Pediatric Surgery, C67 P517-572

Question 1

What is the motto of
pediatric surgery?
P517

Answer 1

“Children are NOT little adults!”

Question 2

What is a simple way to
distract a pediatric patient
when examining the
abdomen for tenderness?
P517

Answer 2

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

Question 3

PEDIATRIC IV FLUIDS AND NUTRITION
What is the estimated blood
volume of infants and children?
P517

Answer 3

≈8% of body weight or ≈80 cc/kg

Question 4

PEDIATRIC IV FLUIDS AND NUTRITION
What is the maintenance IV
fluid for children?
P517

Answer 4

D5 1/4 NS + 20 mEq KCl

Question 5

PEDIATRIC IV FLUIDS AND NUTRITION
Why 1/4 NS?
P517

Answer 5

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

Question 6

PEDIATRIC IV FLUIDS AND NUTRITION
How are maintenance fluid
rates calculated in children?
P517

Answer 6

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 kilogram over
the first 20 (e.g., the rate for a
child weighing 25 kg is 4 x 10 = 40
plus 2 x 10 = 20 plus 1 x 5 = 5,
for an IVF rate of 65 cc/hr)

Question 7

PEDIATRIC IV FLUIDS AND NUTRITION
What is the minimal urine
output for children?
P517

Answer 7

From 1 to 2 mL/kg/hr

Question 8

PEDIATRIC IV FLUIDS AND NUTRITION
What is the best way to
present urine output
measurements on rounds?
P518

Answer 8

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

Question 9

PEDIATRIC IV FLUIDS AND NUTRITION
What is the major difference
between adult and pediatric
nutritional needs?
P518

Answer 9

Premature infants/infants/children need

more calories and protein/kg/day

Question 10

PEDIATRIC IV FLUIDS AND NUTRITION
What are the caloric requirements by age for the following patients:
Premature infants?
P518

Answer 10

80 Kcal/kg/day and then go up

Question 11

PEDIATRIC IV FLUIDS AND NUTRITION
What are the caloric requirements by age for the following patients:
Children younger than
1 year?
P518

Answer 11

≈100 Kcal/kg/day (90–120)

Question 12

PEDIATRIC IV FLUIDS AND NUTRITION
What are the caloric requirements by age for the following patients:
Children ages 1 to 7?
P518

Answer 12

≈85 Kcal/kg/day (75–90)

Question 13

PEDIATRIC IV FLUIDS AND NUTRITION
What are the caloric requirements by age for the following patients:
Children ages 7 to 12?
P518

Answer 13

≈70 Kcal/kg/day (60–75)

Question 14

PEDIATRIC IV FLUIDS AND NUTRITION
What are the caloric requirements by age for the following patients:
Youths ages 12 to 18
P518

Answer 14

≈40 Kcal/kg/day (30–60)

Question 15

PEDIATRIC IV FLUIDS AND NUTRITION
What are the protein requirements by age for the
following patients:
Children younger than 1 year?
P518

Answer 15

3 g/kg/day (2–3.5)

Question 16

PEDIATRIC IV FLUIDS AND NUTRITION
What are the protein requirements by age for the
following patients:
Children ages 1 to 7?
P518

Answer 16

2 g/kg/day (2–2.5)

Question 17

PEDIATRIC IV FLUIDS AND NUTRITION
What are the protein requirements by age for the
following patients:
Children ages 7 to 12?
P518

Answer 17

2 g/kg/day

Question 18

PEDIATRIC IV FLUIDS AND NUTRITION
What are the protein requirements by age for the
following patients:
Youths ages 12 to 18?
P518

Answer 18

1.5 grams/kg/day

Question 19

PEDIATRIC IV FLUIDS AND NUTRITION
How many calories are in
breast milk?
P518

Answer 19

20 Kcal/30 cc (same as most formulas)

Question 20

PEDIATRIC IV FLUIDS AND NUTRITION
PEDIATRIC BLOOD VOLUMES
Give blood volume per kilogram:
Newborn infant?
P518

Answer 20

85 cc

Question 21

PEDIATRIC IV FLUIDS AND NUTRITION
Infant 1–3 months of age?
P518

Answer 21

75 cc

Question 22

PEDIATRIC IV FLUIDS AND NUTRITION
Child?
P518

Answer 22

70 cc

Question 23

FETAL CIRCULATIONFETAL CIRCULATION
What is the number of
umbilical veins?
P519

Answer 23

1 (usually)

Question 24

FETAL CIRCULATIONFETAL CIRCULATION
What is the number of
umbilical arteries?
P519

Answer 24

2

Question 25

FETAL CIRCULATIONFETAL CIRCULATION Which umbilical vessel carries oxygenated blood? P519

Answer 25

Umbilical vein

Question 26

``` FETAL CIRCULATIONFETAL CIRCULATION The oxygenated blood travels through the liver to the IVC through which structure? P519 ```

Answer 26

Ductus venosus

Question 27

``` FETAL CIRCULATIONFETAL CIRCULATION Oxygenated blood passes from the right atrium to the left atrium through which structure? P519 ```

Answer 27

Foramen ovale

Question 28

``` FETAL CIRCULATIONFETAL CIRCULATION Unsaturated blood goes from the right ventricle to the descending aorta through which structure? P519 ```

Answer 28

Ductus arteriosum

Question 29

FETAL CIRCULATIONFETAL CIRCULATION Define the overall fetal circulation. P519 (picture)

Answer 29

(see Picture)

Question 30

``` FETAL CIRCULATIONFETAL CIRCULATION What are the ADULT structures of the following fetal structures: Ductus venosus? P520 ```

Answer 30

Ligamentum venosum

Question 31

``` FETAL CIRCULATIONFETAL CIRCULATION What are the ADULT structures of the following fetal structures: Umbilical vein? P520 ```

Answer 31

Ligamentum teres

Question 32

``` FETAL CIRCULATIONFETAL CIRCULATION What are the ADULT structures of the following fetal structures: Umbilical artery? P520 ```

Answer 32

Medial umbilical ligament

Question 33

``` FETAL CIRCULATIONFETAL CIRCULATION What are the ADULT structures of the following fetal structures: Ductus arteriosus? P520 ```

Answer 33

Ligamentum arteriosum

Question 34

``` FETAL CIRCULATIONFETAL CIRCULATION What are the ADULT structures of the following fetal structures: Urachus? P520 ```

Answer 34

Median umbilical ligament

Question 35

``` FETAL CIRCULATIONFETAL CIRCULATION What are the ADULT structures of the following fetal structures: Tongue remnant of thyroid’s descent? P520 ```

Answer 35

Foramen cecum

Question 36

``` FETAL CIRCULATIONFETAL CIRCULATION What are the ADULT structures of the following fetal structures: Persistent remnant of vitelline duct? P520 ```

Answer 36

Meckel’s diverticulum

Question 37

ECMO What is ECMO? P520

Answer 37

ExtraCorporeal Membrane Oxygenation: chronic cardiopulmonary bypass—for complete respiratory support

Question 38

ECMO What are the types of ECMO? P520

Answer 38

Venovenous: Blood from vein → oxygenated → back to vein Venoarterial: Blood from vein (IJ) → oxygenated → back to artery (carotid)

Question 39

ECMO What are the indications? P520

Answer 39

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

Question 40

ECMO What are the contraindications? P520

Answer 40

Weight <2 kg, IVH (IntraVentricular Hemorrhage in brain contraindicated because of heparin in line)

Question 41

``` NECK What is the major differential diagnosis of a pediatric neck mass? P521 ```

Answer 41

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

Question 42

THYROGLOSSAL DUCT CYST What is it? P521

Answer 42

``` 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 ```

Question 43

THYROGLOSSAL DUCT CYST What is the average age at diagnosis? P521

Answer 43

Usually presents around 5 years of age

Question 44

THYROGLOSSAL DUCT CYST How is the diagnosis made? P521

Answer 44

Ultrasound

Question 45

THYROGLOSSAL DUCT CYST What are the complications? P521

Answer 45

``` Enlargement, infection, and fistula formation between oropharynx or salivary gland; aberrant thyroid tissue may masquerade as thyroglossal duct cyst, and if it is not cystic, deserves a thyroid scan ```

Question 46

THYROGLOSSAL DUCT CYST What is the anatomic location? P522

Answer 46

Almost always in the midline

Question 47

``` THYROGLOSSAL DUCT CYST How can one remember the position of the thyroglossal duct cyst? P522 (picture) ```

Answer 47

Think: thyroGLOSSAL = TONGUE | midline sticking out

Question 48

THYROGLOSSAL DUCT CYST What is the treatment? P522

Answer 48

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

Question 49

BRANCHIAL CLEFT ANOMALIES What is it? P522

Answer 49

Remnant of the primitive branchial clefts in which epithelium forms a sinus tract between the pharynx (second cleft), or the external auditory canal (first cleft), and the skin of the anterior neck; if the sinus ends blindly, a cyst may form

Question 50

BRANCHIAL CLEFT ANOMALIES What is the common presentation? P522

Answer 50

Infection because of communication | between pharynx and external ear canal

Question 51

BRANCHIAL CLEFT ANOMALIES What is the anatomic position? P522

Answer 51

``` Second cleft anomaly—lateral to the midline along anterior border of the sternocleidomastoid, anywhere from angle of jaw to clavicle First cleft anomaly—less common than second cleft anomalies; tend to be located higher under the mandible ```

Question 52

BRANCHIAL CLEFT ANOMALIES What is the most common cleft remnant? P523

Answer 52

Second; thus, these are found most often laterally versus thyroglossal cysts, which are found centrally (Think: Second = Superior)

Question 53

BRANCHIAL CLEFT ANOMALIES What is the treatment? P523

Answer 53

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

Question 54

``` BRANCHIAL CLEFT ANOMALIES What is the major anatomic difference between thyroglossal cyst and branchial cleft cyst? P523 ```

Answer 54

Thyroglossal cyst = midline Branchial cleft cyst = lateral (Think: brAnchial = lAteral)

Question 55

STRIDOR What is stridor? P523

Answer 55

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

Question 56

STRIDOR What are the signs/ symptoms? P523

Answer 56

Dyspnea, cyanosis, difficulty with | feedings

Question 57

STRIDOR What is the differential diagnosis? P523

Answer 57

``` Laryngomalacia—leading cause of stridor in infants; results from inadequate development of supporting laryngeal structures; usually self-limited and treatment is expectant unless respiratory compromise is present Tracheobronchomalacia—similar to laryngomalacia, but involves the entire trachea Vascular rings and slings—abnormal development or placement of thoracic large vessels resulting in obstruction of trachea/bronchus ```

Question 58

STRIDOR What are the symptoms of vascular rings? P523

Answer 58

Stridor, dyspnea on exertion, or dysphagia

Question 59

STRIDOR How is the diagnosis of vascular rings made? P523

Answer 59

Barium swallow revealing typical configuration of esophageal compression Echo/arteriogram

Question 60

STRIDOR What is the treatment of vascular rings? P523

Answer 60

Surgical division of the ring, if the patient | is symptomatic

Question 61

CYSTIC HYGROMA What is it? P524

Answer 61

Congenital abnormality of lymph sac | resulting in lymphangioma

Question 62

CYSTIC HYGROMA What is the anatomic location? P524

Answer 62

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

Question 63

CYSTIC HYGROMA What is the treatment? P524

Answer 63

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

Question 64

CYSTIC HYGROMA What are the possible complications? P524

Answer 64

``` Enlargement in critical regions, such as the floor of the mouth or paratracheal region, may cause airway obstruction; also, they tend to insinuate onto major structures (although not malignant), making excision difficult and hazardous ```

Question 65

``` ASPIRATED FOREIGN BODY (FB) Which bronchus do FBs go into more commonly (left or right)? P524 ```

Answer 65

``` Younger than age 4—50/50 Age 4 and older—most go into right bronchus because it develops into a straight shot (less of an angle) ```

Question 66

ASPIRATED FOREIGN BODY (FB) What is the most commonly aspirated object? P524

Answer 66

Peanut

Question 67

ASPIRATED FOREIGN BODY (FB) What is the associated risk with peanut aspiration? P524

Answer 67

Lipoid pneumonia

Question 68

ASPIRATED FOREIGN BODY (FB) How can an FB result in “air trapping and hyperinflation”? P524

Answer 68

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

Question 69

``` ASPIRATED FOREIGN BODY (FB) How can you tell on A-P CXR if a coin is in the esophagus or the trachea? P524 ```

Answer 69

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

Question 70

ASPIRATED FOREIGN BODY (FB) What is the treatment of tracheal or esophageal FB? P525

Answer 70

Remove FB with rigid bronchoscope or | rigid esophagoscope

Question 71

CHEST What is the differential diagnosis of a lung mass? P525

Answer 71

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

Question 72

``` CHEST What is the differential diagnosis of mediastinal tumor/mass? P525 ```

Answer 72

``` 1. Neurogenic tumor (ganglioneuromas, neurofibromas) 2. Teratoma 3. Lymphoma 4. Thymoma (Classic “four T’s”: Teratoma, Terrible lymphoma, Thymoma, Thyroid tumor) Rare: pheochromocytoma, hemangioma, rhabdomyosarcoma, osteochondroma ```

Question 73

``` PECTUS DEFORMITY What heart abnormality is associated with pectus abnormality? P525 ```

Answer 73

Mitral valve prolapse (many patients | receive preoperative echocardiogram)

Question 74

PECTUS EXCAVATUM What is it? P525 (picture)

Answer 74

Chest wall deformity with sternum caving | inward (Think: exCAVatum = CAVE)

Question 75

PECTUS EXCAVATUM What is the cause? P526

Answer 75

Abnormal, unequal overgrowth of rib | cartilage

Question 76

PECTUS EXCAVATUM What are the signs/ symptoms? P526

Answer 76

Often asymptomatic; mental distress, | dyspnea on exertion, chest pain

Question 77

PECTUS EXCAVATUM What is the treatment? P526

Answer 77

Open perichondrium, remove abnormal cartilage, place substernal strut; new cartilage grows back in the perichondrium in normal position; remove strut 6 months later

Question 78

PECTUS EXCAVATUM What is the NUSS procedure? P526

Answer 78

Placement of metal strut to elevate | sternum without removing cartilage

Question 79

PECTUS CARINATUM What is it? P526 (picture)

Answer 79

Chest wall deformity with sternum outward (pectus = chest, carinatum = pigeon); much less common than pectus excavatum

Question 80

PECTUS CARINATUM What is the cause? P526

Answer 80

Abnormal, unequal overgrowth of rib | cartilage

Question 81

PECTUS CARINATUM What is the treatment? P526

Answer 81

``` Open perichondrium and remove abnormal cartilage Place substernal strut New cartilage grows into normal position Remove strut 6 months later ```

Question 82

ESOPHAGEAL ATRESIA WITHOUT TRACHEOESOPHAGEAL (TE) FISTULA What is it? P527

Answer 82

Blind-ending esophagus from atresia

Question 83

ESOPHAGEAL ATRESIA WITHOUT TRACHEOESOPHAGEAL (TE) FISTULA What are the signs? P527

Answer 83

Excessive oral secretions and inability to | keep food down

Question 84

ESOPHAGEAL ATRESIA WITHOUT TRACHEOESOPHAGEAL (TE) FISTULA How is the diagnosis made? P527

Answer 84

Inability to pass NG tube; plain x-ray shows tube coiled in upper esophagus and no gas in abdomen

Question 85

ESOPHAGEAL ATRESIA WITHOUT TRACHEOESOPHAGEAL (TE) FISTULA What is the primary treatment? P527

Answer 85

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

Question 86

ESOPHAGEAL ATRESIA WITHOUT TRACHEOESOPHAGEAL (TE) FISTULA What is the definitive treatment? P527

Answer 86

``` Surgical with 1 anastomosis, often with preoperative stretching of blind pouch (other options include colonic or jejunal interposition graft or gastric tube formation if esophageal gap is long) ```

Question 87

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA What is it? P527

Answer 87

Esophageal atresia occurring with a fistula to the trachea; occurs in >90% of cases of esophageal atresia

Question 88

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA What is the incidence? P527

Answer 88

One in 1500 to 3000 births

Question 89

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA Define the following types of fistulas/atresias: Type A P527 (picture)

Answer 89

Esophageal atresia without TE fistula (8%)

Question 90

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA Define the following types of fistulas/atresias: Type B P528 (picture)

Answer 90

``` Proximal esophageal atresia with proximal TE fistula (1%) ```

Question 91

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA Define the following types of fistulas/atresias: Type C P528 (picture)

Answer 91

``` Proximal esophageal atresia with distal TE fistula (85%); most common type ```

Question 92

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA Define the following types of fistulas/atresias: Type D P528 (picture)

Answer 92

Proximal esophageal atresia with both proximal and distal TE fistulas (2%) (Think: D = Double connection to trachea)

Question 93

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA Define the following types of fistulas/atresias: Type E P529 (picture)

Answer 93

“H-type” TE fistula without esophageal | atresia (4%)

Question 94

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA How do you remember which type is most common? P529

Answer 94

Simple: Most Common type is type C

Question 95

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA What are the symptoms? P529

Answer 95

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

Question 96

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA What are the signs? P529

Answer 96

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

Question 97

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA How is the diagnosis made? P529

Answer 97

Failure to pass an 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 (tracheoesophageal fistula)

Question 98

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA What is the initial treatment? P529

Answer 98

``` Directed toward minimizing complications from aspiration: 1. Suction blind pouch (NPO/TPN) 2. Upright position of child 3. Prophylactic antibiotics (Amp/gent) ```

Question 99

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA What is the definitive treatment? P529

Answer 99

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

Question 100

``` ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA What can be done to lengthen the proximal esophageal pouch? P530 ```

Answer 100

Delayed repair: with or without G-tube | and daily stretching of proximal pouch

Question 101

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA Which type should be fixed via a right neck incision? P530

Answer 101

“H-Type” (type E) is high in the thorax and can most often be approached via a right neck incision

Question 102

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA What is the workup of a patient with a TE fistula? P530

Answer 102

To evaluate the TE fistula and associated anomalies: CXR, AXR, U/S of kidneys, cardiac echo (rest of workup directed by physical exam)

Question 103

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA What are the associated anomalies? P530

Answer 103

``` VACTERL cluster (present in about 10% of cases): Vertebral or vascular, Anorectal, Cardiac, TE fistula, Esophageal atresia Radial limb and renal abnormalities, Lumbar and limb Previously known as VATER: Vertebral, Anus, TE fistula, Radial ```

Question 104

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL (TE) FISTULA What is the significance of a “gasless” abdomen on AXR? P530

Answer 104

No air to the stomach and, thus, no | tracheoesophageal fistula

Question 105

CONGENITAL DIAPHRAGMATIC HERNIA What is it? P530

Answer 105

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

Question 106

CONGENITAL DIAPHRAGMATIC HERNIA What is the incidence? P530

Answer 106

One in 2100 live births; males are more | commonly affected

Question 107

CONGENITAL DIAPHRAGMATIC HERNIA What are the types of hernias? P530

Answer 107

Bochdalek and Morgagni

Question 108

CONGENITAL DIAPHRAGMATIC HERNIA What are the associated positions? P530

Answer 108

Bochdalek—posterolateral with L > R Morgagni—anterior parasternal hernia, relatively uncommon

Question 109

``` CONGENITAL DIAPHRAGMATIC HERNIA How to remember the position of the Bochdalek hernia? P531 (picture) ```

Answer 109

Think: BOCH DA LEK = “BACK TO | THE LEFT”

Question 110

CONGENITAL DIAPHRAGMATIC HERNIA What are the signs? P531

Answer 110

Respiratory distress, dyspnea, tachypnea, retractions, and cyanosis; bowel sounds in the chest; rarely, maximal heart sounds on the right; ipsilateral chest dullness to percussion

Question 111

CONGENITAL DIAPHRAGMATIC HERNIA What are the effects on the lungs? P531

Answer 111

1. Pulmonary hypoplasia | 2. Pulmonary hypertension

Question 112

CONGENITAL DIAPHRAGMATIC HERNIA What inhaled agent is often used? P531

Answer 112

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

Question 113

CONGENITAL DIAPHRAGMATIC HERNIA What is the treatment? P531

Answer 113

NG tube, ET tube, stabilization, and if patient is stable, surgical repair; if patient is unstable: nitric oxide +/-- ECMO then to the O.R. when feasible

Question 114

PULMONARY SEQUESTRATION What is it? P531

Answer 114

Abnormal benign lung tissue with separate blood supply that DOES NOT communicate with the normal tracheobronchial airway

Question 115

PULMONARY SEQUESTRATION Define the following terms: Interlobar P531

Answer 115

Sequestration in the normal lung tissue | covered by normal visceral pleura

Question 116

PULMONARY SEQUESTRATION Define the following terms: Extralobar P532

Answer 116

Sequestration not in the normal lung | covered by its own pleura

Question 117

PULMONARY SEQUESTRATION What are the signs/ symptoms? P532

Answer 117

Asymptomatic, recurrent pneumonia

Question 118

PULMONARY SEQUESTRATION How is the diagnosis made? P532

Answer 118

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

Question 119

PULMONARY SEQUESTRATION What is the treatment of each type: Extralobar? P532

Answer 119

Surgical resection

Question 120

PULMONARY SEQUESTRATION What is the treatment of each type: Intralobar? P532

Answer 120

Lobectomy

Question 121

``` PULMONARY SEQUESTRATION What is the major risk during operation for sequestration? P532 ```

Answer 121

``` 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 ```

Question 122

``` ABDOMEN What is the differential diagnosis of pediatric upper GI bleeding? P532 ```

Answer 122

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

Question 123

``` ABDOMEN What is the differential diagnosis of pediatric lower GI bleeding? P532 ```

Answer 123

``` Upper GI bleeding, anal fissures, NEC (premature infants), midgut volvulus (usually children younger than 1 year), strangulated hernia, intussusception, Meckel’s diverticulum, infectious diarrhea, polyps, IBD, hemolytic uremic syndrome, Henoch-Schönlein purpura, vascular malformation, coagulopathy ```

Question 124

``` ABDOMEN What is the differential diagnosis of neonatal bowel obstruction? P532 ```

Answer 124

``` 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) ```

Question 125

``` ABDOMEN What is the differential diagnosis of infant constipation? P533 ```

Answer 125

Hirschsprung’s disease, CF (cystic fibrosis), | anteriorly displaced anus, polyps

Question 126

``` INGUINAL HERNIA What is the most commonly performed procedure by U.S. pediatric surgeons? P533 ```

Answer 126

Indirect inguinal hernia repair

Question 127

INGUINAL HERNIA What is the most common inguinal hernia in children? P533

Answer 127

Indirect

Question 128

INGUINAL HERNIA What is an indirect inguinal hernia? P533

Answer 128

``` Hernia lateral to Hesselbach’s triangle into the internal inguinal ring and down the inguinal canal (Think: through the abdominal wall indirectly into the internal ring and out through the external inguinal ring) ```

Question 129

INGUINAL HERNIA What is Hesselbach’s triangle? P533

Answer 129

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

Question 130

``` INGUINAL HERNIA What type of hernia goes through Hesselbach’s triangle? P533 ```

Answer 130

Direct hernia from a weak abdominal floor; rare in children (0.5% of all inguinal hernias)

Question 131

``` INGUINAL HERNIA What is the incidence of indirect inguinal hernia in all children? P533 ```

Answer 131

≈3%

Question 132

INGUINAL HERNIA What is the incidence in premature infants? P533

Answer 132

Up to 30%

Question 133

INGUINAL HERNIA What is the male to female ratio? P533

Answer 133

6:1

Question 134

INGUINAL HERNIA What are the risk factors for an indirect inguinal hernia? P533

Answer 134

``` Male gender, ascites, V-P shunt, prematurity, family history, meconium ileus, abdominal wall defect elsewhere, hypo/epispadias, connective tissue disease, bladder exstrophy, undescended testicle, CF ```

Question 135

INGUINAL HERNIA Which side is affected more commonly? P534

Answer 135

Right ( ≈60%)

Question 136

INGUINAL HERNIA What percentage are bilateral? P534

Answer 136

≈15%

Question 137

``` INGUINAL HERNIA What percentage have a family history of indirect hernias? P534 ```

Answer 137

≈10%

Question 138

INGUINAL HERNIA What are the signs/ symptoms? P534

Answer 138

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

Question 139

INGUINAL HERNIA What is the silk glove sign? P534

Answer 139

Hernia sac rolls under the finger like the | finger of a silk glove

Question 140

INGUINAL HERNIA Why should it be repaired? P534

Answer 140

Risk of incarcerated/strangulated bowel | or ovary; will not go away on its own

Question 141

INGUINAL HERNIA How is a pediatric inguinal hernia repaired? P534

Answer 141

``` 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) ```

Question 142

``` INGUINAL HERNIA Which infants need overnight apnea monitoring/ observation? P534 ```

Answer 142

Premature infants; infants younger than 3 | months of age

Question 143

``` INGUINAL HERNIA What is the risk of recurrence after high ligation of an indirect pediatric hernia? P534 ```

Answer 143

≈1%

Question 144

``` INGUINAL HERNIA Describe the steps in the repair of an indirect inguinal hernia from skin to skin. P534 ```

Answer 144

Cut skin, then fat, then Scarpa’s fascia, then external oblique fascia through the external inguinal ring; find hernia sac anteriomedially and bluntly separate from the other cord structures; ligate sac high at the neck at the internal inguinal ring; resect sac and allow sac stump to retract into the peritoneal cavity; close external oblique; close Scarpa’s fascia; close skin

Question 145

INGUINAL HERNIA Define the following terms: Cryptorchidism P535

Answer 145

Failure of the testicle to descend into the | scrotum

Question 146

INGUINAL HERNIA Define the following terms: Hydrocele P535

Answer 146

Fluid-filled sac (i.e., fluid in a patent processus vaginalis or in the tunica vaginalis around the testicle)

Question 147

``` INGUINAL HERNIA Define the following terms: Communicating hydrocele P535 ```

Answer 147

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

Question 148

``` INGUINAL HERNIA Define the following terms: Noncommunicating hydrocele P535 ```

Answer 148

Hydrocele that does not communicate with the peritoneal cavity; stays about the same size

Question 149

``` INGUINAL HERNIA Define the following terms: Can a hernia be ruled out if an inguinal mass transilluminates? P535 ```

Answer 149

NO; baby bowel is very thin and will | often transilluminate

Question 150

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA From what abdominal muscle layer is the cremaster muscle derived? P535 ```

Answer 150

Internal oblique muscle

Question 151

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA From what abdominal muscle layer is the inguinal ligament (a.k.a. Poupart’s ligament) derived? P535 ```

Answer 151

External oblique

Question 152

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA What nerve travels with the spermatic cord? P535 ```

Answer 152

Ilioinguinal nerve

Question 153

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA Name the 5 structures in the spermatic cord. P535 ```

Answer 153

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

Question 154

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA What is the hernia sac made of? P535 ```

Answer 154

Basically peritoneum or a patent | processus vaginalis

Question 155

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA What is the name of the fossa between the testicle and epididymis? P536 ```

Answer 155

Fossa of Geraldi

Question 156

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA What attaches the testicle to the scrotum? P536 ```

Answer 156

Gubernaculum

Question 157

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA How can the opposite side be assessed for a hernia intraoperatively? P536 ```

Answer 157

``` 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 ```

Question 158

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA Name the remnant of the processus vaginalis around the testicle. P536 ```

Answer 158

Tunica vaginalis

Question 159

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA What is a Littre’s inguinal hernia? P536 ```

Answer 159

Hernia with a Meckel’s diverticulum in | the hernia sac

Question 160

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA What may a yellow/orange tissue that is not fat be on the spermatic cord/testicle? P536 ```

Answer 160

Adrenal rest

Question 161

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA What is the most common organ in an inguinal hernia sac in boys? P536 ```

Answer 161

Small intestine

Question 162

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA What is the most common organ in an inguinal hernia sac in girls? P536 ```

Answer 162

Ovary/fallopian tube

Question 163

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA What lies in the inguinal canal in girls instead of the vas? P536 ```

Answer 163

Round ligament

Question 164

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA Where in the inguinal canal does the hernia sac lie in relation to the other structures? P536 ```

Answer 164

Anteriomedially

Question 165

CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA What is a “cord lipoma”? P536

Answer 165

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

Question 166

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA Within the spermatic cord, do the vessels or the vas lie medially? P537 ```

Answer 166

Vas is medial to the testicular vessels

Question 167

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA What is a small outpouching of testicular tissue off of the testicle? P537 ```

Answer 167

Testicular appendage (a.k.a. the appendix testes); should be removed with electrocautery

Question 168

CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA What is a “blue dot sign”? P537

Answer 168

Blue dot on the scrotal skin from a | twisted testicular appendage

Question 169

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA How is a transected vas treated? P537 ```

Answer 169

Repair with primary anastomosis

Question 170

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA How do you treat a transected ilioinguinal nerve? P537 ```

Answer 170

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

Question 171

``` CLASSIC INTRAOPERATIVE QUESTIONS DURING REPAIR OF AN INDIRECT INGUINAL HERNIA What happens if you cut the ilioinguinal nerve? P537 ```

Answer 171

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

Question 172

UMBILICAL HERNIA What is it? P537

Answer 172

Fascial defect at the umbilical ring

Question 173

UMBILICAL HERNIA What are the risk factors? P537

Answer 173

1. African American infant | 2. Premature infant

Question 174

UMBILICAL HERNIA What are the indications for surgical repair? P537

Answer 174

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

Question 175

GERD What is it? P537

Answer 175

GastroEsophageal Reflux Disease

Question 176

GERD What are the causes? P537

Answer 176

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

Question 177

GERD What are the signs/ symptoms? P538

Answer 177

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

Question 178

GERD How is the diagnosis made? P538

Answer 178

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

Question 179

``` GERD What cytologic aspirate finding on bronchoscopy can diagnose aspiration of gastric contents? P538 ```

Answer 179

Lipid-laden macrophages (from | phagocytosis of fat)

Question 180

GERD What is the medical/ conservative treatment? P538

Answer 180

H(2) blockers Small meals/rice cereal Elevation of head

Question 181

GERD What are the indications for surgery? P538

Answer 181

``` “SAFE”: Stricture Aspiration, pneumonia/asthma Failure to thrive Esophagitis ```

Question 182

GERD What is the surgical treatment? P538

Answer 182

Nissen 360 fundoplication, with or | without G tube

Question 183

CONGENITAL PYLORIC STENOSIS What is it? P538 (picture)

Answer 183

Hypertrophy of smooth muscle of pylorus, | resulting in obstruction of outflow

Question 184

CONGENITAL PYLORIC STENOSIS What are the associated risks? P538

Answer 184

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

Question 185

CONGENITAL PYLORIC STENOSIS What is the incidence? P539

Answer 185

1 in 750 births, M:F ratio = 4:1

Question 186

CONGENITAL PYLORIC STENOSIS What is the average age at onset? P539

Answer 186

``` Usually from 2 weeks after birth to about 2 months (“2 to 2”) ```

Question 187

CONGENITAL PYLORIC STENOSIS What are the symptoms? P539

Answer 187

Increasing frequency of regurgitation, leading to eventual nonbilious projectile vomiting

Question 188

CONGENITAL PYLORIC STENOSIS Why is the vomiting nonbilious? P539

Answer 188

Obstruction is proximal to the ampulla of | Vater

Question 189

CONGENITAL PYLORIC STENOSIS What are the signs? P539

Answer 189

``` Abdominal mass or “olive” in epigastric region (85%), hypokalemic hypochloremic metabolic alkalosis, icterus (10%), visible gastric peristalsis, paradoxic aciduria, hematemesis ( <10%) ```

Question 190

CONGENITAL PYLORIC STENOSIS What is the differential diagnosis? P539

Answer 190

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

Question 191

CONGENITAL PYLORIC STENOSIS How is the diagnosis made? P539

Answer 191

``` Usually by history and physical exam alone U/S—demonstrates elongated ( >15 mm) pyloric channel and thickened muscle wall ( >3.5 mm) If U/S is nondiagnostic, then barium swallow—shows “string sign” or “double railroad track sign” ```

Question 192

CONGENITAL PYLORIC STENOSIS What is the initial treatment? P539

Answer 192

Hydration and correction of alkalosis with D10 NS plus 20 mEq of KCl (Note: the infant’s liver glycogen stores are very small; therefore, use D10; Cl and hydration will correct the alkalosis)

Question 193

CONGENITAL PYLORIC STENOSIS What is the definitive treatment? P539

Answer 193

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

Question 194

CONGENITAL PYLORIC STENOSIS What are the postoperative complications? P540

Answer 194

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

Question 195

CONGENITAL PYLORIC STENOSIS What is the appropriate postoperative feeding? P540

Answer 195

Start feeding with Pedialyte® at 6 to 12 hours postoperatively; advance to full-strength formula over 24 hours

Question 196

CONGENITAL PYLORIC STENOSIS Which vein crosses the pylorus? P540

Answer 196

Vein of Mayo

Question 197

DUODENAL ATRESIA What is it? P540

Answer 197

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

Question 198

DUODENAL ATRESIA What is the anatomic location? P540

Answer 198

85% are distal to the ampulla of Vater, 15% are proximal to the ampulla of Vater (these present with nonbilious vomiting)

Question 199

DUODENAL ATRESIA What are the signs? P540

Answer 199

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

Question 200

DUODENAL ATRESIA What is the differential diagnosis? P540

Answer 200

Malrotation with Ladd’s bands, annular | pancreas

Question 201

DUODENAL ATRESIA How is the diagnosis made? P540

Answer 201

Plain abdominal film revealing “double bubble,” with one air bubble in the stomach and the other in the duodenum

Question 202

DUODENAL ATRESIA What is the treatment? P540

Answer 202

Duodenoduodenostomy or | duodenojejunostomy

Question 203

DUODENAL ATRESIA What are the associated abnormalities? P540

Answer 203

50% to 70% have cardiac, renal, or other gastrointestinal defects; 30% have trisomy 21

Question 204

MECONIUM ILEUS What is it? P540

Answer 204

Intestinal obstruction from solid | meconium concretions

Question 205

MECONIUM ILEUS What is the incidence? P540

Answer 205

Occurs in ≈15% of infants with CF

Question 206

``` MECONIUM ILEUS What percentage of patients with meconium ileus have CF (cystic fibrosis)? P541 ```

Answer 206

>95%

Question 207

MECONIUM ILEUS What are the signs/symptoms of meconium ileus? P541

Answer 207

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

Question 208

MECONIUM ILEUS What is Neuhauser’s sign? P541

Answer 208

A.k.a. “soap bubble” sign: ground glass appearance in the RLQ on AXR from viscous meconium mixing with air

Question 209

MECONIUM ILEUS How is the diagnosis made? P541

Answer 209

``` Family history of CF, plain abdominal films showing significant dilation of similar-sized bowel loops, but few if any air-fluid levels, BE may demonstrate “microcolon” and inspissated meconium pellets in the terminal ileum ```

Question 210

MECONIUM ILEUS What is the treatment? P541

Answer 210

``` 70% nonoperative clearance of meconium using gastrografin enema, +/-- acetylcysteine, which is hypertonic and therefore draws fluid into lumen, separating meconium pellets from bowel wall (60% success rate) ```

Question 211

MECONIUM ILEUS What is the surgical treatment? P541

Answer 211

``` If enema is unsuccessful, then enterotomy with intraoperative catheter irrigation using acetylcysteine (Mucomyst®) ```

Question 212

MECONIUM ILEUS What should you remove during all operative cases? P541

Answer 212

Appendix

Question 213

MECONIUM ILEUS What is the long-term medical treatment? P541

Answer 213

Pancreatic enzyme replacement

Question 214

MECONIUM ILEUS What is cystic fibrosis (CF)? P541

Answer 214

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/liter) and genetic testing

Question 215

MECONIUM ILEUS What is DIOS? P541

Answer 215

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

Question 216

MECONIUM PERITONITIS What is it? P542

Answer 216

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

Question 217

MECONIUM PERITONITIS What are the signs? P542

Answer 217

Calcifications on plain films

Question 218

MECONIUM PLUG SYNDROME What is it? P542

Answer 218

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

Question 219

MECONIUM PLUG SYNDROME What is it also known as? P542

Answer 219

Neonatal small left colon syndrome

Question 220

MECONIUM PLUG SYNDROME What are the signs/ symptoms? P542

Answer 220

Abdominal distention and failure to pass meconium within first 24 hours of life; plain films demonstrate many loops of distended bowel and air-fluid levels

Question 221

MECONIUM PLUG SYNDROME What is the nonoperative treatment? P542

Answer 221

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

Question 222

MECONIUM PLUG SYNDROME What is the major differential diagnosis? P542

Answer 222

Hirschsprung’s disease

Question 223

MECONIUM PLUG SYNDROME Is meconium plug highly associated with CF? P542

Answer 223

No; <5% of patients have CF, in contrast to meconium ileus, in which nearly all have CF (95%)

Question 224

ANORECTAL MALFORMATIONS What are they? P542

Answer 224

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

Question 225

ANORECTAL MALFORMATIONS IMPERFORATE ANUS What is it? P542

Answer 225

Congenital absence of normal anus | complete absence or fistula

Question 226

ANORECTAL MALFORMATIONS IMPERFORATE ANUS Define a “high” imperforate anus. P543

Answer 226

Rectum patent to level above | puborectalis sling

Question 227

ANORECTAL MALFORMATIONS IMPERFORATE ANUS Define “low” imperforate anus. P543

Answer 227

Rectum patent to below puborectalis | sling

Question 228

``` ANORECTAL MALFORMATIONS IMPERFORATE ANUS Which type is much more common in women? P543 ```

Answer 228

Low

Question 229

``` ANORECTAL MALFORMATIONS IMPERFORATE ANUS What are the associated anomalies? P543 ```

Answer 229

Vertebral abnormalities, Anal abnormalities, Cardiac, TE fistulas, Esophageal Atresia, Radial/Renal abnormalities, Lumbar abnormalities (VACTERL; most commonly TE fistula)

Question 230

``` ANORECTAL MALFORMATIONS IMPERFORATE ANUS What are the signs/ symptoms? P543 ```

Answer 230

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

Question 231

ANORECTAL MALFORMATIONS IMPERFORATE ANUS How is the diagnosis made? P543

Answer 231

Physical exam, the classic Cross table “invertogram” plain x-ray to see level of rectal gas (not very accurate), perineal ultrasound

Question 232

``` ANORECTAL MALFORMATIONS IMPERFORATE ANUS What is the treatment of the following conditions: Low imperforate anus with anal fistula? P543 ```

Answer 232

Dilatation of anal fistula and subsequent | anoplasty

Question 233

``` ANORECTAL MALFORMATIONS IMPERFORATE ANUS What is the treatment of the following conditions: High imperforate anus? P543 ```

Answer 233

Diverting colostomy and mucous fistula; | neoanus is usually made at 1 year of age

Question 234

HIRSCHSPRUNG’S DISEASE What is it also known as? P543

Answer 234

Aganglionic megacolon

Question 235

HIRSCHSPRUNG’S DISEASE What is it? P543

Answer 235

``` Neurogenic form of intestinal obstruction in which obstruction results from inadequate relaxation and peristalsis; absence of normal ganglion cells of the rectum and colon ```

Question 236

HIRSCHSPRUNG’S DISEASE What are the associated risks? P543

Answer 236

Family history; 5% chance of having a | second child with the affliction

Question 237

HIRSCHSPRUNG’S DISEASE What is the male to female ratio? P544

Answer 237

What is the male to female | ratio?

Question 238

HIRSCHSPRUNG’S DISEASE What is the anatomic location? P544

Answer 238

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

Question 239

HIRSCHSPRUNG’S DISEASE What are the signs/ symptoms? P544

Answer 239

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

Question 240

HIRSCHSPRUNG’S DISEASE What is the classic history? P544

Answer 240

Failure to pass meconium in the first | 24 hours of life

Question 241

HIRSCHSPRUNG’S DISEASE What is the differential diagnosis? P544

Answer 241

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

Question 242

HIRSCHSPRUNG’S DISEASE What imaging studies should be ordered? P544

Answer 242

AXR: reveals dilated colon Unprepared barium enema: reveals constricted aganglionic segment with dilated proximal segment, but this picture may not develop for 3 to 6 weeks; BE will also demonstrate retention of barium for 24 to 48 hours (normal evacuation = 10 to 18 hours)

Question 243

HIRSCHSPRUNG’S DISEASE What is needed for definitive diagnosis? P544

Answer 243

``` Rectal biopsy: for definitive diagnosis, submucosal suction biopsy is adequate in 90% of cases; otherwise, full-thickness biopsy should be performed to evaluate Auerbach’s plexus ```

Question 244

HIRSCHSPRUNG’S DISEASE What is the “colonic transition zone”? P544

Answer 244

Transition (taper) from aganglionic small colon into the large dilated normal colon seen on BE

Question 245

HIRSCHSPRUNG’S DISEASE What is the initial treatment? P544

Answer 245

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

Question 246

HIRSCHSPRUNG’S DISEASE What is a “leveling” colostomy? P545

Answer 246

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

Question 247

HIRSCHSPRUNG’S DISEASE Describe the following procedures: Swenson P545 (picture)

Answer 247

Primary anastomosis between the anal | canal and healthy bowel (rectum removed)

Question 248

HIRSCHSPRUNG’S DISEASE Describe the following procedures: Duhamel P545 (picture)

Answer 248

Anterior, aganglionic region of the rectum is preserved and anastomosed to a posterior portion of healthy bowel; a functional rectal pouch is thereby created (Think: duha = dual barrels side by side)

Question 249

HIRSCHSPRUNG’S DISEASE Describe the following procedures: Soave P546 (picture)

Answer 249

A.k.a. endorectal pull-through; this procedure involves bringing proximal normal colon through the aganglionic rectum, which has been stripped of its mucosa but otherwise present (Think: SOAVE = SAVE the rectum, lose the mucosa)

Question 250

``` HIRSCHSPRUNG’S DISEASE What is the new trend in surgery for Hirschsprung’s disease? P546 ```

Answer 250

No colostomy; remove aganglionic colon (as confirmed on frozen section) and perform pull-through anastomosis at the same time (Boley modification)

Question 251

HIRSCHSPRUNG’S DISEASE What is the prognosis? P546

Answer 251

Overall survival rate >90%; >96% of patients continent; postoperative symptoms improve with age

Question 252

MALROTATION AND MIDGUT VOLVULUS What is it? P546

Answer 252

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

Question 253

MALROTATION AND MIDGUT VOLVULUS Where is the cecum? P546

Answer 253

With malrotation, the cecum usually ends | up in the RUQ

Question 254

MALROTATION AND MIDGUT VOLVULUS What are Ladd’s bands? P547 (picture)

Answer 254

Fibrous bands that extend from the abnormally placed cecum in the RUQ, often crossing over the duodenum and causing obstruction

Question 255

MALROTATION AND MIDGUT VOLVULUS What is the usual age at onset? P547

Answer 255

33% are present by 1 week of age, 75% | by 1 month, and 90% by 1 year

Question 256

MALROTATION AND MIDGUT VOLVULUS What is the usual presentation? P547

Answer 256

Sudden onset of bilious vomiting (bilious vomiting in an infant is malrotation until proven otherwise!)

Question 257

MALROTATION AND MIDGUT VOLVULUS Why is the vomiting bilious? P547

Answer 257

“Twist” is distal to the ampulla of Vater

Question 258

MALROTATION AND MIDGUT VOLVULUS How is the diagnosis made? P547

Answer 258

Upper GI contrast study showing cutoff in duodenum; BE showing abnormal position of cecum in the upper abdomen

Question 259

MALROTATION AND MIDGUT VOLVULUS What are the possible complications? P547

Answer 259

Volvulus with midgut infarction, leading to death or necessitating massive enterectomy (rapid diagnosis is essential!)

Question 260

MALROTATION AND MIDGUT VOLVULUS What is the treatment? P547

Answer 260

IV antibiotics and fluid resuscitation with LR, followed by emergent laparotomy with Ladd’s procedure; second-look laparotomy if bowel is severely ischemic in 24 hours to determine if remaining bowel is viable

Question 261

MALROTATION AND MIDGUT VOLVULUS What is the Ladd’s procedure? P548

Answer 261

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

Question 262

``` MALROTATION AND MIDGUT VOLVULUS In what direction is the volvulus reduced—clockwise or counterclockwise? P548 ```

Answer 262

Rotation of the bowel in a | counterclockwise direction

Question 263

MALROTATION AND MIDGUT VOLVULUS Where is the cecum after reduction? P548

Answer 263

LLQ

Question 264

``` MALROTATION AND MIDGUT VOLVULUS What is the cause of bilious vomiting in an infant until proven otherwise? P548 ```

Answer 264

Malrotation with midgut volvulus

Question 265

OMPHALOCELE What is it? P548

Answer 265

Defect of abdominal wall at umbilical | ring; sac covers extruded viscera

Question 266

OMPHALOCELE How is it diagnosed prenatally? P548

Answer 266

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

Question 267

OMPHALOCELE What comprises the “sac”? P548

Answer 267

Peritoneum and amnion

Question 268

``` OMPHALOCELE What organ is often found protruding from an omphalocele, but is almost never found with a gastroschisis? P548 ```

Answer 268

The liver

Question 269

OMPHALOCELE What is the incidence? P548

Answer 269

≈1 in 5000 births

Question 270

OMPHALOCELE How is the diagnosis made? P548

Answer 270

Prenatal U/S

Question 271

OMPHALOCELE What are the possible complications? P548

Answer 271

Malrotation of the gut, anomalies

Question 272

OMPHALOCELE What is the treatment? P548

Answer 272

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

Question 273

OMPHALOCELE What is the treatment of a small defect ( <2 cm)? P549

Answer 273

Closure of abdominal wall

Question 274

OMPHALOCELE What is the treatment of a medium defect (2–10 cm)? P549 (picture)

Answer 274

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 to 7 days, as the abdomen accommodates the viscera; then the defect is closed

Question 275

OMPHALOCELE What is the treatment of “giant” defects ( >10 cm)? P549

Answer 275

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

Question 276

OMPHALOCELE What are the associated abnormalities? P550

Answer 276

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

Question 277

OMPHALOCELE Of what “pentalogy” is omphalocele a part? P550

Answer 277

Pentalogy of Cantrell

Question 278

OMPHALOCELE What is the pentalogy of Cantrell? P550

Answer 278

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

Question 279

GASTROSCHISIS What is it? P550

Answer 279

Defect of abdominal wall; sac does not | cover extruded viscera

Question 280

GASTROSCHISIS How is it diagnosed prenatally? P550

Answer 280

Possible at fetal ultrasound after 13 weeks’ | gestation, elevated maternal AFP

Question 281

GASTROSCHISIS Where is the defect? P550

Answer 281

Lateral to the umbilicus | Think: gAstrochisis = lAteral

Question 282

``` GASTROSCHISIS On what side of the umbilicus is the defect most commonly found? P550 ```

Answer 282

Right

Question 283

GASTROSCHISIS What is the usual size of the defect? P550

Answer 283

2 to 4 cm

Question 284

GASTROSCHISIS What are the possible complications? P550

Answer 284

``` Thick edematous peritoneum from exposure to amnionic fluid; malrotation of the gut Other complications include hypothermia; hypovolemia from third-spacing; sepsis; and metabolic acidosis from hypovolemia and poor perfusion, NEC, prolonged ileus ```

Question 285

GASTROSCHISIS How is the diagnosis made? P550

Answer 285

Prenatal U/S

Question 286

GASTROSCHISIS What is the treatment? P551

Answer 286

``` Primary—NG tube decompression, IV fluids (D10 LR), and IV antibiotics Definitive—surgical reduction of viscera and abdominal closure; may require staged closure with silo ```

Question 287

GASTROSCHISIS What is a “silo”? P551

Answer 287

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

Question 288

GASTROSCHISIS What is the prognosis? P551

Answer 288

>90% survival rate

Question 289

GASTROSCHISIS What are the associated anomalies? P551

Answer 289

Unlike omphalocele, relatively uncommon except for intestinal atresia, which occurs in 10% to 15% of cases

Question 290

``` GASTROSCHISIS What are the major differences compared with omphalocele? P551 ```

Answer 290

No membrane coverings Uncommon associated abnormalities Lateral to umbilicus—not on umbilicus

Question 291

``` GASTROSCHISIS How can you remember the position of omphalocele vs. gastroschisis? P551 ```

Answer 291

Think: OMphalocele = ON the | umbilicus

Question 292

``` GASTROSCHISIS How do you remember that omphalocele is associated with abnormalities in 50% of cases? P551 ```

Answer 292

Think: Omphalocele = “Oh no, lots of | abnormalities”

Question 293

``` POWER REVIEW OF OMPHALOCELE AND GASTROSCHISIS What are the differences between omphalocele and gastroschisis in terms of the following characteristics: Anomalies? P551 ```

Answer 293

Common in omphalocele (50%), | uncommon in gastroschisis

Question 294

``` POWER REVIEW OF OMPHALOCELE AND GASTROSCHISIS What are the differences between omphalocele and gastroschisis in terms of the following characteristics: Peritoneal/amnion covering (sac)? P551 ```

Answer 294

Always with omphalocele—never with | gastroschisis

Question 295

``` POWER REVIEW OF OMPHALOCELE AND GASTROSCHISIS What are the differences between omphalocele and gastroschisis in terms of the following characteristics: Position of umbilical cord? P551 ```

Answer 295

On the sac with omphalocele, from skin | to the left of the gastroschisis defect

Question 296

``` POWER REVIEW OF OMPHALOCELE AND GASTROSCHISIS What are the differences between omphalocele and gastroschisis in terms of the following characteristics: Thick bowel? P552 ```

Answer 296

Common with gastroschisis, rare with | omphalocele (unless sac ruptures)

Question 297

``` POWER REVIEW OF OMPHALOCELE AND GASTROSCHISIS What are the differences between omphalocele and gastroschisis in terms of the following characteristics: Protrusion of liver? P552 ```

Answer 297

Common with omphalocele, almost never | with gastroschisis

Question 298

``` POWER REVIEW OF OMPHALOCELE AND GASTROSCHISIS What are the differences between omphalocele and gastroschisis in terms of the following characteristics: Large defect? P552 ```

Answer 298

Omphalocele

Question 299

APPENDICITIS What is it? P552

Answer 299

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

Question 300

APPENDICITIS What is its claim to fame? P552

Answer 300

Most common surgical disease requiring | emergency surgery in children

Question 301

APPENDICITIS What is the affected age? P552

Answer 301

Very rare before 3 years of age

Question 302

APPENDICITIS What is the usual presentation? P552

Answer 302

``` Onset of referred or periumbilical pain followed by anorexia, nausea, and vomiting (Note: Unlike gastroenteritis, pain precedes vomiting, then migrates to the RLQ, where it intensifies from local peritoneal irritation) If the patient is hungry and can eat, seriously question the diagnosis of appendicitis ```

Question 303

APPENDICITIS How is the diagnosis made? P552

Answer 303

History and physical exam

Question 304

APPENDICITIS What are the signs/ symptoms? P552

Answer 304

``` Signs of peritoneal irritation may be present—guarding, muscle spasm, rebound tenderness, obturator and Psoas signs; low-grade fever rising to high grade if perforation occurs ```

Question 305

APPENDICITIS What is the differential diagnosis? P552

Answer 305

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

Question 306

``` APPENDICITIS What is the common bacterial cause of mesenteric lymphadenitis? P553 ```

Answer 306

Yersinia enterocolitica

Question 307

APPENDICITIS What are the associated lab findings with appendicitis? P553

Answer 307

``` Increased WBC ( >10,000 per mm in >90% of cases, with a left shift in most) ```

Question 308

APPENDICITIS What is the role of urinalysis? P553

Answer 308

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

Question 309

APPENDICITIS What is the “hamburger” sign? P553

Answer 309

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

Question 310

APPENDICITIS What radiographic studies may be performed? P553

Answer 310

Often none; CXR to rule out RML or RLL pneumonia; abdominal films are usually nonspecific, but calcified fecalith is present in 5% of cases; U/S to evaluate for ovarian/gynecologic pathology

Question 311

APPENDICITIS What is the treatment? P553

Answer 311

``` Nonperforated—prompt appendectomy and cefoxitin to avoid perforation Perforated—triple antibiotics, fluid resuscitation, and prompt appendectomy; all pus is drained and cultures obtained, with postoperative antibiotics continued for 5 to 7 days, ± drain ```

Question 312

``` APPENDICITIS How long should antibiotics be administered if nonperforated? P553 ```

Answer 312

24 hours

Question 313

APPENDICITIS How long if perforated? P553

Answer 313

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

Question 314

``` APPENDICITIS If a normal appendix is found upon exploration, what must be examined/ ruled out? P553 ```

Answer 314

Meckel’s diverticulum, Crohn’s disease, | intussusception, gynecologic disease

Question 315

APPENDICITIS What is the approximate risk of perforation? P554

Answer 315

≈25% after 24 hours from onset of symptoms ≈50% by 36 hours ≈75% by 48 hours

Question 316

INTUSSUSCEPTION What is it? P554

Answer 316

Obstruction caused by bowel telescoping into the lumen of adjacent distal bowel; may result when peristalsis carries a “leadpoint” downstream

Question 317

INTUSSUSCEPTION What is its claim to fame? P554

Answer 317

Most common cause of small bowel | obstruction in toddlers ( <2 years old)

Question 318

INTUSSUSCEPTION What is the usual age at presentation? P554

Answer 318

Disease of infancy; 60% present from 4 to | 12 months of age, 80% by 2 years of age

Question 319

INTUSSUSCEPTION What is the most common site? P554

Answer 319

Terminal ileum involving ileocecal valve | and extending into ascending colon

Question 320

INTUSSUSCEPTION What is the most common cause? P554

Answer 320

Hypertrophic Peyer’s patches, which act as a lead point; many patients have prior viral illness

Question 321

INTUSSUSCEPTION What are the signs/ symptoms? P554

Answer 321

Alternating lethargy and irritability (colic), bilious vomiting, “currant jelly” stools, RLQ mass on plain abdominal film, empty RLQ on palpation (Dance’s sign)

Question 322

INTUSSUSCEPTION What is the intussuscipiens? P554

Answer 322

Recipient segment of bowel (Think: | recipiens = intussuscipiens)

Question 323

INTUSSUSCEPTION What is the intussusceptum? P554

Answer 323

Leading point or bowel that enters the | intussuscipiens

Question 324

INTUSSUSCEPTION Identify locations 1 and 2 on the following illustration: P554 (picture)

Answer 324

1. Intussuscipiens | 2. Intussusceptum

Question 325

``` INTUSSUSCEPTION How can the spelling of intussusception be remembered? P555 ```

Answer 325

Imagine a navy ship named The | U.S.S. U.S.—INTUSSUSCEPTION

Question 326

INTUSSUSCEPTION What is the treatment? P555

Answer 326

``` Air or barium enema; 85% reduce with hydrostatic pressure (i.e., barium = meter elevation air = maximum of 120 mm Hg); if unsuccessful, then laparotomy and reduction by “milking” the ileum from the colon should be performed ```

Question 327

``` INTUSSUSCEPTION What are the causes of intussusception in older patients? P555 ```

Answer 327

Meckel’s diverticulum, polyps, and tumors, all of which act as a lead point

Question 328

MECKEL’S DIVERTICULUM What is it? P556

Answer 328

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

Question 329

MECKEL’S DIVERTICULUM What is the usual location? P556

Answer 329

Between 45 and 90 cm proximal to the ileocecal valve on the antimesenteric border of the bowel

Question 330

MECKEL’S DIVERTICULUM What is the major differential diagnosis? P556

Answer 330

Appendicitis

Question 331

MECKEL’S DIVERTICULUM Is it a true diverticulum? P556

Answer 331

Yes; all layers of the intestine are found | in the wall

Question 332

MECKEL’S DIVERTICULUM What is the incidence? P556

Answer 332

2% of the population at autopsy, but | >90% of these are asymptomatic

Question 333

MECKEL’S DIVERTICULUM What is the gender ratio? P556

Answer 333

2 to 3x more common in males

Question 334

MECKEL’S DIVERTICULUM What is the usual age at onset of symptoms? P556

Answer 334

Most frequently in the first 2 years of life, | but can occur at any age

Question 335

MECKEL’S DIVERTICULUM What are the possible complications? 556

Answer 335

``` Intestinal hemorrhage (painless)—50% Accounts for 50% of all lower GI bleeding in patients younger than 2 years; bleeding results from ectopic gastric mucosa secreting acid → ulcer → bleeding Intestinal obstruction—25% Most common complication in adults; includes volvulus and intussusception Inflammation ( ± perforation)—20% ```

Question 336

MECKEL’S DIVERTICULUM What percentage of cases have heterotopic tissue? 556

Answer 336

>50%; usually gastric mucosa (85%), but duodenal, pancreatic, and colonic mucosa have been described

Question 337

``` MECKEL’S DIVERTICULUM What is the most common ectopic tissue in a Meckel’s diverticulum? 556 ```

Answer 337

Gastric mucosa

Question 338

``` MECKEL’S DIVERTICULUM What other pediatric disease entity can also present with GI bleeding secondary to ectopic gastric mucosa? 556 ```

Answer 338

Enteric duplications

Question 339

``` MECKEL’S DIVERTICULUM What is the most common cause of lower GI bleeding in children? 556 ```

Answer 339

Meckel’s diverticulum with ectopic | gastric mucosa

Question 340

MECKEL’S DIVERTICULUM What is the “rule of 2s”? 556

Answer 340

``` 2% are symptomatic Found ≈2 feet from ileocecal valve Found in 2% of the population Most symptoms occur before age 2 One of 2 will have ectopic tissue Most diverticula are about 2 inches long Male:female ratio = 2:1 ```

Question 341

MECKEL’S DIVERTICULUM What is a Meckel’s scan? 556

Answer 341

Scan for ectopic gastric mucosa in Meckel’s diverticulum; uses technetium Tc 99m pertechnetate IV, which is preferentially taken up by gastric mucosa

Question 342

NECROTIZING ENTEROCOLITIS What is it also known as? P57

Answer 342

NEC

Question 343

NECROTIZING ENTEROCOLITIS What is it? P57

Answer 343

Necrosis of intestinal mucosa, often with bleeding; may progress to transmural intestinal necrosis, shock/sepsis, and death

Question 344

NECROTIZING ENTEROCOLITIS What are the predisposing conditions? P57

Answer 344

``` PREMATURITY Stress: shock, hypoxia, RDS, apneic episodes, sepsis, exchange transfusions, PDA and cyanotic heart disease, hyperosmolar feedings, polycythemia, indomethacin ```

Question 345

NECROTIZING ENTEROCOLITIS What is the pathophysiologic mechanism? P57

Answer 345

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

Question 346

NECROTIZING ENTEROCOLITIS What is its claim to fame? P57

Answer 346

Most common cause of emergent | laparotomy in the neonate

Question 347

NECROTIZING ENTEROCOLITIS What are the signs/ symptoms? P57

Answer 347

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

Question 348

NECROTIZING ENTEROCOLITIS What are the radiographic findings? P57

Answer 348

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

Question 349

NECROTIZING ENTEROCOLITIS What are the lab findings? P57

Answer 349

Low hematocrit, glucose, and platelets

Question 350

NECROTIZING ENTEROCOLITIS What is the treatment? P57

Answer 350

Most are managed medically: 1. Cessation of feedings 2. OG tube 3. IV fluids 4. IV antibiotics 5. Ventilator support, as needed

Question 351

NECROTIZING ENTEROCOLITIS What are the surgical indications? P57

Answer 351

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

Question 352

NECROTIZING ENTEROCOLITIS Operation? P58

Answer 352

1. Resect | 2. Stoma

Question 353

``` NECROTIZING ENTEROCOLITIS What is an option for bowel perforation in 1000 gram NEC patients? P58 ```

Answer 353

Placement of percutaneous drain | without laparotomy!

Question 354

``` NECROTIZING ENTEROCOLITIS Is portal vein gas or pneumatosis intestinalis alone an indication for operation with NEC? P58 ```

Answer 354

No

Question 355

NECROTIZING ENTEROCOLITIS What are the indications for peritoneal tap? P58

Answer 355

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

Question 356

NECROTIZING ENTEROCOLITIS What are the possible complications? P58

Answer 356

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

Question 357

NECROTIZING ENTEROCOLITIS What is the prognosis? P58

Answer 357

>80% overall survival rate

Question 358

BILIARY TRACT What is “physiologic jaundice”? P558

Answer 358

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

Question 359

BILIARY TRACT What enzyme is responsible for conjugation of bilirubin? P558

Answer 359

Glucuronyl transferase

Question 360

``` BILIARY TRACT How is hyperbilirubinemia from “physiologic jaundice” treated? P558 ```

Answer 360

UV light

Question 361

BILIARY TRACT What is Gilbert’s syndrome? P558

Answer 361

Partial deficiency of glucuronyl transferase, leading to intermittent asymptomatic jaundice in the second or third decade of life

Question 362

BILIARY TRACT What is Crigler-Najjar syndrome? P558

Answer 362

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

Question 363

BILIARY ATRESIA What is it? P559

Answer 363

Obliteration of extrahepatic biliary tree

Question 364

BILIARY ATRESIA What is the incidence? P559

Answer 364

One in 16,000 births

Question 365

BILIARY ATRESIA What are the signs/ symptoms? P559

Answer 365

``` Persistent jaundice (normal physiologic jaundice resolves in <2 weeks), hepatomegaly, splenomegaly, ascites and other signs of portal hypertension, acholic stools, biliuria ```

Question 366

BILIARY ATRESIA What are the lab findings? P559

Answer 366

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

Question 367

``` BILIARY ATRESIA What is the classic “rule of 5s” of indirect bilirubinemia? P559 ```

Answer 367

``` Bizarre: with progressive hyperbilirubinemia, jaundice progresses by levels of 5 from the head to toes: 5 mg/dL = jaundice of head, 10 mg/ dL = jaundice of trunk, 15 mg/dL = jaundice of leg/feet ```

Question 368

BILIARY ATRESIA What is the differential diagnosis? P559

Answer 368

``` Neonatal hepatitis (TORCH); biliary hypoplasia ```

Question 369

BILIARY ATRESIA How is the diagnosis made? P559

Answer 369

``` 1. U/S to 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 ```

Question 370

BILIARY ATRESIA What is the treatment? P559

Answer 370

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

Question 371

BILIARY ATRESIA How does a Kasai work? P559

Answer 371

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

Question 372

BILIARY ATRESIA What if the Kasai fails? P560

Answer 372

Revise or liver transplantation

Question 373

BILIARY ATRESIA What are the possible postoperative complications? P560

Answer 373

``` Cholangitis (manifested as decreased bile secretion, fever, leukocytosis, and recurrence of jaundice), progressive cirrhosis (manifested as portal hypertension with bleeding varices, ascites, hypoalbuminemia, hypothrombinemia, and fat-soluble vitamin K, A, D, E deficiencies) ```

Question 374

BILIARY ATRESIA What are the associated abnormalities? P560

Answer 374

Between 25% and 30% have other anomalies, including annular pancreas, duodenal atresia, malrotation, polysplenic syndrome, situs inversus, and preduodenal portal vein; 15% have congenital heart defects

Question 375

CHOLEDOCHAL CYST What is it? P560

Answer 375

Cystic enlargement of bile ducts; most commonly arises in extrahepatic ducts, but can also arise in intrahepatic ducts

Question 376

CHOLEDOCHAL CYST What is the usual presentation? P560

Answer 376

50% present with intermittent jaundice, RUQ mass, and abdominal pain; may also present with pancreatitis

Question 377

CHOLEDOCHAL CYST What are the possible complications? P560

Answer 377

Cholelithiasis, cirrhosis, carcinoma, and | portal HTN

Question 378

``` CHOLEDOCHAL CYST What are the anatomic variants: I? P560 (picture) ```

Answer 378

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

Question 379

``` CHOLEDOCHAL CYST What are the anatomic variants: II? P561 (picture) ```

Answer 379

Lateral saccular cystic dilation

Question 380

``` CHOLEDOCHAL CYST What are the anatomic variants: III? P561 (picture) ```

Answer 380

Choledochocele represented by an | intraduodenal cyst

Question 381

``` CHOLEDOCHAL CYST What are the anatomic variants: IV? P561 (picture) ```

Answer 381

Multiple extrahepatic cysts, intrahepatic | cysts, or both

Question 382

``` CHOLEDOCHAL CYST What are the anatomic variants: V? P561 (picture) ```

Answer 382

Single or multiple intrahepatic cysts

Question 383

CHOLEDOCHAL CYST How is the diagnosis made? P562

Answer 383

U/S

Question 384

CHOLEDOCHAL CYST What is the treatment? P562

Answer 384

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

Question 385

``` CHOLEDOCHAL CYST What condition are these patients at increased risk of developing? P562 ```

Answer 385

Cholangiocarcinoma often arises in the cyst; therefore, treat by complete prophylactic resection of the cyst

Question 386

CHOLELITHIASIS What is it? P562

Answer 386

Formation of gallstones

Question 387

CHOLELITHIASIS What are the common causes in children? P562

Answer 387

``` Etiology differs somewhat from that of adults; the most common cause is cholesterol stones, but there is an increased percentage of pigmented stones from hemolytic disorders ```

Question 388

CHOLELITHIASIS What is the differential diagnosis? P562

Answer 388

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

Question 389

CHOLELITHIASIS What are the associated risks? P562

Answer 389

Use of oral contraceptives, teenage, | positive family history

Question 390

CHOLELITHIASIS What is the treatment? P562

Answer 390

Cholecystectomy is recommended for all | children with gallstones

Question 391

``` ANNULAR PANCREAS What is an annular pancreas? What is an annular pancreas? P562 ```

Answer 391

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

Question 392

ANNULAR PANCREAS What are the symptoms? P562

Answer 392

Duodenal obstruction

Question 393

ANNULAR PANCREAS What is the treatment? P562

Answer 393

Duodenoduodenostomy bypass of | obstruction (do not resect the pancreas!)

Question 394

``` TUMORS What is the differential diagnosis of pediatric abdominal mass? P563 ```

Answer 394

``` Wilms’ tumor, neuroblastoma, hernia, intussusception, malrotation with volvulus, mesenteric cyst, duplication cyst, liver tumor (hepatoblastoma/hemangioma), rhabdomyosarcoma, teratoma ```

Question 395

TUMORS WILMS’ TUMOR What is it? P563

Answer 395

Embryonal tumor of renal origin

Question 396

TUMORS WILMS’ TUMOR What is the incidence? P563

Answer 396

Very rare: 500 new cases in the United | States per year

Question 397

``` TUMORS WILMS’ TUMOR What is the average age at diagnosis? P563 ```

Answer 397

Usually between 1 and 5 years of age

Question 398

TUMORS WILMS’ TUMOR What are the symptoms? P563

Answer 398

Usually asymptomatic except for abdominal mass; 20% of patients present with minimal blunt trauma to mass

Question 399

TUMORS WILMS’ TUMOR What is the classic history? P563

Answer 399

Found during dressing or bathing

Question 400

TUMORS WILMS’ TUMOR What are the signs? P563

Answer 400

``` Abdominal mass (most do not cross the midline); hematuria (10%–15%); HTN in 20% of cases, related to compression of juxtaglomerular apparatus; signs of Beckwith-Wiedemann syndrome ```

Question 401

``` TUMORS WILMS’ TUMOR What are the diagnostic radiologic tests? P563 ```

Answer 401

Abdominal and chest CT

Question 402

``` TUMORS WILMS’ TUMOR Define the stages: Stage I P563 ```

Answer 402

Limited to kidney and completely resected

Question 403

``` TUMORS WILMS’ TUMOR Define the stages: Stage II P563 ```

Answer 403

Extends beyond kidney, but completely resected; capsule invasion and perirenal tissues may be involved

Question 404

``` TUMORS WILMS’ TUMOR Define the stages: Stage III P563 ```

Answer 404

Residual nonhematogenous tumor after | resection

Question 405

``` TUMORS WILMS’ TUMOR Define the stages: Stage IV P563 ```

Answer 405

``` Hematogenous metastases (lung, distal lymph nodes, and brain) ```

Question 406

``` TUMORS WILMS’ TUMOR Define the stages: Stage V P563 ```

Answer 406

Bilateral renal involvement

Question 407

``` TUMORS WILMS’ TUMOR What are the best indicators of survival? P564 ```

Answer 407

Stage and histologic subtype of tumor; 85% of patients have favorable histology (FH); 15% have unfavorable histology (UH); overall survival for FH is 85% for all stages

Question 408

TUMORS WILMS’ TUMOR What is the treatment? P564

Answer 408

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

Question 409

``` TUMORS WILMS’ TUMOR What is the neoadjuvant treatment? P564 ```

Answer 409

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

Question 410

``` TUMORS WILMS’ TUMOR What are the associated abnormalities? P564 ```

Answer 410

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

Question 411

``` TUMORS WILMS’ TUMOR What is the Beckwith- Wiedemann syndrome? P564 ```

Answer 411

``` Syndrome of: 1. Umbilical defect 2. Macroglossia (big tongue) 3. Gigantism 4. Visceromegaly (big organs) (Think: Wilms’ = Beckwith-Wiedemann) ```

Question 412

TUMORS NEUROBLASTOMA What is it? P564

Answer 412

Embryonal tumor of neural crest origin

Question 413

``` TUMORS NEUROBLASTOMA What are the anatomic locations? P564 ```

Answer 413

``` Adrenal medulla—50% Paraaortic abdominal paraspinal ganglia—25% Posterior mediastinum—20% Neck—3% Pelvis—3% ```

Question 414

``` TUMORS NEUROBLASTOMA With which types of tumor does a patient with Horner’s syndrome present? P564 ```

Answer 414

Neck, superior mediastinal tumors

Question 415

TUMORS NEUROBLASTOMA What is the incidence? P564

Answer 415

One in 7000 to 10,000 live births; most common solid malignant tumor of infancy; most common solid tumor in children outside the CNS

Question 416

``` TUMORS NEUROBLASTOMA What is the average age at diagnosis? P564 ```

Answer 416

≈50% are diagnosed by 2 years of age | ≈90% are diagnosed by 8 years of age

Question 417

TUMORS NEUROBLASTOMA What are the symptoms? P565

Answer 417

Vary by tumor location—anemia, failure to thrive, weight loss, and poor nutritional status with advanced disease

Question 418

TUMORS NEUROBLASTOMA What are the signs? P565

Answer 418

``` Asymptomatic abdominal mass (palpable in 50% of cases), respiratory distress (mediastinal tumors), Horner’s syndrome (upper chest or neck tumors), proptosis (with orbital metastases), subcutaneous tumor nodules, HTN (20%–35%) ```

Question 419

TUMORS NEUROBLASTOMA LABS? P565

Answer 419

24-hour urine to measure VMA, HVA, and metanephrines (elevated in 85%); neuron-specific enolase, N-myc oncogene, DNA ploidy

Question 420

``` TUMORS NEUROBLASTOMA What are the diagnostic radiologic tests? P565 ```

Answer 420

CT scan, MRI, I-MIBG, somatostatin | receptor scan

Question 421

``` TUMORS NEUROBLASTOMA What is the classic abdominal plain x-ray finding? P565 ```

Answer 421

Calcifications ( ≈50%)

Question 422

``` TUMORS NEUROBLASTOMA How do you access bone marrow involvement? P565 ```

Answer 422

Bone marrow aspirate

Question 423

``` TUMORS NEUROBLASTOMA What is the difference in position of tumors in neuroblastoma versus Wilms’ tumors? P565 (picture) ```

Answer 423

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

Question 424

TUMORS NEUROBLASTOMA What is the treatment? P565

Answer 424

Depends on staging

Question 425

``` TUMORS NEUROBLASTOMA Define the stages: Stage I P566 ```

Answer 425

Tumor is confined to organ of origin

Question 426

``` TUMORS NEUROBLASTOMA Define the stages: Stage II P566 ```

Answer 426

Tumor extends beyond organ of origin | but not across the midline

Question 427

``` TUMORS NEUROBLASTOMA Define the stages: Stage III P566 ```

Answer 427

Tumor extends across the midline

Question 428

``` TUMORS NEUROBLASTOMA Define the stages: Stage IV P566 ```

Answer 428

Metastatic disease

Question 429

``` TUMORS NEUROBLASTOMA Define the stages: Stage IVS P566 ```

Answer 429

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

Question 430

``` TUMORS NEUROBLASTOMA What is the treatment of each stage: Stage I P566 ```

Answer 430

Surgical resection

Question 431

``` TUMORS NEUROBLASTOMA What is the treatment of each stage: Stage II P566 ```

Answer 431

Resection and chemotherapy +/-- XRT

Question 432

``` TUMORS NEUROBLASTOMA What is the treatment of each stage: Stage III P566 ```

Answer 432

Resection and chemotherapy/XRT

Question 433

``` TUMORS NEUROBLASTOMA What is the treatment of each stage: Stage IV P566 ```

Answer 433

Chemotherapy/XRT → resection

Question 434

``` TUMORS NEUROBLASTOMA What is the treatment of each stage: Stage IVS P566 ```

Answer 434

In the infant with small tumor and asymptomatic = observe as many will regress “spontaneously”

Question 435

``` TUMORS NEUROBLASTOMA What is the survival rate of each stage: Stage I? P566 ```

Answer 435

≈90%

Question 436

``` TUMORS NEUROBLASTOMA What is the survival rate of each stage: Stage II? P566 ```

Answer 436

≈80%

Question 437

``` TUMORS NEUROBLASTOMA What is the survival rate of each stage: Stage III? P566 ```

Answer 437

≈40%

Question 438

``` TUMORS NEUROBLASTOMA What is the survival rate of each stage: Stage IV? P566 ```

Answer 438

≈15%

Question 439

``` TUMORS NEUROBLASTOMA What is the survival rate of each stage: Stage IVS? P566 ```

Answer 439

Survival rate is >80%! Note: these tumors are basically stage I or II with metastasis to liver, subcutaneous tissue, or bone marrow; most of these patients, if younger than 1 year of age, have a spontaneous cure (Think: Stage IVS = Special condition)

Question 440

``` TUMORS NEUROBLASTOMA What are the laboratory prognosticators? P567 ```

Answer 440

Aneuploidy is favorable! The lower the number of N-myc oncogene copies, the better the prognosis

Question 441

``` TUMORS NEUROBLASTOMA Which oncogene is associated with neuroblastoma? P567 ```

Answer 441

N-myc oncogene | Think: N-myc = Neuroblastoma

Question 442

TUMORS RHABDOMYOSARCOMA What is it? P567

Answer 442

Highly malignant striated muscle sarcoma

Question 443

TUMORS RHABDOMYOSARCOMA What is its claim to fame? P567

Answer 443

Most common sarcoma in children

Question 444

TUMORS RHABDOMYOSARCOMA What is the age distribution? P567

Answer 444

Bimodal: 1. 2–5 years 2. 15–19 years

Question 445

``` TUMORS RHABDOMYOSARCOMA What are the most common sites? P567 ```

Answer 445

1. Head and neck (40%) 2. GU tract (20%) 3. Extremities (20%)

Question 446

``` TUMORS RHABDOMYOSARCOMA What are the signs/ symptoms? P567 ```

Answer 446

Mass

Question 447

TUMORS RHABDOMYOSARCOMA How is the diagnosis made? P567

Answer 447

Tissue biopsy, CT scan, MRI, bone marrow

Question 448

``` TUMORS RHABDOMYOSARCOMA What is the treatment: Resectable? P567 ```

Answer 448

Surgical excision, +/-- chemotherapy and | radiation therapy

Question 449

``` TUMORS RHABDOMYOSARCOMA What is the treatment: Unresectable? P567 ```

Answer 449

Neoadjuvant chemo/XRT, then surgical | excision

Question 450

TUMORS HEPATOBLASTOMA What is it? P567

Answer 450

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

Question 451

``` TUMORS HEPATOBLASTOMA What is the average age at diagnosis? P567 ```

Answer 451

Presents in the first 3 years of life

Question 452

``` TUMORS HEPATOBLASTOMA What is the male to female ratio? P567 ```

Answer 452

2:1

Question 453

TUMORS HEPATOBLASTOMA How is the diagnosis made? P568

Answer 453

``` Physical exam—abdominal distention; RUQ mass that moves with respiration Elevated serum -fetoprotein and ferritin (can be used as tumor markers) CT scan of abdomen, which often predicts resectability ```

Question 454

``` TUMORS HEPATOBLASTOMA What percentage will have an elevated -fetoprotein level? P568 ```

Answer 454

≈90%

Question 455

TUMORS HEPATOBLASTOMA What is the treatment? P568

Answer 455

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

Question 456

``` TUMORS HEPATOBLASTOMA What is the overall survival rate? P568 ```

Answer 456

≈50%

Question 457

``` TUMORS HEPATOBLASTOMA What is the major difference in age presentation between hepatoma and hepatoblastoma? P568 ```

Answer 457

Hepatoblastoma presents at younger than 3 years of age; hepatoma presents at older than 3 years of age and in adolescents

Question 458

PEDIATRIC TRAUMA What is the leading cause of death in pediatric patients? P568

Answer 458

Trauma

Question 459

``` PEDIATRIC TRAUMA How are the vast majority of splenic and liver injuries treated in children? P568 ```

Answer 459

Observation (i.e., nonoperatively)

Question 460

``` PEDIATRIC TRAUMA What is a common simulator of peritoneal signs in the blunt pediatric trauma victim? P568 ```

Answer 460

Gastric distention (place an NG tube)

Question 461

``` PEDIATRIC TRAUMA How do you estimate normal systolic blood pressure (SBP) in a child? P568 ```

Answer 461

80 + 2 x age (e.g., a 5-year-old child | should have an SBP of about 90)

Question 462

``` PEDIATRIC TRAUMA What is the 20–20–10 rule for fluid resuscitation of the unstable pediatric trauma patient? P569 ```

Answer 462

First give a 20-cc/kg LR bolus followed by a second bolus of 20-cc/kg LR bolus if needed; if the patient is still unstable after the second LR bolus, then administer a 10-cc/kg bolus of blood

Question 463

PEDIATRIC TRAUMA What CT scan findings suggest small bowel injury? P569

Answer 463

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

Question 464

PEDIATRIC TRAUMA What is the treatment for duodenal hematoma? P569

Answer 464

Observation with NGT and TPN

Question 465

OTHER PEDIATRIC SURGERY QUESTIONS What is bilious vomiting in an infant? P569

Answer 465

Malrotation, until proven otherwise! (About 90% of patients with malrotation present before the first year of life)

Question 466

OTHER PEDIATRIC SURGERY QUESTIONS What does TORCHES stand for? P569

Answer 466

Nonbacterial fetal and neonatal infections: TOxoplasmosis, Rubella, Cytomegalovirus (CMV), HErpes, Syphilis

Question 467

OTHER PEDIATRIC SURGERY QUESTIONS What is the common pediatric sedative? P569

Answer 467

Chloral hydrate

Question 468

``` OTHER PEDIATRIC SURGERY QUESTIONS What are the contraindications to circumcision? P569 ```

Answer 468

Hypospadias, etc., because the foreskin might be needed for future repair of the abnormality

Question 469

OTHER PEDIATRIC SURGERY QUESTIONS When should an umbilical hernia be repaired? P569

Answer 469

>1.5 cm, after 4 years of age; otherwise observe, because most close spontaneously; repair before school age if it persists

Question 470

OTHER PEDIATRIC SURGERY QUESTIONS What is the cancer risk in the cryptorchid testicle? P569

Answer 470

>10x the normal testicular cancer rate

Question 471

OTHER PEDIATRIC SURGERY QUESTIONS When should orchidopexy be performed? P569

Answer 471

All patients with undescended testicle | undergo orchidopexy after 1 year of age

Question 472

OTHER PEDIATRIC SURGERY QUESTIONS What are some signs of child abuse? P570

Answer 472

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

Question 473

OTHER PEDIATRIC SURGERY QUESTIONS What is the treatment of child abuse? P570

Answer 473

Admit the patient to the hospital

Question 474

OTHER PEDIATRIC SURGERY QUESTIONS What is Dance’s sign? P570

Answer 474

Empty RLQ in patients with ileocecal | intussusception

Question 475

OTHER PEDIATRIC SURGERY QUESTIONS What is the treatment of hemangioma? P570

Answer 475

Observation, because most regress | spontaneously

Question 476

OTHER PEDIATRIC SURGERY QUESTIONS What are the indications for operation in hemangiomas? P570

Answer 476

Severe thrombocytopenia, congestive heart failure, functional impairment (vision, breathing)

Question 477

OTHER PEDIATRIC SURGERY QUESTIONS What are treatment options for hemangiomas? P570

Answer 477

Steroids, radiation, surgical resection, | angiographic embolization

Question 478

``` OTHER PEDIATRIC SURGERY QUESTIONS What is the most common benign liver tumor in children? P570 ```

Answer 478

Hemangioma

Question 479

OTHER PEDIATRIC SURGERY QUESTIONS What is Eagle-Barrett’s syndrome? P570

Answer 479

``` A.k.a. prune belly; congenital inadequate abdominal musculature (very lax and thin) ```

Question 480

OTHER PEDIATRIC SURGERY QUESTIONS What is the Pierre-Robin syndrome? P570

Answer 480

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

Question 481

OTHER PEDIATRIC SURGERY QUESTIONS What is the major concern with Pierre-Robin syndrome? P570

Answer 481

Airway obstruction by the tongue!

Question 482

OTHER PEDIATRIC SURGERY QUESTIONS What are the most common cancers in children? P570

Answer 482

1. Leukemia 2. CNS tumors 3. Lymphomas

Question 483

OTHER PEDIATRIC SURGERY QUESTIONS What is the most common solid neoplasm in infants? P570

Answer 483

Neuroblastoma

Question 484

OTHER PEDIATRIC SURGERY QUESTIONS What is the most common solid tumor in children? P570

Answer 484

CNS tumors

Question 485

``` OTHER PEDIATRIC SURGERY QUESTIONS What syndrome must you consider in the patient with abdominal pain, hematuria, history of joint pain, and a purpuric rash? P571 ```

Answer 485

Henoch-Schönlein syndrome; patient may also have melena (50%) or at least guaiac-positive stools (75%)

Question 486

OTHER PEDIATRIC SURGERY QUESTIONS What is Apley’s law? P571

Answer 486

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

Question 487

OTHER PEDIATRIC SURGERY QUESTIONS What is the most common cause of SBO in children? P571

Answer 487

Hernias

Question 488

OTHER PEDIATRIC SURGERY QUESTIONS What is a patent urachus? P571

Answer 488

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

Question 489

OTHER PEDIATRIC SURGERY QUESTIONS What is a “Replogle tube”? P571

Answer 489

10 French sump pump NG tube for babies (originally designed by Dr. Replogle for suction of the esophageal blind pouch of esophageal atresia)

Question 490

OTHER PEDIATRIC SURGERY QUESTIONS What are “A’s and B’s”? P571

Answer 490

Apnea and Bradycardia episodes in | babies

Question 491

OTHER PEDIATRIC SURGERY QUESTIONS What is the “double bubble” sign on AXR? P571

Answer 491

Gastric bubble and duodenal bubble on AXR; seen with duodenal obstruction (web, annular pancreas, malrotation with volvulus, duodenal atresia, etc.)

Question 492

OTHER PEDIATRIC SURGERY QUESTIONS What is Poland’s syndrome? P571

Answer 492

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

Question 493

``` OTHER PEDIATRIC SURGERY QUESTIONS What is the treatment of ATYPICAL mycobacterial lymph node infection? P571 ```

Answer 493

Surgical removal of the node

Question 494

``` OTHER PEDIATRIC SURGERY QUESTIONS What is the most common cause of rectal bleeding in infants? P571 ```

Answer 494

Anal fissure

Question 495

``` OTHER PEDIATRIC SURGERY QUESTIONS What chromosomal abnormality is associated with duodenal web/atresia/ stenosis? P572 ```

Answer 495

Trisomy 21

Question 496

``` OTHER PEDIATRIC SURGERY QUESTIONS Which foreign body past the pylorus must be surgically removed? P572 ```

Answer 496

Batteries!

Question 497

``` POWER REVIEW What is the usual age at presentation of the following conditions: Pyloric stenosis? P572 ```

Answer 497

2 weeks to 2 months

Question 498

``` POWER REVIEW What is the usual age at presentation of the following conditions: Intussusception? P572 ```

Answer 498

4 months to 2 years ( >80%)

Question 499

``` POWER REVIEW What is the usual age at presentation of the following conditions: Intussusception? P572 ```

Answer 499

1 to 5 years

Question 500

``` POWER REVIEW What is the usual age at presentation of the following conditions: Malrotation? P572 ```

Answer 500

Birth to 1 year ( >85%)

Question 501

``` POWER REVIEW What is the usual age at presentation of the following conditions: Neuroblastoma? P572 ```

Answer 501

≈50% present by 2 years; | >80% present by 8 years

Question 502

``` POWER REVIEW What is the usual age at presentation of the following conditions: Hepatoblastoma? P572 ```

Answer 502

Younger than 3 years

Question 503

``` POWER REVIEW What is the usual age at presentation of the following conditions: Appendicitis? P572 ```

Answer 503

Older than 3 years (but must be | considered at any age!)