Infantile hyperthropic pyloric stenosis

IHPS
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1. بسم الله الرحمن الرحیم 2/8/2021 1

2. Nangarhar University Teaching Hospital Surgery ward Topic to be presented Infantile Hypertrophic Pyloric Stenosis ( IHPS ) Presenter Dr. Abdullah Ihsaas 3 rd Year TMO of GS Guidance Associate prof Dr . Gul aqa sadat Date 1399/11/16 2/8/2021 2

3. Anatomy of stomach Stomach is most dilated part of gastrointestinal tract and has a J-like shape Positioned between the abdominal esophagus and the small intestine Located in epigastrium , Umbilical and lift hypochondrium regions of the abdomen Divided into four regions Cardia Fundus Body Pyloric part: which is divided into Pyloric antrum Pyloric canal Pylorus contained thick circular muscle the pyloric sphincter  symphatitic and ganglion innervation 2/8/2021 3

4. The GI tract contains four layers: Mucosa ( the innermost layer)  epithelium , lamina propria , muscularis mucosa sub mucosa muscularis propria ( smooth muscle layer )   : There are usually two layers the inner layer is circular the outer layer is longitudinal are used for peristalsis (rhythmic waves of contraction) adventitia Or serosa ( the outermost layer ): of loose connective tissue - covered by the visceral peritoneum. Contains blood vessels, lymphatic's and nerves. Layers of the Gastrointestinal Tract 2/8/2021 4

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6. Function of pylorus Food from the stomach, as  chyme, passes through the pylorus to the  duodenum . The pylorus, through the pyloric sphincter , regulates entry of food from the stomach into the duodenum . Gastric Juice : Hydrochloric acid Mucus pepsin Lipase 2/8/2021 6

7. Innervation innervated by intrinsic neurons of the enteric nervous system (ENS) and by the axons of extrinsic sympathetic, parasympathetic and visceral afferent neurons . Both the intrinsic and the extrinsic innervation, to different degrees, are affected by age 2/8/2021 7

8. History Hildanus in 1627 the first auter who described case of IHPS, how ever the history dos not fit, child recover spontaneously it is likely a case of overfeeding Patrick Blair in 1717 described a more typical clinical and postmortem finding of IHPS first complete description was by Herald Hirsch sprung in 1888 and named it angeborener pylorus stenosis ( congenital pyloric stenosis ) Cordua  jejunostomy 1892 Lobker  gastroenterostomy 1898 Nicolle tried  pyloric dilatation via gastrostomy Pyloroplasty and external mucosal pyloroplasty  nicool, Dent, fredet and weber On 23 august 1911 Wilhelm Conrad Ramstad firs performed his pyloromyotomy without transversal suture On 18 June 1912 he repeated his new technique on another male patient and reported these two canes to the national science assembly at Munster, Ramstad operation is nowadays a stander surgical technique for the IHPS. 2/8/2021 8

9. Infantile hypertrophic pyloric stenosis One of the most common surgical condition of the newborn A condition characterised by concentric hypertrophy of the muscle involving the internal circular layer of pylorus on gastric side Produces a gastric outlet obstruction 2/8/2021 9

10. Epidemiology Incidence It occurs at a rate of 1 to 4 per 1000 live births Commonly occur in causation in ( whites ) but is less common in ( Africans and Asians) Males are affected more often with a 4:1 male to female ratio . Present in infants between 3 and 8 weeks of age First born Preterm infants 2/8/2021 10

11. Etiology and risk factors but Genetic and environmental factors appear to play a large role in pathophysiology Environmental factors associated with HPS include The method of feeding Seasonal variability Exposure to erythromycin in first 2 weeks of age has been reported There has been lot of research on this subject and various factors Several gastro intestinal peptides or growth factors that may facilitate pyloric hypertrophy some of these include Substance p – decreased neurotrophins, deficient nitric oxide synthase and gastrin hyper secretion Ultrastructure abnormalities of enteric nerves and the interstitial cells of cajal have been implicated 2/8/2021 11

12. Cont. A cohort study found that treatment of young infants with macrolide antibiotics was strongly associated with infantile hypertrophic pyloric stenosis (IHPS ). Maternal use of macrolides during the first 2 weeks after birth was also associated with an increased risk of IHPS.   2/8/2021 12

13. Risk factors including Family history Maternal Younger maternal age <25y Maternal smoking during pregnancy Fatal Gender Being a first born infant Premature infants are diagnosed with HPS later than term or post term infants 2/8/2021 13

14. Associated anomaly's Golden role in children's Some is Genetically x linked described by spicer esophageal atresia and neuronal dysfunction Turner syndrome Trisomy 18 Other Non Genetic Malformations Inguinoscrotal pathology Encephalocele Hydrocephalus Cardiac malformation Gastro esophageal reflux Hiatus hernia Jaundice described with IHPs usually resolves after pyloromyotomy 2/8/2021 14

15. Clinical manifestation Often start with Vomiting at 3 - 8 week of life, and some time earlier Post prandial – always Forceful – non bilious Remain hunger The vomit contains milk or curds It can have a brownish discoloration or have ‘ ’Coffee ground ” appearance caused by chronic gastritis 2/8/2021 15

16. Physical examination Physical examination mad diagnosis 75 % Start by a feeding of formula or breast feed if the infant state allows it ( alert, active ) Inspection: Visible gastric peristaltic waves that progress successively from the upper left to the mid right of the abdomen . ( golf ball waves ) Palpation: The examiner should stay on the left of the patient while baby is supine in mothers arms , relaxed , warmed reassured 2/8/2021 16

17. Physical examination The left hand is placed are the epigastrium with the mid finger tip just above and to the right of the umbilicus. The right hand is beneath the infant for support , giving a slight extension to the back in order to ease the palpation of the ( olive ) which is pushed forward by the vertebrae. If it is not palpated , then the test is repeated just after an episode of vomiting or decompression of stomach via NGT 10fr, 12fr Jaundice 2 % cases Dehydration and malnutrition such as poor weight gain , weight loss , marasmus, decreased urinary output, lethargy, and shock 2/8/2021 17

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19. Assessment and Diagnostic Findings Diagnosis is usually made on the clinical evidence . Laboratory studies: Electrolytes , pH, BUN, and creatinine levels should be obtained at the same time as intravenous access in patients with pyloric stenosis. Ultrasonography .   If the clinical presentation is typical and an olive is felt , the diagnosis is almost certain however formal ultrasonography is still recommended to confirm the diagnosis. Sensitivity and specificity approach 100% Diagnostic criteria  pyloric muscle thickness of 4mm or more and pyloric channel length of 16mm or more Radiography: Barium swallow show an abnormal retention of barium in the stomach and increased peristalsis . String sign  contrast within the narrow lumen Shoulders  bulging of the pyloric muscle protruding into the antrum 2/8/2021 19

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21. Differential Diagnosis DDx for nonbiloius vomiting should be considered This include medical and surgical conditions Medical Pylorospasm Gastric atony GERD Gastroenteritis Increased intracranial pressure And metabolic disorders Surgical or Anatomic causes Gastric Antral web Pyloric atresia Foregut duplication cyst Gastric tumors or a tumor causing extrinsic gastric compression 2/8/2021 21

22. Pathophysiology Child with uncorrected metabolic alkalosis will hypo ventilate to normalize serum PH ( compensatory respiratory acidosis ) clinically this translates to an inability to wean from mechanical ventilation post operatively. 2/8/2021 22

23. Pre operative management Correction of dehydration and electrolyte abnormalities is obligatory before bringing the infant to operating room Pre operative fluid resuscitation Type of fluid : 5% Dextrose with 0.45% saline. Add 2mmol of KCl in each 100 of fluid Volume: 150 ml/kg of body Wight /24 hr. Correct hypovolaemia with 10ml /kg 0.9% saline The most important factor is serum HCO3 level 25 – 28 mEq/L Close attention is paid to urinary output ,over all perfusion, repeat lab investigation All enteral feedings are held prior to surgery (NPO) There is no need for nasogastric decompression for long time if there was any radiopaque 2/8/2021 23

24. Management Medical Operative management Open approach Laparoscopic approach 2/8/2021 24

25. Medical Reports of medical treatment with atropine and pyloric dilation but require long periods and are often not effective 2/8/2021 25

26. 2. Operative management The mainstay of therapy is typically resuscitation followed by pyloromyotomy . The open approach Several incisions have been described for the open approach The typical right upper quadrant transverse incision seems to be commonly More cosmetically pleasing incision is Omega – shaped incision around the superior portion of the umbilicus Pylorus is delivered through the incision A longitudinal serosal incision is made in the pylorus approximately 2 mm proximal to the junction of the duodenum an is carried onto the anterior ga stric wall for approximately 5 mm. 2/8/2021 26

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28. Cont.. Blunt dissection  divided the firm pyloric fibers using the handle of a scalpel. Until the pyloric sub mucosal layer is seen and slightly protruded Care must be exercised when dividing fibers Ensure independent movement of mucosa in edges The mucosal integrity can be checked by installing air through the NG tube if there are no leakage air should suctioned If there was perforation then close it with omental patch 2/8/2021 28

29. 2. Laparoscopic pyloromyotomy Alain et al first describe extra mucosal pyloromyotomy 2/8/2021 29

30. Laparoscopic vs open approach The large randomized controlled series comparing open vs laparoscopic pyloromyotomies showed less post operative pain, and emesis, and fewer complications in the laparoscopic group but no difference in length of hospital stay or operating time Laparoscopic surgery need great deal of experience 2/8/2021 30

31. Post operative care Rapid resolution of emesis Short duration of analgesic requirement Significant improvement in feeding tolerance  start 15 -30 ml per feed several hour after the conclusion of the operation some other ( 18 – 24 hr. ) Persistent emesis while in the hospital giving H2 blocker or PPI 2/8/2021 31

32. Complications Mucosal perforation Wound infection Emesis Incisional hernia Dehiscence Recurrent pyloric stenosis 2/8/2021 32

33. Outcomes In the past, the mortality from pyloric stenosis was considerable and approached 50%. Today , however, mortality is nearly zero with improvement in neonatal resuscitation and anesthesia as well as surgical techniques . Morbidity is also significantly lower than in the past an overall complication rate between 1–2%. Additionally , 2/8/2021 33

34. Summary points 2/8/2021 34

35. Reference 2/8/2021 35

36. Thanks From Your Attention 2/8/2021 36