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

1. INFANTILE HYPERTROPHIC PYLORIC STENOSIS"IHPS" By DR.HISHAMHUSSEIN,M.D LECTURER OF GENERAL SURGERY BENHA UNIVERSITY HOSPITAL 2010

2. INTRODUCTION ► Hildanus first described pyloric stenosis in 1646. ►Harald Hirschsprungelaborated on the clinical presentation and pathology of the condition in1888. ►Lobker in 1899 performed a gastrojejunostomy, as the first successful surgery to treat an infant with HPS. ►In 1908Fredet 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.

3. INTRODUCTION • 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%.

4. Incidence ☼3:1000 live births ☼ Male: female 4:1 ☼ More in first born babies ☼ More in white infants ☼ More during spring and autumn ☼Four times more in children who are their mothers have had pyloric stenosis. ☼ Usually appears between 3-6 weeks of age and rare after 3 months

5. Pathophysiology A- Macroscopically; ☼There is progressive hypertrophy of the circular muscle layer of the pylorus leads to gastric outlet obstruction. ☼ The pylorus becomes elongated and thickened. ☼ The stomach at first hypertrophic then dilated with gastritis and ulceration in late cases. ☼ The intestine is empty and collapsed.

6. 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.

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

8. Aetiology The precise cause of “IHPS” remains poorly understood but some proposed the following theories; 1- Postnatal work hypertrophy 2-Congenital delay of the pyloric sphincter opening 3-Milk curds obstructing the small pyloric channel 4- Increased production of gastrin or VIP either by the infant or the mother 5-Maternal stress

9. 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)

10. 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.

11. 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”

12. 2- Distension; upper abdominal distension. 3- Visible peristalsis propagating from left to right. 4- Palpable mass in the right upper quadrant in up to 90% of cases (Olive Like). 5- Dehydration and metabolic alkalosis in late cases. 6-Jaundice in 2% of infants that resolves spontaneously. “The child should be examined in a good light and during a feed”

13. Infant with IHPS. Note the protruding rib cage and scaphoid abdomen through which thedistended stomach and prominent peristaltic activity can be seen.

14. 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”

15. Ultrasound criteria of “IHPS”

16. Ultrasound criteria of “IHPS” Redundant mucosa in the narrowed lumen, which creates 2 mucosal outlines Double-track sign

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

18. 2- Upper gastro-intestinal series Supine radiograph in an infant with IHPS shows 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

19. 3- Routine laboratory investigations ☼ Especially HB% and electrolyte esp. Na+ and K+ Finding a palpable “Olive like” mass on Physical examination+ characteristic History can obviate the need for imaging, Thus saving health care cost. NOTE

20. TREATMENT • A- preoperative preparation; * Correction of fluid and electrolyte disturbances. * Correction of anemia. * Stop oral feeding. * Nasogastric tube for decompression. * Application of central venous line. * Careful respiratory assessment. Failure to correct preoperative alkalosis may result in post-anesthetic apnea and respiratory arrest.

21. B- Definitive treatment; I- “Surgery is the gold standard for treatment of IHPS” Pyloromyotomy; Fredet-Ramstedt extra-mucosal Pyloromyotomy Pyloric traumamyoplasty Laparoscopic Open; either Disrupts the hypertrophied Circular muscle in 2 places Using Babcock clamp Intra-corporeal Extra-corporeal Transverse upper Abdominal incision Supra-umbilical Semicircular incision Vertical incision

22. Horizontal Pyloromyotomy scar Vertical Pyloromyotomy scar

23. Laparoscopic pyloromytomy

25. ☼ 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.

26. 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.

27. Outcomes • Surgery for IHPS is usually successful and has minimal complications. • If emesis persists beyond 2 weeks, concern should be raised for GERD or inadequate pyloromyotomy.

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