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320 Achalasia

the lower oesophageal sphincter to relax on swallowing, along with
an absence of peristalsis being the confirmatory features. Endoscopy
Dysphagia, excludes other causes of organic obstruction or infection in the
regurgitation, distal oesophagus.
bronchial asthma, Recurrent cough Refractory An algorithm for the diagnosis of achalasia in children in the
persistent vomiting bronchial asthma developing countries and countries with limited diagnostic facilities,
(infants) seen in Figure 50.2, has been suggested by Chirdan et al. 5

Management
The three therapeutic modalities of achalasia management are pharma-
cological treatment, pneumatic dilatation, and esophageal myotomy.
An overriding consideration is the nutritional status of the child at
outset, and due to the unpredictability of the efficacy of any of the
procedures, restoration of good nutritional status is paramount. That
can be effectively achieved with nasogastric feeding.
Pharmacological Treatment
Per-endoscopic four quadrant injection of botulinum toxin (100 unit)
Plain chest radiograph (to include upper abdomen) has been shown to substantially reduce the lower oesophageal sphinc-
• Widened mediastinum ter pressures (preinjection, 44 mm Hg; postinjection, 16 mm Hg) with
substantial sphincter relaxation. The clear disadvantage of this tech-
• Fluid level in mediastinum nique is the need for its repeated application. 6
• Pneumonic changes in lung fields The calcium channel blocker nifedipine has been used with good
effect in adults, but its use has been limited to those adults unfit for any
• Absent air shadow in gastric fundus other form of intervention. 7
Pneumatic Dilatation in Children
The aim of pneumatic dilatation in children is to relieve obstruction by
a gentle disruption of the lower oesophageal sphincter. This procedure
is carried out under anaesthesia using radiological and manometric
control. An upper gastrointestinal contrast study is performed follow-
ing the procedure to exclude oesophageal perforation. The number
and periodicity of repeat dilatations is patient-specific. It is the recom-
Barium oesophagogram mended initial therapeutic method of choice in older children, but if
• Dilated proximal oesophagus initial attempts fail to provide satisfactory relief, surgical treatment is
• Smooth tapering of distal oesophagus then indicated. Only 25% of children show significant improvement
with pneumatic dilatations alone, and few children under the age of 9
• Ineffective peristalsis in proximal oesophagus
years have responded to this treatment. 8
Oesophageal Myotomy
The operative approaches include an abdominal approach, an open
thoracic approach, or—more recently—an endoscopic approach
9
through the chest or abdomen. These approaches share the same
objective of performing a modified Heller’s procedure, which includes
a myotomy of the lower oesophagus, preserving the integrity of the
oesophageal mucosa. (Heller initially performed his procedure on both
Achalasia sides of the oesophagus; that is, a double oesophagomyotomy.) This is
often accompanied by a subsequent fundoplication to prevent gastro-
oesophageal reflux frequently produced by the Heller’s procedure, and
Source: Adapted from Chirdan LB, et al., Childhood achalasia in Zaria Nigeria. E Afr Med J some authorities restrict the myotomy to 0.5 cm on the cardia, thereby
2001; 78:497–499. reducing the need for antireflux procedures. In those patients for
Figure 50.2: Algorithm for the diagnosis of oesophageal achalasia in
children in countries with limited diagnostic facilities. whom transabdominal or a laparoscopic oesophagomyotomy has been
performed, this is very frequently accompanied by an antireflux pro-
9
cedure, although some recent authors evaluating the long-term results
have suggested that symptomatic improvement in the longer term can
be equally effectively obtained without an antireflux procedure. 10
Surgical Procedure
Oesophageal myotomy through the abdominal route is described in
detail in this section. An antireflux procedure is usually not necessary
because reflux is rare among African children.
Position and Anaethesia
General anaesthetic with endotracheal intubation is used, with pre-
caution taken to avoid aspiration of oesophageal contents, especially
during induction. The child is placed in the supine position. In cen-
11
tres where paediatric endoscopes are available, preoperative oesopha-