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DOI 10.1007/s00383-017-4196-y
REVIEW ARTICLE
Abstract Despite hypertrophic pyloric stenosis (HPS) pyloromyotomy has remained the treatment of choice ever
being one of the most frequently treated pediatric surgical since its description more than a century ago [1]. HPS is a
conditions, its etiology remains incompletely understood. condition that typically manifests between 2 and 12 weeks of
We review the diagnosis and treatment of this condition age where the pyloric sphincter becomes abnormally thick,
with an emphasis on the evolution of surgical techniques resulting in gastric outlet obstruction. The classic presenta-
that led to laparoscopic pyloromyotomy, the most frequently tion is an infant more than two weeks old with postprandial
performed technique for HPS today. In addition, we review projectile vomiting who typically becomes hungry after-
key developments in the understanding of HPS etiology and ward. The incidence of HPS is 2–4 per 1000 live births with
treatment, including the postulated etiology of work-induced geographical variation [2]. It is commonly seen in temper-
hypertrophy of the pylorus, its association with prokinetic ate countries, but rarely seen in the African population near
macrolide antibiotics, and the emerging role of atropine sul- the equator, suggesting an environmental contribution to the
fate as a medical treatment for HPS or a rescue treatment for etiology [2]. This report will review the historical aspects of
incomplete myotomy. pyloric stenosis followed by a brief overview of its epidemi-
ology, current treatment and possible etiologies.
Keywords Pyloric stenosis · Pyloromyotomy ·
Laparoscopic pyloromyotomy · Macrolide
History
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A B C
Fig. 1 Pyloric dilatation. a Orientation of the gastrostomy. b Coronal section through the stomach depicts the hypertrophied pyloric muscle. c
Hegar dilator inserted through gastrostomy site to forcibly dilate the pyloric muscle
longitudinally, leaving the mucosa intact and suturing the defect transversely, however, the sutures tore out through
muscularis transversely [4] (Fig. 2). This invariably did not the muscle and he instead, decided to cover the defect with
lead to satisfactory results because it did not durably disrupt an omental patch. This omental patching was done 26 years
the pyloric ring and relieve the obstruction. Last, gastroen- before Roscoe Reid Graham of Toronto published his 51
terostomy, a procedure that involves the surgical creation cases of perforated peptic ulcers successfully treated with an
of a connection between the stomach and the jejunum was omental ‘Graham’s patch’ [8]. The patient made a success-
employed to bypass the gastric outlet obstruction; however, ful recovery. Ramstedt performed another pyloromyotomy
there was an unacceptably high mortality rate associated a year later, only this time by incising the pylorus muscle
with this procedure (Fig. 3). It was reportedly first performed longitudinally without covering the mucosal bulge with an
by Dr. W. Abel [6]. omental patch. This operation, now known as Ramstedt’s
In 1910, Sir Harold Jalland Stiles, a British surgeon, pyloromyotomy, has remained unchanged since 1912 [4].
performed the first pyloromyotomy [7]. However, the pro- Before Ramstedt’s operation, most infants with pyloric
cedure came to be known from the German surgeon, Dr. stenosis died in an emaciated state. While the approach to
Conrad Ramstedt, who in 1911 operated on the first case achieve a Ramstedt pyloromyotomy has evolved over the
of pyloric stenosis that he encountered [1]. He proceeded years and is currently most frequently done laparoscopically,
with the intent of performing a pyloroplasty. After incising which was first reported in the literature in 1991 [9], the
the pylorus muscle longitudinally, he attempted to close the pyloromyotomy itself has remained unchanged.
A B C
Fig. 2 Technique of pyloroplasty. a Longitudinal incision over the pylorus to split the muscle. b Stay sutures placed superiorly and inferiorly to
apply traction to facilitate transverse closure. c End result of the pyloroplasty
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Diagnosis
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for anesthesia is serum bicarbonate less than 28 mEq/dL, increased risk of incomplete pyloromyotomy and mucosal
serum potassium more than 3.5 mEq/dL and serum chloride perforation compared to the open approach (Fig. 4) [23, 28].
greater than 100 mEq/dL [20]. It is vital that the metabolic
alkalosis is corrected prior to surgery because it can poten-
tially affect the respiratory drive, thus leading to difficulties Postoperative feeding
with extubation at the end of surgery [24].
The routine use of a nasogastric tube (NGT) preopera- The optimum feeding regimen is yet to be determined with
tively has been debated and is mostly based on surgeon and/ feeding strategies varying widely among different institu-
or institution preference. A recent prospective, randomized tions. In the past, it was believed that fasting up to 24 h
controlled pilot study on the use of a NGT preoperatively postoperatively would decrease the number of emesis based
found no effect on postoperative emesis or length of hospital on early gastric motility studies demonstrating normal gas-
stay, concluding its use as unnecessary [25]. tric peristalsis returning 24 h after surgery [29, 30]. How-
The surgical approach for pyloric stenosis varies; how- ever, that dogma has changed recently with most centers
ever, all current techniques follow the same principle of instituting ad libitum postoperative feeding as the enteral
extramucosal longitudinal splitting of the pylorus muscle, feeding of choice [31]. A recent meta-analysis demonstrated
first introduced by Ramstedt in 1912 [1]. An adequate pylo- that ad libitum feeding decreased the length of stay and
romyotomy entails an incision extending from the vein of decreased time to achieve full enteral feeding when com-
Mayo at the duodenal end to the circular fibers of the stom- pared to a structured feeding regimen [31]. The variation in
ach proximally. Once the mucosal bulge is seen with both feeding protocol is based on a surgeon’s preference and the
edges of the pylorus muscle moving freely either side, an air fear for the development of postoperative emesis. Regard-
leak test can be performed by asking the anesthesiologist to less of the postoperative feeding regimen chosen, postop-
insufflate air via the NGT into the stomach while dripping erative emesis will occur [23, 31–33]. One of the important
saline on the mucosa. aspects of the postoperative managements of HPS patients
The three surgical approaches used today are the right is parental education regarding the self-limiting nature of
upper abdominal transverse muscle-splitting incision, cir- postoperative vomiting.
cumbilical approach introduced by Tan and Bianchi in 1986
and lastly the laparoscopic approach [12]. Various studies
have compared laparoscopic to open pyloromyotomy, but Intraoperative and perioperative complications
both techniques have broadly acceptable similar outcomes
[23]. People who advocate for laparoscopic approach sup- The key intraoperative complications associated with
port it because of a shorter time needed (2.27 h) to achieve pyloromyotomy are mucosal perforation and incomplete
full enteral feeding and an earlier home discharge [26, 27]. myotomy. Mucosal perforation most commonly occurs
The downside to laparoscopic approach is a very small at the duodenal end of the pyloric incision because the
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mucosal lining balloons out quite rapidly and the sero- Etiology
sal surface of the pylorus substantially thins as it transi-
tions to the duodenal bulb. Intraoperative recognition of The exact cause of the postnatal pyloric muscle thickening
mucosal perforation is critical, as unrecognized perfo- characteristic of HPS is unclear, but it is mostly likely multi-
ration can lead to postoperative peritonitis, sepsis, and factorial. Duodenal exposure to acid is known to be a potent
death. Limited perforation can be repaired by simple stimulus of pyloric sphincter contraction. Furthermore, high
suturing, while more extensive perforations should be levels of gastrin have been documented after birth in some
repaired by completely closing the pyloromyotomy, rotat- patients with pyloric stenosis. Thus, the gastrin theory pos-
ing the pylorus ninety degrees, and repeating the pyloro- tulates that the high gastrin levels will promote hyperacidity,
myotomy at this alternate location. Incomplete pyloro- which stimulates the cycle of pyloric sphincter contraction
myotomy is most often caused by insufficient disruption and results in muscle hypertrophy [38]. The acid hypersecre-
of the muscular fibers on the gastric end of the pyloric tion phenomenon has been reported by Heine et al. [39], who
incision. Incomplete pyloromyotomy will cause persistent documented higher histamine-induced gastric acid secretion
vomiting postoperatively and should be considered when in infants with pyloric stenosis before and one week after
these symptoms persist beyond one week. The diagno- pyloromyotomy. In addition, animal studies have induced
sis (or exclusion) of incomplete myotomy is most readily pyloric stenosis in newborn dogs after the administration of
made by an upper gastrointestinal study performed when maternal pentagastrin shortly before birth [40]. The hypoth-
postoperative vomiting persists for more than seven days esis of work-induced hypertrophy of the pylorus muscle is
postoperatively [28]. A study suspicious of incomplete further supported by the association between pyloric ste-
myotomy will reveal delayed gastric emptying of contrast nosis and the use of erythromycin and azithromycin. Both
and a “string sign” as contrast transits the persistently macrolide antibiotics are known to act as prokinetic agents
narrowed pylorus [34]. As mentioned above, vomiting due to their stimulation of motilin receptors in the antrum
may persist postoperatively for up to one week despite and pylorus of the stomach, which promotes peristalsis and
adequate pyloromyotomy due to postoperative pyloric gastric emptying [41]. A sevenfold increase in the incidence
edema or gastric atony due to chronic obstruction [35]. of HPS was reported among newborns that received the anti-
biotic erythromycin for post-exposure pertussis prophylaxis
[42]. More recently, a cohort study found a strong associa-
tion between HPS and infants receiving macrolide antibi-
Non‑operative management otics, especially if received within the first two weeks of
life, resulting in a 30-fold increased risk of HPS [43, 44].
The only accepted non-operative management for the Infant consumption from two weeks to four months of age
treatment of HPS is the use of atropine sulfate. This medi- or maternal consumption during the first two weeks of life
cal management is usually reserved for patients who are also resulted in a threefold increased risk [43]. The strong
deemed unfit to undergo general anesthesia due to severe male predominance has remained a mystery, but one clue
medical co-morbidities. Atropine is a parasympatholytic, is the documentation of preterm male babies having more
due its competitive, reversible antagonistic binding to the acid compared to matched females [45]. Another interest-
muscarinic acetylcholine receptors. It inhibits peristalsis ing observation is that bottle-feeding appears to be inde-
and therefore reduces muscular spasms that are thought pendently associated with HPS with a 4.6-fold increase risk
to lead to muscular hypertrophy of HPS [36]. It is also a compared to infants who were breastfed [46]. Other theo-
very strong inhibitor of meal-induced gastric acid secre- ries include deficiency of nitric oxide synthase containing
tion [37]. This presumably will mitigate the acidic envi- neurons within the thickened pylorus [47]. Nitric oxide is a
ronment and thus prevent pyloric sphincter contraction. It major cellular signaling molecule produced by endothelial
has also been used successfully to treat incomplete pylo- cells that relaxes smooth muscle and acts as a major vaso-
romyotomy as a ‘rescue therapy’ [34]. Takeuchi et al. [36] dilator. Hence, the abnormal pyloric innervation is postu-
revealed in their series of 188 patients treated initially lated to result in inadequate relaxation of the pylorus muscle
with atropine, (intravenous form, n = 80; oral form, n = 8), leading to hypertrophy [48]. This led the investigators to
they had an overall success rate of 78.9% (142/180). The postulate the gene for nitric oxide synthetase, NOS1, as a
reported median number of days needed for successful candidate gene for HPS [49].
medical treatment is between 12 and 14 days [36] with Genetic inheritance for HPS is complex and multifacto-
most recommending intravenous dose of 0.06 mg/kg/day. rial. There is increased concordance between monozygotic
twins, when compared to dizygotic twins, and patients with
pyloric stenosis occasionally have a family history of this
condition. Pyloric stenosis is associated with several genetic
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syndromes, including Apert syndrome, Denys-Drash syn- 3. Hirschsprung H (1888) Falle von angeborener pylorus stenose,
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determined [51, 52]. FOXF1 gene mutations have been asso- 5. Gardner W (1889) A case of Loreta’s operation for dilatation of
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malrotation and duodenal stenosis [53]. A recent study on a 6. Nicoll JH (1904) Congenital hypertrophic stenosis of the pylorus.
From an experience of fifteen cases. Br Med J 2:1148–1149
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Acknowledgements This research received no specific grant from (2011) The changing clinical presentation of hypertrophic pyloric
any funding agency in the public, commercial, or not-for-profit sectors. stenosis: the experience of a large, tertiary care pediatric hospital.
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Conflict of interest The authors declare no conflict of interest. evidence-based review. Surg Clin N Am 92:527–539 vii-viii.
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