Pediatric Surgery Flashcards
1
Q
What is the motto of pediatric surgery?
A
Children are not little adults
2
Q
What is a simple way to distract a pediatric patient when examining the abdomen for tenderness?
A
Listen to the abdomen with the stethoscope and then push down on the abdomen with the stethoscope to check for tenderness
3
Q
What is the estimated blood volume of infants and children?
A
8% of body weight or 80 cc/kg
4
Q
What is the maintenance IV fluid for children?
A
D5 1/4 NS + 20 mEq KCl
5
Q
Why 1/4 NS?
A
Children (especially those younger than 4 years) cannot concentrate their urine and cannot clear excess sodium
6
Q
How are maintenance fluid rates calculated in children?
A
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
Q
What is the minimal urine output for children?
A
From 1-2 mL/kg/hr
8
Q
What is the best way to present urine output measurements on rounds?
A
Urine output total per shift, then cc/kg/hr
9
Q
What is the major difference between adult and pediatric nutritional needs?
A
Premature infants/infants/children need more calories and protein/kg/day
10
Q
What are the caloric requirements for premature infants?
A
80 kcal/kg/day (and then go up)
11
Q
What are the caloric requirements for children younger than 1 year?
A
100 kcal/kg/day
12
Q
What are the caloric requirements for children ages 1-7?
A
85 kcal/kg/day
13
Q
What are the caloric requirements for children ages 7-12?
A
70 kcal/kg/day
14
Q
What are the caloric requirements for children ages 12-18?
A
40 kcal/kg/day
15
Q
What are the protein requirements for children younger than 1 year?
A
3 g/kg/day
16
Q
What are the protein requirements for children ages 1-7?
A
2 g/kg/day
17
Q
What are the protein requirements for children ages 7-12?
A
2 g/kg/day
18
Q
What are the protein requirements for children ages 12-18?
A
1.5 g/kg/day
19
Q
How many calories are in breast milk?
A
20 kcal/30 cc
20
Q
What is the blood volume per kg for a newborn infant?
A
85 cc/kg
21
Q
What is the blood volume per kg for an infant 1-3 months?
A
75 cc/kg
22
Q
What is the blood volume per kg for a child?
A
70 cc/kg
23
Q
What is the number of umbilical veins?
A
1 (usually)
24
Q
What is the number of umbilical arteries?
A
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 < 2 kg; intraventricular hemorrhage in brain (heparin in line)
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: No preference
| > 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 (< 1 year), strangulated hernia, intussusception, Meckel's diverticulum, infectious diarrhea, polyps, IBD, HUS, HSP, vascular malformation, coagulopathy
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 (< 2 cm)?
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 (< 2 years)?
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?
< 2 years, but can occur at any age
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
< 2 years of age
1/2 will have ectopic tissue
2 inches long
```
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 < 1000 g NEC patients?
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?
< 3 years
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.
| Hepatoma 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
