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1

embryology: lungs, esophagus, stomach, pancreas liver, gallbladder, bile duct, and duodenum proximal to ampulla

foregut

2

embryology: lungs, esophagus, stomach, pancreas liver, gallbladder, bile duct, and duodenum proximal to ampulla

foregut

3

embryology: duodenum distal to ampulla, small bowel, and large bowel to distal 1/3 of transverse colon

midgut

4

distal 1/3 of transverse colon to anal canal

hindgut

5

how does the midgut develop?

midgut rotates 270 degrees counterclockwise normally

6

low birth weight

7

premature

8

immunity at birth

IgA from mother's milk
IgG crosses the placenta

9

#1 cause of childhood death

trauma

10

trauma bolus

20cc/kg x 2, then give blood 10 cc/kg

11

best indicator of shock

tachycardia (neonate > 150 ; 120 ; rest > 100)

12

trauma: goal urine output

> 2-4cc/kg

13

GFR in children (

children (

14

what causes bone growth in children?

increased alkaline phosphatase in children compared with adults

15

what are the umbilical vessels?

2 arteries and 1 vein

16

maintenance IV fluids

4cc/kg/hr for first 10 kg
2cc/kg/hr for 2nd 10 kg
1cc/kg/hr for everything after that

17

four kinds of congenital cystic disease of the lung

- pulmonary sequestration
- congenital lobar overinflation
- congenital cystic adenoid malformation
- bronchiogenic cyst

18

- 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

19

pulmonary sequestration: more likely to have systemic venous drainage (azygous system)

extra-lobar pulmonary sequestration

20

pulmonary sequestration: more likely to have pulmonary vein drainage

intra-lobar pulmonary sequestration

21

tx: pulmonary sequestration (congenital cystic disease of the lung)

lobectomy

22

tx: pulmonary sequestration (congenital cystic disease of the lung)

lobectomy

23

embryology: duodenum distal to ampulla, small bowel, and large bowel to distal 1/3 of transverse colon

midgut

24

distal 1/3 of transverse colon to anal canal

hindgut

25

how does the midgut develop?

midgut rotates 270 degrees counterclockwise normally

26

low birth weight

27

tx: congenital cystic adenoid malformation

lobectomy

28

immunity at birth

IgA from mother's milk
IgG crosses the placenta

29

#1 cause of childhood death

trauma

30

trauma bolus

20cc/kg x 2, then give blood 10 cc/kg

31

tx: bronchiogenic cyst

resect cyst

32

most common mediastinal tumor in children; usually located posteriorly

neurogenic tumors (neurofibroma, neuroganglioma, neuroblastoma)

33

GFR in children (

children (

34

what causes bone growth in children?

increased alkaline phosphatase in children compared with adults

35

what are the umbilical vessels?

2 arteries and 1 vein

36

maintenance IV fluids

4cc/kg/hr for first 10 kg
2cc/kg/hr for 2nd 10 kg
1cc/kg/hr for everything after that

37

four kinds of congenital cystic disease of the lung

- pulmonary sequestration
- congenital lobar overinflation
- congenital cystic adenoid malformation
- bronchiogenic cyst

38

is thymoma rare in children?

yes, thymoma is rare in children

39

pulmonary sequestration: more likely to have systemic venous drainage (azygous system)

extra-lobar pulmonary sequestration

40

pulmonary sequestration: more likely to have pulmonary vein drainage

intra-lobar pulmonary sequestration

41

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

42

tx: pulmonary sequestration (congenital cystic disease of the lung)

lobectomy

43

- 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)

44

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

45

most commonly affected lobe in congenital lobar overinflation

LUL most commonly affected

46

tx: congenital lobar overinflation (emphysema)

lobectomy

47

- communicates with airway
- alveolar structure is poorly developed, although lung tissue is present
- symptoms: respiratory compromise or recurrent infection

congenital cystic adenoid malformation

48

tx: congenital cystic adenoid malformation

lobectomy

49

- 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

50

how do bronchiogenic cysts present?

usually present with a mediastinal mass filled with milky fluid

51

what problems can bronchiogenic cysts cause?

can compress adjacent structures or become infected; have malignant potential

52

tx: bronchiogenic cyst

resect cyst

53

most common mediastinal tumor in children; usually located posteriorly

neurogenic tumors (neurofibroma, neuroganglioma, neuroblastoma)

54

symptoms common to all mediastinal masses regardless of location

respiratory symptoms, dysphagia

55

anterior mediastinal masses

T cell lymphoma, teratoma, and other germ cell tumors (most common type of anterior mediastinal mad in children), thyroidCA

56

tx: cystic hygroma (lymphangioma)

resection

57

middle mediastinal masses

T cell lymphoma, teratoma, cyst (cardiogenic or bronchiogenic)

58

posterior mediastinal masses

t cell lymphoma, neuroblastoma, neurogenic tumor

59

is thymoma rare in children?

yes, thymoma is rare in children

60

caused by reflux of pancreatic enzymes into the biliary system in utero

choledochal cyst

61

why do choledochal cysts need to be resected in children?

risk of cholangiocarcinoma, pancreatitis, cholangitis, and obstructive jaundice

62

choledochal cyst: (85%): fusiform dilation of entire common bile duct, mildly dilated common hepatic duct, normal intrahepatic ducts

type 1 choledochal cysts

63

tx: choledochal cyst: type 1

resection, hepaticojejunostomy

64

choledochal cyst: (3%) a true diverticulum that hangs off the common bile duct

type 2 choledochal cyst

65

tx: type 2 choledochal cyst

resection off common bile duct; may be able to preserve common bile duct and avoid hepaticojejunostomy

66

choledochal cyst: (1%) dilation of distal intramural common bile duct; involves sphincter of Oddi

type 3 choledochal cyst

67

tx: type 3 choledochal cyst

resection, choledochojejunostomy

68

choledochal cyst: (10%) multiple cysts, both intrahepatic and extrahepatic

type 4 choledochal cyst

69

tx: type 4 choledochal cyst

resection; may need liver lobectomy; possible TXP

70

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

71

tx: type 5 choledochal cyst

resection; may need lobectomy; possible liver TXP

72

usual causes of lymphadenopathy

acute suppurative adenitis associated with URI or pharyngitis

73

management: fluctuant lymphadenopathy

FNA, culture and sensitivity, and antibiotics; may need incision and drainage if it fails to resolve

74

lymphadenopathy: chronic causes

cat scratch fever, atypical mycoplasma

75

management: asymptomatic lymphadenopathy

antibiotics for 10 days -> excisional biopsy if no improvement. this is lymphoma until proven otherwise

76

lymphadenopathy: usually found in lateral cervical regions in neck; gets infected; is usually lateral to the SCM

cystic hygroma (lymphangioma)

77

tx: cystic hygroma (lymphangioma)

resection

78

- 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

79

are there associated anomalies with diaphragmatic hernias and chest wall?

80% have associated anomalies (cardiac and neural tube defects mostly; malrotation)

80

cxr: diaphragmatic hernia

bowel in chest

81

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)

82

diaphragmatic hernia: most common, located posteriorly

bochdalek's hernia

83

diaphragmatic hernia: rare, located anteriorly

morgagni's hernia

84

management: pectus excavatum (sinks in)

sternal osteotomy, need strut; performed if causing respiratory symptoms or emotional stress

85

management: pectus carinatum (pigeon chest)

strut not necessary; repair for emotional stress

86

what can branchial cleft cyst cause?

leads to cysts, sinuses, and fistulas

87

branchial cleft cyst: angle of mandible; may connect with external auditory canal. often associated with facial nerve

1st branchial cleft cyst

88

branchial cleft cyst: on anterior border of mid-SCM muscle. goes through carotid bifurcation into tonsillar pillar

2nd branchial cleft cyst

89

branchial cleft cyst: lower neck, medial to or through the lower SCM

3rd branchial cleft cyst

90

most common branchial cleft cyst

2nd branchial cleft cyst

91

tx for all branchial cysts

resection

92

what causes thyroglossal duct cyst?

from the descent of the thyroid gland from the foramen cecum

93

- may be only thyroid tissue patient has
- presents as a midline cervical mass
- goes through hyoid bone

thyroglossal duct cyst

94

tx: thyroglossal duct cyst

excision of cyst, tract, and hyoid bone (At least the central portion)

95

- appears at birth or shortly after
- rapid growth during first 6-12 months of life, then begins to involute

hemangioma

96

tx: hemangioma

observation - most resolve by age 7-8

97

does wilms tumor metastasize?

frequent metastases to bone and lung

98

#1 solid abdominal malignancy in children

neuroblastoma

99

- usually presents as an asymptomatic mass
- can have secretory diarrhea, raccoon eyes (orbital metastases), HTN, and opsomyoclonus syndrome (unsteady gait)

neuroblastoma

100

where do most neuroblastoma occur?

most often on adrenals; can occur anywhere along the sympathetic chain

101

when do most neuroblastoma present?

most common in 1st 2 yeas of life. children

102

neuroblastoma is derived from what?

derived from neural crest cells

103

lab findings in neuroblastoma

most have increased catecholamines, VMA, HVA, and metanephrines (HTN)

104

how does neuroblastoma affect vasculature?

encases vasculature rather than invades

105

rare metastases of neuroblastoma

go to lung and bone

106

abdominal xr: neuroblastoma

may have stippled calcifications in the tumor

107

wilms tumor: stage 5

bilateral renal involvement

108

neuroblastoma: what is increased in all patients with metastases?

NSE

109

tx: neuroblastoma

resection (Adrenal gland and kidney take; 40% cured)

110

management of unresectable tumors in neuroblastoma

initially unresectable tumors may be resectable after doxorubicin-based chemo

111

neuroblastoma: stage 1

localized, complete excision

112

best prognosis in hepatoblastoma

fetal histology

113

neuroblastoma: stage 3

crosses midline +/ regional nodes

114

neuroblastoma: stage 4

distant metastases (nodes or solid organ)

115

neuroblastoma: stage 4-S

localized tumor with distant metastases

116

usually presents as asymptomatic mass; can have hematuria or HTN; 10% bilateral
- mean age at diagnosis: 3 years

wilms tumor (nephroblastoma)

117

what is prognosis of wilms tumor based on?

prognosis based on tumor grade (anaplastic and sarcomatous variations have worse prognosis)

118

does wilms tumor metastasize?

frequent metastases to bone and lung

119

management of pulmonary mets in wilms tumor?

can resect pulmonary mets if resectable

120

abdominal ct: wilms tumors

replacement of renal parenchyma and not displacement (differentiates it from neuroblastoma)

121

how do you differentiate neuroblastoma and nephroblastoma on ct?

replacement of parenchyma and not displacement in nephroblastoma

122

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

123

chemotherapy regimen for nephroblastoma (wilms tumor)

actinomycin and vincristine based chemo in all unless stage 1 and

124

wilms tumor: stage 1

limited to kidney, completed excised

125

wilms tumor: stage 2

beyond kidney but completely excised

126

upper GIB 1 year to adult

esophageal varices, esophagitis

127

wilms tumor: stage 4

hematogenous metastases

128

wilms tumor: stage 5

bilateral renal involvement

129

most common malignant liver tumor in children; increased AFP in 90%

hepatoblastoma

130

- fractures, precocious puberty (from beta-hcg release)
- better prognosis than hepatocellular CA
- can be pedunculated; vascular invasion common

hepatoblastoma

131

tx: hepatoblastoma

resection optimal; otherwise doxorubicin and cisplatin based chemotherapy -> may downstage tumors and make them resectable

132

what is survival based on in hepatoblastoma?

survival is primarily related to resectability

133

best prognosis in hepatoblastoma

fetal histology

134

#1 children's malignancy overall

leukemia (ALL)

135

#1 solid tumor class

CNS tumors

136

#1 general surgery tumor

neuroblastoma

137

#1 general surgery tumor in child

neuroblastoma

138

#1 general surgery tumor in children > 2 years

wilms tumor

139

#1 cause of duodenal obstruction in newborns (

duodenal atresia

140

#1 cause of duodenal obstruction after newborn period (>1 week) and overall

malrotation

141

#1 cause of colon obstruction

hirschsprung's disease

142

#1 liver tumor in children

hepatoblastoma; 2/3 of liver tumors in children are malignant

143

#1 lung tumor in children

carcinoid

144

#1 cause painful lower GIB

benign anorectal lesions (fissures, etc)

145

#1 cause painless lower GIB

meckel's diverticulum

146

upper GIB 0-1 year

gastritis, esophagitis

147

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

148

found on anti mesenteric border of small bowel
- embryology: persistent vitelline duct
- #1 cause of painless lower GIB in children

meckel's diverticulum

149

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)

150

dx: meckel's diverticulum

can get meckel's diverticulum scan with pertechnetate if suspicious of meckel's diverticulum and having trouble locating

151

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

152

3-12 weeks, firstborn males
projectile vomiting
can feel oliver mass in stomach

pyloric stenosis

153

acid base in pyloric stenosis

hypochloremic, hypokalemia, metabolic alkalosis

154

ultrasound in pyloric stenosis

pylorus >/ 4mm thick
>/ 14 mm long

155

tx: duodenal atresia

resuscitation, duodenuoduodenostomy or duodenojejunostomy

156

why avoid fluid resuscitation with K containing fluids in children with severe dehydration?

hyperkalemia can develop quickly

157

why avoid non salt containing solutions in infants?

hyponatremia can quickly develop

158

why do infants need a maintenance fluid with glucose?

because of their limited reserves for gluconeogenesis and vulnerability for hypogylcemia

159

tx: pyloric stenosis

pyloromyotomy (RUQ incision; proximal extent should be the circular muscles of the stomach)

160

- 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

161

intussusception: lead points in children

enlarged peyer's patches (#1), lymphoma, and meckel's diverticulum

162

rate of recurrence after reduction in intussusception

15%

163

tx: intussusception

reduce with air-contrast enema -80% successful; no surgery required if reduced

164

intussusception: max pressure with air-contrast enema

120 mmHg

165

intussusception: max column height with barium enema

1 meter (3 feet)

166

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

167

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.

168

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

169

- 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

170

dx: intestinal atresia

get rectal biopsy to r/o hirschsprung's before surgery

171

tx: intestinal atresia

resection

172

#1 cause of duodenal obstruction in newborns (

duodenal atresia

173

- 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

174

why check chromosomal studies in duodenal atresia?

20% of these patients have Down's syndrome

175

abdominal xr: duodenal atresia

shows double-bubble sign

176

tx: duodenal atresia

resuscitation, duodenuoduodenostomy or duodenojejunostomy

177

most common type tracheoesophageal fistulas (TEF)

type c: most common type (85%)

178

define type c tracheoesophageal fistula

proximal esophageal atresia (blind pouch) and distal TE fistula

179

symptoms: newborn spits up feeds, has excessive drooling and respiratory symptoms with feeding; cannot place NGT in stomach

tracheoesophageal fistula: type C, type A

180

abdominal xr: TE fistula type c

distended, gas-filled stomach

181

define type a TE fistula

esophageal atresia and no fistula

182

mc types of TE fistula

- type c (MC - 85%)
- type a (2nd MC - 5%)

183

abdominal xr: type a te fistula

patients have gases abdomen

184

define VACTERL syndrome

vertebral, anorectal (imperforate anus), cardiac, TE fistula, radius/renal, and limb abnormalities

185

tx: te fistulas

right expleural thoracotomy for most perform primary repair, and place g-tube
- azygous vein needs to be divided

186

tx te fistula for infants that are premature,

replogle tube, treat respiratory symptoms; place g-tube, delay repair

187

te fistula: complications of repair

GERD, leak, empyema, stricutre, and fistula

188

what is survival related to in te fistula?

survival related to birth weight and associated anomalies

189

malrotation: symptoms

sudden onset of bilious vomiting (ladd's bands cause duodenal obstruction, coming out from the right retroperitoneum)

190

what is volvulus associated with in malrotation?

volvulus associated with compromise of the SMA, leading to infarction of the intestine

191

what causes malrotation?

failure of normal counterclockwise rotation (270 degrees)

192

when does malrotation present?

75% in the first month, 90% present by 1 year of age

193

management of any child with bilious vomiting

needs an UGI to rule out malrotation

194

dx: malrotation

UGI - duodenum does not cross midline; duodenal-jejunal junction displaced to the right

195

tx: malrotation

resect lady's bands, counterclockwise rotation (may require multiple turns), place ceum in LLQ (Cecopexy), place duodenum in RUQ and appendectomy

196

causes distal ileal obstruction, abdominal distention, bilious vomiting, and distended loops of bowel

meconium ileus

197

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

198

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

199

complications of meconium ileus requiring laparotomy

can cause perforation, leading to meconium pseudocyst or free perforation

200

tx: meconium ileus

gastrograffin enema (effective in 80%); can also make the diagnosis and potentially treat the patient
- can also use n-acetylcysteine enema

201

surgical management (if necessary) in meconium ileus

if surgery required, manual decompression and create a vent for n-acetylcysteine antegrade enemas

202

clasically presents with bloody stools after 1st feeding in premature neonate

necrotizing enterocolitis

203

risk factors: necrotizing enterocolitis

prematurity, hypoxia, sepsis

204

symptoms: lethargy, respiratory decompensation, abdominal distention, vomiting, blood per rectum

necrotizing enterocolitis

205

abdominal xr: necrotizing enterocolitis

may show pneumatosis intestinalis, free air, or portal vein air

206

what do you need for monitoring in necrotizing enterocolitis?

need serial lateral decubitus films to look for perforation

207

initial tx: necrotizing enterocolitis

resuscitation, NPO, antibiotics, TPN, and orogastric tube

208

indications for operation: necrotizing enterocolitis

free air, peritonitis, clinical deterioration -> resect dead bowel and bring up ostomies

209

why do you need barium contrast enema before taking down ostomies in necrotizing enterocolitis?

to rule out distal obstruction from stenosis

210

mortality in necrotizing entercolitis

10%

211

indications for surgery in congenital vascular malformation

hemorrhage, ischemia, CHF, nonbleeding ulcers, functional impairment, or limb-length discrepancy

212

tx: congenital vascular malformation

embolization (may be sufficient on its own) and/or resection

213

male vs female: imperforate anus

more common in males

214

what do you need to check for in imperforate anus?

check for associated renal, cardiac, and vertebral (VACTERL) anomalies

215

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

216

tx: low (below levators) imperforate anus - meconium to perineal skin

perform posterior sagittal anoplasty (pull anus down into sphincter mechanism); no colostomy needed

217

post op management of imperforate anus

need post op anal dilatation to avoid stricture; these patients are prone to constipation

218

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

219

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

220

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

221

cantrell pentalogy

- cardiac defects
- pericardium defects (usually at diaphragmatic pericardium)
- sternal cleft or absence of lower sternum
- diaphragmatic septum transversum absence
- omphalocele

222

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

223

what has a better prognosis: gastroschisis vs omphalocele

omphaloceleworse overall prognosis compared with gastroschisis secondary to congenital anomalies

224

is malrotation a risk in omphalocele and gastroschisis?

yes, in both

225

#1 cause of colonic obstruction in infants; more common in males

hirschsprung's disease

226

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)

227

- get distention; occasionally get colitis
- can get explosive release of watery stool with anorectal exam

hirschsprung's disease

228

dx: hirschsprung's disease

rectal biopsy diagnostic (absence of ganglion cells in myenteric plexus

229

what causes hirschsprung's disease?

is due to failure of the neural crest cells (ganglion cells) to progress in caudad direction

230

what do you need to resect in hirschsprung's disease?

need to resect colon until proximal to where ganglion cells appear

231

tx: hirschsprung's disease

may need to bring up a colostomy initially, eventually connect the colon to the anus (Soave or Duhamel procedure)

232

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

233

tx: hirschsprung's colitis

rectal irrigation to try and empty colon; may need emergency colectomy

234

- failure of closure of linea alba; most close by age 3, rare incarceration
- increased in african americans and premature infants

umbilical hernia

235

tx: umbilical hernia

surgery if not closed by age 5, incarceration, or if patient has a vp shunt

236

due to persistent processus vaginalis; 3% of infants, M>F
- right in 60%, left in 30%, bilateral in 10%

inguinal hernia

237

how can you differentiate hernia from hydrocele?

extension of the hernia into the internal ring differentiates hernia from hydrocele

238

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

239

when would you consider exploring the contralateral side in inguinal hernia?

if left sided, female or child

240

- most disappear by 1 year; noncommunicating will resolve; should transilluminate

hydrocele

241

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

242

- most common in ileum; often on mesenteric border
- tx: resect cyst

cystic duplication

243

mcc of neonatal jaundice requiring surgery

biliary atresia

244

jaundice suggesting biliary atresia

progressive jaundice persisting > 2 weeks after birth suggests atresia

245

what can biliary atresia involve?

can involve either the extra hepatic or intrahepatic biliary tree or both

246

dx: biliary atresia

liver biopsy -> periportal fibrosis, bile plugging, eventual cirrhosis
- US and cholangiography can reveal atrocity biliary tree

247

pathway of disease in biliary atresia

get cholangitis, continued cirrhosis and eventual hepatic failure

248

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

249

when do you need to perform the Kasai procedure?

before age 3 months, otherwise get irreversible liver damage

250

- increased AFP and beta-hcg
- neonates: sacrococcygeal
- adolescents: ovarian
- tx: excision

teratoma

251

- 90% benign at birth (almost all have exophytic component)
- great potential for malignancy
- AFP: good marker

sacrococcygeal teratomas

252

what is important about the 2 month mark in sacrococcygeal teratomas?

2 month mark is a huge transition:
- usually benign
- > 2 months -> usually malignant

253

tx: sacrococcygeal teratomas

coccygectomy and long-term follow up

254

when do you treat undescended testicles?

wait until 2 years old to treat

255

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

256

management of bilateral undescended testicles

if undescended bilaterally, get chromosomal studies

257

management of undescended testicles that you cannot feel in the inguinal canal

you need to get an mri to confirm their presence

258

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

259

- elliptical, fragmented tracheal rings instead of c-shaped
- wheezing, usually get better after 1-2 years

tracheomalacia

260

surgical indication: tracheomalacia

dying spell, failure to wean from ventilator, recurrent infections

261

surgery: tracheomalacia

aortopexy (aorta sutured to the back of the sternum, open up trachea)

262

most common cause of airway obstruction in infants

laryngomalacia

263

symptoms: intermittent respiratory distress and stridor exacerbation in the supine position

laryngomalacia

264

what causes laryngomalacia

caused by immature epiglottis cartilage with intermittent collapse of the epiglottis airway

265

when do most children outgrow laryngomalacia?

most children outgrow this by 12 months

266

is surgical tracheostomy the norm in laryngomalacia?

surgical tracheostomy is reserved for a very small number of patients

267

- obstruction of channel opening (nasal passage) by either bone or mucous membrane, usually unilateral
- symptoms: intermittent respiratory distress, poor suckling

choanal atresia

268

tx: choanal atresia

surgical correction

269

- most common tumor of the pediatric larynx
- frequently involutes after puberty

laryngeal papillomatosis

270

tx: laryngeal papillomatosis

can treat with endoscopic removal or laser but frequently comes back

271

what is though to cause laryngeal papillomatosis?

thought to be caused from hpv in the mother during passage through the birth canal

272

is cerebral palsy associated with GERD?

yes, many develop GERD

273

hx: bilious emesis
pe: abdominal distention
dx: plain abdominal film

intestinal atresia or stenosis

274

hx: failure to pass meconium
pe: acholic meconium, gastric distention
dx: contrast enema, plain abdominal film

duodenal atresia or stenosis

275

hx: bilious emesis
pe: trisomy 21
dx: upper gi contrast study

duodenal atresia or stenosis

276

hx: failure to pass meconium
pe: absent anus or visible fistula
dx: plain chest, abdominal film

imperforate anus

277

hx: bilious emesis (late)
pe: abdominal distention
dx: ultrasound kidneys, sacrum,r ectum

imperforate anus

278

hx: high-risk, premature infant / bilious emesis
pe: abdominal distention / hematochezia, guaiac-positive stool
dx: plain abdominal film

necrotizing enterocolitis

279

hx: cystic fibrosis (10%), bilious emesis
pe: acholic meconium, abdominal distention
dx: plain abdominal film, contrast enema

meconium ileus

280

hx: bilious emesis, term, healthy infant
pe: no abdominal distention
dx: plain abdominal film, upper gi contrast study

malrotation

281

hx: delayed passage of meconium, bilious emesis
pe: abdominal distention, trisomy 21
dx: plain abdominal film, contrast enema

hirschsprung's disease