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USMLE Step 2 > Pediatrics: Congenital Gastroenterology > Flashcards

Pediatrics: Congenital Gastroenterology Flashcards

(115 cards)

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1
Q

What is Meconium

A

The first stool of a newborn

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2
Q

What is the appearance of Meconium

A

Dark, thick and sticky

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3
Q

When should Meconium pass by

A

within 24 hours

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4
Q

What is Meconium Aspiration Syndrome

A

meconium passes in-utero and the baby passes through meconium-stained amniotic fluid

leads to newborn respiratory distress

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5
Q

How to treat Meconium Aspiration Syndrome

A

Maintenance Oxygen and Ventilation

Empiric Abx

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6
Q

What does Delayed Meconium Passage indicate

A

Possible Intestinal Obstruction

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7
Q

What diseases should you think of with delayed passage of Meconium

A

Meconium Ileus

Hirschsprung’s

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8
Q

What is Meconium Ileus

A

SBO due to meconium

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9
Q

What causes Meconium Ileus

A

Meconium is too thick and sticky causing a meconium plug to form

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10
Q

How does Meconium Ileus present

A

Abdominal Distension

Bilious Vomit (yellow-green)

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11
Q

What disease are almost all cases of Meconium Ileus associated with

A

Cystic Fibrosis

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12
Q

How do you diagnose Meconium Ileus

A

Abdominal X-ray

Contrast Enama X-ray

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13
Q

What does an abdominal x-ray show in meconium ileus

A

Dilated bowel loops
-soap bubble sign

meconium mixed with air excludes perforation

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14
Q

What does a contrast enema x-ray show in meconium ileus

A

Small colon: unused

partial small bowel filling

pellets of meconium

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15
Q

How do you manage Meconium Ileus

A

NG decompression

Gastrografin Enema

Surgery if hemodynamically unstable

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16
Q

How does a Gastrografin Enema work

A

Hyperosmolar Enema that draws fluid into the bowel to break up meconium

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17
Q

What causes Hirschsprung’s Disease

A

Abnormal Peristalsis of the Colon caused by absent ganglion cells
-fail to migrate properly

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18
Q

How does Hirschsprung’s disease present

A

Obstruction due to no peristalsis

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19
Q

What is Hirschsprung’s Disease associated with

A

Down Syndrome

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20
Q

What are the common clinical features of Hirschsprung’s disease

A

failure to pass Meconium

Abdominal Distension

Bilious vomit

Squirt or blast sign

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21
Q

What is seen on examination in Hirschsprung’s Disease

A

no stool in the rectal vault

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22
Q

What is the Squirt or blast sign

A

rectal exam provides temporary relief of the obstruction leading to rapid emptying of the bowels

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23
Q

How do you diagnose Hirschsprung’s Disease

A

Abdominal X-ray

Contrast enama: Transition zone

Rectal Suction Biopsy

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24
Q

What does an abdominal x-ray show in hirschsprung’s disease

A

dilated bowel with no air in the rectum

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25
What does a contrast enema show in Hirschsprung's Disease
Transition zone -distended proximal bowel -small distal bowel
26
What does a rectal "suction" biopsy show in Hirschsprung' disease
The rectum is always involved need to biopsy full thickness into submucosa reveals absence of ganglion cells
27
How to treat Hirschsprung's Disease
Colon resection to remove the segments without ganglion cells
28
What is Intestinal Atresia
Closed/absent opening anywhere in the GI tract
29
What location is Intestinal Atresia most common
Duodenum
30
How does Intestinal Atresia present
Bilious vomit in newborn abdominal distention
31
What is Intestinal Atresia associated with
Polyhydramnios
32
What is Duodenal Atresia Associated with
Failure of Recanalization Down Syndrome
33
How do you diagnose Duodenal Atresia
X-ray
34
What is seen on x-ray in Duodenal Atresia
Double Bubble Sign -distention of duodenal stump and stomach -tight pylorus between bubbles -no distal gas
35
What is the definitive treatment for Duodenal Atresia
Surgery after stabilization with NGT and Fluids
36
What causes Jejunal-Ileal Atresia
Vascular disruption leads to ischemic necrosis of the intestine -necrotic tissue is resorbed leaving blind ends of bowel Bowel distal to blind end may be curled -apple-peel atresia
37
How do you diagnose Jejunal-Ileal Atresia
X-ray
38
What does an x-ray show for Jejunal-Ileal Atresis
Triple bubble sign -double bubble + 3rd bubble for jejunal distension
39
How do you treat Jejunal-Ileal Atresia
Surgery
40
What causes malrotation
failure of embryonic gut to rotate normally -improper positioning of the bowel -Fibrous adhesion (ladd bands)
41
What can malrotation lead to
Duodenal Obstruction from bands
42
What is the most common presentation of malrotation
Midgut Volvulus
43
What causes a Midgut Volvulus
Small bowel (Ileum) twists around the SMA causing SBO Vascular compromise can lead to ischemia
44
What age does Midgut Volvulus usually present
3 to 7 days
45
How does Midgut Volvulus usually present
Bilious vomiting Mild abdominal distention Blood in stool Sepsis from bowel necrosis
46
What is the best initial test in suspected Midgut Volvulus
Abdominal X-ray -can be normal early -may show dilated bowel loops -excludes perforation
47
How do you diagnose midgut volvulus
Upper GI series with contrast
48
What is seen on an Upper GI series with contrast in Midgut Volvulus
Corkscrew appearance of proximal small bowel
49
How do you treat Midgut Volvulus
Urgent Surgery: Ladd's Procedure -remove adhesions -remove appendix -SB on right -colon on left
50
What is Biliary Atresia
Biliary Obstruction in newborns
51
What causes Biliary Atresia
Biliary ducts do not form or degenerate early in life
52
What age does Biliary Atresia usually present
2 to 8 weeks
53
How does Biliary Atresia Present
Conjugated Hyperbilirubinemia Jaundice, Dark-urine pale stools (acholic) Elevated AST/ALT
54
How do you diagnose Biliary Atresia
Ultrasound
55
What is seen on US in Biliary Atresia
Gallbladder absent/abnormal Absence of common bile duct
56
How do you treat Biliary Atresia
Surgery: Kasai Procedure -hepatoportoenterostomey -create drainage using small intestine Follow with Ursodeoxycholic acid
57
What is Esophageal Atresia
Esophageal narrowing or interruption
58
What are clinical features of Esophageal Atresia
Esophagus does not connect to stomach -polyhydramnios -drool,choke,vomit -symptoms with feeds -cannot pass NG tube Fistula esophagus to trachea -gastric distention -reflux -aspiration PNA -respiratory distress
59
What is the best initial test for Esophageal Atresia
CXR
60
What does a CXR show in Esophageal atresia
NGT is curled with a gas-filled intestinal tract
61
How to treat Esophageal Atresia
Surgical repair
62
What is the V in VACTERL Association
Vertebral Defects
63
What is the A in VACTERL association
Anal atresia
64
What is the C in VACTERL Association
Cardiac Defects
65
What is the T in VACTERl association
Tracheosophageal Fistula
66
What is the R in VACTERL association
Renal Anomalies
67
What is the L in VACTERL association
Limb abnormalies
68
What is needed to diagnose VACTERL
at least 3 of the symptoms
69
What causes Congenital Diaphragmatic hernia (CDH)
Defective formation of the pleuroperitoneal membrane allows abdominal organs to herniate into the chest
70
What can result from in utero herniation in CDH
Pulmonary Hypoplasia Fetal Demise
71
How do you diagnose CDH in utero
Ultrasound
72
How do you diagnose CDH at birth
CXR with abdominal contents in chest
73
How do you initially manage CDH
Stabilize Ventilate
74
What is the definitive treatment for CDH
Surgery
75
What is Hypertrophic Pyloric Stenosis
Pylorus hypertrophy obstructs the gastric outlet
76
How does Hypertrophic Pyloric stenosis present
Projectile non-bilious vomiting Palpable mass -olive sign Metabolic alkalosis -from vomiting "hungry vomit"
77
At what age does Hypertrophic Pyloric Stenosis Present
3-6 weeks
78
What is Hypertrophic Pyloric Stenosis Associated with
1st child Males Macrolide use <2 weeks of age
79
How do you diagnose Hypertrophic Pyloric Stenosis
Ultrasound
80
What does an Ultrasound show in Hypertrophic Pyloric Stenosis
Target or Donut sign Hypertrophied hyperechoic muscle that surround mucosa
81
What do you do to diagnose Hypertrophic Pyloric Stenosis if an US is equivocal
Upper GI contrast X-ray
82
What does an upper GI Contrast X-ray show for Hypertrophic Pyloric Stenosis
String sign from elongated pyloric canal
83
How do you treat Hypertrophic Pyloric Stenosis
Volume resuscitation Surgery
84
What is an Omphalocele
Persistence of normal herniation through the umbilical chord covered by peritoneal membrane
85
What is an easy way to detect Omphalocele
Fetal Ultrasound
86
What genetic conditions are associated with Omphalocele
Trisomy 21 (down) Trisomy 18 (edward) Trisomy 13
87
What other conditions are associated with Omphalocele
Congenital heart defects Orofacial Clefts Neural Tube defects
88
How do you treat Omphalocele
Surgical closure
89
What is Gastroschisis
Extrusion of bowel through abdominal wall defect not covered by peritoneum
90
What side does Gastroschisis usually occur
Right side of the umbilical cord
91
What lab value is Gastroschisis associated with
Elevated maternal AFP
92
What are potential complications of Gastroschisis
Bowel Ischemia and necrosis
93
How do you treat Gastroschisis
Surgery
94
What is Meckel's Diverticulum
Diveticiulum of SB (ileum) due to persistent remnant of the Vitelline duct
95
What type of Diverticulum is Meckel's
True: contains all layers of bowel wall
96
What else is often contained in a Meckel's Diverticulum
Extopic Gastric tissue
97
What is the most common symptom of Meckel's Diverticulum
Painless bleeding -ectopic gastric tissue may secrete acid ---Ulcer, Bleed, Pain
98
What rule is associated with Meckel's
Rule of 2's
99
What is the Rule of 2's
2% of population 2:1 M:F Within 2 feet of ileocecal valve Usually 2 inches in size
100
How do you diagnose Meckel's
Technetium Scan (Meckel's Scan)
101
What does a Technetium Scan show in meckel's
Tracer uptake by gastric cells in diverticulum
102
How do you treat Meckel's
Surgery for symptomatic Patients
103
What is an Intussusception
Telescoping of intestine folding into lumen
104
Where does Intussusception usually occur
Near the Ileocecal Junction
105
What age is common for Intussusception
<2
106
How does Intussuscpetion present
Abdominal Pain GI Bleed; Currant Jelly -blood/mucous
107
How do you diagnose Intussusception
Abdominal US
108
What does an abdominal US show in an Intussusception
Bull's eye or target sign
109
How do you treat Intussusception
Air enema to resolve infolding of intestines Surgery in severe, refractory cases
110
What is an Annular Pancreas
The ventral pancreatic bud fuses around the duodenum
111
What can an Annular Pancreas lead to
SBO Rare; Pancreatitis
112
How do you diagnose an Annular Pancreas
Upper GI series of CT scan
113
How do you treat an annular pancreas
Duodenoduodenostomy -bypass annulus -do not resect because you may cause fistula
114
What is Pancreas Divisum
Dorsal/Ventral Pancreatic ducts do not fuse 2 separate ducts -usually asymptomatic -may cause pancreatitis
115

USMLE Step 2 (40 decks)

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