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RESP 5017 > 11. Neonatal Pathophysiology (Congenital & Surgical) > Flashcards

11. Neonatal Pathophysiology (Congenital & Surgical) Flashcards

1
Q

What does a TEF usually occur with?

What common neonatal population do these occur more frequently in?

A
  1. Esophageal atresia (EA)

2. Preterm delivery, small gestational age (SGA)

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2
Q
  1. The esophagus and the trachea separate during the ___ and ___ week of embryologic development
  2. What does a TEF-EA occur from?
A
  1. 4th and 5th

2. Incomplete separation

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3
Q

What is the most common type of TEF-EA (and how common)? Explain how the fistula is oriented, and is there esophageal atresia present?

A
  1. Type C, EA with distal TEF! (84% of cases)
  • esophagus shaped like pouch superiorly, secretions build here
  • distal esophagus connection to trachea causes air to build up in the stomach
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4
Q

When figuring out the associated anomalies with a TEF-EA, what does VACTERL stand for?

A 
Vertebral defect
Anorectal: imperforate anus
Cardiac defect: congenital heart defect
Tracheal: TEF
Esophageal: EA
Renal anomalies
Limb defects
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5
Q
  1. What do you see on U/S antenatally to aid in diagnoses of TEF-EA? (3 things)
  2. What do you see postnatally? (physically/diagnostic, 7 things)
A
  1. Small or absent stomach, polyhydramnios, dilated upper esophagus
  2. Excessive salivation, drooling, choking, coughing, regurg with feeding, resp. distress from aspiration, excessive gastric distention (type C), high suspicion with other anomalies, X-ray confirmation (unable to pass NG past 10 cm, esophagus is pouch shaped, air in stomach)
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6
Q

How do you manage a baby with TEF-EA before surgerical intervention? (4 things)
What are surgeons occasionally unable to do for these patients, and what do they do about it?

A
  1. ABCs as needed (careful with the airway, gastric distention/leak, intubation with surgeon present), NG to low-intermittent suction to clear pouch and prevent aspiration, keep head elevated at 45 degrees, rule out/manage anomalies (echo, U/S, CXR)
  2. Occasionally unable to connect/stretch esophagus to connect upper and lower portion, so they stretch them out over a few weeks & then try again
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7
Q
  1. How are TEF-EA pts managed post-op? (3 things)

2. What complications can arise?

A
  1. Avoid suction past end ETT, avoid suction into esophagus at level of surgical site, monitor for pneumo
  2. Esophageal strict, GERD, difficult feeding
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8
Q
  1. What is/what happens in Gastroschisis?
  2. What location does it occur in with regards to the umbilical cord?
  3. The abdominal wall defect is relatively _____ compared to _______.
  4. Is this associated with other syndromes/anomalies?
  5. What type of mother are babies with this condition common in?
A
  1. -Congenital abdominal wall defect
    - Herniation of abdominal contents, small intestine mainly, can also be the stomach, colon, ovaries
  2. Lateral, Right of umbilical cord insertion
  3. Small compared to the size of the eviscerated bowel
  4. No!
  5. Young mothers, 1st child
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9
Q
  1. What happens to the embryology of a fetus at 6 weeks?
  2. What happens at 10 weeks?
  3. If this transition at 10 weeks fails, what abnormality can happen to the baby?
A
  1. Abdominal content grows at a rate faster than the body elongates resulting in abdominal contents forming outside the abdominal cavity
  2. Abdominal content returns to abdominal cavity, and the abdominal wall forms
  3. Gastroschisis
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10
Q
  1. How is Gastroschisis diagnosed prenatally, and what is there an increased risk of in the third trimester?
  2. What can happen to the bowel in Gastroschisis, and why does it happen?
  3. Why does the small opening it causes in the abdomen cause problems?
A
  1. Diagnosed via ultrasound, third trimester has risk of fetal HR abnormalities, IUGR (growth restriction), oligohydramnios, gastrointestinal obstruction, preterm labour, still birth
  2. Extruded bowel has a thickened surface, with fibrous layer (peel), due to an inflammatory reaction to amniotic fluid, urine and meconium
  3. Small opening can obstruct BF and leads to bowel edema and intestinal necrosis
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11
Q
  1. Is a baby with gastroschisis usually delivered vaginally or by C/S?
  2. How is the baby managed in the delivery room when it is delivered? (hint: 6 things)
A
  1. Vaginally, C/S saved for usual complications
  2. Initial NRP steps, placed with right side down to decrease tension on mesenteric vessels, watch for effective BF, NG tube to decompress the stomach, sterile warm moist wrap to keep baby covered to minimize insensible fluid loss, IV access w/ increase TFI (total fluid intake)
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12
Q
  1. When does the surgery team see the baby with Gastroschisis, and what do they ensure?
  2. What do they do for small defects, and what do they do for large ones w/ smaller openings?
  3. What are the post-op risks? (hint: 7 things)
A
  1. Shortly after birth, ensures effective circulation
  2. Small: primary repair (shove in and close up)
    Large: “Silo procedure”, staged reductions every few days until repair
  3. Abdominal edema and ↑ intra-abdominal pressure, GI ischemia, bowel infarction, necrotizing enterocolitis, small bowel obstruction/perforation, intestinal atresia/stenosis, short bowel syndrome
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13
Q
  1. What is/what happens in Omphalocele?
  2. What does the sac consist of?
  3. What location does it occur in with regards to the umbilical cord?
  4. How common is it? (1 in every __ - __ births)
  5. What is there a higher incidence of if baby has this?
A
  1. Abdominal wall defect at umbilicus, failure of the lateral ectomesodermal folds to meet in midline of the abdomen b/w 3rd and 4th wks of gestation, bowel formed in a sac outside body
  2. Varying amount of bowel (liver, bladder, stomach, ovary, testis), and consists of amnion, Wharton’s jelly, and peritoneum
  3. Umbilical cord is attached to sac itself
  4. 1 in every 4,000-7,000 live births
  5. Chromosomal and associated anomalies
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14
Q

Because of the high risk of abnormalities with Omphalocele, what in-depth diagnostics are done?
Are babies delivered vaginally or by C/S? When is there an exception?

A

Ultrasound, MRI, Echo
Okay to deliver vaginally, C/S reserved for usual complications, exception when giant omphalocele w/ liver involvement delivery is by C/S

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15
Q

What are the different anomalies associated with Omphalocele, and how often do each occur?

A

Trisomy’s 13, 14, 15, 18, 21 - 20%
Beckwith-Wiedemann (gigantism, macroglossia, hypoglycemia, omphalocele) - 12%
Cardiac defects - 20%

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16
Q

How are babies with Omphalocele managed in the delivery room? (hint: 5 things)

A

NRP, protect sac from rupture (warm, moist towel), NG tube to decompress stomach, IV + replace insensible fluid loss, detailed assessment and management of other abnormalities

17
Q
  1. How are baby’s with Omphalocele treated surgically for a small defect, and how about a larger one?
  2. What can happen post-op in these babies? (hint: 2 things)
A
  1. Small: primary repair
    Large: may take a few surgeries and months to years to allow abdomen to grow, “paint and wait”, silver sulfadiazine cream allows sac to thicken to stop damage.
  2. Abdominal edema and increased intra-abdominal pressure
18
Q

What is the difference between Gastroschisis and Omphalocele based on….

  1. Location?
  2. Sac/No Sac?
  3. Insertion of umbilical cord?
  4. Associated anomalies?
A 
1. G: Lateral - right of the umbilical cord insertion 
O: Ventral - at base of cord 
2. G: Absent
O: Present 
3. G: Normal 
O: Inserts onto sac
4. G: Rare 
O: Common (40-80%)
19
Q
  1. What is Necrotizing Enterocolitis (NEC)?
  2. What is it classified as?
  3. What does it cause damage to, and how does the extent of injury range?
A
  1. Disease of the GI tract characterized by necrosis, often leading to perforation
  2. Acute inflammatory disease
  3. Damage to intestinal tract ranging from mucosal injury to full thickness necrosis and perforation
20
Q

What are the important risk factors for NEC? (Hint: 4 that Greg bolded in black)

(I added the extras in case we wanted to try and guess those too, there are 7)

A
  1. Prematurity
  2. Enteral nutrition (formula feeding)
  3. Ichemia
  4. Infection

Others…
- Umbilical catheterization, perinatal asphyxia, hypothermia, shock, hypoxia, thrombocytopenia, anemia

21
Q

How can we prevent NEC? (hint: 5 things)

A

NPO, trophic feeds (slow feeds), slower advancement of feed volume, supplemental immunoglobulins, probiotics

22
Q
  1. What is the hallmark sign of NEC?

2. What are the other clinical manifestations of NEC? (hint: 7 things)

A
  1. Bloody stool
  2. Signs of sepsis (apnea, bradycardia, hypotension, lethargy, temp instability, blood glucose instability, met. acidosis, thrombocytopenia), feeding intolerance, emesis, abdominal distention, decreased bowel sounds, pain, discomfort, guarding, bilious emesis
23
Q

What are the stages of NEC? (hint: 6 stages)

A
  1. Mild non-specific signs of sepsis
  2. Blood in stool
  3. Pneumatosis intestinalis (air from bacteria into walls of intestines)
  4. Increasing severity of sickness
  5. Severely ill (hypotension, bradycardia, resp failure, met. acidosis, coagulopathy, neutropenia)
  6. Perforated bowel (bowel into abdominal cavity)
24
Q
  1. What is pneumatosis?
  2. How does pneumatosis appear on CXR?
  3. Why does pneumatosis occur?
A
  1. Gas in the intestinal wall
  2. Bubbly appearance
  3. Result of bacterial fermentation
25
Q
  1. What is a pneumoperitoneum and what happens when an infant has this?
  2. How does it present on a supine radiograph? What abut a left lateral decubitus CXR?
A
  1. Full thickness necrosis of the bowel with resultant perforation, results in free air in the peritoneal cavity
  2. Supine: Football sign
    Left lateral decubitus: Free air around the liver (pushes liver down)
26
Q

How is NEC managed/treated? (hint: 6 things, we will talk about surgical management on separate card)

A

Treat based on Bell Stage, treat ABCs (be aware of neg. effects of CPAP, often abdominal edema and decreased lung vol), treat sepsis/shock (fluid bolus, inotropes), ABX, IVIG, blood (if needed)

27
Q

How is NEC treated surgically? (hint: 4 things)

A
  1. Removing large sections of bowel
  2. Laparotomy w/ resection and ostomy
  3. Primary anastomosis
  4. Penrose drains for abdominal compartment syndrome or evacuation of the peritoneal cavity
28
Q

What are the possible complications of NEC? (hint: 4 things)

A
  1. Intestinal stricture and adhesions
  2. Short-gut syndrome
  3. Failure to thrive
  4. Liver disease

RESP 5017 (19 decks)

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