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Surgical Recall > Pediatric Surgery > Flashcards

Pediatric Surgery Flashcards

1
Q

What is the motto of pediatric surgery?

A

Children are not little adults

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2
Q

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

A

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

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3
Q

What is the estimated blood volume of infants and children?

A

8% of body weight or 80 cc/kg

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4
Q

What is the maintenance IV fluid for children?

A

D5 1/4 NS + 20 mEq KCl

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5
Q

Why 1/4 NS?

A

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

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6
Q

How are maintenance fluid rates calculated in children?

A

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

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7
Q

What is the minimal urine output for children?

A

From 1-2 mL/kg/hr

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8
Q

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

A

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

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9
Q

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

A

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

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10
Q

What are the caloric requirements for premature infants?

A

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

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11
Q

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

A

100 kcal/kg/day

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12
Q

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

A

85 kcal/kg/day

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13
Q

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

A

70 kcal/kg/day

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14
Q

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

A

40 kcal/kg/day

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15
Q

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

A

3 g/kg/day

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16
Q

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

A

2 g/kg/day

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17
Q

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

A

2 g/kg/day

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18
Q

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

A

1.5 g/kg/day

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19
Q

How many calories are in breast milk?

A

20 kcal/30 cc

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20
Q

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

A

85 cc/kg

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21
Q

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

A

75 cc/kg

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22
Q

What is the blood volume per kg for a child?

A

70 cc/kg

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23
Q

What is the number of umbilical veins?

A

1 (usually)

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24
Q

What is the number of umbilical arteries?

A

2

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25
Q

Which umbilical vessel carries oxygenated blood?

A

Umbilical vein

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26
Q

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

A

Ductus venosus

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27
Q

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

A

Foramen ovale

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28
Q

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

A

Ductus arteriosus

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29
Q

What does the ductus venosus become?

A

Ligamentum venosum

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30
Q

What does the umbilical vein become?

A

Ligamentum teres

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31
Q

What does the umbilical artery become?

A

Medial umbilical ligament

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32
Q

What does the ductus arteriosus become?

A

Ligamentum arteriosum

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33
Q

What does the urachus become?

A

Median umbilical ligament

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34
Q

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

A

Foramen cecum

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35
Q

What does the persistent remnant of the vitelline duct become?

A

Meckel’s diverticulum

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36
Q

What is ECMO?

A

ExtraCorporeal Membrane Oxygenation:

Chronic cardiopulmonary bypass for complete respiratory support.

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37
Q

What are the types of ECMO?

A

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

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38
Q

What are the indications for ECMO?

A

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

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39
Q

What are the contraindications for ECMO?

A

Weight

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40
Q

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

A

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

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41
Q

What is a thyroglossal duct cyst?

A

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.

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42
Q

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

A

5 years

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43
Q

How is the diagnosis of thyroglossal duct cyst made?

A

U/S

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44
Q

What are the complications of a thyroglossal duct cyst?

A

Enlargement; infection; fistula formation between oropharynx or salivary glands

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45
Q

What is the anatomic location of a thyroglossal duct cyst?

A

Midline

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46
Q

What is the treatment for a thyroglossal duct cyst?

A

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

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47
Q

What is a branchial cleft cyst?

A

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

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48
Q

What is the common presentation of branchial cleft cyst

A

Infection because of communication between pharynx and external ear canal

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49
Q

What is the anatomic position of a branchial cleft cyst?

A

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

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50
Q

What is the most common cleft remnant?

A

2nd

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51
Q

What is the treatment for a branchial cleft cyst?

A

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

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52
Q

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

A

Thyroglossal: midline

Branchial cleft: lateral

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53
Q

What is stridor?

A

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

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54
Q

What are the signs and symptoms of stridor?

A

Dyspnea, cyanosis, difficulty with feeding

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55
Q

What is the differential diagnosis for stridor?

A

Laryngomalacia, tracheobronchomalacia, vascular rings and slings

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56
Q

What is laryngomalacia?

A

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

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57
Q

What are vascular rings?

A

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

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58
Q

What are the symptoms of vascular rings?

A

Stridor, dyspnea on exertion, dysphagia

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59
Q

How is the diagnosis of vascular rings made?

A

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

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60
Q

What is the treatment of vascular rings?

A

Surgical division of the ring, if the patient is symptomatic

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61
Q

What is cystic hygroma?

A

Congenital abnormality of lymph sac resulting in lymphangioma

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62
Q

What is the anatomic location of cystic hygroma?

A

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

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63
Q

What is the treatment for cystic hygroma?

A

Early total surgical removal because they tend to enlarge.

Sclerosis may be needed if the lesion is unresectable.

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64
Q

What are the possible complications of cystic hygroma?

A

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

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65
Q

Which bronchus do foreign bodies go into more commonly?

A

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

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66
Q

What is the most commonly aspirated object?

A

Peanut

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67
Q

What is the associated risk with peanut aspirations?

A

Lipoid pneumonia

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68
Q

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

A

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

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69
Q

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

A

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

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70
Q

What is the treatment of tracheal or esophageal foreign bodies?

A

Remove foreign body with rigid bronchoscope or rigid esophagoscope

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71
Q

What is the differential diagnosis of a lung mass?

A

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

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72
Q

What is the differential diagnosis of mediastinal tumor or mass?

A
  1. Neurogenic tumor (ganglioneuromas, neurofibromas)
  2. Teratoma
  3. Lymphoma
  4. Thymoma
    Rare: pheochromocytoma, hemangioma, rhabdomyosarcoma, osteochondroma
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73
Q

What heart abnormality is associated with pectus abnormality?

A

Mitral valve prolapse (many patients receive preoperative echocardiogram)

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74
Q

What is pectus excavatum?

A

Chest wall deformity with sternum caving inward

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75
Q

What is the cause of pectus excavatum?

A

Abnormal, unequal overgrowth of rib cartilage

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76
Q

What are the signs and symptoms of pectus excavatum?

A

Often asymptomatic.

Mental distress, dyspnea on exertion, chest pain.

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77
Q

What is the treatment for pectus excavatum?

A

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

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78
Q

What is the NUSS procedure?

A

Placement of metal strut to elevate sternum without removing cartilage

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79
Q

What is pectus carinatum?

A

Chest wall deformity with sternum outward.

Much less common that pectus excavatum.

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80
Q

What is the cause of pectus carinatum?

A

Abnormal, unequal overgrowth of cartilage

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81
Q

What is the treatment for pectus carinatum?

A

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

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82
Q

What is esophageal atresia?

A

Blind-ending esophagus from atresia

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83
Q

What are the signs of esophageal atresia without TEF?

A

Excessive oral secretions and inability to keep food down

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84
Q

How is the diagnosis of esophageal atresia without TEF made?

A

Inability to pass NG tube.

Plain XR shows tube coiled in upper esophagus and no gas in abdomen.

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85
Q

What is the primary treatment of esophageal atresia without TEF?

A

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

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86
Q

What is the definitive treatment of esophageal atresia without TEF?

A

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

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87
Q

What is esophageal atresia with TEF?

A

Esophageal atresia occurring with a fistula to the trachea

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88
Q

What is the incidence of esophageal atresia with TEF?

A

1:1500-3000

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89
Q

What is a type A esophageal atresia/fistula?

A

Esophageal atresia without TEF (8%)

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90
Q

What is a type B esophageal atresia/fistula?

A

Proximal esophageal atresia with proximal TEF (1%)

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91
Q

What is a type C esophageal atresia/fistula?

A

Proximal esophageal atresia with distal TEF (85%)

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92
Q

What is a type D esophageal atresia/fistula?

A

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

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93
Q

What is a type E esophageal atresia/fistula?

A

TEF without esophageal atresia (4%)

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94
Q

What are the symptoms of esophageal atresia/TEF?

A

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

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95
Q

What are the signs of esophageal atresia/TEF?

A

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

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96
Q

How is the diagnosis of esophageal atresia/TEF made?

A

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

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97
Q

What is the initial treatment of esophageal atresia/TEF?

A
  1. Suction blind pouch (NPO/TPN)
  2. Upright position of child
  3. Prophylactic antibiotics (amp/gent)
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98
Q

What is the definitive treatment for esophageal atresia/TEF?

A

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

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99
Q

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

A

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

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100
Q

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

A

Type E is high in the thorax

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101
Q

What is the workup of a patient with TEF?

A

To evaluate the TEF and associated anomalies:

CXR, AXR, U/S of kidneys, cardiac echo

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102
Q

What are associated anomalies with TEF?

A

VACTERL cluster:

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

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103
Q

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

A

No air to the stomach and, thus, no TEF

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104
Q

What is a congenital diaphragmatic hernia?

A

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

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105
Q

What is the incidence of congenital diaphragmatic hernia?

A

1:2100 live births

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106
Q

What are the types of congenital diaphragmatic hernia?

A

Bochdale: posterolateral with L > R
Morgagni: anterior parasternal

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107
Q

What are the signs of congenital diaphragmatic hernia?

A

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

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108
Q

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

A
  1. Pulmonary hypoplasia

2. Pulmonary hypertension

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109
Q

What inhaled agent is often used with congenital diaphragmatic hernia?

A

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

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110
Q

What is the treatment for congenital diaphragmatic hernia?

A

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

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111
Q

What is pulmonary sequestration?

A

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

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112
Q

What is interlobar pulmonary sequestration?

A

Sequestration in the normal lung tissue covered by normal visceral pleura

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113
Q

What is extralobar pulmonary sequestration?

A

Sequestration not in the normal lung covered by its own pleura

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114
Q

What are the signs and symptoms of pulmonary sequestration?

A

Asymptomatic, recurrent pneumonia

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115
Q

How is the diagnosis of pulmonary sequestration made?

A

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

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116
Q

What is the treatment for interlobar pulmonary sequestration?

A

Lobectomy

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117
Q

What is the treatment for extralobar pulmonary sequestration?

A

Surgical resection

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118
Q

What is the major risk during operation for pulmonary sequestration?

A

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.

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119
Q

What is the differential diagnosis of pediatric upper GI bleeding?

A

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

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120
Q

What is the differential diagnosis of pediatric lower GI bleeding?

A

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

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121
Q

What is the differential diagnosis of neonatal bowel obstruction?

A

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)

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122
Q

What is the differential diagnosis of infant constipation?

A

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

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123
Q

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

A

Indirect inguinal hernia

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124
Q

What is the most common inguinal hernia in children?

A

Indirect

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125
Q

What is an indirect inguinal hernia?

A

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

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126
Q

What is Hesselbach’s triangle?

A

Triangle formed by:

  1. Epigastric vessels
  2. Inguinal ligament
  3. Lateral border of the rectus sheath
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127
Q

What type of hernia goes through Hesselbach’s triangle?

A

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

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128
Q

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

A

3%

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129
Q

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

A

30%

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130
Q

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

A

6:1

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131
Q

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

A

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

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132
Q

What side is affected more commonly by indirect inguinal hernias?

A

Right (60%)

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133
Q

What percentage of indirect inguinal hernias are bilateral?

A

15%

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134
Q

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

A

10%

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135
Q

What are the signs and symptoms of inguinal hernia?

A

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

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136
Q

What is the silk glove sign?

A

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

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137
Q

Why should an inguinal hernia be repaired?

A

Risk of incarcerated or strangulated bowel or ovary.

Will not go away on its own.

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138
Q

How is a pediatric inguinal hernia repaired?

A

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.)

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139
Q

Which infants need overnight apnea monitoring?

A

Premature infants; infants younger than 3 months of age

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140
Q

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

A

1%

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141
Q

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

A
  1. Cut skin, then fat, then Scarpa’s fascia, then external oblique fascia through the external inguinal ring.
  2. Find hernia sac anteromedially and bluntly separate from the other cord structures.
  3. Ligate sac high at the neck at the internal inguinal ring.
  4. Resect sac and allow sac stump to retract into the peritoneal cavity.
  5. Close external oblique, then Scarpa’s fascia, then skin.
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142
Q

What is cryptorchidism?

A

Failure of the testicle to descend into the scrotum

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143
Q

What is a hydrocele?

A

Fluid-filled sac

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144
Q

What is a communicating hydrocele?

A

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

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145
Q

What is a noncommunicating hydrocele?

A

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

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146
Q

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

A

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

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147
Q

From what abdominal muscle layer is the cremaster muscle derived?

A

Internal oblique muscle

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148
Q

From what abdominal muscle layer is the inguinal ligament derived?

A

External oblique muscle

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149
Q

What nerve travels with the spermatic cord?

A

Ilioinguinal nerve

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150
Q

What 5 structures are in the spermatic cord?

A
  1. Cremasteric muscle fibers
  2. Vas deferens
  3. Testicular artery
  4. Testicular pampiniform venous plexus
  5. With or without hernia sac
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151
Q

What is the hernia sac made of?

A

Basically peritoneum or a patent processus vaginalis

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152
Q

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

A

Fossa of Geraldi

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153
Q

What attaches the testicle to the scrotum?

A

Gubernaculum

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154
Q

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

A

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.

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155
Q

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

A

Tunica vaginalis

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156
Q

What is a LIttre’s inguinal hernia?

A

Hernia with a Meckel’s diverticulum in the hernia sac

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157
Q

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

A

Adrenal rest

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158
Q

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

A

Small intestine

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159
Q

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

A

Ovary/fallopian tube

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160
Q

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

A

Round ligament

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161
Q

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

A

Anteromedially

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162
Q

What is a cord lipoma?

A

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

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163
Q

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

A

Vas is medial to the testicular vessels

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164
Q

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

A

Testicular appendage.

Should be removed with electrocautery.

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165
Q

What is the blue dot sign?

A

Blue dot on the scrotal skin from a twisted testicular appendage

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166
Q

How is a transected vas treated?

A

Repair with primary anastomosis

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167
Q

How do you treat a transected ilioinguinal nerve?

A

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

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168
Q

What happens if you cut the ilioinguinal nerve?

A

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

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169
Q

What is an umbilical hernia?

A

Fascial defect at the umbilical ring

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170
Q

What are the risk factors for umbilical hernia?

A
  1. African-American infant

2. Premature infant

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171
Q

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

A
  1. > 1.5 cm defect
  2. Bowel incarceration
  3. > 4 years
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172
Q

What is GERD?

A

GastroEsophageal Reflux Disease

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173
Q

What are the causes of GERD in children?

A

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

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174
Q

What are the signs and symptoms of GERD in children?

A

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

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175
Q

How is the diagnosis of GERD made?

A

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

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176
Q

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

A

Lipid-laden macrophages (from phagocytosis of fat)

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177
Q

What is the medical treatment of GERD?

A

H2 blockers; small meals; elevation of head

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178
Q

What are the indications for surgery for GERD in children?

A

SAFE:

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

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179
Q

What is the surgical treatment for GERD?

A

Nissen 360 degree fundoplication, +/- G tube

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180
Q

What is congenital pyloric stenosis?

A

Hypertrophy of smooth muscle of pylorus resulting in obstruction of outflow

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181
Q

What are the associated risk factors for congenital pyloric stenosis?

A

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

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182
Q

What is the incidence of congenital pyloric stenosis?

A

1:750 births
M:F = 4:1

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183
Q

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

A

Usually from 2 weeks after birth to about 2 months

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184
Q

What are the symptoms of congenital pyloric stenosis?

A

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

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185
Q

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

A

Obstruction is proximal to the ampulla of Vater

186
Q

What are the signs of congenital pyloric stenosis?

A

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

187
Q

What is the differential diagnosis for congenital pyloric stenosis?

A

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

188
Q

How is the diagnosis of congenital pyloric stenosis made?

A

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

189
Q

What is the initial treatment for congenital pyloric stenosis?

A

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

190
Q

What is the definitive treatment for congenital pyloric stenosis?

A

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

191
Q

What are the postoperative complications of pyloromyotomy?

A

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

192
Q

What is the appropriate postoperative feeding for pyloromyotomy?

A

Start feeding with Pedialyte at 6-12 hours postoperatively.

Advance to full-strength formula over 24 hours.

193
Q

Which vein crosses the pylorus?

A

Vein of Mayo

194
Q

What is duodenal atresia?

A

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

195
Q

What is the anatomic location of duodenal atresia?

A

85% are distal to the ampulla of Vater

196
Q

What are the signs of duodenal atresia?

A

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

197
Q

What is the differential diagnosis of duodenal atresia?

A

Malrotation with Ladd’s bands, annular pancreas

198
Q

How is the diagnosis of duodenal atresia made?

A

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

199
Q

What is the treatment for duodenal atresia?

A

Duodenoduodenostomy or duodenojejunostomy

200
Q

What are the associated abnormalities with duodenal atresia?

A

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

30% have trisomy 21.

201
Q

What is meconium ileus?

A

Intestinal obstruction from solid meconium concretions

202
Q

What is the incidence of meconium ileus?

A

15% of infants with CF

203
Q

What percentage of patients with meconium ileus have CF?

A

95%

204
Q

What are the signs and symptoms of meconium ileus?

A

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

205
Q

What is Neuhauser’s sign?

A

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

206
Q

How is the diagnosis of meconium ileus made?

A

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

207
Q

What is the treatment for meconium ileus?

A

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

208
Q

What is the surgical treatment for meconium ileus?

A

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

209
Q

What should you remove during all operative cases?

A

Appendix

210
Q

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

A

Pancreatic enzyme replacement

211
Q

What is cystic fibrosis?

A

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

212
Q

What is DIOS?

A

Distal Intestinal Obstruction Syndrome:

Intestinal obstruction in older patients with CF from inspissated luminal contents.

213
Q

What is meconium peritonitis?

A

Sign of intrauterine bowel perforation.

Sterile meconium leads to an intense local inflammatory reaction with eventual formation of calcifications.

214
Q

What are the signs of meconium peritonitis?

A

Calcifications on plain films

215
Q

What is meconium plug syndrome?

A

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

216
Q

What is another name for meconium plug syndrome?

A

Neonatal small left colon syndrome

217
Q

What are the signs and symptoms of meconium plug syndrome?

A

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

218
Q

What is the nonoperative treatment for meconium plug syndrome?

A

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

219
Q

What is the major differential diagnosis for meconium plug syndrome?

A

Hirschsprung’s disease

220
Q

Is meconium plug syndrome highly associated with CF?

A

No

221
Q

What are anorectal malformations?

A

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

222
Q

What is an imperforate anus?

A

Congenital absence of normal anus (complete absence or fistula)

223
Q

What is a high imperforate anus?

A

Rectum patent to level above puborectalis sling

224
Q

What is a low imperforate anus?

A

Rectum patent to below puborectalis sling

225
Q

Which type of imperforate anus is more common in women?

A

Low

226
Q

What are the associated anomalies with imperforate anus?

A

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

227
Q

What are the signs and symptoms of imperforate anus?

A

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

228
Q

How is the diagnosis of imperforate anus made?

A

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

229
Q

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

A

Dilatation of anal fistula and subsequent anoplasty

230
Q

What is the treatment for high imperforate anus?

A

Diverting colostomy and mucous fistula.

Neoanus is usually made at 1 year.

231
Q

What is Hirschsprung’s disease also known as?

A

Aganglionic megacolon

232
Q

What is Hirschsprung’s disease?

A

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

233
Q

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

A

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

234
Q

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

A

4:1

235
Q

What is the anatomic location of Hirschsprung’s disease?

A

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

236
Q

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

A

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

237
Q

What is the classic history of Hirschsprung’s disease?

A

Failure to pass meconium in the first 24 hours of life

238
Q

What is the differential diagnosis for Hirschsprung’s disease?

A

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

239
Q

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

A

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

240
Q

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

A

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

241
Q

What is the colonic transition zone?

A

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

242
Q

What is the initial treatment for Hirschsprung’s disease?

A

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

243
Q

What is a leveling colostomy?

A

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

244
Q

What is the Swenson procedure?

A

Primary anastomosis between the anal canal and healthy bowel

245
Q

What is the Duhamel procedure?

A

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

246
Q

What is the Soave procedure?

A

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

247
Q

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

A

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

248
Q

What is the prognosis for Hirschsprung’s disease?

A

Overall survival rate > 90%.

Postoperative symptoms improve with age.

249
Q

What is malrotation and midgut volvulus?

A

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

250
Q

Where is the cecum with malrotation and midgut volvulus?

A

With malrotation, the cecum usually ends up in the RUQ

251
Q

What are Ladd’s bands?

A

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

252
Q

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

A

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

253
Q

What is the usual presentation of malrotation and midgut volvulus?

A

Sudden onset of bilious vomiting

254
Q

Why is the vomiting bilious in malrotation and midgut volvulus?

A

Twist is distal to the ampulla of Vater

255
Q

How is the diagnosis of malrotation and midgut volvulus made?

A 
Upper GI contrast study (cutoff in duodenum).
Barium enema (abnormal position of cecum in the upper abdomen).
256
Q

What are the possible complications of malrotation and midgut volvulus?

A

Midgut infarction, leading to death or necessitating massive enterectomy

257
Q

What is the treatment for malrotation and midgut volvulus?

A
  1. IV antibiotics and fluid resuscitation with LR.
  2. Emergent laparotomy with Ladd’s procedure.
  3. Second-look laparotomy if bowel is severely ischemic in 24 hours to determine if remaining bowel is viable.
258
Q

What is Ladd’s procedure?

A
  1. Counterclockwise reduction of midgut volvulus.
  2. Splitting of Ladd’s bands.
  3. Division of peritoneal attachments to the cecum, ascending colon.
  4. Appendectomy.
259
Q

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

A

Counterclockwise

260
Q

Where is the cecum after midgut volvulus reduction?

A

LLQ

261
Q

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

A

Malrotation with midgut volvulus

262
Q

What is an omphalocele?

A

Defect of abdominal wall at umbilical ring.

Sac covers extruded viscera.

263
Q

How is omphalocele diagnosed prenatally?

A

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

264
Q

What comprises the sac in an omphalocele?

A

Peritoneum and amnion

265
Q

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

A

Liver

266
Q

What is the incidence of omphalocele?

A

1:5000 births

267
Q

How is the diagnosis of omphalocele made?

A

Prenatal U/S

268
Q

What are the possible complications of omphalocele?

A

Malrotation of the gut, anomalies

269
Q

What is the treatment for omphalocele?

A
  1. NG tube for decompression
  2. IV fluids
  3. Prophylactic antibiotics
  4. Surgical repair of the defect
270
Q

What is the treatment of a small omphalocele (

A

Closure of abdominal wall

271
Q

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

A

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

272
Q

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

A

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

273
Q

What are the associated abnormalities with omphalocele?

A

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

274
Q

What is the pentalogy of Cantrell?

A 
D COPS:
Diaphragmatic defect (hernia)
Cardiac abnormality
Omphalocele
Pericardium malformation/absence
Sternal cleft
275
Q

What is gastroschisis?

A

Defect of abdominal wall.

Sac does not cover extruded viscera.

276
Q

How is gastroschisis diagnosed prenatally?

A

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

277
Q

Where is the defect in gastroschisis?

A

Lateral to the umbilicus

278
Q

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

A

Right

279
Q

What is the usual size of the defect in gastroschisis?

A

2-4 cm

280
Q

What are the possible complications of gastroschisis?

A

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

281
Q

How is the diagnosis of gastroschisis made?

A

Prenatal U/S

282
Q

What is the treatment for gastroschisis?

A
  1. NG tube decompression, IV fluids (D10 LR), IV antibiotics.
  2. Surgical reduction of viscera and abdominal closure (may require staged closure with silo).
283
Q

What is a silo?

A

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

284
Q

What is the prognosis for gastroschisis?

A

> 90% survival rate

285
Q

What are the associated anomalies with gastroschisis?

A

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

286
Q

What are the major differences between gastroschisis and omphalocele?

A

No membrane coverings; uncommon associated abnormalities; lateral to umbilicus

287
Q

What is appendicitis?

A

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

288
Q

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

A

Appendicitis

289
Q

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

A

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

290
Q

What is the role of U/A in appendicitis?

A

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

291
Q

What is the hamburger sign?

A

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

292
Q

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

A

24 hours

293
Q

How long should antibiotics be administered for perforated appendicitis?

A

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

294
Q

What is intussusception?

A

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

295
Q

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

A

Intussusception

296
Q

What is the usual age of presentation for intussusception?

A

60% from 4-12 months.

80% by 2 years.

297
Q

What is the most common site for intussusception?

A

Terminal ileus involving ileocecal valve and extending into ascending colon

298
Q

What is the most common cause of intussusception?

A

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

299
Q

What are the signs and symptoms of intussusception?

A

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

300
Q

What is the intussuscipiens?

A

Recipient segment of bowel

301
Q

What is the intussusceptum?

A

Leading point or bowel that enters the intussuscipiens

302
Q

What is the treatment for intussusception?

A

Air or barium enema.

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

303
Q

What are the causes of intussusception in older patients?

A

Meckel’s diverticulum, polyps, and tumors

304
Q

What is Meckel’s diverticulum?

A

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

305
Q

What is the usual location of Meckel’s diverticulum?

A

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

306
Q

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

A

Appendicitis

307
Q

Is Meckel’s diverticulum a true diverticulum?

A

Yes

308
Q

What is the incidence of Meckel’s diverticulum?

A

2%

309
Q

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

A

2-3:1

310
Q

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

A
311
Q

What are the possible complications of Meckel’s diverticulum?

A

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

312
Q

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

A

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

313
Q

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

A

Gastric mucosa

314
Q

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

A

Enteric duplications

315
Q

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

A

Meckel’s diverticulum with ectopic gastric mucosa

316
Q

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

A

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

317
Q

What is a Meckel’s scan?

A

Scan for ectopic gastric mucosa in Meckel’s diverticulum.

Uses technetium Tc 99m pertechnetate IV, which is preferentially taken up by gastric mucosa.

318
Q

What is necrotizing enterocolitis?

A

Necrosis of intestinal mucosa, often with bleeding.

May progress to transmural intestinal necrosis, septic shock, death.

319
Q

What are the predisposing conditions for NEC?

A

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

320
Q

What is the pathophysiologic mechanism of NEC?

A

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

321
Q

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

A

NEC

322
Q

What are the signs and symptoms of NEC?

A

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

323
Q

What are the radiographic findings with NEC?

A

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

324
Q

What are the lab findings with NEC?

A

Low hematocrit, glucose, and platelets

325
Q

What is the treatment for NEC?

A
  1. Cessation of feedings
  2. OG tube
  3. IV fluids
  4. IV antibiotics
  5. Ventilator support, as needed
326
Q

What are the surgical indications for NEC?

A

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

327
Q

What is an option for bowel perforation in

A

Placement of percutaneous drain (without laparotomy)

328
Q

What are the indications for peritoneal tap for NEC?

A

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

329
Q

What are the possible complications of NEC?

A

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

330
Q

What is the prognosis for NEC?

A

> 80% survival rate

331
Q

What is physiologic jaundice?

A

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

332
Q

What enzyme is responsible for conjugation of bilirubin?

A

Glucoronyl transferase

333
Q

How is hyperbilirubinemia from physiologic jaundice treated?

A

UV light

334
Q

What is Gilbert’s syndrome?

A

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

335
Q

What is Crigler-Najjar syndrome?

A

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

336
Q

What is biliary atresia?

A

Obliteration of extrahepatic biliary tree

337
Q

What is the incidence of biliary atresia?

A

1:16,000 births

338
Q

What are the signs and symptoms of biliary atresia?

A

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

339
Q

What are the lab findings with biliary atresia?

A

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

340
Q

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

A

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

341
Q

What is the differential diagnosis for biliary atresia?

A

Neonatal hepatitis, biliary hypoplasia

342
Q

How is the diagnosis of biliary atresia made?

A
  1. U/S: Rule out choledochal cyst and to examine extrahepatic bile ducts and gallbladder.
  2. HIDA scan: Shows no excretion into the GI tract (with phenobarbital preparation).
  3. Operative cholangiogram and liver biopsy.
343
Q

What is the treatment for biliary atresia?

A

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

344
Q

What is a Kasai procedure?

A

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

345
Q

What is done if a Kasai procedure fails?

A

Revise or liver transplantation

346
Q

What are the possible postoperative complications of a Kasai procedure?

A 
Cholangitis (manifested as decreased bile secretion, fever, leukocytosis, recurrence of jaundice).
Progressive cirrhosis (manifested as portal hypertension with bleeding varices, ascites, hypoalbuminemia, hypothrombinemia, and fat-soluble vitamin deficiencies).
347
Q

What are the associated abnormalities for biliary atresia?

A

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

348
Q

What is a choledochal cyst?

A

Cystic enlargement of bile ducts.

Most commonly arises in extrahepatic ducts, but can also arise in intrahepatic ducts.

349
Q

What is the usual presentation of a choledochal cyst?

A

50% with intermittent jaundice, RUQ mass, abdominal pain

350
Q

What are the possible complications of a choledochal cyst?

A

Cholelithiasis, cirrhosis, carcinoma, portal hypertension

351
Q

What is a type I choledochal cyst?

A

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

352
Q

What is a type II choledochal cyst?

A

Lateral saccular cystic dilation

353
Q

What is a type III choledochal cyst?

A

Choledochocele represented by an intraduodenal cyst

354
Q

What is a type IV choledochal cyst?

A

Multiple extrahepatic cysts, intrahepatic cysts, or both

355
Q

What is a type V choledochal cyst?

A

Single or multiple intrahepatic cysts

356
Q

How is the diagnosis of choledochal cyst made?

A

U/S

357
Q

What is the treatment for a choledochal cyst?

A

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

358
Q

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

A

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

359
Q

What is cholelithiasis?

A

Formation of gallstones

360
Q

What are the common causes of cholelithiasis in children?

A

Cholesterol stones, pigmented stones (from hemolytic disorders)

361
Q

What is the differential diagnosis of cholelithiasis in children?

A

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

362
Q

What are the associated risk factors for cholelithiasis?

A

Use of OCPs, teenage, positive family history

363
Q

What is the treatment for cholelithiasis?

A

Cholecystectomy

364
Q

What is an annular pancreas?

A

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

365
Q

What are the symptoms of annular pancreas?

A

Duodenal obstruction

366
Q

What is the treatment for annular pancreas?

A

Duodenoduodenostomy bypass of obstruction

367
Q

What is the differential diagnosis of pediatric abdominal mass?

A

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

368
Q

What is Wilms’ tumor?

A

Embryonal tumor of renal origin

369
Q

What is the incidence of Wilms’ tumor?

A

Rare; 500 cases in US per year

370
Q

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

A

1-5 years

371
Q

What are the symptoms of Wilms’ tumor?

A

Abdominal mass

372
Q

What is the classic history for Wilms’ tumor?

A

Found during bathing or dressing

373
Q

What are the signs of Wilms’ tumor?

A

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

374
Q

What are the diagnostic radiologic tests for Wilms’ tumor?

A

Abdominal and chest CT

375
Q

What is stage I Wilms’ tumor?

A

Limited to kidney and completely resected

376
Q

What is stage II Wilms’ tumor?

A

Extends beyond kidney, but completely resected.

Capsule invasion and perirenal tissues may be involved.

377
Q

What is stage III Wilms’ tumor?

A

Residual non-hematogenous tumor after resection

378
Q

What is stage IV Wilms’ tumor?

A

Hematogenous metastases (lung, distal lymph nodes, brain)

379
Q

What is stage V Wilms’ tumor?

A

Bilateral renal involvement

380
Q

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

A

Stage and histologic subtype of tumor

381
Q

What is the treatment for Wilms’ tumor?

A

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

382
Q

What is the neoadjuvant treatment for Wilms’ tumor?

A

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

383
Q

What are the associated abnormalities with Wilms’ tumor?

A

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

384
Q

What is Beckwith-Wiedemann syndrome?

A
  1. Umbilical defect
  2. Macroglossia
  3. Gigantism
  4. Visceromegaly
385
Q

What is neuroblastoma?

A

Embryonal tumor of neural crest origin

386
Q

What are the anatomic locations of neuroblastomas?

A

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

387
Q

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

A

Neck, superior mediastinal tumors

388
Q

What is the incidence of neuroblastoma?

A

1:7,000-10,000 births

389
Q

What is the most common solid malignant tumor of infancy?

A

Neuroblastoma

390
Q

What is the average age of diagnosis of neuroblastoma?

A

50% by 2 years

90% by 8 years

391
Q

What are the symptoms of neuroblastoma?

A

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

392
Q

What are the signs of neuroblastoma?

A

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

393
Q

What are the lab tests are performed for neuroblastoma?

A

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

394
Q

What are the diagnostic radiologic tests for neuroblastoma?

A

CT, MRI, I-MIBG, somatostatin receptor scan

395
Q

What is the classic AXR finding with neuroblastoma?

A

Calcifications

396
Q

How do you assess bone marrow involvement with neuroblastoma?

A

Bone marrow aspirate

397
Q

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

A

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

398
Q

What is stage I neuroblastoma?

A

Confined to organ of origin

399
Q

What is stage II neuroblastoma?

A

Tumor extends beyond organ of origin but not across the midline

400
Q

What is stage III neuroblastoma?

A

Tumor extends across the midline

401
Q

What is stage IV neuroblastoma?

A

Metastatic disease

402
Q

What is stage IVS neuroblastoma?

A

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

403
Q

What is the treatment for stage I neuroblastoma?

A

Surgical resection

404
Q

What is the treatment for stage II neuroblastoma?

A

Resection and chemotherapy +/- XRT

405
Q

What is the treatment for stage III neuroblastoma?

A

Resection and chemotherapy, XRT

406
Q

What is the treatment for stage IV neuroblastoma?

A

Chemotherapy, XRT followed by resection

407
Q

What is the treatment for stage IVS neuroblastoma?

A

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

408
Q

What is the survival rate of neuroblastoma?

A 
I:  90%
II:  80%
III:  40%
IV:  15%
IVS:  > 80%
409
Q

What are the lab prognosticators for neuroblastoma?

A

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

410
Q

Which oncogene is associated with neuroblastoma?

A

N-myc

411
Q

What is the most common sarcoma in children?

A

Rhabdomyosarcoma

412
Q

What is the age distribution for rhabdomyosarcoma?

A

Bimodal:

  1. 2-5 years
  2. 15-19 years
413
Q

What are the most common sites for rhabdomyosarcoma?

A
  1. Head and neck
  2. GU tract
  3. Extremities
414
Q

What are the signs and symptoms of rhabdomyosarcoma?

A

Mass

415
Q

How is the diagnosis of rhabdomyosarcoma made?

A

Tissue biopsy, CT, MRI, bone marrow

416
Q

What is the treatment for resectable rhabdomyosarcoma?

A

Surgical excision +/- chemotherapy and XRT

417
Q

What is the treatment for unresectable rhabdomyosarcoma?

A

Neoadjuvant chemotherapy, XRT, then surgical excision

418
Q

What is hepatoblastoma?

A

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

419
Q

What is the average age at diagnosis for hepatoblastoma?

A
420
Q

What is the male:female ratio for hepatoblastoma?

A

2:1

421
Q

How is the diagnosis of hepatoblastoma made?

A

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

422
Q

What percentage of hepatoblastomas will have an elevated AFP level?

A

90%

423
Q

What is the treatment for hepatoblastoma?

A

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

424
Q

What is the overall survival rate for hepatoblastoma?

A

50%

425
Q

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

A

Hepatoblastoma presents at 3 years.

426
Q

What is the leading cause of death in pediatric patients?

A

Trauma

427
Q

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

A

Observation

428
Q

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

A

Gastric distention

429
Q

How do you estimate normal systolic BP in a child?

A

80 + 2*age

430
Q

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

A
  1. 20-cc/kg LR bolus, then
  2. 20-cc/kg LR bolus, if still unstable, then
  3. 10-cc/kg of blood, if still unstable
431
Q

What CT findings suggest small bowel injury?

A

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

432
Q

What is the treatment for duodenal hematoma?

A

Observation with NGT and TPN

433
Q

What does TORCHES stand for?

A 
Nonbacterial fetal and neonatal infections:
TOxoplasmosis
Rubella
Cytomegalovirus
HErpes
Syphilis
434
Q

What is the common pediatric sedative?

A

Chloral hydrate

435
Q

What are the contraindications to circumcision?

A

Hypospadias (foreskin may be needed for future repair)

436
Q

When should an umbilical hernia be repaired?

A

> 1.5 cm, after 4 years of age

437
Q

What is the cancer risk in the cryptorchid testicle?

A

> 10-fold

438
Q

When should orchidopexy be performed?

A

All patients with undescended testicle undergo orchidopexy after 1 year

439
Q

What are some signs of child abuse?

A

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

440
Q

What is the treatment of child abuse?

A

Admit the patient to the hospital

441
Q

What is Dance’s sign?

A

Empty RLQ in patients with ileocecal intussusception

442
Q

What is the treatment of hemangioma?

A

Observation, because most regress spontaneously

443
Q

What are the indications for operation in hemangiomas?

A

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

444
Q

What are treatment options for hemangiomas?

A

Steroids, radiation, surgical resection, angiographic embolization

445
Q

What is the most common benign liver tumor in children?

A

Hemangioma

446
Q

What is Eagle-Barrett’s syndrome?

A

Congenital inadequate abdominal musculature (also known as prune belly)

447
Q

What is the Pierre-Robin syndrome?

A
  1. Big, protruding tongue (glossoptosis)
  2. Small mandible (micrognathia)
  3. Cleft palate
448
Q

What is the major concern with Pierre-Robin syndrome?

A

Airway obstruction by the tongue

449
Q

What are the most common cancers in children?

A
  1. Leukemia
  2. CNS tumors
  3. Lymphomas
450
Q

What is the most common solid tumor in children?

A

CNS tumors

451
Q

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

A

HSP

452
Q

What is Apley’s law?

A

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

453
Q

What is the most common cause of SBO in children?

A

Hernias

454
Q

What is a patent urachus?

A

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

455
Q

What is a Replogle tube?

A

10 French sump pump NG tube for babies

456
Q

What is Poland’s syndrome?

A

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

457
Q

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

A

Surgical removal of the node

458
Q

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

A

Anal fissure

459
Q

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

A

Trisomy 21

460
Q

Which foreign body past the pylorus must be surgically removed?

A

Battery

Surgical Recall (68 decks)

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