Full text of DOI:10.1007/s005950050619

Surg Today (2000) 30:438–440
Jejuno-ileal Atresia in Identical Twins: Report of a Case
Yutaro Matsumoto, Kazuhisa Komatsu, and Tosiharu Tabata
Division of Pediatric Surgery, Iwaki Kyoritsu General Hospital, Uchigo-Mimayacho-Kusehara-16, Iwaki-City 973-8555, Japan
Abstract: Small intestinal atresia occurring in twins is ex-
tremely rare. The aims of this study are to report a case of
identical twins with different types of jejunal atresia, to review
six other pairs of twins, and discuss the etiology. Our case was
the seventh in which both twins were diagnosed to have
jejuno-ileal atresia. Furthermore, they are also only the sixth
and seventh patients known to survive. All the other pub-
lished cases involved identical twins except one. Three pairs
had different types of atresia and four pairs did not have any
other anomalies. In addition, to date there have been no fur-
ther indications of any other members of these families being
affected with intestinal atresia. Therefore, we conclude that
most such cases might be due to environmental influences
during gestation.
tory of either malformations or consanguinity. Neither
any drugs nor dye injection into the amnion had been
administered during pregnancy, which was normal until
30 weeks when polyhydramnios was found. At 34 weeks
5 days of gestation, the mother experienced premature
labor and was referred to our hospital. Upon admission,
intestinal atresia in both twins was suspected by fetal
ultrasound scanning, and the babies were delivered by
cesarean section. There was only one placenta and the
babies were later confirmed to be identical twins by
HLA typing.
Since an abdominal radiograph showed a double
bubble sign, twin A, weighing 1736g, was operated
on the first day of life. Type I jejunal atresia after
Grossfeld’s modified classification2 at 8cm distal to the
ligament of Treiz was found. No other malformations,
including malrotation, were found. A resection of the
membrane and transverse closure of the jejunum were
performed. His recovery was excellent and he weighed
2420g when he was discharged on the 52nd day of life.
On the other hand, the condition of twin B, weighing
1790g, was unstable after birth due to mild heart failure
attributed to atrial septal defect. An abdominal radio-
graph showed a triple bubble sign. Again, jejunal atresia
was suspected. He underwent operation on the fifth day
of life. Multiple jejunal atresias with fibrous cord-like
structures which started from 6cm distal to the ligament
of Treiz and a membranous atresia were found (Type IV
jejunal atresia).2 In this case, as well, no other malforma-
tions including malrotation were found. About 30cm of
the jejunum was resected. Then the colon was mobilized
into nonrotation fashion and tapering jejunoplasty by
GIA (United States Surgical, Norwalk, CT, USA) was
performed. Subsequently, the proximal tapered jejunum
was anastomosed to the distal jejunum, and 75cm of
small intestine and the ileocecal valve were preserved
(Fig. 1). The postoperative course of twin B was ex-
tremely complicated. Moderate heart failure continued,
and wound dehiscence and anastomotic breakdown
Key Words: intestinal atresia, jejuno-ileal atresia, identical
twins
Introduction
Jejuno-ileal atresia occurs about once in every 5000 live
births, but rarely in both members of twins. There are
only six such cases reported in the English literature so
far. In 1994, we briefly reported, in Japanese, on one
such case where identical twins were both affected with
different types of jejunal atresia.1 We herein describe
this case in detail and discuss the etiology of these
anomalies with a review of the literature.
Case Reports
In 1992, male twins were born to a healthy 32-year-old,
primigravida Japanese woman. There was no family his-
Reprint requests to: Y. Matsumoto
(Received for publication on May 31, 1999; accepted on Nov.
11, 1999)

Y. Matsumoto et al.: Jejuno-ileal Atresia in Identical Twins
439
Fig. 1. Schema of the operative
findings and procedures in twin B.
Four cord-like atresias without me-
senteric defect and a membranous
atresia were found. Tapering jejun-
oplasty and mobilization of the colon
into nonrotation were done. In-
cidental appendectomy and jejuno-
jejunostomy were performed
occurred on the seventh day after the operation. There-
after a double-barreled jejunostomy was constructed
and a central venous catheter for total parenteral
nutrition (TPN) was inserted. Six months later reana-
stomosis was completed, and he was discharged at the
age of 8 months weighing 4.0kg. At 6 years of age, he was
operated on again due to intestinal adhesion. At present,
both twins are leading a normal life.
of twins among them. Unfortunately, the details of this
case were not reported. In the same year, Esterly and
Talbert reported on a set of twins, both of whom were
affected with different types of jejunal atresia. They
suggested that the etiology of the defects might be an
intrauterine infection during early gestation.6 Further-
more, Olson et al. reported on identical twins with Type
IV jejunal atresia with malrotation. Their mother ex-
perienced anaphylactic shock at 10.5 weeks gestation.7
Both of the patients survived. Olson et al. speculated
the etiology to be vascular incitement during gestation.
The latest such case was reported by Smith et al. in
1991.8 They reported on a set of twins with apple-peel
type jejunal atresia with malrotation. They concluded
that it might be due to genetic etiology because they
could not find any other antenatal event. Our case is the
seventh pair of twins with jejuno-ileal atresia and the
sixth and seventh case who survived.
In the case of twins, many believe that intrauterine
trauma or circulatory accident during gestation may
cause structural defects such as intestinal atresia.
Esterly and Talbert6 and Olson et al.7 gave detailed
explanations of such cases to support this. In addition,
the data from David, Wagner et al., and Moorman-
Voestermans et al. supported this hypothesis.9–11 On the
other hand, the pair of twins reported by Lewis had
Type I atresia.4 Since Type I atresia may occur through
failure of recanalization, he believed that the etiology of
his case was genetic or hereditary malformations. After
Lewis’s report, the possibility of genetic or hereditary
intestinal atresia was reported by Seashore et al.,
Discussion
The reported cases of jejuno-ileal atresia in twins, writ-
ten in English, including our case are summarized in
Table 1. To our knowledge, only seven sets of twins
have been reported up until the end of 1998. They were
all identical twins except for one. Three pairs had differ-
ent types of atresia and four pairs had no other asso-
ciated malformations. There was no family history of
jejuno-ileal atresia in any of these cases.
Denney and Sloan were the first to report on a set of
identical twins with jejuno-ileal atresia, in 1932.3 Both of
these twins had additional multiple fatal malformations
and both died soon after the birth. Lewis reported on a
second set of twins with ileal atresia in 1953.4 In both
cases, the authors speculated that the etiology might be
a result of genetic rather than environmental influences.
In 1969, DeLorimier et al. reviewed the 10-year experi-
ence of the members of Surgical Section of American
Academy of Pediatrics.5 They collected 613 cases of
jejuno-ileal atresia and stenosis, but found only one set

440
Y. Matsumoto et al.: Jejuno-ileal Atresia in Identical Twins
Table 1. Reported cases of jejuno-ileal atresia in twins
Type of
atresiaa
Highest part
of atresia
Associated
malformations
Identical or
nonidentical
First authorRef.
1 Denney3
Year
1932
Outcome
Type I (?)
Type IIIb
Ileal (?)
Jejunal
Malrotationb
Gastroschisis
Colonic atresia
None
None
None
None
No description
None
None
Identical twins
Died
Died
2 Lewis4
1952
1969
Type I
Type I
Type I
Ileal
Ileal
Identical twins
Identical twins
Died
Died
Survived
Died
No description
Survived
Survived
Survived
Survived
Survived
Survived
3 Esterly6
Jejunal
Jejunal
No description
Jejunal
Jejunal
Jejunal
Jejunal
Jejunal
Jejunal
Type IIIa
No description
Type IIIb
Type IIIb
Type IIIb
Type IIIb
Type I
4 DeLorimier5
5 Olson7
1969
1987
No description
Identical twins
6 Smith8
1991
1994
Malrotation
Malrotation
None
Identical twins
Identical twins
7 Tabata1
Type IV
None
a According to Grossfeld’s modified classification2
b Malrotation was found in both twins
Kimble et al., and Lambrecht et al.12–14 However, their
reported cases were often associated with other
multiple anomalies, and rarely occurred in identical
twins, rarely affected both twins, and characteristi-
cally showed fatal outcomes. In 1994, Cragan et al.
researched the prevalence of small intestinal atresia
among twins and singletons in the United States using
two large databases.15 According to their analysis, of a
total of 760 affected infants, 39 were twins. According to
the two databases, the prevalences among twin infants
were 5.5 and 7.3 per 10000 live births, respectively.
These were significantly higher than the prevalences of
2.0 and 2.5 per 10000 live births among singletons, but
there was no set of twins where both were affected. The
authors concluded that since twins have a higher rate of
small intestinal atresia than singletons, it might be due
to vascular disruption, especially in monozygotic twins.
In our case there were no reported accidents during
gestation, and twin A had typical high jejunal membra-
nous atresia. This might suggest genetic anomalies. But
the twins had different types of jejunal atresia. In addi-
tion, they also had no other anomalies, nor family his-
tory, which does not support this hypothesis. We thus
believe that many of the identical twins with jejuno-ileal
atresia might be affected by environmental factors.
Further investigations into the etiology and long-term
follow-up of these families are therefore called for.
English abstract). Nippon Geka Gakkai Zasshi (J Jpn Surg Soc)
95:709–711
2. Grossfeld JL, Ballentine TVN, Schoemaker R (1979) Operative
management of intestinal atresia and stenosis based on pathologic
findings. J Pediatr Surg 14:366–375
3. Denney ES, Sloan LH (1932) Congenital intestinal malformations
in identical twins. Surg Clin North Am 12:227–240
4. Lewis FJ (1953) Intestinal atresia in identical twins. Surgery
33:121–125
5. DeLorimier AA, Fonkalsrud EW, Hays DM (1969) Congenital
atresia and stenosis of the jejunum and ileum. Surgery 65:819–
827
6. Esterly JR, Talbert JL (1969) Jejunal atresia in twins with pre-
sumed congenital rubella. Lancet 2:1028–1029
7. Olson LM, Flom LS, Kierney CMP, Shermeta DW (1987) Identi-
cal twins with malrotation and type IV jejunal atresia. J Pediatr
Surg 22:1015–1016
8. Smith MB, Smith L, Wells W, Shapira E, Hendrickson M,
Moynihan PC (1991) Concurrent jejunal atresia with “apple peel”
deformity in premature twins. Pediatr Surg Int 6:425–428
9. David TJ (1985) Vascular basis for malformations in a twin. Arch
Dis Child 60:166–167
10. Wagner DS, Klein RL, Robinson HB, Novak RW (1990) Placen-
tal emboli from a fetus papyraceous. J Pediatr Surg 25:538–
542
11. Moorman-Voestermans CGM, Heij HA, Vos A (1990) Jejunal
atresia in twins. J Pediatr Surg 25:638–639
12. Seashore JH, Collins FS, Markowitz RI, Seashore MR (1987)
Familial apple peel jejunal atresia: surgical, genetic, and radio-
graphic aspects. Pediatrics 80:540–544
13. Kimble RM, Harding JE, Kolbe A (1995) Jejuno-ileal atresia.
An inherited condition? Pediatr Surg Int 10:400–403
14. Lambrecht W, Kluth D (1998) Hereditary multiple atresias of the
gastrointestinal tract: report of a case and review of the literature.
J Pediatr Surg 33:794–797
15. Cragan JD, Martin ML, Waters GD, Khoury MJ (1994) Increased
risk of small intestinal atresia among twins in the United States.
Arch Pediatr Adolesc Med 148:733–739
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