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Pyloric Stenosis
Marshall Z. Schwartz Etiology
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1022 PART VII ABDOMEN
Nitric Oxide
Nitric oxide can induce smooth muscle relaxation in the
esophagus, stomach, and intestine. It was reasoned that a
deficiency of nitric oxide in the pyloric muscle might result
in failure of relaxation.39–41 Nicotinamide-adenine dinucleo-
tide phosphate (NADPH) diaphorase (essentially identical to
nitric oxide synthase) was measured in biopsy specimens of
hypertrophied pyloric muscle wall from IHPS patients. The
hypertrophied circular muscle did not demonstrate any
NADPH diaphorase activity, but the activity in the longitudinal
muscle was normal. The authors concluded that the lack of
FIGURE 78-1 Laparoscopic view of a hypertrophied pylorus. nitric oxide synthase in pyloric tissue might be responsible
for pylorospasm and lead to IHPS.42
Thus at present no definitive cause of IHPS has been elu-
GROWTH FACTORS AND cidated. Whatever the mechanism, it must take into account
GASTROINTESTINAL PEPTIDES that the process of IHPS generally occurs several weeks after
birth and the circular muscle hypertrophy is transient even
Over the past several decades numerous gastrointestinal pep- without myotomy. It is also apparent that whatever triggers
tides or growth factors have been implicated by direct or IHPS causes a dramatic change in cellular architecture and
indirect methodologies as having a causal relationship inclu- function through what may be a broad array of pathways.
ding gastrin, substance P, epidermal growth factor (EGF),
transforming growth factor-a (TGF-a), insulin-like growth
factor-I, somatostatin, secretin, enteroglucagon, and neuro-
tensin.24,25 Other peptides have been evaluated for their po- Clinical Features and Differential
tential relationship to IHPS. Substance P, a neurotransmitter
responsible for enteric muscle contraction, could produce Diagnosis
chronic pylorospasm leading to muscle hypertrophy.26 This ------------------------------------------------------------------------------------------------------------------------------------------------
peptide is present in higher concentration in the pyloric The typical clinical finding in an infant with IHPS is the onset
muscle of patients with IHPS. Shima and colleagues27–29 of nonbilious vomiting at 2 to 8 weeks of age with a peak oc-
found an increase in the gene expression of EGF, TGF-a, and currence at 3 to 5 weeks. Two reports are at variance with tra-
insulin-like growth factor-I and an increase in immunostaining ditional concepts: IHPS has been diagnosed at birth43 and even
for EGF and TGF-a. Somatostatin, secretin, enteroglucagon, in utero.44 Initially, the emesis may not be frequent or forceful,
and neurotensin have also been implicated.24,30,31 However, but over a period of several days it progresses to nearly every
their role has not been substantiated, and no clear etiologic feeding and becomes forceful (projectile). On occasion, there
relationship has been identified. may be blood in the emesis that gives it a brownish discoloration
or a coffee-ground appearance as a result of gastritis or esopha-
gitis. Infants with IHPS remain hungry after emesis and are oth-
Neurotrophins, Neural Development, erwise not ill appearing or febrile. A significant delay in diagnosis
and Nerve Function leading to severe dehydration, however, results in a lethargic
Neurotrophins, which are important for nerve differentiation infant. Some infants have diarrhea (starvation stools) and are
and survival, have been noted to be decreased in IHPS. Further thought to have gastroenteritis. Approximately 2% to 5% of
studies have shown that one of the specific receptors for these infants have jaundice from indirect hyperbilirubinemia, which
neurotrophins, the tyrosine kinase A receptor c-kit, is not can reach levels as high as 15 to 20 mg/dL. This is believed to
present in IHPS tissue.32–34 Investigators also identified that be secondary to glucuronyl transferase deficiency.45 In prema-
the pylorus in IHPS is deficient in glial-derived growth factors, ture infants, IHPS is generally diagnosed 2 weeks later than in
which may be indicative of immature development of the term infants.19 The emesis may not be projectile and evolves
enteric nervous system.35 more slowly, which frequently leads to a delay in diagnosis.
Other techniques have been applied to the evaluation of Pylorospasm and gastroesophageal reflux may be difficult
pyloric innervation. Okazaki and colleagues36 used monoclo- to differentiate from IHPS without further imaging evaluation.
nal antibodies targeted to nerve terminals and neurofilaments. Other medical causes of nonbilious vomiting include gastro-
They found a reduced density of both neural elements in enteritis, increased intracranial pressure, and metabolic disor-
patients with IHPS. Kobayashi and colleagues37 demonstrated ders. Other surgical causes of nonbilious emesis include antral
a decrease in nerve-supporting cells in both the circular and webs, pyloric atresia, duplication cyst of the antropyloric
longitudinal muscle layers in infants with IHPS. Piotrowska region, and ectopic pancreatic tissue within the pyloric
and colleagues38 found that muscle biopsy specimens from muscle, all far less common than IHPS.
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CHAPTER 78 HYPERTROPHIC PYLORIC STENOSIS 1023
Diagnosis
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1024 PART VII ABDOMEN
In 1998 our group conducted a survey of practice patterns be based on the level of hypokalemia and the rate of infusion
for the diagnosis of IHPS among pediatric surgeons in while keeping in mind that a potassium chloride concen-
community, university, and children’s hospitals.48 The ques- tration exceeding 30 mEq/L is rarely indicated.
tionnaire was distributed to 487 board-certified pediatric It is necessary to monitor urine output and serum elec-
surgeons, with a 69% response rate. Ninety-one percent of trolytes. Normalizing the serum bicarbonate level (with the
the respondents stated that they would not request a further goal of decreasing the level below 30 mEq/dL) usually lags
diagnostic test if a pyloric olive was palpable. US was identi- behind normalization of fluid volume and serum potassium
fied as the diagnostic study of choice for an inconclusive phys- and chloride. Administration of ammonium chloride or
ical examination (86% of the time). Finally, 82% of pediatric dilute hydrochloric acid is rarely necessary. As noted previ-
surgeons would proceed with an operation with a positive US ously, indirect hyperbilirubinemia with clinical signs of
study even if the results of physical examination under anes- jaundice occurs in a small percentage of infants with IHPS.
thesia were negative. The response to this question indicates It is not usually necessary to evaluate the hyperbiliru-
that US has essentially supplanted physical examination as binemia further. The hyperbilirubinemia invariably resolves
the diagnostic test of choice for IHPS.48 postoperatively.
OPERATIVE PROCEDURE
Treatment
------------------------------------------------------------------------------------------------------------------------------------------------ Again, it is important to emphasize that fluid and electrolyte
PREOPERATIVE PREPARATION abnormalities must be corrected preoperatively including
having a serum bicarbonate below 30 mEq/L to avoid respira-
It is most important to prepare the infant adequately for tory depression and prolonged postoperative intubation.
anesthesia and surgical correction of IHPS. The length of prep- In the operating room, before the induction of anesthesia, it
aration depends on the severity of the fluid and electrolyte is important to aspirate the stomach. If a barium UGI study has
abnormalities. Benson and Alpern49 defined three levels of been performed, it is advisable to remove the residual contrast
severity primarily on the basis of the serum carbon dioxide material by gastric irrigation and suctioning to avoid the risk
content (slight, <25 mEq/L; moderate, 26 to 35 mEq/L; and for perioperative aspiration.
severe, >35 mEq/L). In addition to the elevated bicarbonate, The operative procedure of choice remains the Ramstedt
hypokalemia, hypochloremia, dehydration, and possibly pyloromyotomy. This procedure has stood the test of time
malnutrition may be present. because it is straightforward, curative, and associated with
Most infants with IHPS do not have complete gastric outlet remarkably low morbidity. Regardless of abdominal access
obstruction and can therefore tolerate their gastric secretions. techniques, the myotomy created is identical.
Oral feedings should be discontinued. A nasogastric tube
should not be placed routinely because it removes additional Minimal Laparotomy (“Open”) Technique
fluid and hydrochloric acid from the stomach, which per- The standard open approach is a right upper quadrant trans-
petuates the electrolyte and acid-base imbalance. verse incision of approximately 2.5 to 3 cm made either over
Fluid resuscitation should be based on the degree of dehy- or lateral to the right rectus muscle and just superior to the
dration and the extent of electrolyte abnormalities. Most in- edge of the liver. The fascial layers are divided transversely
fants with IHPS should be able to be resuscitated within a over the rectus muscle, and it can be either retracted laterally
24-hour period. With severe metabolic and fluid abnormali- or split longitudinally in the middle. Several other incisions
ties, however, aggressive resuscitation should be avoided be- have been described.3 A commonly used alternative is a
cause it can produce rapid fluid and electrolyte shifts, possibly supraumbilical curved skin incision followed by division of
leading to seizures and other complications. Intravenous ad- the midline fascia superiorly for 1 to 2 cm. The edge of the
ministration of 5% dextrose in 0.45 normal saline containing liver is retracted superiorly to expose the greater curvature
20 mEq/L of potassium chloride is the optimal resuscitation of the stomach (near the pylorus), which is grasped with a
regimen for fluid and electrolyte replacement. Under circum- noncrushing forceps or clamp and brought out through the
stances of extreme hypokalemia, the concentration of potas- incision. A damp gauze sponge can then be used to grasp
sium chloride can be increased to 30 mEq/L, but because the stomach, and with traction inferiorly and laterally to the
the intravenous fluid rate likely will be above maintenance patient’s left, the pylorus can be delivered through the inci-
rates, the serum potassium level should be carefully moni- sion. Grasping the duodenum or pylorus directly with forceps
tored. Withholding potassium chloride in the intravenous can result in injury or perforation of these structures and
fluid while awaiting urine output only delays appropriate re- should therefore be avoided. The pylorus can be stabilized
placement. The exception (rare) is knowledge of preexisting by the index finger of the operating surgeon standing to the
renal impairment or evidence of acute renal compromise. right of the patient. The serosa on the anterior wall of the
Hyponatremia is rarely a problem. Nonetheless, it is common hypertrophied pylorus is incised with a scalpel from the area
to see normal saline given as an initial bolus. However, there is just proximal to the hypertrophied muscle extending just
little rationale for the use of normal saline because it enhances proximal to the pyloric vein (Fig. 78-4). I and many others
the hypokalemia by dilution and provides an excess amount of prefer to use the back of a scalpel handle to complete the
sodium. Intravenous therapy should be correlated with the myotomy by bluntly splitting the hypertrophied muscle down
level of dehydration. An initial rate for fluid resuscitation is to the submucosa (see Fig. 78-4). Other surgeons prefer the
1.25 to 2 times the normal maintenance rate until adequate use of a pyloric spreading clamp such as that described by
fluid resuscitation and urine output are achieved. The concen- Benson.50 It is appropriate to leave a few pyloric muscle fibers
tration of potassium chloride in the intravenous fluid should intact at the duodenal end to reduce the risk for duodenal
ERRNVPHGLFRVRUJ
CHAPTER 78 HYPERTROPHIC PYLORIC STENOSIS 1025
Serosa
Stomach
A Pyloric vein
Duodenum
Submucosa
Serosa
C
FIGURE 78-5 Position of the infant on the operating room table for
laparoscopic pyloromyotomy.
B
ERRNVPHGLFRVRUJ
1026 PART VII ABDOMEN
FIGURE 78-7 View through the laparoscope with the arthrotomy knife
being used to incise the serosa over the hypertrophied pylorus.
FIGURE 78-8 Hypertrophied pyloric muscle after being split with the
spreader.
the published studies to date have been retrospective and
demonstrate a marked degree of variability.
The range for the average time to discharge in the pub- Over the past decade, considerable additional experience
lished literature for laparoscopy is similar to that for open pro- has occurred and laparoscopic pyloromyotomy has become
cedures. Of the four publications that compare time to the standard at the training sites and children’s hospitals in
discharge between the open procedure and laparoscopy, only the United States with low morbidity and short hospital stays.
one group found statistical significance and the difference in The wound infection rate for both groups was similar
discharge time was only 4 hours (Table 78-1).53,55,57–61 and ranged from 0% to 6% in the laparoscopic groups and
There is wide variation in the incidence of postoperative from 0% to 7% in the open groups. None of these studies
emesis. The range for laparoscopy was 1% to 68%, whereas identified a significant difference between the groups (Ta-
for the open procedure it was 25% to 65%, with no statistical ble 78-3).52,54,55,57–59,63
significance observed between the open and laparoscopic The operative time was similar for both groups, with
groups.52,53,57,58,62 laparoscopy taking from 24.3 to 41 minutes and the open
The average time to establish full feeding in the laparoscopic procedure taking from 18.9 to 32.5 minutes. Yagmurlu and col-
group ranged from 16 to 32 hours versus 20 to 61 hours for the leagues56 (the largest study) showed that laparoscopy was faster
open procedure (Table 78-2).52–54,57,62 The results are no dif- by 4.7 minutes on average. Sitsen and colleagues55 and Scorpio
ferent if the Fujimoto outlier study is eliminated.52 and colleagues60 found that laparoscopy was slower by 14.3
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CHAPTER 78 HYPERTROPHIC PYLORIC STENOSIS 1027
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1028 PART VII ABDOMEN
TABLE 78-6
Postpyloromyotomy Feeding Schedule*
Complications
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the initial postoperative period, but this approach is usually After pyloromyotomy, mortality should be a rare occurrence
successful and generally allows for earlier hospital dis- and morbidity should be low. In a large series of infants
charge.64 The feeding schedule shown in Table 78-6 is mod- undergoing open pyloromyotomy, the incidence of perfora-
erately aggressive and allows for hospital discharge tion was 2.3%, wound-related complications occurred in
approximately 24 hours after the feedings are initiated. 1%, and there was one death.50 More recent series have
In the future, infants may be discharged within 3 or 4 hours reported even lower morbidity and mortality.66
after pyloromyotomy, in which case resumption of feeding will As experience with laparoscopic pyloromyotomy has
be undertaken by the family at home. increased, outcomes have become similar to those after the
open approach. With a somewhat aggressive postoperative
feeding schedule, infants should be able to be discharged
within 24 hours of pyloromyotomy.
NONOPERATIVE TREATMENT The long-term sequelae from pyloromyotomy are minimal.
Ludtke and colleagues67 evaluated the presence of gastrointes-
This approach never gained acceptance in North America but tinal symptoms, measured scintigraphic gastric emptying, and
was practiced in the past in some European countries. determined pyloric measurements by US in 36 adults 17 to 27
Although infants with IHPS can be managed with frequent years after pyloromyotomy. Their results were compared with
small feedings, this practice necessitates either a prolonged those of age-matched and gender-matched controls. They
hospital stay or an attentive caregiver at home and may lead identified no differences between the postpyloromyotomy
to aspiration and malnutrition. It may take months for the group and the control group for the parameters measured.
hypertrophied muscle to resolve. The occasional mortality
and the prolonged interval from diagnosis to resolution led The complete reference list is available online at www.
to abandonment of this type of management. expertconsult.com.
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