Keywords
double - duodenum - atresia - abdominal cyst
New Insights and the Importance for the Pediatric Surgeon
Double duodenal atresia is very important to be known.
Introduction
Multiple duodenal atresia is an extremely rare condition that may be either double
or triple. To our knowledge, only 24 papers in the English literature reported this
anomaly. Of these, two patients had triple duodenal atresia and the remainder had
a double duodenal atresia.[1]
Duodenal atresia is thought to occur by failure of embryological recanalization. However,
a multiple atresia is difficult to explain with this theory. Yoshida and Migita described
it as a result of a malrotation that causes a twist and results in pressure necrosis
and atresia in more than one point.[2]
This rare deformity has different presentations ranging from neonatal intestinal obstruction
in case of complete atresia to infantile presentation with incomplete intestinal obstruction.
This is associated with or without a palpable abdominal mass.[3] This anomaly can be associated with other conditions as Zamfir et al reported a
case of double duodenal atresia associated with Cri du Chat (cat cry) syndrome.[4] Also this anomaly may be associated with peptic ulcer.[3]
Case Report
A male newborn (37 weeks of gestation; 2.43 kg) presented with bilious vomiting. Clinical
examination revealed mild epigastric distension with the passage of mucous on digital
rectal examination. A palpable epigastric firm mass measuring 1 × 1 cm was found.
General examination revealed severe respiratory distress due to meconium aspiration
pneumonia with associated signs of sepsis; however, there was no sign of peritonitis.
Meconium aspiration pneumonia was diagnosed after reviewing the records of delivery
that revealed meconium-stained amniotic fluid in association with immediate postnatal
respiratory distress.
Laboratory investigations revealed hypochromic microcytic anemia with moderate leukocytosis
in association with mild hypokalemia and metabolic alkalosis. Hanging abdominal X-ray
showed a hugely distended stomach with a faint double bubble sign and absent gas distribution
in the remaining abdomen as shown in [Fig. 1]. Also, plain X-ray (PXR) chest revealed patches of consolidation and areas of hyperinflation
that raised the suspicion of meconium aspiration pneumonia.
Fig. 1 PXR abdomen standing showing hugely dilated stomach and first part of the duodenum
(double bubble sign).
The mother had an antenatal ultrasound that revealed full stomach in association with
polyhydramnios.
After stabilization of the septic patient, a right supraumbilical explorative laparotomy
was performed. We performed a Kocher maneuver and found a cyst between the first and
the second part of the duodenum as shown in [Figs. 2] and [3].
Fig. 2 Intraoperative double duodenal atresia with duodenal cyst in between.
Fig. 3 Illustration showing the duodenal pathology.
A longitudinal duodenotomy distal to the cyst was performed and the ampulla of Vater
was identified. We advanced a 6-inch French tube through this incision into the distal
intestine with injection of warm saline to exclude further distal intestinal obstructions.
Then transverse duodenotomy proximal to the cyst was applied and we found a duodenal
diaphragm at the proximal end of the cyst causing partial duodenal obstruction; this
diaphragm was resected. Another complete duodenal atresia was found at the distal
end of the cyst that explained the development of this cyst between a proximal partial
obstruction and a distal complete obstruction. This cyst was bypassed by a diamond-shaped
duodeno-duodenal anastomosis between the transverse duodenotomy proximal to it and
the longitudinal duodenotomy distal to it.
Stool was passed on the 5th postoperative day; however, the child still had bilious
reflux, so we performed a contrast passage study, which showed that the contrast passed
to the distal intestine freely. However, with a remaining proximal dilatation the
decision to delay oral intake was taken ([Fig. 4]).
Fig. 4 Postoperative contrast study with the dye passing into the small intestine.
Unfortunately, the baby progressively deteriorated due to the meconium aspiration
pneumonia that was resistant to antibiotic treatment. The condition was associated
with severe uncontrollable sepsis that was progressed to septic shock, disseminated
intravascular coagulation (DIC), and multiorgan failure that ended with death of the
child 15 days postoperatively
Discussion
Double duodenal atresia is an extremely rare condition occurring in 1:5,000 to 1:10,000
live births; Stringer et al published a series of four patients and the postoperative
survival was reported in three cases and only one case died due to the associated
congenital heart disease.[5] Also, along his 20-year experience, Grosfeld and Rescorla described only three patients
with double duodenal atresia.[6]
It usually occurs as a sporadic congenital anomaly and only two reports documented
its presence in siblings.[7]
Besides the previously mentioned presentations, double duodenal atresia may present
postoperatively after the treatment of a first point duodenal obstruction by intestinal
obstruction, perforation, and/or abdominal cyst. This usually develops if the patency
of the distal intestine was not ensured during the first operation.[5]
[8]
In our case, a cystic dilatation between the first and the second part of the duodenum
was found during operative exploration. This is consistent with other similar studies
discussing the same topic like that one belonging to Stringer et al, which was published
in 1992.[5]
Endoscopic incision of double duodenal webs has been advocated by some surgeons as
a new treatment modality in infants presenting with partial double duodenal diaphragms.
However, it is not feasible in newborns because a minimum weight of ∼8 kg is mandatory
for endoscopic intervention as reported by Barabino et al. These authors recorded
a successful endoscopic release of duodenal web in a 11-month-old baby (8 kg).[9] Another study was conducted by Bittencourt et al who documented 9 to 12 months as
a minimum age for endoscopic intervention of infantile duodenal membranes.[10] Finally, an endoscopic dilatation and partial resection of a duodenal web were reported
by Beeks et al in a 15-month-old patient (8 kg).[11]
Reviewing the records of our patient during delivery revealed that delayed cord clamping
was not done in this condition; this may be the cause of hypochromic microcytic anemia.
Our patient died 2 weeks postoperatively from meconium aspiration pneumonia that was
resistant to antibiotic treatment and associated with uncontrollable sepsis. The condition
progressed to septic shock, DIC, and multiorgan failure that ended with death of the
child. Surgical exploration and anastomosis are usually curative in all cases. Mortality
is only known due to other causes, such as associated cardiac malformations or pneumonia
as in our patient.[6]
Conclusion
Double duodenal atresia is a rare condition. Operative detection of one atresia does
not preclude the presence of further distal one, so thorough examination of the distal
intestine with a tube and warm saline injection in case of intestinal atresia is mandatory
to exclude other distal obstructing lesions.
