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

Quiz questions
References
Hypertrophic Pyloric Stenosis

Age
o Usually manifests at 2-8 weeks of life
Clinical
o Nonbilious projectile vomiting with progression over a period of several weeks
after birth (15-20%)
o Palpable olive-shaped mass (80% sensitive in experienced hands)
Positive family history
Nasogastric aspirate >10 ml
UGI findings
o Pyloric wall thickness >10 mm
o Elongation and narrowing of pyloric canal (2-4 cm in length)
o "Double / triple track sign"
Crowding of mucosal folds in pyloric channel
o "String sign"
Passing of small barium streak through pyloric channel
o Twining recess = "diamond sign"
Transient triangular tent-like cleft / niche in midportion of pyloric canal
with apex pointing inferiorly secondary to mucosal bulging between two
separated hypertrophied muscle bundles on the greater curvature side
within pyloric channel
o "Pyloric teat"
Outpouching along lesser curvature due to disruption of antral peristalsis
o "Antral beaking"
Mass impression upon antrum with streak of barium pointing toward
pyloric channel
 Stomach shows double tracking in region of pyloric canal,
indentation on base of bulb and delayed gastric emptying

o Kirklin sign = "mushroom sign"

Indentation of base of bulb (in 50%)
o Gastric distension with fluid
o Active gastric hyperperistalsis
"Caterpillar sign"
Gastric hyperperistaltic waves

US findings
o "Target sign"
Hypoechoic ring of hypertrophied pyloric muscle around echogenic
mucosa centrally on cross-section
o "Cervix sign"
Indentation of muscle mass on fluid-filled antrum on longitudinal section
o "Antral nipple sign"
Redundant pyloric channel mucosa protruding into gastric antrum
o Pyloric volume >1.4 cm3 (= 1/4 x [maximum pyloric diameter]2 x pyloric
length)
Most criteria independent of contracted or relaxed state
o Pyloric length (mm) + 3.64 x muscle thickness (mm) > 25
o Pyloric muscle wall thickness >3 mm
o Pyloric transverse diameter >13 mm with pyloric channel closed
o Elongated pyloric canal >17 mm in length
 o Exaggerated peristaltic waves
o Delayed gastric emptying of fluid into duodenum
Complications
o Hypochloremic metabolic alkalosis
DDx
o Infantile pylorospasm
Muscle thickness between 1.5 and 3 mm
Variable caliber of antral narrowing
Antral peristalsis
Delayed gastric emptying
Elongation of pylorus
Prognosis
Resolves in several days / ? early stage of evolving pyloric
stenosis
Treatment
Effective with metoclopramide hydrochloride
o Milk allergy
o Eosinophilic gastroenteritis