Endoscopic treatment for cicatricial pyloric stenosis in children

Pyloric stenosis
is a narrowing of the opening of the stomach towards the first part of the small intestine (pylorus). [1] Symptoms include projectile vomiting without the presence of bile. [1] This most often occurs after feeding the baby. [1] The typical age at which symptoms become apparent is between two and twelve weeks. [1]

The cause of pyloric stenosis is not clear. [2] Risk factors in infants include birth by cesarean section, preterm birth, bottle feeding, and first-time birth. [3] The diagnosis can be made by feeling an olive-colored mass in the baby's abdomen. [1] This is often confirmed by ultrasound. [4]

Treatment initially begins with addressing dehydration and electrolyte problems. [1] This is usually followed by surgery. [1] The results are generally good in both the short and long term. [1] Some treat this condition without surgery with atropine. [1]

Between one and two in 1,000 babies are affected. [1] Men are affected approximately four times more often than women. [1] The disease is very rare in adults. [6] The first description of pyloric stenosis was in 1888, with surgery first performed in 1912 by Conrad Ramstedt. [1] [2] Before surgery, most infants died. [1]

Signs and symptoms[edit]

Infants with this condition usually present anytime during the first weeks to 6 months of life with progressive vomiting. Vomiting affects first-born children more often in males than in females, by a ratio of 4 to 1. [7] Vomiting is often described as non-bilious vomiting ("non-bilious") and "projectile vomiting" because it is more forceful than normal regurgitation (gastroesophageal reflux) seen at this age. Some babies experience poor feeding and weight loss, but others experience normal weight gain. Dehydration may occur, causing the baby to cry without tears and produce fewer wet or dirty diapers due to lack of urination for several hours or several days. Symptoms usually appear between 3 and 12 weeks of age. Findings include epigastric fullness with visible peristalsis in the upper abdomen from left to right. [8] Constant hunger, belching, and colic are other possible signs that your baby may not be able to eat properly.

Pathophysiology[edit]

Gastric outlet obstruction due to a hypertrophied pylorus also prevents the emptying of gastric contents into the duodenum. As a result, all swallowed food and gastric secretions can only be released through vomiting, which can be projectile in nature. Although the exact cause of hypertrophy remains unknown, one study suggested that neonatal hyperacidity may be involved in the pathogenesis. [10] This physiological explanation for the development of clinical pyloric stenosis at about 4 weeks and its spontaneous long-term resolution without surgery with conservative treatment has recently been further analyzed. [eleven]

Constant vomiting leads to loss of gastric juice (hydrochloric acid). The vomit material does not contain bile because the pyloric obstruction prevents duodenal contents (containing bile) from entering the stomach. The Chloride loss results in low blood chloride levels, which impairs the kidneys' ability to excrete bicarbonate. This is a factor that prevents the correction of alkalosis leading to metabolic alkalosis. [12]

Secondary hyperaldosteronism develops due to a decrease in blood volume. High aldosterone levels cause the kidneys to greedily retain Na+ (to correct intravascular volume depletion) and excrete increased amounts of K+ in the urine (resulting in low blood potassium levels). [ Required citation

] The body's compensatory response to metabolic alkalosis is hypoventilation, leading to an increase in arterial pCO 2 .

Diagnosis[edit]

Pyloric stenosis on ultrasound in a 6-week-old infant [13]
Diagnosis is made based on a careful history and physical examination, often supplemented by radiographic studies. Pyloric stenosis should be suspected in any young child with severe vomiting. During physical examination, palpation of the abdomen may reveal a mass in the epigastrium. This mass, consisting of an enlarged pylorus, is called "olive" [14], and sometimes becomes obvious after the baby is given formula to drink. Rarely, peristaltic waves that can be felt or seen (video on NEJM) occur due to the stomach trying to push its contents through the narrowed pyloric outlet. [ citation needed

]

In most cases, pyloric stenosis is diagnosed/confirmed by ultrasound, if available, which shows a thickened pylorus and failure of gastric contents to pass into the proximal duodenum. A muscle wall thickness of 3 millimeters (mm) or greater and a pyloric length of 15 mm or greater are considered abnormal in infants less than 30 days old. Gastric contents should not be seen passing through the pylorus because in this case pyloric stenosis should be excluded and other differential diagnoses such as pylorospasm should be considered. The position of the superior mesenteric artery and superior mesenteric vein should be noted, as changes in the position of these two vessels may indicate intestinal malrotation rather than pyloric stenosis. [7]

Although the child is exposed to radiation, an upper gastrointestinal series (x-rays taken after the child has drunk a special contrast agent) can be diagnostic, showing a pylorus with an elongated, narrow lumen and an indentation in the duodenal bulb. [7] "String sign" or "railroad sign". For any type of study, there are certain measurement criteria used to identify abnormal results. A regular X-ray of the abdomen sometimes shows an enlarged stomach. Although UGI endoscopy reveals pyloric obstruction, clinicians have difficulty accurately distinguishing hypertrophic pyloric stenosis from pyloric spasm. [ citation needed

]

Blood tests will show low levels of potassium and chloride in the blood combined with elevated blood pH and high blood bicarbonate levels due to loss of stomach acid (which contains hydrochloric acid) due to persistent vomiting. There will be an exchange of extracellular potassium with intracellular hydrogen ions in an attempt to correct the pH imbalance. These results can be seen with severe vomiting for any reason. [ citation needed

]

Etiology

Pyloric stenosis can be acquired or congenital. The most common is acquired pyloric stenosis, which is a consequence of stomach diseases. Congenital pyloric stenosis, classified as a developmental defect, is observed relatively rarely, mainly in children.

The causes of acquired pyloric stenosis are cicatricial changes resulting from peptic ulcer disease (see) or burns with strong acids and alkalis, benign and malignant tumors localized in the pylorus area. Inflammatory and tumor processes located outside the pyloric canal can also lead to pyloric stenosis. It is possible to develop pyloric stenosis with tuberculosis and syphilis of the stomach (see). A rare cause of pyloric stenosis in adults is congenital hypertrophy of the pyloric muscularis.

Treatment[edit]

Vertical pyloromyotomy scar (large) 30 hours after surgery in a 1-month-old baby Horizontal pyloromyotomy scar 10 days after surgery in a 1-month-old baby
Infantile pyloric stenosis is usually treated with surgery; [15] Very few cases are mild enough to treat.

The danger of pyloric stenosis arises from dehydration and electrolyte imbalance, not from the underlying problem itself. Therefore, the child's condition must be initially stabilized by correcting dehydration and the abnormally high blood pH observed in combination with low chloride levels using intravenous fluids. This can usually be done in about 24 to 48 hours. [ citation needed

]

Intravenous and oral atropine can be used to treat pyloric stenosis. The success rate for this method is 85-89%, compared to almost 100% for pyloromyotomy, but it requires lengthy hospitalization, skilled nursing, and close monitoring during treatment. [16] It may be an alternative to surgery in children who have contraindications to anesthesia or surgery, or in children whose parents do not want surgery.

Surgery[edit]

Definitive treatment of pyloric stenosis with surgical pyloromyotomy is known as the Ramstedt procedure (by dividing the muscle from the pylorus to open the gastric outlet). This surgery can be done through a single incision (usually 3-4 cm long) or laparoscopically (through several tiny incisions), depending on the surgeon's experience and preference. [17]

Today, laparoscopic techniques have largely replaced traditional open reconstruction, which involved either a tiny circular incision around the belly button or the Ramstedt procedure. Compared to older open techniques, the complication rate is similar, except for a markedly lower risk of wound infection. [18] This is now considered the standard of care in most children's hospitals in the United States, although some surgeons still use the open technique. After repair, small 3mm incisions are difficult to see.

The vertical incision depicted and listed above is usually no longer required, although in recent years many incisions have been horizontal. As soon as the stomach can descend into the duodenum, feeding can begin again. You can expect some vomiting in the first few days after surgery as your gastrointestinal tract settles. In rare cases, the myotomy procedure is incomplete and projectile vomiting continues, requiring repeat surgery. Pyloric stenosis usually has no long-term side effects and does not affect the child's future. [ citation needed

]

Classification

By etiology:

• due to benign diseases;
• due to malignant diseases.

By stage of development:

• compensated;
• subcompensated;
• decompensated.

Complications:

• hypovolemic shock;
• acute gastrointestinal bleeding (ulcerative or mucosal ruptures due to repeated vomiting);
• perforation of an ulcer or tumor;
• spontaneous rupture of the stomach.

Epidemiology[edit]

Men are affected more often than women, first-born males are affected approximately four times more often, and there is a genetic predisposition to the disease. [19] It is usually associated with people of Scandinavian descent and has multifactorial patterns of inheritance. [9] Pyloric stenosis is more common in Caucasians than in Hispanics, blacks, or Asians. The incidence is 2.4 per 1000 live births in Caucasians, 1.8 in Hispanics, 0.7 in blacks, and 0.6 in Asians. It is also less common among children of mixed-race parents. [20] Caucasian infants with blood type B or O are more susceptible to the disease than children of other types. [19]

Infants exposed to erythromycin are at increased risk of developing hypertrophic pyloric stenosis, especially when the drug is administered after approximately two weeks of life [21] and possibly during late pregnancy and through breast milk in the first two weeks of life. [22]

How does pyloric insufficiency manifest?

The larger the lumen in the pylorus, the more pronounced the signs of the disease are:

• pain a little to the right and below the solar plexus;
• after eating, nausea and vomiting begin;
• the appearance of plaque on the tongue (usually yellow);
• belching with a bitter aftertaste;
• heaviness and discomfort in the epigastric region.

The symptoms are similar to other diseases of the digestive system. In addition to insufficiency, the pylorus of the stomach can be susceptible to spasms, ulcers and erosions, the appearance of polyps and other pathologies. For this reason, each patient is required to undergo examination and consult a gastroenterologist.

Links[edit]

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