Pyloric stenosis

(or infantile hypertrophic pyloric stenosis) is a condition that causes severe vomiting in the first few months of life.
There is narrowing (stenosis) of the opening from the stomach to the intestines, due to enlargement (hypertrophy) of the
muscle surrounding this opening (the pylorus, meaning "gate"), which spasms when the stomach empties. It is uncertain
whether there is a real congenital narrowing or whether there is a functional hypertrophy of the muscle which develops
in the first few weeks of life.

Males are more commonly affected than females, with firstborn males affected about four times as often, and there is a
genetic predisposition for the disease.[1] It is commonly associated with people of Jewish ancestry.[2] Pyloric stenosis is
more common in whites than Hispanics, African Americans, or Asians. The incidence is 2.4 per 1000

live births in whites, 1.8 in Hispanics, 0.7 in African Americans, and 0.6 in Asians. It is also less common amongst
children of mixed race parents.[3] Caucasian babies with blood type B or O are more likely than other types to be
affected.[1]

Pyloric stenosis also occurs in adults where the cause is usually a narrowed pylorus due to scarring from chronic peptic
ulceration. This is a completely different condition from the infantile form.

Symptoms

Babies with this condition usually present any time in the first weeks to months of life with progressively worsening
vomiting. The vomiting is often described as non-bile stained ("non bilious") and "projectile vomiting", because it is
more forceful than the usual spittiness (gastroesophageal reflux) seen at this age. Some infants present with poor feeding
and weight loss, but others demonstrate normal weight gain.

Diagnosis

Diagnosis is via a careful history and physical examination, often supplemented by radiographic studies. There should be
suspicion for pyloric stenosis in any young infant with severe vomiting. On exam, palpation of the abdomen may reveal
a mass in the epigastrium. This mass, which consists of the enlarged pylorus, is referred to as the 'olive,' and is
sometimes evident after the infant is given formula to drink. It is an elusive diagnostic skill requiring much patience and
experience. There are often palpable (or even visible) peristaltic waves due to the stomach trying to force its contents
past the narrowed pyloric outlet.

At this point, most cases of pyloric stenosis are diagnosed/confirmed with ultrasound, if available, showing the thickened
pylorus. Although somewhat less useful, an upper GI series (x-rays taken after the baby drinks a special contrast agent)
can be diagnostic by showing the narrowed pyloric outlet filled with a thin stream of contrast material; a "string sign" or
the "railroad track sign". For either type of study, there are specific measurement criteria used to identify the abnormal
results. Plain x-rays of the abdomen are not useful, except when needed to rule out other problems.

Blood tests will reveal hypokalemic, hypochloremic metabolic alkalosis due to loss of gastric acid (which contain
hydrochloric acid and potassium) via persistent vomiting; these findings can be seen with severe vomiting from any
cause. The potassium is decreased further by the bodies release of aldosterone, in an attempt to compensate for the
hypovolaemia due to the severe vomiting.

Pathophysiology

The gastric outlet obstruction due to the hypertrophic pylorus impairs emptying of gastric contents into the duodenum.
As a consequence, all ingested food and gastric secretions can only exit via vomiting, which can be of a projectile nature.
The vomited material does not contain bile because the pyloric obstruction prevents entry of duodenal contents
(containing bile) into the stomach.
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This results in loss of gastric acid (hydrochloric acid). The chloride loss results in hypochloremia which impairs the
kidney's ability to excrete bicarbonate. This is the significant factor that prevents correction of the alkalosis.[4]

A secondary hyperaldosteronism develops due to the hypovolaemia. The high aldosterone levels causes the kidneys to:

avidly retain Na+ (to correct the intravascular volume depletion)

excrete increased amounts of K+ into the urine (resulting in hypokalaemia).

The body's compensatory response to the metabolic alkalosis is hypoventilation resulting in an elevated arterial pCO2.

Treatment

Pyloromyotomy scar (rather large) 30 hrs post-op in a 1 month-old baby

Infantile pyloric stenosis is typically managed with surgery. It is important to understand that the danger of pyloric
stenosis comes from the dehydration and electrolyte disturbance rather than the underlying problem itself. Therefore, the
baby must be initially stabilized by correcting the dehydration and hypochloremic alkalosis with IV fluids. This can
usually be accomplished in about 24-48 hours.

Very few cases are mild enough to be treated medically. The definitive treatment of pyloric stenosis is with surgical
pyloromyotomy known as Ramstedt's procedure (dividing the muscle of the pylorus to open up the gastric outlet). This is
a relatively straightforward surgery that can possibly be done through a single incision (usually 3-4 cm long) or
laparoscopically (through several tiny incisions), depending on the surgeon's experience and preference.

Today a tiny circular incision around the navel is most commonly performed. This will leave minimal scar tissue. The
vertical incision, pictured and listed above, is no longer usually required.

Once the stomach can empty into the duodenum, feeding can commence. Some vomiting may be expected during the
first days after surgery as the gastro-intestinal tract settles. Very occasionally the myotomy (muscle division) was
incomplete and projectile vomiting continues, requiring repeat surgery. But the condition generally has no longterm side-
effects or impact on the child's future.

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