The condition of Congenital Hypertrophic Pyloric
Stenosis, also commonly called Congenital Pyloric
Stenosis, or Pyloric Stenosis in Infants, may be
shortly defined as "A disease in which symptoms of increasing
pyloric obstruction arise in children under
three months of age" (Parsons and Barling).
In our opinion the full title Congenital Hyper - trophic Pyloric Stenosis is the most desirable in that
it implies the congenital nature of the malady, and
emphasises the muscular hypertrophy of the pyloric
canal so constantly found post mortem and at operation
in these cases.
Our study of Congenital Hypertrophic Pyloris Stenosis
has shown it to be a condition associated with
pathological changes of a constant and well defined
nature. A marked hypertrophy of the pylorus, caused
by local gigantism of the circular muscle fibres constituting
the wall of the pyloric canal, is the most
striking feature of the pathology. We believe that
this circular muscle hypertrophy affects only the pyloric
antrum and canal, and does not occur in the pyloric
ring sphincter. Such hypertrophy of the circular
muscle layer, combined with the protrusion of the
mucosa into the lumen of the pyloric canal, constitutes
a relative obstruction. Our observations have, however,
convinced us that an element of spasm is essential,
if complete obstruction of the pyloric canal is
to occur. The pathological appearances and clinical
features of this condition have led us to support the
view held by John Thomson in regard to the pathogenesis
of Congenital Hyperthrophic Pyloric Stenosis; no
one has yet been able to explain why there is a want
of co- ordination between the sympathetic and parasympathetic
fibres supplying this area, but no doubt the
explanation will emerge as our knowledge of the function
and control of the sympathetic ganglia increases.
There is an impression amongst doctors that Congenital
Hypertrophic Pyloric Stenosis is a rare condition, and
that the clinical diagnosis requires the skill, perception,
and acumen of a paediatric specialist. It is true that careful observation and repeated examination
may be necessary before the four cardinal signs and
symptoms are ascertained, but when present their observation
and significance should at once suggest the
diagnosis to the family doctor. Elaborate clinical
methods of investigation are neither necessary nor desirable
in order to make an accurate diagnosis; the
occurrence of forceful vomiting in a first born, male
infant, under three months of age, is highly suggestive
of the condition and if, in addition, visible gastric
peristalsis can be seen and constipation exists the diagnosis
is almost certain, The palpation of a pyloric
tumour is, in our experience, the least commonly demonstrable
sign; we believe that failure to elicit this
sign is seldom due to lack of perception on the part
of the examiner, but rather to the anatomical position
of the tumour beneath the lower border of the liver.
Consideration of the subsidiary symptoms and signs,
while not essential for clinical diagnosis, will undoubtedly
help to make the diagnosis more certain in
the difficult case. Recent observations on the existence
and. manifestations of alkalosis in this condition
constitute an important advance in our knowledge, by
facilitating diagnosis, and awakening a new consciousness
of the risk of surgery without adequate preoperative
preparation.
The differential diagnosis of IIypertrophic Steno-:
sis from Pyloric Spasm and Duodenal Obstruction should
not present any great difficulty if the different sex incidence, earlier onset, and tendency to hypertonicity
in the former, and the presence of bile in the vomitus
in the latter are borne in mind by the physician.
Turning now to the question of treatment in Congenital
Hypertrophia pyloric Stenosis, we are of the opinion
that the results achieved do not justify the adoption
of medical measures alone in the treatment of this condition.
The optimam chance of recovery for the infant
lies in the closest co- operation between the
physician and the surgeon; the initial stages of treatment
should be medical, so that while the general condition
of the patient is being improved coincident observations
can be made which will ensure the speedy
formation of a definite diagnosis in the physician's
mind. The determination of the psychological moment at
which to operate is a matter which the physician must
decide for himself, aided by his past experience of
these cases; early operation, as soon as a definite
diagnosis has been made and the infant's condition improved,
undoubtedly holds the best promise of a successful
cure . We would strongly urge the desirability of
with
continuation. breast feeding in these cases if at all
possible; early operation to ensure the continuance
of breast feeding during convalescence is a procedure
always attended with the best results. From our limited
experience we have formed the opinion that : umydri.n
is an antispasmodic drug well worthy of a trial, in
the event of careful feeding combined with gastric lavage
proving unsuccessful in diminishing the frequency
of projectile vomiting.
The classical Fredet- Pammstedt operation has now
become the standard method of surgical treatment in
this condition, and has proved to be the simplest and
most efficacious procedure yet devised. The ultimate
success of this operation depends in large measure upon
careful pre -operative preparation, which aims at improving
the :general condition and resistance of the
patient, while also rendering the operation simple in
execution and minimising the serious risk of post -óperative
complications. The adequate administration of
fluid before operation is of considerable importance,
not only does this procedure tend to improve the general
condition of the infant and counteract operative
shock, but it also allays the very real danger of alkalosis
before operation which has recently been stressed
by Maize is and Morris. We believe that blood
transfusion holds pride of place in the pre -operative
measures adopted to compensate for the anaemic, emaciated,
dehydrated state of many of these infants before
operation; the routine adoption of such treatment
would, we believe, very substantially reduce the operative
mortality by putting the infant in the best position
to withstand the shock of operation. Preliminary
gastric lavage and the administration of a mild hypnotic
greatly facilitate the safe and speedy execution
of the operation, by rendering the stomach easy to
handle, and reducing movement to the minimum. In the
presence of these favourable conditions the operative technique is simple in execution and the hazards few,
if the operator. is full;; alive to the danger of perforating
the duodenum or closing the abdomen before
satisfactory haeuostasis has been secured in the operative
field. Once completed the success or failure
of the operation depends in large measure upon the subsequent
nursing of the infant. Small frequent feeds
preferably of breast milk, the supplementary administration
of fluid, and the maximum of sleep in warm surroundings
will go far to ensure a speedy and successful
recovery.
Post -operative complications should be of infrequent
occurrence in the rands of an experienced surgeon,
who has made a careful inspection of the pylorus
before returning it to the abdomen, and has repaired
the abdominal wall to the best of his ability when
closing the wound. A high standard of nursing and the
short time spent in hospital probably explain the fact
that we have not experienced the dread results of enteritis
after operation; this risk can, we believe, be
still further reduced by nursing these cases in an isolation
ward.
Despite the very satisfactory results of surgical
treatment in Congenital Hypertrophie Pyloric Stenosis
the prognosis should always be guarded. At the present
time the lives of many infants suffering from this
condition. are needless lost owing to long delay in
diagnosis, foolhardy experiments with medical treatment,
and a lack of appreciation of the correct time at which to operate. The very low mortality amongst
private cases emphasises the fact that early diagnosis
and prompt treatment affects the mortality very
favourably, and for this reason we hope that in the
future every family doctor will be alive to the possibility
of Congenital Hypertrophic Pyloric Stenosis,
whenever he is called to see an infant with persistent
vomiting, visible gastric peristalsis, and progressive
constipation, and that he will act imrnediately to safeguard the best interests of his patient.
