INFANTILE HYPERTROPHIC PYLORIC STENOSIS.pptx

Ihps

1. INFANTILE HYPERTROPHIC PYLORIC STENOSIS:IHPS Presenter Dr . Mengistu .G ( PSR IV) Moderator Dr.Woubedil (pediatric surgeon ) April,2019 4/16/2019 1

2. Introduction IHPS is one of the most common surgical conditions of early infancy. IHPS is the most common cause of GOO in infants Pyloromyotomy for IHPS is a safe and effective procedure which results in a dramatic and rapid improvement in symptoms Hence is one of the most gratifying procedures for surgeons 4/16/2019 2

3. Historical back ground 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 modified the Fredet procedure 1992 Laparoscopic pyloromyotomy 4/16/2019 3

4. Epidemiology Incidence: 1.5-4:1000 live births Male: female 4:1 More in first born babies, white infants & during spring and autumn Fivefold increased risk if one first- degree relatives is affected . Peak incidence is between 3-6 weeks of age and rare after 3 months More than 90 % are diagnosed before the 10th week of life. Premature infants are diagnosed on average 2 weeks later than term Has also been reported in the first few days of life. 4/16/2019 4

5. Infantile Hypertrophic pyloric stenosis: A 2 years (2011-2012) retrospective study from a Tertiary Hospital in Ethiopia Amezene . T, A. Gadisa The prevalence was 12.9/1000 admissions . 87.3% were male and 12.7% were female; the M:F= 6.86:1 90.9% were breastfed infants,7.3% were bottle fed 56.4% were first born The mean age at onset of symptom was 16.4 day 11(20 %) infants started vomiting very early during the first week The mean length of hospital stay in this series was 12.69days, 4/16/2019 5

6. Etiology The precise cause of IHPS remains poorly understood and considered to be multifactorial but some proposed the following; Postnatal work hypertrophy Maternal stress An association with blood group “O” and “B ”. Environmental factors. Trans pyloric feeding of premature babies Systemic erythromycin given in the 1st 2 weeks of life. Genetic 4/16/2019 6

7. GI hormones excess gastrin & substance P, deficiency of NO Neurotrophins have been noted to be decreased , are important for nerve differentiation and survival increase in the gene expression of EGF, TGF-a, and insulin-like growth factor-I 4/16/2019 7

8. Pathophysiology Macroscopically ; There is progressive hypertrophy of the circular muscle layer 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 4/16/2019 8

9. 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. NADPH /nitric oxide synthetase activity not seen in circular muscle biopsy Muscle layer deficient in quantity of nerve terminals ,markers for nerve supporting cells & peptide containing nerve fibers 4/16/2019 9

10. The pylorus becomes elongated and thickened resulting in narrowing of the lumen : GOO 4/16/2019 10

11. 4/16/2019 11

12. End points Dehydration Malnutrition,anemia Hypochloraemic hypokalaemic metabolic alkalosis Paradoxical aciduria Hyperbilirubinemia 4/16/2019 12

13. Associated Anomalies 4%-7% of infants with IHPS have associated anomalies Hiatal and inguinal hernias. Congenital heart disease. Esophageal atresia, Tracheo esophageal fistulas. Renal abnormalities. Chromosomal abnormalities such as Turner syndrome and trisomy 18. No documented anomalies in previously mentioned study in our set up. 4/16/2019 13

14. Diagnosis 1.Clinical Presentations 2.Investigations 1.The classical clinical presentation is ; History Vomiting (is the cardinal symptom) Projectile and progressive Never bile stained Immediately after Feeding voracious appetite after vomiting starts on the 3rd or 4th week after birth Could also be coffee ground matter occasionally Constipation ,diarrhea/starvation stool ,poor weight gain /loss 4/16/2019 14

15. Physical examination Distension; upper abdominal distension. Visible peristalsis propagating from left to right(golf ball waves) Palpable mass in the right upper quadrant in up to 90% of cases (Olive Like). Signs of Dehydration and metabolic alkalosis in late cases. Jaundice in 2% of infants that resolve spontaneously. Obvious signs of malnutrition 4/16/2019 15

16. 4/16/2019 16

17. 4/16/2019 17

18. 2.Investigations Abdominal ultrasound is the method of choice for the diagnosis & it has a sensitivity and specificity of almost 100%. Pyloric channel length>16mm Pyloric muscle diameter>14mm Pyloric muscle wall thickness>3-4mm Sonographic signs Double tract sign Target sign /doughnut sign/bull eye sign Cervical pyloric sign 4/16/2019 18

19. 4/16/2019 19

20. B arium study 4/16/2019 20

21. 4/16/2019 21

22. Laboratory CBC BG&Rh Serum electrolytes ABG 4/16/2019 22

23. 4/16/2019 23

24. Management Preoperative preparation Operative management Open Laparoscopic Non operative management 4/16/2019 24

25. Preoperative preparation Feedings should be withheld NGT—shouldn’t be routine Correction of fluid electrolyte abnormalities 10–20 mL /kg bolus of NS Then, D5/1/2NS + 20–30 mEq/L KCL at a rate of 1.25 - 2 times the calculated maintenance rate Electrolytes should be checked QID Follow V/S and urine output 4/16/2019 25

26. Markers of adequate resuscitation to proceed to operative treatment Dehydration corrected carbon dioxide <30 mmol /L Chloride >100 mmol /L Potassium 3 .5–5.5 mmol /L Sodium >130 mmol /L Urine output >1.5 mL /kg/h Bicarbonate < 30 mEq/L Benson & Alpern stages of severity alkalosis based on HCO3 levels I slight <25 mEq /l II Moderate 26-35 mEq /l III Severe >35 mEq /l Dangers of in adequate resuscitation ;apnea, respiratory arrest & arrhythmia 4/16/2019 26

27. Surgical management Surgery is the gold standard for treatment of IHPS Make sure resuscitation adequate so that fluid, electrolyte and acid base disturbance is corrected Before the induction of anesthesia aspirate the stomach The operative procedure of choice remains the Ramstedt pyloromyotomy Regardless of abdominal access techniques, the myotomy created is identical 4/16/2019 27

28. 1 2 2 4/16/2019 28

29. 3 4 4 4/16/2019 29

30. 4 5 4/16/2019 30

31. 4/16/2019 31

32. After completing myotomy check for Completeness mucosal bulge independent movement of muscle ends, visualizing the circular muscle of the stomach proximally Leak -- by instilling air through NGT Bleeding -- generally venous and almost always stop 6. wound closure 4/16/2019 32

33. 2. Laparoscopic Umbilical port,& two other access sites (RUQ & LUQ) Arthrotomy knife or extended cautery blade for myotomy (serosa) A grasper through LUQ access and extended cautery blade through RUQ access Grasp the duodenum Pyloric spreader is used to complete the myotomy 4/16/2019 33

34. Open Vs Laparascopic No significant differences in complication rates For surgeons with limited exposure or access to laparoscopic techniques and equipment, the “open” Ramstedt pyloromyotomy remains the gold standard Time to achieve full enteral feeding is shorter ( 18.5hrs) in those treated laparoscopically vs open (23.9 hrs ) & Better cosmesis Open Laparoscopic Average Length of Stay (Hrs) 28-74 28-70 Average Time to Full Feeding (Hrs) 20-61 19-35 Average Operative Time (Minutes) 19-33 24-41 Average Wound Infection Rate 0%-7% 0%-6% Average Perforation Rate 0%-6% 0.4%-10% 4/16/2019 34

35. Post operative care Post op pain management Early Post-operative emesis after Pyloromyotomy is common & Is due to pyloric edema ,gastric atony o r GER it is suggested to ‘feed through it introduce oral feeding at least 4-6h after surgery, advancing feedings slowly and in a controlled manner 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 4/16/2019 35

36. 4/16/2019 36

37. Double y pyeloromyotomy The double-Y pyloromyotomy ( Alayet's pyloromyotomy ) seems to be a good technique for the surgical management of IHPS. It offered a better functional outcome in terms of postoperative vomiting during the first postoperative week and weight gain during the first 10 days in our initial series while having a safety profile similar to Ramstedt's pyloromyotomy . 4/16/2019 37

38. Complications Minimal in experienced hands overall complication rate of this is reported to be 3 %. Complications mainly occur when In adequate preoperative preparation insufficient abnormal muscle fibers are split ( Torkelson’s bands), resulting in persistent obstruction, an overly aggressive myotomy results in mucosal injury . 4/16/2019 38

39. 1. Mucosal perforation Mucosal perforation occurs in 1–2% Perforations are usually identified intraoperatively either by direct visualization of mucosa or leaking gastric content or during insufflation of the stomach at the end of the procedure. If a perforation is missed, patients can present with potentially life-threatening intra abdominal sepsis a few days postoperatively. go undetected especially in laparoscopic . 4/16/2019 39

40. Yagmurlu et al. reported a significantly higher rate of perforation with open pyloromyotomy when compared to the laparoscopic approach (3.6% versus 0.4%). The most common point of mucosal entry is at the distal part of the incision at the duodenal–pyloric junc ­ tion . is appropriate to leave a few pyloric muscle fibers intact at the duodenal end to reduce the risk for duodenal perforation 4/16/2019 40

41. Management Repairing the mucosal tear with interrupted fine absorbable sutures followed by placement of an omental patch if perforation is small & at deodnal end closing the myotomy and performing a new myotomy at a different site 90–180 ° from the original incision if perforation is big and at the middle. The stomach should be decompressed by nasogastric tube 4/16/2019 41

42. Feedings should be held for 24 hours and then restarted and a contrast study might also performed to document the absence of a leak prior to resuming feeds. Patients also benefit from postoperative prophylactic antibiotics. Time of recognition of perforation is very crucial in predicting prognosis 4/16/2019 42

43. 2. Persistent vomiting Persitent dehydration , weight loss and severe electrolyte imbalance . beyond 3-4 days suspect Incomplete myotomy . An UGI imaging study with oral contrast seems like the logical first step. Unfortunately , an UGI study is not as such useful during the immediate postoperative period If persists >1 week, re exploration & redo surgery. laparoscopic pyloromyotomy seems to be associated with a higher risk of incomplete myotomy when compared to the open approach(2.2 vs 0) recurrence is more likely on the gastric side (incomplete myotomy ) 4/16/2019 43

44. 3.deserosalization or full thickness damage to the duodenal wall more common with the laparoscopic technique and trans umbilical approach of open type 4.Continued postoperative bleeding 4/16/2019 44

45. 5.wound infection similar to other clean operation 2% risk factors for wound infection are a poorly epithelialized umbilical stump , mucosal perforation and trans umbilical approach In a trans umbilical approach 7%, & use of prophylactic antibiotics when using the trans umbilical approach is recommended Local wound care 6.Other complication such as incisional hernia ,post operative adhesion 4/16/2019 45

46. Prognosis Excellent. Once adequately treated, pyloric stenosis does not recur. Mortality is rare after pyloromyotomy Infantile Hypertrophic pyloric stenosis: A 2 years (2011-2012) retrospective study from a Tertiary Hospital in Ethiopia Amezene . T, A. Gadisa There were 6 (10.9%) intra-operative mucosal perforation. 6(10.9 %) wound infections , 2(3.8 %) pneumonia. Two patients died(3.6%) 4/16/2019 46

47. Non operative management Are currently not recommended 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, Endoscopic balloon dilatation . Endoscopic Pyloromyotomy using an electroscopic needle knife or sphincterotome . 4/16/2019 47

48. R eferences 4/16/2019 48

49. THANK YOU !! 4/16/2019 49