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Imaging of Congenital Anomalies of the Gastrointestinal

Arun Kumar G u p t a and B h u v n e s h G u g l a n i

Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India.

A b s t r a c t . The radiological imaging plays a vital role in the evaluatin of patients with congenital anomalies of the
gastrointestinal tract. The evaluation of these patients, most of which present early after birth, frequently requires the use of
various imaging modalities for making the correct diagnosis and planning surgical correction. This article reviews the common
congenital anomalies of the gastrointestinal tract including obstructive lesions, anomalies of rotation and fixation, anorectal
anomalies, and intestinal duplications. The plain radiograph is often diagnostic in neonates with complete gastric of upper
intestinal obstruction and further radiologic evaluation may be unnecessary. An upper gastrointestinal series should be
performed in all patients with incomplete intestinal obstruction. Sonography is useful in the evaluation of many congenital
anomalies affecting pediatric gastrointestinal tract especially hypertrophic pyloric stenosis, enteric duplication cysts, midgut
malrotation, meconium ileus and meconium peritonitis. Moreover, CT and MRI has assumed a greater importance as these
provide excellent anatomic details which may be necessary for correct diagnosis as well as treatment planning. This is
particularly true in evaluation of congenital anomalies such as esophageal/enteric duplications, vascular rings and anorectal
anomalies. It is important to be familiar with the role nad usefulness of the various imaging modalities so that these can be used
judiciously to avoid unnecessary radiation exposure while minimizing the patient discomfort.
[Indian J Pediatr 2005; 72 (5) : 403-414]

Key words : GIT; Congenital anomalies; Intestinal abnormalities

A wide spectrum of congenital anomalies m a y affect Attributed to in utero vascular (ischemic) complication
gastrointestinal tract, some of which manifest early after 9 Jejuno-ileal atresia
birth while others may not present till late childhood or 9 Colonic atresia or stenosis
a d u l t h o o d . Imaging plays a very useful role in these 9 Complicated meconium ileus
d e v e l o p m e n t a l lesions, most of which p r e s e n t with
o b s t r u c t i o n . In m o s t cases, plain r a d i o g r a p h y ,
gastrointestinal contrast study or Ultrasound (US) suffice. 9 Meconium plug syndrome and its variants
C o m p u t e d t o m o g r a p h y (CT) and Magnetic Resonance 9 Megacystis-microcolon-intestinal hypoperistalsis
Imaging (MRI) are increasingly proving more useful to
provide an accurate diagnosis specially in difficult cases. Structural and Functional Combined
Developmental lesions of the neonatal gastrointestinal 9 Hypertrophic pyloric stenosis
tract can be grouped as follows: 1 9 Midgut vovulus (complicating midgut
9 Uncomplicated meconium ileus
Attributed to embryologic maldevelopment 9 Colonic aganglionosis
9 Esophageal atresia with or without fistula
9 Antro-pyloric atresia In a n o r m a l n e o n a t e , s w a l l o w i n g b e g i n s a l m o s t
9 Antral diaphragm immediately after birth and gas should be present in the
9 Duodenal atresia stomach within few minutes. 3Within 3 hours of birth, the
9 Duodenal stenosis entire small bowel usually contains gas while the sigmoid
Intrinsic: windsock duodenum colon is seen only after 8-9 hours. 4 Disruption of this
Extrinsic: annular pancreas c o m m o n pattern is seen in obstruction or presence of
9 Midgut malrotation with peritoneal bands underlying illness such as brain damage, septicemia or
9 Duplication or mesenteric cyst hypoglycemia.
9 Anorectal atresia Plain r a d i o g r a p h is a useful, s i m p l e and m o s t
inexpensive tool in the evaluation of the neonate with
Correspondence and Reprint requests : Dr. Arun Kumar Gupta,
Professor, Department of Radiodiagnosis, All India Institute of gastrointestinal (GI) obstruction. Unlike adults and older
Medical Sciences,New Delhi-110029.Fax : 91-26588641,26588663 children, in neonates the small and large bowel usually

Indian Journal of Pediatrics, Volume 72--May, 2005 403

Arun Kumar Gupta and Bhuvnesh Guglani

cannot be distinguished. The gas is distributed omental cysts}

throughout the small and large bowel where a little fluid US is the modality of choice for prenatal screening, but
is present resulting in sharp bowel-air interfaces which occasionally additional imaging information is needed. 6
appear as multiple closely a p p o s e d r o u n d e d or MRI is being increasingly used for prenatal imaging of
polyhedral structures on plain radiograph. 2 The precise congenital anomalies. The use of fast sequences like single
level of obstruction may be evident in high GI obstruction shot fast spin echo and echoplanar imaging has enabled
but is difficult to determine in a low gut obstruction. High successful prenatal MR imaging. Esophageal, duodenal,
GI obstruction occurs most commonly at the level of or small bowel atresia can be diagnosed on antenatal MRI.
duodenum and proximal jejunum and plain radiography On MRI, the signal intensity differences of the dilated
alone is often diagnostic. bowel may provide additional information comple-
Bilious vomiting indicates obstruction distal to the mentary to the US in identifying the site of obstruction.
ampulla of Vater. Although there is little role of contrast The proximal small bowel (fluid content) appears
examintion in high gut obstruction, 5 GI contrast hyperintense on single-shot fast spin echo (SSFSE) and
examination should be performed in obstructions hypointense on T l - w e i g h t e d fast spin echo (FSE)
presenting beyond first few days of life to rule out imaging. In contrast, the distal small bowel and colon
malrotation and midgut volvulus. The stomach is appear hypointense on SSFSE and hyperintense on T1-
emptied through a nasog'astric tube before upper GI weighted FSE imaging due to presence of meconium. 7~
contrast study is undertaken.
Barium suspension is not used in cases of suspected ESOPHAGUS
perforation or if there is a risk of barium inspissations.
Aspiration into lungs shoufd be avoided while using Esophageal Atresia (EA) and Tracheo-Esophageal
contrast agents because commonly used high osmolality fistula (TEF)
ionic contrast may produce severe pulmonary edema. The presence of polyhydramnios, reduced intrahiminal
Hypertonic water-soluble ionic contrast media may be liquid in the fetal gut and inability to detect the fetal
useful in relieving obstruction in meconium ileus by stomach on prenatal ultrasound may provide an early
drawing water into the bowel lumen. However, this may clue to the possibility of esophageal atresia. 9
cause fluid and electrolyte imbalance. Therefore, infant The chest radiographs (AP and Lateral) demonstrate
should be hydrated and serum electrolytes monitored proximal esophageal pouch filled with air. The abdomen
before the procedure. Non-ionic low osmolality contrast is always included in the radiograph to look for air in the
media are preferred in most circumstances. Barium is gastrointestinal tract. The absence of gas in stomach or
preferred in cases of suspected Hirschsprung's disease or intestinal tract is suggestive of pure esophageal atresia
other conditions where delayed films have diagnostic (EA) without fistula or EA with proximal tracheo-
value. esophageal fistula. The position of aortic arch should be
High Intestinal Obstruction: The usual presentation is conveyed to the surgeon as it may help determine the
vomiting which may be bile stained if obstruction is distal surgical approach. When plain radiographs fail to
to ampulla of Vater. The plain radiograph is often indicate the side of the arch, and as a preoperative work-
diagnostic and further radiological evaluation may be up, echocardiography should be performed before
unnecessary. However, an upper gastrointestinal series surgery to prognosticate and plan successful outcome} ~
must be performed in all patients with incomplete Chest radiograph may show consolidation due to
intestinal obstruction. The causes of high obstruction aspiration pneumonia especially in the dependant upper
include pyloric atresia, duodenal atresia, malrotation with lobes in supine position.
midgut volvulus or Ladd's bands and proximal jejunal Radiographically, there is inability to pass a red rubber
atresia. 2 Partial obstruction results from jejunal stenosis, catheter into the stomach. The proximal pouch may be
peritoneal bands, duplication cyst, malrotation and outlined due to swallowed air (Fig la). Gentle injection of
Meckel's diverticulum. 4 air via the tube may distend the proximal pouch. The
Low Intestinal Obstruction: Failure to pass meconium in distance between proximal and distal esophageal pouch
the first 24-48 hours of life may be due to structural or can be assessed only after a gastrostomy in a staged
functional reasons. The causes include ileal or colon procedure. Isolated long gap esophageal atresia is known
atresia, Anorectal malformations, Hirschsprung's disease, to be associated with 13 pair of ribs} 2
meconium plug syndrome, and neonatal small left colon The diagnosis is frequently delayed in H-type fistula.
syndrome. The fistula is more precisely termed as N-type fistula as it
Ultrasonography (US) is often the first modality to be courses obliquely from the esophagus upwards to the
used in investigation of child with abdominal lump or trachea (Fig lb). Most H-fistulas are at thoracic inlet (T2-
suspected hypertrophic pyloric stenosis. US is highly 3). Tube esophagogram under fluoroscopic guidance with
accurate in the diagnosis of hypertrophic pyloric stenosis patient in the lateral or steep prone oblique position is the
and extremely useful in the investigation of mass lesions best way to demonstrate H-type fistula. The nasogastric
such as enteric duplication cysts and mesenteric or tube placed at GE junction is gradually withdrawn while

404 Indian Journal of Pediatrics, Volume 72--May, 2005

Imaging of Congenital Anomalies of the Gastrointestinal Tract

Fig lb. H-type tracheoesophageal fistula. The contrast study

demonstrates the superiorly angulated fistula (arrow) from
the oesophagus to the trachea.

up radiographs may be obtained if initial findings are

Associated anomalies are f r e q u e n t l y seen in such
individuals including features of the VACTREL spectrum
(vertebral anomalies, anorectal malformation,
cardiovascular malformation, tracheo-esophageal fistula
w i t h e s o p h a g e a l atresia, renal a n o m a l i e s a n d limb
defects). The other recognized associations are trisomy 18,
trisomy 21, CHARGE syndrome, Potter syndrome and
presence of 13 pair of ribs. 3,15
Fig la. Oesophageal Atresia with distal Tracheosophageal fistula. The dilatation of upper pouch may persist for months
Lateral radiograph shows air filled dilated proximal f o l l o w i n g r e p a i r of atresia. P a t i e n t s w i t h r e p a i r e d
oesophageal pouch. The presence of air in the stomach and esophageal atresia show a high incidence of abnormal
the bowel indicate distal tracheosophageal fistula.
peristalsis in the e s o p h a g u s , w h i c h m a y cause
d y s p h a g i a . 3,16 A n a s t o m o t i c s t r i c t u r e s a n d gastro-
injecting contrast at various levels? ~ A small amount of esophageal reflux occur c o m m o n l y after EA and TEF
isotonic non-ionic contrast medium or properly diluted repair. The incidence of stricture increases in patients with
barium should be used. The contrast should be aspirated long gap EA with a delayed primary repair 17 and reflux.
back immediately after the study.
Esophageal stenosis and webs may be associated with STOMACH
tracheoesophageal fistula? 3,14Up to one quarter of patients
w i t h EA & TEF h a v e associated w i t h GI a n o m a l i e s Gastric Atresia, Antro-pyloric Membranes: Congenital
including pyloric stenosis, duodenal/small bowel atresia, gastric o b s t r u c t i o n is rare~ Isolated pyloric atresia,
anorectal malformations. There should be high index of p y l o r i c / p r e p y l o r i c m e m b r a n e , or antral webs are
suspicion for associated anomalies. The pattern of air uncommon causes of gastric obstruction in the newborn.
distribution on plain radiographs may provide clue to Pyloric atresia is classified into three types: (a) complete
these anomalies. Air confined to the stomach and or atresia with no connection b e t w e e n the stomach and
d u o d e n u m suggests associated duodenal atresia. Follow d u o d e n u m , (b) complete atresia with a fibrous b a n d

Indian Journal of Pediatrics, Volume 72--May, 2005 405

Arun Kumar Gupta and Bhuvnesh Guglani

connecting the stomach and duodenum, (c) and a gastric

membrane or diaphragm2 8,19The congenital antro-pyloric
webs can be diagnosed on UGI contrast study where
they are seen as linear defects resulting in' pseudo double
bulb' appearance, as the inflowing barium outlines the
space between the antrum and pylorus first and then the
duodenal bulb. 2~The membrane may be detected on
sonography if the stomach is filled with clear fluid.
Congenital Hypertrophic Pyloric Stenosis (CHPS):
CHPS is characterized by hypertrophy of the circular
muscles. Recent work suggests that impaired neuronal
nitric oxide synthase synthesis in the myenteric plexus is
an important contributing factor in the pathogenesis of
infantile hypertrophic pyloric stenosis as well as of
achalasia, diabetic gastroparesis, Hirschsprung's disease,
and Chaga's disease. 21 The mainstay of diagnosis remains
palpation of the hypertrophied pylorus in a quietly
feeding baby. Ultrasonography is the primary imaging
method, if required, and has replaced contrast evaluation.
There is controversy over exact measurements of the
Fig 2b. Hypertrophicpyloric stenosis (HPS): On tranverse view,
gastric outlet in normal and hypertrophic pylorus. The hypertrophied pylorus has a doughnut appearance. The
thickness of the muscle is the most discriminating and mucosa is echogenicwith surroundinghypoechoicmuscle
accurate criterion for the diagnosis of hypertrophic layer (betweencursors).
pyloric stenosis. 22
Ultrasonographically the antro-pyloric muscle of each pylorospasm may overlap those accepted as positive for
patient is measured in the midlongitudinal plane (Fig 2a). hypertrophic pyloric stenosis. In pylorospasm, there is
The stomach is usually well distended allowing easy considerable variability in measurement or image
identification of the antro-pyloric region. The water or appearance with time during the s t u d y . 24 This is an
glucose solution may be given in cases of inadequate important clue for diagnosing pylorospasm. The
distention but should be removed after examination to sonographic double-track sign can be seen in cases of
prevent vomiting and aspiration. The antro-pyloric pylorospasm as well as hypertrophic pyloric stenosis. It is
muscle less than 2 mm thick should be considered not pathognomonic for hypertrophic pyloric stenosisY
unequivocally normal. 23The muscle thickness of 3 mm or
A barium study may be performed if ultrasound is
more is abnormal (Fig 2b) and diagnostic for pyloric inconclusive or if gastro-oesophageal reflux is suspected
stenosis, whereas muscle thickness from 2mm to less than (Fig 2c). The hypertrophied muscle mass causes
3 mm is abnormal but not specifically diagnostic for elongation and narrowing of pyloric canal ('string sign')
pyloric stenosis. 24 The sonographic measurements in as well as a bulge in the distal antrum with streak of

Fig 2c. Hypertrophicpyloricstenosis(HPS):Upper GIbariumstudy

Fig 2a. Hypertrophicpyloricstenosis (HPS):Longitudinalview on shows narrowed and elongated pyloric canal. The
sonographyshows elongatedpyloriccanal w~h thickened hypertrophied muscle mass produces indentation on the
hypoechoeicmuscle (arrow). duodenal cap and the antrum.

406 Indian Journal of Pediatrics, Volume 72--May, 2005

Imaging of Congenital Anomalies of the Gastrointestinal Tract

barium pointing towards pyloric channel ('beak sign'). Jejuno-ileal atresia results from vascular occlusion and
The b a r i u m m a y outline c r o w d e d mucosal folds as ischemic injury to the developing gut. Jejuno-ileal atresia
parallel lines ('double/triple track sign'). These findings, is classified into four types. Type l is a simple intraluminal
if transient, should be interpreted with caution. The d i a p h r a g m c o m p o s e d of m u c o s a l and s u b m u c o s a l
i m p o r t a n c e of clinical e x a m i n a t i o n and test feed is elements. No interruption of the muscularis is present.
e m p h a s i z e d especially in cases w i t h equivocal Type II represents an atresia with solid fibrous cord (all
sonographic or barium findings. This will reduce the risk the layers of intestinal wall interrupted) connecting the
of false-positive diagnoses and negative laparotomies. 2~ blind ends of the bowel. Type III is an atresia with
complete separation of the blind ends and an associated
DUODENUM mesenteric defect. The familial form of multiple atresias is
considered as Type IV. 1~3o
Duodenal Atresia/Stenoses The d i s p r o p o r t i o n a t e d i l a t a t i o n of the bowel
Complete duodenal obstruction is much more common immediately proximal to the atresia results in a bulbous
than congenital gastric obstruction. Vomiting is usually c o n t o u r s u g g e s t i v e of c o n g e n i t a l small bowel
bilious as obstruction is often distal to the ampulla of obstruction. 31Plain radiography is usually diagnostic and
Vater. The plain r a d i o g r a p h of the a b d o m e n shows further radiological "evaluation (upper GI series) is not
'double bubble' sign corresponding to air in stomach and necessary (Fig. 3). In chses of partial obstruction little
dilated proximal duodenum with absence of air in distal amount of distal gas is usually present. A small amount of
gastrointestinal tract. This condition may be diagnosed air may be injected through a nasogastric tube to confirm
with antenatal ultrasound showing fluid filled double- complete or partial jejunal obstruction. In isolated
bubble and polyhydramnios. Fetal karyotype should be proximal atresia of the duodenum or jejunum, the colon
o b t a i n e d as up to 30% of patients have D o w n ' s is of normal size because the remaining bowel distal to
syndrome. 9 There is a frequent association with other the atresia produces sufficient intestinal secretions to
a n o m a l i e s in up to 50% of cases. Partial d u o d e n a l produce a normal caliber colon? In ileal atresia, the colon
obstruction is caused by annular pancreas, Ladd's bands, has a n o r m a l location b u t the caliber is r e d u c e d
m i d g u t volvulus, p r e - d u o d e n a l portal vein and (functional microcolon) 32. In low intestinal obstruction
duplication cyst. contrast enema should be done to exclude colonic lesion,
A pre-duodenal portal vein (persistent left vitelline meconium ileus or Hirschsprung's disease especially
vein) results from n o r m a l situs a s y m m e t r y , and is
c o m m o n l y f o u n d in patients w i t h h e t e r o t a x y . The
resultant portal vein courses anterior to the pancreas and
d u o d e n u m . The diagnosis is made by identifying the
prepancreatic course of the portal vein on sonography, CT
or MR imaging. It is now believed that in most cases of
duodenal obstruction associated with preduodenal portal
vein, the obstruction is due to a primary, obstructing
duodenal lesion such as intraluminal membrane or web
and such a lesion should be suspected in these patients if
duodenal obstruction is present. 2,27


Jejunal/ileal atresia
Intestinal atresias account for about one third of all cases
of n e o n a t a l intestinal obstruction. Prenatal
ultrasonography is more reliable in detection of duodenal
atresia than the more distal lesions. The survival rate has
improved significantly in most of the series with the
operative mortality being less than 1%. More distal the
atresia better is the survival. An increased mortality is
observed in multiple atresias, 'apple peel' syndrome, and
w h e n atresia is associated w i t h m e c o n i u m ileus,
meconium peritonitis and gastroschisis.28Intestinal atresia
occurs in approximately 10% to 20% of neonates with Fig 3. Small bowel atresia: Plain radiograph demonstratesmultiple
gastroschisis and may be missed at the initial closure if a dilated bowel loops and fluid levels.Peritonealcalcifications
thick 'peel' obscures the bowel. 29 are seen as a result of meconiurnperitonitis.

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Arun Kumar Gupta and Bhuvnesh Guglani

when dilated small bowel or colon cannot be Any duodenal obstruction that occurs after immediate
differentiated on plain radiography.4,10Sonography may postnatal period should be assumed to be midgut
be useful in differentiating meconium ileus and ileal volvulus until proved otherwise. 38 The upper gastro-
atresia. In meconium ileus, the dilated bowel loops are intestinal examination shows a typical corkscrew appea-
filled with echogenic material, whereas in ileal atresia the rance of the duodenum and proximal jejunum (Fig 4). The
bowel contents are echo-poor.33 distal jejunum lies to the right of midline. The Ladd's
The 'apple peel' s y n d r o m e is thought to follow bands result in Z-shaped duodeno-jejunal configuration
intrauterine occlusion of the distal superior mesenteric and usually obstruct at the third portion of the duodenum
artery (SMA) resulting in a proximal jejunal atresia with but can obstruct more distally.ByA barium meal should be
absence of mid-small bowel and the dorsal mesentery. done for suspected malrotation since a normal barium
The distal bowel derives its blood supply from the enema does not exclude malrotation. The position of
proximal SMA. The distal small bowel spirals around its caecum may be normal in a significant number of patients
single vascular supply and resembles an "apple peel. "34,35 with malrotation. 2~The UGI examination remains the
Intestinal stenoses are much less common than gold standard for the diagnosis of malrotation
intestinal atresias. Stenoses result from three major
causes: external compression of the bowel lumen,
intramural narrowing resulting from rests of heterotopic
tissue, and incompletely perforated intraluninal webs. l~
Stenoses caused by extrinsic impression on the bowel
lumen are almost entirely confined to the duodenum as
a result of annular pancreas ~ or peritoneal bands.
In the fetal life, the primitive midgut herniates into the
extraembryonic celom and later returns into the
abdominal cavity between 6-10 weeks. During this
process, the primitive midgut rotates anticlockwise
through 270 degrees around the axis of the superior
mesenteric artery. Both, duodeno-jejunal junction and
caecal pole undergo 270 degree anti-clockwise rotation
followed by fixation of duodeno-jejunal junction in the
left upper quadrant and the ileo-caecal junction in the
right lower quadrant. Interruption of this process at any
point results in a narrow base of small bowel mesentery
with predisposition to volvulus.2 The term 'malrotation'
or 'Nonrotation' is a misnomer as it results from lack of Fig 4. Midgutvolvulus:Upper GI barium study demonstratesthe
complete intestinal rotation The Ladd's bands or classic 'corkscrew' appearance of the duodenum and
hyperfixation bands can form due to faulty peritoneal proximaljejunum.
attachments as a result of malfixation and malrotation of
the intestine. Rotational abnormalities are seen in Superior mesenteric vein (SMV) normally lies to the
approximately 70% of patients with situs ambiguous right of superior mesenteric artery (SMA). Malrotation
(heterotaxy). may be suggested on US if the SMV is to the left or
A neonate with bilious vomiting on the first day of life anterior to the artery. However, this is neither a sensitive
and radiographic evidence of complete d u o d e n a l nor a specific sign. The sonographic "whirlpool sign" is
obstruction does not require further radiological objective and a definite sign as volvulus, is shown at color
evaluation. On the other hand a neonate with bilious Doppler US as clockwise spiraling of the mesentery and
vomiting who has been normal for first few days or with SMV around the SMA.~, 4~Recent literature is replete with
radiographic evidence of incomplete obstruction many diagnostic criteria for various imaging modalities.
(presence of gas in distal part of gut) requires further The multiplicity of the findings is a testimony to the
evaluation.4 An upper gastrointestinal series is usually unsatisfactory nature of their yield.
performed initially to demonstrate the level and nature of Meconium peritonitis
obstruction. On a supine radiograph the normal C- loop
crosses the midline and duodeno-jejunal junction lies to Meconium peritonitis results from an in-utero bowel
the left of the left vertebral pedicles at the level of the perforation proximal to a complete obstruction. Although
duodenal bulb. The normal duodeno-jejunal junction may commonly associated with bowel atresias, it may be
be mobile and easily displaceable in infancy, especially in present in asymptomatic neonates with in-utero sealed
children less than 4 months of age. 37 bowel perforation. Plain abdominal radiograph may

408 Indian Journal of Pediatrics, Volume 72--May, 2005

Imaging of Congenital Anomalies of the Gastrointestinal Tract

show peritoneal calcifications due to calcified meconium

(Fig 5). The small bowel loops may be dilated in cases of
associated bowel atreasia. These calcific densities may be
seen in the scrotum also if the processus vaginalis is
patent. On ultrasound, the calcifications are seen as
echogenic densities'outside the bowel wall.

Fig 6. Sonogram of a distal ileal duplication cyst. An anechoic

lesion with typical wall characteristics is seen-inner
echogenicmucosal stripe and outer hypoechoic muscle layer

length of the gut where they lie along the mesenteric

b o r d e r and share a c o m m o n muscle wall and blood
supply. The duplications commonly occur in the distal
ileum (Fig 7) and esophagus. Duplication cysts are usually
s p h e r i c a l lesions and less often t u b u l a r . The
communication with the adjacent bowel is uncommon in
spherical lesions and is more likely to occur with tubular
duplications. 43

Fig 5. Meeonium ileus: plain radiograph shows multiple dilated

bowel loops (low intestinal obstruction) and 'soap bubble'
appearance in the right lower quadrant.

Meconium ileus
Meconium ileus is a low intestinal obstruction caused by
inspissated meconium usually in the terminal ileum and
is almost always associated with cystic fibrosis. The plain
abdominal radiograph usually shows low small bowel
obstruction with dilated small bowel loops but absent or
scant fluid levels. Although the absence of air-fluid levels
strongly suggests meconium ileus, the presence of air
fluid levels does not e x c l u d e it as some cases do
d e m o n s t r a t e air-fluid levels, e s p e c i a l l y those w i t h
associated complications such as volvulus and stenosis or
atresia.lS,42Volvulus of a meconium laden heavy bowel
loop is c o m m o n and can lead to intestinal stenoses,
atresias, gangrene and perforation. The "soap bubble"
appearance caused by admixture of air and meconium is Fig 7. Hirschsprung's disease (recto-sigmoid): Contrast enema
frequently seen (Fig 6). It can, however, also be seen with (lateral radiograph) shows abnormal rectosigmoid index and
ileal atresia, colon atresia, Hirschsprung's disease and the transition zone with narrow caliber of the rectosigmoid and
meconium plug syndrome. TMCalcifications or pseudocyst dilated proximal ganglionated sigmoid colon. The extensive
may be seen due to meconium peritonitis and localized ulcerations are due to enterocolitis.
ascitis Ultrasound is the imaging modality of choice for the
Enteric Duplication Cyst evaluation of an abdominal mass in the neonate. The most
common clinical manifestation of intra-abdominal enteric
Enteric duplication cysts can occur anywhere along the duplication cysts is intestinal obstruction. Occasionally,

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Arun Kumar Gupta and Bhuvnesh Guglani

haemorrhage or gastrointestinal bleeding may occur due from obstruction of the distal ileum. The right colon is
to ectopic gastric mucosa which is seen in 10-20% of cases most commonly affected. 53 Plain radiograph
which makes it visible with technetium-99m demonstrates features of a low intestinal obstruction with
pertechnetate scanning and may be of diagnostic value. air-fluid levels or mottled pattern due to retained
The cyst shows the echogenic inner rim of mucosa and meconium. Contrast enema shows a distal microcolon
hypoechoic outer rim of the muscle layer (double wall and complete obstruction at the level of atresia. On US,
sign). This is most easily identified in the dependent dilatation of the distal small bowel and proximal colon
portion of the cyst. 44Peristalsis of the cyst wall when with echogenic contents due to retained meconium may
visible, is another useful sign.45The cyst is fluid filled, be seen. 4
usually unilocular and anechoic. Occasionally, internal
Hirschsprung's Disease
echoes and septations may be seen due to hemorrhage or
inspissated mucoid material. Rarely, the cyst is Approximately one-fifth of neonatal intestinal
completely echogenic and gives false impression of a obstructions are due to Hirschsprung's disease.2In about
solid lesion. Multiple duplication cysts may be present in three quarters of these cases, the area of aganglionosis is
15-20% of cases. 46 limited to the rectum and sigmoid (short segment
Esophageal duplication cyst: duplications of the disease). 54 Long segment disease involves a variable
esophagus are the second most common duplication of portion of the colon proximal to the sigmoid and in total
the gastrointestinal tract after ileal duplications. 43,47An aganglionosis coli the entire colon and a part of the
esophageal duplication cyst may present with pressure terminal ileum2~ is involved. Total colonic aganglionosis
symptoms such as dysphagea, stridor, or breathing and 'ultra short segment' disease is rare.
difficulty. On chest radiograph, duplication cyst is seen as The majority of patients present within the first 6
posterior mediastinal mass. Ultrasound may demonstrate weeks in life. Neonates present with failure to pass
double-layered wall especially in large cysts abutting meconium, abdominal distention, vomiting or
posterior chest wall. CT and MRI demonstrate well enterocolitis. Enterocolitis is the leading cause of death in
delineated outlines with non-enhancing attenuation Hirschsprung's disease. 55Enterocolitis and perforation is
values of water. Endoscopic US can accurately diagnose more common in the long segment disease. The
duplication cyst by demonstrating contiguity of the perforation occurs most commonly in the ascending colon
muscularis propria of the esophagus with the muscle or in the appendixfi6,s7
layer of the cyst wall.~ Imaging Features: Plain radiograph shows features of
Duodenal duplication cyst is a rare congenital anomaly low bowel obstruction with colonic dilatation out of
and usually presents with symptoms of obstruction, but proportion to the small bowelY There may be absence of
may cause biliary obstruction and pancreatitis. 49The rectal gas or small amount of gas may be seen in rectum
differential diagnosis of duodenal duplication includes a (especially on prone films) on plain radiograph. However,
choledochocele, a pancreatic pseudo.cyst and intralurninal the absence of rectal gas is not specific for Hirschsprung's
dlverticulum.5~ disease, being more commonly seen in infants with sepsis
Mesenteric Cyst (Lymphangioma) and necrotizing enterocolitis. 59
Digital examination of the rectum or enemas,in
Mesenteric cyst is a congenital malformation arising due patients scheduled for barium studies should not be done
to sequestration of lymphatic vessels. 51These are usually as it may distort the findings.1~For the same reason, the
seen in the mesentery and less often in omentum and study (enema) is never performed with an inflated Foley
retroperitoneum. Children usually present in the first catheter in the rectum. TM Barium enema is performed
decade, with increasing abdominal girth or a palpable carefully on an unprepared patient by inserting a straight-
abdominal mass. Sonography reveals a thin-walled tipped catheter to a point just beyond the anal sphincter.
unilocular or multilocular cystic lesion, whereas CT and The patient should be placed in lateral position and
MRI demonstrate variable characteristics of the cyst barium infused slowly as rapid infusion can distend and
contents (usually water-to fat) depending upon whether mask the transition zone. The contrast agent should be
fluid is chylous, infected or haemorrhagic. Ultrasound is prepared with normal saline to avoid possibility of water
particularly useful to demonstrate the thin septations absorption from the large surface area of dilated colon?
which may not be well seen on CT. Rarely, a mesenteric The diagnostic feature in short segment disease is funnel
lymphangioma may contain calcification mimicking a shaped transition zone and reversal of the recto-sigmoid
mesenteric teratoma. 52 ratio (Fig 7). Normally, the rectum is the most distensible
portion of the bowel and has a diameter greater than that
LARGE BOWEL of the sigmoid colon (recto: sigmoid ratio >1). 2~The
radiological transition zone is commonly found distal to
Colonic Obstruction the pathological transition zone.6~There may be irregular,
Colonic atresia is quite rare and often indistinguishable uncoordinated contractions in the aganglionic segment
(20% of cases) which may be mistaken for mucosal

410 Indian Journal of Pediatrics, Volume 72--May, 2005

Imaging of Congenital Anomalies of the Gastrointestinal Tract

ulcerations. Delayed films after 24 hrs may demonstrate

prolonged retention of barium in the sigmoid colon (not
rectum which normally holds barium for 24 hours). This
is a strong indicator of Hirschsprung's disease, even when
the enema findings have been inconclusive. Rectal biopsy
is always necessary for confirmation.
The diagnosis of total colonic aganglionosis is much
more difficult. The radiological findings viz.
foreshortening, rounding of the colonic flexures and small
caliber colon2are relatively nonspecific. The prolonged
retention of barium on delayed films should alert one to
suspect the diagnosis. 61Presence of skip lesions in
Hirschsprung's disease is extremely rare (controversial).
Awareness of this variant aids in the interpretation of the
barium enema in children with signs and symptoms of
aganglionosis, especially when the rectal biopsy is
normal. ~2The associations of Hirschsprung's disease
include Down's syndrome, ileal and colon atresia and
Anorectal A n o m a l i e s
Fig 8. Lateralviewof distalcologramshowsrectourethralfistulain
The anorectal malformations are divided into high and a maleinfantwitha highanorectalmalformation.Associated
low types depending upon whether the termination of sacral dysgenesisis seen.
h i n d g u t is above or below the puborectalis sling.
Anorectal anomalies are often associated with fistulous
determining the level of atresia. 68The use of invertogram
tracts opening into the genitourinary system. Although
is to be discouraged as it causes unnecessary stress.
the distinction between high and low lesions can be made
During invertogram, the baby usually keeps crying
clinically, the radiologic study is required to evaluate the
causing contraction of puborectalis sling and obliteration
internal anatomy. This is usually accomplished using
of the lower rectum. Also, the rectum may be pulled in the
contrast studies. In both male and female infants with low
cephalic direction resulting in error of classification. In
lesions, there is usually a visible perineal opening and the
those anomalies, which are associated with fistulae with
communication with the genitourinary tract is absent 1~176
the urinary or genital tract, the fistula becomes the highest
However, there are cases with imperforate anus with
point of rectum in an invertogram and gas may escape
external signs of a low lesion with demonstrable fistula
resulting in less distension of the rectum. In prone
into the anterior urethra either at surgery or by
position, the fistula is lowest and therefore rectum is
radiographic studies. 66These cases should be considered
better distended causing adequate delineation of the
an intermediate form of imperforate anus with external
rectal gas 6s. The pubococcygeal line and M line are
signs of a low lesion but with fistula characteristically to
imaginary lines used to represent the level of puborectalis
the membranous or bulbous urethra. 65High lesions are
sling on lateral radiographs. The "M" line is a better
treated with initial diverting colostomy and definitive
anatomic correlate of puborectalis sling. The "M" line
repair at a later age. A pressure augmented distal
passes horizontally through the junction of the lower third
colostogram is recommended prior to definitive repair,
and upper two thirds of the ischium.
both to confirm the level of rectal atresia and to define any
Ultrasonography can assess the distance of the rectal
associated fistulous communication. 67 Water- soluble
pouch in relation to the perineal surface. A pouch perineal
contrast medium is injected under mild pressure through
distance of < 1.5 cm is indicative of low anomalies, while
a Foley's catheter with its balloon inflated to occlude the
a pouch that terminates above the base of the bladder
stoma. The fistulous tract not identified on the
(superior extent of urogenital diaphragm) is indicative of
conventional contrast colostogram (Fig 8) may be
a high lesion. 69However, the distinction between high
delineated when an augmented-pressure modification of
and intermediate anomaly cannot be made out with this
the technique is utilized. 66Micturating cystourethro-
technique. Recently, anal endosonography has been used
graphy (MCU) and/or retrograde urethrography (RGU)
as an accurate alternative to MRI in the assessment of
are also useful to demonstrate fistula and presence of
anorectal malformations after repair, y~
vesicoureteric reflux.
CT and MRI are the modalities of choice to delineate
The easily available plain radiograghy is simple and is
pelvic anatomy including puborectalis muscle and
often the preliminary investigation to be used in
external sphincter. Congenital anorectal malformations
evaluation of these patients. Prone cross table lateral view
are found in many forms, and are frequently associated
with babies kept in genu-pectoral position is useful in

Indian Journal of Pediatrics, Volume 72--May, 2005 411

Arun Kumar Gupta and Bhuvnesh Guglani

with other anomalies, especially of the spine, spinal cord, radiologist aimed at providing best information and help
and urogenital system. 71 Magnetic resonance imaging to the treating clinician to p l a n out the m o s t suitable
has proven to be the single stop modality to answer all the treatment efficaciously.
crucial questions such as level and type of malformation,
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Book Review

Hyperalgesia: Molecular Mechanism and Clinical Implications

I read the book "Hyperalgesia: Molecular Mechanism and Clinical Implications" with a great interest.
Pain is the terrible fear of mankind and it is more terrible than death itself. Pain relief is the most important
task of a physician. There are various theory and treatment associated with acute and chronic pain, still chronic
pain most of the time and in most of the world remain untreated, may be because of lack of understanding of
its etiology and mechanism. Before treating pain at each level it is important to understand mechanism of action
at peripheral and central level.
This book consists of 26 contributions submitted by renowned researchers from different parts of the world.
The book is divided into five sections, the first section discusses the basic question and defined very well about
hyper excitability. This section also gives very good insight about which nomenclature should be used in various
conditions of pain.
Second section describes the exiting n e w developments in molecular research and c o m m o n p a t h w a y
emerging to understand peripheral mechanism of hyperalgesia like discovery of the capsaicin receptor on the
u n d e r s t a n d i n g of p e r i p h e r a l sensitization, contribution of tetrodotoxin-resistant s o d i u m channels in
development of peripheral hyperalgesia, VR-1 receptors or TRPV1 receptors is a key substrate in hyperalgesia.
Third section describes transudation mechanism of nociceptors following the pathological process. For
example, mechanism of cold hyperalgesia induced by inflammatory process, visceral pain and hyperalgesia,
ASIC3 is responsible for development of mechanical hyperalgesia following muscle injury. This section also
explains w h y it is difficult to adequately treat pain in cancer patients.
The fourth section describes the role of central nervous system in hyperalgesia and explains very well the
molecular mechanism of central hyperalgesia and its neurobiological features.
The fifth section explains that m a n y brain specific functions also contribute to hyperalgesia and pain
It is a good reference book for clinician and basic scientist working in the area of molecular biology,
neurobiology, molecular pharmacology.
It is also a good reference book for people working in the area of genetics and trying to find out the cause of
pain at genetic level. In addition, it is a useful reference book for pharmaceutical industries involved in making
analgesic/and doing pain research.
For general practitioner and clinician it m a y be of limited use as the main emphasis is on molecular aspect
of pain and its mechnism.

Dr. Sushma Bhatnagar,

Associate Professor,
Unit of Anaesthesiology
New Delhi.

414 Indian Journal of Pediatrics, Volume 72--May, 2005