CONGENITAL
HYPERTROPHIC PYLORIC
STENOSIS
Dr. kundan
Department of surgery
Patna medical college & Hospital
HYPERTROPHIC PYLORIC STENOSIS
One of most common GI disorders during
early infancy.
The history of what we now refer to as
infantile hypertrophic pyloric stenosis dates
back to the early 1700s.
Blair described an infant with postmortem
findings consistent with hypertrophic pyloric
stenosis in 1717
Described by Hirschsprung in 1888.
Hypertrophy of circular muscles of pylorus
results in constriction and obstruction of
gastric outlet.
EPIDEMIOLOGY AND ETIOLOGY
• Incidence: 1-2/1000 live births
• Epidemiology: more in first born males
M:F - 4-5:1
• Etiology: Unknown
• Genetic- 11q14-22 and Xq23
• Familial
• Gender
• Ethnic origin- more in whites
• As new technology and concepts have
evolved,additional associations that involve IHPS
and gastrointestinal peptides, growth factors,
neurotrophins, changes in neural development,
and nitric oxide have been described.
•
ASSOCIATED ANOMALIES
Esophageal atresia
Tracheoesophageal fistula
Hirschsprung disease
Exomphalos
Inguinal hernia
Hypospadias
Undescended testis
CLINICAL PRESENTATION
History: 2nd - 8th week of life
Projectile, frequent episodes of non-bilious
vomiting 30-60 minutes after feeding
Weight loss
Persistent hunger
Jaundice (2%)- due to
decreased hepatic glucoronosyl
transferase associated
with starvation
Examination:
Palpable olive shaped mass (1.5-2cm) to
the right of epigastric area.
Visible gastric peristalsis
from Lt. upper quadrant to
epigastrium
s/o dehydration
PATHOPHYSIOLOGY
• Vomiting → loss of H⁺ and Cl⁻ →
Hypochloremic hypokalemic metabolic
alkalosis
• Protracted vomiting → ECF volume deficit →
urinary excretion of K⁺ and H⁺ to preserve Na⁺
and water
• Initial alkalotic urine becomes acidotic-
Paradoxical aciduria
• Hypochloremic hypokalemic metabolic
alkalosis with paradoxical aciduria with
secondary respiratory acidosis
• Hyponatremia may not be evident because of
hypovolemia
DIAGNOSIS
History
and physical examination
Abdominal USG:
Pyloric muscle thickness >3-4mm
pyloric length > 15-18mm in presence
of functional gastric outlet obstruction
Upper GI study when atypical presentation
or negative USG
Diagnostic: narrowed, elongated pyloric
channel with pyloric mass effect on
stomach and duodenum – String/ Double
tract/ Beak/ Pyloric teat sign
BARIUM SWALLOW
Air filled fundus
Duodenal bulb
Barium filled antrum
Narrowed pyloric channel
Normal stomach
String sign
DIFFERENTIAL DIAGNOSIS
Gastroesophageal reflux, with or without hiatal
hernia.
Differentiated by radiologic studies. Also
amount of vomitus is smaller, and the infant does
not usually lose weight.
Adrenal insufficiency.
Differentiated by absence of
metabolic acidosis, hyperkalemia, and elevated
urinary sodium.
Viral gastroenteritis.
Unusual in infants less than 6
weeks of age. Associated with significant diarrhea
and sick contacts.
• Treatment: medical emergency but NOT
surgical emergency
• Definitive treatment: Ramstedt
Pyloromyotomy
• Anaesthetic considerations
• Patient related: infant age group
severe dehydration
electrolyte imbalance
• Surgery related: open/ laparoscopic
Celiac reflex
• Anaesthesia related: pulmonary aspiration
PONV
PREOPERATIVE PREPARATION
• Correction of fluid deficits- over 24-48 hrs
• Deficit: isotonic fluid 0.9% saline (20ml/kg
bolus)
• Maintenance: 0.45% saline in 5% Dextrose at
1.5 times maintenance rate +10-40 meq/L
KCL added once urine output established
• Correction of electrolyte imbalances
• Prevention of aspiration: aspiration through
NGT
Surgery should only take place when
dehydration corrected, normal S. Na and K, Cl⁻
> 90mmol/L, HCO₃ <28mmol/L and BE <+2.
SURGICAL MANAGEMENT
Once resuscitated the infant can undergo the
Fredet-Ramstedt pylormyotomy, which is the
procedure of choice.
Ramstedt described this operative procedure to
alleviate the condition in 1907
It consist of incision in to the sphincter muscle of
pylorus.
NG tube is passed and gastric content are
aspirated just prior to surgery.
SURGERY
Laparoscopic Procedure
1 2 3
COMPLICATIONS
Complications after pyloromyotomy should be
minimal if performed by experienced surgeons.
Perforation (In a large series of infants
undergoing open pyloromyotomy, the incidence of
perforation was 2.3%).
Wound-related complications occurred in 1%.
POSTOPERATIVE CARE
• Post op pain relief:
Acetaminophen 30-40mg/kg rectal
suppository
LA infiltration of surgical incision
• Post op concerns:
Respiratory depression and apnea due to CSF
alkalosis and intraop hyperventilation
Hypoglycemia
Hypothermia
Therefore post op monitoring for 12 hrs is recommended in
these patients.
