GROUP I. INFANTILE HYPERTROPHIC PYROLIC STENOSIS AND HIRSCHSPRUNG'S DISEASE.pptx

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1. INFANTILE HYPERTROPHIC PYLORIC STENOSIS VS HIRSCHSPRUNG’S DISEASE BY GROUP I

2. GROUP I MEMBERS LAMBERT MURWANASHYAKA 218003320 FABRICE BYRINGIRO NDAHIGWA 218003291 HERITIER THIERRY NSHIZIRUNGU 218006773 JEAN BOSCO MANISHIMWE 218001489 SHABAN MURISA 218002755 GENTILLE UWAMAHORO 217045499

3. INTRODUCTION Infantile hypertrophic pyloric stenosis Hypertrophy and hyperplasia of the muscular layers of the pylorus Gastric outlet obstruction First case reported in 1717, clearly described by Hirschprung in 1887 Hirschsprung’s disease Aganglionosis of the colon Result in failure of the colon to relax First described i n 1886, Harold Hirschsprung as a cause of constipation in early infancy

4. INTRODUCTION Infantile hypertrophic pyloric stenosis Hirschsprung’s disease

5. EPIDEMIOLOGY Infantile hypertrophic pyloric stenosis 2 to 3.5 per 1000 live births Male to female ratio 4:1 Age: 3-6 weeks, rare after 12 weeks Common in whites than blacks and rare in asians Hirschsprung’s disease 1 Per 5000 live births Male to female ratio 4:1 90% newborn, 10% other age groups Common in Asian Americans

6. ETIOLOGY Infantile hypertrophic pyloric stenosis Unknown etiology Risk factors : Family history( genetic) Use of macrolides antibiotics(erythromycin and azithromycin) Bottle feeding Hirschsprung’s disease Unknown etiology Risk factors : Family history (genetic) Congenital anomalies (Down syndrome and other abnormalities present at birth)

7. PATHOPHYSIOLOGY Infantile hypertrophic pyloric stenosis Hypertrophy of muscles of pylorus Narrowing of the lumen of gastric antrum Gastric outlet obstruction Hirschsprung’s disease Aganglionosis due to neural crest cells : Defect in the migration Failure in proliferation Apoptosis Improper differentiation Failure in relaxation of colon

8. CLINICAL PRESENTATION Infantile hypertrophic pyloric stenosis Postprandial Non-bilious projectile vomiting Persisting hunger( hungry vomiters) Weight loss Lethargy Olive like mass in RUQ Visible gastric peristalsis Signs of dehydration 14% cases: jaundice Hirschsprung’s disease Bilious vomiting Failure to pass meconium > 48hrs Poor feeding Distended abdomen Chronic constipation in infants and toddlers Fever in 10% of cases Failure to thrive

9. CLINICAL PRESENTATION Infantile hypertrophic pyloric stenosis Hirschsprung’s disease

10. DIFFERENTIAL DIAGNOSIS Infantile hypertrophic pyloric stenosis Gastroesophageal reflux Cow's milk protein intolerance Adrenal crisis Intestinal obstruction Liver disease Hirschsprung’s disease Hypothyroidism Intestinal atresia Intestinal malrotation Small left colon syndrome Functional ileus Meconium plug syndrome

11. DIAGNOSIS Infantile hypertrophic pyloric stenosis Ultrasound is gold standard Diagnostic criteria Pyloric muscle thickness (PMT)> 3-4mm pyloric muscle length (PML) >15-19mm Barium meal: string sign, beak sign Hirschsprung’s disease Rectal biopsy: absence of ganglionic cells( the gold standard) Barium enema: distended upper colon and thin terminal colon Anorectal manometry

12. DIAGNOSIS Infantile hypertrophic pyloric stenosis (Ultrasound Diagnostic criteria) Hirschsprung’s disease (Barium enema)

13. Infantile hypertrophic pyloric stenosis Barium meal: string sign, beak sign

14. MANAGMENT Infantile hypertrophic pyloric stenosis Fluid and electrolytes resuscitation NGT to empty the stomach Foley catheter to monitor urine out put Surgical: Pyloromyotomy Hirschsprung’s disease Fluid resuscitation Rectal irrigation(enema) Antibiotics Surgery: Swenson Procedure, Soave Procedure,Colostomy

15. COMPLICATIONS Infantile hypertrophic pyloric stenosis Severe dehydration Hypovolemic shock Aspiration pneumonia Mucosal perforation Incomplete pyloromyotomy Postop infections Hirschsprung’s disease Enterocolitis Sepsis Intestinal perforation Post-op fecal incontinence( anal sphincter dysfunction)

16. REFERENCES von Sochaczewski CO, Muensterer OJ. The incidence of infantile hypertrophic pyloric stenosis nearly halved from 2005 to 2017: analysis of German administrative data. Pediatric Surgery International. 2021 May;37(5):579-85. Chirdan LB, Ameh EA, Hughes-Thomas A. Infantile hypertrophic pyloric stenosis. InPediatric Surgery 2020 (pp. 631-637). Springer, Cham. Zvizdic Z, Halimic T, Milisic E, Jonuzi A, Halimic JA, Vranic S. Infantile hypertrophic pyloric stenosis in Bosnia and Herzegovina: A retrospective cohort study from the largest tertiary care facility. Asian Journal of Surgery. 2021 Nov 17. Nelson Textbook of Pediatrics, 21st Edition 2020 https://onlinelibrary.wiley.com/doi/epdf/10.1002/bdra.23269 https://www.uptodate.com/contents/congenital-aganglionic-megacolon-hirschsprung-disease?search=Hirschsprung%27s%20disease&source=search_result&selectedTitle=1~70&usage_type=default&display_rank=1 https://emedicine.medscape.com/article/178493-overview#showall https://pubmed.ncbi.nlm.nih.gov/6481580/