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General anesthesia + regional anesthesia (spinals, etc.) (at the same time) =

Medico-legally a bad idea (more dangerous as pt unable to make a response if cord is hit by needle) but the pediatric world of anesthesia argues with this.

The most common method of regional anesthesia in pediatrics

"Kiddie Caudal"

Dose (concentration & volume) for "kiddie caudal" & expected effect

Concentration: 1/4% Bupivicaine (2.5 mg/mL)
Volume: (more than adult because tissue not so densely packed)
-0.5 mL/kg for belly-button level
-1 mL/kg for nipple line

=Will suppress pain but not adequate to suppress movement

Why would you be cautious when placing needle for a "kiddie caudal"?

-Cord may have not yet moved up in the spinal column or could even be tethered

Condition characterized by an incomplete diaphragm that allows abdominal organs (mostly intestines) to push up into thorax

Congenital Diaphragmatic Hernia (CDH)

Condition characterized by organs (intestines, etc.) protruding through the abdominal wall that are covered by a protective sac

Omphalocele

Condition characterized by organs (intestines, etc.) protruding through the abdominal wall (no protective sac)

Gastrochisis

Condition characterized by failed vertebral column formation that results in a sac-like protrusion of spinal cord and membrane

Meningomyelocele

Condition characterized by failed vertebral column formation that results in a sac-like protrusion of membrane but no neural tissue

Meningocele

Gestation week # when diaphragm normally forms and divides the chest from the abdomen

5-10 weeks

Incidence of congenital diaphragmatic hernia (CDH)

1:4000 live births
1:2000 (total including still births)

Incidence of Left vs Right sided congenital diaphragmatic hernia (CDH)

Left: 90% (Bochdalek)
Right: 10% (Morgagni)

Mortality of congenital diaphragmatic hernia (CDH)

40-50% (25% if born alive)

Names for L & R congenital diaphragmatic hernia (CDH) foramen

Left: Bochdalek (90% of CDH)
Right: Morgagni

3 consequences of a loss of space within the thoracic cavity caused by a congenital diaphragmatic hernia (CDH)

-Pulmonary hypoplasia (underdeveloped lung)
-Hyper-reactive pulmonary vasculature (?)
-LV hypoplasia (underdeveloped heart)

Clinical symptoms of congenital diaphragmatic hernia (CDH) (x3)

-Respiratory insufficiency
-↓ breath sounds (usually on L) [also bowel sounds in chest]
-Scaphoid abdomen (abdomen sucked inwards)

Why a "scaphoid" abdomen makes repairing a congenital diaphragmatic hernia (CDH) difficult

After moving intestines, etc. back into abdomen, there may not be enough tissue to close abdomen & may need to stage the repair.

(scaphoid abdomen = belly sucked inwards because some abdominal organs up in chest)

Is airway protection a concern with congenital diaphragmatic hernia (CDH) & why

Airway management is important to protect them from aspirating (only 1 good lung usually)

How to ventilate a newborn during a congenital diaphragmatic hernia (CDH) repair (x3)

-Permissive hypercapnia (PaCO₂ to 60-65 mmHg)
-Peak inspiratory pressures less than 35 cmH₂O
-Arterial saturation (preductal) can go as low as 90%

How to measure a preductal arterial saturation

Sample blood from the right arm

(1st aortic bifurcation is innominate which supplies R carotid & subclavian & carries blood to the arm that has not had a chance to mix with blood from the ductus arteriosus)

How to set peak inspiratory pressure on a newborn with congenital diaphragmatic hernia (CDH)

Keep PIP [peak inspiratory pressure] less than 35 cm H₂O
(Protect good lung from Barotrauma)

Method to help relieve intrathoracic pressure for congenital diaphragmatic hernia & why (x2)

Decompress stomach
-less intrathoracic pressure because less abdominal pressure = more lung expansion
-also makes it easier to close abdomen at end of repair surgery

There is a high suspicion for ? with congenital diaphragmatic hernia repair

Suspect pneumothorax

How to help prevent pulmonary hypertension with congenital diaphragmatic hernia (CDH) (x2)

Avoid hypothermia & acidosis

(they precipitate pulmonary hypertension which constricts pulmonary circulation & may be the final straw to put them over the edge)

Anesthesia agents to use (x4) for a congenital diaphragmatic hernia (CDH) repair & 1 agent to avoid

-Inhalation agents as tolerated
-Narcotics OK (use age-appropriate)
-Use muscle relaxants (will help when closing abdomen)
-Consider regional pain management if early extubation possible
-*Avoid Nitrous Oxide (closed air space)

How urgent is a congenital diaphragmatic hernia repair

Not a surgical emergency, medical stabilization prior to OR

Anesthesia concerns with congenital diaphragmatic hernia (x7)

-Airway (aspiration = bad)
-Barotrauma (keep PIP < 35 mmH₂O)
-Overventilation (PaO₂ of 90%, PaCO₂ 60 ok)
-Pneumothorax (high incidence intraop)
-Pulm HTN (avoid acidosis or hypothermia which make worse)
-Nitrous oxide = closed air space problem
-Abdominal/thoracic cavity pressures (decompress stomach)

What is eventration in relation to the diaphragm

Incomplete muscularization of the diaphragm (also possibly related to denervation [atony]) that results in a hernia sac of intra-obdominal pressure into the thorax.

When does a problem in fetal development occur that results in oomphalocele & what happens?

5-10th week
-abdominal contents fail to return back into abdomen

2 outwardly visible characteristics of oomphalocele

-abdominal contents covered by membrane (amnion)
-umbilical cord at the apex of the sac

This malformation frequently has associated congenital anomalies

Oomphalocele (25% occurance of congenital heart disease)

A major associated congenital anomaly with oomphalocele & incidence

25% risk for congenital heart disease

Conditions associated with Beckwith-Wiedemann syndrome (x5)

-Oomphalocele
-Congenital heart disease
-Large tongue
-Mental retardation
-*Hypoglycemia (may be on test)

This condition is characterized by:
-Oomphalocele
-Congenital heart disease
-Large tongue
-Mental retardation
-Hypoglycemia

Beckwith-Wiedemann syndrome

When does a problem in fetal development occur that results in gastrochisis & what happens?

-Interruption of omphalomesenteric artery leads to ischemia & atrophy at base of umbilical cord & gut herniates
-Occurs after abdominal contents return to abdomen (after week 10)

2 outwardly visible characteristics of gastrochisis

-Not covered by membrane
-Umbilical cord found to one side of contents

Is gastrochisis associated with other congenital anomalies?

No

Problems associated with gastrochisis (x2)

-High risk of infection
-Associated with other problems with the GI tract

A high alpha-fetoprotein (AFP) would indicate

Failure of closure of abdominal wall or neural tube

Urgency of gastrochisis vs omphalocele

Gastrochisis is more urgent (infection risk & fluid loss)

Preop care for gastrochisis (x5)

-ABCs
-Bagging abdominal contents in "silo"
-Prevent infection
-Watch fluids & electrolytes
-CVP, a-line possibly

Preop care for oomphalocele (x5)

-ABCs
-Search for associated anomalies (Cong. H. Defect, etc.)
-Prevent respiratory failure (↑ intrathoracic pressure d/t contents being enclosed by membrane + possible heart defect)
-Watch fluids/electrolytes
-CVP, a-line possibly

Why HTN & lower extremity edema occur during & after repair of oomphalocele & gastrochisis

RAAS is intact in newborns:
-↑ intra-abdominal pressure ↓s renal blood flow stimulating release of renin = HTN + edema

Anesthesia drugs for oomphalocele or gastrochisis repair (x3)

(similar to an open abdomen case)
-Fluid management
-Give muscle relaxants (abdominal relaxation for closure)
-No nitrous oxide

Monitoring during an oomphalocele or gastrochisis repair (x3)

-*Lower extremity NBP & pulse oximiter
(in case too much intra-abdominal pressure when trying to close cause obstruction of arterial supply to legs)
-Arterial line
-Central venous line

Description & timing of failure in fetal development that leads to a tracheoesophageal fistula

-Failure of the lung bud to separate from the foregut at 4-6 weeks.

Incidence of tracheoesophageal fistula

1:3000 live births

! Incidence of congenital anomalies associated with tracheoesophageal fistulas

50% (VATER or VACTERL)

Congenital anomalies associated with tracheoesophageal fistulas (x6)

V: Vertebral defects
A: Anal defects (atresia)
C: Cardiac (15-20%)
TE: TE fistula
R: Renal anomalies
L: Limb (radial)

(Also VATER)

Incidence of congenital heart defects with tracheoesophageal fistulas

15-20% (25-30% in another part of notes)

! What is the most common type of TE fistula

Gross type C (87%)
=Esophageal atresia with distal TEF (just above carina)

Define polyhydramnios & how does it relate to TE fistula?

Excessive amniotic fluid
-can be caused by inability for fetus to swallow amniotic fluid when a TE fistula is present

Clinical symptoms of a TE fistula (x3)

-Drooling
-Coughing / choking
-Cyanotic spells
(especially if it occurs after 1st feed)

! Major preoperative concerns for TE fistula (x2)

Aspiration
Dehydration

Most important to anesthesia in regards to a TE fistula repair

*Don't ventilate the stomach

! Boards answer for induction / intubation for TE fistula repair & more practical method

Boards:
-Spontaneous breathing, surgeon opens chest & ligates fistula, then you take over breathing (easier said than done)
More practical:
-Rapid sequence intubation, R mainstem the tube then come up so tip is just above carina (most fistulas above carina)

What type of ETT to use during TE fistula repair

Magill tube (uncuffed) with no Murphy's eye

Where will incision be for TE fistula repair

Thoracotomy on side opposite the aortic arch

Why early extubation helpful for TE fistula repair

Positive pressure ventilation can cause "suture stress" on the newly repaired & friable esophageal tissue

Describe a 1-stage vs 2-stage repair of TE fistula

1-stage: thoracotomy with ligation of fistula & anastomosis of esophagus
2-stage (not enough esophagus): ligate fistula & exteriorize pouch until 9 kg or 12 months

Incidence of meningomyelocele

1:1000

Description & timing of failure in fetal development that leads to a meningomyelocele

Failure of neural tube closure during 4th week of gestation

Most common location for meningomyelocele

Lumbosacral or sacral

Arnold-Chiari malformation definition & classes

Condition in which the cerebellum protrudes into the spinal canal
Type 1: adulthood
Type 2: meningomyelocele
Type 3: cervical meningocele
Type 4: hypoplasia w/o herniation

!What is significant about an Arnold-Chiari type 2 malformation

Associated with myelomeningocele

Relationship of meningomyelocele & hydrocephalus

Hydrocephalus requiring shunting occurs 90% with meningomyelocele

Arnold Chiari Type 1 Malformation characteristics (x3)

-Causes headaches, vomiting, difficulty swallowing, hoarseness
-Occurs in young adults
-Syrinx formation (syringohydromyelia) = fluid cavity in spinal cord

2 secondary issues from myelomeningocele

-Paralysis of legs & (less commonly) arms
-Hydrocephalus (can cause mental impairment)

Term for a fluid collection (tubular cyst) in the spinal cord

Syrinx
(syringohydromyelia is associated with an Arnold-Chiari type 1 malformation in most cases)

Causes of syringomyelia (x6)

-Arnold-Chiari malformation
-hydrocephalus
-tethered spinal cord
-trauma
-tumor
-idiopathic (a very large number of them are idiopathic)

Define Syringomyelia

A disorder in which a cyst (syrinx) forms within the spinal cord which expands and elongates over time, destroying the center of the spinal cord

A term associated with: tight filum terminale, lipomeningomyelocele, split cord malformations, dermal sinus tracts, demoids, and cystoceles

Tethered spinal cord

Define tethered spinal cord

A tugging of the spinal cord at the base of the spinal canal

Why meningomyelocele is repaired usually within first 72 hours of life (x2)

Risk of ventriculitis or progressive neurologic defect

Meningomyelocele often requires an additional surgery for

Shunt for hydrocephalus

Preop care for meningomyelocele (x5)

-Prevent infection (antibiotics & keep covered)
-Prevent trauma to malformation (prone position, "donut")
-Fluid management (replace CSF leak with saline)
-Look for other congenital anomalies
-Latex precautions

How to replace leaking CSF for a meningomyelocele

With saline

Intraoperative care for meningomyelocele (x6)

-Latex precautions
-Positioned prone for surgery with "donut"
-Minimize abdominal pressure (impairs venous return)
-Succinylcholine ok (but avoid if nerve stimulation to be used)
-Watch temperature & monitors

Postop concerns for meningomyelocele (x3)

-Vent as needed
-Watch for brain stem issues?
-Watch for hyrdocephalus issues?

Term for a fault in the spinal column in which one or more vertebrae fail to form properly leaving a gap or split

Spina bifida

3 types of spina bifida

-Spina bifida occulta (hidden)
-Spina bifida cystica (meningocele & meningomyelocele)
-Cranium bifida (encephalocele, anencephaly & iniencephaly [chin & back of head fused to body & face upward])

A sac that forms in cranium bifida is known as

an encephalocele (contains tissue, CSF & sometimes part of brain)

The 2 most severe forms of cranium bifida

anencephaly (no cranium)
iniencephaly (chin & base of head fused to body)

What is the most common cause of intestinal obstruction in children between 3 months & 6 years of age?

Intussusception

3 types of intussusception & which is most common

-Ileocolic (most common)
-Illeoileal
-Colocolic

Symptoms related to intussusception (x6)

-Sudden onset
-Colicky (knee-chest position then playing with toys)
-N/V
-Fever
-Bloody stools (currant jelly)
-Distended abdomen

2 diseases that can cause intussusception

-Cystic Fibrosis (thick secretions stick to wall)
-Henoch-Schonlein purpura (hematoma in intestine)

Intussusception treatments (x2)

-Enema in radiology (reduction) (for pediatrics mostly)
-Surgery (most adults)

Air vs liquid enemas for reduction of intussusception

Air is better than liquid enemas
-Higher success rates
-Fewer perforations (2 in 1000 vs 25 in 1000)

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