then give blood 10 cc/kg
best indicator of shock
tachycardia (neonate > 150 ; 120 ; rest > 100)
trauma: goal urine output
> 2-4cc/kg
GFR in children (
children (
what causes bone growth in children?
increased alkaline phosphatase in children compared with adults
what are the umbilical vessels?
2 arteries and 1 vein
maintenance IV fluids
4cc/kg/hr for first 10 kg
2cc/kg/hr for 2nd 10 kg
1cc/kg/hr for everything after that
four kinds of congenital cystic disease of the lung
- pulmonary sequestration
- congenital lobar overinflation
- congenital cystic adenoid malformation
- bronchiogenic cyst
- lung tissue has anomalous systemic arterial supply (thoracic aorta or abdominal aorta through inferior pulmonary ligament)
- have either systemic venous or pulmonary vein drainage
- do not communicate with tracheobronchial tree
pulmonary sequestration
pulmonary sequestration: more likely to have systemic venous drainage (azygous system)
extra-lobar pulmonary sequestration
pulmonary sequestration: more likely to have pulmonary vein drainage
intra-lobar pulmonary sequestration
tx: pulmonary sequestration (congenital cystic disease of the lung)
lobectomy
tx: pulmonary sequestration (congenital cystic disease of the lung)
lobectomy
embryology: duodenum distal to ampulla, small bowel, and large bowel to distal 1/3 of transverse colon
midgut
distal 1/3 of transverse colon to anal canal
hindgut
how does the midgut develop?
midgut rotates 270 degrees counterclockwise normally
low birth weight
tx: congenital cystic adenoid malformation
lobectomy
immunity at birth
IgA from mother’s milk
IgG crosses the placenta
1 cause of childhood death
trauma
trauma bolus
20cc/kg x 2, then give blood 10 cc/kg
tx: bronchiogenic cyst
resect cyst
most common mediastinal tumor in children; usually located posteriorly
neurogenic tumors (neurofibroma, neuroganglioma, neuroblastoma)
GFR in children (
children (
what causes bone growth in children?
increased alkaline phosphatase in children compared with adults
what are the umbilical vessels?
2 arteries and 1 vein
maintenance IV fluids
4cc/kg/hr for first 10 kg
2cc/kg/hr for 2nd 10 kg
1cc/kg/hr for everything after that
four kinds of congenital cystic disease of the lung
- pulmonary sequestration
- congenital lobar overinflation
- congenital cystic adenoid malformation
- bronchiogenic cyst
is thymoma rare in children?
yes, thymoma is rare in children
pulmonary sequestration: more likely to have systemic venous drainage (azygous system)
extra-lobar pulmonary sequestration
pulmonary sequestration: more likely to have pulmonary vein drainage
intra-lobar pulmonary sequestration
how does pulmonary sequestration present? (congenital cystic disease of the lung)
most commonly presents with infection; can also have respiratory compromise or an abnormal CXR
tx: pulmonary sequestration (congenital cystic disease of the lung)
lobectomy
- cartilage fails to develop in bronchus, leading to air trapping with expiration
- vascular supply and other lobes are normal (except compressed by hyper inflated lobe)
congenital lobe overinflation (emphysema)
why can hemodynamic instability or respiratory compromise develop in congenital lobar overinflation (emphysema)?
same mechanism as tension PTX - vascular supply is compressed by hyper inflated lobe
most commonly affected lobe in congenital lobar overinflation
LUL most commonly affected
tx: congenital lobar overinflation (emphysema)
lobectomy
- communicates with airway
- alveolar structure is poorly developed, although lung tissue is present
- symptoms: respiratory compromise or recurrent infection
congenital cystic adenoid malformation
tx: congenital cystic adenoid malformation
lobectomy
- most common cysts of the mediastinum; usually posterior to the carina
- are extra-pulmonary cysts formed from bronchial tissue and cartilage wall
- occasionally are intra-pulmonary
bronchiogenic cyst
how do bronchiogenic cysts present?
usually present with a mediastinal mass filled with milky fluid
what problems can bronchiogenic cysts cause?
can compress adjacent structures or become infected; have malignant potential
tx: bronchiogenic cyst
resect cyst
most common mediastinal tumor in children; usually located posteriorly
neurogenic tumors (neurofibroma, neuroganglioma, neuroblastoma)
symptoms common to all mediastinal masses regardless of location
respiratory symptoms, dysphagia
anterior mediastinal masses
T cell lymphoma, teratoma, and other germ cell tumors (most common type of anterior mediastinal mad in children), thyroidCA
tx: cystic hygroma (lymphangioma)
resection
middle mediastinal masses
T cell lymphoma, teratoma, cyst (cardiogenic or bronchiogenic)
posterior mediastinal masses
t cell lymphoma, neuroblastoma, neurogenic tumor
is thymoma rare in children?
yes, thymoma is rare in children
caused by reflux of pancreatic enzymes into the biliary system in utero
choledochal cyst
why do choledochal cysts need to be resected in children?
risk of cholangiocarcinoma, pancreatitis, cholangitis, and obstructive jaundice
choledochal cyst: (85%): fusiform dilation of entire common bile duct, mildly dilated common hepatic duct, normal intrahepatic ducts
type 1 choledochal cysts
tx: choledochal cyst: type 1
resection, hepaticojejunostomy
choledochal cyst: (3%) a true diverticulum that hangs off the common bile duct
type 2 choledochal cyst
tx: type 2 choledochal cyst
resection off common bile duct; may be able to preserve common bile duct and avoid hepaticojejunostomy
choledochal cyst: (1%) dilation of distal intramural common bile duct; involves sphincter of Oddi
type 3 choledochal cyst
tx: type 3 choledochal cyst
resection, choledochojejunostomy
choledochal cyst: (10%) multiple cysts, both intrahepatic and extrahepatic
type 4 choledochal cyst
tx: type 4 choledochal cyst
resection; may need liver lobectomy; possible TXP
choledochal cyst: (1%) Caroli’s disease: intrahepatic cysts; get hepatic fibrosis; may be associated with congenital hepatic fibrosis and medullary sponge kidney
type 5 choledochal cyst
tx: type 5 choledochal cyst
resection; may need lobectomy; possible liver TXP
usual causes of lymphadenopathy
acute suppurative adenitis associated with URI or pharyngitis
management: fluctuant lymphadenopathy
FNA, culture and sensitivity, and antibiotics; may need incision and drainage if it fails to resolve
lymphadenopathy: chronic causes
cat scratch fever, atypical mycoplasma
management: asymptomatic lymphadenopathy
antibiotics for 10 days -> excisional biopsy if no improvement. this is lymphoma until proven otherwise
lymphadenopathy: usually found in lateral cervical regions in neck; gets infected; is usually lateral to the SCM
cystic hygroma (lymphangioma)
tx: cystic hygroma (lymphangioma)
resection
- overall survival 50%
- increased on left side (80%); can have severe pulmonary HTN
- diagnosis can be made with prenatal ultrasound
- symptoms: respiratory distress
diaphragmatic hernias and chest wall
are there associated anomalies with diaphragmatic hernias and chest wall?
80% have associated anomalies (cardiac and neural tube defects mostly; malrotation)
cxr: diaphragmatic hernia
bowel in chest
tx: diaphragmatic hernia
high-frequency ventilation; inhaled nitric oxide; may need ECMO
- stabilize these patients before operation
- need to reduce bowel and repair defect +/ mesh (abdominal approach)
- look for visceral anomalies (runt he bowel)
diaphragmatic hernia: most common, located posteriorly
bochdalek’s hernia
diaphragmatic hernia: rare, located anteriorly
morgagni’s hernia
management: pectus excavatum (sinks in)
sternal osteotomy, need strut; performed if causing respiratory symptoms or emotional stress
management: pectus carinatum (pigeon chest)
strut not necessary; repair for emotional stress
what can branchial cleft cyst cause?
leads to cysts, sinuses, and fistulas
branchial cleft cyst: angle of mandible; may connect with external auditory canal. often associated with facial nerve
1st branchial cleft cyst
branchial cleft cyst: on anterior border of mid-SCM muscle. goes through carotid bifurcation into tonsillar pillar
2nd branchial cleft cyst
branchial cleft cyst: lower neck, medial to or through the lower SCM
3rd branchial cleft cyst
most common branchial cleft cyst
2nd branchial cleft cyst
tx for all branchial cysts
resection
what causes thyroglossal duct cyst?
from the descent of the thyroid gland from the foramen cecum
- may be only thyroid tissue patient has
- presents as a midline cervical mass
- goes through hyoid bone
thyroglossal duct cyst
tx: thyroglossal duct cyst
excision of cyst, tract, and hyoid bone (At least the central portion)
- appears at birth or shortly after
- rapid growth during first 6-12 months of life, then begins to involute
hemangioma
tx: hemangioma
observation - most resolve by age 7-8
does wilms tumor metastasize?
frequent metastases to bone and lung
1 solid abdominal malignancy in children
neuroblastoma
- usually presents as an asymptomatic mass
- can have secretory diarrhea, raccoon eyes (orbital metastases), HTN, and opsomyoclonus syndrome (unsteady gait)
neuroblastoma
where do most neuroblastoma occur?
most often on adrenals; can occur anywhere along the sympathetic chain
when do most neuroblastoma present?
most common in 1st 2 yeas of life. children
neuroblastoma is derived from what?
derived from neural crest cells
lab findings in neuroblastoma
most have increased catecholamines, VMA, HVA, and metanephrines (HTN)
how does neuroblastoma affect vasculature?
encases vasculature rather than invades
rare metastases of neuroblastoma
go to lung and bone
abdominal xr: neuroblastoma
may have stippled calcifications in the tumor
wilms tumor: stage 5
bilateral renal involvement
neuroblastoma: what is increased in all patients with metastases?
NSE
tx: neuroblastoma
resection (Adrenal gland and kidney take; 40% cured)
management of unresectable tumors in neuroblastoma
initially unresectable tumors may be resectable after doxorubicin-based chemo
neuroblastoma: stage 1
localized, complete excision
best prognosis in hepatoblastoma
fetal histology
neuroblastoma: stage 3
crosses midline +/ regional nodes
neuroblastoma: stage 4
distant metastases (nodes or solid organ)
neuroblastoma: stage 4-S
localized tumor with distant metastases
usually presents as asymptomatic mass; can have hematuria or HTN; 10% bilateral
- mean age at diagnosis: 3 years
wilms tumor (nephroblastoma)
what is prognosis of wilms tumor based on?
prognosis based on tumor grade (anaplastic and sarcomatous variations have worse prognosis)
does wilms tumor metastasize?
frequent metastases to bone and lung
management of pulmonary mets in wilms tumor?
can resect pulmonary mets if resectable
abdominal ct: wilms tumors
replacement of renal parenchyma and not displacement (differentiates it from neuroblastoma)
how do you differentiate neuroblastoma and nephroblastoma on ct?
replacement of parenchyma and not displacement in nephroblastoma
tx: wilms tumor (nephroblastoma)
nephrectomy (90% cured)
- 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, which will increase stage
chemotherapy regimen for nephroblastoma (wilms tumor)
actinomycin and vincristine based chemo in all unless stage 1 and
wilms tumor: stage 1
limited to kidney, completed excised
wilms tumor: stage 2
beyond kidney but completely excised
upper GIB 1 year to adult
esophageal varices, esophagitis
wilms tumor: stage 4
hematogenous metastases
wilms tumor: stage 5
bilateral renal involvement
most common malignant liver tumor in children; increased AFP in 90%
hepatoblastoma
- fractures, precocious puberty (from beta-hcg release)
- better prognosis than hepatocellular CA
- can be pedunculated; vascular invasion common
hepatoblastoma
tx: hepatoblastoma
resection optimal; otherwise doxorubicin and cisplatin based chemotherapy -> may downstage tumors and make them resectable
what is survival based on in hepatoblastoma?
survival is primarily related to resectability
best prognosis in hepatoblastoma
fetal histology
1 children’s malignancy overall
leukemia (ALL)
1 solid tumor class
CNS tumors
1 general surgery tumor
neuroblastoma
1 general surgery tumor in child
neuroblastoma
1 general surgery tumor in children > 2 years
wilms tumor
1 cause of duodenal obstruction in newborns (
duodenal atresia
1 cause of duodenal obstruction after newborn period (>1 week) and overall
malrotation
1 cause of colon obstruction
hirschsprung’s disease
1 liver tumor in children
hepatoblastoma; 2/3 of liver tumors in children are malignant
1 lung tumor in children
carcinoid
1 cause painful lower GIB
benign anorectal lesions (fissures, etc)
1 cause painless lower GIB
meckel’s diverticulum
upper GIB 0-1 year
gastritis, esophagitis
what do you think about in adults presenting with intussusception?
patient most likely has malignant lead point (i.e. colon ca in cecum) -> OR for resection
found on anti mesenteric border of small bowel
- embryology: persistent vitelline duct
- # 1 cause of painless lower GIB in children
meckel’s diverticulum
rule of 2s in meckel’s diverticulum
2 ft from ileocecal vale, 2% population, 2% asymptomatic, 2 tissue types (pancreatic - most common; gastric - most likely to be symptomatic) and 2 presentations (diverticulitis and bleeding)
dx: meckel’s diverticulum
can get meckel’s diverticulum scan with pertechnetate if suspicious of meckel’s diverticulum and having trouble locating
tx: meckel’s diverticulum
resection with symptoms, suspicion of gastric mucosa, or narrow neck
- diverticulitis involving th ebase or if the base is > 1/3 the size of the bowel, need to perform segmental resection
3-12 weeks, firstborn males
projectile vomiting
can feel oliver mass in stomach
pyloric stenosis
acid base in pyloric stenosis
hypochloremic, hypokalemia, metabolic alkalosis
ultrasound in pyloric stenosis
pylorus >/ 4mm thick
>/ 14 mm long
tx: duodenal atresia
resuscitation, duodenuoduodenostomy or duodenojejunostomy
why avoid fluid resuscitation with K containing fluids in children with severe dehydration?
hyperkalemia can develop quickly
why avoid non salt containing solutions in infants?
hyponatremia can quickly develop
why do infants need a maintenance fluid with glucose?
because of their limited reserves for gluconeogenesis and vulnerability for hypogylcemia
tx: pyloric stenosis
pyloromyotomy (RUQ incision; proximal extent should be the circular muscles of the stomach)
- usually 3 months to 3 years
- currant jelly stools (from vascular congestion, not an indication for resection), sausage mass, abdominal distention, RUQ pain, and vomiting
- invagination of one loop of intestine into another
intussusception
intussusception: lead points in children
enlarged peyer’s patches (#1), lymphoma, and meckel’s diverticulum
rate of recurrence after reduction in intussusception
15%
tx: intussusception
reduce with air-contrast enema -80% successful; no surgery required if reduced
intussusception: max pressure with air-contrast enema
120 mmHg
intussusception: max column height with barium enema
1 meter (3 feet)
intussusception: high perforation risk beyond 120 mmHg air contrast enema and 1 meter height barium enema
need to go to OR if you have reached these values
when must you go to OR for intussusception?
- risk of perforation with air contrast or barium enema
- peritonitis or free air or if unable to reduce
when reducing in OR, do not place traction on proximal limb of bowel, need to apply pressure to the distal limb. usually do not require resection unless associated with lead point.
what do you think about in adults presenting with intussusception?
patient most likely has malignant lead point (i.e. colon ca in cecum) -> OR for resection
- develop as a result of intrauterine vascular accidents
- symptoms: bilious emesis, distention; most do not pass meconium
- more common in jejunum; can be multiple
intestinal atresia
dx: intestinal atresia
get rectal biopsy to r/o hirschsprung’s before surgery
tx: intestinal atresia
resection
1 cause of duodenal obstruction in newborns (
duodenal atresia
- usually distal to ampulla of Vater and causes bilious vomiting, feeding intolerance
- associated with polyhydramnios in mother
- a/w cardiac, renal, and other gi anomalies
duodenal atresia
why check chromosomal studies in duodenal atresia?
20% of these patients have Down’s syndrome
abdominal xr: duodenal atresia
shows double-bubble sign
tx: duodenal atresia
resuscitation, duodenuoduodenostomy or duodenojejunostomy
most common type tracheoesophageal fistulas (TEF)
type c: most common type (85%)
define type c tracheoesophageal fistula
proximal esophageal atresia (blind pouch) and distal TE fistula
symptoms: newborn spits up feeds, has excessive drooling and respiratory symptoms with feeding; cannot place NGT in stomach
tracheoesophageal fistula: type C, type A
abdominal xr: TE fistula type c
distended, gas-filled stomach
define type a TE fistula
esophageal atresia and no fistula
mc types of TE fistula
- type c (MC - 85%)
- type a (2nd MC - 5%)
abdominal xr: type a te fistula
patients have gases abdomen
define VACTERL syndrome
vertebral, anorectal (imperforate anus), cardiac, TE fistula, radius/renal, and limb abnormalities
tx: te fistulas
right expleural thoracotomy for most perform primary repair, and place g-tube
- azygous vein needs to be divided
tx te fistula for infants that are premature,
replogle tube, treat respiratory symptoms; place g-tube, delay repair
te fistula: complications of repair
GERD, leak, empyema, stricutre, and fistula
what is survival related to in te fistula?
survival related to birth weight and associated anomalies
malrotation: symptoms
sudden onset of bilious vomiting (ladd’s bands cause duodenal obstruction, coming out from the right retroperitoneum)
what is volvulus associated with in malrotation?
volvulus associated with compromise of the SMA, leading to infarction of the intestine
what causes malrotation?
failure of normal counterclockwise rotation (270 degrees)
when does malrotation present?
75% in the first month, 90% present by 1 year of age
management of any child with bilious vomiting
needs an UGI to rule out malrotation
dx: malrotation
UGI - duodenum does not cross midline; duodenal-jejunal junction displaced to the right
tx: malrotation
resect lady’s bands, counterclockwise rotation (may require multiple turns), place ceum in LLQ (Cecopexy), place duodenum in RUQ and appendectomy
causes distal ileal obstruction, abdominal distention, bilious vomiting, and distended loops of bowel
meconium ileus
rate of meconium ileus in cystic fibrosis
occurs in 10% of children with cystic fibrosis
- need sweat chloride test or PCR for Cl channel defect
abdominal XR: meconium ileus
dilated loops of small bowel without air-fluid levels (because the meconium is too thick to separate from bowel wall); can have ground glass or soapsuds appearance
complications of meconium ileus requiring laparotomy
can cause perforation, leading to meconium pseudocyst or free perforation
tx: meconium ileus
gastrograffin enema (effective in 80%); can also make the diagnosis and potentially treat the patient - can also use n-acetylcysteine enema
surgical management (if necessary) in meconium ileus
if surgery required, manual decompression and create a vent for n-acetylcysteine antegrade enemas
clasically presents with bloody stools after 1st feeding in premature neonate
necrotizing enterocolitis
risk factors: necrotizing enterocolitis
prematurity, hypoxia, sepsis
symptoms: lethargy, respiratory decompensation, abdominal distention, vomiting, blood per rectum
necrotizing enterocolitis
abdominal xr: necrotizing enterocolitis
may show pneumatosis intestinalis, free air, or portal vein air
what do you need for monitoring in necrotizing enterocolitis?
need serial lateral decubitus films to look for perforation
initial tx: necrotizing enterocolitis
resuscitation, NPO, antibiotics, TPN, and orogastric tube
indications for operation: necrotizing enterocolitis
free air, peritonitis, clinical deterioration -> resect dead bowel and bring up ostomies
why do you need barium contrast enema before taking down ostomies in necrotizing enterocolitis?
to rule out distal obstruction from stenosis
mortality in necrotizing entercolitis
10%
indications for surgery in congenital vascular malformation
hemorrhage, ischemia, CHF, nonbleeding ulcers, functional impairment, or limb-length discrepancy
tx: congenital vascular malformation
embolization (may be sufficient on its own) and/or resection
male vs female: imperforate anus
more common in males
what do you need to check for in imperforate anus?
check for associated renal, cardiac, and vertebral (VACTERL) anomalies
tx: high (above levators) imperforate anus (meconium in urine or vagina - fistula to bladder/vagina/prostatic urethra)
colostomy, later anal reconstruction with posterior sagittal anoplasty
tx: low (below levators) imperforate anus - meconium to perineal skin
perform posterior sagittal anoplasty (pull anus down into sphincter mechanism); no colostomy needed
post op management of imperforate anus
need post op anal dilatation to avoid stricture; these patients are prone to constipation
intrauterine rupture of umbilical vein; does not have a peritoneal sac
- decreased congenital anomalies (only 10%) except malrotation
- to the right of midline, no peritoneal sac, stiff bowel from exposure to amniotic fluid
gastroschisis
tx: gastroschisis
initially place saline-soaked gauzes and resuscitate the patient; can lose a lot of fluid from the exposure bowel; TPN; NPO.
- repair when patient is stable
- at operation, try to place bowel back in abdomen, may need vicryl mesh silo
- primary closure at a later date if silo used
failure of embryonal development; has peritoneal sac with cord attached
- increased congenital anomalies (50%); midline defect
- sac can contain intra-abdominal structures other than bowel (liver, spleen, etc)
omphalocele
cantrell pentalogy
- cardiac defects
- pericardium defects (usually at diaphragmatic pericardium)
- sternal cleft or absence of lower sternum
- diaphragmatic septum transversum absence
- omphalocele
tx: omphalocele
initially place saline-soaked gauzes and resuscitate the patient; can lose a lot of fluid from the exposed bowel; TPN; NPO
- repair when patient is stable
- at operation, try to place bowel back in abdomen, may need vicryl mesh silo
- primary closure at a later date if silo used
what has a better prognosis: gastroschisis vs omphalocele
omphaloceleworse overall prognosis compared with gastroschisis secondary to congenital anomalies
is malrotation a risk in omphalocele and gastroschisis?
yes, in both
1 cause of colonic obstruction in infants; more common in males
hirschsprung’s disease
most common sign of hirschsprung’s
infants fail to pass meconium in 1st 24 hours
- can also present in older age groups as chronic constipation (age 2-3)
- get distention; occasionally get colitis
- can get explosive release of watery stool with anorectal exam
hirschsprung’s disease
dx: hirschsprung’s disease
rectal biopsy diagnostic (absence of ganglion cells in myenteric plexus
what causes hirschsprung’s disease?
is due to failure of the neural crest cells (ganglion cells) to progress in caudad direction
what do you need to resect in hirschsprung’s disease?
need to resect colon until proximal to where ganglion cells appear
tx: hirschsprung’s disease
may need to bring up a colostomy initially, eventually connect the colon to the anus (Soave or Duhamel procedure)
hirschsprung’s disease: may be rapidly progressive; manifested by abdominal distention and foul-smelling diarrhea
- lethargy and signs of sepsis may be present
hirschsprung’s colitis
tx: hirschsprung’s colitis
rectal irrigation to try and empty colon; may need emergency colectomy
- failure of closure of linea alba; most close by age 3, rare incarceration
- increased in african americans and premature infants
umbilical hernia
tx: umbilical hernia
surgery if not closed by age 5, incarceration, or if patient has a vp shunt
due to persistent processus vaginalis; 3% of infants, M>F
- right in 60%, left in 30%, bilateral in 10%
inguinal hernia
how can you differentiate hernia from hydrocele?
extension of the hernia into the internal ring differentiates hernia from hydrocele
tx: inguinal hernia
emergent operation if not able to reduce; otherwise, elective repair with high ligation
- consider exploring the contralateral side if left sided, female or child
when would you consider exploring the contralateral side in inguinal hernia?
if left sided, female or child
- most disappear by 1 year; noncommunicating will resolve; should transilluminate
hydrocele
tx: hydrocele
surgery at 1 year if not resolved or if thought to be communication (Waxing and waning size); resect hydrocele and ligate processus vaginalis
- most common in ileum; often on mesenteric border
- tx: resect cyst
cystic duplication
mcc of neonatal jaundice requiring surgery
biliary atresia
jaundice suggesting biliary atresia
progressive jaundice persisting > 2 weeks after birth suggests atresia
what can biliary atresia involve?
can involve either the extra hepatic or intrahepatic biliary tree or both
dx: biliary atresia
liver biopsy -> periportal fibrosis, bile plugging, eventual cirrhosis
- US and cholangiography can reveal atrocity biliary tree
pathway of disease in biliary atresia
get cholangitis, continued cirrhosis and eventual hepatic failure
what is the Kasai procedure?
for biliary atresia - hepaticoportojejunostomy
(1/3 get better, 1/3 go on to liver transplant, 1/3 die)
- involves resecting the extra hepatic bile duct segment
when do you need to perform the Kasai procedure?
before age 3 months, otherwise get irreversible liver damage
- increased AFP and beta-hcg
- neonates: sacrococcygeal
- adolescents: ovarian
- tx: excision
teratoma
- 90% benign at birth (almost all have exophytic component)
- great potential for malignancy
- AFP: good marker
sacrococcygeal teratomas
what is important about the 2 month mark in sacrococcygeal teratomas?
2 month mark is a huge transition:
- usually benign
- > 2 months -> usually malignant
tx: sacrococcygeal teratomas
coccygectomy and long-term follow up
when do you treat undescended testicles?
wait until 2 years old to treat
ca risk in undescended testicles
- higher risk of testicular ca in these children
- cancer risk stays the same even if testicles brought into scrotum
- get seminoma
management of bilateral undescended testicles
if undescended bilaterally, get chromosomal studies
management of undescended testicles that you cannot feel in the inguinal canal
you need to get an mri to confirm their presence
tx: undescended testicles
orchiopexy through inguinal incision; if not able to get testicles down -> close and wait 6 months and try again; if will not come down, perform division of spermatic vessels
- elliptical, fragmented tracheal rings instead of c-shaped
- wheezing, usually get better after 1-2 years
tracheomalacia
surgical indication: tracheomalacia
dying spell, failure to wean from ventilator, recurrent infections
surgery: tracheomalacia
aortopexy (aorta sutured to the back of the sternum, open up trachea)
most common cause of airway obstruction in infants
laryngomalacia
symptoms: intermittent respiratory distress and stridor exacerbation in the supine position
laryngomalacia
what causes laryngomalacia
caused by immature epiglottis cartilage with intermittent collapse of the epiglottis airway
when do most children outgrow laryngomalacia?
most children outgrow this by 12 months
is surgical tracheostomy the norm in laryngomalacia?
surgical tracheostomy is reserved for a very small number of patients
- obstruction of channel opening (nasal passage) by either bone or mucous membrane, usually unilateral
- symptoms: intermittent respiratory distress, poor suckling
choanal atresia
tx: choanal atresia
surgical correction
- most common tumor of the pediatric larynx
- frequently involutes after puberty
laryngeal papillomatosis
tx: laryngeal papillomatosis
can treat with endoscopic removal or laser but frequently comes back
what is though to cause laryngeal papillomatosis?
thought to be caused from hpv in the mother during passage through the birth canal
is cerebral palsy associated with GERD?
yes, many develop GERD
hx: bilious emesis
pe: abdominal distention
dx: plain abdominal film
intestinal atresia or stenosis
hx: failure to pass meconium
pe: acholic meconium, gastric distention
dx: contrast enema, plain abdominal film
duodenal atresia or stenosis
hx: bilious emesis
pe: trisomy 21
dx: upper gi contrast study
duodenal atresia or stenosis
hx: failure to pass meconium
pe: absent anus or visible fistula
dx: plain chest, abdominal film
imperforate anus
hx: bilious emesis (late)
pe: abdominal distention
dx: ultrasound kidneys, sacrum,r ectum
imperforate anus
hx: high-risk, premature infant / bilious emesis
pe: abdominal distention / hematochezia, guaiac-positive stool
dx: plain abdominal film
necrotizing enterocolitis
hx: cystic fibrosis (10%), bilious emesis
pe: acholic meconium, abdominal distention
dx: plain abdominal film, contrast enema
meconium ileus
hx: bilious emesis, term, healthy infant
pe: no abdominal distention
dx: plain abdominal film, upper gi contrast study
malrotation
hx: delayed passage of meconium, bilious emesis
pe: abdominal distention, trisomy 21
dx: plain abdominal film, contrast enema
hirschsprung’s disease