Congenital Gastric Outlet Obstruction
By Colin C.M. Moore
Riyadh, Saudi Arabia
9 Two additional cases of congenital gastric outlet
obstruction are presented. A comprehensive review of the
literature was undertaken and as a result a classification
for congenital gastric outlet obstruction is suggested. The
management of the cases reported in the literature has
also been reviewed together with the genetics of pyloric
atresia and associated dermatologic lesions. Guidelines are
given for the management of congenital gastric outlet
obstruction with and without associated, inherited derma-
tologic conditions.
9 1989 by W.B. Saunders Company.
INDEX WORDS: Congenital gastric outlet obstruction;
pyloric atresia; epidermolysis bullosa; antral webs.
O N G E N I T A L G A S T R I C outlet obstruction due
C to pyloric atresia or membraneous antral web
represents less than 1% of all atresias and diaphragms
of the alimentary tract? Cook and Rickham 2 reported
an incidence of 0.003% in a period of 25 years at the
Liverpool Regional Neonatal Centre. After an exten-
sive search of the English, German, and Japanese
medical literature, I found only 104 cases of congenital
gastric outlet obstruction alone, 3 cases of congenital
outlet obstruction associated with esophageal atresia,
and 18 cases of congenital gastric outlet obstruction
associated with epidermolysis bullosa for a total of 125
cases.
CASE REPORTS
Case 1
M.A. was a full-term baby boy with a birth weight of 2.5 kg.
Polyhydramnios was not recorded. Nonbilious vomiting began with
the first feed, and since no pyloric tumor could be felt, the preopera-
tive diagnosis of proximal duodenal atresia was made on the basis of
a plain x-ray of the abdomen, which showed a large gastric bubble
and no air distally.
At operation on the second day of life the pylorus was seen to be
obstructed by a thick web of tissue 1 m m wide. A Heineke-Mikulicz
pyloroplasty and excision of the web was performed. Histologic
examination of the solid web showed smooth muscle with mucosa at
each end (Fig 1F). The patient made an uneventful recovery and 2
years later continues to thrive.
Case 2
R.D. is a 3-year-old boy who for most of his life vomited if fed
normally. His mother therefore fed him small amounts of food at
frequent intervals. He thrived on this regimen and his only symptom
was epigastric fullness after meals; his family complained of the
curtailment of their activities due to his frequent feeds.
Radiographic studies (a barium meal) showed a normal esopha-
gus and a huge stomach that emptied slowly through an apparently
normal pylorus. Gastroscopy showed a large spinnaker-like antral
diaphragm with a small eccentric hole about 2 m m in diameter. At
Journal of Pediatric Surgery, Vol 24, No 12 (December), 1989: pp 1241-1246
laparotomy a gastrotomy showed the antral diaphragm, which was
excised, and the gastrotomy closed (Fig 1H). His postoperative
recovery was uneventful, and a barium meal on the third postopera-
tive day confirmed normal gastric emptying. It was 3 months before
he could tolerate feeds normal for his age, but this was more likely
due to apprehension than organic dysfunction.
The membrane consisted of two layers of gastric mucosa with a
supporting stroma of fibrous tissue.
DISCUSSION
The total number of reported cases of congenital
gastric outlet obstruction due to pyloric atresia or
gastric antral web alone is 106 and of congenital
gastric outlet obstruction with other abnormalities is
21, for a grand total of 127 cases, including the two
reported here.
Classification
Gerber and Aberdeen I have proposed a limited
classification of pyloric atresia. In 1977, Korber and
Glasson 3 reported the first two cases of pyloric atresia
associated with epidermolysis bullosa; a total of 19
such cases have now been reported. 313
Also in 1977, Crowe and Sumner 14 reported a case
of what is now regarded as a pyloric gap atresia type II
(Fig IC) associated with esophageal atresia; in 1980
Friedman et a115 reported a case with almost identical
anatomy. A third case of pyloric atresia with esopha-
geal atresia but without tracheoesophageal fistula has
also been reported]
All these cases indicate a need for a new classifica-
tion, which is suggested in Table 1 and Fig 1.
History and Pathologic Anatomy
The first description of pyloric atresia is attributed
to Calder in 17492; subsequently, in 1828, Crooks 2
described the first pyloric membrane. Wuensche in
1875 z and Neele in 18842 each described solid atresia
of the pylorus. The first incomplete prepyloric mem-
brane was described by Laudener in 1897. 2 Since then
From the Section of Paediatric Surgery, Department of Surgery,
King Faisat Specialist Hospital and Research Centre, Riyadh,
Saudi Arabia.
Address reprint requests to Colin C.M. Moore, MD, Consultant
Paediatric Surgeon, Department of Surgery, King Faisal Specialist
Hospital and Research Centre, PO Box 3354, Riyadh 11211, Saudi
Arabia.
9 1989 by W.B. Saunders Company.
0022-3468/89/2412-0006503.00/0
1241
1242 COLIN C.M. MOORE

Normal Anatomy

Type I
/
Type III ~
(D) Pyloric Gap Atresia (E) Pyloric Septum
(Solid Atresia)

Membrane (G) Pyloric

Membrane 1
Type I Type II

(H) Antral Membrane

(Web) (Wind Sock)~
(I) Antral Membrane ~ _ ~ ~

Fig 1. The pathophysiological classifi-

cation of congenital gastric outlet obstruc-
tion due to pyloric atresia or gastric antral
web.

there have been a number of reports of various forms of
obstruction with the diagnosis of pyloric atresia, gas-
tric web, or membraneous stenosis, v74 Their pathophy-
siology is shown in Fig 1.
Kume et a116 collected 84 cases whose distribution
was pyloric membrane (Fig 1F and G) or solid atresia
(Fig 1E) (67%), pyloric atresia of a gap variety (Fig
IB, C, and D) (27%), antral membrane (Fig 1H and I)
(5%), and antral atresia of the gap type (Fig 1A)
(1%).
antral web, but to date there is no convincing experi-
mental proof. The available evidence v687 favors the
second theory.
Clinical Features
Distribution by sex is about equal) 6'63'64 There is a
high p r o p o r t i o n of i n f a n t s w i t h low b i r t h
weight 16'22'23'29'35"64 and polyhydramnios is reported
commonly (6 1 r 16,21,z4,29,63,64,88
The prominent symptom is nonbilious vomiting

Etiology
The etiology of pyloric atresia is obscure; the
pathoembryogenesis is usually explained by the reca-
distress.
222,3
usually in the first few days of life. There may also be
upper abdominal distention with or without respiratory

A plain x-ray of the abdomen will usually show a

nalization failure theory of Tandler 75 or the fetal large distended stomach with a single gas bubble and
ischemic necrosis theory of Lowe and Bernard. v* Both no air distal to the stomach. If vomiting has been
theories are applicable to pyloric atresia and gastric vigorous, the abdomen may be gasless. In cases of
CONGENITAL GASTRIC OUTLET OBSTRUCTION 1243

Table 1. Congenital Gastric Outlet Obstruction concluded, in concurrence with Bar-Maor, 18that there
Simple Complex is strong evidence to support a genetic determination in
Pyloric Associated with esophageal an autosomal recessive mode. There is further support
atresia for a genetic determination in the literature on the
Solid atresia (septum) Pyloric atresia with gap pyloric atresia-epidermolysis bullosa syndromeY 1
(Fig 1E) Type I (Fig 1B)
Epidermolysis bullosa letalis is a rare autosomal
Type II (Fig 1C)
Atresia with gap
recessive disease. 89 Gedde-Dahl 9~has shown that some
Type I (Fig IB) of the clinical variants of epidermolysis bullosa are the
Type II (Fig 1C) result of nonidentical recessive alleles and that some, if
Type III (Fig 1D) not all, of the several forms of recessive epidermolysis
Membraneous diaphragm
bullosa may be at the same locus. Eighteen cases of
Type I (Fig 1F)
Type II (Fig 1G) pyloric atresia-epidermolysis bullosa syndrome have
been reported since Korber and Glasson's initial report
Antral (1 cm or more proximal Associated with aplasia cutis
to pylorus) congenita
in 1977. 3 Neither pyloric atresia nor epidermolysis
Atresia with gap (Fig 1A) Solid pyloric atresia (Fig 1E) bullosa has been reported as occurring separately in
Membraneous diaphragm families with the pyloric atresia-epidermolysis bullosa
(Fig 1 H and I with opening) syndrome. The coincidental occurrence of these two
Pyloric atresia epidermolysis diseases in so many patients seems unlikely. The
bullosa syndrome existence of this syndrome as a separate clinical entity,
Pyloric with an autosomal recessive genetic determinant, the
Solid (Fig 1E)
pleiotropic manifestation of a single gene, is a most
Atresia with gap
Type I (Fig 1B) attractive explanation.
Type II (Fig 1C) The reports of Crowe and Sumner 14 and Friedman
Membraneous diaphragm et al ~5 in 1977 of two separate cases of pyloric atresia
type I (Fig 1F) (long gap variety) (Fig 1B and C) associated with
esophageal atresia raise the possibility of yet another
antral web (Fig 1H and 1) upper gastrointestinal pyloric atresia-associated syndrome. Furthermore, in
radiographic studies may be helpful. 25'52'6~Bell et a152 1982 Carmi et al 7 reported a case of aplasia cutis
claim that these studies are 90% accurate in the congenita associated with pyloric atresia and esopha-
diagnosis of antral web. The typical appearance of a geal atresia, the product of a consanguineous mating.
web is a thin, membraneous septum projecting into the Aplasia cutis congenita is a rare condition in two
antral lumen perpendicular to its longitudinal axis 1 main forms. One form, confined to the scalp, is inher-
cm or 2 cm proximal to the pyloric canal. Gastroscopy ited as an autosomal dominant gene. 91,92 The second
is also a very useful diagnostic tool in older infants and form affects larger areas and is inherited as an autoso-
children, as in the second case reported here, where the real recessive gene. 93
appearance was of a distended, smooth antrum with an Gedde-Dahl and Lambrecht 94 have shown that
apparent small gastric outlet anteriosuperiorly into families with the pyloric atresia-epidermolysis bullosa
which it was impossible to pass the gastroscope. syndrome are of two ethnic groups, American Indian
Rupture of the stomach in pyloric atresia may occur and Lebanese-Turkish, thus favoring genetic linkage
as early as 12 hours after birth, z3'64 Furthermore, it is between two autosomal recessive genes and not a single
important in cases of antral web to pass a Foley syndrome. The consanguineous mating in the case
catheter from the stomach as far as possible distally reported by Carmi et al 7 supports a linkage between
and then to inflate the balloon and withdraw. Win- the two autosomal recessive genes for aplasia cutis
dsock pyloric membranes (Fig lI) protruding into the congenita and pyloric atresia, particularly since this
duodenum have been reported 68 and would be missed child has a full sibling with only aplasia cutis congeni-
at laparotomy by simple inspection of the gastric ta. However, other reports 3'9"~~favor the opposite view
lumen. that the pyloric atresia-epidermolysis bullosa syn-
drome is a single autosomal recessive disorder.
Inheritance Reports of more cases of these disorders are needed
Familial occurrence of pyloric atresia has been in order to clarify their genetic origins. However, it is
reported, lg'21'39'45"64'66 E1 Shafie et a111 reviewed the clear that parents of a child with pyloric atresia-
literature in 1979 and found 6 1 cases of pyloric atresia. epidermolysis bullosa syndrome have a 1:4 risk of
Fifteen of these occurred in seven families. They recurrence in subsequent children, with autosomal
1244
recessive gene inheritance, be it a single or linked
gene.
The need for genetic counselling and the potential
for prenatal diagnosis must be considered. Fortunate-
ly, epidermolysis bullosa without pyloric atresia can be
diagnosed prenatally by electronmicroscopy of fetal
skin biopsy specimens taken at 18 weeks' gestation. 95
A fetal skin biopsy positive for epidermolysis bull-
osa in a family with known pyloric atresia-epidermoly-
sis bullosa syndrome is an indication that the fetus has
the syndrome. This is particularly likely since there is,
so far, no record of either disorder occurring separately
in families with the pyloric atresia-epidermolysis bull-
osa syndrome. The decision on whether to continue the
pregnancy could be considered in view of the fact that
early recognition can allow the use of newer modes of
treatment for epidermolysis bullosa with phenytoin 96
or hormones. 97 Conversely, since there is only one
survivor over the age of II months 3 in all reported
cases of pyloric atresia-epidermolysis bullosa syn-
drome, this syndrome is very lethal.
Treatment
The treatment of congenital gastric outlet obstruc-
tion is surgical. Preoperative preparation should con-
sist of relief of gastric distention by nasogastric suction
supplemented by lavage with warm normal saline to
reduce the gastritis if there is much mucous or if
feeding has been attempted. Correction of dehydration
and metabolic alkalosis will also be required in most
cases.
Operation is best performed through a supraumbili-
cal transverse muscle-cutting incision. However, the
pathology may not be recognized until the stomach has
been opened unless the lesion is one of the gap atresia
types (Fig 1A, B, C, and D). Passage of a Foley
catheter distally will exclude a windsock diaphragm
(Fig 1I) at this stage.
Simple excision of the membrane is all that is
required in the antral membrane type (Fig 1H and I).
However, the pyloric membrane type I (Fig 1F) or type
II (Fig 1G) will also require a pyloroplasty. A Hei-
neke-Mikulicz pyloroplasty is the procedure of choice,
as in case t reported here, and has good results.
Kadowaki et a166caution against too generous a pyloro-
plasty because of the subsequent risk of bile reflux and
the recent implication of the latter in the pathogenesis
of gastric ulcer. I concur with this view and therefore
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COLIN C.M. MOORE
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In gap type atresia (Fig 1A, B, C, and D) and
pyloric septum (Fig 1E), two types of operation have
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