CLINICAL CORRESPONDENCE
Figure 3: Microscopic view of the pylorus showing hyperplasia of
Figure 2: Axial CT. Arrow: thickened pylorus.
Discussion
Idiopathic hypertrophic pyloric stenosis
(IHPS) is a disease usually seen in infants.
Adult IHPS (AIHPS) is rare, and was first
described by Jean Cruveilhier in 1835.1 It
is generally classified as either primary or
secondary.
AIHPS presents in adult life without any
apparent cause, and with no history of
infantile vomiting, suggestive of pyloric
stenosis of infancy. Microscopically, there
is total or segmental hypertrophy of the
smooth muscle of the pylorus, without any
identifiable underlying disease.2
Secondary hypertrophic pyloric stenosis
is as a result of other diseases of the upper
gastrointestinal tract, such as peptic ulcer
disease, malignancy and inflammatory
diseases. Microscopically, there is localised
replacement by fibrous tissue, and minimal
or no hypertrophy of the muscularis propria.
The aetiology of AIHPS remains unknown.
Most authors believe it is likely due to the
persistence of a mild infantile form into adult
life.3 Infantile and adult IHPS have a similar
anatomical and histological appearance.
Diagnosis is based upon history, clinical
and radiological findings and endoscopic
appearance. The predominant symptom is
postprandial abdominal pain and distension.
The discomfort tends to be relieved by
vomiting. Weight loss and anorexia are
common. Unlike infantile IHPS, an abdominal
mass is not usually palpable. It may be
mistaken radiologically and endoscopically
for a gastric gastrointestinal stromal tumour,
or a diffuse infiltrating adenocarcinoma given
the normal overlying mucosa.
the muscularis propria.
Endoscopically, the pylorus is fixed,
narrowed and has a smooth border. Its
appearance has been described as the
“cervix sign” by Schuster and Smith4.
Biopsies should always be taken, however
they are frequently normal as the gastric
mucosa is unaffected and therefore submu-
cosal malignancies cannot be excluded.
An endoscopic ultrasound and fine-needle
aspirate or core biopsy may be performed,
predominantly to exclude other submu-
cosal tumours. Biopsies, however, may be
inconclusive and currently there are no
clear guidelines on their use. Dilatation
can be performed, but results are usually
temporary and recurrence high.
Surgery is indicated in the treatment
of AIHPS. Partial gastrectomy,
gastroenterostomy, pyloromyotomy and
pyloroplasty have all been proposed as
treatments.2, 3, 5 In many cases, malignancy
cannot be excluded, therefore gastric
resection with either Billroth I or II
reconstruction may be performed, and is
preferred by most clinicians. Pyloroplasty
is generally favoured over pyloromyotomy,
due to the risk of mucosal laceration and
subsequent diverticulum formation with
pyloromyotomy. Pyloroplasty can be
successfully performed laparoscopically.5
Conclusion
AIHPS is a rare condition with less than
300 case reports in the literature. Its aeti-
ology is unclear, but may be an attenuated
form of infantile pyloric stenosis. It may
be treated endoscopically, however most
patients proceed to surgery and a partial
gastrectomy is preferred by most clinicians.
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NZMJ 1 July 2016, Vol 129 No 1437
ISSN 1175-8716 © NZMA
www.nzma.org.nz/journal
 CLINICAL CORRESPONDENCE

Competing interests:
Nil
Author information:
Simon Richards, General Surgical Registrar, Taranaki Base Hospital, New Plymouth; Glenn
Farrant, General Surgeon, General Surgery, Taranaki Base Hospital, New Plymouth; Gerard
McCarthy, Pathologist, Taranaki MedLab, New Plymouth, New Zealand.
Corresponding author:
Simon Richards, Department of General Surgery, Taranaki Base Hospital, David Street, New
Plymouth, New Zealand.
simon.richards@cdhb.health.nz
URL:
www.nzma.org.nz/journal/read-the-journal/all-issues/2010-2019/2016/vol-129-no-1437-1-
july-2016/6935

REFERENCES:
1. Cruveilhier J. Anato-
mie Pathologique du
Corps Humain. Paris:
Bailliere, 1835.
2. Hellan M, Lee T, Lerner
T. Diagnosis and therapy
of primary hypertrophic
pyloric stenosis in adults:
case report and review of
literature. J Gastrointest
Surg 2006;10:265-9.
3. Graadt van Roggen JF,
van Krieken JH. Adult
hypertrophic pyloric
stenosis: case report and
review. Journal of Clinical
Pathology 1998;51:479-480.
4. Schuster MM, Smith VM.
The pyloric “cervix sign”
in adult hypertrophic
pyloric stenosis. Gastroin-
test Endosc 1970;16:210-1.
5. Danikas D, Geis WP, Gina-
lis EM, et al. Laparoscopic
Pyloroplasty in Idiopathic
Hypertrophic Pyloric
Stenosis in an Adult. JSLS:
Journal of the Society
of Laparoendoscopic
Surgeons 2000;4:173-175.

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NZMJ 1 July 2016, Vol 129 No 1437
ISSN 1175-8716 © NZMA
www.nzma.org.nz/journal