INFANTILE
HYPERTROPHIC
PYLORIC STENOSIS
CASE
28 days old infant with repeated projectile vomiting
Dehydrated
? Tumor felt in right hypochondrium
1.What is it?
Hypertrophy of the smooth muscle of the
pylorus, resulting in the obstruction of
outflow
2. Risk Factors?
Family Hx,firstborn males,decreased
incidence in African-American population.
3. Average Age
Usually from 2 weeks after birth to about 2
months.
CHPS
INCIDENCE
2- 4 in 1,000 live births
more commonly in males
30 percent of cases occur in firstborn
genetic predisposition
Sibling has 5 times more incidence
environmental factors
Neonatal hypergastrinemia and gastric
hyperacidity may have a role
HISTORICAL PERSPECTIVE
First described by Hirschsprung in 1888
Ramstedt described an operative procedure to alleviate the condition
in 1907 – the procedure used to this day to treat pyloric stenosis
ETIOLOGY
Abnormal muscle innervation
Nitric oxide synthetase defect
Neonatal hypergastrinemia
Gastric hyperacidity
Erythromycin
Milk curd theory
Infantile Pyloric Stenosis.pptx
NORMAL PYLORUS
IHPS
PATHOLOGY
Thickened and elongated Pyloric channel
Hypertrophied and redundant pyloric mucosa
Gastritis
Decreased nerve cells, NO syntetase activity in the muscle
MUCOSAL THICKNESS
PATHOPHYSIOLOGY
Failure of pylorus relaxation, Absence, reduction, or delayed maturation of Interstitial
cells of canal ( Intestinal pacemaker)
Increased gastric acid secretion and an increased parietal cell mass with enhanced
release of secretin and cholecystokinin have been described
Prostaglandin E2 was increased in the gastric juice
Increased amounts of insulin-like growth factor, transforming growth factor, platelet
derived growth factor, epidermal growth factor, as well as growth factor receptors in
hypertrophied circular and longitudinal smooth muscle of patients with pyloric
stenosis.
Abnormal innervation of smooth muscle cells of the pylorus in pyloric stenosis.
Recently, interest has been directed at the role of nitric oxide in pyloric stenosis, It
mediates relaxation of smooth muscle,
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").
PALPABLE MASS
The mass is most easily felt immediately
after emesis because it may otherwise be
obscured by a distended antrum and/or
tensed abdominal muscles
VGP(VISIBLE GASTRIC
PARISTALSIS)
Peristaltic waves may be seen progressing
across the child's upper abdomen from left
to right just before emesis.
METABOLIC CHANGES
Repeated vomiting: loss of water, sodium, potassium, chloride, and
hydrogen ions; develops hypochloremic metabolic alkalosis with
hyponatremia and hypokalemia
Sodium Conservation: The urine is initially alkalotic from excreted
bicarbonate, Sodium is mostly reabsorbed in an attempt to maintain
extracellular volume, but there is some obligatory sodium loss
accompanying bicarbonate in the urine, result in paradoxical
aciduria in the presence of a systemic metabolic alkalosis
Metabolic alkalosis from pyloric stenosis can cause hypoventilation
with a resultant hypercapnia.
As dehydration progresses, metabolic acidosis, starvation ketosis,
and possibly hyperkalemia from prerenal failure may be seen
METABOLIC CHANGES
Bicarbonate > 29 mEq/l and serum chloride
< 98 mEq/l are considered indicative of
hypochloremic metabolic alkalosis in pyloric
stenosis.
The severity of dehydration and metabolic
alkalosis
□Mild: serum bicarbonate < 32 mEq/l, serum
chloride > 100 mEq/l,
□Moderate: serum bicarbonate 32-42 mEq/l, serum
chloride 90-100 mEq/1,
□Severe: serum bicarbonate > 42 mEq/l, serum
chloride < 90 mEq/l,
D/D
GER (Gastroesophageal reflex)
Hiatal hernia
Pylorospasm
Preampulary duodenal obstruction
DIAGNOSIS
History collection
Physical examination
ultrasound or upper gastrointestinal (UGI)
contrast study
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
Infantile Pyloric Stenosis.pptx
BARIUM STUDIES
Infantile Pyloric Stenosis.pptx
Infantile Pyloric Stenosis.pptx
DOUBLE TRACK SIGN
NON-SURGICAL
MANAGEMENT
Balloon catheter dilatation of the stenotic pylorus,
Injection of botulinum toxin,
Antispasmodics to relieve pylorospasm,
Transpyloric nasoduodenal feeding
Atropine:Oral or intravenous atropine has been used
successfully in patients with pyloric stenosis.
Atropine temporarily suppresses spastic
contractions of pyloric muscle in pyloric stenosis,
and it resulted in cessation of vomiting and eventual
regression of pyloric hypertrophy.
RAMSTEDT
PYLOROMYOTOMY
OPERATIVE TECHNIQUE
Open approach
□Transverse RUQ(right upper quadrant)
incision
□Supraumbilical curvilinear incision with
midline fascial opening
Laparoscopic
□Decreased post-op emesis
□Less post-op pain and analgesic use
NURSING MANAGEMENT
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. Surgery should be delayed
in the setting of dehydration and/or electrolyte
derangements
HIGH ANAESTHETIC RISK
Small infant
Metabolic derangements
Full stomach
PRE-OP MANAGEMENT
Rehydration:
□May require initial saline bolus
□45% DNS twice the maintanance
□Add potassium 20 meq/L after infant passes urine
□Correction of alkalosis is important: serum chloride of
100 mEq/l and higher generally reflects satisfactory
rehydration,
POST-OP MANAGEMENT
Watch for apnea
Intravenous administration of 5% dextrose in 0.25 to 0.45%
NaCl solution at a maintenance rate
Blood sugar levels may be checked intermittently
COMPLICATIONS OF
SURGERY
Most frequent complications as GER (11 percent), duodenal
perforation (8 percent), and wound infection (5 percent). The
incidence of wound dehiscence and bleeding is very low .
Infantile Pyloric Stenosis.pptx

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Infantile Pyloric Stenosis.pptx

  • 2. CASE 28 days old infant with repeated projectile vomiting Dehydrated ? Tumor felt in right hypochondrium
  • 3. 1.What is it? Hypertrophy of the smooth muscle of the pylorus, resulting in the obstruction of outflow 2. Risk Factors? Family Hx,firstborn males,decreased incidence in African-American population. 3. Average Age Usually from 2 weeks after birth to about 2 months. CHPS
  • 4. INCIDENCE 2- 4 in 1,000 live births more commonly in males 30 percent of cases occur in firstborn genetic predisposition Sibling has 5 times more incidence environmental factors Neonatal hypergastrinemia and gastric hyperacidity may have a role
  • 5. HISTORICAL PERSPECTIVE First described by Hirschsprung in 1888 Ramstedt described an operative procedure to alleviate the condition in 1907 – the procedure used to this day to treat pyloric stenosis
  • 6. ETIOLOGY Abnormal muscle innervation Nitric oxide synthetase defect Neonatal hypergastrinemia Gastric hyperacidity Erythromycin Milk curd theory
  • 10. PATHOLOGY Thickened and elongated Pyloric channel Hypertrophied and redundant pyloric mucosa Gastritis Decreased nerve cells, NO syntetase activity in the muscle
  • 12. PATHOPHYSIOLOGY Failure of pylorus relaxation, Absence, reduction, or delayed maturation of Interstitial cells of canal ( Intestinal pacemaker) Increased gastric acid secretion and an increased parietal cell mass with enhanced release of secretin and cholecystokinin have been described Prostaglandin E2 was increased in the gastric juice Increased amounts of insulin-like growth factor, transforming growth factor, platelet derived growth factor, epidermal growth factor, as well as growth factor receptors in hypertrophied circular and longitudinal smooth muscle of patients with pyloric stenosis. Abnormal innervation of smooth muscle cells of the pylorus in pyloric stenosis. Recently, interest has been directed at the role of nitric oxide in pyloric stenosis, It mediates relaxation of smooth muscle,
  • 13. 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").
  • 14. PALPABLE MASS The mass is most easily felt immediately after emesis because it may otherwise be obscured by a distended antrum and/or tensed abdominal muscles
  • 15. VGP(VISIBLE GASTRIC PARISTALSIS) Peristaltic waves may be seen progressing across the child's upper abdomen from left to right just before emesis.
  • 16. METABOLIC CHANGES Repeated vomiting: loss of water, sodium, potassium, chloride, and hydrogen ions; develops hypochloremic metabolic alkalosis with hyponatremia and hypokalemia Sodium Conservation: The urine is initially alkalotic from excreted bicarbonate, Sodium is mostly reabsorbed in an attempt to maintain extracellular volume, but there is some obligatory sodium loss accompanying bicarbonate in the urine, result in paradoxical aciduria in the presence of a systemic metabolic alkalosis Metabolic alkalosis from pyloric stenosis can cause hypoventilation with a resultant hypercapnia. As dehydration progresses, metabolic acidosis, starvation ketosis, and possibly hyperkalemia from prerenal failure may be seen
  • 17. METABOLIC CHANGES Bicarbonate > 29 mEq/l and serum chloride < 98 mEq/l are considered indicative of hypochloremic metabolic alkalosis in pyloric stenosis. The severity of dehydration and metabolic alkalosis □Mild: serum bicarbonate < 32 mEq/l, serum chloride > 100 mEq/l, □Moderate: serum bicarbonate 32-42 mEq/l, serum chloride 90-100 mEq/1, □Severe: serum bicarbonate > 42 mEq/l, serum chloride < 90 mEq/l,
  • 18. D/D GER (Gastroesophageal reflex) Hiatal hernia Pylorospasm Preampulary duodenal obstruction
  • 19. DIAGNOSIS History collection Physical examination ultrasound or upper gastrointestinal (UGI) contrast study 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
  • 25. NON-SURGICAL MANAGEMENT Balloon catheter dilatation of the stenotic pylorus, Injection of botulinum toxin, Antispasmodics to relieve pylorospasm, Transpyloric nasoduodenal feeding Atropine:Oral or intravenous atropine has been used successfully in patients with pyloric stenosis. Atropine temporarily suppresses spastic contractions of pyloric muscle in pyloric stenosis, and it resulted in cessation of vomiting and eventual regression of pyloric hypertrophy.
  • 27. OPERATIVE TECHNIQUE Open approach □Transverse RUQ(right upper quadrant) incision □Supraumbilical curvilinear incision with midline fascial opening Laparoscopic □Decreased post-op emesis □Less post-op pain and analgesic use
  • 28. NURSING MANAGEMENT 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. Surgery should be delayed in the setting of dehydration and/or electrolyte derangements
  • 29. HIGH ANAESTHETIC RISK Small infant Metabolic derangements Full stomach
  • 30. PRE-OP MANAGEMENT Rehydration: □May require initial saline bolus □45% DNS twice the maintanance □Add potassium 20 meq/L after infant passes urine □Correction of alkalosis is important: serum chloride of 100 mEq/l and higher generally reflects satisfactory rehydration,
  • 31. POST-OP MANAGEMENT Watch for apnea Intravenous administration of 5% dextrose in 0.25 to 0.45% NaCl solution at a maintenance rate Blood sugar levels may be checked intermittently
  • 32. COMPLICATIONS OF SURGERY Most frequent complications as GER (11 percent), duodenal perforation (8 percent), and wound infection (5 percent). The incidence of wound dehiscence and bleeding is very low .