Jejunal and/or ileal
atresia occurs in about 1 of 3,000 live births and is
believed to be caused by an interruption of blood flow to the affected segment (11).
- Jejunal atresia - the atresias tend to be multiple and the jejunum has the
capacity to dilate considerably before perforation occurs.
- Ileal atresias tend to be
solitary and perforation occurs more readily with very little dilatation,
(11-13). Fetal ascites seen in the setting of
suspected bowel obstruction is likely to be caused by bowel perforation.
- The
position of a dilated bowel loop is a poor predictor of whether or not it
is small or large bowel.
- Focally
dilated, isolated loops of bowel are sometimes seen in utero
in the setting of small bowel atresia and may
mimic cysts (14).
- Ileal atresia may be occult
on early antenatal sonography and to come to
attention only after delivery (15).
- Mechanical obstruction:
- Intestinal atresia (usually due to an in utero
vascular accident, secondary to hypotension, vascular accidents, intussusception, or vascular malformation).
- Intestinal stenosis.
- Intestinal Volvulus.
- Meconium
ileus.
- Non - obstructive causes:
- Megacystis-microcolon-intestinal
hypoperistalsis syndrome.
- Congenital chloridorrhea (1,2)
- Most intestinal obstructive
disorders do not occur until the third trimester (a second trimester scan
is usually normal) (3).
- Multiple interconnecting overdistended bowel loops.
- Individual loops of bowel >15
mm in length or 7 mm in diameter (3,4).
- There is considerable
variability in the appearance of the bowel in third trimester, such that a
fetus with a prenatal diagnosis of intestinal obstruction, based solely on
the presence of bowel dilatation may prove to be normal at birth (5).
- The number of dilated bowel
loops depends on the level of obstruction (the lower the level the greater
the number).
- Polyhydramnios
(more common in higher obstructions).
- Peristalsis in the
obstructed loops confirms that we are indeed looking at bowel.
- High small bowel
obstructions may not be apparent until the late second or third trimester.
- In midgut
volvulus a whirlpool or snail sign is seen in utero (6-8).
The whirlpool sign is produced by the loop of bowel and its accompanying
mesentery and mesenteric vessels that wrap around the superior mesenteric
artery. Color or power doppler
may help, by demonstrating the twisted mesenteric vessels that accompany
the bowel and mesentery. Midgut volvulus and intestinal atresia
often co-exist. Volvulus occurs when a loop of
bowel rotates on an axis, causing an obstruction to the lumen and the
mesenteric vessels. In utero, volvulus may occur when the mesentery is incompletely
affixed to the retroperitoneum (9). The
resultant ischemia leads to atresia of the
associated bowel.
- Complications include bowel
obstruction, perforation and ischemic necrosis due to vascular compromise.
|
|
|
Postnatal
Barium study of Proximal Jejunal Atresia/
Dilated Jejunum and Microcolon
|
|
Jejunal web
|
|
Proximal ileal obstruction – atresia
at surgery.
Etiology
probably vascular
|
Rotational Abnormalities
|
Non Rotation
|
Malrotation
|
Reversed Rotation
|
·
Midgut returns to
abdominal cavity after rotating only 1800 and not 2700.
·
Post-arterial (colonic) limb reenters the
abdominal cavity first instead of last.
|
·
Rotation occurs but is incomplete.
·
Prearterial segment
returns to the abdomen first and is usually in a normal position.
|
·
Postarterial segment
of the midgut returns to the abdomen first.
·
This unwinds the normal counterclockwise
rotation that occurred during the first stage and substitutes a final
clockwise rotation of 900.
|
·
Small bowel on right side of abdomen.
·
Colon
and cecum on left side of abdomen.
|
·
Degree of malrotation
is indicated by position of cecum:
·
Cecum may be on left
side, higher than normal on the right side or in an intermediate position.
|
·
Transverse colon lies behind the duodenum and
is separated from it by the SMA.
|
Malrotation with midgut volvulus
If mesenteric fixation of bowel does not occur in a normal
fashion, nonrotation or malrotation
of bowel has occurred.
Without normal fixation of bowel into position, there is
increased risk of displacement and subsequent bowel ischemia because of
twisting of mesenteric vessels. This can occur at any time beginning in fetal
life and continuing on into adulthood.
- If midgut volvulus occurs in
the fetus, it may be sonographically visible as dilated
loops of bowel (16-18).
- Unfortunately,
it is difficult, if not impossible, to distinguish midgut
volvulus, a surgical emergency, from other nonurgent causes of bowel obstruction.
- Malrotation has been described in fetuses shown to
have large intraabdominal masses, suggesting
that the presence of the mass did not allow normal bowel fixation to occur
(19).
- Multicystic
renal dysplasia.
- Dilated tortuous ureter.
- Normal caliber large bowel
(large bowel has a more peripheral location).
- Langer JC, Winthrop AL,
Burrows RF et.al. False diagnosis of intestinal
obstruction in a fetus with congenital chloride diarrhea. J Pediatr Surg 1991;26:1282-1284.
- Lundkvist
K, Ewald U, Lindgren PG. Congenital chloride diarrhoea: a prenatal differential diagnosis of small
bowel atresia. Acta Pediatr 1996;85:295-298.
- Ogunyemi
D. Prenatal ultrasonographic diagnosis of ileal atresia and volvulus in a twin pregnancy. J Ultrasound Med 2000;19:723-726.
- Nyberg DA, Mack LA, Patten
RM et.al. Fetal bowel - normal sonographic findings. J Ultrasound Med 1987;6:3-6.
- Lau TK, Fung
HYM, Fung TK. Fetal bowel dilatation: Report of
three cases with different outcomes. Aust NZ J Obstet Gynaecol 1997;37:323.
- Pracros
JP, Sann L, Genin G et.al. Ultrasound diagnosis of midgut
volvulus: the whirlpool sign. Pediatr Radiol 1992;22:18-20.
- Shimanuki
Y, Aihara T, Takano H et.al.
Clockwise whirlpool sign at color doppler
US: an objective and definitive sign of midgut volvulus. Radiology 1996;199:261-264.
- Yoo
S -J, Park W, Cho SY et.al.
Definitive diagnosis of intestinal volvulus in utero. Ultrasound Obstet Gynecol 1999;13:200-203.
- Nyberg
DA, Mahony BS, Pretorius
DH. Diagnostic ultrasound of fetal anomalies: text and atlas. St Louis, Mosby-Year Book;1990:356.
- K.L. Moore and T.V.N. Persaud, The digestive system. In: K.L. Moore and
T.V.N. Persaud, Editors, The Developing Human:
Clinically Oriented Embryology ((ed 7).),
Saunders, Philadelphia,
PA (2003), pp.
255–285.
- R.J. Touloukian,
Diagnosis and treatment of jejunoileal atresia. World J Surg 17
(1993), pp. 310–317.
- S. Lyrenas,
S. Cnattingius and B. Lindberg, Fetal jejunal atresia and intrauterine
volvulus—A case
report. J Perinat Med 10
(1982), pp. 247–248.
- O. Bahgat,
M. Lev-Gur and M.Y. Divon,
Prenatal ultrasound diagnosis of intestinal obstruction: A case report. Am
J Perinatol 6 (1989), pp.
324–325.
- A. Kubota, T. Nakayama, T. Yonekura et al., Congenital ileal
atresia presenting as a single cyst-like lesion
on prenatal sonography. J Clin
Ultrasound 28 (2000), pp. 206–208.
- R.B. Parad,
K. Applegate, P.M. Doubilet et al., Occult fetal
bowel obstruction: Ileal atresia
presenting in a newborn infant after normal antenatal sonography.
J Ultrasound Med 14 (1995), pp. 161–163.
- N. Samuel, D. Dicker, D.
Feldberg et al., Ultrasound
diagnosis and management of fetal intestinal obstruction and volvulus in utero. J Perinat Med
12 (1984), pp. 333–337.
- B. Weinberg and E.E. Diakoumakis, Three complex cases of foregut atresia: Prenatal sonographic
diagnosis with radiographic correlation. J Clin Ultrasound 13
(1985), pp. 481–484.
- S.J. Yoo,
K.W. Park, S.Y. Cho et al., Definitive diagnosis of intestinal volvulus
in utero. Ultrasound
Obstet Gynecol 13
(1999), pp. 200–203.
- R. Littlewood
Teele, P.W. Pease and R.S. Rowley, Malrotation in newborns following antenatal diagnosis
of intra-abdominal cyst. Pediatr Radiol 28 (1998), pp.
717–721.