Pyloric stenosis or pylorostenosis is narrowing (stenosis) of the opening from the stomach to the

first part of the small intestine known as the duodenum, due to enlargement (hypertrophy) of the
muscle surrounding this opening (the pylorus, meaning "gate"), which spasms when the stomach
empties. This condition causes severe projectile non-bilious vomiting. It most often occurs in the first
few months of life, when it may thus be more specifically labeled as infantile hypertrophic pyloric
stenosis.[1] The thickened pylorus is felt classically as an olive-shaped mass in the middle upper part
or right upper quadrant of the infant's abdomen. In pyloric stenosis, it is uncertain whether there is a
true congenital anatomic narrowing or whether there is merely a functional hypertrophy of the pyloric
sphincter muscle. This condition typically develops in male babies in the first 2 to 6 weeks of life.
Pyloric stenosis also occurs in adults, where the cause is usually a narrowed pylorus due to scarring
from chronic peptic ulceration. This is a different condition from the infantile form.

Signs and symptoms[edit]

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. Dehydration may occur which causes a baby to cry without having
tears and to produce less wet or dirty diapers due to not urinating for hours or for a few days.
[2]

Constant hunger, belching, and colic are other possible signs that the baby is unable to eat

properly.

Cause[edit]
Pyloric stenosis seems to be multifactorial, with some genetic and some environmental components.
It is four times more likely to occur in firstborn males.[3] Rarely, infantile pyloric stenosis can occur as
anautosomal dominant condition.[4]

Diagnosis[edit]
Diagnosis is via a careful history and physical examination, often supplemented by radiographic
imaging studies. Pyloric stenosis should be suspected in any young infant with severe vomiting. On
physical 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',[5] and is sometimes evident after the
infant is given formula to drink. Rarely, there are peristaltic waves that may be felt or seen (video on
NEJM) due to the stomach trying to force its contents past the narrowed pyloric outlet.

Most cases of pyloric stenosis are diagnosed/confirmed with ultrasound, if available, showing the
thickened pylorus. Muscle wall thickness 3 millimeters (mm) or greater and pyloric channel length
14 mm or greater are considered abnormal in infants younger than 30 days.
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
sometimes shows a dilated stomach as shown here.
Although UGI endoscopy would demonstrate pyloric obstruction, physicians would find it difficult to
differentiate accurately between hypertrophic pyloric stenosis and pylorospasm.
Blood tests will reveal low blood levels of potassium and chloride in association with an increased
blood pH and high blood bicarbonate level due to loss of stomach acid (which contains hydrochloric
acid) from persistent vomiting. There will be exchange of extracellular potassium with intracellular
hydrogen ions in an attempt to correct the pH imbalance. These findings can be seen with severe
vomiting from any cause.

Pathophysiology[edit]
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. While the exact cause of the hypertrophy remains
unknown, Rogers has assembled compelling evidence that neonatal hyperacidity is involved. [6]
This physiological explanation for the development of clinical pyloric stenosis at around 4 weeks and
its spontaneous long term cure without surgery if treated conservatively, has recently been further
reviewed.[7]The vomited material does not contain bile because the pyloric obstruction prevents entry
of duodenal contents (containing bile) into the stomach.
Persistent vomiting results in loss of stomach acid (hydrochloric acid). The chloride loss results in
a low blood chloride level which impairs the kidney's ability to excrete bicarbonate. This is the
significant factor that prevents correction of the alkalosis.[8]
A secondary hyperaldosteronism develops due to the decreased blood volume. The
high aldosterone levels causes the kidneys to avidly retain Na+ (to correct the intravascular volume
depletion), and excrete increased amounts of K+ into the urine (resulting in a low blood level of
potassium).
The body's compensatory response to the metabolic alkalosis is hypoventilation resulting in an
elevated arterial pCO2.

Treatment[edit]

Vertical Pyloromyotomy scar (large) 30 hrs post-op in a one-month-old baby

Horizontal Pyloromyotomy scar 10 days post-op in a one-month-old baby

Infantile pyloric stenosis is typically managed with surgery; [9] very few cases are mild enough to be
treated medically.
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 the abnormally high blood pH seen in combination with low chloride levels with IV
fluids. This can usually be accomplished in about 2448 hours.
Intravenous and oral atropine may be used to treat pyloric stenosis. It has a success rate of 85-89%
compared to nearly 100% for pyloromyotomy, however it requires prolonged hospitalization, skilled
nursing and careful follow up during treatment.[10] It might be an alternative to surgery in children who
have contraindications for anesthesia or surgery, or in children whose parents do not want surgery.

Surgery[edit]
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 surgery can be
done through a single incision (usually 34 cm long) or laparoscopically (through several tiny
incisions), depending on the surgeon's experience and preference. [11]

Today, the laparoscopic technique has largely supplanted the traditional open repairs which involved
either a tiny circular incision around the navel or the Ramstedt procedure. Compared to the older
open techniques, the complication rate is equivalent, except for a markedly lower risk of wound
infection.[12] This is now considered the standard of care at the majority of children's hospitals across
the US, although some surgeons still perform the open technique. Following repair, the small 3mm
incisions are hard to see.
The vertical incision, pictured and listed above, is no longer usually required, though many incisions
have been horizontal in the past years.
Once the stomach can empty into the duodenum, feeding can begin again. Some vomiting may be
expected during the first days after surgery as the gastrointestinal tract settles. Rarely,
the myotomy procedure performed is incomplete and projectile vomiting continues, requiring repeat
surgery. Pyloric stenosis generally has no long term side-effects or impact on the child's future.

Epidemiology[edit]
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.[13] It is commonly associated with people
of Scandinavian ancestry, and has multifactorial inheritance patterns. [4] Pyloric stenosis is more
common in Caucasians than Hispanics, Blacks, or Asians. The incidence is 2.4 per 1000 live births
in Caucasians, 1.8 in Hispanics, 0.7 in Blacks, and 0.6 in Asians. It is also less common amongst
children of mixed race parents.[14] Caucasian babies with blood type B or O are more likely than other
types to be affected.[13]
Infants exposed to erythromycin are at increased risk for developing hypertrophic pyloric stenosis,
especially when the drug is taken around two weeks of life[15] and possibly in late pregnancy and
through breastmilk in the first two weeks of life