IHPS

•  Infantile hypertrophic pyloric stenosis (IHPS) occurs in

approximately 2 to 3.5 per 1000 live births, although rates and
trends vary markedly from region to region [1-7].
• It is more common in males than females (4:1 to 6:1) [1,6-11]
and in infants born preterm as compared with those born at
term [12-14].
• Approximately 30 to 40 percent of cases occur in first-born
children (approximately 1.5-fold increased risk) [8,9,12,15], and
• it is less common in infants of older mothers [16]. Symptoms
usually begin between 3 and 5 weeks of age, and very rarely
occur after 12 weeks of age
• The etiology of infantile hypertrophic pyloric
stenosis (IHPS) is unclear but probably is
multifactorial, involving genetic predisposition
and environmental factors.
• Neonatal hypergastrinemia and gastric
hyperacidity may play a role [10].
• Prematurity (<37 weeks gestation) may be a
risk factor [13];
 Environmental factors 
•  Maternal smoking during pregnancy increases the risk for IHPS by 1.5 to
2.0-fold [12,13,16,20].
• Several studies have suggested that bottle feeding rather than
breastfeeding increases the risk for IHPS [14,16,21-24]. In particular, a
large population-based study from Denmark found that bottle feeding
during the first four months of life increased the risk for IHPS more than
fourfold (hazard ratio [HR] 4.62, 95% CI 2.78-7.65), and a similar increase
in risk was seen for infants that were both bottle- and breastfed [23].
Most of the included subjects used formula for bottle feeds rather than
expressed breast milk, so the study did not delineate whether the
increased risk is related to the formula or to the mechanism of bottle
feeding. Similarly, a large study from Washington state also found that
bottle feeding increased the risk for IHPS after adjustment for sex and
maternal smoking (odds ratio [OR] 2.31, 95% CI 1.81-2.95) [16].
 Genetic factors
•  Familial aggregation of pyloric stenosis was examined in a
large population registry from Denmark [6].
• Significant familial aggregation was noted, with a nearly
200-fold higher rate among monozygotic twins and a 20-
fold increase among dizygotic twins or siblings, as
compared with individuals with no affected relatives.
• The heritability estimate from this study was 87 percent.
• The maternal and paternal contributions to heritability
were similar, suggesting that the intrauterine
environment was not an important causal factor.
 Macrolide antibiotics
• Both erythromycin and azithromycin are
associated with increased risk of IHPS,
particularly when administered to infants
younger than two weeks of age [7,27-31].
• The risk of IHPS with clarithromycin is not
known
• Several studies have reported an association
between the use of macrolide antibiotics by
women during late pregnancy or while
breastfeeding and the development of IHPS in
their offspring [31,33-35].
• One study reported that the association was
stronger among female as compared with
male offspring [35].
 Classic presentation 
• The classic presentation of infantile hypertrophic pyloric stenosis
(IHPS) is the three- to six-week-old infant who develops immediate
postprandial vomiting that is nonbilious and forceful (often
described as "projectile" vomiting).
• The infant then demands to be re-fed soon afterwards (a "hungry
vomiter"). These characteristics were seen in a series of 132 infants
who were diagnosed with IHPS in the 1970s, in which 91 percent
presented with projectile vomiting after feedings [8].
• The majority (83 percent) were boys, and 31 percent were firstborn.
• In another review of infants diagnosed between 1957 and 1969, the
mean age in which the infants began vomiting was 22 days [36].
• IHPS rarely occurs after 12 weeks of age [17].
• Patients were classically described as being
emaciated and dehydrated with a palpable "olive-
like" mass at the lateral edge of the rectus
abdominis muscle in the right upper quadrant of
the abdomen (see 'Palpation of the “olive”' below).
• The frequency of palpation of the "olive" typically
was quite high (up to 92 percent) in a report from
1975 [8], but has been noted to be less common in
subsequent reports [37].
• Laboratory evaluation classically showed a
hypochloremic, metabolic alkalosis due to the loss
of large amounts of gastric hydrochloric acid, the
severity of which depended upon the duration of
symptoms prior to initial evaluation [38].
• Similarly, hypokalemia is common in infants who
have been vomiting for longer than three weeks
but typically is not seen in those with a more
recent onset of symptoms
 Earlier presentation
• The typical presentation has changed over time, probably because infants are
diagnosed earlier than in the past. Vomiting is still the most common symptom,
but infants tend to be better nourished and generally present without significant
electrolyte imbalances [40].
• This difference was illustrated in a study comparing the presentation of a total of
283 infants diagnosed in the decades prior to 1975, 1985, and 1995 [41].
• The mean age at presentation was significantly younger in the more recent
decades (mean age was 5.4 weeks in 1975 versus 3.4 weeks in 1995).
• Electrolyte abnormalities were absent in most infants (88 percent) in all groups.
• A minority of infants present before two weeks of age, and these infants are
more likely to have a positive family history of IHPS [42]. These infants tend to
have more subtle ultrasonographic abnormalities compared with those
presenting after two weeks of age.
• The earlier diagnosis of IHPS might be explained by advances in
diagnostic imaging or to an increased awareness of the disorder
among clinicians.
• One study found that clinicians in the modern era were less likely to
be able to palpate the pyloric "olive" (from 87 percent to 49 percent in
the two different time periods) [37].
• This finding is probably due to ready availability and reliance on
ultrasound to make the diagnosis immediately upon suspicion, so that
clinicians in the modern era have less practice at palpating the pyloric
"olive" [43].
• In addition, infants are now more likely to be evaluated earlier in the
course of the disease, when they are well nourished, which makes
palpation of "olive" more difficult.
 Atypical presentation
•  In addition to the presenting symptoms
described above, the diagnosis of IHPS should
be considered in young infants with repeated
nonbilious vomiting, hypochloremic
alkalosis, and/or rapid clinical improvement
after rehydration, even in the absence of
projectile vomiting or a palpable "olive" [44,45].
• In a few infants with IHPS (<2 percent), the
vomiting may be bilious [46].
• In infants with medical or surgical problems, the
presentation may be atypical or the symptoms initially
attributed to other etiologies.
• In infants with congenital anomalies that affect
swallowing (eg, central nervous system anomalies,
cleft lip and palate), vomiting may not be projectile [
44].
• In infants with gastrointestinal anomalies,
postoperative vomiting may initially be attributed to
adhesions or obstruction at an anastomotic site [44].
• In premature infants with IHPS, vomiting may be less forceful, voracious
appetite and exaggerated gastric peristalsis may be lacking, and
ultrasonographic criteria for diagnosis may not apply [44,45,47,48].
• Premature infants may also present at an older chronological age than
term infants [19,49].
• One study found a mean age at presentation of 7.6 weeks in infants
born prior to 34 weeks gestation versus 5.4 weeks in term infants [49].
• In hospitalized premature infants, non-projectile vomiting, weight loss,
and lethargy may initially be attributed to sepsis; in this case, negative
cultures, rapid improvement with intravenous fluids, and metabolic
alkalosis (rather than acidosis) in such infants should prompt
consideration of IHPS [44].
 History 
• Vomiting in IHPS is typically forceful, nonbilious, and
tends to occur immediately after feeding.
• The force and timing can help to distinguish IHPS from
physiologic gastroesophageal reflux, in which most
episodes of vomiting are not forceful, and may occur
10 minutes or more after the meal.
• A history of bilious vomiting does not exclude IHPS
but should raise concern about more distal intestinal
obstruction, such as malrotation with volvulus or
Hirschsprung disease.
 Palpation of the “olive”
•  The hypertrophied pylorus is palpable in 50 to 90
percent of infants with IHPS.
• When present, it is virtually pathognomonic of the
disease (99 percent sensitivity in one study [72]), and
many surgeons will proceed directly to the operating
room based on this finding, without further testing.
• However, identification of a palpable pylorus (called
the "olive") has fallen from 73 to 27 percent over time,
with clinicians in the current era making the diagnosis
primarily by pyloric ultrasonography [73].
• The olive is felt as a firm mass at the lateral edge of the rectus abdominis
muscle in the right upper quadrant of the abdomen, about the size and shape
of an olive.
• To palpate the "olive," the abdomen should be examined when the infant is
quiet to avoid interference from tensed abdominal muscles;
• providing a pacifier dipped in a sucrose solution may be helpful to quiet the
infant during the examination. Ideally, the examination should be performed
immediately after emesis because the mass is less likely to be obscured by a
distended antrum.
• As an alternative, the gastric contents can be emptied with a nasogastric
tube, which helps to decompress the distended stomach and enhances the
palpability of the pyloric mass.
• Peristaltic waves may be seen progressing across the child's upper abdomen
from left to right just before emesis.
• The abdomen should also be evaluated for distension and bowel
sounds.
• Abdominal distension or high-pitched bowel sounds suggest
intestinal obstruction rather than IHPS, especially if associated
with bilious vomiting.
• Such infants should be evaluated promptly with abdominal
radiographs.
• A stool should be visually inspected for mucus and blood, and
tested for occult blood.
• Rectal bleeding raises the possibility of cow's milk protein
intolerance in a healthy-appearing infant, or of intussusception in
an unwell infant.
 Electrolytes
• Patients with recent onset of symptomatic IHPS usually have
normal laboratory results. Those with prolonged symptoms tend
to have low serum chloride and potassium, and elevated
bicarbonate (a hypochloremic alkalosis). Either hypernatremia
or hyponatremia may be present. One study showed that serum
pH >7.45, chloride <98, and base excess >+3 gave a positive
predictive value of 88 percent in diagnosing IHPS in infants
presenting with vomiting [74].
• By contrast, an infant with a hyperkalemic acidosis is unlikely to
have IHPS and should be urgently evaluated for other causes,
including adrenal crisis (eg, congenital adrenal hyperplasia).
(See 'Differential diagnosis' below.)
• Blood urea nitrogen (BUN) and creatinine – To help assess for
dehydration and renal insufficiency (eg, pre-renal azotemia due to
dehydration)
• CBC – The CBC should be normal in infants with uncomplicated IHPS.
Abnormal results do not exclude IHPS, but should raise suspicion of
another cause of vomiting (eg, infection).
• Bilirubin – Infants with jaundice should be evaluated for total and
conjugated bilirubin, aspartate aminotransferase (AST), alanine
aminotransferase (ALT) and alkaline phosphatase or gamma-glutamyl
transpeptidase (GGTP).
• Unconjugated hyperbilirubinemia is consistent with IHPS
• If there are elevations in conjugated bilirubin, ALT or AST, the infant
should be evaluated for underlying liver disease.
 Ultrasonography 
• Ultrasound is the procedure of choice if a
sonographer with experience in detecting pyloric
stenosis is available.
• In experienced hands, the sensitivity and specificity
of ultrasonography for IHPS are above 95 percent,
but the accuracy is operator-dependent [76-78].
• If the ultrasound is negative or equivocal but the
patient remains symptomatic, the study should be
repeated a few days to a week later.
• IHPS is characterized by a classic "target" sign on
transverse view.
• The measurements most commonly used are pyloric
muscle thickness (PMT), pyloric muscle length (PML),
and pyloric diameter (PD).
• Criteria defining the upper limits of normal range
from PMT 3 to 4 mm, PML 15 to 19 mm, and PD 10 to
14 mm .
• Measurements within or above these ranges support
the diagnosis of IHPS
• These measurements may not be applicable in
premature infants .
• Normal values vary with the size of the infant, so
results should be interpreted with caution in
particularly small or young infants .
• Each of these measurements has been touted as
the most reliable of the three .
• However, all three measurements typically are
used together in practice
 TREATMENT

• Pyloromyotomy — Definitive management of infantile

hypertrophic pyloric stenosis (IHPS) is surgery.
• The timing of surgery depends upon the clinical status
of the infant.
• If the child is well-hydrated with normal electrolytes,
and if surgeons with expertise in the procedure are
available, surgery may take place on the day of
diagnosis [97].
• Surgery should be delayed in the setting of
dehydration and/or electrolyte derangements [98].
• Infants presenting with normal electrolyte values and mild
dehydration, as is the case with more than 60 percent of
patients, should receive maintenance intravenous fluids
such as 5 percent dextrose with ¼ normal saline (0.22
percent NaCl) and 2 mEq KCl per 100 mL.
• Infants with moderate or severe dehydration require more
intensive fluid management with higher NaCl
concentrations (½ normal saline [0.45 percent NaCl] or
normal saline [0.9 percent NaCl]) and higher rates of
administration (1.5 to 2 times maintenance), perhaps
combined with initial administration of a fluid bolus
• In severely dehydrated infants, renal function
should be assessed prior to adding potassium
to the intravenous fluids.
• If alkalosis is present, this should be corrected
prior to surgery because it has been
associated with an increased risk of post-
operative apnea
• Feeding can be resumed in most infants within
a few hours after surgery. Modest
regurgitation occurs in as many as 80 percent
of infants after pyloromyotomy [8] and should
not delay feedings. Vigorous post-operative
vomiting is infrequent.
• In a meta-analysis, infants offered ad-lib
feedings four hours after operation tolerated
full feedings sooner and had a shorter hospital
stay compared with infants receiving an
incremental feeding schedule, despite having
more emesis episodes [107].
• Radiologic evaluation should be performed if
vomiting persists beyond five days
postoperatively [108], with the understanding
that interpretation of the study may be
difficult because of postoperative swelling.
• Infants with IHPS are at risk for apnea
postoperatively because of their young age
and effects of anesthesia; we suggest
monitoring for apnea for at least 24 hours
postoperatively.
• Those with underlying alkalosis may be at
increased risk based on limited and inferential
data [100]. As mentioned above, this is one
reason why alkalosis and other electrolyte
abnormalities should be corrected prior to
surgery.
• The differential diagnosis of IHPS includes
physiologic gastroesophageal reflux and cow's milk
protein intolerance, which are relatively benign.
• More severe disorders that may present with
vomiting in the first two months of life, include
adrenal crisis, intestinal obstruction (eg, due to
malrotation, Hirschsprung disease, or
intussusception), and liver disease (eg, biliary
atresia or metabolic defects).
• These disorders should be considered in
infants with atypical presenting features,
including hypotension, vomiting that is bilious,
abdominal distension, or jaundice with
conjugated hyperbilirubinemia