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Pyloric stenosis: evolution from pylorospasm?

Article in Pediatric Surgery International · November 1990

DOI: 10.1007/BF00174341 · Source: OAI

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Pediatr Surg Int (1990) 5:425-428
Pediatric
Surgery International
© Springer-Verlag1990

Original article
Pyloric stenosis: evolution from pylorospasm?
John R. Wesley, Michael A. DiPietro, and Arnold G. Coran
Section of Pediatric Surgery,and Sectionof Pediatric Radiology,C. S. Mort Children's Hospital, and the Universityof Michigan Medical School,
Ann Arbor, Michigan, USA

AcceptedMay 15, 1990

Abstract. Over a 10-year period, we have performed py-

loromyotomy on 260 infants with hypertrophic pyloric ste-
nosis (HPS), 10 of whom had a history suggestive of py-
loric stenosis but initially had neither the physical nor
radiological findings to confirm the diagnosis. All 10 de-
monstrated pylorospasm on upper gastrointestinal series
(UGIS), were treated medically without improvement, and
subsequently developed classic HPS confirmed by repeat
UGIS. Age at diagnosis ranged from 3 to 16 weeks (mean
8 weeks). Vomiting was progressively more projectile and
severe from the onset until diagnosis and operation, with a
duration of 5 - 5 0 days (mean 24 days). In 9 of the
10 patients a second UGIS demonstrated the diagnostic
signs of HPS in 8 and suggested an antral web in the 9th.
The interval between the two UGIS ranged from 2 to
46 days (mean 13 days). The 10th patient had a palpable
hypertrophic pyloric muscle 9 days after the first UGIS and
was operated upon without a follow-up UGIS. All
10 patients had classic HPS at operation. We conclude that
although most infants with pylorospasm on UGIS improve
with medical management, a small but significant number
go on to develop HPS. Awareness of this variant of pyloric
stenosis and appropriate follow-up UGIS will help to avoid
undue delay in correctly diagnosing infants with persistent
non-bilious vomiting.

Key words: Pyloric stenosis - Pylorospasm - Evolution

Introduction

Hypertrophic pyloric stenosis (HPS) is the most common

surgical disorder causing vomiting in infancy. Eighty-five
to 90% of patients with a history suggestive of HPS can be
diagnosed by palpation of the hypertrophied pyloric Fig. 1. PatientRN. A At 46 days of age: narrowedand elongatedpyloms
(arrows) and contractedantrum (a), which opened with prompt passage
of barium (D = duodenum): diagnosis pylorospasm.B At 49 days of age:
Offprint requests to: J. R. Wesley,Universityof Michigan F7516 Mott persistently hartowed and elongated pyloric channel with prominent
Children's Hospital, Box 0245, Section of Pediatric SurgeryAnn Arbor, shoulder sign (arrows) and minimalpassage of barium: diagnosispyloric
MI 48 109-0245, USA stenosis
426

Fig. 2. Patient RM. A At 1 month of age: antral-pyloric narrowing (ar- narrow pylorus (arrow) with prominent shoulder (closed arrowheads)
rows) with intermittent good passage of barium into the duodenum (open and tit (open arrowhead) signs; poor gastric emptying: diagnosis pyloric
arrowhead = duodenal bulb; d = duodenal sweep): diagnosis py- stenosis
lorospasm. B At 3 months of age: elongated (here incompletely filled),

they evidenced a form of pylorospasm that later evolved

into classic HPS.

Clinical presentation and methods

Between September 1977 and September 1987, 260 pyloromyotomies

were performed at C. S. Mort Children's Hospital, Ann Arbor. Of this
group, 10 infants (3.8%) had a history typical of pyloric stenosis but on
initial examination had neither the physical nor radiological findings of
HPS. On UGIS all 10 demonstrated either pyloric narrowing without
much hold-up of barium or pylorospasm with intermittent hold-up. They
were treated medically for varying lengths of time with no improvement,
and subsequent UGIS revealed classic HPS. All 10 infants then under-
went pyloromyotomy.

Results

There were 7 males and 3 females ranging in age at diag-

nosis from 3 to 16 weeks (mean 8 weeks). Vomiting was
progressively more severe and projectile from the onset
until diagnosis and operation, with a duration o f 5 - 50 days
(mean 24 days). In 9 of the patients, a second U G I S de-
Fig. 3. Patient JP - 1 month of age, vomiting since birth. UGIS normal; monstrated the diagnostic signs of HPS in 8 (Fig. 1 and 2)
ultrasound showed transient pyloric thickening (arrowheads; one wall and persistent narrowing with only moderate hold-up sug-
{between +'s} = 4 ram) without significant elongation. Diagnosis py- gestive of an antral web in the 9th. The interval between the
lorospasm. Vomiting resolved over 4 weeks. White arrow marks the two U G I S ranged from 2 to 46 days (mean 13 days). The
pyloric lumen. Arrows point anteriorly (A) and rightward (R) for orienta- 10th patient had a readily palpable hypertrophic pyloric
tion muscle 9 days after the first contrast study and was
operated upon shortly thereafter without a follow-up
muscle. The remaining cases can usually be diagnosed by UGIS. All 10 patients had classic HPS at operation and
observing the classic radiological findings on upper gastro- recovered uneventfully from surgery.
intestinal series (UGIS) [11]. More recently, the diagnosis
has been quite accurately made by ultrasonography [2, 8].
Since 1977, however, in spite of the ease of preoperative Discussion
diagnosis, we have cared for 10 infants with a history sug-
gestive o f HPS but with neither a palpable pyloric "olive" The precise etiology of HPS is unknown, and it is still
nor the characteristic changes of HPS on UGIS. Rather, uncertain whether this is an acquired or a congenital lesion.
 427

Most infants develop symptoms 2 to 8 weeks after birth, Pyloric stenosis is most often demonstrated radio-
and cases have first presented as late as 8 months of age graphically by the classic well-known findings of an elon-
[6, 13]. A genetic predisposition may play a role in some gated pylorus producing a string sign and the hypertro-
cases in that several instances of familial involvement have phied pyloric muscle impression on the distal antrum creat-
been reported [1, 3], and pyloric stenosis has occurred in ing the shoulder sign, the pyloric tit, and the beak sign [20].
twins and triplets [9, 14]. However, there are no convincing Less often seen, but equally important to accurate diagno-
data to suggest that, overall, genetics or familial tendencies sis, are radiographic demonstrations of typical or in-
are overriding etiological factors. complete muscle hypertrophy such as the double-track sign
One theory holds that the ganglion cells of the pyloric [7], the lesser curve mass, the spiculated antrum, the funnel
myenteric plexus are abnormal in either number or func- antrum, and the pyloric niche [21]. In addition, there are a
tion [5, 15], however, this is more likely the result of number of patients who have pyloric stenosis with no
degeneration secondary to overactivity and hypertrophy of roentgenographically identifiable hypertrophied muscle
the pyloric muscle than to intrinsic factors [19]. Further- mass but definite short-segment pyloric narrowing and ob-
more, a recent electron microscopic study of myenteric struction [22]. These infants generally have a small muscle
plexus cells in infants with HPS concluded that they were mass at the time of operation that was not readily palpable
normal [10]. There is very little evidence, therefore, to when the patient was awake. Most of these patients re-
implicate abnormalities in the structure or number of gan- spond to pyloromyotomy, but in a few cases so little
glion cells as causative factors in HPS. muscle hypertrophy is present that pyloroplasty may be the
More recent evidence is accumulating that suggests preferred procedure [22]. The etiology of short-segment
that overactivity or prolonged spasm of the pyloric muscle pyloric stenosis with minimal muscle hypertrophy is un-
may be a common pathway by which infants develop HPS. known, but it may represent an early stage of classic HPS
This may be caused by increased sensitivity of the pyloric that, for as yet unknown reasons, smolders along without
ganglion cells to the gastrointestinal hormones secretin and progressing to the usual degree of muscle hypertrophy.
cholecystokinin, which are known to cause both pyloric Having taken into account all of the above possibilities,
muscle spasm and hypertrophy [ 16]. The secretion of these we made the diagnosis of "pylorospasm" in the 10 cases
hormones, in turn, is stimulated by gastric acid secreted by reported, which when followed clinically did not respond
the parietal cells of the stomach, and many infants with to medication or the passage of time, but developed into
HPS have been documented to have increased parietal cell classic pyloric stenosis as confirmed by a subsequent diag-
mass [ 16]. Secretion of excess gastrin and vagal overstimu- nostic UGIS. As physicians and pediatricians consider py-
lation have also been implicated in the etiology of HPS, loric stenosis earlier in infants presenting with non-bilious
both having been shown to induce pyloric muscle spasm vomiting, we may see more cases of HPS in evolution from
and subsequent hypertrophy [4, 11, 12, 18]. Exogenous pylorospasm. The best clinical approach in these patients,
causes of liPS have also been documented, as in the case of based on the experience gained from this series of 10 in-
five newborns who developed vomiting with pylorospasm fants, is to follow the patients until the vomiting resolves or
following the administration of erythromycin estolate, until a definite diagnosis of HPS can be made. Definite
which over a period of days developed into HPS [17]. HPS should be documented either by physical examination
Prolonged pyloric muscle spasm leading to muscular or by repeat diagnostic imaging (ultrasound or UGIS) so
hypertrophy appears to be a common denominator in all of that adequate pyloric muscle hypertrophy is present to
the proposed mechanisms and described causes of HPS, allow the surgeon to perform a standard pyloromyotomy.
both intrinsic and extrinsic. Further support for this being Otherwise, if the operation is done too early in the course
the predominant problem is found in the successful medi- of the disease, a more complicated operation such as a
cal treatment of HPS, which involves placing the pyloric pyloroplasty may be required to alleviate the infant's ga-
muscle at rest with gastric decompression and anticholiner- stric obstruction.
gics [23]. Our experience adds more evidence to the theory that
If, when evaluating an infant with the classic symptoms pylorospasm is an important early finding in the develop-
of HPS, an abdominal mass (pyloric "olive") cannot be ment of HPS. Occasionally infants presenting early may
palpated, then some form of diagnostic imaging is indi- have negative ultrasound or UGIS, and if symptoms persist
cated. During most of the 10 years encompassed by this a repeat study is indicated. Awareness of this early clinical
review, we used the UGIS in this situation. Although we picture in the course of HPS will help avoid undue delay in
have recently been using ultrasound as the first diagnostic correctly diagnosing infants with persistent non-bilious
study when physical examination is equivocal or nonre- vomiting.
vealing (Fig. 3) [2, 8], we continue to use the UGIS if
ultrasound fails to reveal a classic hypertrophic pyloric
olive. The UGIS not only demonstrates the antral, pyloric,
and duodenal anatomy, but also permits evaluation of References
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