Pyloric Stenosis (HPS)

Congenital Hypertrophic,
Infantile hypertrophic,

Clinical features

One of the most common surgical

conditions in the first few months of life,
in the western world.
There are racial variations, being rare in
blacks, Chinese and Indians.
Genetic factor seem to be positive with
increased incidence in infants born to
mothers and fathers who had the
disease as infants.

Clinical features

Palpable 'tumor' in right upper

quadrant best felt from left during
test feed
Visible peristalsis often seen
Diagnosis can be confirmed by
abdominal ultrasound
Biochemically a hypochloremic
alkalosis exists

Clinical features

M:F 4-6:1,
overall incidence is 3/1000 live
birth

Infantile hypertrophic pyloric

stenosis

Strong genetic factor

Risk
Risk
7%
Risk
Risk
2%

to son if affected mother = 20%

to daughter if affected mother =
to son if affected father = 5%
to daughter if affected father =

Clinical features

Usually presents between 3 - 6 weeks of age

Late presentation up to 6 months can occur
Rapidly progressive projectile vomiting without
bile
Child is hungry and often feeds immediately
after vomiting
Dehydration and alkalosis are prominent
clinical features

Clinical features

Clinical features: age 3-6 weeks,

progressive, persistent, projectile
nonbilious vomiting,
Associated with good appetite, chronic
dehydration , loss of weight, constipation
and gastritis with hematemesis.
Olive sign. An enlarged pylorus is
palpable.
Succution splash.

Pathophysiology

Diffuse hypertrophy and hyperplasia

of the smooth muscle of the antrum
of the stomach and pylorus proper
narrow the channel, which then
become easily obstructed.
In response to outflow obstruction
and vigorous peristalsis, stomach
musculature becomes uniformly
hypertrophied and dilated.

Pathophysiology

Gastritis, hematemesis and

dehydration with
Hypokalemic, hypochloremic,
metabolic alkalosis.

Etiology

Unknown, many theories, multifactorial,

with proven genetic x-linked factor as
well as uncertain environmental factors.
Failure of relaxation of the pyloric
musculature.
Abnormal ganglion innervation.
Deregulation of VIP and nitric oxide.

Etiology

No definite cause has been found.

Regardless of mechanism, it is a
predictable process, occurring
several weeks after birth and
resolving after transitory muscle
hypertrophy, even when not
treated with myotomy.

Diagnosis

Clinical features in 80-90%, gastric

peristalsis, palpation of an olive like
mass in the right Para umbilical region.
plain X-ray, Ba. swallow and meal,
(string sign), U/S doughnut sign, serum
electrolytes, blood PH.

Differential Diagnosis

Over feeding,
Pylorospasm,
GER,
Delayed gastric emptying,
Duodenal stenosis,
Duplication and
Systemic diseases,
Metabolic disorders,
Inborn error of metabolism,
CNS lesions and
Sepsis.

Fluid and Electrolytes disturbances

and kidney response:

Vomiting of CL rich fluid(130-150meq/l), Na(60100meq/l), and K(10-15meq/l)plus loss of HCL,

result is HYPOKALEMIC HYPOCHLOREMIC
METABOLIC ALKALOSIS.
Initial response of the kidney is to maintain
blood PH, by excreting alkaline urine.
This with loss of Na and K results in increased
resorption of H ion by the renal tubules.

Fluid and Electrolytes

disturbances and kidney
response:

With continued vomiting leads to

volume depletion- the response of
the kidney- is to expand the ECV.
There is increased resorption of NA
and marked loss of K (Aldosterone
mediated).
Hypokalemia leads to excretion of H
ion that gives rise to paradoxical
aciduria.

Treatment

Correct dehydration over a 24 - 72 hour

period
Nasogastric tube often required
Ramstedt's pyloromyotomy first described in
1911
Transverse right upper quadrant or
circumumbilical incision
Longitudinal incision in pylorus down to
mucosa

Treatment

Incision extend from duodenum

onto the gastric antrum
Need to try and avoid mucosal
perforation
Feeding re-established within 1224 hours of surgery
Recurrence does not occur

Treatment

Medical: correction of fluid and

electrolyte imbalance, depending on the
degree of dehydration and disturbance.
LR,0.9%NS, 0.45%NS are the solutions of
choice at 1.5-2 times the maintenance.
Adequate amount of K, and CL to correct
metabolic alkalosis.
Surgical: by Pyloromyotomy (Ramstedt)

Prognosis

good.
Mortality less than 1%.
Perforation of pyloric mucosa less
than 3%

Infantile hypertrophic pyloric

stenosis

Due to failure of nitric oxide synthesis

Results in gastric outflow obstruction,
vomiting and dehydration
Affects 3 per 1000 live births
Male : female 4:1
Most common in first born males
Multifactorial inheritance