Mod VI: Intra-abdominal Malformations Flashcards
Intra-abdominal Malformations
Faulty separation of primitive trachea and esophagus (commonly occur together)
Esophageal Atresia & Tracheoesophageal Fistula
Esophageal Atresia & Tracheoesophageal Fistula
Incidence of Esophageal Atresia & Tracheoesophageal Fistula:
1:4,000 live births
Esophageal Atresia & Tracheoesophageal Fistula
What’s the Most common form of Esophageal Atresia & Tracheoesophageal Fistula?
Type IIIB
Location of fistula variable
Esophageal Atresia & Tracheoesophageal Fistula
Clinical presentation of Esophageal Atresia & Tracheoesophageal Fistula
Classic triad
Coughing, choking, cyanosis
Drooling
Regurgitation/Aspiration
Respiratory distress
Unable to pass NGT into stomach
Abdominal distention/gas
No abdominal gas: EA w/o fistula
Increased incidence pneumonia H-type
Esophageal Atresia & Tracheoesophageal Fistula
Anomalies associated with Esophageal Atresia & Tracheoesophageal Fistula: VACTERL
V: Vertebral (6 lumbar vertebrae, 13 pair ribs)
A: Anal atresia (imperforated anus)
C: Cardiac
T: TracheoEsophageal Fistula
E: Esophageal atresia
R: Renal agenesis
L: Limb defects
Esophageal Atresia & Tracheoesophageal Fistula
Picture showing the different types of Esophageal Atresia & Tracheoesophageal Fistula
See picture
Note Type IIIB, the most common presentation
Esophageal Atresia & Tracheoesophageal Fistula
Treatment of Esophageal Atresia & Tracheoesophageal Fistula:
Dependent on stability of infant
Surgery*
Delay surgery if pneumonia present until lungs improved (antibiotics, O2)
Gastrostomy tube placed under local
Reduce aspiration
NPO - NGT to LS - ↑ HOB - Intubate and MV if severe
Esophageal Atresia & Tracheoesophageal Fistula
Primary surgery for Esophageal Atresia & Tracheoesophageal Fistula involves:
Ligation of fistula with esophageal anastomosis
Esophageal Atresia & Tracheoesophageal Fistula
Staged surgery for Esophageal Atresia & Tracheoesophageal Fistula involves:
Gastrostomy with fistula diversion,
and later
Repair of esophagus
Esophageal Atresia & Tracheoesophageal Fistula
Anesthetic considerations/management during Induction w/ Esophageal Atresia & Tracheoesophageal Fistula
Prevention aspiration critical
Maintain upright position
Awake suction of proximal pouch prior to induction
Place gastrostomy to water seal if present
AFOI or inhalation induction
Maintains SV/avoids need for PPV
Avoid muscle relaxation and PPV with bag/mask
Esophageal Atresia & Tracheoesophageal Fistula
Anesthetic considerations/management during ET tube placement w/ Esophageal Atresia & Tracheoesophageal Fistula
Difficult if TEF present
Goal: Below fistula and above carina
1st = mainstem right bronchus
2nd= withdrawal ET slowly until BBS heard over L thorax
Esophageal Atresia & Tracheoesophageal Fistula
Anesthetic considerations/management during Maintenance w/ Esophageal Atresia & Tracheoesophageal Fistula
Inhalation anesthetic with SV until gastrostomy performed
Monitor inspiratory pressures: Avoid High!
Correct F/E disturbances/cont’d resuscitation efforts
Esophageal Atresia & Tracheoesophageal Fistula
ET tube placement in Esophageal Atresia & Tracheoesophageal Fistula
See picture
Intra-abdominal Malformations
Intra-abdominal malformation characterrized by failed migration of intestine into abdomen & failed closure of abdominal wall @ 6-8 weeks gestation; typically occurs at base of umbilicus and is known as:
Omphalocele
Viscera outside abdominal wall
Intact membrane (amnion)
Intra-abdominal Malformations - Omphalocele
Incidence of Omphalocele:
1:6,000
Intra-abdominal Malformations - Omphalocele
Associated congenital anomalies w/ Omphalocele
Cardiac lesions (20%)
Exstrophy bladder
Beckwith-Wiedemann syndrome
(mental retardation, hypoglycemia, congenital heart dx, large tongue)
Intra-abdominal Malformations - Omphalocele
What does Omphalocele look like at birth?
See picture
Intra-abdominal Malformations
A defect of abdominal wall on right lateral aspect of umbilicus w/ failed closure @ 12-18 weeks gestation is known as:
Gastroschisis
Lacks peritoneal coverage, exposed bowel
Highly susceptible to ECF loss and infection
Usually lateral to umbilicus
Intra-abdominal Malformations - Gastroschisis
Incidence of Gastroschisis:
1:30,000
Intra-abdominal Malformations - Gastroschisis
Why is Gastroschisis more urgent of a surgery?
Risk of fluid loss!!!
Lacks peritoneal coverage, exposed bowel
Highly susceptible to ECF loss and infection
Intra-abdominal Malformations - Gastroschisis
T/F: Gastroschisis is a/w Less incidence of concurrent anomalies
True
Although it is associated with prematurity
Intra-abdominal Malformations - Gastroschisis
What does Gastroschisis look like?
See picture
Omphalocele & Gastroschisis
Goals of Medical stabilization w/ Omphalocele & Gastroschisis include:
Protect defect
Minimize fluid & heat loss
IV hydration
Requires large amounts (150 ml/kg/day) of full-strength BSS plus colloids
Protection of viscera before surgical repair
NGT drainage
Omphalocele & Gastroschisis
How should the viscera of Omphalocele be protected before surgical repair in order to prevent increased heat loss?
Cover sac with sterile, warm, saline soaked gauze
Omphalocele & Gastroschisis
How should the viscera of Gastroschisis be protected before surgical repair in order to prevent Increased heat & ECF loss, and risk of infection?
Apply warm
Saline soaked gauze to exposed viscera
Wrap infant in warm
Sterile towels
Omphalocele & Gastroschisis
For Surgical repair of Omphalocele & Gastroschisis, hat are 2 major concerns with Primary closure?
Ventilation
Circulation
Omphalocele & Gastroschisis
For Surgical repair of Omphalocele & Gastroschisis and after primary closure, when is reopening and staged procedure indicated?
Intragastric pressures > 20 mmHg
24 hrs after primary closure
Omphalocele & Gastroschisis
For Surgical repair of Omphalocele & Gastroschisis, when is Staged procedure indicated? Describe it!
Staged with Silastic “silo” with larger defects
Silo size reduced every 2-3 days
Spontaneous ventilation maintained with intubation
Monitor O2 saturation, pulses, & BP to determine appropriate reduction size that allows adequate ventilation and circulation
Ketamine 0.5 – 1mg/kg common
Omphalocele & Gastroschisis
Silo size reduced every 2-3 days, pushing bowel back inside the abdomen
See picture
Omphalocele & Gastroschisis
Silo size reduced every 2-3 days, pushing bowel back inside the abdomen
See picture
Omphalocele & Gastroschisis
Silo size reduced every 2-3 days, pushing bowel back inside the abdomen
Abdominal pressure will be decreased prior to the fascia closure
See picture
Omphalocele & Gastroschisis
Anesthetic considerations/management w/ Omphalocele & Gastroschisis:
Aspiration risk
Induce/intubate in semi-upright position
Decompress stomach before
AFOI indicated
↑ inspiratory pressures may be necessary d/t inc abd cavivity pressure
Consider cuffed ETT
Maintain high suspicion for pneumothorax
SaO2/BP/ETC02
Muscle relaxation usually required
Work w/ surgeon to figure out what the true intraabdominal pressure is
Avoid N2O
To prevent any bowel distension
Correct F/E disturbance/Hypovolemic shock
Monitor CVP and U/O for adequacy of volume resuscitation
Thermoregulatory instability = warming measures
Omphalocele & Gastroschisis
Summary table
See picture
Intra-abdominal Malformations
Hypertrophy of pyloric smooth muscle → gradual obstruction of gastric outlet. This condition is also known as:
Pyloric Stenosis
Intra-abdominal Malformations - Pyloric Stenosis
When does Pyloric Stenosis present?
2nd or 3rd week of life
Intra-abdominal Malformations - Pyloric Stenosis
Clinical presentation of Pyloric Stenosis
Persistent, projectile, nonbilious vomiting
Metabolic disturbance?
Hyponatremic, Hypokalemic hypochloremic metabolic alkalosis with compensatory respiratory acidosis
Intolerance to feeding
Palpable abdominal mass (“olive”)
Gastric dilation via X-ray
Intra-abdominal Malformations - Pyloric Stenosis
GI assessment of Pyloric Stenosis
Palpable abdominal mass (“olive”)
Gastric dilation via X-ray
Intra-abdominal Malformations - Pyloric Stenosis
Metabolic disturbance a/w Pyloric Stenosis include:
Hyponatremic
Hypokalemic hypochloremic metabolic alkalosis
with compensatory respiratory acidosis
Intra-abdominal Malformations - Pyloric Stenosis
Confirmation of Pyloric Stenosis diagnosis
US
Barium swallow
Intra-abdominal Malformations - Pyloric Stenosis
What does Pyloric Stenosis look like?
See picture
Note enlarge pylorus causing the stenosis
Intra-abdominal Malformations - Pyloric Stenosis
T/F: Pyloric Stenosis is a surgical emergency
False
Pyloric Stenosis is a Medical Emergency = NOT Surgical
Intra-abdominal Malformations - Pyloric Stenosis
Correction of F/E deficits in the medical management of Pyloric Stenosis involves:
Resuscitate with full-strength BSS (NS)
Add K+ after infant begins to urinate
Intra-abdominal Malformations - Pyloric Stenosis
Which lab values indicate that the infant is adequately prepared and medically optimized for the OR?
Plasma Na+ > 130 mEq/L
Plasma K+ > 3.0 mEq/L
Plasma Cl- > 85 mEq/L
U/O = 1-2 ml/kg/hr
Normal skin turgor/tear production
Intra-abdominal Malformations - Pyloric Stenosis
Surgical repair of Pyloric Stenosis inculde:
Pyloromyotomy
Short procedure/small incision
Intra-abdominal Malformations - Pyloric Stenosis
Anesthetic considerations/management to address ↑Risk for aspiration w/ Pyloric Stenosis include:
Awake OG/NGT suction in supine & left lateral position
RSI or AFOI
Intra-abdominal Malformations - Pyloric Stenosis
Muscle relaxation required at 2 points during surgery to correct Pyloric Stenosis. What are those two points?
Delivery of pylorus at beginning
Return of pylorus at end of surgical procedure
Intra-abdominal Malformations - Pyloric Stenosis
Anesthsia technique: Controlled ventilation or Caudal?
Caudal provides needed muscle relaxation,
reduces anesthetic requirements, &
provides postoperative analgesia
Intra-abdominal Malformations - Pyloric Stenosis
T/F: Deep extubation is recommended for infants after surgery to correct Pyloric Stenosis
False
Infant should be fully awake and breathing adequately before removing ETT
Intra-abdominal Malformations
The developmental abnormality a/w spontaneous abnormal rotation of midgut around mesentery, which could cause complete or partial duodenal obstruction is also known as:
Intestinal Malrotation & Volvulus
Intra-abdominal Malformations - Intestinal Malrotation & Volvulus
Incidence of Intra-abdominal Malformations:
1:500 live births
Intra-abdominal Malformations - Intestinal Malrotation & Volvulus
What’s the only definitive treatment for Intestinal Malrotation & Volvulus?
Surgery provides only definitive treatment
Midgut volvulus is a Surgical Emergency
Intra-abdominal Malformations - Intestinal Malrotation & Volvulus
Anesthetic Considerations w/ Intestinal Malrotation & Volvulus:
Increased risk for aspiration
Decompress stomach
Keep hydrated
Invasive lines may be necessary
Intra-abdominal Malformations
T/F: Necrotizing Enterocolitis may appear in the absence of functional or anatomical lesions
True
Intra-abdominal Malformations - Necrotizing Enterocolitis
Incidence of Necrotizing Enterocolitis - Age group most affected:
5-15 % of infants
Predominantly in premature infants (90%)
Intra-abdominal Malformations - Necrotizing Enterocolitis
Mortality rate in Necrotizing Enterocolitis:
40%
Intra-abdominal Malformations - Necrotizing Enterocolitis
Pathogenesis of Necrotizing Enterocolitis:
Immature intestine has ↓ability to absorb substrates→ stasis
→ bacterial infection→ ischemia→ necrosis of intestinal mucosa
→ perforation, gangrene, fluid loss, peritonitis, septicemia, DIC
Intra-abdominal Malformations - Necrotizing Enterocolitis
Clinical features of Necrotizing Enterocolitis:
Abdominal distention/ileus
↑gastric aspirate
Bloody stools
Temperature instability
Cardiorespiratory instability
Hypovolemia/oliguria
Metabolic acidosis
Intra-abdominal Malformations - Necrotizing Enterocolitis
How does Necrotizing Enterocolitis look like?
See picture
Intra-abdominal Malformations - Necrotizing Enterocolitis
Treatment for Necrotizing Enterocolitis is primarily medical and includes:
Cessation of oral intake (NPO for 10-14 days)
Decompression of stomach
F/E therapy
Full-strength BSS - Correct metabolic acidosis
Peritoneal drain
Intubation/ventilation
Intra-abdominal Malformations - Necrotizing Enterocolitis
How is Necrotizing Enterocolitis Nonresponsive to medical treatment managed?
Exploratory laparotomy to remove gangrenous bowel
Intra-abdominal Malformations - Necrotizing Enterocolitis
Anesthetic considerations/management w/ Necrotizing Enterocolitis
Most challenging cases in pediatric anesthesia
Infants are critically ill, usually already intubated on mechanical ventilation
Continue resuscitative measures
Provide muscle relaxation and careful titration of anesthetic drugs*
Infuse full-strength BSS to maintain BP and U/O
Transfuse blood if HCT <30%
Intra-abdominal Malformations - Necrotizing Enterocolitis
Anesthetic agents used in the Tx of Necrotizing Enterocolitis include:
Ketamine 0.5-1ug/kg every 20-30” to start
Fentanyl may be added if condition improves
Small doses of volatile agent if dramatic improvement
Intra-abdominal Malformations - Necrotizing Enterocolitis
Which Anesthetic agent must be avoided in the Tx of Necrotizing Enterocolitis include:
N2O
Nitrous oxide
Intra-abdominal Malformations - Intestinal Obstruction
The congenital absence or complete closure of a portion of the lumen of the duodenum that causes increased levels of amniotic fluid during pregnancy (polyhydramnios) and intestinal obstruction in newborn babies is also known as:
Duodenal atresia
Upper GI tract obstruction - Duodenal atresia
When does Duodenal atresia become evident?
Within first 24 hrs of life when feeding is initiated
Upper GI tract obstruction - Duodenal atresia
Clinical features of Duodenal atresia include:
Bile-stained emesis
Upper abdominal distention
Increased volume of gastric aspirate
Associated with trisomy 21 and other intestinal malformations
Upper GI tract obstruction - Duodenal atresia
Anesthetic considerations/management w/ Duodenal atresia
Risk of aspiration: RSI
Ensure adequate relaxation for exploration
Preferred technique: combined GETA with Caudal
Extubation at end of surgery can be anticipated
When in doubt, leave ET in with PEEP
Upper GI tract obstruction - Duodenal atresia
What does Duodenal atresia look like?
See picture
Note “Double bubble effect”
Upper GI tract obstruction - Duodenal atresia
Another look of what Duodenal atresia looks like:
See picture
Intestinal Obstruction
Possible Lower GI tract obstructions include:
Terminal ileum
Imperforate anus
Intestinal Obstruction - Lower GI tract obstructions
When do Lower GI tract obstructions become evident?
Between 2 & 7 days of age
Intestinal Obstruction - Lower GI tract obstructions
Clinical features of Lower GI tract obstructions include:
Progressive distention
Little or no stool passed
Vomiting
Fluid/electrolyte abnormalities
Enormous amount fluids sequestered into intestinal tract
Intestinal Obstruction - Lower GI tract obstructions
Anesthetic considerations/management w/ Lower GI tract obstructions include:
Correct F/E deficits
Na+ : >130 - U/O: 1-2 ml/kg/hr
Avoid N2O
Provide adequate muscle relaxation
Accomplished with various anesthetic techniques
Extubation criteria same as upper GI obstruction
Pediatric condition that can cause indubation issues
Small/ underdeveloped mandible (micrognathia or mandibular hypoplasia - can be used interchangeably), a tongue that falls back and downwards (glossoptosis) when supine, a/w Cleft palate, neonate w/ this condition often requires intubation for airway protection. The condition is also known as:
Pierre-Robin Syndrome
Pediatric condition that can cause indubation issues
Condition characterized by Small mouth, Small/ underdeveloped mandible, Nasal airway is blocked by tissue (choanal atresia), Ocular and auricular anomalies. This condition is also known as:
Treacher-Collins Syndrome
Pediatric condition that can cause indubation issues
Condition characterized by Small mouth, Large tongue, Atlantoaxial instability, Small subglottic diameter (subglottic stenosis). This condition is also known as:
Down Syndrome or Trisomy 21
Down Syndrome or Trisomy 21
Airway characteristics in Down Syndrome or Trisomy 21
Small mouth
Large tongue
Palate is narrow with a high arch
Midface hypoplasia
Atlantoaxial instability
C1 & C2 subluxation
Use glidescope
Avoid neck flexion during laryngoscopy
Child should receive cervical x-ray screening between 3-5 years of age
Subglottic stenosis
Increased risk of postintubation croup
Use a smaller ETT
Obstructive sleep apnea on extubation
Chronic pulmonary infection
Down Syndrome or Trisomy 21
CV issues a/w Down Syndrome or Trisomy 21
Co-existing congenital heart disease is common
Most common = AV septal defect (endocardial cushion defect)
Second most common = VSD
Bradycardia is very common during sevoflurane induction (tx = anticholinergic)
Low levels of circulating catecholamines
Down Syndrome or Trisomy 21
What’s the Most common CV issues a/w Down Syndrome or Trisomy 21
AV septal defect
(endocardial cushion defect)
Down Syndrome or Trisomy 21
Other physiologic issues a/w Down Syndrome or Trisomy 21
Intellectual disability
Epilepsy
Strabismus
Low muscle tone (hypotonia)
Hyperflexible joints (careful with positioning)
GERD
Thyroid disease
Increased incidence of leukemia
Pediatric condition that can cause indubation issues
Condition characterized by, Small/ underdeveloped mandible, Cervical spine abnormality
Goldenhar syndrome
Start w/ glidescope to minimize amount of neck mobility
Pediatric condition that can cause indubation issues
Condition characterized by large tongue
Beckwith Syndrome
Pediatric condition that can cause indubation issues
Condition charaterized by Small/ underdeveloped mandible, Laryngomalacia, Strider
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