Cases in
Claude Abdallah, MD, MSc
Children’s National Medical Center
Washington DC
On Call
A newborn with a
combination of congenital
Abdallah, C. et al.: Management of a neonate with a rare combination of unrepaired
major malformations. Annual Meeting of the American Society of Anesthesiologists, San
Francisco, CA.
Case Presentation
 One day old female transferred from another hospital.
 Called to schedule ASAP for rigid bronchoscopy, gastrostomy
(concern of tracheoesophageal fistula), with possible closure
of an omphalocele.
 Admitted to NICU few hours ago:
o Fluid and electrolyte status optimized
(as per NICU)
o NPO and head elevated
o Bradycardia episodes resolving with stimulation
Embryologic process: Failure of
abdominal wall to develop.
Defect: Central defect;
Membranous sac covers the gut;
however, the sac may rupture.
(more frequent)
Incidence of prematurity: 30%
Associated congenital anomalies:
Embryologic process: Intrauterine
occlusion of the omphalo mesenteric
Defect: In abdominal wall lateral to
No sac covers intestine
Incidence: 1/15,000-1/30,000
Incidence of prematurity: 60%
Associated congenital anomalies: Rare
Anomalies Associated
with Omphalocele
• Gastrointestinal: Biliary atresia, imperforated anus.
• Cardiovascular: Tetralogy of Fallot, Septal defects.
• Urinary: Bladder extrophy, vesicointestinal fistula.
• Craniofacial: Cleft lip/palate-
• Oro/maxillary tumor--
Potential difficult
Differential Syndromes
Beckwith-Wiedemann syndrome:
(infantile gigantism)
- 50% born prematurely,
- Birth weight>4kg
- Macroglossia, exophtalmos
- Visceromegaly, omphalocele
- Congenital heart disease
- Neonatal hypoglycemia
Potentially difficult airway
( Pancreatic islet cell hyperplasia) Requires constant glucose infusion, with
close monitoring of blood glucose
- Medullary Renal dysplasia
Impaired renal drug excretion
- Polycythemia
Differential Syndromes
• Vater Syndrome or VACTERL
association: combines:
- V: Vertebral Anomalies
- A: Imperforate Anus/ Anal
- C: Congenital heart disease
(VSD usually)
- TE: Tracheo-esophageal fistula
- R: Renal dysfunction
- L: Limb defects, Absent radius
Differential Syndromes
Pentalogy of Cantrell:
Association of:
Defects in abdominal wall
Diaphragmatic abonormalities: Hernia
Sternal deformities
Cardiac and Pericardial defects
Request Cardiac
Hypoplastic LV, coarctation of the
aorta with hypoplastic transverse
arch, hypoplastic anomalous left
pulmonary artery branch,
hypoplastic mitral valve, large
atrial septal defect and patent
ductus arteriosus: Hypoplastic Left
Heart Syndrome: HLHS
Cardiac Anesthesia Considerations
 The neonate’s survival is
dependent upon ductal
patency, as mixed blood in
the RV is ejected out the
single great vessel (PA) to
both the lungs and
(through the PDA) the
systemic circulation.
 Severe congestive failure
may be present.
The right ventricle functions as the systemic ventricle,
• The ratio of pulmonary to systemic blood flow depends on the
balance between systemic vascular resistance (SVR) and
pulmonary vascular resistance (PVR). PVR is affected by
changes in Paco2, Pao2, acid-base status, body temperature,
and lung volumes.
• Insure patency of the ductus arteriosus
Cardiac Anesthesia Considerations
 Maintenance of cardiac output and oxygen delivery
o Inotropic support, if needed
o Blood transfusion
 Meticulous administration of IV fluids
 Narcotic/air/oxygen technique
 Adequate muscle relaxation
Unusual Presentation: Defects Association
with TEF
Congenital diaphragmatic Hernia/ lung hypoplasia
Linked to genetic background (trisomy 13,18, deletion at chromosome
15q24-26) and environmental factors (insecticides), deficit in Vit A.
One Infant with
similar three midline
esophageal atresia,
fistula, tetralogy of
fallot. Pediatr. Surg. Int.
Anesthesia Considerations
Tracheoesophageal Fistula
Esophageal atresia with distal TEF
(close to the carina) is most common.
mask ventilation.
GOAL: Endotracheal tube (ETT) distal to the
fistula an above the carina: Place the ETT into
the right mainstem bronchus and slowly
withdrawing until bilateral breath sounds are
Aim is to avoid gastric distension,
which may decrease ventilation and
venous return to the heart.
Anesthesia Considerations
AVOID Aspiration pneumoniae (Cardiac!)
AVOID OG/NG suctioning (TEF!!)
Rapid sequence induction? Modified rapid
sequence induction ? Awake intubation?
Rigid BRONCHOSCOPY part with
spontaneous ventilation!
Induction Technique:
- Anesthetic plan to balance multiple organ system function
- Spontaneous ventilation
- General Anesthesia
- Difficulty monitoring level of anesthesia
- Titrate to effect, while avoiding coughing /movement and
laryngospam major consequences in this patient.
- Avoid urgent gastrostomy with present omphalocele
- Increased risk of pulmonary complications from aspiration
-Anesthetic plan to maintain hemodynamic stability,
• Antagonizes the NMDA receptor
central dissociation of the
cortex from the limbic system
good sedation and analgesia
while preserving upper airway
muscular tone and respiratory
• Relaxes the smooth musculature
of the airway .
• Increase the amount of oral
secretions (laryngospasm).
• Correlation of ketamine with
increased neuronal apoptosis
during rapid synaptogenesis
after birth.
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Safe hemodynamic Profile
Rapid onset and Recovery
Versatile: Short or prolonged effect
Titration for all levels of sedation as well as for general
• No side effects: Nausea/ Vomiting/ Delirium…..
• >DRUG OF CHOICE : MRI sedation with
spontaneous ventilation.
Delivery Method: Manual Titration Versus Infusion
Total Amount of Fluid Infused
Total Amount of Fluid Infused (ml/min)
*corrected to the time of infusion
*including volume of propofol infused
• The desired level of sedation was not
statistically significant between both
• Both infusion techniques, preserved
hemodynamic stability .
Other anesthesia agents
• Etomidate: Good Hemodynamic stability
?SuppressionAdrenal function
• Dexmedetomidine: Spontaneous Ventilation
? Hemodynamic stability
• Topical local anesthetics to vocal cords during
 IV induction with ketamine/propofol titration.
 Cover omphalocele with warm sterile saline-soaked gauze and plastic
 Maintain a neutral thermal environment
 Prevention of infection: High risk of sepsis would delay cardiac repair, and
increased mortality-- Aseptic technique + Strict Antibiotic regimen.
 Rigid bronchoscopy/ Endotracheal Intubation : Muscle relaxation
(rocuronium) and opioid based technique (fentanyl).
 Surgical Intent: Primary Closure of omphalocele
NO access to umbilical vessels
Arterial line- Baseline and Follow Up: Hematocrit/acid base
status/ Electrolytes:
 Avoid Hemoconcentration and Metabolic Acidosis: Albumin
Anesthesia Considerations
 Omphalocele closure (primary closure) effect on:
- pulmonary ventilation pressures
- venous return, cardiac output and other hemodynamic
parameters 1) Bowel ischemia and eventual wound
dehiscence 2) Renal compromise: Oliguria, HTN.
 Post operative mechanical ventilation management:
- At least 24-48 hours
- Immature lungs
- Limitation of PEEP application …
 Consider TPN ( even with gastrostomy, postoperative ileus)
Bronchoscopy revealed
complete tracheal rings below
the trachoesophgeal fistula
(TEF) and tracheal stenosis.
 Patient underwent a rigid
bronchoscopy, gastrostomy
and omphalocele closure.
 Stable to NICU
 A week later, a TEF,
tracheostomy and an open
heart surgery repair were
 For long term growth:
Gastrojejunostomy tube in
preparation for esophageal
atresia repair.
Post Anesthesia Care Unit
Review of the anesthesia
12 yrs. old female patient , 29 kgs,
Severe developmental delay, Severe scoliosis
ORIF of lower extremity fracture
Lennox-Gastaut syndrome (Difficult-to-treat form of
childhood- onset epilepsy, characterized by frequent and
different types of seizures.)
• Previous surgical history: Botox injections, G tube placement.
• Medications: Multivitamins, Valproic acid (Depakote) .
Intraoperative Course
• Easy mask inhalation, difficult IV insertion , maintenance with
sevoflurane , rocuronium , fentanyl 3mcg/kg and morphine 0.2mg/kg.
• Estimated blood loss intraoperative: 80 ml of blood loss.
• 500 cc of LR given.
• To Recovery Room, uneventful stay for >1 hour then patient became more
tired with discrepancy in blood pressure cuff measurements (100/5080/40-70/30 changed to LE 80/30…)
On assessment, patient was noticed to be pale with blood saturation
of surgical dressing
A bedside hemocue showed a hemoglobin of 4 g/dl.
Rescuscitation with albumin and PRBC transient improvement
followed by deterioration in vital signs, treated with vasopressors.
Central line placed.
Transferred to PICU.
In PICU, management necessitating supplementary investigative
laboratory testing along with intensive management with blood, blood
products and coagulation factors.
Patient subsequently recovered after 3 days and was discharged home.
Valproic acid
• Valproic acid (VPA) is one of the
most frequently prescribed
antiepileptic drugs.
• Effective in the management of
Lennox-Gastaut syndrome and
infantile spasms.
• Inhibition of repetitive firing of
neurons by blockade of voltagesensitive sodium channels,
increasing membrane potassium
• Increase GABA brain concentrations
(>synthesis , blocking conversion )
Abdallah C.: Valproic acid and acquired
coagulopathy. Ped. Anaesth. In print.
Acquired Coagulopthy
- Thrombocytopenia,
abnormal platelet
- Hypofibrinogenemia, and
decreased concentrations
of von Willebrand factor.
- Decreased factor VII and
VIII, XIII levels, Protein C,
and increased lipoprotein
(a) levels even during
short-term therapy (1).
- The incidence of
coagulation disorders
related to VPA in children:
4% -20.7% (2, 3).
1) J Child Neurol. 2009 Dec;24(12):1493-8
2) Epilepsia. 2006 Jul; 47(7):1136-43
3) J Child Neurol. 2002 Jan; 17(1):41-3
4) Epilepsia. 1992 Jan-Feb; 33(1):178-84.
• Valproate may cause a variety of laboratory abnormalities affecting
Always associated with thrombocytopenia? NO
• The mechanism of VPA- induced coagulopathy is not well identified
• Relationship between plasma VPA levels, duration of therapy and
incidence of VPA induced coagulopathy in patients receiving VPA not well
defined (VPA dose, length of treatment).
• Special attention to this side effect in the preoperative assessment would be
highly recommended
Acute Epiglottitis
• Inflammatory edema of the arytenoids, aryepiglottic
folds and the epiglottis; therefore, supraglottitis may be
used instead or preferred to the term acute epiglottitis.
• A late referral to an acute care setting with its serious
• Life-threatening disorder: Potential for laryngospasm
and irrevocable loss of the airway.
• Acute epiglottitis can occur at any age.
Incidence and Pathogens
• Used to be Hemophilus influenzae type B (Hib)
• Infection with group A b-hemolytic Streptococci has become more
frequent after the widespread use of Hemophilus influenzae vaccination.
• Incidence of acute epiglottitis in adults : 0.97 to 3.1 per 100,000.
Mortality of approximately 7.1%.
• The mean annual incidence of acute epiglottitis per 100,000 adults
significantly increased from 0.88 (from 1986 to 1990) to 2.1 (from 1991
to 1995) and to 3.1 (from 1996 to 2000). [2],[3]
• There is more diversity in the cause of epiglottitis in adults. with
often negative sputum cultures and negative blood cultures to Hib.
• Some cases of epiglottitis have been attributed to Candida spp.
• Noninfectious causes of epiglottitis may include trauma by foreign
objects, inhalation and chemical burns, thermal or caustic injury (
mental disorders or communication difficulties), or are associated
with systemic disease or reactions to chemotherapy.
• In young adults, acute epiglottitis has been described as being
caused by inhalation of heated objects when smoking illicit drugs.
Pediatric Patient
Review of epiglottitis admissions: 8-year retrospective (19982006):
- Epiglottitis continues to be a significant entity,
- Two uniquely vulnerable populations: Infants (<1 year
old) and the elderly (>85 years old).
-Examining the pediatric cohort of patients (patients
<18 years of age), 34.4% were <1 year of age.
- This category of age <1 year seemed to have increased
in frequency: 26.8% of pediatric patients in 1998 to 41.1% in
2006. [5]
- A case of epiglottitis with negative cultures has been
reported in a neonate within hours of birth. [6]
Differential Diagnosis
Viral laryngotracheobronchitis,
swelling of the mucosa in the
subglottic area of the larynx,
more prevalent during the
Croup has a more gradual onset
than acute epiglottitis,
commonly associated with lowgrade fever.
Same symptoms of inspiratory
stridor, suprasternal, intercostal and
substernal retractions and
differentiation in early illness is
possible by additional observation
of barking cough and absence of
drooling and dysphagia in croup.
• no seasonal predilection
• and by the additional
observation of drooling
and dysphagia with
absence of coughing in
• Additional reliable signs
of epiglottitis are a
preference to sit,
dysphagia and refusal to
The radiological "thumb sign" in
acute epiglottitis
Alphabet P sign" formed by acoustic
shadow of hyoid bone
" (HY), swollen
epiglottis (pointed by white arrows).
Hung TY et al. Am J Emerg Med. 2011; 29:359.
Inflammatory edema of the arytenoids, aryepiglottic folds and the epiglottis.
Tracheal intubation of a patient with epiglottitis
must be regarded as a potentially difficult
• Difficulty in breathing and stridor are common signs of
epiglottitis in children, but are less frequent in adults.
• The most common presenting symptom in adults is
odynophagia (100%), followed by dysphagia (85%) and
voice change (75%).
• In adults, stridor is regarded as a warning sign for
occlusion of the upper airway.
• Stridor, tachycardia, tachypnea, rapid onset of symptoms
and a "thumb-sign" present in 79% of the cases on lateral
X-rays of the neck are significant predictors for
imminent airway compromise with rapid clinical
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