By By DR.HISHAM HUSSEIN,M.D LECTURER OF GENERAL SURGERY BENHA UNIVERSITY HOSPITAL 2010.

ByBy

DR.HISHAM HUSSEIN,M.D LECTURER OF GENERAL SURGERY

BENHA UNIVERSITY HOSPITAL

2010

INTRODUCTION ► Hildanus first described pyloric stenosis in 1646.

► Harald Hirschsprung elaborated on the clinical presentation and pathology of the condition in 1888.

► Lobker in 1899 performed a gastrojejunostomy, as the first successful surgery to treat an infant with HPS.

► In 1908 Fredet advocate longitudinal submucosal division of the thickened pyloric muscle, but recommended suturing the defect transversely.

► In 1912 German surgeon Conrad Ramstedt simplified the Fredet procedure by omitting the transverse suturing, leaving the mucosa exposed in the longitudinal seromuscular defect.

Pyloric stenosis is one of the most common surgical conditions of early infancy.

The condition was previously treated medically using a combination of gastric lavage, antispasmodic drugs, dietary manipulation and the application of local heat, because the surgical mortality rate was almost 100%.

INTRODUCTION

Incidence

☼ ☼ 3:10003:1000 live births live births ☼☼ Male: female Male: female 4:14:1 ☼☼ More in More in first bornfirst born babies babies ☼☼ More in More in whitewhite infants infants ☼☼ More duringMore during spring spring and and autumnautumn ☼☼ Four times moreFour times more in children who are their in children who are their mothers have had pyloric stenosis.mothers have had pyloric stenosis. ☼ ☼ Usually appears between Usually appears between 3-6 weeks3-6 weeks of age and rare of age and rare after 3 monthsafter 3 months

Pathophysiology

A- Macroscopically;A- Macroscopically;

☼☼ There is There is progressive hypertrophyprogressive hypertrophy of the of the circularcircular muscle muscle layer of the pylorus leads to gastric outlet obstruction.layer of the pylorus leads to gastric outlet obstruction.

☼☼ The The pylorus becomes becomes elongatedelongated and and thickenedthickened.. ☼☼ The The stomach at at first hypertrophicfirst hypertrophic then then dilated dilated with with gastritis and ulceration in late cases.gastritis and ulceration in late cases.

☼☼ TheThe intestine is is emptyempty and and collapsedcollapsed..

B-Microscopically;B-Microscopically;

* The circular muscle hypertrophies, with increased

connective tissue in the septa between the muscle

bundles.

* An increase of chondroitin sulfate within the

extracellular matrix may account for the cartilaginous

quality of the pyloric tumor.

Pyloric Antrum in “IHPS”. Note the circumferentially thickened muscle , and the lumen is shown as a narrowed canal

Aetiology

The precise cause of “IHPS” remains poorly understood but some proposed the following theories;

1- Postnatal work hypertrophy2- Congenital delay of the pyloric sphincter opening3- Milk curds obstructing the small pyloric channel4- Increased production of gastrin or VIP either by

the infant or the mother5- Maternal stress

6- An association with blood group “O” and “B”.

7- Systemic erythromycin given in the 1st 2 weeks of life.8- Environmental factors.

9- Abnormalities in nitric oxide synthetase.

10- Abnormalities in the interstitial cells of Cajal ( pacemaker cells)

Associated Anomalies with “IHPS” Approximately 4%-7% of infants with pyloric stenosis have

associated anomalies as;

• Hiatal and inguinal hernias being the most common.

• Congenital heart disease. • Esophageal atresia.• Tracheoesophageal fistulas.• Renal abnormalities.• Rubella.• Chromosomal abnormalities such as Turner syndrome and trisomy

18.

Diagnosis Depends on;

A-Clinical PresentationsB- Investigations

A-The classical clinical presentation is ; 1- Vomiting ”it’s the cardinal symptom” * Projectile and explosive * Never bile stained * Immediately after meals * voracious appetite after meal * starts on the 3rd or 4th week after birth

“Any neonate presenting with projectile non bile stained vomiting, with associated hunger and firm stools

should be considered to have IHPS until prove otherwise”

2- 2- Distension;; upper abdominal distension. upper abdominal distension.3- 3- Visible peristalsis propagating from left to propagating from left to right.right.4- 4- Palpable mass in the right upper in the right upper quadrant in up to 90% of cases (Olive quadrant in up to 90% of cases (Olive Like).Like).5- 5- Dehydration and metabolic alkalosis in and metabolic alkalosis in late cases.late cases.6-6- Jaundice in 2% of infants that resolves in 2% of infants that resolves spontaneously.spontaneously.

““The child should be examined in a good light and during a feed”The child should be examined in a good light and during a feed”

Infant with IHPS. Note the protruding rib cage and scaphoid abdomen through which the

distended stomach and prominent peristaltic activity can be seen.

B-Investigations

In addition to careful clinical examination; is almost 100% sensitive for

the diagnosis of “IHPS”. The U/S criteria is;

* Pyloric channel length>17mm * Pyloric muscle diameter>14mm * Pyloric muscle wall thickness>3-4mm

1-Abdominal Ultrasound;

The most common abnormality that mimics “IHPS” is pylorospasm both have been shown the sonographic “Double-track sign”

Ultrasound criteria of “IHPS”

Ultrasound criteria of “IHPS”

Redundant mucosa in the narrowed lumen, which creates 2 mucosal

outlines

Double-track sign

Ultrasound criteria of “IHPS”

Transverse sonographic Transverse sonographic image in a patient with image in a patient with proven hypertrophic proven hypertrophic pyloric stenosis pyloric stenosis demonstrates the demonstrates the “target sign” and heterogeneous and heterogeneous echo texture of the echo texture of the muscular layer.muscular layer.

Transverse sonographic Transverse sonographic image in a patient with image in a patient with proven hypertrophic proven hypertrophic pyloric stenosis pyloric stenosis demonstrates the demonstrates the “target sign” and heterogeneous and heterogeneous echo texture of the echo texture of the muscular layer.muscular layer.

2- Upper gastro-intestinal series

Supine radiograph in an infant with IHPSshows the “caterpillar sign” (A markedly dilated stomach with exaggerated incisura ).

“String sign” or “shouldering” of the hypertrophied pyloric

Muscles bulging into the Gastric lumen

Pylorospasm also mimics IHPS in this imaging modality

☼ Especially HB% and electrolyte esp. Na+ and K+

3- Routine laboratory investigations

Finding a palpable “Olive like” mass onPhysical examination+ characteristic

History can obviate the need for imaging,Thus saving health care cost.

NOTE

TREATMENTTREATMENT A- preoperative preparation; * * Correction of fluid and electrolyte disturbances.Correction of fluid and electrolyte disturbances.

* Correction of anemia.* Correction of anemia.

* Stop oral feeding.* Stop oral feeding.

* Nasogastric tube for decompression.* Nasogastric tube for decompression.

* Application of central venous line.* Application of central venous line.

* Careful respiratory assessment.* Careful respiratory assessment.

Failure to correct preoperative alkalosis may result in post-anesthetic apnea and respiratory arrest.

B- Definitive treatment; I- “Surgery is the gold standard for treatment of IHPS”

Pyloromyotomy;Fredet-Ramstedt extra-mucosal Pyloromyotomy Pyloric traumamyoplasty

Open; either Laparoscopic

Disrupts the hypertrophiedCircular muscle in 2 places

Using Babcock clamp

Transverse upper

Abdominal incision

Intra-corporeal Extra-corporeal

Supra-umbilical

Semicircular incision Vertical incision

Vertical Pyloromyotomy scar Horizontal Pyloromyotomy scar

Laparoscopic pyloromytomyLaparoscopic pyloromytomy

☼ II- Non operative strategies are currently not recommended;

A- I.V Atropine sulfate at an initial dose of 0.4 mg/kg/day and advanced at increments of 0.1 mg/kg/day over an 8 days period until vomiting ceases, then maintained on oral atropine for 2

weeks, this regimen reported to resolve the symptoms but requires prolonged hospitalization and I.V nutrition.

B- Endoscopic balloon dilatation.

C- Endoscopic Pyloromyotomy using an electroscopic needle knife or sphincterotome.

Post-operative care

* Post-operative emesis after Pyloromyotomy is not uncommon may be due to pyloric edema and ileus and usually subsides within the first week.

* Most surgeons introduce oral feeding at least 2-6h after surgery, initially low volume feedings (15 cc) of pedialyte or glucose water then volumes are advanced until the infant is able to tolerate 60 cc to 90 cc of breast milk twice without significant vomiting .

* Most infants are discharged within 24-36h of surgery.

Outcomes

Surgery for IHPS is usually successful and Surgery for IHPS is usually successful and has minimal complications.has minimal complications.

If emesis persists beyond 2 weeks, If emesis persists beyond 2 weeks, concern should be raised for GERD or concern should be raised for GERD or inadequate pyloromyotomy.inadequate pyloromyotomy.