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Achalasia 321

goscopy is used to ensure complete evacuation of retained food and exposed for several centimetres. A myotomy is performed on the distal
secretions from the oesophagus. A nasogastric tube of appropriate size oesophagus vertically with extension just distal to the cardio-oesopha-
is passed into the stomach. geal junction. The muscle is elevated on either side of the myotomy with
Incision and Exposure graspers anchoring the edge of the muscle and with the dissection of the
An upper midline abdominal incision extending from the xiphisternum muscle from the mucosa performed with endoscopic scissors.
to the umbilicus is used. The incision could be extended to just below Postoperative Care
the umbilicus to give more access. Once the peritoneum is opened, Intravenous fluid is stopped after 3–4 days, and the nasogastric tube is
adequate exposure of the abdominal oesophagus is gained by retracting removed by day 4.
the left lobe of the liver with a wide liver retractor anterosuperiorly. The most worrying postoperative complication is leakage from an
Alternatively, the left triangular ligament is divided in its avascular undetected perforation of the oesophageal mucosa, making a subse-
plane, and the left lobe of the liver is retracted towards the midline. quent contrast study a requirement of the postoperative period.
Mobilisation Prognosis and Outcomes
The next stage of the operation is the exposure of the oesophageal hiatus
The vast majority of patients have an immediate and lasting benefit from
and mobilisation of the distal oesophagus. For those who want to add
their surgery. Failure of the initial operation should be managed by an
a floppy Nissen fundoplication, the fundus of the stomach is freed by
attempt at a second myotomy (on the contralateral side of the oesopha-
ligating and dividing the short gastric vessels in the gastrosplenic liga-
gus), but more than 80% of patients have long-term relief of symptoms
ment. The phrenicoesophageal membrane is then stretched by apply-
and resumption of appropriate growth following the first operation.
ing downward traction on the stomach and retracting the diaphragm
upwards. The avascular membrane is incised with a scissors, exposing Evidence-Based Research
the muscularis of the oesophagus, and the anterior vagus nerve is seen Due to the rarity of achalasia in children, no prospective studies exist
on the oesophagus. The exposed distal oesophagus is now encircled by that compare the various modalities of treatment and their long-term
using a combination of blunt and sharp dissection, taking care not to outcome in Africa. Guidance is derived mainly from retrospec-
injure the posterior vagus nerve. A rubber sling or nylon tape is then tive experiences. Table 5.1 presents evidence-based research using
placed around the distal oesophagus, and 5–8 cm of the oesophagus is Heller’s procedure.
exposed by using blunt dissection.
Table 50.1: Evidence-based research.
Myotomy
The myotomy is done on the anterior oesophagus extending to 1 cm of Title Evaluating long term results of modified Heller limited
esophagomyotomy in children with esophageal
the fundus of the stomach. An incision is made in the distal oesophagus; achalasia
the divided muscle is then parted with a blunt haemostat, exposing the Authors Vaos G, Demetriou L, Velaoras C, et al.
mucosa. The muscle is separated from the underlying mucosa by pledget Institution Second Department of Pediatric Surgery, P and A
dissection. This is continued to about at least half the circumference, free- Kyriakou Children’s Hospital, Athens, Greece;
ing the oesophagus from the constricting muscle. This is extended to 1 cm
of the fundus. The stomach and oesophagus are then distended with air First Department of Pediatric Surgery, P and A
Kyriakou Children’s Hospital, Athens, Greece;
from the nasogastric tube, and the exposed mucosa is carefully inspected
for perforation. In the event of a perforation, the mucosal defect is closed Department of Pediatric Surgery, Penteli General
Children’s Hospital, Athens, Greece
with polyglycolic acid sutures. Finally, the hiatus is narrowed posteriorly
by placing deep sutures through the diaphragmatic crura, leaving sufficient Reference J Pediatr Surg 2008; 43:1262–1269
space along the oesophagus that can admit the tip of the finger. Problem The role of modified transabdominal Heller’s myotomy
in the long-term outcome of children with achalasia.
Wound Closure Intervention Heller’s limited oesophagomyotomy.
The abdominal wound is closed in layers with nylon sutures. The skin Comparison To evaluate long-term symptom relief after intervention
is closed with subcuticular suturing. using subjective outcome, Ba esophagogram,
Laparoscopic Approach esophageal pH, and oesophageal manometry.
When a laparoscope is available, the pneumoperitoneum is established Outcome/effect Excellent to good results observed in 93.3% of patients,
through standard procedure and a 5–10 mm camera port is placed late Ba oesophagogram showed a significant decrease in
oesophageal diameter compared to preoperative values
through the infraumbilical skin crease. Three to four additional 5-mm (p < 0.01), and the late oesophageal manometry showed
ports are placed to permit retraction and dissection of the oesophageal a significant decrease of lower oesophageal sphincter
hiatus. The same principles are adhered to as in the open operation, with pressure (p < 0.05).
mobilisation of the distal oesophagus under direct vision. Following Historical This report, although retrospective and in a small
circumferential encircling of the oesophagus with the umbilical tape and significance/ population of children, showed that the long-term
outcome of children treated with modified Heller’s
with caudal retraction on this tape, the distal oesophagus is dissected and Comments myotomy can be quite satisfactory.

Key Summary Points

1. Achalasia is often diagnosed late in childhood. 4. Heller’s myotomy (open or laparoscopic) should follow
correction of nutritional deficit, with good results being
2. An awareness of the condition as it affects children is key to
diagnosis. expected.
3. A combination of failure to thrive, respiratory symptoms, 5. Strict attention must be paid to ensuring mucosal integrity.
and food aversion should prompt investigation of upper
gastrointestinal (GI) tract.