Pyloric stenosis

Dr Behrang Amini et al.

Hypertrophic pyloric stenosis (HPS) refers to the idiopathic thickening of gastric pyloric musculature which
then results in progressive gastric outlet obstruction.

Epidemiology
Pyloric stenosis is relatively common and has a male predilection (M:F ~4:1), and is more commonly seen in
Caucasians 4. It typically occurs between the first week to 3 months of age. There may be a positive family
history.

Incidence of hypertrophic pyloric stenosis is approximately 2-5 per 1,000 births per year in most white
populations. HPS is less common in India and among black and other Asian populations.

Clinical presentation
Clinical presentation is typical with non-bilious projectile vomiting. The hypertrophied pylorus can be palpated
as an olive-sized mass in the right upper quadrant. A succussion splash may be audible, and although
common, is only relevant if heard hours after the last meal 6.

Risk factors

maternal history of pyloric stenosis 10

Pathology
HPS is the result of both hyperplasia and hypertrophy of the pyloric circular muscles fibres. The pathogenesis
of this is not understood. There are four main theories 9:

immunohistochemical abnormalities
genetic abnormalities
infectious cause
hyperacidity theory

Associations

Turner syndrome

tracheo-oesophageal fistula

oesophageal atresia

trisomy 18 10

Radiographic features
Plain radiograph
Abdominal x-ray findings are non-specific but may show a distended stomach with minimal distal intestinal
bowel gas.
 Fluoroscopy
An upper gastrointestinal series (barium meal) excludes other, more serious causes of pathology, but the
findings of a UGI series infer rather than directly visualise the hypertrophied muscle. On upper gastrointestinal
fluoroscopy:

delayed gastric emptying

peristaltic waves (caterpillar sign)
elongated pylorus with a narrow lumen (string sign) which may appear duplicated due to puckering of
the mucosa (double-track sign)
the pylorus indents the contrast-filled antrum (shoulder sign) or base of the duodenal bulb (mushroom
sign)
the entrance to the pylorus may be beak-shaped (beak sign)

Ultrasound
Ultrasound is the modality of choice in the right clinical setting because of its advantages over a barium meal
are that it directly visualises the pyloric muscle and does not use ionising radiation. Unfortunately, it is
incapable of excluding other diagnoses such as midgut volvulus. Easy ultrasound technique is to find
gallbladder then turn the probe obliquely sagittal to the body in an attempt to find pylorus longitudinally 7.

The hypertrophied muscle is hypoechoic, and the central mucosa is hyperechoic. Diagnostic measurements
include (mnemonic "number pi"):

pyloric muscle thickness, i.e. diameter of a single muscular wall on a transverse image: >3 mm (most
accurate 3)
length, i.e. longitudinal measurement: >15-17 mm
pyloric volume: >1.5 cc
pyloric transverse diameter: >13 mm
With the patient right side down the pylorus should be watched and should not be seen to open.

Described sonographic signs include:

antral nipple sign

cervix sign
target sign

Treatment and prognosis

Initial medical management is essential with rehydration and correction of electrolyte imbalances. This should
be completed prior to surgical intervention.

Treatment is surgical with a pyloromyotomy in which the pyloric muscle is divided down to the submucosa. This
can be performed both open and laparoscopically. The operation is curative and has very low morbidity 4-5.

Differential diagnosis
There is usually little differential when imaging findings are appropriate. Of course, clinically it is important to
consider other causes of vomiting in infancy.
 A degree of pylorospasm is common in infancy and is responsible for some delay in gastric emptying. The
pylorus, however, appears sonographically normal. In cases where the doubts persist, fluid gastric distention
can be performed to "open" a tapered pylorus.

Gastro-oesophageal reflux which represents the cause of vomiting in two-thirds of infants referred to
radiology 8.

Other causes of proximal gastrointestinal obstruction can be considered 8:

midgut volvulus
gastric antral web
duodenal web/stenosis
annular pancreas
bezoar

A pyloromyotomy is a surgical procedure in which an incision is made in the longitudinal and

circular muscles of the pylorus. It is used to treat hypertrophic pyloric stenosis. Hypertrophied
muscle is cut along the whole length until the mucosa bulges out.

first-born male infant (common)

Considered a key risk factor. The incidence is
approximately 4 times greater in male infants
than in female infants. [16]
Usually affects the first-born male.

non-bilious projectile vomiting (common)

Occurs soon after feeding.

3 to 6 weeks old (common)

Most infants present between 3 to 6 weeks of
age. Typically, diagnosed in term infants. It may
be more difficult to diagnose in premature
infants, who may have atypical symptoms or
ultrasound findings.
upper abdominal mass (common)
Confirms the diagnosis if present on examination.
Also known as an 'olive'.

peristaltic waves (uncommon)

Waves travelling from left to right across the
abdomen. Due to the stomach attempting to force
its contents past the narrowed pyloric outlet.

Other diagnostic factorsshow all

FHx of pyloric stenosis (common)

Considered a risk factor. Non-Mendelian familial
pattern has been well described. [16] [20]

multiple formula changes (common)

Early symptoms may be vague and confused with
intolerance to certain formulas.

tachycardia (common)
Delayed presentation can lead to severe volume
depletion.

decreased wet nappies (common)

Delayed presentation can lead to severe volume
depletion.

dry mucous membranes (common)

Delayed presentation can lead to severe volume
depletion.

flat or depressed fontanelles (common)

Delayed presentation can lead to severe volume
depletion.
constipation (common)
Can be seen with delay in diagnosis; due to
volume depletion and poor oral intake.

poor weight gain (common)

Can be seen with delay in diagnosis.

irritability (common)

Usually the infant is not calm and is crying. This
is because the infant is extremely hungry and
does not get fed effectively.

Classically, the infant with pyloric stenosis has nonbilious vomiting or
regurgitation, which may become projectile (in as many as 70% of
cases), after which the infant is still hungry. [11]

Emesis may be intermittent initially, or occur after each feeding.

Emesis should not be bilious as the obstruction is proximal to the
common bile duct. The emesis may become brown or coffee color due
to blood secondary to gastritis or a Mallory-Weiss tear at the
gastroesophageal junction.

As the obstruction becomes more severe, the infant begins to show
signs of dehydration and malnutrition, such as poor weight gain, weight
loss, marasmus, decreased urinary output, lethargy, and shock.

The infant may develop jaundice, which is corrected upon correction of
the disease.
CAUSE

In as many as 60-80% of the infants with infantile hypertrophic pyloric
stenosis (IHPS), a firm, nontender, and mobile hard pylorus that is 1-2
cm in diameter, described as an "olive," may be present in the right
upper quadrant at the lateral edge of the rectus abdominis muscle. This
is best palpated after the infant has vomited and when calm, or when
the gastric contents have been removed via nasogastric tube. The
palpation of an olive mass has a 99% positive predictive value for IHPS
diagnosis. [12]

Clinicians may also observe gastric peristalsis just prior to emesis as
the peristaltic waves try to overcome the obstruction.

Signs of dehydration include depressed fontanelles, dry mucous
membranes, decreased tearing, poor skin turgor, and lethargy.

Due to the widespread early use of ultrasonography to aid in diagnosis,
the classic signs of infantile hypertrophic pyloric stenosis are becoming
less common. The mean age of presentation is getting significantly
younger, and infants are not developing the physical signs or electrolyte
abnormalities they were in the past.