Infantile hypertrophic pyloric stenosis

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Infantile hypertrophic pyloric stenosis

• DR KAZEMI
• Alzahra hospital
• pediatric
• kmazemi_at_med.mui.ac.ir.com
• 1382

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Infantile hypertrophic pyloric stenosis

• Infantile hypertrophic pyloric stenosis (IHPS) is
  a condition of hypertrophy of the pylorus, with
  elongation

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Infantile hypertrophic pyloric stenosis
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etiology

• 3 in 1,000 live births
• more commonly in males
• 30 percent of cases occur in firstborn
• genetic predisposition
• environmental factors
• Neonatal hypergastrinemia and gastric
  hyperacidity may have a role

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etiology

• An association between IHPS and the
  administration of oral erythromycin given for
  postexposure prophylaxis for pertussis has been
  described 11-14.

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CLINICAL MANIFESTATIONS

• The classic presentation of IHPS is the three- to
  six-week-old baby who develops immediate
  postprandial, non-bilious, often projectile
  VOMITING and demands to be re-fed soon afterwards
• (a "hungry vomiter").

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CLINICAL MANIFESTATIONS

• In another review of infants diagnosed between
  1957 to 1969, the mean age in which the infants
  began
• vomiting was 22 days 15

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CLINICAL MANIFESTATIONS

• olive" typically was quite high
• (up to 92 percent) in a report from 1975, but is
  less common in more recent reports 4,5. The
  "olive" is most easily felt immediately after
  emesis because it may otherwise be obscured by a
  distended antrum and/or tensed abdominal muscles

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CLINICAL MANIFESTATIONS

• Peristaltic waves may be seen progressing across
  the child's upper abdomen from left to right just
  before emesis.

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Laboratory evaluation

• hypochloremic, metabolic alkalosis
• loss of large amounts of gastric hydrochloric
  acid
• hypokalemia typically is not seen early on but is
  quite common in babies who have been vomiting for
  longer than three weeks

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Clinical associations

• One of the most common associations is an
  unconjugated hyperbilirubinemia
• concomitant alpha-1-antitrypsin deficiency

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Clinical associations

• Midgut malrotation has been described in up to 5
  percent
• An association with malrotation, congenital short
  bowel, and IHPS also has been reported but is
  much less common

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Clinical associations

• An absent or hypoplastic mandibular frenulum, as
  well as joint hypermobility, has been observed

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Clinical associations

• eosinophilic gastroenteritis 28, gastric ulcer
  29, hiatal hernia 30, diaphragmatic hernia
  31, congenital heart disease 32, propionic
  acidemia 33, congenital nephrotic syndrome
  34, and congenital hypothyroidism 35. At
  least one case of congenital pyloric stenosis has
  been diagnosed in an infant with an intrauterine
  history of polyhydramnios 36.

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Clinical associations

• A clinical picture of IHPS was described in a
  one-month-old infant with familial hyperlipidemia
  who had an intense hyperechogenicity of the
  thickened pyloric muscle on ultrasound and fatty
  infiltration of the pyloric muscle layer at
  surgical exploration 37. The symptoms resolved
  after implementation of a fat-restricted diet.

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DIAGNOSIS

• "olive" is palpable
• ultrasound or upper gastrointestinal (UGI)
  contrast study 10,38
• pyloric muscle length (PML), and pyloric diameter
  (PD). Published criteria have ranged from 3 to 4
  mm for PMT, 15 to 19 mm for PML, and 10 to 14 mm
  for PD 39-41.

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Infantile hypertrophic pyloric stenosis
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Barium studies

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An upper GI series reveals a point of obstrution
at the fourth part of the duodenum
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An upper GI series demonstrates malposition
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surgical
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Ultrasound upprr gi
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Upper endoscopy
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DIAGNOSIS

• gastric volume and serum acid-base
  determinations. In one study, IHPS was present in
  nearly 92 percent of infants with a nasogastric
  aspirate of 10 mL or more after three to four
  hours of fasting 55.

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DIAGNOSIS

• GER was diagnosed in nearly 86 percent of those
  with aspirates of less than 10 mL. Another study
  showed that serum pH gt7.45, chloride gt98, and
  base excess gt3 gave a positive predictive value
  of 88 percent in diagnosing IHPS in infants
  presenting with vomiting 56.

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TREATMENT

• Oral and intravenous atropine sulfate (which
  relaxes the pyloric musculature) also have been
  described but are uncommonly used 58,59.
  Because of the safety and efficacy of surgery,
  conservative management should be reserved for
  infants in whom a surgical approach is either not
  advisable or feasible.

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TREATMENT

• The timing of surgery depends upon the clinical
  status of the infant. If the diagnosis is made
  early and the child is well-hydrated with normal
  electrolytes, surgery may take place on the day
  of diagnosis 60. Surgery should be delayed in
  the setting of dehydration and/or electrolyte
  derangements 61

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TREATMENT

• . Infants with moderate or severe dehydration
  require more involved fluid percent management
  with higher NaCl concentrations (0.5 to normal
  saline) and higher rates of administration (1.5
  to 2 times maintenance), perhaps combined with
  bolus administration initially.

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TREATMENT

• The correction of alkalosis prior to surgery is
  imperative because alkalosis has been associated
  with an increased risk of post-operative apnea
  62.

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TREATMENT

• Caution must be taken in the more severely
  dehydrated infants to ensure the kidneys are
  functional prior to the addition of KCl to the
  intravenous fluids. The correction of alkalosis
  prior to surgery is imperative because alkalosis
  has been associated with an increased risk of
  post-operative

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TREATMENT

• Endoscopically-guided balloon dilation for IHPS
  has been described

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TREATMENT

• An earlier study cited its most frequent
  complications as GER (11 percent), duodenal
  perforation (8 percent), and wound infection (5
  percent) 70. The incidence of wound dehiscence
  and bleeding is very low 71.

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TREATMENT

• regurgitation can be as high as nearly 80 percent
  5, and its presence should not delay the
  institution of post-operative feedings.

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