Congenital duodenal diaphragm in eight children.

Abstract

Congenital duodenal obstruction (CDO) is a common and usually easy to diagnose cause of intestinal obstruction in the newborn, except when the cause of the obstruction is a duodenal diaphragm. We describe our experience with eight children who had intrinsic duodenal obstruction secondary to a duodenal diaphragm.
The medical records of 22 children with the diagnosis of congenital intrinsic duodenal obstruction were reviewed for age at diagnosis, sex, gestation, birth weight, clinical features, associated anomalies, method of diagnosis, treatment and outcome. Operative findings and procedures were obtained from the operative notes.
Eight of the 22 children (36.4%) had congenital duodenal diaphragm (CDD). In all children, the diagnosis was made from plain abdominal X-ray, which showed the classic double-bubble appearance, and barium meal, which showed duodenal obstruction. Four patients had associated anomalies, including two with Down's syndrome. Intraoperatively, five patients were found to have duodenal diaphragm with a central hole, while the other three had complete duodenal diaphragms. Postoperatively, all patients did well. Six required total parenteral nutrition.
The 100% survival rate among these children is comparable to that in Western countries, and can be attributed to the lack of major associated abnormalities, good perioperative management, and the availability of total parenteral nutrition.

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ORIGINAL ARTICLE
193
Congenital duodenal diaphragm in eight children
Akhtar Nawaz*, MD, FRCSI; Hilal Matta*, MD, FCRS; Alic Jacobsz*, MD, FRCS; Omar Trad**, MD, MBBS;
Ahmed H. Al Salem*, FRCSI, FACS, FICS
and type of duodenal diaphragm as well as the operative
procedures, were obtained from the operative notes.
Results
Of the 22 children with congenital duodenal obstruction
(CDO), 8 (36.4%), 4 males and 4 females, had CDD (Table 1).
All had a full-term normal vaginal delivery except one who had
30 weeks gestation. All presented with bile-stained vomiting.
Six were newborns and all except one did not pass meconium.
The one who passed meconium was found to have a CDO
secondary to a diaphragm with a central hole. Three were
jaundiced and three had upper abdominal distension. Three
were dehydrated and one (patient No. 5, Table 1) had Klebsiella
septicemia at the time of presentation, which necessitated
treatment with antibiotics for 8 days prior to surgery. In all
children, the diagnosis was made from plain abdominal X-
ray, which showed the classic double-bubble appearance, and
barium meal, which showed duodenal obstruction. In three
children, the obstruction was complete (Figure 1), while in the
other five the obstruction was incomplete, with gas present
distally in the rest of the bowel and streaks of contrast material
passed into the intestines (Figure 2). Four (50%) of our
patients had associated anomalies, as shown in Table 1. Two
(25%) had Down’s syndrome.
AB
From the *Department of Obstetrics
and Gynaecology and
**Department of Pathology,
Aga Khan University Hospital
Karachi, Pakistan.
Correspondence to :
Shahnaz Wasti, FRCOG
Department of Obstetrics
and Gynaecology
Khoula Hospital
P.B.NO: 90, Post Code: 116,
Mina Al Fahal,
Sultanate of Oman
Accepted for publication: July 2003
Ann Saudi Med 24(3):193-197
Background: Congenital duodenal obstruction (CDO) is a common and usually easy to
diagnose cause of intestinal obstruction in the newborn, except when the cause of the
obstruction is a duodenal diaphragm. We describe our experience with eight children
who had intrinsic duodenal obstruction secondary to a duodenal diaphragm.
Methods: The medical records of 22 children with the diagnosis of congenital intrinsic
duodenal obstruction were reviewed for age at diagnosis, sex, gestation, birth weight,
clinical features, associated anomalies, method of diagnosis, treatment and outcome.
Operative findings and procedures were obtained from the operative notes.
Results: Eight of the 22 children (36.4%) had congenital duodenal diaphragm (CDD).
In all children, the diagnosis was made from plain abdominal X-ray, which showed
the classic double-bubble appearance, and barium meal, which showed duodenal
obstruction. Four patients had associated anomalies, including two with Down’s
syndrome. Intraoperatively, five patients were found to have duodenal diaphragm
with a central hole, while the other three had complete duodenal diaphragms.
Postoperatively, all patients did well. Six required total parenteral nutrition.
Conclusions: The 100% survival rate among these children is comparable to that in
Western countries, and can be attributed to the lack of major
associated abnormalities, good perioperative management, and the availability of total
parenteral nutrition.
Key words: Congenital duodenal obstruction, congenital duodenal diaphragm, Oman
C
case, however, when the cause of obstruction is a duodenal
diaphragm, which can be missed both preoperatively and
intraoperatively. Preoperatively, the cause can be missed in
patients with a duodenal diaphragm with a central aperture,
where the diagnosis can sometimes be delayed until adult
life; intraoperatively the cause can be missed in patients
with a duodenal diaphragm without an external duodenal
wall deformity or if there is a wind sock abnormality.1,2,3,4
We describe our experience with eight children who had
intrinsic duodenal obstruction secondary to a duodenal
diaphragm, with an emphasis toward an early diagnosis.
ongenital duodenal diaphragm (CDO) is one
of the common causes of intestinal obstruction
in the newborn, and as an isolated lesion, the
diagnosis is usually not difficult. This is not the
Methods
Between 1983 and 1998, 22 children with the diagnosis
of congenital intrinsic duodenal obstruction were treated
at our hospital. Eight were diagnosed with CDD. The
medical records of these children were reviewed for age
at diagnosis, sex, gestation, birth weight, clinical features,
associated anomalies, method of diagnosis, treatment
and outcome. The operative findings, including the site

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CONGENITAL DUODENAL DIAPHRAGM
One patient was 3 1/2-years old at the time of presentation
to our hospital. She had Down’s syndrome and presented
with vomiting, sometimes bile stained, and upper abdominal
distension. She had a laparotomy at another hospital for
intestinal obstruction one year prior to her presentation at our
hospital. The causes of obstruction as well as type of treatment
were not known. A plain abdominal X-ray showed a dilated
stomach and duodenum with a double-bubble appearance, but
air was present in the rest of the bowel. The barium meal showed
incomplete duodenal obstruction, with streaks of contrast
material passing into the intestine. She was operated on and
found to have a duodenal obstruction in the second part of the
duodenum secondary to a duodenal diaphragm, with a central
hole (Figure 3), which was obstructed by date seeds (Figure 4).
Another patient presented at the age of 2 months with
vomiting since birth. The vomiting was on and off and on
many occasions non-bile stained. A plain abdominal X-ray and
barium meal from another hospital were reported as normal.
When repeated, the tests showed a classic double-bubble
appearance indicative of duodenal obstruction, but there was
gas in the rest of bowel. A barium meal showed incomplete
duodenal obstruction with a small amount of barium passed
into the intestines. Intraoperatively, she was found to have
CDO in the second part of duodenum secondary to a duodenal
diaphragm with a central hole.
All our patients were operated on, and intraoperatively, five
were found to have duodenal diaphragm with a central hole,
Figure 1. Upper gastrointestinal study showing duodenal obstruction
secondary to a complete duodenal diaphragm.
Figure 2. Upper gastrointestinal study showing incomplete duodenal
obstruction with contrast material passing distally through a central
hole in a duodenal diaphragm.
while the other three had complete duodenal diaphragms. Six
of our patients had the duodenal diaphragm in the second
part of the duodenum and two had a duodenal diaphragm
in the third part of the duodenum. None of our patients
had a windsock abnormality. All except one had excision of
the duodenal diaphragm and duodenoplasty by closing the
longitudinal incision in the duodenum transversely. Because
of the close proximity of the duodenal diaphragm to the
ampulla of Vater, one patient had a dudeno-duodenostomy.
Another, who had situs inversus, had apendicectomy as
well. Four of our patients had silastic transanastomotic
tubes. In one child, the tube recoiled into the stomach and
was removed. This patient underwent a gastrostomy. In
two patients, pieces of duodenal diaphragms were sent for
histology. The samples revealed duodenal mucosa on both
sides with stunted villi, crypts and Brunner’s glands. In one
sample, there were hemorrhages as well as an infiltrate of
polymorphs, lymphocytes and plasma cells.
Postoperatively, all our patients did well. Six required
total parenteral nutrition. There was no morbidity or
mortality. Hospital stays ranged from 10 to 28 days (mean
20.3 days).
Discussion
The exact incidence of CDO is not known, but it is
reported to occur in between 1:3,000 and 1:10,000
newborns.5,6 In recent years, the survival of neonates with

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CONGENITAL DUODENAL DIAPHRAGM
Figure 3. Upper gastrointestinal study showing a dilated duodenum
that is incompletely obstructed, with contrast material passing
distally through a central hole in a duodenal diaphragm.
Figure 4. Operative photograph of the date seeds that caused obstruction of an aperture in a duodenal diaphragm in a 3-1/2-
year-old girl with Down’s syndrome.
CDO has improved markedly as a result of early diagnosis,
improved perioperative management, including advances
in total parenteral nutrition, and appropriate surgical
techniques. However, prematurity and associated major
congenital anomalies still contribute to mortality.7
Early diagnosis of CDO based on prompt recognition
of symptoms and accurate interpretation of radiological
investigations is important, as delayed diagnosis is known
to be associated with increased morbidity and a prolonged
hospital stay.1 The diagnosis of complete CDO is usually
easy, and in the majority of cases this can be established with
plain film radiographs and upper gastrointestinal barium
studies.6 Although the classic double-bubble appearance
with absent air distally on plain abdominal radiographs
is pathognomonic of CDO, it is not specific, and is also
seen in patients with small bowel volvulus, and sometimes
in patients with tight duodenal stenosis.6 The importance
of differentiating CDO from malrotation, with its risk of
midgut volvulus and gangrene, needs to be emphasized.
A barium meal is helpful in this regard if it shows the
typical coil spring appearance of small bowel volvulus, or
if the duodenojejunal junction is noted as low and to the
right of midline, suggesting malrotation, which can be
confirmed by barium enema.6,8,9 The presence of CDO
with a doublebubble appearance and distal gastrointestinal
air is one of the contributing factors for delayed diagnosis

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CONGENITAL DUODENAL DIAPHRAGM
Table 1. Demographic and clinical features of eight children with congenital duodenal diaphragm.
Birth
weight
(kg)
Vomitting, failure
to pass mecnium,
No
Age at
diagnosis
Sex Gestation Clinical features
Associated
anomalies
Site of
diaphragm
Type of
diaphragm
Treatment
12 daysM36 weeks2.28
jaundice,
dehydration
Vomiting, upper
abdominal
distension,
dehydration
None
3rd part of
duodenum
Complete
diaphragm
Excision +
Duodenoplasty
+ Gastronomy
21 dayM37 weeks1.57None
2nd part of
duodenum
Complete
diaphragm
Excision +
Duodenoplasty
+ Stent
3 2 monthsF38 weeks 2.44
Vomiting
dehydration
None
2nd part of
duodenum
Diaphragm
with a
central
hole
Diaphragm
with a
central
hole
Diaphragm
with a
central
hole
Diaphragm
with a
central
hole
Diaphragm
with a
central
hole
Excision +
Duodenoplasty
+ Stent
4 6 daysF 38 weeks 3.8VomitingSitus inversus
2nd part of
duodenum
Excision +
Duodenoplasty
+ Stent +
Appendectomy
5 39 weeksF39 weeks3.0
Vomitiing,
jaundice,
klebsiella
septicemia
Vomiting, upper
abdominal
distension,
jaundice
None
2nd part of
duodenum
Excision +
Duodenoplasty
6 8 daysM 38 weeks 2.29
Down’s
syndrome
2nd part of
duodenum
Duodeno-
Duodenoplasty
7 3 yearsF38 weeks-
Vomiting, upper
abdominal
distension
Sickle cell
trait, Down’s
syndrome
2nd part of
duodenum
Excision +
Duodenoplasty
82 daysF 30 weeks1.44Vomiting
Polydactly,
Meckel’s
diverticulum
3rd part pf
duodenum
Complete
diaphragm
Excision +
Duodenoplasty
as it may not be possible to differentiate between the various
etiological factors, which include congenital duodenal
stenosis secondary to annular pancreas or Ladd’s bands,10,11
complete duodenal obstruction with the possibility of bifid
pancreaticobiliary duct,12,13 duodenal diaphragm with a central
aperture,4 and duodenal diaphragm with microperforations.14
This was the case in two of our patients with a diaphragm and
a central hole where the diagnosis was delayed. In one patient,
a previous contrast study prior to presentation at our hospital
was reported as normal and the other patient, a 3-1/2-year-old,
had undergone a negative laparotomy for intestinal obstruction
one year prior to presentation. In addition, depending on the
size of the aperture in the diaphragm, the symptomatology
may not be specific or persistent, and not uncommonly, the
patient will have non-bile stained vomiting. The diagnosis in
some patients may be delayed until adult life.2 To obviate this
delay, the possibility of CDD with a central hole must always
be kept in mind, especially in children with repeated attacks of
vomiting even if not bile stained. An experienced radiologist
should evaluate the radiograms.
CDD is a rare and variable cause of CDO. Eustace et al
had only one duodenal diaphragm among 53 (1.9%) neonates
with CDO treated over a 4-year period, which formed 3.7%
of patients with intrinsic duodenal obstruction.6 Al Salem et
al treated five newborns with CDD among 19 (26.3%) with
intrinsic CDO.5 Adeyemi treated six newborns with CDD
among 30 (20%) with CDO, which were 28.6% of 21 with
intrinsic CDO.15 We treated eight (36.4%) children with
CDD among 22 with intrinsic CDO. The reason for the
relatively high frequency of CDD in our series is not known.
The incidence of CDO and associated anomalies is
also variable, ranging from 13%16 to as high as 78%.7 The
association of CDO and Down’s syndrome is well known, with
an incidence of about 30% to 50%,5,17 but a low incidence of
11%18 and a high incidence of 70%7 were also reported. Two
of our patients with CDD (25%) had Down’s syndrome.
Abdominal situs inversus is one of the rare but interesting
anomalies associated with CDO.19 One of our patients
who had duodenal diaphragm with a central hole diagnosed
preoperatively on upper gastrointestinal study and proven
intraoperatively, had abdominal situs inversus with partial
duodenal obstruction.
Although preampullary diaphragms and diaphragms
in the third part of duodenum have been reported, the

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diaphragm was located in the third part of duodenum. Like
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duodenoplasty.1,4 Intraoperatively, the site of the duodenal
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of the duodenal diaphragm should proceed from the lateral
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side, but if this proves difficult then duodeno-duodenostomy
becomes the procedure of choice. For this reason, others
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patients had silastic transanastomotic nasojejunal tubes that
were used for early postoperative enteral feeding. Although
the use of trans-anastomotic feeding tubes is said to lead
to earlier full preanastomotic feeding,24 the tubes are not
without complications, namely difficulty in manipulation
at the time of insertion, recoiling as happened in one of
our patients, and anastomatic breakdown.5 The likelihood
of prolonged ileus in these patients calls for early total
parenteral nutrition.
The survival rate of 100% in our patients is comparable to
that reported from Western centres.7,25 We think the survival
rate is attributable to the lack of major associated anomalies in
our patients and good perioperative management, including
the availability of total parenteral nutrition.
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