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APSP J Case Rep 2010; Vol. 1 (2)
Intrauterine Intussusception Causing Ileal
Yogesh Kumar Sarin
Department of Pediatric Surgery, Maulana Azad Medical
College New Delhi, India
*Corresponding Author's E-mail address: email@example.com
APSP J Case Rep 2010; 1: 18
Intrauterine intussusception (IUI) is the one of
the rarest recognized causes of jejuno-ileal atresia (JIA). We report on a
15-day old full-term neonate presenting with features of intestinal obstruction,
wherein on exploration, a visible ileo-ileal intussusception resulting in ileal
atresia was found. The relevant literature has been
Intrauterine Intussusception, Jejuno-ileal atresia, Neonatal intestinal
HOW TO CITE
YK. Intrauterine intussusception causing ileal atresia. APSP J Case Rep
IUI has been described as a
rare cause of intestinal atresia. Evans, in a review of almost 1500 cases of
intestinal atresias found only 9 (0.6%) cases due to IUI. However,
the pathophysiology of this condition remains unclear because the diagnosis is
usually made postnatally based on the intra-operative findings of neonatal
IUI as cause of JIA was first recognized by
Chiari in 1888, and then reported for the first time in English literature by
Davis and Poynter in 1922. In 1983, Pavri et al could find less than 30
adequately documented cases of JIA resulting from IUI. An extensive review of
world literature revealed less than 100 cases of this association till date;
only 2 cases reported from India hitherto. Only isolated case reports are
available; though two series from Japan have reported more than 10 cases of JIA
resulting from IUI in the last decade [3-9].
In this report a full-term
neonate who was diagnosed to have JIA resulting from IUI intra-operatively, is
A male neonate, born to a
19-year-old primigravida at full term, of a consanguineous marriage, through
normal vaginal delivery at home by a traditional birth attendant, presented at
15th day of life with bilious vomiting, failure to pass meconium and
progressive abdominal distension since birth. The mother had not received any
antenatal care. The baby was being breast fed all this while and he had only
received some intravenous fluids for few hours before admission. His general
condition was poor. A clinical diagnosis of intestinal obstruction was made, and
confirmed by plain abdominal radiographs [Image 1].
resuscitation, laparotomy was undertaken that revealed adhesions and calcareous
deposits suggestive of meconium peritonitis and type IIIa ileal atresia, 15cm
proximal to ileocecal valve. The distal collapsed ileal segment revealed an
obvious intussusception [Image 2]; a 5 cm long viable ileal segment could be
retrogradely reduced from this segment of ileum. No pathologic lesion as lead
point could be identified. An 8 cm of dilated proximal and 6cm of the distal
ileum was resected and end- to-back anastomosis was performed. Histopathology of
the resected specimen showed transmural inflammation of both proximal and distal
bowel segments. The postoperative period remained stormy including sepsis,
disseminated intravascular coagulation and meningitis. The child however
survived and discharged on 17th postoperative day.
Mid and low JIA is generally considered to result from
intrauterine vascular disruption(s) in a part of the developed intestine
occurring relatively late in gestation. These disruptive intrauterine events may
include volvulus, herniation, constriction, thrombosis of mesenteric vessels and
rarely intussusception. At least on one occasion, both intrauterine volvulus and
IUI have been concomitantly noted in the same neonate with JIA. No local
vascular disruptive event, however, is known to occur in patients with
apple-peel atresia (APA) or multiple atresias (MA). Genetic,
placental, or other causes have been speculated for APA and MA. The exact
incidence of IUI is not known. The underlying IUI has been noted in 0.6 to 25%
cases of JIA reported previously [1,9,10].
IUI has rarely been detected
by prenatal ultrasonography after 25-30 weeks of pregnancy. Isolated
or transient fetal ascites has been suggested as a pointer to IUI. Other
sonographic features described include dilated intestinal loops with high
echogenicity and a "target-like" appearance. Abdominal calcifications may also
be seen. Though there are no reports of fetal therapy once an antenatal
diagnosis of IUI has been made, but an early diagnosis would definitely result
in referral to a tertiary neonatal surgical center and early surgical
intervention for better outcome [2,11,12]. Although survival in the present case
is encouraging, but non-availability of antenatal care and approaching medical
facility so late could only be condemned.
IUI causing JIA is usually
seen in full-term neonates but has been reported rarely in prematurely born
neonates. The condition is considered to occur very late in pregnancy, which is
supported by presence of normal or near-normal sized colon due to passage of
meconium in majority of these neonates and absence of associated anomalies
A definitive preoperative diagnosis may be difficult, if not
impossible. Though the usual presentation is that of neonatal intestinal
obstruction, and features of peritonitis are usually absent, dilated proximal
bowel may perforate and lead to pneumoperitoneum. The diagnosis is usually made
at surgery or more commonly at histopathological examination [7,14].
due to IUI is single, usually of types II and IIIa, and of mid and low
jejuno-ileum. IUI has never been encountered along with high jejunal, APA or
MA. Pathologically, the lesion is identical to classic, idiopathic
intussusceptions as seen in infants and older children; the presence of lead
point such as Meckel’s diverticulum has been reported rarely
IUI and resultant JIA was readily evident as in our case and
in few instances before, so a cause-effect relationship could be established,
but this may not always be the case. In those situations, IUI may be revealed
only on histopathological examination. On histology, usually a polypoid
intussusceptum, with relatively good preservation of the structure of the
intestine, is observed at the obstructed end on the distal side. In few cases
with the markedly necrotic tissue, polypoid lesion was found located apart from
the blind end. Rarely, intussusceptum and intussuscipiens have been seen to be
The present case reaffirms that IUI may occur at
late stage of pregnancy and cause impairment of blood supply to a segment of
intestine leading to its resorption and atresia.
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