Atresia of Jejunum and Ileum: Is it the Same Disease?

By H.A. Heij, C.G.M. Moorman-Voestermans, and A. Vos

Amsterdam, The Netherlands

0 A retrospective analysis of 21 patients with jejunal Table 1. Annual Incidence of Jejunal and lleal Atresia
atresia and 24 with iieal atresia showed more differences 1978 to 1997
than similarities between the two categories. The inci- Year Jejunal lieal Total
dence of jejunal atresia appeared to be increasing during
1978 0 6
the period of observation (1978 to 19871. In the past 2 6
1979 3 5
years, many children with jejunal atresia. who belonged to 2(1’)
0 2 2
a set of heterozygotic twins, were admitted to this institu- 1980

tion. The mean birth weight and gestational age in jejunal 1981 0 1 1
atresia were significantly lower than in ileal atresia. The 1982 l(l’) 4 5
majority of jejunal atresias were multiple, whereas most 1983 2llC) 0 2
ileal atresias were single. Antenatal perforation occurred 1984 3 1 4
rather frequently (10 cases) in ileal atresia but only twice in 1985 4(3’) 2 6
jejunal atresia. The postoperative course was more often 1986 312t1 4 7
prolonged in jejunal than in ileal atresia. Mortality was 1987 6(6tl 1 7
Total 21 24 45
higher in jejunal atresia: three deaths, all in apple peel
atresia, versus one death in ileal atresia. An explanation for *Apple peel type atresia.
some of these observations can be found in a difference of tTwins.
compliance of the bowel wall between jejunum and ileum:
the more compliant jejunal wall allows for massive dilata- perforation, one associated with cystic fibrosis. Operative treatment
tion with subsequent loss of peristaltic activity. It is
was undertaken as soon as the diagnosis was made. In all cases a
postulated that because of the many differences, jejunal primary end-to-end anastomosis was performed where necessary
and ileal atresia may be considered as separate diseases. after resection and/or tapering of the dilated proximal loop. The
o 1990 by W.B. Saunders Company.
postoperative course was related to the type of atresia. The children
with a single atresia made a quicker recovery (mean total parenteral
INDEX WORDS: Intestinal atresia. nutrition [TPN] duration, 34 days) than children with multiple
atresias (mean TPN duration, 50 days) or apple peel type (mean

T RADITIONALLY, jejunal and ileal atresia have

been grouped together as intestinal atresia. This
category of atresias has been distinguished from esoph-
ageal, duodenal, and anal atresia by epidemiological
and etiologic characteristics.’ From our experience
with 45 cases of jejunal and ileal atresia as reported
herein, we have reasons to emphasize the differences
between these two types of atresia.
MATERIALS AND METHODS
From 1978 to 1987 we treated 45 newborns with atresia of
jejunum or ileum.
Table 1 illustrates a gradual increase over the years in the number
of cases of jejunal atresia relative to ileal atresia. Obstetric data
demonstrated no specific pattern. Polyhydramnios was recorded in
five cases, three of jejunal and two of ileal atresia. Antenatal
ultrasonography was done with increasing frequency during the
period of observation and had demonstrated signs of atresia in 13
cases.
There was a preponderance of premature births (57%) among the
babies with jejunal at&a. The mean birth weight in this group was
significantly lower than that of children with ileal atresia: 2,244 f
674 g versus 3,032 f 640 g. When the twins in the group of jejunal
atresia are excluded, the difference in birth weights remains signifi-
cant: average birth weight of singletons, 2,380 2 688 g (n = 13);
average birth weight of twins, 2,023 * 587 g (n = 8). The family
history for bowel atresia was negative in all cases.
Table 2 lists the details of 21 children with jejunal atresia. There
were four cases of membranous atresia. Four children had associated
congenital malformations: cystic fibrosis, omphalocele, situs inver-
sus, and malrotation. There were two children with antenatal bowel
TPN duration, 94 days). Also, the need for reoperation was higher in
the group of multiple and apple peel atresias.
Indications for reoperation were anatomical (2) or functional
obstruction (3) of the anastomosis and leakage due to a persistent
membrane more distally (1). Subsequently, all these children made a
good recovery. Mortality occurred in three cases with apple peel
atresia. Two children died with septicemia 7 and 150 days after
surgery. In the latter case, short-bowel syndrome necessitated
long-term TPN, which had led to cholestasis and venous access
problems. A third child died on the first postoperative day due to
persistent fetal circulation. Thus the mortality in type IIIb (apple
peel) atresia was three of 6, or 50%. In the whole group of jejunal
atresia, mortality was three of 2 1, or 14%.
Table 3 presents details of 24 patients with ileal atresia. Eighteen
had single, six had multiple atresias. Five children had membranous
atresias (type I). In five children other congenital malformations
were found: omphalocele (2), gastroschisis (1). malrotation (l), and
urethral atresia with severe pulmonary hypoplasia (1). The latter
child died 11 days after operative correction of the ileal atresia. Ten
children had antenatal ileal perforations with meconium peritonitis,
two of them had cystic fibrosis. Parenteral nutrition was necessary
for a relatively short period in most cases, except for three patients
From the Department of Pediatric Surgery, Free University
Hospital and Emma Children’s Hospital, Amsterdam, The Nether-
lands.
Date accepted: June 14. 1989.
Address reprint requests to H.A. Heij. MD, Department of
Pediatric Surgery, Free University Hospital, PO Box 7057. 1007
MB Amsterdam, The Netherlands.
o 1990 by W.B. Saunders Company.
0022-3468/90/2506-0013$03.00/0

Journal of Pediatric Surgery, Vol 25, No 6 (June), 1990: pp 635-637 635

 HEIJ, MODRMAN-VOESTEAMANS, AND VOS

Table 2. 21 Cases of Jejunal Atresia Table 4. Differences Between Jejunal and lleal Atresia Patients

Tvoe of Atresia Jsjunum Ileum

shgls A&s Peel Multiple Number 21 24

Number 7 6 8 Single atresia 7 18
F/M 314 313 315 Multiple atresias 8 6
Apple peel 6 -
Bwt (a) 2,519 zt 981 1.970 + 657 2,246 f 537
Twins 2 0 6 Twins 8 0
Other congenital Gestational age (weeks) 35 + 5 39 f 2
maifcrmations 1 (CF) 2 1 Preterm delivery (n) 15 5
Antenatal perforation 2 0 0 Birth weight (g) 2,244 f 674 3,032 f 640
TPN(days) 34 (8-65) 94 (7-2 10) 50 (16-102) Antenatal perforation 2 10
Reoperations 0 2 of 4 4 Duration TPN (days) 53 * 45 14 + 8 (n = 21)
Mortality 0 3 0 Mortalitv 3 (14%) 1 (4%)

Abbreviations: F, female; M, male: Bwt, birth weight; TPN, total

parenterel nutrition: CF. cystic fibrosis.

The epidemiological data showed a gradual increase

with a complicated postoperative course. The first exception was a in the incidence of jejunal atresia, both absolute and
child with gastroschisis who had long-term functional obstruction relative to the number of patients with ileal atresia. A
that only gradually resolved after construction of an ileostoma. The high number of children with jejunal atresia in recent
continuity of the bowel was restored at the age of 9 months, The
second exception was a child with cystic fibrosis and meconium
years belonged to a set of heterozygotic twins, but no
peritonitis who needed conversion of a very distal end-to-end twinning occurred among the patients with ileal atresia.
ileoileostomy into an ileocolostomy with good recovery. The third The obstetric data of jejunal atresia patients showed
exception was a boy with an omphalocele who had the same problem. a significantly lower gestational age and birth weight
Table 4 summarizes the differences between patients with jejunal
compared with children with ileal atresia. There was
and ileal atresia. Single atresias were much more often observed in
ileal than in jejunal atresia. The number of discordant twins with no higher incidence of polyhydramnios in the jejunal
jejunal atresia was striking. atresia group to explain these findings.
Birth weight and duration of pregnancy were significantly lower The characteristics of the atresias also showed
among.children with jejunal atresia, .even after correction for the several differences. Jejunal atresias were frequently
twins. Antenatal perforation occurred more frequently in ileal (10
cases) than in jejunal atresia (two cases). The postoperative course in
(67%) multiple as opposed to ileal atresias (25%
jejunal atresia patients was more protracted. The mean duration of multiple). Membranous atresias occurred with equal
TPN in jejunal atresia was 53 days. If the three patients who frequency in both groups. The incidence of multiple
suffered complications are excluded, the mean duration of TPN in atresias in our series is high compared with the
ileal atresia was only 14 days. Mortality rate in jejunal atresia.was
observations of others.2 Although multiple membra-
three of 21 versus one of 24 in ileal atresia.
nous atresias have been ascribed to intrauterine
DISCUSSION inflammations,3 the mesenteric gaps observed in most
In this review of 45 children with intestinal atresia, of these patients suggest a vascular origin of the
we found more differences than similarities between atresias.4
patients with jejunal and with ileal atresia. Antenatal perforation occurred only twice in pa-
tients with jejunal atresia, but was a frequent complica-
tion in ileal atresia. This can be explained by assuming
Table 3. 24 Cases of Neal Atresia
a difference in compliance between jejunal and ileal
Type of Atresia
wall. The jejunum appears capable of massive disten-
Single Multiple
sion in case of atresia, whereas the ileum is never very
Number 18 6 dilated but tends to perforation. It is possible that the
F/M 1117 214
3,035 f 511
less optimal vascularisation of the ileum compared
Bwt (a) 3,178 + 689
Twins 0 0 with the jejunum’ is involved.
Other congenital malformations 3 2 The postoperative course in jejunal atresia was much
Antenatal perforation 7 3 longer than in most cases of ileal atresia. This can be
CF 2 0
explained partly by the difference in bowel wall compli-
TPNfdays) 14 (7-21) 19 (9-40)
(16 pat) (5 pat) ance. The very dilated jejunum has lost its peristaltic
Reoperations 3 2 activity and requires specific measures (like tapering)
Mortalitv 1 0 in order to regain its function. In ileal atresia, where
Abbreviations: F, female; M, male; Bwt, birth weight; CF. cystic the bowel is never distended to the same degree, the
fibrosis; TPN, total parenteral nutrition; pat, patients. function is restored much sooner. Other relevant fac-
ATRESIA OF JEJUNUM AND ILEUM
tots in determining the postoperative course are gesta-
tional age and birth weight.
In conclusion, many differences were found between
children with jejunal and ileal atresias. Jejunal atresia
is characterized in our experience by a rising incidence,
lower gestational age and birth weight, and a high
incidence of nonidentical twinning. There were more
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