Pyloric stenosis

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Pyloric stenosis
Classification and external resources

Outline of stomach, showing its anatomical
landmarks, including the pylorus.
ICD-10 K31.1, Q40.0
ICD-9 537.0, 750.5
DiseasesDB 11060 29488
MedlinePlus 000970
eMedicine emerg/397 radio/358
MeSH D046248
Pyloric stenosis (or infantile hypertrophic pyloric stenosis)
[1]
is a condition that causes severe
projectile non-bilious vomiting in the first few months of life. There is narrowing (stenosis) of
the opening from the stomach to the first part of the small intestine known as the duodenum, due
to enlargement (hypertrophy) of the muscle surrounding this opening (the pylorus, meaning
"gate"), which spasms when the stomach empties. This hypertrophy is felt classically as an olive-
shaped mass in the middle upper part or right upper quadrant of the infant's abdomen. In pyloric
stenosis, it is uncertain whether there is a real congenital narrowing or whether there is a
functional hypertrophy of the pyloric sphincter muscle. This condition typically develops in male
babies in the first 26 weeks of life.
Pyloric stenosis also occurs in adults where the cause is usually a narrowed pylorus due to
scarring from chronic peptic ulceration. This is a different condition from the infantile form.
Contents
1 Signs and symptoms
2 Diagnosis
3 Pathophysiology
4 Treatment
o 4.1 Surgery
5 Epidemiology
6 References
7 External links
Signs and symptoms
Babies with this condition usually present any time in the first weeks to months of life with
progressively worsening vomiting. The vomiting is often described as non-bile stained ("non
bilious") and "projectile vomiting", because it is more forceful than the usual spittiness
(gastroesophageal reflux) seen at this age. Some infants present with poor feeding and weight
loss, but others demonstrate normal weight gain. Dehydration also can occur causing the baby to
cry without having tears, and having less wet or dirty nappies such as going hours or a couple
days without having anything.
[2]
Constant hunger, belching, and colic are other possible signs as
the baby is not able to eat properly.
Diagnosis
Diagnosis is via a careful history and physical examination, often supplemented by radiographic
studies. There should be suspicion for pyloric stenosis in any young infant with severe vomiting.
On exam, palpation of the abdomen may reveal a mass in the epigastrium. 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. It is an elusive diagnostic skill requiring much patience and
experience. There are often palpable (or even visible) peristaltic waves due to the stomach trying
to force its contents past the narrowed pyloric outlet.
Most cases of pyloric stenosis are diagnosed/confirmed with ultrasound, if available, showing the
thickened pylorus. Although somewhat less useful, an upper GI series (x-rays taken after the
baby drinks a special contrast agent) can be diagnostic by showing the narrowed pyloric outlet
filled with a thin stream of contrast material; a "string sign" or the "railroad track sign". For
either type of study, there are specific measurement criteria used to identify the abnormal results.
Plain x-rays of the abdomen are not useful, except when needed to rule out other problems.
Blood tests will reveal hypokalemic, hypochloremic metabolic alkalosis due to loss of gastric
acid (which contain hydrochloric acid and potassium) via persistent vomiting; these findings can
be seen with severe vomiting from any cause. The potassium is decreased further by the body's
release of aldosterone, in an attempt to compensate for the hypovolaemia due to the severe
vomiting.
Pathophysiology
The gastric outlet obstruction due to the hypertrophic pylorus impairs emptying of gastric
contents into the duodenum. As a consequence, all ingested food and gastric secretions can only
exit via vomiting, which can be of a projectile nature. The exact cause of the hypertrophy
remains unknown.
[3]

The vomited material does not contain bile because the pyloric obstruction prevents entry of
duodenal contents (containing bile) into the stomach.
This results in loss of gastric acid (hydrochloric acid). The chloride loss results in hypochloremia
which impairs the kidney's ability to excrete bicarbonate. This is the significant factor that
prevents correction of the alkalosis.
[4]

A secondary hyperaldosteronism develops due to the hypovolemia. The high aldosterone levels
causes the kidneys to avidly retain Na
+
(to correct the intravascular volume depletion), and
excrete increased amounts of K
+
into the urine (resulting in hypokalaemia).
The body's compensatory response to the metabolic alkalosis is hypoventilation resulting in an
elevated arterial pCO
2
.
Treatment


Vertical Pyloromyotomy scar (large) 30 hrs post-op in a 1 month-old baby


Horizontal Pyloromyotomy scar 10 days post-op in a 1 month-old baby


Laparoscopic Plyoromyotomy scar, 6hrs post-op
Infantile pyloric stenosis is typically managed with surgery;
[3]
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 hypochloremic alkalosis with IV fluids. This can usually be accomplished in
about 2448 hours.
Intravenous and oral atropine 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.
[5]
It might be an
alternative to surgery in children who have contraindications for anesthesia or surgery.
Surgery
The definitive treatment of pyloric stenosis is with surgical pyloromyotomy known as Ramstedt's
procedure (dividing the muscle of the pylorus to open up the gastric outlet). This is a relatively
straightforward surgery that can possibly be done through a single incision (usually 34 cm long)
or laparoscopically (through several tiny incisions), depending on the surgeon's experience and
preference.
[6]

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.
[7]
This is now considered the standard of care at the majority of Children
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, feeding can commence. Some vomiting may be
expected during the first days after surgery as the gastro-intestinal tract settles. Very occasionally
the myotomy was incomplete and projectile vomiting continues, requiring repeat surgery. But the
condition 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 for the disease.
[8]
It is commonly associated with
people of Jewish ancestry, and has multifactorial inheritance patterns.
[9]
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.
[10]
Caucasian babies with blood type B or O are more
likely than other types to be affected.
[8]

Infants exposed to erythromycin are at increased risk for developing hypertrophic pyloric
stenosis, especially when the drug is taken around two weeks of life.
[11]

References
1. ^ Hulka F, Campbell TJ, Campbell JR, Harrison MW (1997). "Evolution in the
recognition of infantile hypertrophic pyloric stenosis". Pediatrics 100 (2): E9.
doi:10.1542/peds.100.2.e9. PMID 9233980.
2. ^ "Pyloric stenosis: Symptoms". MayoClinic.com. 2010-08-21. Retrieved 2012-02-21.
3. ^
a

b
Askew, Nathan (October 2010). "An overview of infantile hypertrophic pyloric
stenosis.". Paediatric nursing 22 (8): 27-30. PMID 21066945. Retrieved 30 August 2012.
4. ^ Kerry Brandis, Acid-Base Physiology. Retrieved December 31, 2006.
5. ^ Aspelund G, Langer JC (February 2007). "Current management of hypertrophic pyloric
stenosis". Semin. Pediatr. Surg. 16 (1): 2733. doi:10.1053/j.sempedsurg.2006.10.004.
PMID 17210480.
6. ^ "Medical News:Laparoscopic Repair of Pediatric Pyloric Stenosis May Speed
Recovery - in Surgery, Thoracic Surgery from". MedPage Today. 2009-01-16. Retrieved
2012-02-21.
7. ^ Sola JE, Neville HL (August 2009). "Laparoscopic vs open pyloromyotomy: a
systematic review and meta-analysis". J. Pediatr. Surg. 44 (8): 16317.
doi:10.1016/j.jpedsurg.2009.04.001. PMID 19635317.