INFANTILE HYPERTROPHIC

PYLORIC STENOSIS
HENG Sophea, MD
Phnom Penh, Cambodia
Post-Graduated Program
2020
 Learning Outcomes

• Introduction
• Etiology
• Pathology
• Diagnosis
• Differential diagnosis
• Treatment
• Complications
• References

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 Introduction
• Infantile hypertrophic pyloric stenosis (IHPS) is a common
surgical condition encountered in early infancy, occurring
in 2–3 per 1,000 live births.

• Characterized by hypertrophy of the circular muscle,

causing pyloric narrowing and elongation and producing
partial or complete luminal occlusion.

• The incidence of the disease

– varies widely with geographic location, season, and
ethnic origin.
– Boys are affected four times more than girls.
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 Etiology
The causes : still obscure/unknown, various hypotheses :

•Evidence of a genetic predisposition,

– associated with a number of inherited syndromes
– Nonsyndromic IHPS shows familial aggregation. Siblings of
patients with IHPS are 15 times more likely to suffer the
condition than children who have no family history of IHPS.

•Reported in several areas and countries, have highlighted the

importance of environmental factors.

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• The debate over a genetic or environmental origin
of IHPS has not yet reached a final conclusion.

• The cause of hypertrophic circular muscle of the

pylorus in IHPS
– is thought to result from the failure to relax the
sphincter smooth muscle.
– IHPS may be a developmental abnormality in
which the pyloric muscle hypertrophies after
birth.

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 Pathology
• The characteristic gross pathological feature :
– thickening of the antropyloric portion of the
stomach (“Olive like mass”) and
– crowding of redundant and edematous mucosa
within the lumen.

• Abnormally circumferentially thickened antropyloric

muscle (thickness​ : 4–6 mm, length : 16–20 mm,
diameter > 10-14 mm)

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• It stops abruptly at both the ends.

• The rigid antropyloric canal is

unable to accommodate the
redundant mucosa, which
protrudes into the gastric antrum.

• These anatomical abnormalities

cause obstruction to the passage
of gastric contents.

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 Histologically, IHPS is characterized by

• Thickened, hypertrophied, and edematous mucosa

• its relationship to the underlying hypertrophied
musculature,
• primarily involving the circular muscle.
• Various results of immunohistochemical studies in
relation to the enteric nervous system in IHPS.

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 Diagnosis
• Usually based on
– Clinical history,
– Physical examination,
– Imaging studies such as
• ultrasonography
• barium meal study.

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 Clinical Features

• The usual onset of symptoms occurs between 2 and

8 weeks of age with peak occurrence at 3–5 weeks
of age.

• Rarely reported in premature infants, especially

extremely low birth weight infants, and these
premature infants with IHPS present the signs and
symptoms 2–4 weeks later as compared to normal
term infants.

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Vomiting •
•
may not be frequent and forceful,
but over several days
• progresses to every feeding
• becomes forceful nonbilious vomiting
described as “projectile”.
• The emesis consists of gastric contents,
ejecting breast milk or formula up.
• Vomiting might be mild at first
• May become blood tinged with
protracted vomiting and likely related
to gastritis, with “coffee-ground”
appearance (17–18% of cases).

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• Infants with IHPS do not appear ill or febrile in the
early stages.
• A significant delay in diagnosis leads to severe
dehydration and weight loss due to inadequate fluid
and calorie intake.
• Severe starvation can exacerbate diminished
glucoronyl transferase activity and jaundice
associated with indirect hyperbilirubimemia as seen
in 2–5% of infants with IHPS.
• Associate anomalies are seen in 6–20% of patients.

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 Physical Exam
• Diagnosis alone in 80–90% on clinical
features
• The important diagnostic features are
– visible gastric peristaltic waves in the left
upper abdomen and a palpable enlarged
pylorus (“olive” like mass).

• Physical examination requires a calm and

cooperative infant with a relaxed abdomen. It
would be easy to observe the gastric
peristaltic waves after test feeding in a warm
environment.
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• Very difficult if the
stomach distends.
• Aspiration using the
nasogastric tube facilitates
the successful palpation of
an enlarged pylorus.

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Palpation
• After the edge of the liver has been identified with the
finger tip, applied gentle pressure deep to the liver and
progress caudally to reveal an enlarged pylorus

• In most cases, an enlarged pylorus located just above the

umbilicus at the lateral border of the rectal muscle below
the liver edge.
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Diagnostic Imaging

Plain Abdominal X-Ray

•In many cases, difficult to detect this
pyloric “olive” by hand.
– In these cases, many physicians may
obtain a plain x-ray of the abdomen

•Result of abdominal X-ray

– a large, air filled stomach
– but never a complete absence of gas
throughout the rest of the abdomen.
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 Ultrasonography
• The most common imaging study for the diagnosis

• Easily carried out without any sedation or radiation

Visualization :
• Longitudinal ultrasonography shows
– a variable degree of hypertrophied muscle and
– the intervening mucosa that protrudes into the fluid-
filled antrum.

• Cross-sectional study shows circumferential muscular

thickening surrounding the central channel filled with
mucosa
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• The most commonly used criteria
are pyloric muscle (IHPS)
– thickness of 4 mm or more
– pyloric channel length of 16
mm or more.
– Diameter >10 cm
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Barium meal study

• Still a highly sensitive examination

for the diagnosis of IHPS.

• The characteristic : a narrowed

elongated pyloric canal giving a
“string” or “double track” sign
caused by compressed invaginated
folds of mucosa in the pyloric
canal.
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• Contrast meal, showing
– the string sign
– Double track sign

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• Barium meal study provides indirect
information about the antropyloric canal
status.
– Failure of the relaxation of the antropyloric lesion,
known as pylorospasm, demonstrates the same
findings as those of IHPS.
– The emptying speed of the barium meal to the
distal bowel will be important to differentiate
these two conditions.

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 Differential Diagnosis
• Several conditions must be considered if the patient demonstrates non-
bilious vomiting.
– Surgical Conditions :
• Gastroesophageal reflux
• Gastic volvulus
• Antral web
• Preampullar duodenal stenosis
• Duplication cyst
• Ectopic pancreas within the pyloric muscle
– Medical conditions :
• Gastroenteritis
• Increased intracranial pressure
• Metabolic disease
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• Vomiting, differential diagnosis in newborn or infants :
– Gastroesophageal reflux
– IHPS
– Bowel obstruction : volvulus, intussusception, enterocolitis,
bowel atresia..
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Differential Diagnosis between GER
and IHPS

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 Treatment

• Once the diagnosis of IHPS has been

confirmed, surgical referral is made rapidly.

– Pre-Operative Management

– Operation

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 Pre-Operative Treatment
• Recurrent and persistent vomiting in these patients
results in chloride and potassium depletion with
metabolic alkalosis.
• Evaluate the state of dehydration and acid–base
abnormalities : serum electrolyte level, urea
nitrogen level, hematocrit, and blood gases

• Rehydration : fluid resuscitation, based on

– Degree of hydration
– Extent of electrolyte abnormalities
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 Pre-Operative Treatment
• many babies with IHPS do not show any clinical
evidence of dehydration and electrolyte
abnormalities on admission.
• If mild dehydration and hypochloremic alkalosis,
maintenance fluid
– with 5% dextrose in 0.45% normal saline
containing 20–40 mEq/l of potassium chloride

• Most of the patients with IHPS should be able to be

resuscitated within 24 h.
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 Operative Treatment
• The operation for IHPS is not an emergency
– should never be undertaken until serum
electrolytes level and acid–base balance have
returned to normal.
• Pyloromyotomy for IHPS, the most commonly used
technique, described by Ramstedt in 1912.

• Ramstedt’s pyloromyotomy for IHPS

– universally accepted, and
– the safest procedure.
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 Operative Treatment

• NGT must be placed before the induction of

anaesthesia if the tube was not placed pre-
operatively.

• The surgery is performed under GA

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 Ramstedt’s pyloromyotomy

• Technique
– Skin Incision : 2.5 – 3 cm
• The standard approach is
the right upper quadrant
transverse incision.
• Another incision that is
commonly used is an
umbilical fold incision.

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• The hypertrophied antropyloric lesion is
delivered by gentle traction through the
surgical wound

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• Then a longitudinal serosal
incision is made on
– the antero-superior aspect of
the pylorus beginning
approximately 1–2 mm
proximal to the duodenum and
Pyloric vein
– extended into the
nonhypertrophied antrum.
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• The blunt end of the scalpel handle is used to
initially disrupt the muscle fiber.
• The hypertrophied circular muscle is then
further disrupted down to the mucosa using
the Benson spreader.
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• When the pyloric muscle is adequately split, the mucosa can be seen to be
bulging.
• To test for the mucosal injury, the stomach is inflated through the
nasogastric tube (160–180 ml) as is usually done in open techniques.
Bulging of the mucosal layer with no evidence of defect should be
confirmed.
• Greenish or yellowish fluid at the myotomy area is a sign of mucosal
tear.

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 Laparoscopy for IHPS
• Recently, laparoscopy has been used as
an alternative access for pyloromyotomy.

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 Post-operative Care
• After successful pyloromyotomy,
– NGT is removed after completing surgery
• Results of surgical treatment of pyloric stenosis are highly
satisfactory.
• Symptomatic relief is complete

• Most patients are discharged within 24–48 hours

postoperatively.

• Mortality is virtually zero, and most complications are

minor.
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 Complications
• Acid-base and electrolyte imbalances.
– Hypokalemic, hypochloremic metabolic alkalosis
– If no surgery or delay treatment

• Minor degrees of postoperative vomiting are not

uncommon and need not deter feeding.
– Persistent and severe postoperative vomiting, should
alert clinicians to underlying problems such as gastritis,
gastro-oesophageal reflux, mucosal perforation or
incomplete myotomy

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• Major post-op complications :
– wound infection, mucosal perforation, and
adequate pyloromyotomy
– Mucosal perforation : 1-2 %
• Reoperation : recurrent vomiting (4%)
– Wound infection : 1-5%
– Incidence of complications after
pyloromyotomy is extremely low at present.

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 Take home message
• IHPS is characterized by hypertrophy of the circular muscle,
causing pyloric narrowing and elongation and producing
partial or complete luminal occlusion.

• The causes : unknown, mostly a result from the failure to

relax the sphincter smooth muscle.
• The clinical presentation is finding with vomiting in baby
between 2-8 weeks of life and palpable enlarged pylorus
(“olive” like mass),
• Vomiting is characterized by projectile/forceful milk and
gastric contents, progressively every feeding. The diagnosis is
confirmed by abdominal ultrasound or barium study.
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– The operation for IHPS is not an emergency, never
be undertaken until serum electrolytes level and
acid–base balance have returned to normal.
• Ramstedt’s pyloromyotomy for IHPS is the most
common used method
• The acid-base and electrolyte imbalances are the
fatal pre-operative complications, must be
corrected
• Other complications : GE reflux, infection,
perforation, peritonitis.

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Summary

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 References
1. Pediatric Surgery – Holder-Arnold, second edition,
2005.
2. Pediatric Surgery: diagnosis and management –
Prem Puri, Michael Höllwarth, Springer-Verlag Berlin
Heldelberg 2009.
3. Pediatric Surgery – Prem Puri MS, Michael
E.Höllwarth; Springer-Verlag Berlin Heidelberg 2006,
4. Pediatric Surgery Digest – Zachiaria Zachariou;
Springer-Verlag Berlin Heidelberg 2009.

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Questions ?

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