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Flashcards in Pediatric Surgery Deck (460):
1

What is the motto of pediatric surgery?

Children are not little adults

2

What is a simple way to distract a pediatric patient when examining the abdomen for tenderness?

Listen to the abdomen with the stethoscope and then push down on the abdomen with the stethoscope to check for tenderness

3

What is the estimated blood volume of infants and children?

8% of body weight or 80 cc/kg

4

What is the maintenance IV fluid for children?

D5 1/4 NS + 20 mEq KCl

5

Why 1/4 NS?

Children (especially those younger than 4 years) cannot concentrate their urine and cannot clear excess sodium

6

How are maintenance fluid rates calculated in children?

4, 2, 1 per hour:
4 cc/kg for the first 10 kg of body weight
2 cc/kg for the second 10 kg of body weight
1 cc/kg for every kg over the first 20 kg

7

What is the minimal urine output for children?

From 1-2 mL/kg/hr

8

What is the best way to present urine output measurements on rounds?

Urine output total per shift, then cc/kg/hr

9

What is the major difference between adult and pediatric nutritional needs?

Premature infants/infants/children need more calories and protein/kg/day

10

What are the caloric requirements for premature infants?

80 kcal/kg/day (and then go up)

11

What are the caloric requirements for children younger than 1 year?

100 kcal/kg/day

12

What are the caloric requirements for children ages 1-7?

85 kcal/kg/day

13

What are the caloric requirements for children ages 7-12?

70 kcal/kg/day

14

What are the caloric requirements for children ages 12-18?

40 kcal/kg/day

15

What are the protein requirements for children younger than 1 year?

3 g/kg/day

16

What are the protein requirements for children ages 1-7?

2 g/kg/day

17

What are the protein requirements for children ages 7-12?

2 g/kg/day

18

What are the protein requirements for children ages 12-18?

1.5 g/kg/day

19

How many calories are in breast milk?

20 kcal/30 cc

20

What is the blood volume per kg for a newborn infant?

85 cc/kg

21

What is the blood volume per kg for an infant 1-3 months?

75 cc/kg

22

What is the blood volume per kg for a child?

70 cc/kg

23

What is the number of umbilical veins?

1 (usually)

24

What is the number of umbilical arteries?

2

25

Which umbilical vessel carries oxygenated blood?

Umbilical vein

26

The oxygenated blood travels through the liver to the IVC through which structure?

Ductus venosus

27

Oxygenated blood passes from the right atrium to the left atrium through which structure?

Foramen ovale

28

Unsaturated blood goes from the right ventricle to the descending aorta through which structure?

Ductus arteriosus

29

What does the ductus venosus become?

Ligamentum venosum

30

What does the umbilical vein become?

Ligamentum teres

31

What does the umbilical artery become?

Medial umbilical ligament

32

What does the ductus arteriosus become?

Ligamentum arteriosum

33

What does the urachus become?

Median umbilical ligament

34

What does the tongue remnant of the thyroid's descent become?

Foramen cecum

35

What does the persistent remnant of the vitelline duct become?

Meckel's diverticulum

36

What is ECMO?

ExtraCorporeal Membrane Oxygenation:
Chronic cardiopulmonary bypass for complete respiratory support.

37

What are the types of ECMO?

Venovenous: blood from vein gets oxygenated, then returned to venous system.
Venoarterial: blood from vein (IJ) gets oxygenated, then returned to artery (carotid)

38

What are the indications for ECMO?

Severe hypoxia, usually from congenital diaphragmatic hernia, meconium aspiration, persistent pulmonary hypertension, sepsis

39

What are the contraindications for ECMO?

Weight

40

What is the major differential diagnosis of a pediatric neck mass?

Thyroglossal duct cyst (midline), branchial cleft cyst (lateral), LAD, abscess, cystic hygroma, hemangioma, teratoma/dermoid cyst, thyroid nodule, lymphoma/leukemia, parathyroid tumor, neuroblastoma, histiocytosis X, rhabdomyosarcoma, salivary gland tumor, neurofibroma

41

What is a thyroglossal duct cyst?

Remnant of the diverticulum formed by migration of thyroid tissue.
Normal development involves migration of thyroid tissue from the foramen cecum at the base of the tongue through the hyoid bone to its final position around the tracheal cartilage.

42

What is the average age of diagnosis for thyroglossal duct cyst?

5 years

43

How is the diagnosis of thyroglossal duct cyst made?

U/S

44

What are the complications of a thyroglossal duct cyst?

Enlargement; infection; fistula formation between oropharynx or salivary glands

45

What is the anatomic location of a thyroglossal duct cyst?

Midline

46

What is the treatment for a thyroglossal duct cyst?

Antibiotics if infection is present, then Sistrunk procedure (excision, which must include the mid-portion of the hyoid bone and entire tract to foramen cecum)

47

What is a branchial cleft cyst?

Remnant of the primitive branchial clefts in which epithelium forms a sinus tract between the pharynx (2nd cleft), or the external auditory canal (1st cleft), and the skin of the anterior neck.
If the sinus ends blindly, a cyst may form.

48

What is the common presentation of branchial cleft cyst

Infection because of communication between pharynx and external ear canal

49

What is the anatomic position of a branchial cleft cyst?

2nd cleft anomaly: lateral to midline along anterior border of the SCM, anywhere from angle of jaw to clavicle.
1st cleft anomaly: less common than 2nd cleft, tend to be located higher under the mandible.

50

What is the most common cleft remnant?

2nd

51

What is the treatment for a branchial cleft cyst?

Antibiotics if infection is present, then surgical excision of cyst and tract once inflammation is resolved

52

What is the major anatomic difference between thyroglossal cyst and branchial cleft cyst?

Thyroglossal: midline
Branchial cleft: lateral

53

What is stridor?

Harsh, high-pitched sound heard on breathing caused by obstruction of the trachea or larynx

54

What are the signs and symptoms of stridor?

Dyspnea, cyanosis, difficulty with feeding

55

What is the differential diagnosis for stridor?

Laryngomalacia, tracheobronchomalacia, vascular rings and slings

56

What is laryngomalacia?

Results from inadequate development of supporting laryngeal structures.
Usually self-limited and treatment is expectant unless respiratory compromise is present.

57

What are vascular rings?

Abnormal development or placement of thoracic large vessels resulting in obstruction of trachea/bronchus

58

What are the symptoms of vascular rings?

Stridor, dyspnea on exertion, dysphagia

59

How is the diagnosis of vascular rings made?

Barium swallow (revealing typical configuration of esophageal compression); Echo/arteriogram

60

What is the treatment of vascular rings?

Surgical division of the ring, if the patient is symptomatic

61

What is cystic hygroma?

Congenital abnormality of lymph sac resulting in lymphangioma

62

What is the anatomic location of cystic hygroma?

Occurs in sites of primitive lymphatic lakes and can occur virtually anywhere in the body, most commonly in the floor of the mouth, under the jaw, or in the neck, axilla, or thorax

63

What is the treatment for cystic hygroma?

Early total surgical removal because they tend to enlarge.
Sclerosis may be needed if the lesion is unresectable.

64

What are the possible complications of cystic hygroma?

Enlargement in critical regions, such as the floor of the mouth or paratracheal region, may cause airway obstruction.
Tend to insinuate onto major structures (although not malignant) making excision difficult.

65

Which bronchus do foreign bodies go into more commonly?

4 years: Right (develops into straight shot, less of an angle)

66

What is the most commonly aspirated object?

Peanut

67

What is the associated risk with peanut aspirations?

Lipoid pneumonia

68

How can a foreign body result in air trapping and hyperinflation?

By forming a ball valve (i.e. air in, no air out) as seen on CXR as a hyper inflated lung on expiratory film

69

How can you tell on AP CXR if a coin is in the esophagus or the trachea?

Coin in esophagus results in the coin lying en face with face of the object viewed as a round object because of compression by anterior and posterior structures.
Coin in trachea is viewed as a side projection due to the U-shaped cartilage with membrane posteriorly.

70

What is the treatment of tracheal or esophageal foreign bodies?

Remove foreign body with rigid bronchoscope or rigid esophagoscope

71

What is the differential diagnosis of a lung mass?

Bronchial adenoma (carcinoid is most common); pulmonary sequestration; pulmonary blastoma; rhabdomyosarcoma; chondroma; hamartoma; leiomyoma; mucus gland adenoma; metastasis

72

What is the differential diagnosis of mediastinal tumor or mass?

1. Neurogenic tumor (ganglioneuromas, neurofibromas)
2. Teratoma
3. Lymphoma
4. Thymoma
Rare: pheochromocytoma, hemangioma, rhabdomyosarcoma, osteochondroma

73

What heart abnormality is associated with pectus abnormality?

Mitral valve prolapse (many patients receive preoperative echocardiogram)

74

What is pectus excavatum?

Chest wall deformity with sternum caving inward

75

What is the cause of pectus excavatum?

Abnormal, unequal overgrowth of rib cartilage

76

What are the signs and symptoms of pectus excavatum?

Often asymptomatic.
Mental distress, dyspnea on exertion, chest pain.

77

What is the treatment for pectus excavatum?

Open perichondrium, remove abnormal cartilage, place substernal strut.
New cartilage grows back in the perichondrium in normal position.
Remove strut in 6 months.

78

What is the NUSS procedure?

Placement of metal strut to elevate sternum without removing cartilage

79

What is pectus carinatum?

Chest wall deformity with sternum outward.
Much less common that pectus excavatum.

80

What is the cause of pectus carinatum?

Abnormal, unequal overgrowth of cartilage

81

What is the treatment for pectus carinatum?

Open perichondrium and remove abnormal cartilage, place substernal strut.
New cartilage grows into normal position.
Remove strut in 6 months.

82

What is esophageal atresia?

Blind-ending esophagus from atresia

83

What are the signs of esophageal atresia without TEF?

Excessive oral secretions and inability to keep food down

84

How is the diagnosis of esophageal atresia without TEF made?

Inability to pass NG tube.
Plain XR shows tube coiled in upper esophagus and no gas in abdomen.

85

What is the primary treatment of esophageal atresia without TEF?

Suction blind pouch, IVFs, (gastrostomy to drain stomach if prolonged preoperative esophageal stretching is planned)

86

What is the definitive treatment of esophageal atresia without TEF?

Surgical with primary anastomosis, often with preoperative stretching of the blind pouch.
Other options include: colonic or jejunal interposition graft or gastric tube formation if esophageal gap is long.

87

What is esophageal atresia with TEF?

Esophageal atresia occurring with a fistula to the trachea

88

What is the incidence of esophageal atresia with TEF?

1:1500-3000

89

What is a type A esophageal atresia/fistula?

Esophageal atresia without TEF (8%)

90

What is a type B esophageal atresia/fistula?

Proximal esophageal atresia with proximal TEF (1%)

91

What is a type C esophageal atresia/fistula?

Proximal esophageal atresia with distal TEF (85%)

92

What is a type D esophageal atresia/fistula?

Proximal esophageal atresia with both proximal and distal TEF (2%)

93

What is a type E esophageal atresia/fistula?

TEF without esophageal atresia (4%)

94

What are the symptoms of esophageal atresia/TEF?

Excessive secretions cause by an accumulation of saliva (may not occur with type E)

95

What are the signs of esophageal atresia/TEF?

Obvious respiratory compromise, aspiration pneumonia, postprandial regurgitation, gastric distention as air enters the stomach directly from the trachea

96

How is the diagnosis of esophageal atresia/TEF made?

Failure to pass NG tube (although this will not be seen with type E); Plain film demonstrates tube coiled in the upper esophagus; Pouchogram (contrast in esophageal pouch); gas on AXR with TEF

97

What is the initial treatment of esophageal atresia/TEF?

1. Suction blind pouch (NPO/TPN)
2. Upright position of child
3. Prophylactic antibiotics (amp/gent)

98

What is the definitive treatment for esophageal atresia/TEF?

Surgical correction via a thoracotomy, usually through the right chest with division of fistula and end-to-end esophageal anastomosis, if possible

99

What can be done to lengthen the proximal esophageal pouch in esophageal atresia?

Delayed repair; with or without G tube and daily stretching of proximal pouch

100

Which type of esophageal atresia/TEF should be fixed via a right neck incision?

Type E is high in the thorax

101

What is the workup of a patient with TEF?

To evaluate the TEF and associated anomalies:
CXR, AXR, U/S of kidneys, cardiac echo

102

What are associated anomalies with TEF?

VACTERL cluster:
Vertebral and Vascular, Anorectal, Cardiac, TE fistula, Esophageal atresia, Radial limb and Renal, Lumbar and Limb

103

What is the significance of a gasless abdomen on AXR when evaluating for esophageal atresia/TEF?

No air to the stomach and, thus, no TEF

104

What is a congenital diaphragmatic hernia?

Failure of complete formation of the diaphragm, leading to a defect through which abdominal organs are herniated

105

What is the incidence of congenital diaphragmatic hernia?

1:2100 live births

106

What are the types of congenital diaphragmatic hernia?

Bochdale: posterolateral with L > R
Morgagni: anterior parasternal

107

What are the signs of congenital diaphragmatic hernia?

Respiratory distress, dyspnea, tachypnea, retractions, cyanosis, bowel sounds in the chest, maximal hearts sounds on right (rare), ipsilateral chest dullness to percussion

108

What are the effects on the lungs with congenital diaphragmatic hernia?

1. Pulmonary hypoplasia
2. Pulmonary hypertension

109

What inhaled agent is often used with congenital diaphragmatic hernia?

Inhaled nitric oxide (pulmonary vasodilator), which decreases the shunt and decreases pulmonary hypertension

110

What is the treatment for congenital diaphragmatic hernia?

NG tube, ET tube, stabilization.
If stable: surgical repair.
If unstable: nitric oxide +/- ECMO then to OR when feasible.

111

What is pulmonary sequestration?

Abnormal benign lung tissue with separate blood supply that does not communicate with the normal tracheobronchial airway

112

What is interlobar pulmonary sequestration?

Sequestration in the normal lung tissue covered by normal visceral pleura

113

What is extralobar pulmonary sequestration?

Sequestration not in the normal lung covered by its own pleura

114

What are the signs and symptoms of pulmonary sequestration?

Asymptomatic, recurrent pneumonia

115

How is the diagnosis of pulmonary sequestration made?

CXR, chest CT, A-gram, U/S with Doppler flow to ascertain blood supply

116

What is the treatment for interlobar pulmonary sequestration?

Lobectomy

117

What is the treatment for extralobar pulmonary sequestration?

Surgical resection

118

What is the major risk during operation for pulmonary sequestration?

Anomalous blood supply from below the diaphragm (can be cut and retracted into the abdomen and result in exsanguination).
Always document blood supply by A-gram or U/S with Doppler flow.

119

What is the differential diagnosis of pediatric upper GI bleeding?

Gastritis, esophagitis, gastric ulcer, duodenal ulcer, esophageal varices, foreign body, epistaxis, coagulopathy, vascular malformation, duplication cyst

120

What is the differential diagnosis of pediatric lower GI bleeding?

Upper GI bleeding, anal fissures, NEC (premature infants), midgut volvulus (

121

What is the differential diagnosis of neonatal bowel obstruction?

Malrotation with volvulus, intestinal atresia, duodenal web, annular pancreas, imperforate anus, Hirschsprung's disease, NEC, intussusception (rare), Meckel's diverticulum, incarcerated hernia, meconium ileus, meconium plug, maternal narcotic abuse (ileus), maternal hypermagnesemia (ileus), sepsis (ileus)

122

What is the differential diagnosis of infant constipation?

Hirschsprung's disease, CF, anteriorly displaced anus, polyps

123

What is the most commonly performed procedure by US pediatric surgeons?

Indirect inguinal hernia

124

What is the most common inguinal hernia in children?

Indirect

125

What is an indirect inguinal hernia?

Hernia lateral to Hesselbach's triangle into the internal inguinal ring and down the inguinal canal

126

What is Hesselbach's triangle?

Triangle formed by:
1. Epigastric vessels
2. Inguinal ligament
3. Lateral border of the rectus sheath

127

What type of hernia goes through Hesselbach's triangle?

Direct hernia from a weak abdominal floor (rare in children)

128

What is the incidence of indirect inguinal hernias in all children?

3%

129

What is the incidence of indirect inguinal hernias in premature infants?

30%

130

What is the male:female ratio for indirect inguinal hernias?

6:1

131

What are the risk factors for an indirect inguinal hernia in children?

Male, ascites, VP shunt, prematurity, family history, meconium ileus, abdominal wall defect, hypospadias, epispadias, connective tissue disease, bladder exstrophy, undescended testicle, CF

132

What side is affected more commonly by indirect inguinal hernias?

Right (60%)

133

What percentage of indirect inguinal hernias are bilateral?

15%

134

What percentage of patients with indirect inguinal hernia have a positive family history?

10%

135

What are the signs and symptoms of inguinal hernia?

Groin bulge, scrotal mass, thickened cord, silk glove sign

136

What is the silk glove sign?

Hernia sac rolls under the finger like the finger in a silk glove

137

Why should an inguinal hernia be repaired?

Risk of incarcerated or strangulated bowel or ovary.
Will not go away on its own.

138

How is a pediatric inguinal hernia repaired?

High ligation of hernia sac.
No repair of the abdominal wall floor, which is a big difference between the procedure in children vs. adults.
(High refers to high position on the sac neck next to the peritoneal cavity.)

139

Which infants need overnight apnea monitoring?

Premature infants; infants younger than 3 months of age

140

What is the recurrence after high ligation of an indirect pediatric hernia?

1%

141

What are the steps in the repair of an indirect inguinal hernia from skin to skin?

1. Cut skin, then fat, then Scarpa's fascia, then external oblique fascia through the external inguinal ring.
2. Find hernia sac anteromedially and bluntly separate from the other cord structures.
3. Ligate sac high at the neck at the internal inguinal ring.
4. Resect sac and allow sac stump to retract into the peritoneal cavity.
5. Close external oblique, then Scarpa's fascia, then skin.

142

What is cryptorchidism?

Failure of the testicle to descend into the scrotum

143

What is a hydrocele?

Fluid-filled sac

144

What is a communicating hydrocele?

Hydrocele that communicates with the peritoneal cavity and thus fills and drains peritoneal fluid or gets bigger, then smaller

145

What is a noncommunicating hydrocele?

Hydrocele that does not communicate with the peritoneal cavity.
Stays about the same size.

146

Can a hernia be rule out if an inguinal mass transilluminates?

No (baby bowel is very thin and will often transilluminate)

147

From what abdominal muscle layer is the cremaster muscle derived?

Internal oblique muscle

148

From what abdominal muscle layer is the inguinal ligament derived?

External oblique muscle

149

What nerve travels with the spermatic cord?

Ilioinguinal nerve

150

What 5 structures are in the spermatic cord?

1. Cremasteric muscle fibers
2. Vas deferens
3. Testicular artery
4. Testicular pampiniform venous plexus
5. With or without hernia sac

151

What is the hernia sac made of?

Basically peritoneum or a patent processus vaginalis

152

What is the name of the fossa between the testicle and epididymis?

Fossa of Geraldi

153

What attaches the testicle to the scrotum?

Gubernaculum

154

How can the opposite side be assessed for a hernia intra-operatively?

Many surgeons operatively explore the opposite side when they repair the affected side.
Laparoscope is placed into the abdomen via the hernia sac and the opposite side internal inguinal ring is examined.

155

What is the remnant of the processus vaginalis around the testicles?

Tunica vaginalis

156

What is a LIttre's inguinal hernia?

Hernia with a Meckel's diverticulum in the hernia sac

157

What may a yellow/orange tissue that is not fat be on the spermatic cord/testicle?

Adrenal rest

158

What is the most common organ in an inguinal hernia sac in boys?

Small intestine

159

What is the most common organ in an inguinal hernia sac in girls?

Ovary/fallopian tube

160

What lies in the inguinal canal in girls instead of the vas deferens?

Round ligament

161

Where in the inguinal canal does the hernia sac lie in relation to the other structures?

Anteromedially

162

What is a cord lipoma?

Preperitoneal fat on the cord structures (pushed in by the hernia sac).
Not a real lipoma.
Should be removed surgically, if feasible.

163

Within the spermatic cord, do the vessels or the vas lie medially?

Vas is medial to the testicular vessels

164

What is a small out-pouching of testicular tissue off of the testicle?

Testicular appendage.
Should be removed with electrocautery.

165

What is the blue dot sign?

Blue dot on the scrotal skin from a twisted testicular appendage

166

How is a transected vas treated?

Repair with primary anastomosis

167

How do you treat a transected ilioinguinal nerve?

Should not be repaired, many surgeons ligate it to inhibit neuroma formation

168

What happens if you cut the ilioinguinal nerve?

Loss of sensation to the medial aspect of the inner thigh and scrotum/labia.
Loss of cremasteric reflex.

169

What is an umbilical hernia?

Fascial defect at the umbilical ring

170

What are the risk factors for umbilical hernia?

1. African-American infant
2. Premature infant

171

What are the indications for surgical repair of an umbilical hernia?

1. > 1.5 cm defect
2. Bowel incarceration
3. > 4 years

172

What is GERD?

GastroEsophageal Reflux Disease

173

What are the causes of GERD in children?

LES malfunction or malposition, hiatal hernia, gastric outlet obstruction, partial bowel obstruction, common in cerebral palsy

174

What are the signs and symptoms of GERD in children?

Spitting up, emesis, URTI, pneumonia, laryngospasm from aspiration of gastric contents into the tracheobronchial tree, FTT

175

How is the diagnosis of GERD made?

24-hour pH probe, bronchoscopy, UGI (manometry, EGD, U/S)

176

What cytologic aspirate finding on bronchoscopy can diagnose aspiration of gastric contents?

Lipid-laden macrophages (from phagocytosis of fat)

177

What is the medical treatment of GERD?

H2 blockers; small meals; elevation of head

178

What are the indications for surgery for GERD in children?

SAFE:
Stricture, Aspiration (pneumonia, asthma), FTT, Esophagitis

179

What is the surgical treatment for GERD?

Nissen 360 degree fundoplication, +/- G tube

180

What is congenital pyloric stenosis?

Hypertrophy of smooth muscle of pylorus resulting in obstruction of outflow

181

What are the associated risk factors for congenital pyloric stenosis?

Family history, firstborn males are affected most commonly, decreased incidence in African American population

182

What is the incidence of congenital pyloric stenosis?

1:750 births
M:F = 4:1

183

What is the average age at onset of congenital pyloric stenosis?

Usually from 2 weeks after birth to about 2 months

184

What are the symptoms of congenital pyloric stenosis?

Increasing frequency of regurgitation, leading to eventual non-bilious projectile vomiting

185

Why is the vomiting with congenital pyloric stenosis non-bilious?

Obstruction is proximal to the ampulla of Vater

186

What are the signs of congenital pyloric stenosis?

Abdominal mass or "olive" in epigastric region, hypokalemic hypochloremic metabolic alkalosis, icterus, visible gastric peristalsis, paradoxic aciduria, hematemesis

187

What is the differential diagnosis for congenital pyloric stenosis?

Pylorospasm, milk allergy, increased ICP, hiatal hernia, GERD, adrenal insufficiency, uremia, malrotation, duodenal atresia, annular pancreas, duodenal web

188

How is the diagnosis of congenital pyloric stenosis made?

Usually H&P.
U/S : demonstrates elongated (> 15 mm) pyloric channel and thickened muscle wall (> 3.5 mm)
If U/S is non diagnostic, then barium swallow (string or double-railroad sign)

189

What is the initial treatment for congenital pyloric stenosis?

Hydration and correction of alkalosis with D10 NS plus 20 mEq of KCl

190

What is the definitive treatment for congenital pyloric stenosis?

Surgical, via Fredet-Ramstedt pyloromyotomy (division of circular muscle fibers without entering the lumen/mucosa)

191

What are the postoperative complications of pyloromyotomy?

Unrecognized incision through the duodenal mucosa, bleeding, wound infection, aspiration pneumonia

192

What is the appropriate postoperative feeding for pyloromyotomy?

Start feeding with Pedialyte at 6-12 hours postoperatively.
Advance to full-strength formula over 24 hours.

193

Which vein crosses the pylorus?

Vein of Mayo

194

What is duodenal atresia?

Complete obstruction or stenosis of duodenum caused by an ischemic insult during development or failure of recanalization

195

What is the anatomic location of duodenal atresia?

85% are distal to the ampulla of Vater

196

What are the signs of duodenal atresia?

Bilious vomiting (if distal to the ampulla), epigastric distention

197

What is the differential diagnosis of duodenal atresia?

Malrotation with Ladd's bands, annular pancreas

198

How is the diagnosis of duodenal atresia made?

AXR: "double bubble", with one or more air bubble in the stomach and the other in the duodenum

199

What is the treatment for duodenal atresia?

Duodenoduodenostomy or duodenojejunostomy

200

What are the associated abnormalities with duodenal atresia?

50-70% have cardiac, renal, or other GI defects.
30% have trisomy 21.

201

What is meconium ileus?

Intestinal obstruction from solid meconium concretions

202

What is the incidence of meconium ileus?

15% of infants with CF

203

What percentage of patients with meconium ileus have CF?

95%

204

What are the signs and symptoms of meconium ileus?

Bilious vomiting, abdominal distention, failure to pass meconium, Neuhauser's sign, peritoneal calcifications

205

What is Neuhauser's sign?

Ground glass appearance in the RLQ on AXR from viscous meconium mixing with air

206

How is the diagnosis of meconium ileus made?

Family history of CF, AXR showing significant dilation of similar-sized bowel loops, but few if any air-fluid levels, barium enema may demonstrate "microcolon" and inspissated meconium pellets in the terminal ileum

207

What is the treatment for meconium ileus?

70% nonoperative clearance of meconium using gastrografin enema, +/- acetylcysteine, which is hypertonic and therefore draws fluid into lumen, separating meconium pellets from bowel wall

208

What is the surgical treatment for meconium ileus?

If enema is unsuccessful, then enterotomy with intra-operative catheter irrigation using acetylcysteine (Mucomyst).

209

What should you remove during all operative cases?

Appendix

210

What is the long-term medical treatment for meconium ileus?

Pancreatic enzyme replacement

211

What is cystic fibrosis?

Inherited disorder of epithelial Cl transport defect affecting sweat glands, airways, and GI tract (pancreas, intestine).
Diagnosed by sweat test: elevated levels of NaCl > 60 mEq/L) and genetic testing.

212

What is DIOS?

Distal Intestinal Obstruction Syndrome:
Intestinal obstruction in older patients with CF from inspissated luminal contents.

213

What is meconium peritonitis?

Sign of intrauterine bowel perforation.
Sterile meconium leads to an intense local inflammatory reaction with eventual formation of calcifications.

214

What are the signs of meconium peritonitis?

Calcifications on plain films

215

What is meconium plug syndrome?

Colonic obstruction from unknown factors that dehydrate meconium, forming a plug

216

What is another name for meconium plug syndrome?

Neonatal small left colon syndrome

217

What are the signs and symptoms of meconium plug syndrome?

Abdominal distention and failure to pass meconium within first 24 hours of life.
AXR demonstrates many loops of distended bowel and air-fluid levels.

218

What is the nonoperative treatment for meconium plug syndrome?

Contrast enema is both diagnostic and therapeutic.
It demonstrates "microcolon" to the point of dilated colon (usually in transverse colon) and reveals copious intraluminal material.

219

What is the major differential diagnosis for meconium plug syndrome?

Hirschsprung's disease

220

Is meconium plug syndrome highly associated with CF?

No

221

What are anorectal malformations?

Malformations of the distal GI tract in the general categories of anal atresia, imperforate anus, and rectal atresia

222

What is an imperforate anus?

Congenital absence of normal anus (complete absence or fistula)

223

What is a high imperforate anus?

Rectum patent to level above puborectalis sling

224

What is a low imperforate anus?

Rectum patent to below puborectalis sling

225

Which type of imperforate anus is more common in women?

Low

226

What are the associated anomalies with imperforate anus?

VACTERL:
Vertebral abnormalities, Anal abnormalities, Cardiac, TEF, Esophageal atresia, Radial and Renal abnormalities, Lumbar abnormalities

227

What are the signs and symptoms of imperforate anus?

No anus, fistula to anal skin or bladder, UTI, fistula to vagina or urethra, bowel obstruction, distended abdomen, hyperchloremic acidosis

228

How is the diagnosis of imperforate anus made?

Physical, classic Cross table invertogram plain XR to see level of rectal gas, perineal U/S

229

What is the treatment for low imperforate anus with anal fistula?

Dilatation of anal fistula and subsequent anoplasty

230

What is the treatment for high imperforate anus?

Diverting colostomy and mucous fistula.
Neoanus is usually made at 1 year.

231

What is Hirschsprung's disease also known as?

Aganglionic megacolon

232

What is Hirschsprung's disease?

Neurogenic form of intestinal obstruction in which obstruction results from inadequate relaxation and peristalsis.
Absence of normal ganglion cells of the rectum and colon.

233

What are the associated risk factors for Hirschsprung's disease?

Family history (5% chance of having second child with the affliction)

234

What is the male:female ratio for Hirschsprung's disease?

4:1

235

What is the anatomic location of Hirschsprung's disease?

Aganglionosis begins at the anorectal line and involves rectosigmoid in 80% of cases (10% to splenic flexure, 10% entire colon)

236

What are the signs and symptoms of Hirschsprung's disease?

Abdominal distention and bilious vomiting.
95% present with failure to pass meconium in the first 24 hours.
May also present later with constipation, diarrhea, and decreased growth.

237

What is the classic history of Hirschsprung's disease?

Failure to pass meconium in the first 24 hours of life

238

What is the differential diagnosis for Hirschsprung's disease?

Meconium plug syndrome, meconium ileus, sepsis with adynamic ileus, colonic neuronal dysplasia, hypothyroidism, maternal narcotic abuse, maternal hypermagnesemia (tocolysis)

239

What imaging studies should be ordered for Hirschsprung's disease?

AXR (dilated colon).
Unprepared barium enema (constricted aganglionic segment with dilated proximal segment, but this picture may not develop for 3-6 weeks).

240

What is needed for definitive diagnosis of Hirschsprung's disease?

Rectal biopsy (submucosal suction biopsy is adequate in 90% of cases; full-thickness biopsy should be performed to evaluate Auerbach's plexus)

241

What is the colonic transition zone?

In Hirschsprung's disease, transition from aganglionic small colon into the large dilated normal colon seen on barium enema

242

What is the initial treatment for Hirschsprung's disease?

In neonates, a colostomy proximal to the transition zone prior to correction, to allow for pelvic growth and dilated bowel to return to normal size

243

What is a leveling colostomy?

Colostomy performed for Hirschsprung's disease at the level of normally innervated ganglion cells as ascertained on frozen section intraoperatively

244

What is the Swenson procedure?

Primary anastomosis between the anal canal and healthy bowel

245

What is the Duhamel procedure?

Anterior, aganglionic region of the rectum is preserved and anastomosed to a posterior portion of healthy bowel.
A functional rectal pouch is thereby created.

246

What is the Soave procedure?

The proximal normal colon is brought through the aganglionic rectum, which has been stripped of its mucosa but is otherwise present (also called endorectal pull-through)

247

What is the new trend in surgery for Hirschsprung's disease?

No colostomy, remove aganglionic colon and perform pull-through anastomosis at the same time

248

What is the prognosis for Hirschsprung's disease?

Overall survival rate > 90%.
Postoperative symptoms improve with age.

249

What is malrotation and midgut volvulus?

Failure of the normal bowel rotation, with resultant abnormal intestinal attachments and anatomic positions

250

Where is the cecum with malrotation and midgut volvulus?

With malrotation, the cecum usually ends up in the RUQ

251

What are Ladd's bands?

Fibrous bands that extend from the abnormally placed cecum in the RUQ (from malrotation and midgut volvulus), often crossing over the duodenum and causing obstruction

252

What is the usual age at onset for malrotation and midgut volvulus?

33% are present by 1 week; 75% by 1 month; 90% by 1 year

253

What is the usual presentation of malrotation and midgut volvulus?

Sudden onset of bilious vomiting

254

Why is the vomiting bilious in malrotation and midgut volvulus?

Twist is distal to the ampulla of Vater

255

How is the diagnosis of malrotation and midgut volvulus made?

Upper GI contrast study (cutoff in duodenum).
Barium enema (abnormal position of cecum in the upper abdomen).

256

What are the possible complications of malrotation and midgut volvulus?

Midgut infarction, leading to death or necessitating massive enterectomy

257

What is the treatment for malrotation and midgut volvulus?

1. IV antibiotics and fluid resuscitation with LR.
2. Emergent laparotomy with Ladd's procedure.
3. Second-look laparotomy if bowel is severely ischemic in 24 hours to determine if remaining bowel is viable.

258

What is Ladd's procedure?

1. Counterclockwise reduction of midgut volvulus.
2. Splitting of Ladd's bands.
3. Division of peritoneal attachments to the cecum, ascending colon.
4. Appendectomy.

259

In what direction is midgut volvulus reduced: clockwise or counterclockwise?

Counterclockwise

260

Where is the cecum after midgut volvulus reduction?

LLQ

261

What is the cause of bilious vomiting in an infant until proven otherwise?

Malrotation with midgut volvulus

262

What is an omphalocele?

Defect of abdominal wall at umbilical ring.
Sac covers extruded viscera.

263

How is omphalocele diagnosed prenatally?

May be seen on fetal U/S after 13 weeks gestation, with elevated maternal AFP

264

What comprises the sac in an omphalocele?

Peritoneum and amnion

265

What organ is often found protruding from an omphalocele, but is almost never found with gastroschisis?

Liver

266

What is the incidence of omphalocele?

1:5000 births

267

How is the diagnosis of omphalocele made?

Prenatal U/S

268

What are the possible complications of omphalocele?

Malrotation of the gut, anomalies

269

What is the treatment for omphalocele?

1. NG tube for decompression
2. IV fluids
3. Prophylactic antibiotics
4. Surgical repair of the defect

270

What is the treatment of a small omphalocele (

Closure of abdominal wall

271

What is the treatment of a medium omphalocele (2-10 cm)?

Removal of outer membrane and placement of a silicone patch to form a silo, temporarily housing abdominal contents.
The silo is then slowly decreased in size over 4-7 days, as the abdomen accommodates the viscera.
Then the defect is closed.

272

What is the treatment of a giant omphalocele (> 10 cm)?

Skin flaps or treatment with Betadine spray, mercurochrome, or silver sulfadiazine (Silvadene) over defect.
This allows an eschar to form, which epithelializes over time, allowing opportunity for future repair months to years later.

273

What are the associated abnormalities with omphalocele?

50% of cases occur with abnormalities of the GI tract, cardiovascular system, GU tract, musculoskeletal system, CNS, and chromosomes

274

What is the pentalogy of Cantrell?

D COPS:
Diaphragmatic defect (hernia)
Cardiac abnormality
Omphalocele
Pericardium malformation/absence
Sternal cleft

275

What is gastroschisis?

Defect of abdominal wall.
Sac does not cover extruded viscera.

276

How is gastroschisis diagnosed prenatally?

Possible at fetal U/S after 13 weeks gestation, elevated maternal AFP

277

Where is the defect in gastroschisis?

Lateral to the umbilicus

278

On what side of the umbilicus is a gastroschisis defect most commonly found?

Right

279

What is the usual size of the defect in gastroschisis?

2-4 cm

280

What are the possible complications of gastroschisis?

Thick edematous peritoneum from exposure to amniotic fluid; malrotation of the gut; hypothermia; hypovolemia for 3rd-spacing; sepsis; metabolic acidosis from hypovolemia and poor perfusion; NEC; prolonged ileus

281

How is the diagnosis of gastroschisis made?

Prenatal U/S

282

What is the treatment for gastroschisis?

1. NG tube decompression, IV fluids (D10 LR), IV antibiotics.
2. Surgical reduction of viscera and abdominal closure (may require staged closure with silo).

283

What is a silo?

Silastic silo is a temporary housing for external abdominal contents.
Silo is slowly tightened over time.

284

What is the prognosis for gastroschisis?

> 90% survival rate

285

What are the associated anomalies with gastroschisis?

Relatively uncommon, except intestinal atresia (10-15%)

286

What are the major differences between gastroschisis and omphalocele?

No membrane coverings; uncommon associated abnormalities; lateral to umbilicus

287

What is appendicitis?

Obstruction of the appendiceal lumen (fecalith, lymphoid hyperplasia), producing a closed loop with resultant inflammation that can lead to necrosis and perforation

288

What is the most common reason for emergency surgery in children?

Appendicitis

289

What is the differential diagnosis for appendicitis in children and adolescents?

Intussusception, volvulus, Meckel's diverticulum, Crohn's disease, ovarian torsion, cyst, tumor, perforated ulcer, pancreatits, PID, ruptured ectopic pregnancy, mesenteric lymphadenitis

290

What is the role of U/A in appendicitis?

To evaluate for possible pyelonephritis or renal calculus, but mild hematuria and pyuria are common in appendicitis because of ureteral inflammation

291

What is the hamburger sign?

Ask patients with suspected appendicitis if they would like a hamburger or favorite food.
If they can eat, seriously question the diagnosis.

292

How long should antibiotics be administered for non-perforated appendicitis?

24 hours

293

How long should antibiotics be administered for perforated appendicitis?

Usually 5-7 days or until WBCs are normal and patient is afebrile

294

What is intussusception?

Obstruction caused by bowel telescoping into the lumen of adjacent distal bowel may result when peristalsis carries a lead-point downstream

295

What is the most common cause of small bowel obstruction in toddlers (

Intussusception

296

What is the usual age of presentation for intussusception?

60% from 4-12 months.
80% by 2 years.

297

What is the most common site for intussusception?

Terminal ileus involving ileocecal valve and extending into ascending colon

298

What is the most common cause of intussusception?

Hypertrophic Peyer's patches, which act as a lead point

299

What are the signs and symptoms of intussusception?

Alternating lethargy and irritability (colic), bilious vomiting, currant jelly stools, RLQ mass on AXR, empty RLQ on palpation

300

What is the intussuscipiens?

Recipient segment of bowel

301

What is the intussusceptum?

Leading point or bowel that enters the intussuscipiens

302

What is the treatment for intussusception?

Air or barium enema.
If unsuccessful, laparotomy and reduction by milking the ileum from the colon should be performed.

303

What are the causes of intussusception in older patients?

Meckel's diverticulum, polyps, and tumors

304

What is Meckel's diverticulum?

Remnant of the vitelline duct, which connects the yolk sac with the primitive midgut in the embryo

305

What is the usual location of Meckel's diverticulum?

Between 45 and 90 cm proximal to the ileocecal valve on the anti-mesenteric border of the bowel

306

What is the major differential diagnosis for Meckel's diverticulum?

Appendicitis

307

Is Meckel's diverticulum a true diverticulum?

Yes

308

What is the incidence of Meckel's diverticulum?

2%

309

What is the male:female ratio for Meckel's diverticulum?

2-3:1

310

What is the usual age at onset of symptoms for Meckel's diverticulum?

311

What are the possible complications of Meckel's diverticulum?

Intestinal hemorrhage (painless), intestinal obstruction, inflammation +/- perforation

312

What percentage of Meckel's diverticulum cases have heterotopic tissue?

> 50% (usually gastric mucosa, but duodenal, pancreatic, and colonic mucosa have been described)

313

What is the most common ectopic tissue in Meckel's diverticulum?

Gastric mucosa

314

Besides Meckel's diverticulum, what other pediatric disease entity can present with GI bleeding secondary to ectopic gastric mucosa?

Enteric duplications

315

What is the most common cause of lower GI bleeding in children?

Meckel's diverticulum with ectopic gastric mucosa

316

What is the rule of 2s for Meckel's diverticulum?

2% are asymptomatic
2 feet from ileocecal valve
2% of population

317

What is a Meckel's scan?

Scan for ectopic gastric mucosa in Meckel's diverticulum.
Uses technetium Tc 99m pertechnetate IV, which is preferentially taken up by gastric mucosa.

318

What is necrotizing enterocolitis?

Necrosis of intestinal mucosa, often with bleeding.
May progress to transmural intestinal necrosis, septic shock, death.

319

What are the predisposing conditions for NEC?

Prematurity and stress (shock, hypoxia, RDS, apneic episodes, sepsis, exchange transfusions, PDA, cyanotic heart disease, hyperosmolar feedings, polycythemia, indomethacin)

320

What is the pathophysiologic mechanism of NEC?

Probable splanchnic vasoconstriction with decreased perfusion, mucosal injury, and probable bacterial infection

321

What is the most common cause of emergent laparotomy in the neonate?

NEC

322

What are the signs and symptoms of NEC?

Abdominal distention, vomiting, heme positive or gross rectal bleeding, fever or hypothermia, jaundice, abdominal wall erythema (consistent with perforation and abscess formation)

323

What are the radiographic findings with NEC?

Fixed, dilated intestinal loops; pneumatosis intestinalis (air in the bowel wall); free air; portal vein air (sign of advanced disease)

324

What are the lab findings with NEC?

Low hematocrit, glucose, and platelets

325

What is the treatment for NEC?

1. Cessation of feedings
2. OG tube
3. IV fluids
4. IV antibiotics
5. Ventilator support, as needed

326

What are the surgical indications for NEC?

Free air in abdomen revealing perforation, and positive peritoneal tap revealing transmural bowel necrosis

327

What is an option for bowel perforation in

Placement of percutaneous drain (without laparotomy)

328

What are the indications for peritoneal tap for NEC?

Severe thrombocytopenia, distended abdomen, abdominal wall erythema, unexplained clinical downturn

329

What are the possible complications of NEC?

Bowel necrosis, gram-negative sepsis, DIC, wound infection, cholestasis, short bowel syndrome, strictures, SBO

330

What is the prognosis for NEC?

> 80% survival rate

331

What is physiologic jaundice?

Hyperbilirubinemia in the first 2 weeks of life from inadequate conjugation of bilirubin

332

What enzyme is responsible for conjugation of bilirubin?

Glucoronyl transferase

333

How is hyperbilirubinemia from physiologic jaundice treated?

UV light

334

What is Gilbert's syndrome?

Partial deficiency of glucoronyl transferase, leading to intermittent asymptomatic jaundice in the 2nd or 3rd decade

335

What is Crigler-Najjar syndrome?

Rare genetic absence of glucoronyl transferase activity, causing unconjugated hyperbilirubinemia, jaundice, and death from kernicterus (usually within the first year)

336

What is biliary atresia?

Obliteration of extrahepatic biliary tree

337

What is the incidence of biliary atresia?

1:16,000 births

338

What are the signs and symptoms of biliary atresia?

Persistent jaundice, hepatomegaly, splenomegaly, ascites, acholic stools, biliuria

339

What are the lab findings with biliary atresia?

Mixed jaundice is always present (i.e. both direct and indirect bilirubin increased), with an elevated serum alkaline phosphate level

340

What is the classic rule of 5s of indirect hyperbilirubinemia?

5 mg/dL = jaundice of head
10 mg/dL = jaundice of trunk
15 mg/dL = jaundice of leg/feet

341

What is the differential diagnosis for biliary atresia?

Neonatal hepatitis, biliary hypoplasia

342

How is the diagnosis of biliary atresia made?

1. U/S: Rule out choledochal cyst and to examine extrahepatic bile ducts and gallbladder.
2. HIDA scan: Shows no excretion into the GI tract (with phenobarbital preparation).
3. Operative cholangiogram and liver biopsy.

343

What is the treatment for biliary atresia?

Early laparotomy by 2 months of age with a modified form of the Kasai hepatoportoenterostomy

344

What is a Kasai procedure?

Anastomosis of the porta hepatis and the small bowel, allowing drainage of bile via many microscopic bile ducts in the fibrous structure of the porta hepatis

345

What is done if a Kasai procedure fails?

Revise or liver transplantation

346

What are the possible postoperative complications of a Kasai procedure?

Cholangitis (manifested as decreased bile secretion, fever, leukocytosis, recurrence of jaundice).
Progressive cirrhosis (manifested as portal hypertension with bleeding varices, ascites, hypoalbuminemia, hypothrombinemia, and fat-soluble vitamin deficiencies).

347

What are the associated abnormalities for biliary atresia?

Annular pancreas, duodenal atresia, malrotation, polysplenic syndrome, situs inversus, preduodenal portal vein

348

What is a choledochal cyst?

Cystic enlargement of bile ducts.
Most commonly arises in extrahepatic ducts, but can also arise in intrahepatic ducts.

349

What is the usual presentation of a choledochal cyst?

50% with intermittent jaundice, RUQ mass, abdominal pain

350

What are the possible complications of a choledochal cyst?

Cholelithiasis, cirrhosis, carcinoma, portal hypertension

351

What is a type I choledochal cyst?

Dilation of common hepatic and common bile duct, with cystic duct entering the cyst (90%)

352

What is a type II choledochal cyst?

Lateral saccular cystic dilation

353

What is a type III choledochal cyst?

Choledochocele represented by an intraduodenal cyst

354

What is a type IV choledochal cyst?

Multiple extrahepatic cysts, intrahepatic cysts, or both

355

What is a type V choledochal cyst?

Single or multiple intrahepatic cysts

356

How is the diagnosis of choledochal cyst made?

U/S

357

What is the treatment for a choledochal cyst?

Operative cholangiogram to clarify pathologic process and delineate the pancreatic duct, followed by complete resection of the cyst and a Roux-en-Y hepatojejunostomy

358

What conditions are patients with choledochal cysts at increased risk of developing?

Cholangiocarcinoma often arises in the cyst (treat by complete resection of cyst)

359

What is cholelithiasis?

Formation of gallstones

360

What are the common causes of cholelithiasis in children?

Cholesterol stones, pigmented stones (from hemolytic disorders)

361

What is the differential diagnosis of cholelithiasis in children?

Hereditary spherocytosis, thalassemia, pyruvate kinase deficiency, sickle-cell disease, cystic fibrosis, long-term TPN, idiopathic

362

What are the associated risk factors for cholelithiasis?

Use of OCPs, teenage, positive family history

363

What is the treatment for cholelithiasis?

Cholecystectomy

364

What is an annular pancreas?

Congenital pancreatic abnormality with complete encirclement of the duodenum by the pancreas

365

What are the symptoms of annular pancreas?

Duodenal obstruction

366

What is the treatment for annular pancreas?

Duodenoduodenostomy bypass of obstruction

367

What is the differential diagnosis of pediatric abdominal mass?

Wilms' tumor, neuroblastoma, hernia, intussusception, malrotation with volvulus, mesenteric cyst, duplication cyst, liver tumor (hepatoblastoma or hemangioma), rhabdomyosarcoma, teratoma

368

What is Wilms' tumor?

Embryonal tumor of renal origin

369

What is the incidence of Wilms' tumor?

Rare; 500 cases in US per year

370

What is the average age at diagnosis for Wilms' tumor?

1-5 years

371

What are the symptoms of Wilms' tumor?

Abdominal mass

372

What is the classic history for Wilms' tumor?

Found during bathing or dressing

373

What are the signs of Wilms' tumor?

Abdominal mass (most do not cross midline); hematuria; hypertension (compression of juxtaglomerular apparatus); signs of Beckwith-Wiedemann syndrome

374

What are the diagnostic radiologic tests for Wilms' tumor?

Abdominal and chest CT

375

What is stage I Wilms' tumor?

Limited to kidney and completely resected

376

What is stage II Wilms' tumor?

Extends beyond kidney, but completely resected.
Capsule invasion and perirenal tissues may be involved.

377

What is stage III Wilms' tumor?

Residual non-hematogenous tumor after resection

378

What is stage IV Wilms' tumor?

Hematogenous metastases (lung, distal lymph nodes, brain)

379

What is stage V Wilms' tumor?

Bilateral renal involvement

380

What are the best indicators of survival from Wilms' tumor?

Stage and histologic subtype of tumor

381

What is the treatment for Wilms' tumor?

Radical resection of affected kidney with evaluation for staging, followed by chemotherapy (low stages) and radiation (higher stages)

382

What is the neoadjuvant treatment for Wilms' tumor?

Large tumors may be shrunk with chemotherapy/XRT to allow for surgical resection

383

What are the associated abnormalities with Wilms' tumor?

Aniridia, hemihypertrophy, Beckwith-Wiedemann syndrome, neurofibromatosis, horseshoe kidney

384

What is Beckwith-Wiedemann syndrome?

1. Umbilical defect
2. Macroglossia
3. Gigantism
4. Visceromegaly

385

What is neuroblastoma?

Embryonal tumor of neural crest origin

386

What are the anatomic locations of neuroblastomas?

Adrenal medulla, para-aortic abdominal para-spinal ganglia, posterior mediastinum, neck, pelvis

387

With which types of tumor does a patient with Horner's syndrome present?

Neck, superior mediastinal tumors

388

What is the incidence of neuroblastoma?

1:7,000-10,000 births

389

What is the most common solid malignant tumor of infancy?

Neuroblastoma

390

What is the average age of diagnosis of neuroblastoma?

50% by 2 years
90% by 8 years

391

What are the symptoms of neuroblastoma?

Vary by tumor location: anemia, FTT, weight loss, poor nutritional status with advanced disease

392

What are the signs of neuroblastoma?

Asymptomatic abdominal mass, respiratory distress (mediastinal), Horner's syndrome (upper chest, neck), proptosis (orbital mets), subcutaneous tumor nodules, hypertension

393

What are the lab tests are performed for neuroblastoma?

24-hour urine (VMA, HVA, metanephrines), neuron-specific enolase, N-myc oncogene, DNA ploidy

394

What are the diagnostic radiologic tests for neuroblastoma?

CT, MRI, I-MIBG, somatostatin receptor scan

395

What is the classic AXR finding with neuroblastoma?

Calcifications

396

How do you assess bone marrow involvement with neuroblastoma?

Bone marrow aspirate

397

What is the difference in position of tumors in neuroblastoma vs. Wilms' tumor?

Neuroblastoma may cross the midline, but Wilms' tumors do so only rarely

398

What is stage I neuroblastoma?

Confined to organ of origin

399

What is stage II neuroblastoma?

Tumor extends beyond organ of origin but not across the midline

400

What is stage III neuroblastoma?

Tumor extends across the midline

401

What is stage IV neuroblastoma?

Metastatic disease

402

What is stage IVS neuroblastoma?

Infants: localized primary tumor does not cross the midline, but remote disease is confined to the liver, subcutaneous/skin, and bone marrow

403

What is the treatment for stage I neuroblastoma?

Surgical resection

404

What is the treatment for stage II neuroblastoma?

Resection and chemotherapy +/- XRT

405

What is the treatment for stage III neuroblastoma?

Resection and chemotherapy, XRT

406

What is the treatment for stage IV neuroblastoma?

Chemotherapy, XRT followed by resection

407

What is the treatment for stage IVS neuroblastoma?

In the infant with small tumor and asymptomatic: observe, as many will regress spontaneously

408

What is the survival rate of neuroblastoma?

I: 90%
II: 80%
III: 40%
IV: 15%
IVS: > 80%

409

What are the lab prognosticators for neuroblastoma?

Aneuploidy is favorable; the fewer N-myc oncogene copies, the better

410

Which oncogene is associated with neuroblastoma?

N-myc

411

What is the most common sarcoma in children?

Rhabdomyosarcoma

412

What is the age distribution for rhabdomyosarcoma?

Bimodal:
1. 2-5 years
2. 15-19 years

413

What are the most common sites for rhabdomyosarcoma?

1. Head and neck
2. GU tract
3. Extremities

414

What are the signs and symptoms of rhabdomyosarcoma?

Mass

415

How is the diagnosis of rhabdomyosarcoma made?

Tissue biopsy, CT, MRI, bone marrow

416

What is the treatment for resectable rhabdomyosarcoma?

Surgical excision +/- chemotherapy and XRT

417

What is the treatment for unresectable rhabdomyosarcoma?

Neoadjuvant chemotherapy, XRT, then surgical excision

418

What is hepatoblastoma?

Malignant tumor of the liver (derived from embryonic liver cells)

419

What is the average age at diagnosis for hepatoblastoma?

420

What is the male:female ratio for hepatoblastoma?

2:1

421

How is the diagnosis of hepatoblastoma made?

Physical (abdominal distention, RUQ mass that moves with respiration); elevated AFP and ferritin; abdominal CT

422

What percentage of hepatoblastomas will have an elevated AFP level?

90%

423

What is the treatment for hepatoblastoma?

Resection by lobectomy or trisegmentectomy is the treatment of choice (plus postoperative chemotherapy).
Large tumors may require preoperative chemotherapy and subsequent hepatic resection.

424

What is the overall survival rate for hepatoblastoma?

50%

425

What is the major difference in age presentation between hepatoma and hepatoblastoma?

Hepatoblastoma presents at 3 years.

426

What is the leading cause of death in pediatric patients?

Trauma

427

How are the vast majority of splenic and liver injuries treated in children?

Observation

428

What is a common simulator of peritoneal signs in the blunt pediatric trauma victim?

Gastric distention

429

How do you estimate normal systolic BP in a child?

80 + 2*age

430

What is the 20-20-10 rule for fluid resuscitation of the unstable pediatric trauma patient?

1. 20-cc/kg LR bolus, then
2. 20-cc/kg LR bolus, if still unstable, then
3. 10-cc/kg of blood, if still unstable

431

What CT findings suggest small bowel injury?

Free fluid with no evidence of liver or spleen injury; free air; contrast leak; bowel thickening; mesentery streaking

432

What is the treatment for duodenal hematoma?

Observation with NGT and TPN

433

What does TORCHES stand for?

Nonbacterial fetal and neonatal infections:
TOxoplasmosis
Rubella
Cytomegalovirus
HErpes
Syphilis

434

What is the common pediatric sedative?

Chloral hydrate

435

What are the contraindications to circumcision?

Hypospadias (foreskin may be needed for future repair)

436

When should an umbilical hernia be repaired?

> 1.5 cm, after 4 years of age

437

What is the cancer risk in the cryptorchid testicle?

> 10-fold

438

When should orchidopexy be performed?

All patients with undescended testicle undergo orchidopexy after 1 year

439

What are some signs of child abuse?

Cigarette burns, rope burns, scald to posterior thighs and buttocks, multiple fractures or old fractures, genital trauma, delay in accessing health care system

440

What is the treatment of child abuse?

Admit the patient to the hospital

441

What is Dance's sign?

Empty RLQ in patients with ileocecal intussusception

442

What is the treatment of hemangioma?

Observation, because most regress spontaneously

443

What are the indications for operation in hemangiomas?

Severe thrombocytopenia, CHF, functional impairment (vision, breathing)

444

What are treatment options for hemangiomas?

Steroids, radiation, surgical resection, angiographic embolization

445

What is the most common benign liver tumor in children?

Hemangioma

446

What is Eagle-Barrett's syndrome?

Congenital inadequate abdominal musculature (also known as prune belly)

447

What is the Pierre-Robin syndrome?

1. Big, protruding tongue (glossoptosis)
2. Small mandible (micrognathia)
3. Cleft palate

448

What is the major concern with Pierre-Robin syndrome?

Airway obstruction by the tongue

449

What are the most common cancers in children?

1. Leukemia
2. CNS tumors
3. Lymphomas

450

What is the most common solid tumor in children?

CNS tumors

451

What syndrome must you consider in the patient with abdominal pain, hematuria, history of joint pain, and a purpuric rash?

HSP

452

What is Apley's law?

The further a chronically recurrent abdominal pain is from the umbilicus, the greater the likelihood of an organic cause for the pain

453

What is the most common cause of SBO in children?

Hernias

454

What is a patent urachus?

Persistence of the urachus, a communication between the bladder and umbilicus.
Presents with urine out of the umbilicus and recurrent UTIs.

455

What is a Replogle tube?

10 French sump pump NG tube for babies

456

What is Poland's syndrome?

Absence of pectoralis major or minor muscle.
Often associated with ipsilateral hand malformation.
Nipple/breast/right-breast hypoplasia.

457

What is the typical treatment of atypical mycobacterial lymph node infection?

Surgical removal of the node

458

What is the most common cause of rectal bleeding in infants?

Anal fissure

459

What chromosomal abnormality is associated with duodenal web/atresia/stenosis?

Trisomy 21

460

Which foreign body past the pylorus must be surgically removed?

Battery