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Pyloric stenosis is a narrowing of the opening from the
stomach The stomach is a muscular, hollow organ in the gastrointestinal tract of humans and many other animals, including several invertebrates. The stomach has a dilated structure and functions as a vital organ in the digestive system. The stomach i ...
to the first part of the
small intestine The small intestine or small bowel is an organ in the gastrointestinal tract where most of the absorption of nutrients from food takes place. It lies between the stomach and large intestine, and receives bile and pancreatic juice through the ...
(the
pylorus The pylorus ( or ), or pyloric part, connects the stomach to the duodenum. The pylorus is considered as having two parts, the ''pyloric antrum'' (opening to the body of the stomach) and the ''pyloric canal'' (opening to the duodenum). The ''pylori ...
). Symptoms include projectile
vomiting Vomiting (also known as emesis and throwing up) is the involuntary, forceful expulsion of the contents of one's stomach through the mouth and sometimes the nose. Vomiting can be the result of ailments like food poisoning, gastroenteritis ...
without the presence of
bile Bile (from Latin ''bilis''), or gall, is a dark-green-to-yellowish-brown fluid produced by the liver of most vertebrates that aids the digestion of lipids in the small intestine. In humans, bile is produced continuously by the liver (liver bile ...
. This most often occurs after the baby is fed. The typical age that symptoms become obvious is two to twelve weeks old. The cause of pyloric stenosis is unclear. Risk factors in babies include birth by
cesarean section Caesarean section, also known as C-section or caesarean delivery, is the surgical procedure by which one or more babies are delivered through an incision in the mother's abdomen, often performed because vaginal delivery would put the baby or m ...
,
preterm birth Preterm birth, also known as premature birth, is the birth of a baby at fewer than 37 weeks gestational age, as opposed to full-term delivery at approximately 40 weeks. Extreme preterm is less than 28 weeks, very early preterm birth is between ...
,
bottle feeding A baby bottle, nursing bottle, or feeding bottle is a bottle with an attached ''teat'' (also called a ''nipple'' in the US) on the top opening, on which can be suckled, and from thereby drunk directly. It is typically used by infants and young ...
, and being first born. The diagnosis may be made by feeling an olive-shaped mass in the baby's abdomen. This is often confirmed with
ultrasound Ultrasound is sound waves with frequencies higher than the upper audible limit of human hearing. Ultrasound is not different from "normal" (audible) sound in its physical properties, except that humans cannot hear it. This limit varies ...
. Treatment initially begins by correcting
dehydration In physiology, dehydration is a lack of total body water, with an accompanying disruption of metabolic processes. It occurs when free water loss exceeds free water intake, usually due to exercise, disease, or high environmental temperature. Mil ...
and
electrolyte problems Electrolyte imbalance, or water-electrolyte imbalance, is an abnormality in the concentration of electrolytes in the body. Electrolytes play a vital role in maintaining homeostasis in the body. They help to regulate heart and neurological function, ...
. This is then typically followed by surgery, although some treat the condition without surgery by using
atropine Atropine is a tropane alkaloid and anticholinergic medication used to treat certain types of nerve agent and pesticide poisonings as well as some types of slow heart rate, and to decrease saliva production during surgery. It is typically given ...
. Results are generally good both in the short term and in the long term. About one to two per 1,000 babies are affected, and males are affected about four times more often than females. The condition is very rare in adults. The first description of pyloric stenosis was in 1888 with surgery management first carried out in 1912 by Conrad Ramstedt. Before surgical treatment most babies died.


Signs and symptoms

Babies with this condition usually present any time in the first weeks to 6 months of life with progressively worsening
vomiting Vomiting (also known as emesis and throwing up) is the involuntary, forceful expulsion of the contents of one's stomach through the mouth and sometimes the nose. Vomiting can be the result of ailments like food poisoning, gastroenteritis ...
. It is more likely to affect the first-born with males more commonly than females at a ratio of 4 to 1. The vomiting is often described as non-bile stained ("non bilious") and "projectile vomiting", because it is more forceful than the usual spitting up (
gastroesophageal reflux Gastroesophageal reflux disease (GERD) or gastro-oesophageal reflux disease (GORD) is one of the upper gastrointestinal chronic diseases where stomach content persistently and regularly flows up into the esophagus, resulting in symptoms and/ ...
) seen at this age. Some infants present with poor feeding and weight loss but others demonstrate normal weight gain. Dehydration may occur which causes a baby to cry without having tears and to produce less wet or dirty diapers due to not urinating for hours or for a few days. Symptoms usually begin between 3 and 12 weeks of age. Findings include epigastric fullness with visible peristalsis in the upper abdomen from the infant's left to right. Constant hunger, belching, and
colic Colic or cholic () is a form of pain that starts and stops abruptly. It occurs due to muscular contractions of a hollow tube (small and large intestine, gall bladder, ureter, etc.) in an attempt to relieve an obstruction by forcing content out. ...
are other possible signs that the baby is unable to eat properly.


Cause

Rarely, infantile pyloric stenosis can occur as an
autosomal dominant In genetics, dominance is the phenomenon of one variant (allele) of a gene on a chromosome masking or overriding the effect of a different variant of the same gene on the other copy of the chromosome. The first variant is termed dominant and ...
condition. It is uncertain whether it is a
congenital A birth defect, also known as a congenital disorder, is an abnormal condition that is present at birth regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can ...
anatomic narrowing or a functional hypertrophy of the pyloric sphincter muscle.


Pathophysiology

The
gastric outlet obstruction Gastric outlet obstruction (GOO) is a medical condition where there is an obstruction at the level of the pylorus, which is the outlet of the stomach. Individuals with gastric outlet obstruction will often have recurrent vomiting of food that has ...
due to the
hypertrophic Hypertrophy is the increase in the volume of an organ or tissue due to the enlargement of its component cells. It is distinguished from hyperplasia, in which the cells remain approximately the same size but increase in number.Updated by Linda J. ...
pylorus impairs emptying of gastric contents into the
duodenum The duodenum is the first section of the small intestine in most higher vertebrates, including mammals, reptiles, and birds. In fish, the divisions of the small intestine are not as clear, and the terms anterior intestine or proximal intestine ...
. As a consequence, all ingested food and gastric secretions can only exit via vomiting, which can be of a projectile nature. While the exact cause of the hypertrophy remains unknown, one study suggested that neonatal hyperacidity may be involved in the pathogenesis. This physiological explanation for the development of clinical pyloric stenosis at around 4 weeks and its spontaneous long term cure without surgery if treated conservatively, has recently been further reviewed. Persistent vomiting results in loss of stomach acid (
hydrochloric acid Hydrochloric acid, also known as muriatic acid, is an aqueous solution of hydrogen chloride. It is a colorless solution with a distinctive pungent smell. It is classified as a strong acid. It is a component of the gastric acid in the digestiv ...
). The vomited material does not contain
bile Bile (from Latin ''bilis''), or gall, is a dark-green-to-yellowish-brown fluid produced by the liver of most vertebrates that aids the digestion of lipids in the small intestine. In humans, bile is produced continuously by the liver (liver bile ...
because the pyloric obstruction prevents entry of duodenal contents (containing bile) into the stomach. The
chloride The chloride ion is the anion (negatively charged ion) Cl−. It is formed when the element chlorine (a halogen) gains an electron or when a compound such as hydrogen chloride is dissolved in water or other polar solvents. Chloride salts ...
loss results in a low blood chloride level which impairs the kidney's ability to excrete bicarbonate. This is the factor that prevents correction of the
alkalosis Alkalosis is the result of a process reducing hydrogen ion concentration of arterial blood plasma (alkalemia). In contrast to acidemia (serum pH 7.35 or lower), alkalemia occurs when the serum pH is higher than normal (7.45 or higher). Alkalosis ...
leading to metabolic alkalosis.Kerry Brandis
Acid-Base Physiology
. Retrieved December 31, 2006.
A secondary
hyperaldosteronism Hyperaldosteronism is a medical condition wherein too much aldosterone is produced by the adrenal glands, which can lead to lowered levels of potassium in the blood (hypokalemia) and increased hydrogen ion excretion ( alkalosis). This cause of m ...
develops due to the decreased blood volume. The high
aldosterone Aldosterone is the main mineralocorticoid steroid hormone produced by the zona glomerulosa of the adrenal cortex in the adrenal gland. It is essential for sodium conservation in the kidney, salivary glands, sweat glands, and colon. It plays a c ...
levels causes the kidneys to avidly retain Na+ (to correct the intravascular
volume depletion Hypovolemia, also known as volume depletion or volume contraction, is a state of abnormally low extracellular fluid in the body. This may be due to either a loss of both salt and water or a decrease in blood volume. Hypovolemia refers to the los ...
), and excrete increased amounts of K+ into the urine (resulting in a low blood level of potassium).The body's compensatory response to the metabolic alkalosis is hypoventilation resulting in an elevated arterial pCO2.


Diagnosis

Diagnosis is via a careful history and physical examination, often supplemented by radiographic imaging studies. Pyloric stenosis should be suspected in any young infant with severe vomiting. On physical exam,
palpation Palpation is the process of using one's hands to check the body, especially while perceiving/diagnosing a disease or illness. Usually performed by a health care practitioner, it is the process of feeling an object in or on the body to determine ...
of the abdomen may reveal a mass in the
epigastrium In anatomy, the epigastrium (or epigastric region) is the upper central region of the abdomen. It is located between the costal margins and the subcostal plane. Pain may be referred to the epigastrium from damage to structures derived from the fo ...
. This mass, which consists of the enlarged pylorus, is referred to as the 'olive', and is sometimes evident after the infant is given formula to drink. Rarely, there are
peristaltic Peristalsis ( , ) is a radially symmetrical contraction and relaxation of muscles that propagate in a wave down a tube, in an anterograde direction. Peristalsis is progression of coordinated contraction of involuntary circular muscles, which ...
waves that may be felt or seen
video on NEJM
due to the stomach trying to force its contents past the narrowed pyloric outlet. Most cases of pyloric stenosis are diagnosed/confirmed with
ultrasound Ultrasound is sound waves with frequencies higher than the upper audible limit of human hearing. Ultrasound is not different from "normal" (audible) sound in its physical properties, except that humans cannot hear it. This limit varies ...
, if available, showing the thickened pylorus and non-passage of gastric contents into the proximal duodenum. Muscle wall thickness 3 millimeters (mm) or greater and pyloric channel length of 15 mm or greater are considered abnormal in infants younger than 30 days. Gastric contents should not be seen passing through the pylorus because if it does, pyloric stenosis should be excluded and other differential diagnoses such as pylorospasm should be considered. The positions of
superior mesenteric artery In human anatomy, the superior mesenteric artery (SMA) is an artery which arises from the anterior surface of the abdominal aorta, just inferior to the origin of the celiac trunk, and supplies blood to the intestine from the lower part of the d ...
and
superior mesenteric vein In human anatomy, the superior mesenteric vein (SMV) is a blood vessel that drains blood from the small intestine (jejunum and ileum). Behind the neck of the pancreas, the superior mesenteric vein combines with the splenic vein to form the hepa ...
should be noted because altered positions of these two vessels would be suggestive of intestinal malrotation instead of pyloric stenosis. Although the baby is exposed to radiation, an
upper GI series An upper gastrointestinal series, also called a barium swallow, barium study, or barium meal, is a series of radiographs used to examine the gastrointestinal tract for abnormalities. A contrast medium, usually a radiocontrast agent such as bari ...
(X-rays taken after the baby drinks a special
contrast agent A contrast agent (or contrast medium) is a substance used to increase the contrast of structures or fluids within the body in medical imaging. Contrast agents absorb or alter external electromagnetism or ultrasound, which is different from radioph ...
) can be diagnostic by showing the pylorus with elongated, narrow lumen and a dent in the
duodenal bulb The duodenal bulb is the portion of the duodenum closest to the stomach. It normally has a length of about 5 centimeters. The duodenal bulb begins at the pylorus and ends at the neck of the gallbladder. It is located posterior to the liver and the ...
. This phenomenon caused " string sign" or the "railroad track/double track sign" on X-rays after contrast is given. Plain x-rays of the abdomen sometimes shows a dilated stomach. Although upper gastrointestinal endoscopy would demonstrate pyloric obstruction, physicians would find it difficult to differentiate accurately between hypertrophic pyloric stenosis and pylorospasm. Blood tests will reveal low blood levels of
potassium Potassium is the chemical element with the symbol K (from Neo-Latin ''kalium'') and atomic number19. Potassium is a silvery-white metal that is soft enough to be cut with a knife with little force. Potassium metal reacts rapidly with atmosp ...
and
chloride The chloride ion is the anion (negatively charged ion) Cl−. It is formed when the element chlorine (a halogen) gains an electron or when a compound such as hydrogen chloride is dissolved in water or other polar solvents. Chloride salts ...
in association with an increased blood pH and high blood bicarbonate level due to loss of
stomach acid Gastric acid, gastric juice, or stomach acid is a digestive fluid formed within the stomach lining. With a pH between 1 and 3, gastric acid plays a key role in digestion of proteins by activating digestive enzymes, which together break down the ...
(which contains
hydrochloric acid Hydrochloric acid, also known as muriatic acid, is an aqueous solution of hydrogen chloride. It is a colorless solution with a distinctive pungent smell. It is classified as a strong acid. It is a component of the gastric acid in the digestiv ...
) from persistent vomiting. There will be exchange of extracellular potassium with intracellular hydrogen ions in an attempt to correct the pH imbalance. These findings can be seen with severe vomiting from any cause.


Treatment

Infantile pyloric stenosis is typically managed with surgery; very few cases are mild enough to be treated medically. The danger of pyloric stenosis comes from the dehydration and electrolyte disturbance rather than the underlying problem itself. Therefore, the baby must be initially stabilized by correcting the dehydration and the abnormally high blood pH seen in combination with low chloride levels with IV fluids. This can usually be accomplished in about 24–48 hours. Intravenous and oral
atropine Atropine is a tropane alkaloid and anticholinergic medication used to treat certain types of nerve agent and pesticide poisonings as well as some types of slow heart rate, and to decrease saliva production during surgery. It is typically given ...
may be used to treat pyloric stenosis. It has a success rate of 85-89% compared to nearly 100% for pyloromyotomy, however it requires prolonged hospitalization, skilled nursing and careful follow up during treatment. It might be an alternative to surgery in children who have contraindications for anesthesia or surgery, or in children whose parents do not want surgery.


Surgery

The definitive treatment of pyloric stenosis is with surgical
pyloromyotomy Pyloromyotomy is a surgical procedure in which a portion of the muscle fibers of the pyloric muscle are cut. This is typically done in cases where the contents from the stomach are inappropriately stopped by the pyloric muscle, causing the stomac ...
known as Ramstedt's procedure (dividing the
muscle Skeletal muscles (commonly referred to as muscles) are organs of the vertebrate muscular system and typically are attached by tendons to bones of a skeleton. The muscle cells of skeletal muscles are much longer than in the other types of muscle ...
of the
pylorus The pylorus ( or ), or pyloric part, connects the stomach to the duodenum. The pylorus is considered as having two parts, the ''pyloric antrum'' (opening to the body of the stomach) and the ''pyloric canal'' (opening to the duodenum). The ''pylori ...
to open up the gastric outlet). This surgery can be done through a single incision (usually 3–4 cm long) or
laparoscopically Laparoscopy () is an operation performed in the abdomen or pelvis using small incisions (usually 0.5–1.5 cm) with the aid of a camera. The laparoscope aids diagnosis or therapeutic interventions with a few small cuts in the abdomen.Medlin ...
(through several tiny incisions), depending on the surgeon's experience and preference. Today, the laparoscopic technique has largely supplanted the traditional open repairs which involved either a tiny circular incision around the navel or the Ramstedt procedure. Compared to the older open techniques, the complication rate is equivalent, except for a markedly lower risk of wound infection. This is now considered the standard of care at the majority of children's hospitals across the US, although some surgeons still perform the open technique. Following repair, the small 3mm incisions are hard to see. The vertical incision, pictured and listed above, is no longer usually required, though many incisions have been horizontal in the past years. Once the stomach can empty into the
duodenum The duodenum is the first section of the small intestine in most higher vertebrates, including mammals, reptiles, and birds. In fish, the divisions of the small intestine are not as clear, and the terms anterior intestine or proximal intestine ...
, feeding can begin again. Some vomiting may be expected during the first days after surgery as the gastrointestinal tract settles. Rarely, the
myotomy Myotomy is a surgical procedure in which muscle is cut. A common example of a myotomy is the Heller myotomy. See also * List of surgeries by type Many surgical procedure names can be broken into parts to indicate the meaning. For example, i ...
procedure performed is incomplete and projectile vomiting continues, requiring repeat surgery. Pyloric stenosis generally has no long term side-effects or impact on the child's future.


Epidemiology

Males are more commonly affected than females, with firstborn males affected about four times as often, and there is a
genetic predisposition A genetic predisposition is a genetic characteristic which influences the possible phenotypic development of an individual organism within a species or population under the influence of environmental conditions. In medicine, genetic susceptibilit ...
for the disease. It is commonly associated with people of Scandinavian ancestry, and has multifactorial inheritance patterns. Pyloric stenosis is more common in Caucasians than 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 amongst children of mixed race parents. Caucasian male babies with
blood type A blood type (also known as a blood group) is a classification of blood, based on the presence and absence of antibodies and inherited antigenic substances on the surface of red blood cells (RBCs). These antigens may be proteins, carbohydra ...
B or O are more likely than other types to be affected. Infants exposed to
erythromycin Erythromycin is an antibiotic used for the treatment of a number of bacterial infections. This includes respiratory tract infections, skin infections, chlamydia infections, pelvic inflammatory disease, and syphilis. It may also be used during ...
are at increased risk for developing hypertrophic pyloric stenosis, especially when the drug is taken around two weeks of life and possibly in late pregnancy and through breastmilk in the first two weeks of life.


References


External links

*
Peristaltic waves video on NEJM
{{Congenital malformations and deformations of digestive system Congenital disorders of digestive system Wikipedia medicine articles ready to translate Stomach disorders