Congenital hypertrophic pyloric stenosis

Pyloric stenosis.

Stomach a) fundus b) corpus c) cardia d) antrum pyloricum e) canalis pyloricus f) duodenum g) oesophagus.

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Hypertrophic pyloric stenosis

Hypertrophic pyloric stenosis is an acquired diffuse hypertrophy and hyperplasia of the smooth muscle of the pylorus and the entire stomach.

Etiology[edit | edit source]

Etiology is unknown, polygenic inheritance and environment are assumed. Familial occurrence was demonstrated in 15%. It is sometimes associated with hiatal hernia, esophageal atresia or Turner syndrome. The incidence of the disease varies greatly geographically, with around 1:5000 live births in the Czech Republic, it is up to five times more often in first-born boys. Newborns and infants between 3-6 weeks of life are affected. Children older than 3 months are rarely affected.

Clinical picture[edit | edit source]

Explosive arc vomiting dominates, projectile (up to 1 m);
Vomit contains acidic gastric juices, usually the content is digested milk, vomit is free of bile;
there is dehydration, the child has a large appetite, he/she drinks eagerly; he/she loses weight (dehydration, insufficient caloric intake)
the child is lethargic, has constipated or hungry stools, has an olf-fashioned appearance;
can lead to severe hypochloremic alkalosis and hypokalemia - severe condition, shallow breathing, loss of consciousness, convulsions - coma pyloricum;
A peristaltic wave (from left to right epigastrium) can be observed on the abdomen immediately after drinking;
with gentle palpation, about 70% of children have palpable resistance = olive, the size of a cherry in the epigastrium, to the right of the midline - tumor pylori;
icterus may occur rarely

Laboratory examination[edit | edit source]

typically hypochloremic alkalosis with hypokalemia, hyponatremia and dehydration;
hypochloremia can reach extreme values (below 75 mmol / l), its degree better reflecting potassium loss than potassium;
elevated gastrin levels

Diagnostics[edit | edit source]

clinical picture
abdominal ultrasound
- the length (17 mm and more) and width (4 mm and more) of the pyloric channel are measured
- sensitivity 97%
X-ray contrast examination (GIT passage) is currently used for diagnostic doubts
- stomach dilatation
- elongated and narrow pyloric canal (so-called rail or shoelace image)
- contrast medium must be aspirated with a nasogastric tube after examination (possible aspiration)
- rapid passage of contrast through the stomach eliminates pyloric stenosis
- this test may reveal other causes of vomiting without bile:
  - gastric atony
  - delayed gastric emptying,
  - gastroesophageal reflux

Differential diagnosis[edit | edit source]

other causes of explosive vomiting:
- intracranial hypertension,
- pyloric atresia,
- antral membrane,
- stomach duplications,
- gastric atony
- delayed gastric emptying,
- gastroesophageal reflux;
other causes of similar metabolic breakdown:
- acute adrenal insufficiency - bleeding or congenital hyperplasia - MAc,
- hyperkalemia,
- sodium loss in urine;
hereditary metabolic disorders – Metabolic alkalosi s can cause disorders of AMK metabolism (urea cycle disorder).

Therapy[edit | edit source]

a conservative approach is not recommended
the treatment is surgical
- longitudinal pyloromyotomy of hypertrophic pyloric muscle is most often performed (Weber-Ramstedt operation):
  - it begins with a transverse laparotomy in the right part of the epigastrium
  - the straight abdominal muscles and the oblique abdominal muscles are intersected longitudinally
  - after opening the peritoneal cavity, a hypertrophic pylorus is luxated into the surgical wound
  - a sharp longitudinal incision is made of serosa and superficial muscle fibers (the incision starts 1-2 mm from the pyloroduodenal junction and ends in the area of the pyloric junction in the stomach)
  - the muscle fibers are separated (along the entire length of the incision) by blunt dissection
  - complication is perforation of the mucosa - this must be sutured with absorbable material and covered with omentum
- patients with more than 5% weight loss, metabolic alkalosis and hypochloremia must parenterally rehydrate and correct the internal environment within 24 hours before surgery
can also be performed by laparoscopic technique
prognosis - good with timely operation.