329

, : I
Esophageal Atresia and Hypertrophic Pyloric Stenosis:
Sequential Coexistence of Disease (Case Report)
Arthur D. Magilner1 2
Additional congenital anomalies in association with esoph-
ageal atresia and tracheoesophageal fistula (TEE) are well
known. The most commonly associated lesions are other
atresias of the gastrointestinal tract and imperforate anus,
but other combinations of the VATER (vertebral defects, anal
atresia, TEE with esophageal atresia, radial and renal dyspla-
sia) complex are not uncommon [1 ]. Hypertrphic pyloric ste-
nosis complicating the postoperative course of tracheoeso-
phageal fistula is less well known. There have been 35 cases
of this combination reported [2-4]. These have appeared in
the surgical and pediatric literature, with the most recent
reports published in 1 970. Two recent cases of pyloric ste-
nosis after surgery for esophageal atresia and TEE form the
basis of this report.
Case Report
This 4.1-kg boy had corrective surgery for esophageal atresia and
TEF on the first day of life (Fig. 1). The postoperative course was
uneventful, and small feedings were begun on the seventh day after
surgery. The patient was about to be discharged when, on the 18th
day after surgery, he began to regurgitate his feedings. A barium
swallow to evaluate the anastomosis showed no stricture, but there
was marked delay in emptying the stomach. Delayed films demon-
strated the typical appearance of hypertrophic pyloric stenosis (Fig.
2). Surgical correction of the pyloric stenosis was performed, and no
further difficulties with feedings developed.
Discussion
The frequency of additional congenital anomalies in pa-
tients with esophageal atresia and TEE is nearly 50% [2].
W hile the association with other anomalies of the gastrointes-
tinal tract is well known, the occurrence of pyloric stenosis
as a possible complicating factor during the postoperative
period is less well appreciated. The frequency of pyloric
stenosis is approximately one in 400 births. However, in
patients with esophageal atresia and TEE, its frequency has
been reported as ranging from 1 to 1 0% [2-4].
Fig. 1 -Barium-filled proximal esoph-
ageal pouch typical of esophageal atresia.
Fig. 2.-Antral and pyloric changes
charactertistic of hypertrophic pyloric ste-
nosis 18 days after surgery.
Received January 16, 1986; accepted after revision March 11,1986.
1 Department of Diagnostic Radiology. Albert Einstein Medical Center, Northern Division, York and Tabor Ads., Philadelphia, PA 19141 . Address reprint requests
to A. D. Magilner.
2 Department of Radiology, Temple lktiversity School of Medicine, 3400 N. Broad St., Philadelphia, PA 19140.
AJR 147:329-330, August 1986 0361-803X/86/1472-0329 C American Roentgen Ray Society
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330 MAGILNER ET AL. AJR:147, August 1986
W e also saw a second case, which is not detailed in the
current report. In this case, the symptoms of hypertrophic
pyloric stenosis did not appear and a diagnosis was not made
until 6 weeks after the first surgery for esophageal atresia
and TEE.
Most of the literature suggests that pyloric stenosis is an
acquired entity and that its onset usually occurs 3 to 6 weeks
after birth. This, of course, is distinctly different from most
other anomalies associated with esophageal atresia and TEE
that are apparent in the neonatal period.
The combination of delayed onset of symptoms and the
tendency to attribute the vomiting to a postoperative compli-
cation often obscures the real cause of the problem. Pyloric
stenosis should be considered in the evaluation of vomiting
in an infant who has had recent surgery for esophageal atresia
and TEE. In addition to evaluating the esophageal anasta-
mosis and esophageal motility, the stomach, pylorus, and
duodenum should be studied for pyloric stenosis.
REFERENCES
1 . Quan L, Smith DW . The VATER association. Vertebral defects,
anal atresia, T-E fistula with esophageal atresia, radial and renal
dysplasia: a spectrum of associated defects. J Pediatr
1973;82: 104-107
2. Raffensperger J. Gastrointestinal defects associated with trach-
eoesophageal fistula and esophageal atresia. Arch Surg
1970;101 :241 -244
3. Ahmed S. Infantile pyloric stenosis associated with major anom-
alies of the gastrointestinal tract. J Ped Surg 1970;5: 660-666
4. Franken EA, Saldino AM. Hypertrophic pyloric stenosis compli-
cating esophageal atresia with tracheoesophageal fistula. Am J
Surg 1969;1 17:647-649
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