What is the role of achalasia in infancy ?
A. Appignani, E. Paico, M. Lima, M. Ferrari, R. Domini (Bologna)
Achalasic megaesophagus is a disease whose etiology is unknown, typically caused by absence of peristalsis along the viscus and by inability of the lower esophageal sphincter (LES) to relax during ingestion. Approximately 5 p. cent of the patients with achalasic megaesophagus are less than 14 years old ; hence this disease is uncommon during childhood. According to Vane [10], the pediatric patients reported in a world-wide review in this century amount to almost 250.
Opinions vary as to the best treatment in adults; some believe repeated dilatation of the strictured lower esophagus to be the most effective solution while some consider surgical approach as mandatory.
Results obtained with dilatation in children are not completely successful, nevertheless cases are still described of children previously treated by dilatation, and finally submitted to surgery.
Like many others [1, 2, 3, 5, 7, 10] we believe the Heller esophagomyotomy combined with an antireflux plasty to be the most effective treatment in children affected by achalasic megaesophagus.
This paper presents our experience in 16 children under our care, since 1972.
Material and methods
From 1972 to 1988, 16 patients with achalasic megaesophagus were treated in the Department of Pediatric Surgery of Bologna: 9 girls and 6 boys. The ages of the patients ranged from 14 months to 13 years, 4 were under 2 (table 1). Symptoms were as reported, regurgitation being the most common.
Radiograms with contrast medium allowed diagnosis in all cases , showing proximal enlargement and distal stricture of the viscus (figure /). The last 5 cases were also been submitted to manometric studies, which showed an increase of LES pressure.
Table 1. Symptoms of 16 children with esophageal achalasia observed in the institute of pediatric surgery, university of Bologna Italy, 1972-1989.
|
Symptoms |
n° cases |
% |
||
|
Regurgitation |
12 |
75 |
||
|
Weight loss |
9 |
56 |
||
|
Dysphagia |
5 |
31 |
||
|
Retrosternal pain |
5 |
31 |
||
|
Regurgitation/ vomiting |
2 |
12 |
||
|
Recurrent infections |
/ |
/ |
||
|
Pulmonary disease |
1 |
6 |
||
Figure 1. Proximal enlargement and distal esophageal stricture.
Peristalsis was therefore abnormal and ineffective, the LES showing no relaxation. Two of our patients had been treated previously in other medical centers: one with dilatations and the other with drug therapy, both with poor results.
Once the diagnosis was made, all the patients were submitted to modified Heller esophagomyotomy combined with an antireflux plasty using Lortat-Jacob's technique.
Details of surgical technique
The subdiaphragmatic esophagus is mobilized through an abdominal approach (xipho-umbilical incision) and pulled downwards with a tape; then the muscular wall of this segment is incised ; the incision continues proximally to the first portion of the mediastinal esophagus and distally no further than 1 cm from the gastro-
esophageal junction ; the incision is therefore no longer than 6-8 cm. The muscular wall is divided from the mucosa through a dissection of almost 50 p. cent of the circumference (figures 2, 3).
Figure 2.
Figure 3.
Figure 4.
The second step is the reconstruction of the hiatus of the esophagus, followed by the antireflux plasty performed by suturing the left edge of the myotomy to the right side of the gastric fundus (figure 4).
The operation never lasted more than 2 hours; neither perforation of the esophageal mucosa, nor any other kind of complication ever occurred.
Histologic examination was made in all cases and invariably showed absence of ganglion cells.
The surgical results were excellent. In a 1-10 yrs follow-up (mean 5 yrs): 15 patients (93.7 %) had complete relief of symptoms from the early postoperative period. Radiograms revealed good transit of contrast medium (figure 5).
Figure 5.
Only one case presented symptoms at the tenth postoperative day and was again submitted to surgical intervention, during which the previous, inadequate myotomy was enlarged ; subsequently the patient recovered.
Discussion
There are different theories of the etiology of esophageal achalasia, the most popular is the neurological one, supported by the histopathologic finding of absence or deficiency of ganglion cells in the Auerbach plexus of the distal tract of the esophagus. Ellis [5] found 3 children affected in the same family. Others report about a family where 7 children, younger than 1 yr, were probably to be affected, suggesting that the disease could be familial or congenital.
Clinical presentation of achalasia in children depends on the age, being similar to gastroesophageal reflux in infants, i.e. regurgitation, choking, apnea, pneumonitis, impaired growth [2, 3]. In older children, clinical signs are more similar to those of adults, with dysphagia as the earliest and most common one, regurgitations, and loss of weight [1,2, 3].
Radiograms with contrast medium usually allow diagnosis; the typical picture is an obstruction at the esophagogastric junction with proximal dilatation. As the disease progresses, the radiographic signs become more characteristic : very enlarged esophagus, cone-shaped stricture of the lumen, extending to the stenotic segment. Esophagoscopy is necessary to differentiate achalasia from peptic disease.
Clinical diagnosis is confirmed by esophageal manometry, which reveals that, at rest, the LES has pressures two or three times the normal range, as has the central part of the viscus, while the upper part is normal. The lower sphincter has no relaxation, and the central part shows no peristalsis.
In the past, the results of attempts to improve symptoms of affected patients with medical therapy have been poor: thus, Yon reported 14 cases treated by drugs therapy: all but one have been unsuccessful. Recently, others have used calcium antagonists (nifedipine) in children, with good results. Maksimak recommends the use of nifedipine for short periods, as a basis for correct therapy ; in any case, calcium antagonists can be used for longer period, for those unfit for dilatations or surgery. We neither used, nor considered pharmacotherapy, which affords only temporary relief of symptoms.
The use of dilators to heal achalasia is as old as the disease. In 1664, Thomas Willis described megaesophagus and used the first rudimentary dilator made of whale bone. Since then, many mechanical, hydrostatic and pneumatic dilators have been used. Adults obtain 65 p. cent to 85 p. cent improvement with dilatations and only 4-5 p. cent occurrence of complications, such as perforation [11]. However, children do not have much reliefs of symptoms (only 30 %) and suffer many complications (20 % perforations) [1]. In the cases reported by Vane [10], 4 patients were submitted to dilatation (mean 6 per patient), but the long-term follow-up was unsatisfactory, so that operation was performed. Other authors reported remarkable improvement in 2 cases out of 17 submitted to dilatation, while 5 had moderate results. Azizkhan [1], at Boston Children's Hospital Center, submitted 20 children to dilatation ; only 5 (older than 9 yrs) improved shortly after, while the remaining 15 a bad response and 12 needed a Heller modified myotomy.
Despite apparent advantages, dilatation also presents many risks such as perforation, gastroesophageal reflux and recurrence, and furthermore, children are uncooperative and general anesthesia is required for these maneuvers [1, 2]. It should be stressed that most follow-ups are short-term, so that many recurrences are not recorded in these reports. We are against dilatation, and consider surgery to be the best treatment in these children.
Many techniques to treat patients with esophageal achalasia have been proposed, most involving destruction of the LES ; theses have been abandoned because they invariably led to serious reflux esophagitis.
At present, the surgical technique we prefer is the myotomy (anterior and posterior) proposed for the first time by Ernest Heller [6] in 1913. Since then, this has been modified many times : in 1918 by De Brune-Groenveldt [4] who supported
the efficacy of anterior myotomy alone, and in 1967 by Ellis et al. [5], who performed myotomy only in the distal segment of the viscus. Ellis and Olsen compared the results obtained with the Heller myotomy (94 %) to those obtained with hydrostatic dilatation (65 %). Payne et al. came also to the same conclusions.
The results obtained with the modified Heller myotomy are far better in children than in adults: in a review of 78 affected children, who underwent Heller myotomy, Azizkhan et al. [1] observed excellent results in 68 cases (87%), good in 8 (10%), and eventual recurrence in 2 cases. Vane [10] submitted 21 children to Heller's modified myotomy (follow-up 1-14 yrs): 18 (86%) were cured, 1 improved after dilatation, and 2 were reoperated.
16 patients were operated in our Institute: 15 (93.7 %) had complete resolution of symptoms (follow-up 1-10 yrs, mean 5 yrs), the remaining child had recurrence due to incomplete esophagomyotomy. Since the results of modified Heller myotomy are good, dilatation is unnecessary (table 2).
Table 2. Cases of children with esophageal achalasia, treated with modified Heller esophagomyotomy + Lortat-Jacob antireflux plasty, in the institute of pediatric surgery of the university of Bologna. Italy.
|
Patient |
Age |
Sex |
Date 1st operation |
Complication |
F. up |
||
|
S.R. |
9 yrs |
F |
19-05-72 |
none |
O.K. |
||
|
S.G. |
12 yrs |
F |
20-07-72 |
none |
O.K. |
||
|
PS. |
8 yrs |
M |
13-02-78 |
none |
O.K. |
||
|
A.A. |
10 yrs |
F |
23-07-79 |
none |
O.K. |
||
|
L.S. |
14 mths |
F |
10-06-81 |
recur.* |
O.K. |
||
|
M.S. |
10 yrs |
F |
4-05-82 |
none |
O.K. |
||
|
C.G. |
13 yrs |
M |
25-10-83 |
none |
O.K. |
||
|
P.Z. |
21 mths |
M |
16-11-84 |
none |
O.K. |
||
|
P. A. |
8 yrs |
F |
25-02-85 |
none |
O.K. |
||
|
CM. |
4 yrs |
F |
10-10-85 |
none |
O.K. |
||
|
S.L. |
9 yrs |
F |
14-10-87 |
none |
O.K. |
||
|
S.C. |
10 yrs |
M |
16-12-87 |
none |
O.K. |
||
|
M.S. |
3 yrs |
M |
30-10-87 |
none |
O.K. |
||
|
F.M. |
18 mths |
M |
22-02-88 |
none |
O.K. |
||
|
R.M. |
12 yrs |
M |
14-06-88 |
none |
O.K. |
||
|
L.V. |
1 yrs |
F |
30-06-88 |
none |
O.K. |
||
* Reoperated on with esophagomyotomy and anti-reflux plasty.
The surgical approach for esophagomyotomy can be thoracic or abdominal.
Temple considers abdominal access the most suitable, as the cardia is intra abdominal. This approach has been criticized because it is difficult to sufficiently mobilize the strictured esophageal segment, and the myotomy may prove too short or insufficient so that symptoms recur; on the contrary, a too extensive myotomy can lead to gastroesophageal reflux. Ballantine et al. use the abdominal approach : they maintain that appropriate dilatation of the hiatus allows a 6-8 cm incision.
Obviously, the length of the incision varies with the age of the patient, but should not extend further than 1 cm on the stomach. When this access is used and the hiatus is disrupted, it is necessary to add an antireflux procedure.
Those who advocate the thoracic access consider it better in patients with a compromised proximal segment and severe esophageal spasm. Data in the literature confirm the validity of both approaches [2, 5].
Finally, there are different opinions on the need to add an antireflux plasty to the Heller modified myotomy: the incidence of gastroesophageal reflux in adults is variable, and reported as from 3 to 60 p. cent. On the other hand, the presence of gastroesophageal reflux seems to increase progressively as time goes by.
In a group of 145 patients operated with modified Heller myotomy, 24 p. cent developed gastroesophageal reflux in the first year, and 48 p. cent in the following 10 years.
The incidence of reflux after esophagomyotomy in children is 20 p. cent [1,2, 5].
Gastroesophageal reflux is due to injuries of the hiatus during mobilization of the subdiaphragmatic esophagus or to excessively extended myotomy. If distal myotomy extends too far (more than 1 cm) along the esophagogastric junction, the patients will develop gastroesophageal reflux unless a plasty is added. Obviously, an antireflux procedure should always be done when there is a pre-existing reflux or hiatal hernia.
To prevent reflux, we personally combine the Lortat-Jacob technique, because it is effective and easy to perform. We disagree with the use of Nissen fundoplication as this can cause unpredictable obstruction and impairment of esophageal motility.
Conclusions
Esophageal achalasia is a rare disease in children. Diagnosis is mainly radiological, and elective treatment in these patients is by Heller's modified esophagomyotomy combined with an antireflux plasty, which guarantees definitive cure.
References
4. De Brune-Groenveldt JR (1918) Over cardiospasmus. Ned tijdscher. Geneeskd 54: 1281-1282.
9. Taylor RG, Myers NA (1989) Achalasia of the cardia in children. Pediatr Surg 4 : 386-389.
S.G. Jolley(Las Vegas)
Achalasia is uncommon in childhood, and suspected to be quite rare in infancy. The absence of esophageal symptoms during infancy predominates the symptom complex of most older children reported with achalasia [1-4]. In an attempt to estimate the incidence of achalasia in infancy and childhood, a large group of infants and children referred for an evaluation of esophageal symptoms is presented.
Population and methods
Since august 1977, the author has evaluated 892 infants and children with esophageal symptoms referred to Primary Children's Medical Center (Salt Lake City, Utah), the Children's Hospital of Oklahoma (Oklahoma City, Oklahoma), and Humana Children's Hospital-Las Vegas (Las Vegas, Nevada). Regurgitation of feedings was present in 544 patients, acute or chronic respiratory symptoms in 612 patients, and poor growth or dysphagia in 187 patients.
There were 519 boys and 373 girls with ages ranging between two weeks and 16 years. Six hundred and fifty eight patients were infants (less than one year of age). Associated disorders were present in 367 patients. The most common associated disorders were central nervous system disease, bronchopulmonary dysplasia related to prematurity, repaired esophageal atresia with tracheo-esophageal fistula, and congenital heart disease.
All patients had a barium esophagram performed. With the exception of two patients referred with known achalasia, all patients had 18-24 hour esophageal pH monitoring performed as described previously [6] to evaluate for gastroesophageal reflux (GER). Esophageal manometry [7] was performed in 117 patients, including all patients with achalasia suspected from radiographic studies or esophageal pH monitoring. Personal follow-up information on clinical symptoms and the clinical course of all evaluated patients was available for two weeks to eleven years (average of three years). In 575 patients, follow-up was for at least one year (average of four years).
Achalasia was defined as esophageal dilatation with distal esophageal narrowing unrelated to stricture. During esophageal pH monitoring, GER was absent and apple juice feedings (300 ml/sq meter BSA) could not be cleared completely from the esophagus within three hours (figure I). Manometric findings in achalasia included 1. an elevated resting lower esophageal sphincter (LES) pressure with incomplete or no relaxation with swallowing, and 2. absent or low amplitude primary peristaltic waves in the esophageal body.
Figure 1. A recording of distal esophageal pH in a six years old boy with achalasia is shown.
The child was given a feeding (300 mL/sq meter BSA) of apple juice (pH 3.0-4.0, upper recording) and of milk (pH 6.0-7.0, lower recording) while maintained in the upright position. Note that the esophageal pH remains similar to the pH of the ingested liquid for at least three hours.
Results
Of the 892 patients with esophageal symptoms evaluated, only six (0.7 %) were found to have achalasia. None of the 658 infants were found to have achalasia, as compared to six (2.6 %) of the 234 children (older than one year of age).
If patients with associated disorders were excluded, six (5.7 %) of the 106 children and none of the 419 infants had achalasia. The children with achalasia were between four and thirteen years of age and had no history of esophageal symptoms during infancy.
Only one full-term female infant was found to have an achalasia-like condition. She presented with regurgitation of feedings, drooling, poor growth and apneic spells. This one month old girl had a dilated esophagus with distal esophageal narrowing on esophagram, incomplete clearance of apple juice from the esophagus after three hours and no GER on esophageal pH monitoring, and an elevated resting LES pressure (30 mm Hg) with normal relaxation and normal primary peristaltic
waves with swallowing. She was treated only with strict postural therapy, maintaining the upright (30-45 degrees) or prone-elevated position at all times.
The apneic spells resolved immediately and all symptoms disappeared within two weeks. The postural therapy was discontinued at one year of age. Ten years later, this patient remains asymptomatic with normal growth and development.
Discussion
Achalasia appears to be rare in infancy in spite of reported series in children with up to 20 p. cent of cases in infants [1,3, 5]. None of the large group of infants selected for evaluation because of esophageal symptoms were found to have achalasia.
Only one infant had achalasia-like findings, but all symptoms resolved with postural therapy. When found in childhood, achalasia appears primarily in older children.
The rare appearance of achalasia in infancy and the usual absence of esophageal symptoms during infancy in older children with achalasia serve to characterize achalasia as an acquired or degenerative, rather than congenital, disorder.
References