Link to Normal Anatomy
DUODENAL DUPLICATION CYSTS
Link to Duodenal Duplication Cysts
obstruction, including both stenosis and atresia, is the most common intestinal
obstruction in newborns and has an approximate incidence of 1 in 10 000 live
births (8). Most obstructions involve the second and third parts of the
duodenum and are located close to the orifice of the bile duct (9), and may be
associated with numerous etiologies (8-13).
congenital anomalies are found in about 50% of the cases and approximately 30%
have trisomy 21 (8, 10-16). Familial cases of duodenal obstruction have been
reported and a genetic origin has been discussed (8, 14-16).
- Intrinsic atresia
(complete or partial failure if canalization)
- Duodenal stenosis /
- Duodenal web /
- Duodenal duplication.
- Pyloric web or
- Extrinsic compression
from annular pancreas, Ladd's bands, volvulus, preduodenal portal vein
intestinal duplication or contiguous renal mass.
- Duodenum is never normally
- Duodenal obstruction has been
reported as early as 14 weeks gestation (1) but is rarely evident before
20 weeks gestation. The diagnosis of duodenal
obstruction is rarely made early in pregnancy. This is thought to be due
to immaturity of autonomic function resulting in immature gastric emptying (16), Fetal
gastric motility is sporadic and infrequent before 20 weeks of
gestation. Both gastric motility and gastric emptying significantly
increase at around 24 weeks of pregnancy. The mean time of diagnosis
in a recent study (22) was rather late in pregnancy (29+2 weeks) and
only eight of their cases (28%) were diagnosed before 26 weeks. Any
dilatation of duodenum in the second trimester might be abnormal (19) and
serial scans may be valuable (20). It has been proposed that the benefits
of prenatal diagnosis might justify a routine ultrasound examination also
in the third trimester. Even though the diagnosis is rarely made at
routine fetal examination in the second trimester, nine out of 11 cases
(82%) from the unselected population from the Trondheim area were
diagnosed prenatally. Other clinical signs, such as polyhydramnios may
raise the suspicion
Duodenal Atresia – Down Syndrome
Atresia – Second part of duodenum -
Upper GI Series:
- Dilated stomach.
- Obstruction of duodenum at the level of
the proximal third portion.
- Duodenal web at surgery.
- 52-78% have significant
associated anomalies (2,3).
- Cardiac defects -
- Trisomy 21 - 30%.
- Biliary malformations
(biliary atresia, choledochal cyst, gallbladder agenesis).
- Malrotation occurs in
up to 40% of cases.
atresia may be associated with duodenal atresia (7% in one surgical
The combination of both conditions is
associated with several other conditions:
association with duodenal atresia (5).
syndrome (oculodigitoesophago-duodenal syndrome) (6):
Microcephaly, tracheo-esophageal fistula and
(7): Microcephaly-oculo-digito-esophageal-duodenal syndrome
Duodenal atresia and congenital left
sided diaphragmatic hernia (Bochdalek type)
- Petrikovsky BM. First
trimester diagnosis of duodenal atresia. Am J Obstet Gynecol
- Grosfeld JL, Rescorla FJ. Duodenal
atresia and stenosis: Reassessment of treatment and outcome based on
antenatal diagnosis, pathologic variance, and long-term follow up. World J
- Akhtar J, Guiney EJ.
Congenital duodenal obstruction. Br J Surg 1992;79:133.
RJ, Mahour GH. Gastrointestinal anomalies associated with esophageal
atresia or tracheoesophageal fistula. Arch Surg 1979;114(10):1125-1128
T, Mahour GH. Surgical problems in patients with VATER-associated
anomalies. J Pediatr Surg 1984;19(5):550-554.
W, Levi S, Verbelen F et.al. Feingold syndrome: report of a new family and
review. Am J Med Genet 1997;73(1):55-60.
M, Katz M, Cabot SG et.al. MODED:
microcephaly-oculo-digito-esophageal-duodenal syndrome. Am J Med Genet
- Fonkalsrud EW. Duodenal atresia or stenosis. In Bergsma
D, ed. Birth Defects Compendium. New York: Alan R. Liss, 1979: 350
- Boyden EA, Cope JG, Bill AH. Anatomy and embryology of
congenital intrinsic obstruction of the duodenum. Am J Surg 1967; 114:
- Kirillova IA, Kulazhenko VP, Kulazhenko LG, Lazjuk GI,
Novikova IV. Pancreas annulare in human embryos. Acta Anat 1984;
- Merrill JR, Raffensberger JG. Pediatric annular
pancreas: twenty years' experience. J Pediatr Surg 1976; 11: 921-5
- Young DG, Wilkinson AW. Abnormalities associated with
neonatal duodenal obstruction. Surgery 1968; 63: 832-6
- Fonkalsrud EW, de Lorimer AA, Hayes DM. Congenital
atresia and stenosis of the duodenum. A review compiled from the members
of the surgical section of the American Academy of Pediatrics. Pediatrics
1969; 43: 79-83
- Grosfeld JL, Rescorla FJ. Duodenal atresia and
stenosis: reassessment of treatment and outcome based on antenatal
diagnosis, pathologic variance, and long-term follow-up. World J Surg
1993; 17: 301-9
- Young DG, Wilkinson AW. Mortality in neonatal duodenal
obstruction. Lancet 1966; 2: 18-20
- Lawrence MJ, Ford WD, Furness ME, Hayward T, Wilson T.
Congenital duodenal obstruction: early antenatal ultrasound diagnosis. Pediatr
Surg Int 2000; 16: 342-5
- Sase M, Tamura H, Ueda K, Kato H. Sonographic
evaluation of antepartum development of fetal gastric motility. Ultrasound
Obstet Gynecol 1999; 13: 323-6
- Sase M, Nakata M, Tashima R, Kato H. Development of
gastric emptying in the human fetus. Ultrasound Obstet Gynecol
2000; 16: 56-9
- Levine D, Goldstein RB, Cadrin C. Distention of the
fetal duodenum: abnormal finding? J Ultrasound Med 1998; 17: 213-15
- Nelson LH, Clark CE, Fishburne JI, Urban RB, Penry MF.
Value of serial sonography in the in utero detection of duodenal
atresia. Obstet Gynecol 1982; 59: 657-60
- Brace RA, Wolf EJ. Normal amniotic fluid volume changes
throughout pregnancy. Am J Obstet Gynecol 1989; 161: 382-8
H-G. K. Blaas H-G K,. SalvesenKA et.al. Fetal duodenal obstructions: increased risk of prenatal
sudden death. Ultrasound
2002; 20: 439