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Peadiatric surgery
emergencies
Yanal Abaza.MD

*Pyloric Stenosis
* Congenital Diaphragmatic
Hernia
*Tracheoesophageal Fistula
*Abdominal Wall Defects
*Necrotizing Enterocolitis
(NEC)

Maternal and perinatal history
Recreational drug use
Birth history
Minimum labs: glucose and CBC
Look for associated anomalies
Cardiac and respiratory status
Metabolic and electrolyte imbalance
Hydration status
Coagulation profile
IV access
Preoperative Evaluation
Pyloric Stenosis
Most common GI obstructive anomaly in neonates

Hypertrophy of the muscular layer of the pylorus

a true surgical not A medical emergency but
emergency

Incidence: 1 3 :1,000 live births

2 - 5x more common in first born, M > F (4:1)
Pyloric Stenosis
Etiology : unknown
? acquired condition with hereditary
predisposition

Symptoms are apparent between 2
nd
-6
th
wk of
life

Presents with nonbilious projectile vomiting,
signs of dehydration, jaundice (2%)
Pyloric Stenosis
Physical Exam

visible gastric peristalsis
palpable olive-shaped mass to the right
of the epigastric area
signs of dehydration

Labs: CBC serum electrolytes EKG
ABG BUN
Pyloric Stenosis
Diagnosis
history and physical exam
abdominal ultrasound
upper GI series with barium contrast
not recommended

pathological
pyloric wall thickness 4 mm
pyloric length of > 16 cm
Pyloric Stenosis
Pyloric Stenosis
Metabolic Abnormalities
hyponatremia
hypochloremia
hypokalemia
1 metabolic alkalosis
compensatory respiratory acidosis
paradoxical acidic urine
Preoperative Preparation
supportive treatment
surgical management
check labs
Pyloric Stenosis
Pyloric Stenosis
Preoperative Preparation
Supportive therapy
Correction of fluid deficits
maintenance: D
5
0.2% NaCl + KCl
20 - 40 mEq/L
replacement: albumin, normal saline
Correction of electrolyte imbalance
Prevention of aspiration : NGT
Surgical Management

Pyloromyotomy
definitive treatment
open or laparoscopic

Lab indices for safe anesthesia

serum Cl >100 mEq/L
HCO3 < 28 mEq/L
Pyloric Stenosis
Pyloric Stenosis

Concerns :
* pulmonary aspiration
* severe dehydration
* metabolic alkalosis
Congenital Diaphragmatic
Hernia
Congenital Diaphragmatic Hernia
Herniation of abdominal viscera into the thorax
Result from failure of the pleuroperitoneal canal
to close at ~ 8th wk of gestation or early
return of midgut to the peritoneal cavity
Most challenging and frustrating of all neonatal
surgical emergencies
50% mortality regardless of the method of
treatment

Incidence: 1:2,000-5,000 live births

M<F 1:1.8, frequently full term

Etiology: unknown
no genetic factors have been implicated

Antenatal history: polyhydramnios
Congenital Diaphragmatic Hernia



Congenital Diaphragmatic Hernia
Classification
Absent diaphragm : rare
Diaphragmatic hernia
80% posterolateral L >R
(Bochdalek)
2% anterior (Morgagni)
15 - 20% paraesophageal
Eventration (15 - 20%)
Congenital Diaphragmatic Hernia
Associated anomalies (20-50%)
cardiovascular 13 - 23%
CNS 28%
gastrointestinal 20%
genitourinary 15%
increase the mortality rate
Congenital Diaphragmatic Hernia
Classic Triad
Dyspnea
Cyanosis
Apparent dextrocardia
Congenital Diaphragmatic Hernia
Physical Exam
scaphoid abdomen and barrel chest
bowel sounds in the chest
displaced heart sounds
Laboratory Studies
CBC ABG
electrolytes calcium
glucose
Congenital Diaphragmatic Hernia
Diagnosis: chest x-ray
loops of bowel in the
chest
mediastinal shift
absent lung markings
Congenital Diaphragmatic Hernia
IMMEDIATE

Intubation

+

Stomach Decompression
Congenital Diaphragmatic Hernia
Determinants of Survival
degree of pulmonary hypoplasia
ipsilateral lung > contralateral lung
development pulmonary vasculature
Congenital Diaphragmatic Hernia
Goals of Management
maximize arterial oxygenation
mechanical ventilation: use low inflating
pressures
increases pulmonary blood flow
prevention of pain
fentanyl infusion 3-10 mcg/kg/hr
correction of acidosis
Standard Management Strategy
Reduce pulmonary HTN
Moderate alkalosis
pCO
2
< 40 mmHg
PaO
2
>100 mmHg
Congenital Diaphragmatic Hernia
Recent Strategy
Permissive hypercapnia and hypoxemia
Pressure-limited ventilation (<25 cmH
2
O)
Postductal pCO
2
40-65 mmHg
Preductal SpO
2
85-90%
Postductal SpO
2
ignored unless pH is
< 7.20 or pCO
2
> 65
Congenital Diaphragmatic
Hernia
Congenital Diaphragmatic Hernia
Bohn (1986)

reevaluation of the traditional mad dash
surgical strategy

recommended 24 48 hrs medical stabilization

assessment of efficacy of delayed approach

infants unresponsive to initial therapy will fail to
survive with surgery or any other treatment
including ECMO
The Relationship Between PaCO
2
and Ventilation
Parameters in Predicting Survival in CHD

Arterial CO
2
accurately reflects the degree of
lung development
Poor survival in the presence of severe
pulmonary hypoplasia
CO
2
retention and severe preductal shunting
have 90% mortality
Bohn, DJ, et al
J of Pedia Surg 19: 666-671, 1884
Congenital Diaphragmatic Hernia
nomogram:

to predict the degree of pulmonary hypoplasia in
the infants and chance of survival
used the preop PaCO
2
and an index of ventilation
(Vi)

If PaCO
2
< 40 and Vi < 1000: survival almost universal
If PaCO
2
> 40 and Vi > 1000: death virtually inevitable

Vi = mean airway pressure x respiratory rate
Congenital Diaphragmatic Hernia
High Mortality

pH < 7.0
pCO2 >60 mmHg
pO2 < 50 mmHg

Boix-Ochoa J, et al
J Pediatric Surg 9:49-57, 1974

Congenital Diaphragmatic Hernia
Acid Base Balance and Blood Gases in
Prognosis and Therapy of CHD
Indications of Surgical Repair

Reversal of ductal shunting
O
2
index of < 40
Arterial pCO
2
maintainable under
40 mmHg
Hemodynamic stability
Congenital Diaphragmatic Hernia
Congenital Diaphragmatic Hernia
Preoperative Preparation
Look for associated anomalies
Labs: CBC, electrolytes, ABG, glucose,
blood type and crossmatch
CXR, Echo
Venous access: upper extremities
preferred
Prevention of hypothermia



Surgical repair
primary closure
staged procedure
Transabdominal subcostal incision
Thoracoscopic repair has been reported
Congenital Diaphragmatic Hernia
Intraoperative
Congenital Diaphragmatic Hernia
Intraoperative
Potential Problems
Hypoxemia
distension of stomach
1 pulmonary hypoplasia / pulmonary HTN
Contralateral pneumothorax
Hypotension or IVC compression
Cardiac arrest
Congenital Diaphragmatic Hernia
Postoperative Care
Ventilatory support
Close fluid management
Hemodynamic monitoring

Honeymoon Period followed by deterioration
increase abdominal pressure
impaired peripheral and visceral perfusion
limited diaphragmatic excursion
worsening of pulmonary compliance
Tracheoesophageal
Fistula
Tracheoesophageal Fistula
Incidence: 1:4000 live births

M > F (25:3)

10-40% are preterm

Antenatal history: polyhydramnios (60%)

Etiology: failure in mesenchymal separation of
upper foregut
Tracheoesophageal Fistula
Clinical Presentation
choking on 1st feed
coughing
cyanosis
excessive salivation
aspiration pneumonia
Tracheoesophageal Fistula
Diagnosis
inability to pass a suction catheter
into the stomach
CXR: coiled orogastric tube in the
cervical pouch; air in the stomach
and intestine
Tracheoesophageal Fistula
Esophageal Atresia Tracheoesophageal
Fistula
Turnage RH, et al, Sabiston Textbook of Surgery,17
th
Ed. 2004
TracheoEsophageal Fistula
5 Types (Gross and Vogt)
Gregory GA, ed, Pediatric Anesthesia, 3
rd
edition, 1996
7.7% 0.8% 86% 0.7% 4.2%
Tracheoesophageal Fistula
35-65% have associated anomalies
VATER and VACTERL
V vertebral anomalies or VSD
A anorectal malformation
C cardiac anomalies (common)
T TEF
E esophageal atresia
R renal abnormalities
L limb/radial malformation
Tracheoesophageal Fistula
Preoperative Preparation
Minimize pulmonary complication
npo
head-up position
sump tube (repogle) on low continuous suction
gastrostomy under local anesthesia
CXR, abdominal x-ray, renal ultrasound
12-L EKG and Echocardiogram : mandatory
IV access arterial line
Laboratory studies
CBC
Electrolytes
Glucose
Calcium
ABGs
Tracheoesophageal Fistula
Preoperative Preparation
Tracheoesophageal Fistula
Preoperative Preparation
24-48 hr medical stabilization
Antibiotics: ampicillin and gentamicin
Ensure availability of blood in the OR
Optimize volume status and metabolic state
Intubation preferably in the operating room
under controlled situation
Tracheoesophageal Fistula
Intraoperative Management
Main Concern
oxygenation and ventilation
securing the airway
Monitors
ASA standard
invasive : arterial line
Tracheoesophageal Fistula
Intraoperative Management
Surgical repair
ligation of fistula
check air leak in suture line
esophageal repair
identify the pouch
placement of feeding tube
chest tube placement and closure of
thoracic cavity
Tracheoesophageal Fistula
Postoperative Management


Early extubation desirable
caution: disruption of surgical repair with
reintubation

Postop Pain Management
1. IV narcotics
2. epidural infusion: 0.1% bupivacaine +
fentanyl 0.5 mcg/ml at 01.-0.2 ml/kg/hr
3. rectal Tylenol + LA infiltration of incision
Tracheoesophageal Fistula
Main Cause of Mortality
associated anomalies
survival rates 85-90%

Long Term Complications
GE reflux
anastomotic stricture
tracheomalacia
Abdominal Wall
Defects
Gastroschisis

Omphalocoele
Gastroschisis


Greek word for belly cleft
Evisceration of gut through a 2-3 cm defect in
the anterior abdominal wall lateral to the
umbilicus, usually on the right
Absence of covering or sac
chemical peritonitis infection
ECF loss heat loss
Incidence: 1:15,000-30,000 live births
Gastroschisis
Etiology
exact cause unknown
Theories
intrauterine occlusion of omphalo-
mesenteric artery ischemia and
atrophy of abdominal muscles
early fetal rupture of an omphalocoele
Gastroschisis
Gastroschisis
rupture of umbilical cord at the site
of the resorbed right umbilical vein
? Maternal: smoking, ETOH,
recreational drugs, medications
(NSAIDS, pseudoephredrine)
associated anomalies - rare
Omphalocoele
Omphalocoele
External herniation of abdominal viscera into
the base of the umbilical cord through a
central defect
Defect: small or large
Umbilical cord is inserted into the apex of the
lesion
Presence of covering or sac (amnion and
peritoneum)
Incidence: 1-5,000-10,000 live births
Omphalocoele
Embryology
Failure of the midgut to return to the
abdominal cavity by the 10
th
wk of
gestation
Omphalocoele
Omphalocoele
Etiology
incomplete return of the gut to the
abdominal cavity due to an abdominal
lateral fold defect
Failure of migration and fusion of cranial,
caudal and/or lateral folds of the embryonic
disc at ~ 3
rd
wk of gestation
Omphalocoele
Cranial Fold : Pentalogy of Cantrell
Epigastric omphalocoele
Sternum cleft
Diaphragmatic defect
Ectopia cordis
Cardiac anomaly
Omphalocoele
Lateral Fold
omphalocoele with cord coming of the
center of the sac
Caudal Fold
Hypogastric omphalocoele
Extrophy of the bladder
Imperforate anus
Colonic agenesis
Vesicointestinal fistula
Omphalocoele
Associated Congenital Anomalies: 75-80%
chromosomal: trisomy 13, 15, 21
cardiac anomalies: 20%
craniofacial
gastrointestinal
Beckwith-Wiedeman Syndrome
omphalocoele microcephaly
visceromegaly hypoglycemia
macroglossia hyperviscosity
Pentalogy of Cantrell
Omphalocoele
Survival: 20% with heart disease
70% without heart disease
Major cause of mortality
cardiac defects
prematurity
Definitive Treatment: surgical repair
Gastroschisis Omphalocoele

Incidence 1:15,000-30,000 1:6,000


Peritoneal absent present
covering/sac

Location of periumbilical within the umbilical
defect cord

Herniated matted, edematous normal
bowel

Associated low (10-15%) high (40-60%)
anomalies
intestinal atresia congenital heart dis.
(15%) Beckwith-Weidman
syndrome
Gastroschisis
Omphalocoele
Hydration / fluid status
warm moist sterile saline-soaked gauze
plastic bowel bag
initial fluid requirement 10 -15 ml/kg/hr; higher with
gastroschisis 100-200 ml/kg/hr
Heat loss : neutral thermal environment
Difficulties of surgical closure
Associated congenital anomalies & prematurity
Abdominal Wall Defects
Preoperative Management
Infection and postop nutrition
Postoperative ventilation
Airway
Metabolic status
Aspiration precautions
Direct trauma to herniated organ
Abdominal Wall Defects
Preoperative Management
Abdominal Wall Defects
Preoperative Management
Lab workup
CBC
Electrolytes and Glucose
ABG
CXR
Echocardiography
Prevent hypothermia
full access body hugger heating blanket
increase room temp plastic wrap
fluid warmer
Fluid requirement
maintenance: D
5
0.2% NS
3
rd
space loss replacement
isotonic fluid 10 -15 ml/kg/hr
blood loss from adhesions
Abdominal Wall Defects
Intraoperative Management
Abdominal Wall Defects
Intraoperative Management
Surgical Closure
optimal method remains controversial
1) primary fascial closure : 80%
intraop and postop muscle paralysis
2) staged repair
silicone elastometer pouch
primary skin closure
Closure dependent on the
1) size of the defect
2) development of abdominal wall
3) presence of associated anomalies
Abdominal Wall Defects
Intraoperative Management
Abdominal Wall Defects
Intraoperative Management
Unsafe for Primary Abdominal Closure
Intragastric pressure > 20 cmH
2
O
Intravesical pressure > 20 cmH
2
O
Change in CVP 4 mmHg
Abdominal Wall Defects
Intraoperative Management
Staged Reduction
Dacron reinforced silastic silo
Gradual reduction over 1- 2 weeks
Ketamine or opioid muscle relaxant in
intubated patients or
Titration of ketamine 0.5 -1 mg/kg IV with
spontaneous breathing unintubated infants
Final closure in the OR
Abdominal Wall Defects
Intraoperative Management
Silo closure
Necrotizing Enterocolitis
(NEC)
Necrotizing Enterocolitis (NEC)
Life-threatening intestinal inflammation
or injury
Caused by bacterial invasion of previously
injured or ischemic bowel wall
Incidence: 5 -10% in infants <1500g birth
weight
Mortality rate: 10 - 30%
Single most important factor
PREMATURITY
Can occur in:
premature infants
LBW infants
Full term infants
fed and unfed infants
Necrotizing Enterocolitis (NEC)
Other factors
ischemia
bacterial infection
GI endotoxemia
enteral feeding
use of hyperosmolar formula
congenital heart disease
hx of umbilical arterial catheterization
hx of exchange transfusion
Necrotizing Enterocolitis (NEC)
Early signs
gastric residuals with feedings
temperature instability
poor feeding
bilious vomiting
lethargy
mucoid or bloody stool
apnea and bradycardia
Necrotizing Enterocolitis (NEC)
Late Signs
Hemodynamic instability
Anemia
Thrombocytopenia
Coagulopathy, DIC
Prerenal azotemia
Metabolic acidosis
Necrotizing Enterocolitis
Physical Exam
distended and tender abdomen
Labs:
CBC
electrolytes and glucose
platelets and coagulation profile
DIC profile
ABG
Necrotizing Enterocolitis (NEC)
Abdominal X-ray
signs of bowel obstruction
ileus with edematous
bowel
Pneumatosis intestinalis
or intramural air (arrow)
portal vein air
pneumoperitoneum
Necrotizing Enterocolitis (NEC)
Medical Management
initial treatment, for 7-10 days
75% successful
Surgical Treatment
10 - 50% mortality
Necrotizing Enterocolitis (NEC)



Necrotizing Enterocolitis (NEC)
Medical Management
No enteral feedings for 10-14 days
NGT on intermittent suction
Hydration and correction of electrolytes
Ventilatory support
Antibiotics
Blood and platelet transfusion if needed
Necrotizing Enterocolitis (NEC)
Surgical Indications
Absolute Indications
1) bowel perforation
peritoneal drains under
local anesthesia
2) intestinal gangrene
Relative Indications
clinical condition
metabolic acidosis
respiratory failure
oliguria, hypovolemia
thrombocytopenia
leucopenia, leukocytosis
air in the portal vein
bowel wall edema
persistent dilated bowel loops
Necrotizing Enterocolitis (NEC)
Necrotizing Enterocolitis
Non-Surgical Indications
severe GI hemorrhage
abdominal tenderness
intestinal obstruction
gasless abdomen with ascites
Necrotizing Enterocolitis (NEC)
Preoperative Management
Electrolyte imbalance: hyperkalemia
Coagulopathy: thrombocytopenia
Respiratory failure
Sepsis / hemodynamic instability
inotropic support
dopamine infusion
Summary
Almost all neonatal surgical emergencies
are really urgencies
Immaturity of organ system in neonates
alters pharmacology and physiology
Thorough preop assessment is required in
all neonates
One anomaly mandates a search for others
Murmurs necessitate a cardiology consult
Successful perioperative outcome depends
on open communication and teamwork
between neonatologist, anesthesiologist and
surgeon
Initial resuscitation of neonatal surgical
candidates includes:
airway protection
adequate IV access
fluid resuscitation
temperature stabilization
gastric decompression
administration of antibiotics
identify associated anomalies
Thank you