Mod VI: Intra-abdominal Malformations

Question 1

_{Intra-abdominal Malformations}

Faulty separation of primitive trachea and esophagus (commonly occur together)

Answer 1

Esophageal Atresia & Tracheoesophageal Fistula

Question 2

_{Esophageal Atresia & Tracheoesophageal Fistula}

Incidence of Esophageal Atresia & Tracheoesophageal Fistula:

Answer 2

1:4,000 live births

Question 3

_{Esophageal Atresia & Tracheoesophageal Fistula}

What’s the Most common form of Esophageal Atresia & Tracheoesophageal Fistula?

Answer 3

Type IIIB

Location of fistula variable

Question 4

_{Esophageal Atresia & Tracheoesophageal Fistula}

Clinical presentation of Esophageal Atresia & Tracheoesophageal Fistula

Answer 4

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

Question 5

_{Esophageal Atresia & Tracheoesophageal Fistula}

Anomalies associated with Esophageal Atresia & Tracheoesophageal Fistula: VACTERL

Answer 5

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

Question 6

_{Esophageal Atresia & Tracheoesophageal Fistula}

Picture showing the different types of Esophageal Atresia & Tracheoesophageal Fistula

Answer 6

_{See picture}

Note Type IIIB, the most common presentation

Question 7

_{Esophageal Atresia & Tracheoesophageal Fistula}

Treatment of Esophageal Atresia & Tracheoesophageal Fistula:

Answer 7

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}

Question 8

_{Esophageal Atresia & Tracheoesophageal Fistula}

Primary surgery for Esophageal Atresia & Tracheoesophageal Fistula involves:

Answer 8

Ligation of fistula with esophageal anastomosis

Question 9

_{Esophageal Atresia & Tracheoesophageal Fistula}

Staged surgery for Esophageal Atresia & Tracheoesophageal Fistula involves:

Answer 9

Gastrostomy with fistula diversion,

_{and later}

Repair of esophagus

Question 10

_{Esophageal Atresia & Tracheoesophageal Fistula}

Anesthetic considerations/management during Induction w/ Esophageal Atresia & Tracheoesophageal Fistula

Answer 10

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}

Question 11

_{Esophageal Atresia & Tracheoesophageal Fistula}

Anesthetic considerations/management during ET tube placement w/ Esophageal Atresia & Tracheoesophageal Fistula

Answer 11

Difficult if TEF present

Goal: Below fistula and above carina

1st = mainstem right bronchus

2nd= withdrawal ET slowly until BBS heard over L thorax

Question 12

_{Esophageal Atresia & Tracheoesophageal Fistula}

Anesthetic considerations/management during Maintenance w/ Esophageal Atresia & Tracheoesophageal Fistula

Answer 12

Inhalation anesthetic with SV until gastrostomy performed

Monitor inspiratory pressures: Avoid High!

Correct F/E disturbances/cont’d resuscitation efforts

Question 13

_{Esophageal Atresia & Tracheoesophageal Fistula}

ET tube placement in Esophageal Atresia & Tracheoesophageal Fistula

Answer 13

See picture

Question 14

_{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:

Answer 14

Omphalocele

Viscera outside abdominal wall

Intact membrane (amnion)

Question 15

_{Intra-abdominal Malformations - Omphalocele}

Incidence of Omphalocele:

Answer 15

1:6,000

Question 16

_{Intra-abdominal Malformations - Omphalocele}

Associated congenital anomalies w/ Omphalocele

Answer 16

Cardiac lesions (20%)

Exstrophy bladder

Beckwith-Wiedemann syndrome

^{(mental retardation, hypoglycemia, congenital heart dx, large tongue)}

Question 17

_{Intra-abdominal Malformations - Omphalocele}

What does Omphalocele look like at birth?

Answer 17

See picture

Question 18

_{Intra-abdominal Malformations}

A defect of abdominal wall on right lateral aspect of umbilicus w/ failed closure @ 12-18 weeks gestation is known as:

Answer 18

Gastroschisis

Lacks peritoneal coverage, exposed bowel

Highly susceptible to ECF loss and infection

Usually lateral to umbilicus

Question 19

_{Intra-abdominal Malformations - Gastroschisis}

Incidence of Gastroschisis:

Answer 19

1:30,000

Question 20

_{Intra-abdominal Malformations - Gastroschisis}

Why is Gastroschisis more urgent of a surgery?

Answer 20

Risk of fluid loss!!!

Lacks peritoneal coverage, exposed bowel

Highly susceptible to ECF loss and infection

Question 21

_{Intra-abdominal Malformations - Gastroschisis}

T/F: Gastroschisis is a/w Less incidence of concurrent anomalies

Answer 21

True

Although it is associated with prematurity

Question 22

_{Intra-abdominal Malformations - Gastroschisis}

What does Gastroschisis look like?

Answer 22

See picture

Question 23

_{Omphalocele & Gastroschisis}

Goals of Medical stabilization w/ Omphalocele & Gastroschisis include:

Answer 23

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

Question 24

_{Omphalocele & Gastroschisis}

How should the viscera of Omphalocele be protected before surgical repair in order to prevent increased heat loss?

Answer 24

Cover sac with sterile, warm, saline soaked gauze

Question 25

_{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?

Answer 25

Apply warm Saline soaked gauze to exposed viscera Wrap infant in warm Sterile towels

Question 26

_{Omphalocele & Gastroschisis} For Surgical repair of Omphalocele & Gastroschisis, hat are 2 major concerns with Primary closure?

Answer 26

**Ventilation** **Circulation**

Question 27

_{Omphalocele & Gastroschisis} For Surgical repair of Omphalocele & Gastroschisis and after primary closure, when is _reopening_ and _staged procedure_ indicated?

Answer 27

_Intragastric pressures_ **\> 20 mmHg** 24 hrs after primary closure

Question 28

_{Omphalocele & Gastroschisis} For Surgical repair of Omphalocele & Gastroschisis, when is Staged procedure indicated? Describe it!

Answer 28

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

Question 29

_{Omphalocele & Gastroschisis} Silo size reduced every 2-3 days, pushing bowel back inside the abdomen

Answer 29

See picture

Question 30

_{Omphalocele & Gastroschisis} Silo size reduced every 2-3 days, pushing bowel back inside the abdomen

Answer 30

See picture

Question 31

_{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

Answer 31

See picture

Question 32

_{Omphalocele & Gastroschisis} Anesthetic considerations/management w/ Omphalocele & Gastroschisis:

Answer 32

**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**

Question 33

_{Omphalocele & Gastroschisis} Summary table

Answer 33

See picture

Question 34

_{Intra-abdominal Malformations} Hypertrophy of pyloric smooth muscle → gradual obstruction of gastric outlet. This condition is also known as:

Answer 34

**Pyloric Stenosis**

Question 35

_{Intra-abdominal Malformations - Pyloric Stenosis} When does Pyloric Stenosis present?

Answer 35

**2nd or 3rd** week of life

Question 36

_{Intra-abdominal Malformations - Pyloric Stenosis} Clinical presentation of Pyloric Stenosis

Answer 36

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

Question 37

_{Intra-abdominal Malformations - Pyloric Stenosis} GI assessment of Pyloric Stenosis

Answer 37

Palpable abdominal **mass** (_“olive”_) **Gastric dilation** via X-ray

Question 38

_{Intra-abdominal Malformations - Pyloric Stenosis} Metabolic disturbance a/w Pyloric Stenosis include:

Answer 38

**Hyponatremic** _Hypokalemic hypochloremic metabolic alkalosis_ with compensatory *_respiratory acidosis_*

Question 39

_{Intra-abdominal Malformations - Pyloric Stenosis} Confirmation of Pyloric Stenosis diagnosis

Answer 39

US Barium swallow

Question 40

_{Intra-abdominal Malformations - Pyloric Stenosis} What does Pyloric Stenosis look like?

Answer 40

See picture Note enlarge pylorus causing the stenosis

Question 41

_{Intra-abdominal Malformations - Pyloric Stenosis} T/F: _Pyloric Stenosis_ is a surgical emergency

Answer 41

**False** Pyloric Stenosis is a **Medical Emergency** = NOT Surgical

Question 42

_{Intra-abdominal Malformations - Pyloric Stenosis} Correction of F/E deficits in the medical management of Pyloric Stenosis involves:

Answer 42

Resuscitate with full-strength **BSS** (NS) **Add K+** after infant begins to _urinate_

Question 43

_{Intra-abdominal Malformations - Pyloric Stenosis} Which lab values indicate that the infant is adequately prepared and medically optimized for the OR?

Answer 43

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

Question 44

_{Intra-abdominal Malformations - Pyloric Stenosis} Surgical repair of Pyloric Stenosis inculde:

Answer 44

**Pyloromyotomy** Short procedure/small incision

Question 45

_{Intra-abdominal Malformations - Pyloric Stenosis} Anesthetic considerations/management to address ↑Risk for aspiration w/ Pyloric Stenosis include:

Answer 45

**Awake OG/NGT suction** in _supine_ & _left lateral position_ **RSI** or **AFOI**

Question 46

_{Intra-abdominal Malformations - Pyloric Stenosis} Muscle relaxation required at 2 points during surgery to correct Pyloric Stenosis. What are those two points?

Answer 46

_Delivery of pylorus_ at beginning _Return of pylorus_ at end of surgical procedure

Question 47

_{Intra-abdominal Malformations - Pyloric Stenosis} Anesthsia technique: Controlled ventilation or Caudal?

Answer 47

**Caudal** provides _needed muscle relaxation_, reduces _anesthetic requirements_, & provides postoperative _analgesia_

Question 48

_{Intra-abdominal Malformations - Pyloric Stenosis} T/F: Deep extubation is recommended for infants after surgery to correct Pyloric Stenosis

Answer 48

**False** Infant should be fully awake and breathing adequately before removing ETT

Question 49

_{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:

Answer 49

**Intestinal Malrotation** & **Volvulus**

Question 50

_{Intra-abdominal Malformations - Intestinal Malrotation & Volvulus} Incidence of Intra-abdominal Malformations:

Answer 50

**1:500** live births

Question 51

_{Intra-abdominal Malformations - Intestinal Malrotation & Volvulus} What's the only definitive treatment for Intestinal Malrotation & Volvulus?

Answer 51

**Surgery** provides only definitive treatment _Midgut volvulus_ is a **Surgical Emergency**

Question 52

_{Intra-abdominal Malformations - Intestinal Malrotation & Volvulus} Anesthetic Considerations w/ Intestinal Malrotation & Volvulus:

Answer 52

Increased risk for aspiration ^{Decompress stomach} Keep hydrated Invasive lines may be necessary

Question 53

_{Intra-abdominal Malformations} T/F: Necrotizing Enterocolitis may appear in the absence of functional or anatomical lesions

Answer 53

**True**

Question 54

_{Intra-abdominal Malformations - Necrotizing Enterocolitis} Incidence of Necrotizing Enterocolitis - Age group most affected:

Answer 54

**5-15 %** of infants Predominantly in **_premature infants_** (90%)

Question 55

_{Intra-abdominal Malformations - Necrotizing Enterocolitis} Mortality rate in Necrotizing Enterocolitis:

Answer 55

**40%**

Question 56

_{Intra-abdominal Malformations - Necrotizing Enterocolitis} Pathogenesis of Necrotizing Enterocolitis:

Answer 56

Immature intestine has _↓ability to absorb substrates_→ **stasis** → **_bacterial infection_**→ ischemia→ necrosis of intestinal mucosa → **perforation,** _gangrene_**, fluid loss,** _peritonitis_**, septicemia, DIC**

Question 57

_{Intra-abdominal Malformations - Necrotizing Enterocolitis} Clinical features of Necrotizing Enterocolitis:

Answer 57

Abdominal distention/ileus ↑gastric aspirate Bloody stools Temperature instability Cardiorespiratory instability Hypovolemia/oliguria Metabolic acidosis

Question 58

_{Intra-abdominal Malformations - Necrotizing Enterocolitis} How does Necrotizing Enterocolitis look like?

Answer 58

See picture

Question 59

_{Intra-abdominal Malformations - Necrotizing Enterocolitis} Treatment for Necrotizing Enterocolitis is primarily medical and includes:

Answer 59

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**

Question 60

_{Intra-abdominal Malformations - Necrotizing Enterocolitis} How is Necrotizing Enterocolitis Nonresponsive to medical treatment managed?

Answer 60

**Exploratory laparotomy** to remove gangrenous bowel

Question 61

_{Intra-abdominal Malformations - Necrotizing Enterocolitis} Anesthetic considerations/management w/ Necrotizing Enterocolitis

Answer 61

_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%**

Question 62

_{Intra-abdominal Malformations - Necrotizing Enterocolitis} Anesthetic agents used in the Tx of Necrotizing Enterocolitis include:

Answer 62

_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

Question 63

_{Intra-abdominal Malformations - Necrotizing Enterocolitis} Which Anesthetic agent must be avoided in the Tx of Necrotizing Enterocolitis include:

Answer 63

**N₂O** Nitrous oxide

Question 64

_{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:

Answer 64

**Duodenal atresia**

Question 65

_{Upper GI tract obstruction - Duodenal atresia} When does Duodenal atresia become evident?

Answer 65

Within first **24 hrs** of life when feeding is initiated

Question 66

_{Upper GI tract obstruction - Duodenal atresia} Clinical features of Duodenal atresia include:

Answer 66

**Bile-stained** emesis Upper abdominal **distention** Increased **volume** of gastric aspirate Associated with **trisomy 21** and other intestinal malformations

Question 67

_{Upper GI tract obstruction - Duodenal atresia} Anesthetic considerations/management w/ Duodenal atresia

Answer 67

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**

Question 68

_{Upper GI tract obstruction - Duodenal atresia} What does Duodenal atresia look like?

Answer 68

See picture Note "Double bubble effect"

Question 69

_{Upper GI tract obstruction - Duodenal atresia} Another look of what Duodenal atresia looks like:

Answer 69

See picture

Question 70

_{Intestinal Obstruction} Possible Lower GI tract obstructions include:

Answer 70

Terminal ileum **Imperforate anus**

Question 71

_{Intestinal Obstruction - Lower GI tract obstructions} When do Lower GI tract obstructions become evident?

Answer 71

Between **2 & 7 days** of age

Question 72

_{Intestinal Obstruction - Lower GI tract obstructions} Clinical features of Lower GI tract obstructions include:

Answer 72

Progressive **distention** Little or **no stool** passed **Vomiting** **Fluid/electrolyte** abnormalities Enormous amount fluids sequestered into intestinal tract

Question 73

_{Intestinal Obstruction - Lower GI tract obstructions} Anesthetic considerations/management w/ Lower GI tract obstructions include:

Answer 73

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

Question 74

_{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:

Answer 74

**Pierre-Robin Syndrome**

Question 75

_{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:

Answer 75

**Treacher-Collins Syndrome**

Question 76

_{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:

Answer 76

**Down Syndrome** or Trisomy 21

Question 77

_{Down Syndrome or Trisomy 21} Airway characteristics in Down Syndrome or Trisomy 21

Answer 77

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

Question 78

_{Down Syndrome or Trisomy 21} CV issues a/w Down Syndrome or Trisomy 21

Answer 78

Co-existing **congenital heart disease** is common Most common = **AV septal defec**t (endocardial cushion defect) Second most common = **VSD** **Bradycardia** is very common during sevoflurane induction (tx = anticholinergic) Low levels of circulating **catecholamines**

Question 79

_{Down Syndrome or Trisomy 21} What's the Most common CV issues a/w Down Syndrome or Trisomy 21

Answer 79

**AV septal defect** | (endocardial cushion defect)

Question 80

_{Down Syndrome or Trisomy 21} Other physiologic issues a/w Down Syndrome or Trisomy 21

Answer 80

Intellectual disability Epilepsy Strabismus Low muscle tone (hypotonia) Hyperflexible joints (careful with positioning) GERD Thyroid disease Increased incidence of leukemia

Question 81

_{Pediatric condition that can cause indubation issues} Condition characterized by, Small/ underdeveloped mandible, Cervical spine abnormality

Answer 81

**Goldenhar syndrome** Start w/ glidescope to minimize amount of neck mobility

Question 82

_{Pediatric condition that can cause indubation issues} Condition characterized by large tongue

Answer 82

**Beckwith Syndrome**

Question 83

_{Pediatric condition that can cause indubation issues} Condition charaterized by Small/ underdeveloped mandible, Laryngomalacia, Strider

Answer 83

**Cri du Chat**