1. 1. Dr raghavendra. Fellow in neonatology
2. 2. Pramod
3. 3. Pt name: Promod, age : 1month 18 days s/o nagraj kumbarpet DOB:17/12/2014 DOA :05/02/2015. Single/live/term /AGA term male baby born via naturalis at WCH hospital, davangere on 17/12/2014 at 08.45 AM. Baby cried immediately after birth. Birth wt 2.3 kg
4. 4. C/C : Vomiting after each feed since 7days projectile, non- bilious in nature. Distension of abdomen since 3 days . Obstetric history: Regular ANCs taken, three scan done USGs normal,. taken iron and folicacid supplements, taken 2 dose of TT. Pregnancy uneventful.. Family history: ML: 5 years, 3rd NCM G2P2L1, No h/o of similar complains in parents
5. 5. GPE: 48 day old male baby with vomiting & excessive crying with visible lump in epigastrium well hydrated and alert. Vitals: HR-128 bpm RR- 38 cpm CFT < 3secs Head to toe examination: AF- at level Visible lump over epigastrium Visible gastric peristalsis from lt to rt hypochondrium
6. 6. Anthropometry : Wt-2.3 kg HC-34cm Length-47cm O/E : Colour - pink Cry - Good Activity - good
7. 7. S/E: o CVS: S1 & S2 heard, no murmur o R/S: B/L air entry equal, no added sounds. o CNS: Normal o P/A: Inspection- visible lump over epigastrium, visible gastric peristalsis from lt to rt hypochondrium Palpation palpable mobile mass, firm in cosistency in epigastrium measuring about 3 cm below liver edge Auscultation- bowel sounds present
8. 8. INVESTIGATIONS Complete hemogram TLC- 10740 cells/cumm N- 24% Hb- 11.3 gm/dl L- 58% PCV- 37.9% Platelet count- 3.5 lakhs/cumm CRP - Non Reactive PT, APTT Normal. BBG: AB positive. HBsAg:negative. HIV:negative
9. 9. Discussion 48 days old male baby presenting with projectile non bilious vomiting immediately after feed with visible lump over epigastrium, visible gastric peristalsis from lt to rt hypochondrium with ABG showing metabolic alkalosis,hypokalemia & USG abdomen showing hypertrophic elongated pylorus with dilated stomach favours the diagnosis of IHPS
10. 10. Treatment IV antibiotics and IV fluids Isolyte-P maintainence for 20hrs. Surgery laparoscopic pyloromyotomy done on 06/02/2015. Post operative 5ml feeds BM started after 5 hrs of surgery, increasing slowly every 6 hrs.
11. 11. Introduction Infantile hypertrophic pyloric stenosis (IHPS) is the most common cause of gastric outlet obstruction in children & is one of the most frequent conditions requiring surgery in infants. IHPS is an acquired condition in which the circumferential muscle layer of the pyloric sphinter becomes thickened, resulting in narrowing & elongation of the pyloric channel. This produces a high-grade gastric outlet obstruction with compensatory dilation, hypertrophy & hyperperistalsis of stomach
12. 12. Epidemology The incidence of IHPS is approximately 2 to 5 per 1,000 births per year in most white populations. Less common in India, and among black and Asian populations, with a frequency that is one third to one-fifth that in the white population. The male-to-female ratio is approximately 4:1. There is a familial link, but a hereditary propensity to the development of IHPS is likely polygenic with no single locus accounting for the fivefold increase in the risk of first-degree relatives.
13. 13. Male and female children of affected fathers carry a risk of 5% and 2.5% respectively, of developing IHPS Male and female children of affected mothers carry a risk of 19% and 7%, respectively of developing IHPS Concordance in monozygotic twins is 0.25-0.44 and that in dizygotic twins is 0.05-0.10
14. 14. Normal pylorus
15. 15. In IHPS the pyloric ring is no longer a clearly defined separation between the pyloric canal and duodenum Instead the muscle of the pyloric antrum is hypertrophic (3 or more mm), which separates the normal antrum (1mm thickness) from the duodenum The lumen is filled with compressed and redundant mucosa, which obstructs the passage of gastric contents
16. 16. IHPS anatomy
17. 17. Etiology It has been found that, when compared to controls, in IHPS specimens, the muscle layer is deficient in: the quantity of nerve terminals markers for nerve-supporting cells (Neurotrophins- peptides that govern survival of ENS neurons) nitric oxide synthase activity mRNA production for nitric oxide synthase. Axonal degeneration in both myentric plexus & intramuscular nerves
18. 18. One more hypothesis is that there is dyscoordination b/w gastric peristalsis & pyloric relaxation resulting in, simultaneous gastric & pyloric contraction, & work hypertrophy of the pyloric muscle. More recently an association b/w maternal & infant exposure to erythromycin that stimulates phase 3 MMC (migrating myoelectric complex) activity exposing immature pylorus to high gastric pressures was found leading to development of IHPS . Gastric outlet obstruction due to pylorospasm has been reported in neonates receiving PGE infusions
19. 19. Clinical presentation The typical clinical presentation in a term male infant between 3-6 wks of age having progressive, nonbilious, projectile vomiting and demands to be re-fed soon afterwards ("hungry vomiter") Vomiting consistently follows every feeding & is forceful leading to significant dehydration. Starvation can exacerbate diminished hepatic glucoronyl transferase activity, and indirect hyperbilirubinemia may be seen in 1-2% of affected infants.
20. 20. Prolonged vomiting leads to the loss of large quantities of gastric secretions rich in H+ and Cl- . As a result of dehydration, the kidney attempts to conserve Na+ to maintain volume, by exchanging them for K+ and H+ (paradoxical aciduria). The net result is a loss of H+ and K+, which results in hypokalemic, hypochloremic metabolic alkalosis. Significant hypoglycemia can occur which may precipitate seizures
21. 21. Diagnosis Initially suggested by the typical clinical presentation. The hallmark of diagnosis is the finding of a small, mobile, ovoid mass referred to as an olive in the epigastrium in a calm infant with relaxed abdominal muscles best palpated from the left, located in the midepigastrium beneath the liver edge.. The olive can be felt to roll under the fingertips during sweeping motion- no other structure gives this sensation. If the olive is not palpable in an infant who has a clinical picture suggestive of IHPS, further studies are warranted.
22. 22. Ultrasonography is used to measure the thickness of the pyloric wall and the length of the pyloric canal normal wall thickness 4 mm normal length of the pyloric canal 17 mm - diameter in IHPS > 17mm. If USG is not diagnostic and IHPS remains a concern, the next test of choice is an upper GI contrast studies. The canal is outlined by a string of contrast material coursing through spaces between redundant mucosa (string sign)
23. 23. Double track signShoulder signString sign
24. 24. Differential diagnosis Overfeeding GER Hiatal hernia Pylorospasm Preampulary duodenal obstruction Salt-wasting adrenogenital syndrome
25. 25. Treatment The preoperative treatment is directed towards correcting the fluid, acid-base, and electrolyte losses. Intravenous fluid therapy is begun with 0.450.9% saline, in 5 10% dextrose, with the addition of KCl in concentrations of 3050mEq/L. the infant is rehydrated till normal urine output and the serum bicarbonate concentration is less than 30mEq/dL, which implies that the alkalosis has been corrected. Correction of the alkalosis is essential to prevent postoperative apnea, which may be associated with anaesthesia
26. 26. Infant can undergo the Fredet-Ramstedt pylormyotomy, which is the procedure of choice. If the mucosa is entered (usually on the duodenal side), it can be primarily repaired and reinforced with an omental patch. Large perforations are managed by closing the pyloromyotomy, rotating the pylorus 90, and repeating the myotomy. Mortality and morbidity of less that 0.5%
27. 27. Fredet-Ramstedt pylormyotomy
28. 28. Highly effective simple, elegant, and inexpensive operation. Described as 'one of the most easy and gratifying procedures performed by pediatric surgeons, the most consistently successful operation ever described. Post-operative complications: Wound infection Incomplete myotomy, treated by repeat myotomy or endoscopic balloon dilation
29. 29. Laparoscopic pyloromyotomy is performed for improved cosmesis and shorter operative time. Endoscopic balloon dilation has been used to treat IHPS, most patients failed balloon dilation and were treated with pyloromyotomy
30. 30. Feeding can be resumed within 6 to 12 hours postoperatively with small volume of sugar water advancing volume & osmolarity every 2-3 hrs until infant is taking breast milk Post-operative vomiting may occur in up to 50% of infants, thought to be secondary to edema of the pylorus at the incision site Most infants will tolerate full feeds within 24 to 48 hrs
31. 31. Non-surgical treatment Injection of botulinum toxin, Antispasmodics to relieve pylorospasm Transpyloric nasoduodenal feeding Atropine: Oral or IV atropine has been used in a dose of 0.06 mg/kg/day in eight divided doses, increased by 0.15 mg/kg/day till vomiting ceased and remained so for a period of 24 hours at a stretch, then substituted by oral atropine at double the effective IV dose for 3 weeks
32. 32. Atropine temporarily suppresses spastic contractions of pyloric muscle in pyloric stenosis, and it resulted in cessation of vomiting and eventual regression of pyloric hypertrophy. Medical management may be successful in less severe cases, but treatment takes longer and the complication rate from medical management was higher. Surgical therapy is considered superior to conservative management, since complications are seldom, long-term results are equal, and patients get better sooner.
33. 33. Prognosis Most children treated for HPS have excellent short & long-term outcomes Postoperative USG studies have documented a return to normal muscle thickness within 4 wks a/w healing of pyloric muscle & return of function
34. 34. References Christine A Gleason, Sherin U Devaskar. Averys diseases of newborn: 9th edition. 2012; 984-85 Richard J Martin, Avroy A Fanaroff, Michel C Walsh. Fanaroff & Martins Neonatal and Perinatal medicine: 9th edition 2011; 1415-18 Ashcraft, Keith W; Murphy.J Patrick. Pediatric Surgery: 6th edition.2000; 391-94 Keith T.Oldham, Paul M.Colombani, Robert P.Foglia, Michael A.Skinner. Principles & Practice of Pediatric Surgery: 2005; 1168-72 Google images
35. 35. References Christine A Gleason, Sherin U Devaskar. Averys diseases of newborn: 9th edition. 2012; 984-85 Richard J Martin, Avroy A Fanaroff, Michel C Walsh. Fanaroff & Martins Neonatal and Perinatal medicine: 9th edition 2011; 1415-18 Ashcraft, Keith W; Murphy.J Patrick. Pediatric Surgery: 6th edition.2000; 391-94 Keith T.Oldham, Paul M.Colombani, Robert P.Foglia, Michael A.Skinner. Principles & Practice of Pediatric Surgery: 2005; 1168-72 Google images