GI Tract issues Flashcards
(148 cards)
1
Q
What is the greatest risk factor for Necrotizing Enterocolitis (NEC)?
A
Premature delivery
1
Q
How does Necrotizing Enterocolitis (NEC) Present?
A
Sudden changes in feeding tolerance, abdominal distension, bilious gastric retention vomiting, rectal bleed and diarrhea
2
Q
What physical exam findings may be present in Necrotizing Enterocolitis (NEC)?
A
Abdominal wall erythema, crepitus and induration
3
Q
What are the most common late GI complications of NEC?
A
Strictures and short bowel syndrome
4
Q
What is the best initial diagnostic test for suspected NEC?
A
Abdominal x-ray showing pneumatosis intestinalis, pneumoperitoneum or hepatobiliary gas
5
Q
How do you manage NEC?
A
Stop all feeds
Decompress with NG tube
Abx to cover aerobic and anaerobic intestinal bacteria
Surgery if perforation or failure to improve with medical therapy
6
Q
What is a Diaphragmatic Hernia?
A
Hole in the diaphragm that allows the abdominal contents to move into the thorax
7
Q
How can you diagnose a diaphragmatic hernia?
A
Bowel sounds heard in the chest on auscultation
air fluid levels seen on CXR
8
Q
What is an Omphalocele?
A
defect where intestines and organs form beyond the abdominal wall with a sac covering
9
Q
What causes an Omphalocele?
A
Failure of the GI sac to retract at 10-12 weeks gestation
10
Q
How do you screen for Omphalocele?
A
Maternal AFP levels and US
11
Q
How do you treat Omphalocele?
A
Surgical reintroduction of the contents
12
Q
What syndrome is highly associated with Omphalocele?
A
Trisomy 18: Edwards Syndrome
13
Q
What causes an Umbilical Hernia?
A
congenital weakness of the rectus abdominus muscle allows vessels and bowel to protrude
14
Q
What condition is highly associated with an Umbilical Hernia?
A
Congenital Hypothyroidism
15
Q
At what age is surgical intervention needed to repair an Umbilical Hernia?
A
4 years of age to prevent bowel strangulation and subsequent necrosis
16
Q
What is Gastroschisis?
A
Wall defect lateral to the midline with intestines and organs forming beyond the abdominal wall with no sac covering
17
Q
How do you treat Gastroschisis?
A
Immediate surgical intervention with gradual introduction of bowel and silo formation
18
Q
What can result from overly aggressive surgical reintroduction of bowel in Gastroschisis?
A
Third spacing and bowel infarction
19
Q
What is the most common abdominal mass in children?
A
Wilms Tumor
20
Q
What causes a Wilms Tumor?
A
hemihypertrophy of one kidney due to increased vascular demands
21
Q
What is felt on physical exam with a Wilms tumor?
A
large palpable abdominal mass
22
Q
What are common features of a Wilms tumor?
A
Anirdia: absence of Iris
constipation, abdominal pain, nausea, vomiting
23
Q
How is the best initial test for a Wilms tumor?
A
Abdominal US
24
What is the most accurate test for a Wilms tumor?
Contrast-enhanced CT
25
How is a Wilms tumor treated?
Total Nephrectomy with chemo and radiation based on staging
B/L involvement indicated partial nephrectomy
26
What is WAGR Syndrome?
Wilms Tumor
Anirdia
Genitourinary malformations
Intellectual Disability
27
What is the most common cancer in infancy and the most common extracranial solid malignancy?
Neuroblastoma
28
What is a Neuroblastoma?
Adrenal Medulla Tumor similar to a pheochromocytoma
29
How do you diagnose a Neuroblastoma?
Increased VMA and Metanephrines on urine collection
30
When is Hyperbilirubinemia considered Pathologic in a newborn?
On the first day of life
Bilirubin rises >5mg/dL/day
Bilirubin rises above 19.5mg/dL in a term child
Direct Bilirubin rises above 2mg/dL at any time
Hyperbilirubinemia persists after the 2nd week of life
31
What is the most serious complication of Hyperbilirubinemia?
Kernicterus
32
What is Kernicterus?
Deposition of Bilirubin in the basal ganglia
33
How does Kernicterus Present?
Hypotonia, seizures, choreoathetosis and hearing loss
34
If Jaundice Presents in the first 24 hours of life what should your workup include?
Total and Direct Bili
Blood type of mom/infant: check for ABO or Rh incompatibility
Direct Coombs Test
CBC, Retic Count, Blood smear: assess for Hemolysis
UA and UCx: assess for sepsis with elevated direct Bili
35
If you see increased Unconjugated Bilirubin what should you think of?
UTI/infection
Bilirubin Conjugation abnormalities
Hemolysis
Intrinsic RBC membrane or enzyme defects
36
If you see increased Conjugated Bilirubin what should you think of?
Cholestasis: check LFT, US and Liver biopsy
37
How do you treat Hyperbilirubinemia?
Photherapy for Bili >10-12
Exchange transfusion in suspected Bilirubin Encephalopathy
38
Which type of Bilirubin can cross the BBB to enter the brain?
Unconjugated: causes cell death by apoptosis and or necrosis
39
How many phases of Acute Bilirubin Encephalopathy are there?
3
40
How does Phase 1 of Acute Bilirubin Encephalopathy present?
Infant is sleepy but arousable with a high-pitched cry
41
How does phase 2 of Acute Bilirubin Encephalopathy present?
Infant more lethargic: poor sucking reflex, hypertonia, beginning with backward arching of neck (Retrocollis) and trunk (opisthotonos) with stimulation
needs emergent exchange transfusion
42
How do you manage the symptoms seen in Phase 2 of Acute Bilirubin Encephalopathy?
Emergent Exchange Transfusion
43
How does Phase 3 of Acute Bilirubin Encephalopathy Present?
Apnea, unable to feed, fever, seizures and coma
may lead to death from respiratory failure or intractable seizures
44
What is Kernicterus?
Chronic and permanent neurologic sequelae of Bilirubin-Induced Neurologic Dysfunction (BIND)
45
How does Kernicterus present?
Choreoathetoid cerebral palsy
Significant hearing loss due to auditory neuropathy
Gaze palsy, upward gaze
Dental enamel dysplasia
46
What is the most common inherited disorder of Bilirubin Glucuronidation?
Gilbert Syndrome
47
What causes Gilbert Syndrome?
mutation in the gene coding for Enzyme UGT1A1 which is resonsible for the conjugation of bilirubin with glucuronic acid
48
How does Gilbert Syndrome present?
recurrent episodes of jaundice triggered by high physical stress
Typically no symptoms aside from jaundice
49
How do you treat Gilbert Syndrome?
no treatment required
50
What Causes Dubin-Johnson Syndrome?
AR mutation of the MRP2 gene
51
How does Dubin-Johnson Syndrome present?
Decreased hepatic clearance of Conjugated Bilirubin
Grossly black liver
Impaired biliary excretion of bilirubin glucuronides
52
What are the relevant lab findings in Dubin-Johnson Syndrome?
Increased Conjugated Bilirubin
Increased Coproporphyrin I levels
53
What does imaging show in Dubin-Johnson Syndrome?
Unable to visualize the GB and biliary system on oral cholecystography
54
How do you treat Dubin-Johnson Syndrome?
Generally no treatment as patients are asymptomatic
55
What is the cause of Rotor Syndrome?
AR mutation of SLCO1B1 and SLCO1B3 genes
56
How does Rotor Syndrome present?
Decreased hepatic excretion of conjugated bilirubin
Defect in hepatic storage of conjugated bilirubin
57
What are the relevant labs in Rotor Syndrome?
Increased Conjugated Bilirubin
Increased total urine Coproporphyrin levels
58
What does imaging show in Rotor Syndrome?
Ability to visualize GB via oral cholecystogram
59
What is Biliary Atresia?
Inflammatory obliterative cholangiopathy that affects intra and extahepatic bile ducts in neonates
60
How does an infant with Biliary Atresia Present?
jaundice and icterus by 8 weeks with acholic stools
61
What do labs show in Biliary Atresia?
Elevated Conjugated Bilirubin
Very high GGTP
Coagulopathy due to vitamin K deficiency
62
What is the best initial test for Biliary Atresia?
ultrasound
63
What is the most accurate test for Biliary Atresia?
Livery biopsy
64
What is the gold standard test for Biliary Atresia?
ERCP with Cholangiogram: only performed if biopsy is not definitive
65
How do you treat Biliary Atresia?
Kasai Procedure (hepatoportoenterostomy) followed by Ursodeoxycholic acid
66
What is Esophageal Atresia?
the esophagus ends blindly
67
What is a Tracheoesophageal Fistula (TEF)?
Esophagus communicates with the trachea through a fistula: occurs in 90% of esophageal atresia cases
68
How does a child with Esophageal Atresia Present?
Vomiting with the first feeding or chocking/ coughing and cyanosis
hx of possible polyhydramnios
69
What can result from TEF?
recurrent aspiration pneumonia from food and secretions entering the lung
70
What is the most accurate test to diagnose Esophageal Atresia?
CT or esophagram with water-soluble contrast
71
What is seen on x-ray in esophageal atresia?
Gastric air bubble and esophageal air bubble
72
What happen if you try to pass an NG tube On CXR in esophageal atresia?
CXR will show coiling of the tube with an inability to pass it into the stomach
73
What syndromes are often associated with Esophageal atreasia/TEF?
CHARGE syndrome and VACTERL syndrome
74
What tests should be performed in all patients diagnosed with esophageal atresia/TEF?
Echocardiography and renal ultrasound
75
How do you treat esopahgeal atresia/tef?
Surgical separation through ligation of the fistula
Abx to cover anaerobes
Fluid resuscitation before surgery
76
What is the most common complication of surgery to correct a TEF?
Dyaphagia and GERD
77
What is Pyloric Stenosis?
Hypertrophic Pyloric sphincter prevents proper passage of GI contents from the stomach into the duodenum
78
What is the most common cause of Pyloric Stenosis?
Idiopathic
79
When does Pyloric Stenosis typically present?
between the first month and 6th month of life
80
What are common features of Pyloric stenosis?
Succussion splash on auscultation
Nonbilious projectile vomiting (hallmark)
Olive sign: palpable mass size of olive in epigastric region
81
What causes the Succussion splash in pyloric stenosis?
Stomach contents slapping into the pylorus like waves on a beach
82
What causes the nonbilious projectile vomiting in pyloric stenosis?
Hypchloremia, Hypokalemic metabolic alkalosis
Aldosterone release in response to hypovolemia worsens potassium loss by increasing urinary excretion
83
What is the most accurate test for Pyloric stenosis?
Abdominal US showing a thickened Pyloric Sphincter
84
If an Abdominal US is not diagnostic for Pyloric Stenosis what is the next best step?
Upper GI series
85
What 4 signs can be seen on Upper GI series for Pyloric Stenosis?
String sign
Shoulder sign
Muschroom sign
Railroad track sign
86
What is the string sign in Pyloric stenosis?
Thin column of barium leaking through the tightened muscle
87
What is the shoulder sign in pyloric stenosis?
Filling defect in antrum due to prolapse of muscle inward
88
What is the mushroom sign in pyloric stenosis?
Hypertrophic pylorus against the duodenum
89
What is the railroad track sign in pyloric stenosis?
Excess mucosa in the pyloric lumen results in 2 columns of barium
90
How is pyloric stenosis treated?
Replace lost volume with IV fluids
NG Tube to decompress the bowel
Surgical Myotomy
91
What is Choanal Atresia?
Infant is born with a membrane between the nostrils and pharyngeal space that prevents breathing during feeding
92
What condition is Choanal Atresia associated with?
CHARGE syndrome
93
What is CHARGE Syndrome?
Set of congenital defects seen together
-Coloboma of the eye, CNA anomalies
-Heart Defects
-Atresia of the choanae
-Retardation of growth and/or development
-Genital and/or urinary defects (hypogonadism)
-Ear anomalies and/or deafness
94
how does Choanal Atresia Present?
Child will turn blue when feeding and pink when crying
95
How is Choanal Atresia diagnosed?
CT scan to confirm diagnosis
96
What is the definitive treatment for Choanal Atresia?
Surgical intervention to perforate the membrane and reconnect the pharynx to the nostrils
97
What is Hirschsprung Disease?
Congenital lack of innervation of the distal bowel by Auerbach plexus leading to a constant contracture of muscle tone
98
What condition is frequently associated with Hirschsprung disease?
Down syndrome
99
How does Hirschsprung disease typically present?
First meconium passed >48 hours or not at all
Extreme constipation followed by large bowel obstruction
Extremely tight sphincter on rectal exam
100
How can you diagnose Hirschsprung disease?
Plain X-rays
Manometry
Biopsy
101
What do plain x-rays show in Hirschsprung disease?
Distended bowel loops with a lack of air in the rectum
contrast enemas show retention of barium for >24 hours
102
What is seen on Manometry in Hirschsprung disease?
High pressures in the anal sphincter
103
What is the mainstay of diagnosis for Hirschsprung disease?
Full thickness biopsy that reveals a lack of ganglionic cells in the submucosa
104
How do you treat Hirschsprung disease?
Surgical repair
105
What is an Imperforate anus?
The opening to the anus is missing and the rectum ends in a blind pouch with conservation of the sphincter
106
What condition is highly associated with an imperforate anus?
Down Syndrome
107
How do you diagnose an Imperforate anus?
Complete failure to pass meconium and no anus on physical exam
108
How do you treat an imperforate anus?
Surgery
109
What Syndrome is a impeforate anus a component of?
VACTERL syndrome
110
What are the components of VACTERL syndrome?
Vertebral anomalies
Anal Atresia
Cardiovascular Anomalies
Treacheoesophageal Fistula
Esophageal Atresia
Renal Anomalies
Limb Anomalies
111
What is Duodenal Atresia?
Improper canalization of the lumen of the duodenum due to absence of apotosis
112
What conditions are associated with duodenal Atresia?
Annular Pancreas and Down Syndrome
113
How does Duodenal Atresia typically present?
Bilious vomiting within 12 hours of birth
114
What is the most accurate test for Duodenal Atresia?
Upper GI contrast study with water-soluble contrast
115
What is another way to test for Duodenal Atresia?
Abdominal X-ray with double bubble sign
116
How do you treat Duodenal Atresia?
Replace lost volume with IVF
NG tube to decompress
Surgical Duodenostomy
117
What is the definitive treatment for Duodenal Atresia?
Surgery; classical Duodenostomy
118
What is Intussusception?
Part of the bowel telescopes into another segment more distal to it
119
What are possible causes of Intussusception?
Polyp, hard stool, lymphoma, viral origin, Most are idopathic
120
How does Intussusception typically present?
Colicky abdominal Pain, bilous vomiting and currant jelly stool
Right-sided sausage-shaped mass can be palpated
121
What can be palpated in Intussusception?
A right sided, sausage-shaped mass
122
What is the best initial test for Intussusception?
Ultrasound showing a donut sign or target sign
-due to concentric alternating echogenic (mucosa) and Hypoechogenic (submucosa) bands
123
What is the most accurate test for Intussusception?
Barium Enama
-diagnostic and therapeutic
124
What are contraindications to a barium enema in Intussusception?
Signs of Peritonitis, shock or perforation
125
How do you treat Intussusception?
Fluid resuscitation and electrolyte rebalancing
NG tube decompression
Barium enema; must carefully monitor
Emergent surgical intervention to prevent bowel necrosis
126
What is the most important initial step in treating Intussusception?
Fluid Resuscitation and rebalancing electrolytes
127
What is Meckel Diverticulum?
The only true congenital diverticulum where the vitelline ducts persists in the small intestinal tract
128
What is the most common congenital anomaly of the GI Tract?
Meckel Diverticulum
129
What is the rule of 2's?
Meckel Diverticulum
-affects 2% of the population
-2 feet from the ileocecal valve
-2 types of ectopic tissue: gastric and pancreatic
-Male 2x more common
-Patient <2 years
-2% are symptomatic
-2 inches long
130
How does Meckel Diverticulum classically present?
Painless rectal bleeding: massive frank bright red blood per rectum due to gastric acid secretion from the ectopic tissue searing the nearby small bowel tissue
131
What types of ectopic tissue are seen in Meckel Diverticulum?
Gastric tissue
132
What is the most accurate test for Meckel Diverticulum?
Technetrium 99m Scan
133
What is the only curative treatment for Meckel Diveticulum?
Surgical removal
134
What are the 3 major causes of Bilious Vomiting?
Duodenal Atresia
Volvulus
Intussusception
135
High yield Duodenal Atresia Facts: Onset, Initial test, First STep and Treatment?
Onset: within first day of life
Initial step: abdominal Xray
First Step: double Bubble: IV fluids
Treat: surgery
136
High yield Volvulus facts: onset, initial test, first step and treatment?
Onset: within 1st year of life
Initial test: Abdominal xray
First step: IV fluids
treatment: surgery
137
High yield Intussusception facts: Onset, Initial Test, First Step, and Treatment?
Onset: within 1st year of life
Initial test: US: doughnut sign
First step: IV fluids
Treatment: Air enema
138
What is the 2nd most common cause of infant death worldwide?
Acute diarrhea leading to loss of fluids and electrolytes
139
What is Gastroenteritis?
Inflammation of the GI tract secondary to microbiologic infiltrate and spread
140
How does inflammatory diarrhea present?
Fever, abdominal pain and possible bloody diarrhea
141
How does noninflammatory diarrhea present?
Vomiting, crampy abdominal pain and watery diarrhea
142
What are the common diagnostic tests for Diarrhea and Gastroenteritis?
Send stool for blood and leukocyte count to detect presence of invasive toxins
Stool PCR to help ID the causative agent
PCR for C. diff
143
What is the most important step in treating Diarrhea and Gastroenteritis?
Rehydration
-Mild: PO fluids
-severe (vomit/fever): IV Fluids
144
What is Small Left colon syndrome?
Congenital smaller descending colon that leads to distension from constipation
145
How is small left colon syndrome diagnosed?
Barium study
146
How do you treat Small left colon syndrome?
Smaller, more frequent feeds
147
What is small left colon syndrome typically a complication of?
Mothers with diabetes
