Oesophageal
achalasia in an unusual lesion in childhood and even more rare in
infancy. We present a case report of a seven-month-old child who was
brought with regurgitation of feeds, recurrent chest infection and
failure to thrive. Diagnosis was confirmed on contrast oesophagogram,
which revealed dilated oesophagus, with smooth narrowing of the oesophagogastric
junction. Child was treated successfully by trans-abdominal oesophagomyotomy
with fundoplication.
Introduction
Achalasia cardia is a pathological condition characterized by failure
of relaxation of distal oesophagus and oesophagogastric junction.1,2
It could be primary in which there is absence of ganglion cells in
the myenteric plexus or it could be secondary as seen in Chaga’s
disease where there is progressive degeneration of ganglion cells.1
Usually the patients affected are middle aged females,3
however, the lesion can be seen occasionally in children. The cause
of achalasia is unknown but the entity is also reported to occur in
families. The child commonly presents with regurgitation, vomiting,
recurring chest infection and rarely with respiratory distress and
near miss sudden infant death syndrome.1,2,4-11 We present
a case of achalasia cardia who was managed successfully by surgery.
Case Report
A seven-month-old female child presented with frequent regurgitation
of feeds, recurrent chest infection and failure to thrive for the
last four months. The patient was second born full term normally delivered,
with a birth weight of 2.8 kg. There was no history of similar complaints
in the other sibling. On examination, the patient was febrile, malnourished
and pale, and weighed 3.5 kg. Patient’s heart rate was 100/min
and respiratory rate was 20/min. On chest auscultation, crepitations
were heard in the right lower zone.
 |
| Fig. : Contrast oesophagogram
showing dilated oesophagus and abrupt, smooth narrowing of the
oesophagogastric junction. |
Abdominal examination
was normal. Laboratory investigations revealed a haemoglobin of 9
gm%, total leucocyte count of 25000/mm3 and differential
count showed leucocytosis with 80% polymorphs. A plain X-ray chest
revealed right lower lobe pneumonitis. After treatment of chest infection
with injectable antibiotics and chest physiotherapy an upper gastrointestinal
contrast study was done, which revealed a dilated oesophagus with
an abrupt and smooth narrowing of oesophagogastric junction which
was suggestive of achalasia cardia (Fig.).
A modified Heller’s oesophagocardiomyotomy with Thal’s
fundoplication was done. Post operatively feeds were started on the
second day, nasogastric tube was removed on the third day and the
patient went home on the fourth day. At 2 months of follow-up, the
patient is asymptomatic, doing well, and has gained weight.
Discussion
Thomas Willis first described achalasia cardia in 1674.12
This lesion which is commonly seen in middle aged females is an uncommon
condition in children and more so in infancy.1,2,4-11 Less
than 5% patients present before the age of 15 years.10
The youngest patient with achalasia being reported is a newborn who
did well after oesophagocardiomyotomy.13 It needs special
consideration as it is associated with a high incidence of recurrent
respiratory tract infection and failure to thrive. As the clinical
presentation of achalasia cardia mimics other causes of oesophageal
obstruction, it challenges the clinical acumen of the treating physician.
In the past, few others have mistaken it for congenital oesophageal
stenosis and have done subtotal resection of oesophagus and gastrostomy
which is not mandated for achalasia.9,14 Therefore, one
should always consider achalasia cardia in the differential diagnosis
of oesophageal obstruction along with gastro-oesophageal reflux, milk
allergy, duplication cyst of the oesophagus and stomach, congenital
oesophageal stenosis and diffuse oesophageal spasm.
Diagnosis of achalasia cardia is made on contrast oesophagogram which
shows dilated oesophagus with smooth narrowing of the distal oesophagus
and oesophagogastric junction also described as “bird-beak”
sign5 or with the help of manometry, in which there is
a failure of relaxation of the lower oesophagogastric junction with
swallowing. However, it may be difficult to differentiate achalasia
from congenital oesophageal stenosis, since both may have similar
symptoms and radiological appearance. Manometry is performed very
infrequently in children and requires experience in interpretation.
We suggest trying to negotiate a wide bore infant feeding tube across
the oesophagogastric junction, which will pass in the former, as there
is a functional obstruction but not in the later, as there is a mechanical
obstruction. It was possible to negotiate the nasogastric tube into
stomach in our case.
The goal of treatment of achalasia is to relieve functional obstruction
in the distal oesophagus and oesophagogastric junction. A modified
Heller’s oesophagocardiomyotomy is considered to be the gold
standard in the management of achalasia cardia.15 However,
the addition of anti-reflux operation is still a matter of controversy.
Some surgeons find an added anti-reflux procedure unnecessary, but
because of the documented risks of Barrett’s syndrome and severe
GORD syndrome seen in 36% children, we feel that it’s a useful
adjunct, as long as it doesn’t cause distal oesophageal obstruction.16,17
A modified Heller’s oesophagocardiomyotomy with fundoplication
can be done by open method or by laparoscopy.1,4,7,10 Other
modalities of treatment described with varying success rate are pharmacological
and mechanical. Pharmacotherapy includes the use of smooth muscle
relaxants like isosorbide dinitrate or calcium channel blockers like
nifedipine or local injection of botulin toxin which has been tried
with some success in adults.18,19 Mechanical therapy includes
forceful pneumatic dilatation of the oesophagus,20,21 however,
symptomatic improvement is mostly seen in older children. Recurrence
of symptoms within six months and oesophageal perforation are the
main concerns of the procedure.22
To summarize, one should always keep achalasia cardia in the differential
diagnosis of an infant presenting with signs and symptoms of oesophageal
obstruction and should promptly get an upper gastrointestinal contrast
study to confirm the disease. A properly done transabdominal oesophagocardiomyotomy
with an anti-reflux procedure leads to a successful outcome.
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DRUG-RESISTANT
SALMONELLA
‘The appearance of this resistant S choleraesuis is a
serious threat to public health, and thus constant surveillance
is warranted’
Salmonella enterica serotype choleraesuis (S choleraesuis) usually
causes systemic infections that need antimicrobial treatment.
This strain proved to be resistant to all antimicrobials commonly
used to treat salmonellosis, including ceftriaxone and ciprofloxacin.
The investigators conclude that the public-health implications
of this multidrug-resistant form of salmonella are serious and
suggest that constant surveillance is needed to prevent its
further spread.
Lancet, 2004; 5 : 1285. |
