Congenital hypertrophic pyloric stenosis

congenital hypertrophic pyloric stenosis

1. CONGENITAL
HYPERTROPHIC PYLORIC
STENOSIS
Dr. kundan
Department of surgery
Patna medical college & Hospital

2. 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.

3. 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.
•

4. ASSOCIATED ANOMALIES
Esophageal atresia
Tracheoesophageal fistula
Hirschsprung disease
Exomphalos
Inguinal hernia
Hypospadias
Undescended testis

5. CLINICAL
PRESENTATION
History: 2
nd
- 8
th
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

6. 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

7. 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

8. 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

9. BARIUM SWALLOW
Duodenal bulb
Air filled fundus
Barium filled antrum
Narrowed pyloric channel
String sign
Normal stomach

10. 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.

11. •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

12. 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, HCO <28mmol/L and BE
⁻ ₃
<+2.

13. 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.

14. SURGERY

15. Laparoscopic Procedure
1 2 3

16. 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%.

17. 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.