Seminars in Pediatric Surgery 31 (2022) 151145

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Seminars in Pediatric Surgery

journal homepage: www.elsevier.com/locate/sempedsurg

Current management of pyloric stenosis

Melissa E Danko∗, Parker T Evans, Jeffrey S Upperman
Monroe Carell Jr. Children’s Hospital, Vanderbilt

Introduction and epidemiology potential factor, with a deficiency in nitric oxide synthase (NOS)
leading to impaired physiologic relaxation of the pyloric sphinc-
Hypertrophic pyloric stenosis (HPS) is a condition of infants ter, pylorospasm, and hypertrophy9 , 10 . Only a fraction of HPS pa-
characterized by hypertrophy of the pyloric sphincter. This leads tients have been shown to have abnormal NOS function11 , 12 , but
to stenosis of the pyloric canal causing gastric outlet obstruction. murine models have demonstrated a strong relationship between
HPS manifests as the classic presentation of progressive postpran- NOS dysfunction and pyloric hypertrophy13 . Additional proposed
dial nonbilious projectile emesis. mechanisms include poor pyloric muscle innervation, decreased
Hypertrophic pyloric stenosis was first described as early as the intestinal-pacemaker cells of Cajal, and infectious etiologies9 , 14 .
17th century. Both Hildanus and Blair have been attributed with HPS also carries a strong familial pattern of aggregation2 , 15 ,
the earlier descriptions of HPS, but our contemporary understand- which has led to the search for potential genetic factors. A study
ing stems from Hirschsprung’s work in the late 1800s. HPS remains utilizing a large population database in Denmark demonstrated
a common condition of newborns, with a historical incidence of 2 significantly higher rates of disease among family members (high-
to 5 cases per 10 0 0 live births in the United States1 , 2 . A recent est in monozygotic twins at a 200-fold higher rate), with an
cross-sectional study of the National Inpatient Sample found an overall heritability estimate of 87%15 . Notable loci on chromo-
overall incidence of 1.56 per 10 0 0 live births from 2012 to 2016, somes 2, 3, 5, 7, 11, and 12 have all be implicated, including the
with a statistically significant decrease in incidence across that APOA1 gene cluster (apolipoprotein A1) and the loci near MBNL1
time period (1.76 in 2012 to 1.57 per 10 0 0 in 2016)3 . A lower inci- and NKX2-59 , 16-20 . However, genetic factors cannot explain the
dence has been reported in Africa and Asia at <1 cases per 10 0 02 . large variations in incidence and environmental factors associated
The disease is significantly more common in male (5:1) and first- with the development of HPS. These environmental factors sug-
born children (30-40%)3-6 . Incidence has also been reported to be gest that the condition must be at least partially acquired. Ma-
higher in white and Hispanic children compared to African Amer- ternal smoking during pregnancy carries an increased risk of 1.5
ican patients (1.75 and 1.97 vs 0.82 per 10 0 0, respectively) and to 2 times the general popultation5 , 21 , 22 . Furthermore, multiple
in patients with lower socioeconomic status3 . Presentation is most studies have identified bottle feeding rather than breast milk as
common between the ages of 2 and 12 weeks, with a mean and an independent risk factor for the HPS, with one study demon-
median age at presentation of 5 weeks4 . Historically, this condi- strating a hazard ratio of 4.6 for patients who were never breast-
tion was almost always fatal with a mortality of almost 100%, but fed22-24 . Neonatal exposure to macrolide antibiotics including ery-
it has evolved into a disease with a surgical correction leading to thromycin and azithromycin has also been demonstrated to be
an excellent prognosis. associated with increased risk for HPS, especially when adminis-
tered prior to two weeks of age25 . Two systematic meta-analyses
Etiology and pathogenesis have demonstrated statistically significant association between di-
rect post-natal exposure to macrolides and pyloric stenosis, but as-
The etiology of pyloric stenosis has been studied extensively, sociations with pre-natal exposure and post-natal maternal expo-
and multiple genetic and environmental factors have been impli- sure (i.e. through breastfeeding) were not significant26 , 27 . Neona-
cated. However, the exact cause remains uncertain. One proposed tal exposure to prostaglandin infusions has also been associated
contributory mechanism is that babies with pyloric stenosis secrete with the development of pyloric stenosis, but this seems to be a
more acid. This hyperacidity then leads to increased and repeated transient phenomenon28 . Other reported risk factors include young
pyloric sphincter contractions. The more the sphincter contracts, maternal age, maternal hyperthyroidism, prematurity, small for
the greater hypertrophy develops. Furthermore, reactionary over- gestational age, and cesarian section5 , 22 .
feeding of vomiting babies by parents further exacerbates this sit-
uation7 , 8 . The nitric oxide pathway has also been identified as a Presentation and pathophysiology

∗
Classically, a patient with HPS will present with a recent his-
Corresponding author at: Department of Pediatric Surgery, Monroe Carell Jr.
Children’s Hospital at Vanderbilt, 2200 Children’s Way, Suite 7100, Nashville, TN tory of forceful projectile nonbilious emesis after feeding. The most
37232, United States common patient is a firstborn male at 4-6 weeks of age and many
E-mail address: [email protected] (M.E. Danko). have a family history of pyloric stenosis. The character and timing

https://doi.org/10.1016/j.sempedsurg.2022.151145
1055-8586/© 2022 Published by Elsevier Inc.

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M.E. Danko, P.T. Evans and J.S. Upperman Seminars in Pediatric Surgery 31 (2022) 151145

of emesis helps distinguish pyloric stenosis from other causes of surgeon experience with physical exam. When reliably palpated
neonatal emesis. In HPS, emesis is typically forceful and occurs im- by experienced hands, presence of the “olive” has been associated
mediately after feeding. Parents may report a worsening of these with a specificity as high as 99%9 , 34 .
symptoms over a period of weeks, despite attempts at changing In the same vein, hypertrophic pyloric stenosis should be con-
feeding regimens or formulations. Typically, the emesis does not sidered in all patients with repeated emesis, even in the absence
contain bile, but bilious emesis should not exclude pyloric steno- of all classic laboratory or exam findings. It is possible for eme-
sis as it has been reported to occur in up to 4% of cases of HPS6 . sis to be bilious6 , though this should also raise concern for in-
Additionally, infants often have a strong appetite despite consistent testinal obstruction caused by malrotation, Hirschsprung disease,
post-prandial emesis. Dehydration can be suggested by eliciting a and intestinal atresias. Notably, there is a reported association of
history of decreased urine output with fewer wet diapers. hypertrophic pyloric stenosis with malrotation. This has been de-
Physical exam is often notable for a dehydrated and some- scribed at rates higher than that of the general population, though
times emaciated appearing infant with a palpable “olive” mass in the effect of surveillance bias is uncertain35 Other neonatal con-
the right upper quadrant. Abdominal exam may also show peri- ditions can dampen the symptoms of HPS, including less forceful
staltic waves moving from left to right caused by the stomach emesis that can be seen with prematurity, cleft lip/palate, and cen-
attempting to pass contents past the pyloric obstruction. Other tral nervous system abnormalities. A history of anatomic gastroin-
characteristics can include plateauing weight and length due to testinal anomalies such as intestinal atresia can also make the di-
poor nutrition, dry mucous membranes and poor skin turgor as agnosis challenging. A common clinical association of HPS is hy-
signs of dehydration, as well as tachycardia and irritability. Pa- perbilirubinemia, most commonly unconjugated, called icteropy-
tients may also demonstrate jaundice related to icteropyloric syn- loric syndrome. This may be present in as many as 14% of cases
drome, but this may also be a sign of liver disease. Laboratory of HPS and typically resolves with treatment of pyloric steno-
workup demonstrates a hypochloremic, hypokalemic metabolic al- sis, though it can be an early sign of Gilbert syndrome (biliru-
kalosis, with more severe derangements found with a longer dura- bin uridine diphosphate-glucuronosyltransferase mutation)36 . Close
tion of symptoms prior to presentation. Elevated blood urea nitro- observation and follow-up of infants with continued emesis is es-
gen (BUN) and creatinine are also helpful markers of dehydration. sential.
Complete blood counts (CBC) should be normal, and with the ex-
ception of unconjugated hyperbilirubinemia, abnormalities in liver Differential diagnosis
function tests should raise concern for liver disease.
Hypochloremic, hypokalemic metabolic alkalosis is a physi- It is common for infants with hypertrophic pyloric stenosis to
ologic response to persistent emesis. Persistent vomiting leads receive other diagnoses, especially early in the disease course, and
to depletion of sodium, potassium, chloride, and hydrogen ions. the differential diagnosis of neonatal emesis is broad. Attention
Dehydration and eventual intravascular hypovolemia stimulate to historical factors such as age, character of emesis, and associ-
the renin-angiotensin-aldosterone system, causing increased aldos- ated symptoms and workup can distinguish the diagnosis of py-
terone production and renal absorption of sodium and water. The loric stenosis. Gastroesophageal reflux (GERD) is a common con-
kidneys also conserve sodium and potassium at the expense of dition with similar symptoms of emesis after feeding, which can
hydrogen causing a paradoxical aciduria. Hypochloremia inhibits also lead to failure to thrive. Compared to HPS, emesis related to
the kidneys’ ability to excrete bicarbonate attempting to main- GERD is generally less forceful and smaller in volume (the “happy
tain a normal pH, so more bicarbonate is reabsorbed, generat- spitter”), and it is uncommon for GERD to be associated with sig-
ing a metabolic alkalosis. Fluid and electrolyte shifts also lead to nificant electrolyte abnormalities. If in doubt, a diagnosis of GERD
potassium derangements, generally hypokalemia (although hyper- can be confirmed with an upper gastrointestinal series, endoscopy,
kalemia can also be seen likely from hemolysis)14 . and pH/impedance monitoring. Overfeeding can contribute to post-
There is increasing evidence that this classic presentation de- prandial emesis in newborns and can be clarified by examina-
scribed has become less common over time, especially in regards tion of feeding volumes. Patients with overfeeding will not exhibit
to electrolyte abnormalities and physical exam. In a retrospective failure to thrive, laboratory abnormalities, or the ultrasound find-
review by Tutay et al., low potassium (K), and chloride (Cl) were ings associated with pyloric stenosis14 . Infectious gastroenteritis is
observed in only 8%, and 25% of patients at presentation, respec- also a consideration, though this is usually associated with diar-
tively. The proportion of patients with HPS that presented with el- rhea (compared to constipation in pyloric stenosis). Children with
evated serum bicarbonate (CO2 ) was only 18% compared to 62% gastrointestinal infections can be very ill upon presentation with
of patients with HPS presenting with normal serum CO2 29 . It electrolyte disturbances resembling those of pyloric stenosis. Pa-
has been hypothesized that this change is due to earlier diag- tients may exhibit other infectious signs such as fever and ele-
nosis, at least in part due to the increased availability of ultra- vated white blood cell count, and stool cultures may implicate spe-
sound. Papadakis et al. compared groups of infants treated for py- cific pathogens. Food allergies such as milk protein allergy may
loric stenosis from 1975, 1985, and 1995 and did not find statisti- also present with both vomiting and diarrhea, often with tinge
cally significant differences in abnormal lab results across the three of blood in the stool. Allergies should respond to dietary adjust-
cohorts30 , indicating that earlier diagnosis may not be solely at- ment14 . Adrenal crisis, such as in patients with congenital adrenal
tributable to ultrasonography. However, more recent studies have hyperplasia (CAH), may also present with emesis and dehydration.
continued to demonstrate earlier diagnosis and fewer lab abnor- This condition is life-threatening, and the associated electrolyte
malities upon admission when compared to older cohorts6 . Despite abnormality is typically hyperkalemic acidosis as opposed to hy-
these changes, hypochloremic, hypokalemic metabolic alkalosis is pokalemic alkalosis seen in HPS. If CAH is the cause, external signs
still present in a significant portion of patients with pyloric steno- such as virilization or ambiguous genitalia may be present. As dis-
sis and should prompt further investigation31 . cussed previously, infants with conjugated hyperbilirubinemia or
Another change in the presentation of pyloric stenosis is in the elevated transaminases should be evaluated for possible liver dis-
presence of the palpable “olive” on exam. Previously reported to be ease.
present in as many as 87% of patients32 , more recent studies have In any child with bilious emesis, malrotation with or without
found that number as low as 13.6%33 . It has been proposed that, midgut volvulus should be ruled out urgently, as this can be a sur-
as with the decrease in laboratory abnormalities, the prevalence of gical emergency. As discussed above, pyloric stenosis can present
ultrasound technology has led to earlier diagnosis and decreased with bilious emesis, but other diagnoses should be considered

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M.E. Danko, P.T. Evans and J.S. Upperman Seminars in Pediatric Surgery 31 (2022) 151145

Fig. 1. Ultrasound documenting (1) pyloric muscle thickness 5.2 mm and (2) pyloric channel length 17.1 mm.

first6 . Upper GI fluoroscopy (UGI) demonstrating a duodenojeju- trasound in experienced hands is a safe and powerful tool in the
nal junction failing to cross midline is diagnostic of malrotation. diagnosis of pyloric stenosis.
Gastrointestinal atresias (pyloric, duodenal, jejunoileal) are also as- Alternative imaging options if ultrasonography is inconclusive
sociated with bilious emesis. Most cases, especially neonates with or if other gastrointestinal anomalies are higher on the differen-
complete atresia, are diagnosed at a younger age, usually immedi- tial include upper GI contrasted studies and endoscopy. In pa-
ately after initiation of oral feeding. Diagnosis can be made with tients with HPS, an upper GI study demonstrates a thin “string”
abdominal radiographs, with contrast if necessary14 . Other causes of contrast at the site of the narrowed pylorus. It may also reveal
of bilious emesis and intestinal obstruction include Hirschsprung the “shoulder sign,” an external compression seen on the gastric
disease and intussusception. antrum caused by the thickened muscle. In addition to exposing
the infant to radiation through fluoroscopy, UGI is a more invasive
Imaging procedure than ultrasound. Ingested contrast may cause further
emesis and increased risk for aspiration. For these reasons, UGI is
Since its first description in 1977, the use of ultrasound to diag- typically reserved for cases in which diagnosis remains uncertain
nose hypertrophic pyloric stenosis has become widespread37 , 38 . Its after ultrasound or in hospitals without pediatric ultrasound ex-
benefits include accessibility and lack of exposure to radiation. Py- pertise39 . Similarly, endoscopy is not used as a primary diagnostic
loric diameter (PD) was originally described as the diagnostic cri- tool except in inconclusive cases or when other diseases are sus-
terion, with an upper limit of normal of 10-14 mm39 . Currently, a pected as it is an invasive procedure and also involves sedation.
positive diagnosis of HPS on ultrasound is characterized by pyloric Endoscopic findings in patients with HPS include thickened mu-
muscle thickness (PMT) > 3 mm and pyloric canal length ≥ 15 cosa of the antrum and pylorus.
mm (Fig. 1). Other less commonly used criteria include pyloric vol-
ume (PV) and pyloric ratio (PR), which are calculated values based
on the above measurements. Notably, pyloric measurements have Pre-operative and medical management
been shown to vary with the weight and age of the patient, and
premature or particularly small infants may not fit within stan- Definitive treatment of pyloric stenosis is surgical pyloromy-
dard diagnostic criteria. This should be taken into consideration otomy. Pre-operative resuscitation and correction of metabolic de-
when interpreting ultrasound results for these infants, as they may rangements are critically important prior to surgical intervention.
still have true pyloric stenosis despite technically “normal” mea- Surgery should not be undertaken until the infant is appropri-
surements40 , 41 . Real-time pyloric function should also be observed ately resuscitated. The goal of initial fluid resuscitation is to cor-
on ultrasound, which will demonstrate abnormal flow and peri- rect dehydration and metabolic derangements. The recommended
stalsis in an infant with pyloric stenosis. Importantly, it will also pre-operative resuscitation fluid is an isotonic fluid such as 0.45%
reveal failure of relaxation of the pyloric canal42 . Both sensitiv- or 0.9% normal saline with 5% dextrose at 1.5 to 2 times the main-
ity and specificity of ultrasonography for the diagnosis of pyloric tenance rate or approximately 150mL/kg/day9 , 43 , 44 . Patients with
stenosis have been shown to be excellent, as high as 95% and in severe volume depletion may also benefit from a bolus with 10-
some studies even approaching 100% depending on the skill and 20 mL/kg of 0.9% normal saline prior to or alongside continu-
experience of the examiner39 , 40 . Interestingly data has shown that ous infusion45 . Potassium should be held out of the maintenance
point of care ultrasound used by emergency medicine providers fluid infusion until urine output has normalized at 1-2 mL/kg/hour
and surgeons can also be accurate, with rates comparable to the due to the risk of rebound hyperkalemia. After urine output im-
radiologists9 . There are several potential causes of inconclusive or proves, potassium should be added at a concentration of 10 to 20
incorrect results from ultrasound. Temporary causes of pyloric ex- mEq/L14 , 44 . If a nasogastric tube (NGT) is placed preoperatively and
pansion such as pylorospasm or filling of the antrum and/or duo- output is high, replacement of this fluid should also be consid-
denal bulb with fluid may generate measurements consistent with ered (ex. 1 mL to 1 mL replacement with 0.9% normal saline with
pyloric stenosis, a false-positive result. Alternatively, falsely nega- 10 mEq/L potassium)46 , 47 . Preoperative observation with telemetry
tive or inconclusive results may be obtained when the pylorus is monitoring in an intensive care setting may be warranted with se-
poorly visualized, such as with overlying bowel gas. However, ul- vere hypokalemia.

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As discussed previously, upon diagnosis many patients with Surgical management

HPS have some component of a hypochloremic, hypokalemic
metabolic alkalosis and may also be dehydrated with nutritional The extramucosal longitudinal splitting of the pyloric muscle for
deficiency and weight loss. Patients taken to the operating room hypertrophic pyloric stenosis was first described as early as 1910
prior to resuscitation have been shown to have worse outcomes by Stiles and made known by Ramstedt in 191255 , 56 . Regardless of
than those who undergo pre-operative fluid resuscitation44 , 48 , 49 . the approach, the goal of the pyloromyotomy is longitudinal divi-
Metabolic alkalosis can affect the infantile respiratory drive, lead- sion of the pylorus down to the submucosal layer, from the circu-
ing to difficulty in extubation and apnea. Despite these potentially lar fibers of the stomach wall proximally to the prepyloric vein of
life-threatening consequences, there is currently limiting data sup- Mayo on the duodenum distally. Adequacy can be tested by dis-
porting what an acceptable level of alkalosis. is in order for an in- tension of the stomach with air which should pass into the duode-
fant to undergo safe general anesthesia50 . Metabolic alkalosis has num, and this maneuver also tests for leak at the site of pyloromy-
also been shown to correlate with increased postoperative emesis, otomy. The two edges of pyloric muscle can be seen to move inde-
time to goal feeds, and length of stay44 , 49 , 51 . pendently after adequate pyloromyotomy.
Adequacy of resuscitation is determined by a combination of Multiple surgical approaches have been described, and this re-
physical exam and laboratory markers. Patients should have im- mains an ongoing topic of discussion and innovation. The tradi-
provement in skin turgor, moist mucous membranes, normaliza- tional open approach is a transverse incision in the right upper
tion of urine output, and improvement in hypotension and tachy- quadrant. This incision provides excellent exposure to the pylorus
cardia if present. The goal should be electrolyte correction prior but leaves a scar that can become significant as it grows with
to pyloromyotomy. Exact thresholds for electrolyte improvement the patient. An alternative open access to the abdomen utilizes a
vary among practices, but common levels include a serum bicar- semicircular incision superior to the umbilicus, followed by skin
bonate of less than 26-28 mEq/L and chloride of greater than 95- flap formation, and midline division of the linea alba superiorly
100 mEq/L44 . In a 2020 international consensus study by van den (Fig. 2A, 2B). This circumumbilical approach introduced by Tan and
Bunder et al, an expert panel of pediatric surgeons, pediatric anes- Bianchi has a more favorable cosmetic outcome, but pyloromy-
thetists, and pediatricians were surveyed on recommendations for otomy can be technically challenging57 . With modern development
preoperative care of HPS. The study recommends following labora- of laparoscopic surgery in the 1980’s and 90’s, the laparoscopic
tory thresholds prior to proceeding with surgery: pH ≤ 7.45, base pyloromyotomy was introduced by Alain et al58 . Typical incision
excess ≤ 3.5, bicarbonate < 26 mmol/L, sodium ≥ 132 mmol/L, placement includes an umbilical laparoscope port (3 to 5 mm) and
potassium ≥ 3.5 mmol/L, chloride ≥ 100 mmol/L, and glucose ≥ 72 two lateral working incisions, one on each side abdomen slightly
mg/dL43 . Acceptable electrolyte levels are generally reached within superior to the camera (Fig. 3A, 3B, 3C, 3D). As with other la-
24 hours of fluid resuscitation9 . paroscopic procedures, laparoscopic pyloromyotomy has a learning
In addition to correction of alkalosis, other pre- and periop- curve for consistent successful performance estimated to be ap-
erative anesthesia considerations include management of aspira- proximately 35 procedures9 , 59 .
tion risk and intraoperative anesthetic technique. Patients should The comparative risks and benefits of open pyloromyotomy (OP)
be kept nil per os (NPO) prior to surgery due to the risk of as- vs laparoscopic pyloromyotomy (LP) has been extensively studied
piration. It has also become widely accepted to empty an infant’s and debated. Five randomized controlled trials were performed be-
stomach with orogastrasic or nasogastric suction immediately prior tween 1997 and 2012 with study sizes ranging from 20 to 200
to anesthesia. The debate continues as to whether preoperative na- patients. Studies had different designs and did not all report on
sogastric tube (NGT) placement is beneficial. Proponents of early the same outcomes, but in general they reported multiple bene-
nasogastric decompression cite the risk of aspiration from gastric fits of LP including shorter time to full feeds, reduced pain, lower
secretions, whereas others feel that most infants can handle their analgesic requirement, shorter length of stay, and higher cosmetic
own secretions and NG tubes exacerbate electrolyte abnormalities. satisfaction9 . A 2012 review and meta-analysis of 4 of these stud-
A small 2015 randomized controlled trial (RCT) comparing patients ies (excluding the study from 2012) found a total incidence of
with and without NGT placement prior to surgery (POINTS trial, complications of 4.9% in the LP group (n = 12 compared to 5 in
50 patients enrolled) found that infants with preoperative NGT had the OP group) but did not show statistical difference. The analy-
slightly higher rates of postoperative emesis, though the study was sis indicated a shorter time to full feeds (p = 0.02) and possibly
underpowered to demonstrate statistical significance52 . In a 2019 shorter length of stay (p = 0.20) in the LP group60 . A 2020 RCT
multicenter retrospective cohort study (481 patients), Lee et al. enrolling 80 patients demonstrated shorter operative time, lower
associated early NGT placement with a longer time to electrolyte pain scores, shorter time to full feeds, shorter hospital stay, and
correction and surgery as well as longer postoperative length of greater satisfaction in the LP group61 .
stay but did not see increased risk of postoperative feeding intoler- Most recently, a 2021 Cochrane Database review was published
ance46 . Another classic teaching for the reduction of aspiration risk by Staerkle et al. comparing open to laparoscopic pyloromyotomy.
has been to perform rapid sequence induction (RSI) of anesthesia, The review included 7 randomized controlled trials for a total of
as opposed to a gas induction. However, a true rapid sequence in- 720 patients (357 open, 363 laparoscopic). The report concluded
duction can be challenging in young infants, with the risk of hy- that evidence suggested a possible small increase in the risks of
poxemia and failed intubation. Scrimgeour et al. showed in a ret- mucosal perforation and incomplete pyloromyotomy with laparo-
rospective cohort study of 269 patients that few patients receive scopic surgery but rated the evidence as “low-certainty”. Other
a true RSI in practice and that gas induction is a safe alternative outcomes included length of stay, time to full feeds, operation
with minimal aspiration risk in the hands of experienced pediatric time, and incidence of wound infections, abscess, and hernia. The
anesthesiologists53 . In the setting of open pyloromyotomy, thoracic results for all of these outcomes were reported as inconclusive,
epidural anesthesia can be an alternative to general anesthesia. In a with “very low-certainty evidence”. Certainty was downgraded for
review of 97 patients undergoing open pyloromyotomy, Opfermann most outcomes due to limitations in study designs and impreci-
et al. found higher mean oxygen saturation and fewer desaturation sion. The authors concluded that there is insufficient unbiased data
events (<90% oxygen saturation) in the epidural anesthesia group to make firm conclusions in favor of one operation or the other.
compared to general anesthesia, illustrating the safety of this tech- Taken in summary, it is apparent that there may be postoper-
nique54 . ative benefits to a laparoscopic approach including shorter length

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M.E. Danko, P.T. Evans and J.S. Upperman Seminars in Pediatric Surgery 31 (2022) 151145

Fig. 2. Open pyloromyotomy through circumumbilicalincision. A) Hypertrophic pylorus exteriorized via circumumbilicalincision. B) Circumumbilicalincision in the supraum-
bilical fold. From the collection of Jeffrey Upperman, used with permission.

Fig. 3. Laparoscopic pyloromyotomy. A) Laparoscopic incisions. B) Appearance of hypertrophic pylorus. C)Longitudinalpyloromyotomy incision. D) Spreading of muscle expos-
ing underlying submucosa. From the collection of Irving Zamora, used with permission.

of stay, shorter time to full feeds, and better cosmetic outcome at scopic pyloromyotomy (EP), also known as peroral pyloromyotomy
a possible small increased risk of mucosal perforation or incom- or gastric peroral endoscopic myotomy (G-POEM), is performed by
plete pyloromyotomy compared to open pyloromyotomy. Given the insertion of an upper endoscope followed by mucosal incision at
uncertainty of the data, a surgeon is justified in choosing either the proximal antrum, creation of a submucosal tunnel, and full-
approach, and the most effective and safest approach is likely to thickness pyloromyotomy. The mucosal entry site is closed with
be that with which the surgeon is most comfortable. endoscopic clips. Data reporting outcomes of this technique are
Several additional more recently developed techniques have limited to early case series and previous animal work, but appears
also been described. These include single-incision laparoscopic py- to be feasible65 , 66 .
loromyotomy, microlaparoscopic pyloromyotomy, and endoscopic
pyloromyotomy. Single incision LP utilizes a single umbilical inci- Postoperative management
sion for the introduction of a laparoscope and two 3mm instru-
ments. Available data indicates that outcomes are likely similar to After pyloromyotomy, patients require continued inpatient ob-
traditional laparoscopic pyloromyotomy9 , 62 . Operative times may servation and pain control. Even well-resuscitated infants are still
be longer and the learning curve steeper than standard LP, al- at risk for postoperative apnea due to their age and the effects of
though Harmon suggests that the learning is less from LP to SLP anesthesia, and those who experienced significant alkalosis preop-
than from OP to LP61 . Microscopic pyloromyotomy (MP) is per- eratively may have an even higher risk. Thus, all patients should
formed in a similar manner to LP, though with smaller instruments be closely monitored after pyloromyotomy, generally for at least
(2 mm instruments and 1.7 to 2.4 mm laparoscope)63 , 64 . Data is 24 hours.
confined to small retrospective case series but MP seems to have An important and widely studied postoperative consideration is
similar complication rates, improved cosmesis, and shorter opera- the timing and regimen of reintroduction of enteral feeds. Many
tive times when compared to traditional laparoscopy62 , 63 . Endo- different strategies have been described, ranging from feeding im-

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M.E. Danko, P.T. Evans and J.S. Upperman Seminars in Pediatric Surgery 31 (2022) 151145

mediately after recovery from anesthesia to periods of fasting of GERD, enteric infections (such as rotavirus), or rarely incomplete
up to 24 hours9 . Regardless of the exact feeding protocol, evi- myotomy72 . The use of atropine as a pyloric antispasmodic for pro-
dence suggests that implementation of a well-defined hospital- tracted vomiting has also been explored with reported success in
wide protocol may improve patient care and hospital length of preventing unnecessary reoperations73 . However, if a patient con-
stay67 . Acker et al. compared the postoperative care and length tinues to have persistent emesis and intolerance to feeds after 4
of stay between two different hospital units and found statisti- or 5 days, the possibility of incomplete pyloromyotomy should be
cally significant differences in both time to ad libitum feeds and considered. If suspected, radiologic evaluation can be useful, and
postoperative length of stay (LOS), suggesting that a hospital-wide patients may require a return to the operating room for further ex-
protocol may improve discrepancies67 . Clayton et al. performed a ploration. Patients may be discharged once pain is controlled and
retrospective analysis combined with a prospective implementa- they are feeding well at goal volumes, which is a factor of their
tion of a postoperative care protocol for HPS patients. Comparing weight. In practice, there are often other contributory factors such
pre- and post-implementation outcomes, the post-implementation as social and logistical considerations that factor into the timing of
group had shorter time to feeds, less emesis, and shorter length of discharge.
stay68 . By the time of follow-up in 2-3 weeks, there should be no more
There has also been increasing evidence to suggest that infants emesis and the incision site(s) should be healing appropriately.
progress faster on feeding protocols that emphasize ab libitum There are generally no serious long-term sequelae of HPS, and af-
feeding, rather than inflexible incremental feeding protocols. In a ter returning to regular feeding most infants can return to routine
2013 review, Graham et al. examined two questions about feed- pediatric care. Growth and weight gain should be monitored. Some
ing patterns: immediate vs delayed feeding; and incremental vs ad children may develop benign gastroesophageal reflux, but prolon-
lib feeding. They concluded that immediate postoperative feeding gation or recurrent of more severe symptoms should prompt fur-
within 6-8 hours postoperatively depending on the study was as- ther workup.
sociated with increased emesis, though there is likely no impact
on time to discharge, and recommended a fasting period of about Non-surgical management
4 hours. Regarding ad lib feeds, the majority of studies reviewed
demonstrated a shorter time to feeding without a significant in- While surgical management is the gold standard in treatment
crease in postoperative emesis and most likely with a shorter time of hypertrophic pyloric stenosis, there are nonoperative treatment
to discharge, leading the authors to recommend ad lib feeding reg- options for cases in which a patient cannot undergo surgery. Med-
imens over incremental69 . In 2014, a prospective randomized trial ical treatment with atropine sulphate can be given intravenously
was published comparing ad libitum feeds to a specific protocol. or orally as a pyloric antispasmodic agent. In theory, atropine
The ad lib group, which was allowed 60 mL formula or breast milk suppresses muscular contractions and gastrointestinal peristalsis
2 hours postop, were found to achieve goal feeds about 7 hours which lessens the muscular spasm that is thought to cause the
faster than the protocolized group (2 rounds of Pedialyte, followed muscular hypertrophy of HPS. Atropine treatment was initially re-
by 2 rounds of half strength formula/breast milk, followed by 2 ported to be successful at resolving HPS in as many as 90% of
rounds of full strength formula/breast milk, followed by home reg- cases, though other studies have reported rates as low as 70%
imen) but did not achieve a shorter postoperative length of stay70 . (compared with nearly 100% resolution for patients treated sur-
This was explained by the fact that hospital stay is not solely deter- gically)74-76 . Medical treatment has also been associated with a
mined by the time taken to achieve and tolerate full enteral feeds. significantly longer length of hospital stay (10 or more vs 5 days
Social and logistical factors all play a role in time of discharge. The with pyloromyotomy) and may require weeks to months of oral
ad lib group also did not pause feeds for emesis, and there was atropine therapy prior to pyloric muscle normalization74 , 76 , 77 . This
no significant difference between the rate of post-operative vom- long hospitalization also requires the use of parenteral nutrition
iting between the two groups69 . In a 2016 review and metanaly- or the ability to deliver postpyloric feeds. Atropine therapy has
sis, Sullivan et al. again assessed outcomes in studies comparing consistently been shown to be safe with minimal side effects74 , 76 .
early to late feeding and ad lib to structured feeding regimens. Given the broad surgical experience and relative efficacy with py-
They found a statistically significant shorter length of stay to be loromyotomy, treatment of HPS with atropine should only be con-
associated with early feeding and with ad lib protocols, though sidered in patients with very high surgical risk or in regions where
early feeding was associated with a higher rate of postoperative neonatal surgery is not available74 . Interesting there has been a
emesis71 , 72 . In summary, immediate postoperative feeding is may case report of successful atropine use as a ‘rescue therapy’ two
be associated with increased vomiting, but shorter fasting periods weeks post-operatively in an infant who underwent an incomplete
and shorter times to full enteral feeds. Ad lib feeding protocols and pyloromyotomy78 . Endoscopic balloon dilation is another potential
hospital wide implementation of postoperative care protocols are treatment that is less invasive than pyloromyotomy. However, early
associated with shorter time to goal feeds and shorter postopera- experience with dilation was unsuccessful in many cases with a
tive length of stay. high risk of pyloric perforation14 , 79 . This procedure may be con-
As an example of a post-operative feeding protocol for HPS, sidered in special circumstances but should be approached with
the current protocol at the writers’ institution is summarized here. caution2 .
Infants are kept NPO in the post-operative anesthesia care unit
(PACU). Once they return to the acute care floor, they are initiated Outcomes and complications
on ad lib breast-feeding or formula feeds. Feeds are not held for
emesis unless severe. If a patient experiences emesis of >15 mL Outcomes for patients with hypertrophic pyloric stenosis are
for 3 consecutive feeds, the covering provider is contacted and vol- generally excellent after pyloromyotomy, with the vast majority of
ume is backed to 30 mL until feeds are tolerated without emesis. patients achieving complete resolution of symptoms within days
Patients are considered appropriate for discharge after tolerating 2 after surgery. Pyloromyotomy has low morbidity and mortality.
consecutive 60 mL feeds of breast milk or formula without projec- Significant complications include mucosal perforation, incomplete
tile emesis. myotomy, and surgical site infection. Overall complication rates are
Postoperative emesis is common in infants after pyloromyotomy between 4.6 and 12%9 .
and is generally self-limited. The cause is not always known, but Mucosal perforation is a rare but significant complication that
most cases are thought to result from aggressive feeding protocols, may be recognized intraoperatively by visual inspection or a leak

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M.E. Danko, P.T. Evans and J.S. Upperman Seminars in Pediatric Surgery 31 (2022) 151145

test, or postoperatively by worsening clinical condition including infants undergoing pyloromyotomy showed increasing numbers of
fevers and sepsis. Perforation requires surgical repair of the mu- pyloromyotomies being performed at pediatric hospitals from 57%
cosal perforation, generally with omental patch reinforcement. If in 1987 to 99% in 200975 . The odds of postoperative complications
closure of the pyloromyotomy is necessary to contain the perfo- were also significantly lower at pediatric hospitals, and they con-
ration, repeat pyloromyotomy should be performed on the oppo- cluded that optimal outcomes occur with pediatric surgeons75 .
site side of the pylorus, 180 degrees from the site of the origi- Cost analysis in pyloromyotomy has recently been investigated.
nal myotomy. Rates of perforation have been reported from 0 to The total cost of hospitalization for patients with pyloric stenosis
11.5%, possibly with a slightly higher incidence with laparoscopic has been reported at around $5,0 0 0, assuming an uncomplicated
repair80 . A 2014 multicenter study by Hall et al. examining al- course with a single operation39 , 91 . A 2016 study of inpatient cost
most 30 0 0 pyloromyotomies reported an overall perforation rate and charge data from the Kid’s Inpatient Database (KID) found an
of 0.63%, with a statistically insignificant higher rate with laparo- average cost of $5,351, which was higher in urban hospitals, hospi-
scopic pyloromyotomy81 . tals with fewer beds, and the western region of the United States91 .
Incomplete myotomy should be considered in patients with One study demonstrated higher total costs in open surgery com-
emesis persisting days after surgery and reintroduction of feeds. pared to laparoscopic pyloromyotomy due to longer time to full
This complication has been reported at rates of 0 to 5.5% in laparo- feeds and hospital stay92 . Finally, costs have been reported to be
scopic surgery and 0 to 1.9% for open pyloromyotomy80 . Hall et al. higher when surgery is performed by general surgeons than by
found an overall rate of 0.85% with a slightly higher incidence in pediatric surgeons, presumably due to differences in complication
the laparoscopic group81 . Evaluation for incomplete myotomy can rates39 .
be done with repeat RUQ ultrasound or upper GI study. However,
even with successful pyloromyotomy, swelling in the pyloric area
Conclusions
can persist for weeks, and imaging should be interpreted with cau-
tion. While atropine has been reported as a possible treatment for
Hypertrophic pyloric stenosis is a common disease of infants
incomplete pyloromyotomy, the definitive solution is reoperation
that remains an important area of investigation. Although the etiol-
with completion myotomy73 .
ogy continues to be elusive, studies indicate an acquired combina-
The rate of postoperative surgical site infection is reported to be
tion of genetic and environmental factors. The classic presentation
between 0.3 and 12% and can generally be treated with antibiotics.
of a wasted infant with hypochloremic, hypokalemic metabolic al-
In the event of abscess formation, incision and drainage or opening
kalosis is less common today, likely due to earlier presentation and
of surgical incisions may be required14 , 80 .
imaging modalities. This in combination with excellence in both
Postoperative emesis is common with an incidence ranging
surgical and postoperative care have made HPS a curable disease
from 25-90% and typically resolves without additional interven-
in nearly 100% of cases with very small complication rates in the
tion52 . Postoperative feeding regimens may need to be adjusted for
hands of specialized centers. It remains a task for the pediatric
significant or persistent emesis.
community to disseminate these advances with the goal of pro-
The discussed complications are rare and correctable, making
viding equivalent care to infants of socioeconomically challenged
the prognosis of HPS extremely good with an almost 0% overall
backgrounds and in developing regions of the world.
mortality rate80 . Earlier recognition of HPS with the aid of ultra-
sound technology has reduced the incidence of presentation with
severe electrolyte disturbances, reducing perioperative morbidity. References
Vulnerable populations such as premature infants and those with
1. Mazurak M, Patkowski D. A history of the surgical correction of pyloric stenosis.
cardiac conditions have been shown to have increased morbid-
J Pediatr Surg. 2021;56:1904–1907.
ity and postoperative length of stay82 , 83 . Notably, mortality rates 2. Galea R, Said E. Infantile Hypertrophic Pyloric Stenosis: An Epidemiological Re-
have been reported to be higher in the developing world, as view. Neonatal Netw. 2018;37:197–204.
3. Donda K, Asare-Afriyie B, Ayensu M, et al. Pyloric Stenosis: National Trends in
high as 4.9% in one publication from Tanzania. The majority of
the Incidence Rate and Resource Use in the United States From 2012 to 2016.
deaths were thought to be related to delayed presentation with se- Hosp Pediatr. 2019;9:923–932.
vere metabolic derangements84 . Even in the United States, African 4. Aboagye J, Goldstein SD, Salazar JH, et al. Age at presentation of common pedi-
American race and uninsured status have been reported to be asso- atric surgical conditions: reexamining dogma. J Pediatr Surg. 2014;49:995–999.
5. Krogh C, Gortz S, Wohlfahrt J, Biggar RJ, Melbye M, Fischer TK. Pre- and Peri-
ciated with higher bicarbonate levels, lower chloride levels, longer natal Risk Factors for Pyloric Stenosis and Their Influence on the Male Predom-
time to operation, and longer length of stay compared to white and inance. Am J Epidemiol. 2011;176:24–31.
insured patients, reflecting inequalities in access to care85 . 6. Taylor ND, Cass DT, Holland AJA. Infantile hypertrophic pyloric stenosis: Has
anything changed? J Paediatr Child Health. 2012;49:33–37.
The majority of patients have no long-term sequelae of HPS, 7. Rogers IM. Hyperacidity, overfeeding, prostaglandins, and pyloric stenosis of in-
though long-term trends including higher rates of chronic abdom- fancy. J Pediatr Surg. 2019;54:2636–2637.
inal pain and possible effects on cognitive development have been 8. Rogers IM. The cause of pyloric stenosis of infancy: A hyperacidity pathogenesis.
Med Hypotheses. 2020;141:108–116.
reported86 , 87 . A subsequent large database retrospective cohort 9. Jobson M, Hall NJ. Contemporary management of pyloric stenosis. Semin Pediatr
study indicates that effects on cognition are likely minimal, but Surg. 2016;25:219–224.
that if present may be related to increased susceptibility to neona- 10. Kusafuka T, Puri P. Altered messenger RNA expression of the neuronal nitric
oxide synthase gene in infantile hypertrophic pyloric stenosis. Pediatr Surg Int.
tal anesthesia88 .
1997;12:576–579.
Overall success of pyloromyotomy approaches 100% in patients 11. Subramaniam R, Doig CM, Moore L. Nitric oxide synthase is absent in only a
with hypertrophic pyloric stenosis, especially in high volume cen- subset of cases of pyloric stenosis. J Pediatr Surg. 2001;36:616–619.
12. Serra A, Schuchardt K, Genuneit J, Leriche C, Fitze G. Genomic variants in the
ters with specialty trained pediatric surgeons89 . A systemic review
coding region of neuronal nitric oxide synthase (NOS1) in infantile hypertrophic
analyzed patient outcomes after pyloromyotomy impacted by dif- pyloric stenosis. J Pediatr Surg. 2011;46:1903–1908.
ferent surgical specialties, case volume, and hospital type. Evans 13. Boybeyi O, Soyer T, Atasoy P, Gunal YD, Aslan MK. Investigation of the ef-
et al. suggested that surgical volume was the best indicator of fects of enteral hormones on the pyloric muscle in newborn rats. J Pediatr Surg.
2015;50:408–412.
outcomes90 . General surgeons higher operative volumes had out- 14. Hunter CJ, Klonoski SC. Pyloric Stenosis. BMJ Best Practice. 2020.
comes comparable to pediatric surgeons. They also stated that pe- 15. Krogh C, Fischer TK, Skotte L, et al. Familial aggregation and heritability of py-
diatric surgeons had lower complications rates including a lower loric stenosis. JAMA. 2010;303:2393–2399.
16. Feenstra B, Geller F, Carstensen L, et al. Plasma Lipids, Genetic Variants
risk of duodenal perforation. Access to a children’s unit was also Near APOA1, and the Risk of Infantile Hypertrophic Pyloric Stenosis. JAMA.
important for improved outcomes. A more recent review of 3500 2013;310:714–721.

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16, 2022. For personal use only. No other uses without permission. Copyright ©2022. Elsevier Inc. All rights reserved.
M.E. Danko, P.T. Evans and J.S. Upperman Seminars in Pediatric Surgery 31 (2022) 151145

17. Feenstra B, Geller F, Krogh C, et al. Common variants near MBNL1 and 49. Kamata M, Cartabuke RS, Tobias JD. Perioperative care of infants with pyloric
NKX2-5 are associated with infantile hypertrophic pyloric stenosis. Nat Genet. stenosis. Paediatr Anaesth. 2015;25:1193–1206.
2012;44:334–337. 50. Peters B, Oomen MWN, Bakx R, Benninga MA. Advances in infantile hyper-
18. Everett KV, Chung EMK. Confirmation of two novel loci for infantile trophic pyloric stenosis. Expert Rev Gastroenterol Hepatol. 2014;8:533–541.
hypertrophic pyloric stenosis on chromosomes 3 and 5. J Hum Genet. 51. St Peter SD, Tsao K, Sharp SW, Holcomb 3rd GW, Ostlie DJ. Predictors of emesis
2013;58:236–237. and time to goal intake after pyloromyotomy: analysis from a prospective trial.
19. Svenningsson A, Soderhall C, Persson S, et al. Genome-wide linkage analysis in J Pediatr Surg. 2008;43:2038–2041.
families with infantile hypertrophic pyloric stenosis indicates novel susceptibil- 52. Flageole HH, Pemberton J. Post-Operative Impact of Nasogastric Tubes on length
ity loci. J Hum Genet. 2012;57:115–121. of stay in infants with pyloric Stenosis (POINTS): A prospective randomized
20. Everett KV, Chioza BA, Georgoula C, Reece A, Gardiner RM, Chung EMK. Infantile controlled pilot trial. J Pediatr Surg. 2015;50:1681–1685.
hypertrophic pyloric stenosis: evaluation of three positional candidate genes, 53. Scrimgeour GE, Leather NW, Perry RS, Pappachan JV, Baldock AJ. Gas induction
TRPC1, TRPC5 and TRPC6, by association analysis and re-sequencing. Hum Genet. for pyloromyotomy. Paediatr Anaesth. 2015;25:677–680.
2009;126:819–831. 54. Opfermann P, Wiener C, Schmid W, et al. Epidural versus general anesthesia for
21. Svenningsson A, Svensson T, Akre O, Nordenskjold A. Maternal and pregnancy open pyloromyotomy in infants: A retrospective observational study. Paediatr
characteristics and risk of infantile hypertrophic pyloric stenosis. J Pediatr Surg. Anaesth. 2021;31:452–460.
2014;49:1226–1231. 55. Keys C, Johnson C, Teague W, MacKinlay G. One hundred years of pyloric
22. Zhu J, Zhu T, Lin Z, Qu Y, Mu D. Perinatal risk factors for infantile hypertrophic stenosis in the Royal Hospital for Sick Children Edinburgh. J Pediatr Surg.
pyloric stenosis: A meta-analysis. J Pediatr Surg. 2017;52:1389–1397. 2015;50:280–284.
23. Krogh C, Biggar RJ, Fischer TK, Lindholm M, Wohlfahrt J, Melbye M. Bottle-feed- 56. Rammstedt C. Zur operation der angeborenen pylorusstenose. Med Klin.
ing and the Risk of Pyloric Stenosis. Pediatrics. 2012;130:943–949. 1912;8:1702–1705.
24. Wayne C, Hung JH, Chan E, Sedgwick I, Bass J, Nasr A. Formula-feeding and 57. Tan KC, Bianchi A. Circumumbilical incision for pyloromyotomy. Br J Surg.
hypertrophic pyloric stenosis: is there an association? A case-control study. J 1986;73:399.
Pediatr Surg. 2016;51:779–782. 58. Alain JL, Grousseau D, Terrier G. Extramucosal pylorotomy by laparoscopy. J Pe-
25. Eberly MD, Eide MB, Thompson JL, Nylund CM. Azithromycin in early infancy diatr Surg. 1991;26:1191–1192.
and pyloric stenosis. Pediatrics. 2015;135:483–488. 59. Oomen MWN, Hoekstra LT, Heij HA. Learning curves for pediatric laparoscopy:
26. Almaramhy HH, Al-Zalabani AH. The association of prenatal and postnatal how many operations are enough? The Amsterdam experience with laparo-
macrolide exposure with subsequent development of infantile hypertrophic py- scopic pyloromyotomy. Surg Endosc. 2010;24:1829–1833.
loric stenosis: a systematic review and meta-analysis. Ital J Pediatr. 2019;45:20. 60. Oomen MWN, Hoekstra LT, Bakx R, Ubbink DT, Heij HA. Open versus la-
27. Murchison L, De Coppi P, Eaton S. Post-natal erythromycin exposure and risk of paroscopic pyloromyotomy for hypertrophic pyloric stenosis: a systematic
infantile hypertrophic pyloric stenosis: a systematic review and meta-analysis. review and meta-analysis focusing on major complications. Surg Endosc.
Pediatr Surg Int. 2016;32:1147–1152. 2012;26:2104–2110.
28. Soyer T, Yalcin S, Bozkaya D, Yigit S, Tanyel FC. Transient hypertrophic pyloric 61. Ismail I, Elsherbini R, Elsaied A, Aly K, Sheir H. Laparoscopic vs. Open Pyloromy-
stenosis due to prostoglandin infusion. J Perinatol. 2014;34:800–801. otomy in Treatment of Infantile Hypertrophic Pyloric Stenosis. Front Pediatr.
29. Tutay GJ, Capraro G, Spirko B, Garb J, Smithline H. Electrolyte profile of 2020;21:1–7.
pediatric patients with hypertrophic pyloric stenosis. Pediatr Emerg Care. 62. Harmon CM. Single-site umbilical laparoscopic pyloromyotomy. Seminars in Pe-
2013;29:465–468. diatric Surgery. 2011;20:208–211.
30. Papadakis K, Chen EA, Luks FI, Lessin MS, Wesselhoeft CWJ, DeLuca FG. The 63. Turial S, Enders J, Schier F. Microlaparoscopic pyloromyotomy in children: initial
changing presentation of pyloric stenosis. Am J Emerg Med. 1999;17:67–69. experiences with a new technique. Surg Endosc. 2011;25:266–270.
31. Vinycomb TI, Laslett K, Gwini SM, Teague W, Nataraja RM. Presentation and 64. Turial S, Enders J, Schier F, Santos M. Comparison of a novel technique of
outcomes in hypertrophic pyloric stenosis: An 11-year review. J Paediatr Child the microlaparoscopic pyloromyotomy to circumbilical and Weber-Ramstedt ap-
Health. 2019;55:1183–1187. proaches. J Gastrointest Surg. 2011;15:1136–1142.
32. Macdessi J, Oates RK. Clinical diagnosis of pyloric stenosis: a declining art. BMJ. 65. Ibarguen-Secchia E. Endoscopic pyloromyotomy for congenital pyloric stenosis.
1993;306:553–555. Gastrointest Endosc. 20 05;61:598–60 0.
33. Glatstein M, Carbell G, Boddu SK, Bernardini A, Scolnik D. The changing clinical 66. Kawai M, Peretta S, Burckhardt O, Dallemagne B, Marescaux J, Tanigawa N. En-
presentation of hypertrophic pyloric stenosis: the experience of a large, tertiary doscopic pyloromyotomy: a new concept of minimally invasive surgery for py-
care pediatric hospital. Clin Pediatr. 2011;50:192–195. loric stenosis. Endoscopy. 2012;44:169–173.
34. White MC, Langer JC, Don S, DeBaun MR. Sensitivity and cost minimization 67. Acker SN, Kulungowski AM, Hodges M, Crombleholme TM, Somme S, Par-
analysis of radiology versus olive palpation for the diagnosis of hypertrophic trick DA. Pyloric stenosis–postoperative care on a nonsurgical ward. J Surg Res.
pyloric stenosis. J Pediatr Surg. 1998;33:913–917. 2015;199:149–152.
35. Croitoru D, Neilson I, Guttman FM. Pyloric stenosis associated with malrotation. 68. Clayton JT, Reisch JS, Sanchez PJ, Fickes JL, Portillo CM, Chen LE. Postopera-
J Pediatr Surg. 1991;26:1276–1278. tive Regimentation Of Treatment Optimizes Care and Optimizes Length of Stay
36. Hua L, Shi D, Bishop PR, Gosche J, May WL, Nowicki MJ. The role of UGT1A1∗ 28 (PROTOCOL) after pyloromyotomy. J Pediatr Surg. 2015;50:1540–1543.
mutation in jaundiced infants with hypertrophic pyloric stenosis. Pediatr Res. 69. Graham KA, Laituri CA, Markel TA, Ladd AP. A review of postoperative
2005;58:881–884. feeding regimens in infantile hypertrophic pyloric stenosis. J Pediatr Surg.
37. Teele RL, Smith EH. Ultrasound in the diagnosis of idiopathic hypertrophic py- 2013;48:2175–2179.
loric stenosis. N Engl J Med. 1977;296:1149–1150. 70. Adibe OO, Iqbal CW, Sharp SW, et al. Protocol versus ad libitum feeds af-
38. Markowitz RI. Olive without a cause: the story of infantile hypertrophic pyloric ter laparoscopic pyloromyotomy: a prospective randomized trial. J Pediatr Surg.
stenosis. Pediatr Radiol. 2014;44:202–211. 2013;49:129–132.
39. Hernanz-Schulman M. Pyloric stenosis: role of imaging. Pediatr Radiol. 71. Sullivan KJ, Chan E, Vincent J, et al. Feeding Post-Pyloromyotomy: A Meta-Anal-
2009;39:S134–S139. ysis. Pediatrics. 2016;137:1–11.
40. Iqbal CW, Rivard DC, Mortellaro VE, Sharp SW, St Peter SD. Evaluation of ultra- 72. Castellani C, Peschaut T, Schippinger M, Saxena AK. Postoperative emesis after
sonographic parameters in the diagnosis of pyloric stenosis relative to patient laparoscopic pyloromyotomy in infantile hypertrophic pyloric stenosis. Acta Pae-
age and size. J Pediatr Surg. 2012;47:1542–1547. diatr. 2014;103:e84–e87.
41. Said M, Shaul DB, Fujimoto M, Radner G, Sydorak RM, Applebaum H. Ultrasound 73. Cubas RF, Longshore S, Rodriguez S, Tagge E, Baerg J, Moores D. Atropine: A
measurements in hypertrophic pyloric stenosis: don’t let the numbers fool you. Cure for Persistent Post Laparoscopic Pyloromyotomy Emesis? J Neonatal Surg.
Perm J. 2012;16:25–27. 2017;6:1–4.
42. Cohen HL, Zinn HL, Haller JO, Homel PJ, Stoane JM. Ultrasonography of py- 74. Mercer AK, Phillips R. Question 2: can a conservative approach to the treatment
lorospasm: findings may simulate hypertrophic pyloric stenosis. J Ultrasound of hypertrophic pyloric stenosis with atropine be considered a real alternative
Med. 1998;17:705–711. to surgical pyloromyotomy? Arch Dis Child. 2013;98:474–477.
43. van den Bunder FAIM, Hall NJ, van Heum LWE, Derikx JPM. A Delphi Analysis 75. Lukac M, Antunovic SS, Vujovic D, et al. Is Abandonment of Nonoperative
to Reach Consensus on Preoperative Care in Infants with Hypertrophic Pyloric Management of Hypertrophic Pyloric Stenosis Warranted. Eur J Pediatr Surg.
Stenosis. Eur J Pediatr Surg. 2020;30:497–504. 2013;23:80–84.
44. Zaghal A, El-Majzoub N, Jaafar R, Aoun B, Jradi N. Brief Overview and Updates 76. Wu S, Lin H, Huang F, et al. Efficacy of Medical Treatment for Infantile Hyper-
on Infantile Hypertrophic Pyloric Stenosis: Focus on Perioperative Management. trophic Pyloric Stenosis: A Meta-analysis. Pediatr Neonatol. 2016;57:515–521.
Pediatr Ann. 2021;50:e136–e141. 77. Lauriti G, Cascini V, Chiesa PL, Pierro A, Zani A. Atropine Treatment for Hyper-
45. Dalton BGA, Gonzalez KW, Boda SR, Thomas PG, Sherman AK, St Peter SD. trophic Pyloric Stenosis: A Systematic Review and Meta-Analysis. Eur J Pediatr
Optimizing fluid resuscitation in hypertrophic pyloric stenosis. J Pediatr Surg. Surg. 2018;28:393–399.
2016;51:1279–1282. 78. Owen RP, Almond SL, Humphrey GME. Atropine sulphate: rescue therapy for
46. Lee LK, Burns RA, Dhamrait RS, et al. Retrospective Cohort Study on the Optimal pyloric stenosis. BMJ Case Rep. 2012:2012.
Timing of Orogastric Tube/Nasogastric Tube Insertion in Infants With Pyloric 79. Hayashi AH, Giacomantonio JM, Lau HY, Gillis DA. Balloon catheter dilatation
Stenosis. Anesth Analg. 2019;129:1079–1086. for hypertrophic pyloric stenosis. J Pediatr Surg. 1990;25:1119–1121.
47. Elanahas A, Pemberton J, Yousef Y, Flageole HH. Investigating the use of pre- 80. Aspelund G, Langer JC. Current management of hypertrophic pyloric stenosis.
operative nasogastric tubes and postoperative outcomes for infants with pyloric Semin Pediatr Surg. 2007;16:27–33.
stenosis: a retrospective cohort study. J Pediatr Surg. 2010;45:1020–1023. 81. Hall NJ, Eaton S, Seims A, et al. Risk of incomplete pyloromyotomy and mu-
48. Taghavi K, Powell E, Patel B, McBride CA. The treatment of pyloric stenosis: Evo- cosal perforation in open and laparoscopic pyloromyotomy. J Pediatr Surg.
lution in practice. J Paediatr Child Health. 2017;53:1105–1110. 2014;49:1083–1086.

Downloaded for LAM AN ([email protected]) at Thammasat University from ClinicalKey.com by Elsevier on September
16, 2022. For personal use only. No other uses without permission. Copyright ©2022. Elsevier Inc. All rights reserved.
M.E. Danko, P.T. Evans and J.S. Upperman Seminars in Pediatric Surgery 31 (2022) 151145

82. Costanzo CM, Vinocur C, Berman L. Prematurity Affects Age of Presentation of 88. Hansen TG, Pedersen JK, Henneberg SW, Morton NS, Christensen K. Educational
Pyloric Stenosis. Clin Pediatr (Phila). 2017;56:127–131. outcome in adolescence following pyloric stenosis repair before 3 months of
83. Miyata S, Cho J, Matsushima K, Fower A, Bliss DW. Operative outcomes of in- age: a nationwide cohort study. Paediatr Anaesth. 2013;23:883–890.
fantile hypertrophic pyloric stenosis in patients with congenital heart disease. J 89. McAteer JP, LaRiviere CA, Oldham KT, Goldin AB. Shifts towards pediatric spe-
Pediatr Surg. 2016;51:1755–1758. cialists in the treatment of appendicitis and pyloric stenosis: trends and out-
84. Chalya PL, Manyama M, Kayange NM, Mabula JB, Massenga A. Infantile hyper- comes. J Pediatr Surg. 2014;49:123–127.
trophic pyloric stenosis at a tertiary care hospital in Tanzania: a surgical expe- 90. Evans C, van Woerden HC. The effect of surgical training and hospital character-
rience with 102 patients over a 5-year period. BMC Res Notes. 2015;8:1–6. istics on patient outcomes after pediatric surgery: a systematic review. J Pediatr
85. Feliz A, Holub JL, Azarakhsh N, Bachier-Rodriguez M, Savoie KB. Health dispari- Surg. 2011;46:2119–2127.
ties in infants with hypertrophic pyloric stenosis. Am J Surg. 2017;214:329–335. 91. Hajiran CJ, Hobbs GR, Vona-Davis LC, Nakayama DK. Cost of Hospitalization for
86. Saps M, Bonilla S. Early life events: infants with pyloric stenosis have a Infantile Pyloric Stenosis. Am Surg. 2016;82:E3–E5.
higher risk of developing chronic abdominal pain in childhood. J Pediatr. 92. Carrington EV, Hall NJ, Pacilli M, et al. Cost-effectiveness of laparoscopic versus
2011;159:551–554. open pyloromyotomy. J Surg Res. 2012;178:315–320.
87. Walker K, Halliday R, Holland AJA, Karskens C, Bawawi N. Early developmental
outcome of infants with infantile hypertrophic pyloric stenosis. J Pediatr Surg.
2010;45:2369–2372.

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