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ABSITE > 43 Pediatrics > Flashcards

43 Pediatrics Flashcards

1
Q

foregut

A

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

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2
Q

midgut

A

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

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3
Q

hindgut

A

distal 1/3 of t colon to anal canal

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4
Q

rotation of gut during embryonic development

A

midgut rotation 270 degrees counterclockwise normally

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5
Q

define low birth weight

A

<2500 g

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6
Q

define premature

A

< 37 weeks

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7
Q

immunity at birth

A

IgA from mom’s milk … IgG crosses placenta

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8
Q

1 cause of childhood death

A

trauma

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9
Q

trauma bolus in kids

A

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

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10
Q

best indicator of shock

A

tachycardia …. neonat >150, <1yr >120, rest >100

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11
Q

goal UOP in trauma

A

> 2-4 cc/kg/hr

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12
Q

renal function in kids

A

kids <6mo only have 25% GFR cpacity of adults, poor concentrating ability

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13
Q

mechanism of bone growth in kids

A

inc alk phos in kids relative to adults

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14
Q

umbilical vessels

A

2 arteries, 1 vein

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15
Q

mIVF amounts

A

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

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16
Q

congenital cystic disease of the lung: list types

A

pulmonary sequestration, congenital lobar overinflation (emphysema), congenital cystic adenoid malformation, bronchiogenic cyst

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17
Q

congenital cystic disease of the lung: pulmonary sequestration - describe

A

lung tissue has anomalous systemic arterial supply (via thoracic aorta or abdominal aorta through inferior pulmonary ligament), have either systemic venous or pulmonary vein drainage

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18
Q

congenital cystic disease of the lung: pulmonary sequestration - locations

A

extra-lobar = more likely to have systemic venous drainage (azygous system) …. vs … intra-lobar = more likely to have pulmonary vein drainage

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19
Q

congenital cystic disease of the lung: pulmonary sequestration - px

A

does NOT communicate with tracheobronchial tree … MC p/w infection, can also have resp compromise or abnormal CXR … tx w lobectomy

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20
Q

congenital cystic disease of the lung: congenital lobar overinflation (emphysema) - pathophys

A

cartilage fails to develop in bronchus —> air trapping with expiration … vascular supply and other lobes are normal (except to the extent to which they are compressed by the hyperinflated lung) … can develop hemodynamic instability (same mech as tension PTX) or respiratory compromise

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21
Q

congenital cystic disease of the lung: congenital lobar overinflation (emphysema) - MC lobe affected

A

LUL

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22
Q

congenital cystic disease of the lung: congenital lobar overinflation (emphysema) - tx

A

lobectomy

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23
Q

congenital cystic disease of the lung: congenital cystic adenoid malformation - pathophys

A

communicates with airway, alveolar structure is poorly developed, although lung tissue is present

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24
Q

congenital cystic disease of the lung: congenital cystic adenoid malformation - sx

A

respiratory compromise or recurrent infection

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25
Q

congenital cystic disease of the lung: congenital cystic adenoid malformation - tx

A

lobectomy

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26
Q

congenital cystic disease of the lung: bronchiogenic cyst - describe

A

MC cysts of the mediastinum, usually posterior to the carina, extra-pulmonary cysts formed from bronchial tissue and cartilage wall

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27
Q

congenital cystic disease of the lung: bronchiogenic cyst - px

A

usually p/w mediastinal mass filled with milky liquid, can compress adjacent structures or become infected, have malignant potential, occasionally are intra-pulmonary

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28
Q

congenital cystic disease of the lung: bronchiogenic cyst - tx

A

resect cyst

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29
Q

mediastinal masses in children: MC

A

neurogenic tumors (neurofibroma, neuroganglioma, neurobalstoma) are the MC mediastinal tumors in kids, usually located posteriorly

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30
Q

mediastinal masses in children: px

A

respiratory sx, dysphagia - common to all mediastinal masses, regardless of location

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31
Q

mediastinal masses in children: list regions

A

anterior, middle, posterior

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32
Q

mediastinal masses in children: anterior masses

A

T cell lymphoma, teratoma, other germ cell tumors (MC anterior mediastinal mass in kids), thyroid CA

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33
Q

mediastinal masses in children: middle masses

A

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

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34
Q

mediastinal masses in children: posterior

A

T cell lymphoma, neuroblastoma, neurogenic tumor

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35
Q

mediastinal masses in children: thymoma

A

RARE in kids

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36
Q

choledochal cyst: casued by what?

A

reflux of pancreatic enzymes int the biliary system

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37
Q

choledochal cyst: inc risk for what?

A

cholangiocarcinoma, pancreatitis, cholangitis, obstructive jaundice

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38
Q

choledochal cyst: mgmt

A

need to resect

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39
Q

choledochal cyst: list types and frequencies

A

1 = 85%, 2 = 3%, 3 = 1%, 4 = 10%, 5 = 1%

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40
Q

choledochal cyst: rate, description, tx - type 1

A

85% …. fusiform dilation of entire CBD, mildly dilated common hepatic duct, normal intrahepatic ducts … tx w resection, hepaticojejunostomy

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41
Q

choledochal cyst: rate, description, tx - type 2

A

3% …. true diverticulum that hangs off CBD … resection off CBD, may be able to preserve CBD and avoid hepaticojejunostomy

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42
Q

choledochal cyst: rate, description, tx - type 3

A

1% …. dilation of distal intramural CBD, invovled sphincter of Oddi … resection, choledochojejunostomy

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43
Q

choledochal cyst: rate, description, tx - type 4

A

10% … multiple cysts, both intrahepatic and extrahepatic … resection, may need liver lobectomy, possible TXP

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44
Q

choledochal cyst: rate, description, tx - type 5

A

1% … caroli’s disease - intrahepatic cysts, get hepatic fibrosis, may be assoc with congenital hepatic fibrosis and medullary sponge kidney … resection, may need lobectomy, possible liver txp

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45
Q

lymphadenopathy: MC cause

A

acute suppurative adenitis assoc with URI or pharyngitis

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46
Q

lymphadenopathy: mgmt if fluctuant

A

FNA, culture and sensitivity, and abx … may need I&D if fails to resolve

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47
Q

lymphadenopathy: chronic causes

A

cat scratch fever, atypical mycoplasma (often fluctuant)

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48
Q

lymphadenopathy: mgmt if a-sx

A

abx x10d —> excisional bx if not improvement … dx is lymphoma until proven otherwise

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49
Q

lymphadenopathy: cystic hygroma

A

aka lymphangioma, usually found in lateral cervical regions in neck, gets infected, is usually lateral to SCM muscle, tx w resection

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50
Q

diaphragmatic hernia and chest wall: overall survival rate

A

50%

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51
Q

diaphragmatic hernia and chest wall: px

A

80% on L side … can have severe pulm HTN …. 80% have assoc anomalies (cardiac and neural tube defects mostly, malrotation)

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52
Q

diaphragmatic hernia and chest wall: dx

A

can be made with prenatal U/S

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53
Q

diaphragmatic hernia and chest wall: sx

A

respiratory distress

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54
Q

diaphragmatic hernia and chest wall: CXR

A

bowel in chest

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55
Q

diaphragmatic hernia and chest wall: tx

A

high frequency ventilation, inhaled nitric oxide, may need ECMO … stabilize before OR …. need to reduce bowel and repair defect +/- mesh (abdominal approach) … look for visceral anomalies (run the bowel)

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56
Q

Bochdalek’s vs Morgagni’s hernia

A

Bochdalek’s = MC, located posteriorly …. Morgagni’s = rare, located anteriorly

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57
Q

pectus excavatum vs carinatum

A

excavatum = sinks in, sternal osteotomy, need strut, performed if causing respiratory sx or emotional stree … carinatum = pigeon chest, strut not necessary, repair for emotional stress

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58
Q

branchial cleft cysts: lead to what

A

cysts, sinuses, fistulas

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59
Q

branchial cleft cysts: types, MC

A

1st, 2nd, 3rd branchial cleft cysts … 2nd = MC

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60
Q

1st branchial cleft cysts

A

angle of the mandible, may connect with external auditory canal, often assoc with facial nerve

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61
Q

2nd branchial cleft cysts

A

MC, on anterior border of mid-SCM muscle, goes through carotid bifurcation into tonsillar pillar

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62
Q

3rd branchial cleft cysts

A

lower neck, medial to or through the lower SCM

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63
Q

branchial cleft cysts: tx

A

resection

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64
Q

branchial cleft cysts: px

A

sinuses and fistulas usually in infants and young kids … cysts usually appear at later age

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65
Q

thyroglossal duct cyst: pathophys, px, tx

A

from the descent of the thyroid gland from the foramen cecum, may be only thyroid tissue pt has, presents as a midline cervical mass, goes through the hyoid bone, tx w excision of cyst, tract, and hyoid bone (at least central portion)

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66
Q

hemangioma: px, tx

A

appears at birth or shortly after, rapid growth during first 6-12 months of life, then begins to involute … tx = observation, most resolve by age 7-8 … if lesion has uncontrollable growth, impairs function (eyelid or ear canal) or is persistent after age 8 - can tx w oral steroids –> laser or resection if steroids are not successful

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67
Q

1 solid abdominal malignancy in kids

A

neuroblastoma

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68
Q

CT of neuroblastoma vs nephroblastoma

A

displacement vs replacement

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69
Q

neuroblastoma: px

A

a-sx abd mass in kids (MC) … can also have secretory diarrhea, raccoon eyes (orbital mets), HTN, and opsomyoclonus syndrome (unsteady gait)

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70
Q

neuroblastoma: location

A

MC is adrenal, can occur anywhere along sympathetic chain

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71
Q

neuroblastoma: age

A

MC in 1st 2 years of life

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72
Q

neuroblastoma: prognosis

A

best in kids <1yr

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73
Q

neuroblastoma: labs

A

inc catecholamines, VMA, HVA, metanephrines (HTN)

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74
Q

neuroblastoma: cell of origin

A

neural crest cells

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75
Q

neuroblastoma: vascular involvement

A

encases vasculature rather than invading

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76
Q

neuroblastoma: mets

A

rare, goes to lung and bone

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77
Q

neuroblastoma: abd xray findings

A

may show stippled calcifications in the tumor

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78
Q

neuroblastoma: worse prognosis

A

NSE, LDH, HVA, diploid tumors, N-myc amplification (>3 copies)

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79
Q

neuroblastoma: seen in pts with mets

A

NSE increase

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80
Q

neuroblastoma: tx

A

resection (adrenal gland and kidney), 40% cure rate … intially unresectable tumors may be resectable after doxorubicin-based chemo

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81
Q

neuroblastoma: staging

A

1 = localized, complete excision …. 2 = incomplete excision but does not cross midline … 3 = crosses midline +/- regional nodes …. 4 = distant mets (nodes or solid organ) … 4s = localized tumor with distant mets

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82
Q

Wilms tumor: aka

A

nephroblastoma

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83
Q

Wilms tumor aka nephroblastoma: px

A

usually px as a=sx mass, can have hematuria or HTN, 10% b/l

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84
Q

Wilms tumor aka nephroblastoma: mean age at dx

A

3yo

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85
Q

Wilms tumor aka nephroblastoma: prognosis

A

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

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86
Q

Wilms tumor aka nephroblastoma: mets

A

frequent, to bone and lung

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87
Q

Wilms tumor aka nephroblastoma: mgmt of pulm mets

A

resect if resectable

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88
Q

Wilms tumor aka nephroblastoma: abd CT findings

A

replacement of renal parenchyma and NOT displacement (differentiates from neuroblastoma)

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89
Q

Wilms tumor aka nephroblastoma: tx

A

nephrectomy (90% cure rate) … if venous extension occurs in the renal vein, the tumor can be extracted from the vein … need to examine the contralateral kidney and look for peritoneal implants … avoid rupture of tumor with resection (this would increase stage) …. actinomycin and vincristine based chemo in all unless stage 1 and <500g tumor

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90
Q

Wilms tumor aka nephroblastoma: staging

A

1 = limited to kidney, complete excised … 2 = beyond kidney but completely excised … 3 = residual nonhematogenous tumor … 4 = hematogenous mets … 5 = b/l renal involvement

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91
Q

MC malignant tumor in kids

A

hepatoblastoma

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92
Q

hepatoblastoma: labs

A

inc AFP in 90%

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93
Q

hepatoblastoma: px

A

fractures, precocious puberty (from beta-hcg release)

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94
Q

hepatoblastoma: prognosis

A

better than HCC

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95
Q

hepatoblastoma: describe mass

A

can be pedunculated, vascular invasion is common

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96
Q

hepatoblastoma: tx

A

resection optimal, o/w doxorubicin and cisplatin based chemo –> may downstage tumors and make them resectable

97
Q

hepatoblastoma: survival

A

primarily related to resectability

98
Q

hepatoblastoma: best prognosis

A

fetal histology

99
Q

1 kids malignancy overall

A

leukemia (ALL)

100
Q

1 solid tumor class

A

CNS tumors

101
Q

1 general surgery tumor in kids … in kids <2yo … in kids >2yo

A

neuroblastoma …. neuroblastoma … Wilms tumor

102
Q

1 cause of duodenal obstruction in newborns (<1week) vs after newborn period (>1week) and overall

A

duodenal atresia … malrotation

103
Q

1 cause of colon obstruction

A

Hirschsprung’s disease

104
Q

1 liver tumor in kids

A

hepatoblastoma … 2/3 of liver tumors in kids are malignant

105
Q

1 lung tumor in kids

A

carcinoid

106
Q

painful vs painless lower GI bleeding

A

painful = anorectal lesions (i.e. fissues, etc) … painless = Meckel’s

107
Q

upper GI bleeding 0-1yo vs 1yo-adult

A

gastritis, esophagitis … esophageal varcies, esophagitis

108
Q

meckel’s diverticulum: location

A

antimesenteric border of small bowel

109
Q

meckel’s diverticulum: embyrology

A

persistent vitelline duct

110
Q

meckel’s diverticulum: rule of 2s

A

2 feet from ileocecal valve, 2% of population, 2% symptomatic, 2 tissue types (pancreatic MC, gastric most liklely to be sympomatic), and 2 presentations (diverticulitis and bleeding)

111
Q

meckel’s diverticulum: #1 cause of

A

painless lower GI bleeding in kids

112
Q

meckel’s diverticulum: workup

A

can get Meckel’s scan with pertechnetate if suspicious of diverticulum but having hard time locating

113
Q

meckel’s diverticulum: tx

A

resection with sx, suspicion of gastric mucosa, narrow neck … diverticulitis involving base or if the base if >1/3 the size of the bowel, need to perform segmental resection

114
Q

pyloric stenosis: typical pt

A

firstborn M, 3-12 weeks

115
Q

pyloric stenosis: px

A

projectile vomiting … get hypoCl, hypoK metabolic alkalosis … feel olive mass in stomach

116
Q

pyloric stenosis: U/S findings

A

pylorus >=4mm thick, >=14mm long

117
Q

pyloric stenosis: fluid mgmt - boluses, what to avoid, infants

A

severe dehydration —> resuscitate with NS boluses until making urine —> switch to D5 NS with 10mEq K for maintenance …. AVOID fluid resus with K containing fluids in kids with severe dehydration b/c hyperK develops quickly, AVOID non-alt containing solutions in infants b/c hypoNa can quickly develop …. infants should always have a maintenance fluid with glucose b/c of their limited reserves for gluconeogenesis and vulnerability for hypoglycemia

118
Q

pyloric stenosis: tx

A

pyloromyotomy = RUQ incision, proximal extent should be the circular muscles of the stomach

119
Q

intussusception: typical age

A

3mo to 3yrs

120
Q

intussusception: px

A

currant jelly stools (from vascular congestion, NOT an indication for resection), sausage mass, abdominal distention, RUQ pain, vomiting

121
Q

intussusception: pathophys

A

invagination of one loop of intestine into another … lead points in kids = enlarged peyer’s patches (#1), lymphoma, Meckel’s diverticulum

122
Q

intussusception: recurrence rate

A

15% after reduction

123
Q

intussusception: tx

A

reduce with air contrast enema, 80% successful and no surgery needed if reduced … if fails, peritonitis present, or free air present, then go to OR

124
Q

intussusception: enema characeristics

A

max P with air contrast enema is 120mmHg … max column height with barium enema is 1meter (3ft) … high perforation risk beyond

125
Q

intussusception: surgical indications

A

peritonitis, free air, unable to reduce

126
Q

intussusception: surgical technique

A

when reducing in OR, do NOT place traction on proximal limb of bowel - need to apply P to distal limb …. usually NO resection required unless associated with lead point (Meckel’s)

127
Q

intussusception: mgmt if px in adults

A

most likely malignant lead point (i.e. colon CA in cecum) —> OR for resection

128
Q

intestinal atresia: caused by what, sx, location, mgmt and tx

A

develop 2/2 intrauterine vascular accidents … sx = bilious emesis, distention, most do NOT pass meconium … MC in jejunum, can be multiple … get rectal bx to r/o Hirschsprung’s preop … tx w resection

129
Q

1 cause of duodenal obstruction in newborns

A

duodenal atresia

130
Q

duodenal atresia: location, px

A

usually distal to ampulla of vater … p/w bilious emesis, feeding intolerance

131
Q

duodenal atresia: associated with what

A

polyhydramnios in mom … cardiac, renal, other GI anomalies … 20% with Down’s syndrome (check chromosomal studies)

132
Q

duodenal atresia: xray

A

abd xr shows double-bubble sign

133
Q

duodenal atresia: tx

A

resuscitation, duodenoduodenostomy or duodenojejunostomy

134
Q

tracheoesophageal fistulas (TEF): MC type, 2nd MC type

A

type C = 80% = proximal esophageal atresia (blind pouch) and distal TE fistula … type A = 5% = esophageal atresia and no fistula

135
Q

tracheoesophageal fistulas (TEF): type C - describe, sx, imaging

A

MC type (80%), proximal esophageal atresia (blind pouch) and distal TE fistula … sx = newborn spits up feed, excessive drooling, respiratory sx with feeding, cannot place NGT in stomach … abd xray = distended, gas-filled stomach

136
Q

tracheoesophageal fistulas (TEF): type A - describe, sx, imaging

A

2nd MC type (5%), esophageal atresia and no fistula … sx similar to type C = newborn spits up, XS drooling, resp sx w feeds, can’t place NGT in stomach … abd xray = gasless abdomen

137
Q

tracheoesophageal fistulas (TEF): tx

A

R extrapleural thoracotomy for most, perform primary repair, placed G tube, azygous vein often needs to be divided

138
Q

tracheoesophageal fistulas (TEF): mgmt of infants that are premature, <2500g, or sick

A

replogle tube, treat resp sx, place G tube, delay repair

A Replogle tube is a medical device used in the treatment of babies with oesophageal atresia. It is a double-lumen tube which is inserted through the baby’s nostril into the blind-ending oesophageal pouch and used to drain saliva

139
Q

tracheoesophageal fistulas (TEF): complications of repair

A

GERD, leak, empyema, stricture, fistula

140
Q

tracheoesophageal fistulas (TEF): survival related to what

A

birth weight, assoc anomalies

141
Q

describe VACTERL syndrome

A

Vertebral, Anorectal (imperforate anus), Cardiac, TE fistula, Radius/renal, Limb anomalies

142
Q

malrotation: px

A

sudden onset bilious vomiting (Ladd’s bands cause duod obstruction, coming out from the R retroperitoneum)

143
Q

malrotation: pathophys

A

assoc with compromise of SMA –> infarct of the intestine …. failure of normal COUNTERclockwise rotation (270 degrees) … Ladd’s bands cause duo obstruction, coming out from the R retroperitoneum

144
Q

malrotation: age at presentation

A

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

145
Q

malrotation: dx

A

UGI - duo does NOT cross midline, duo-jej junction displaced to the R

146
Q

malrotation: tx

A

resect Ladd’s bands, counterclockwise rotation (may require multiple turns), place cecum in LLQ (cecopexy), place duo in RUQ, and appendectomy

147
Q

meconium ileus: leads to what

A

distal ileal obstruction, abd distention, bilious vomiting, distended loops of bowel

148
Q

meconium ileus: assoc with what

A

CF - occurs in 10% of children with CF

149
Q

meconium ileus: workup

A

need sweat chloride test or PCR for Cl channel defect (b/c of frequency of occurrence with CF)

150
Q

meconium ileus: imaging

A

abd xr: dilated loops of small bowel without air-fluid levels - b/c meconium is too thick to separate from the bowel wall, can have ground glass or soapsuds appearance

151
Q

meconium ileus: complications

A

can cause perforation, leading to meconium pseudocyst or free perforation —> requires laparotomy

152
Q

meconium ileus: tx

A

gastrografin enema (effective in 80%), can also make the dx and potentially treat the pt … can also use N-acetylcysteine enema … if surgery required, manueal decompression and create a vent for N-acetylcysteine antegrade enemas

153
Q

necrotizing enterocolitis (NEC): px

A

bloody stools after 1st feeding in premature neonate

154
Q

necrotizing enterocolitis (NEC): risk factors

A

prematurity, hypoxia, sepsis

155
Q

necrotizing enterocolitis (NEC): sx

A

lethargy, resp decompensation, abd distention, vomiting, blood per rectum

156
Q

necrotizing enterocolitis (NEC): imaging

A

abd xr may show pneumatosis intestinalis, free air, or portal vein air … need serial lateral decubitus films to look for perforation

157
Q

necrotizing enterocolitis (NEC): initial tx

A

resuscitation, NPO, abx, TPN, orogastric tube

158
Q

necrotizing enterocolitis (NEC): surgical indications

A

free air, peritonitis, clinical deterioration

159
Q

necrotizing enterocolitis (NEC): surgical approach

A

resect dead bowel and bring up ostomies … need Ba contrast enema before taking down ostomy to r/o distal obstruction from stenosis

160
Q

necrotizing enterocolitis (NEC): mortality rate

A

10%

161
Q

congenital vascular malformation: surgical indications, tx

A

surgery for hemorrhage, ischemia, CHF, nonbleeding ulcers, functional impairment, limb-length discrepancy … tx w embolization (may be sufficient on its own) and/or resection

162
Q

imperforate anus: MC patients

A

M

163
Q

imperforate anus: workup

A

check for associated VACTERL anomalies (vertebral, cardiac, TE fistula, Radius/Renal, Limb)

164
Q

imperforate anus: types

A

high (above levators) … low (below levators)

165
Q

imperforate anus: high type - px, tx

A

high = above levators … meconium in urine or vagina (fistula to bladder/vagina/prostatic urethra) … tx w colostomy, later anal reconstruction with posterior sagittal anoplasty (pull anus down into sphincter mechanism) … need postop anal dilatation to void structure, these pts are prone to constipation

166
Q

imperforate anus: low type - px, tx

A

low = below levators … meconium to perineal skin … perform posterior sagittal anoplasty (pull anus down into sphincter mechanism), NO colostomy needed … need postop anal dilatation to void structure, these pts are prone to constipation

167
Q

imperforate anus: px, tx - high vs low

A

high = above levators, low = below … high = mec in urine or vagina (fistula to bladder/vag/prostatic urethra), low = mec to perineal skin … BOTH = tx w posterior sagittal anoplasty (pull anus into sphincter mechanism), but high = colostomy first and later recon, low = no colostomy … BOTH = need postop anal dilatation to void structure, these pts are prone to constipation

168
Q

gastrochisis vs omphalocele

A

gastro = intrauterine rupture of umbilical vein, does NOT have peritoneal sac, 10% assoc congenital anomalies, to R of midline … omphalo = failure of emb development, WITH peritoneal sac, 50% with assoc abnL, midline defect … malrotation may occur with both … omphalo has worse prognosis 2/2 assoc abnormalities

169
Q

gastroschisis: pathophys

A

intrauterine rupture of umbilical vein, does NOT have a peritoneal sac

170
Q

gastroschisis: describe appearance

A

to R of midline, NO peritoneal sac, stiff bowel from exposure to amniotic fluid

171
Q

gastroschisis: assoc congential anomalies

A

only about 10%, malrotation

172
Q

gastroschisis: tx

A

initially place saline-soaked gauze and resuscitate pt (can lose a lot of fluid from the exposed bowel), TPN, NPO … repair when pt is stable … at operation may try to place bowel in abdomen, +/- vicryl mesh silo - primary closure at a later date if use silo

173
Q

Cantrell pentalogy

A

(1) cardiac defect, (2) pericardium defects (usually at disphragmatic pericardium), (3) sternal cleft or absence of lower sternum, (4) diaphragmatic septum transversum absence, (5) omphalocele

174
Q

omphalocele: pathophys

A

failure of embryoneal development, HAS peritoneal sac with cord attached

175
Q

omphalocele: describe appearance

A

midline defect, WITH peritoneal sac with cord attached, ac can contain structures other than bowel (i.e. liver, spleen, etc)

176
Q

omphalocele: assoc congenital abnormalities

A

50%, midline defect

177
Q

omphalocele: tx

A

initially place saline-soaked gauze and resuscitation pt, can lose a lot of fluid from exposed bowel, TPN, NPO … repair with pt is stable … at operration, try to placed bowel back in abdomen, may need Vicryl mesh silo, primary closure at a later date if mesh used

178
Q

omphalocele: prognosis

A

worse than gastroschisis 2/2 congenital anomalies

179
Q

1 cause of colonic obstruction in infants

A

Hirschsprung’s disease

180
Q

Hirschsprung’s disease: gender

A

MC in M

181
Q

Hirschsprung’s disease: MC sign

A

(#1 cause of colonic obstruction in infants), MC sign = infants fail to pass meconium in 1st 24 hours … can also p/w chronic constipation in ages 2-3

182
Q

Hirschsprung’s disease: px

A

infant fails to pass meconium in 1st 24hours, chronic constipation in ages 2-3 …. distention, occasionally colitis … explosive release of watery stool with anorectal exam

183
Q

Hirschsprung’s disease: dx

A

rectal bx = absence of ganglion cells in myenteric plexus

184
Q

Hirschsprung’s disease: pathophys

A

failure of neural crest cells (ganglion cells) to progress in caudad direction (which is why you see the absence of ganglion cells in myenteric plexus on rectal bx)

185
Q

Hirschsprung’s disease: surgery

A

resect colon proximally to the point where ganglion cells appear … tx = may need to bring up initial colostomy, then eventually connect the colon to the anus (Soave or Duhamel procedures)

186
Q

Hirschsprung’s colitis: describe, px, tx

A

may be rapidly progressive …abd distention, foul-smelling diarrhea, +/- lethargy and signs of sepsis …. tx = rectal irrigation and try to empty colon, may need emergency colectomy

187
Q

umbilical hernia: describe, MC pts, tx

A

failure of closure of linea alba, most close by age 3, rare incarceration … inc in Af American and premature infants … tx = surgery if not closed by age 5, incarceration, pt w VP shunt

188
Q

inguinal hernia: pathophys

A

persistent processus vaginalis

The vaginal process (or processus vaginalis) is an embryonic developmental outpouching of the parietal peritoneum. It is present from around the 12th week of gestation, and commences as a peritoneal outpouching.

In males, it precedes the testes in their descent down within the gubernaculum, and closes. This closure (also called fusion) occurs at any point from a few weeks before birth, to a few weeks after birth. The remaining portion around the testes becomes the tunica vaginalis. If it does not close in females, it forms the canal of Nuck.

189
Q

inguinal hernia: rate in infants, MC gender

A

3% of infants, M > F

190
Q

inguinal hernia: laterality

A

60% on R, 30% on L, 10% b/l

191
Q

inguinal hernia vs hydrocele

A

extension of hernia into the internal ring

192
Q

inguinal hernia: tx

A

non reducible —> emergent operation … o/w elective repair with high ligation … consider exploring the contralateral side IF L sided, F, chidl <1yo …. need operation within 24 hours of reduction

193
Q

hydrocele: describe, tx

A

most disappear by 1yo, noncommunicating will resolve, shoudl transilluminate … tx = surgery at 1 year if not resolved or if through to be communicating (waxing and waning size), resect hydrocele and ligate processus vaginalis

194
Q

cyst duplication: MC site, tx

A

MC in ileum, often on mesenteric border, tx = resect cyst

195
Q

MC cause of neonatal jaundice requiring surgery

A

biliary atresia

196
Q

biliary atresia: px

A

progressive jaundice persisting >2weeks after birth is suggestive

197
Q

biliary atresia: pathophys

A

can involve the extrahepatic, intrahepatic, or both biliary trees

198
Q

biliary atresia: dx

A

liver bx –> periportal fibrosis, bile plugging, eventual cirrhosis

199
Q

biliary atresia: imaging

A

U/S and cholangiography can reveal atretic biliary tree

200
Q

biliary atresia: progression

A

get cholangitis, continued cirrhosis, eventual liver failure

201
Q

biliary atresia: surgical tx

A

Kasai procedure (hepaticoportojejunostomy, resect atretic extrahepatic bile duct segment) … BEFORE 3mo old, o/w get irreversible liver damage

202
Q

biliary atresia: surgical outcomes

A

1/3 get better, 1/3 need liver txp, 1/3 die

203
Q

teratoma: labs

A

inc AFP and b-HCG

204
Q

teratoma: MC site

A

neonates = sacrococcygeal … adolescents = ovarian

205
Q

teratoma: tx

A

excision

206
Q

sacrococcygeal teratomas: malignancy, marker, tx

A

90% benign at birth (almost all have exophytic component), but HIGH malignant potential … 2 months is an important transition point: <2mo usually benign, >2mo usually malignant … AFP = good marker … tx = coccygectomy and long-term follow up

207
Q

undescended testicles: treatment age

A

wait until 2yo to treat

208
Q

undescended testicles: inc risk for what

A

testicular cancer … inc cancer risk stay the same even after the testicles are brought into the scrotum … get seminomas

209
Q

undescended testicles: mgmt if b/l

A

get chromosomal studies

210
Q

undescended testicles: imaging needed

A

if you cannot feel the testes in the inguinal canal, then get MRI to confirm their presence

211
Q

undescended testicles: tx

A

orchiopexy through inguinal incision … if unable to get testicles down –> close and wait 6mo before trying again … if they will not come down then perform division of spermatic vessels

212
Q

tracheomalacia: describe

A

elliptical, fragmented tracheal rings instead of C shape

213
Q

tracheomalacia: px

A

wheezing, usually improves after 1-2 years

214
Q

tracheomalacia: surgical indications

A

dying spell, failure to wean from vent, recurrent infections

215
Q

tracheomalacia: surgical approach

A

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

216
Q

MC cause of airway obstruction in infants

A

laryngomalacia

217
Q

laryngomalacia: pathophys

A

immature epiglottis cartilage with intermittent collapse of the epiglottis airway

218
Q

laryngomalacia: sx

A

intermittent respiratory distress and stridor exacerbation in the supine position

219
Q

laryngomalacia: natural history

A

most children outgrow this by 12months

220
Q

laryngomalacia: surgical mgmt

A

surgical tracheostomy for very few patients

221
Q

choanal atresia: describe

A

obstruction of choanal opening (nasal passage) by either bone or mucus membrane, usually unilateral

222
Q

choanal atresia: sx

A

intermittent resp distress, poor suckling

223
Q

choanal atresia: tx

A

surgical correction

224
Q

MC tumor of the pediatric larynx

A

laryngeal papillomatosis

225
Q

laryngeal papillomatosis: natural hx

A

frequently involutes after puberty

226
Q

laryngeal papillomatosis: tx

A

can tx w endoscopic removal or laser, but frequently recurs

227
Q

laryngeal papillomatosis: caused by what

A

thought to be 2/2 HPV in the mom during passage through the birth canal

228
Q

GI issue assoc with cerebral palsy

A

GERD

229
Q

dx studies and likely dx with following hx and PE: bilious emesis, abd distention

A

plain abd film … dx = intestinal atresia or stenosis

230
Q

dx studies and likely dx with following hx and PE: failure to pass meconium, bilious emesis, acholic meconium, gastric distention, trisomy 21

A

contrast enema, plain abd film, upper GI contrast study … dx = duodenal atresia or stenosis

231
Q

dx studies and likely dx with following hx and PE: failure to pass meconium, bilious emesis (late), absent anus or visible fistula, abd distention

A

plain chest and abd films, U/S of kidneys, sacrum, rectum … dx = imperforate anus

232
Q

dx studies and likely dx with following hx and PE: high-risk premature infant, bilious emesis, abd distention, hematochezia, guaiac positive stool

A

plain abd film … dx = necrotizing enterocolitis

233
Q

dx studies and likely dx with following hx and PE: CF (10%), bilious emesis, acholic meconium, abd distention

A

plain abd film, contrast enema … dx = meconium ileus

234
Q

dx studies and likely dx with following hx and PE: bilious emesis, term healthy infant, no abd distention

A

plain abd film … dx = malrotation

235
Q

dx studies and likely dx with following hx and PE: delayed passage of meconium, bilious emesis, abd distention, trisomy 21

A

plain abd film, contrast enema … dx = Hirschsprung’s disease

236
Q

GI anomalies assoc with trisomy 21

A

Hirschsprung’s, duodenal atresia

237
Q

ddx of bilious emesis in infant

A

intestinal atresia (also w abd distention) … duodenal atresia (also w failure to pass or acholic mec, gastric distention, trisomy 21) … imperforate anus (late bilious emesis, also w failure to pass mec) … NEC (in high risk, premature infant w abd distention and hematochezia) … mec ileus (assoc w CF) … malrotation (no abd distention, healthy term infant) … Hirschprung’s (with delayed mec, abd distention, trisomy 21)

238
Q

ddx of failure to pass meconium in infant

A

duodenal atresia or stenosis, imperforate anus, meconium ileus (10% of CF patients), Hirschsprung’s (delayed passage, >24hr)

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