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OESO©2015
 
Volume: The Esophagogastric Junction
Chapter: Achalasia
 

What are the features of esophageal dysmotility in children?

J.A. Tovar, G. Prieto, M. Molina, J. Arana (Madrid)

Achalasia is a disorder of the upper gastrointestinal tract in which progression of the bolus through the lower esophagus is blocked by a functional obstruction at this level associated with a lack of peristaltic activity. This leads to esophageal dilation (megaesophagus) and to several symptoms including regurgitation, dysphagia, weight loss, etc. The etiology of achalasia is unclear, but the functional disturbances in adult patients are well known: lack or incompleteness of deglutitory relaxation of the lower esophageal sphincter (LES), absent or disorganized peristalsis and, frequently, increased pressure of the LES.

Some clinical features are different in children in whom achalasia is occasionally associated with other diseases and responds poorly to forceful dilation. Due to its rarity and to the obvious difficulties of manometry in children, only a few cases reported in the pediatric literature include data on sphincteric and esophageal body function in this condition and the question arises of whether motor disturbances observed in them are identical to those of adult patients. This study summarizes the experience in achalasia of two centers with particular interest on esophageal problems in children and reviews the stationary manometry data and some preliminary findings on ambulatory manometry.
Table I. Symptoms in 17 cases of achalasia in ch

Patients and methods

We reviewed the 17 patients treated at the Hospital Infantil "La Paz", Madrid, Spain
(n = 10) and at the Hospital Materno-Infantil NaSa de Aranzazu, San Sebastián, Spain
(n = 7) in the last 30 years (1966-1996).

Male-to-female ratio was 2.2 (11 male and 6 female), mean age at the onset of symptoms was 6.9 years (range 6 months to 15 years), and the diagnostic delay was relatively long (20.7 ± 23 months, mean ± SD). The symptoms leading to diagnosis are listed in Table I and were consistent with those observed in other reports. Only in one instance achalasia was diagnosed in a 9 year-old girl treated during 2 years for selective glucocorticoid deficiency and alacrimia ("triple A" or Allgrove's syndrome). Gastrointestinal symptoms in her were identical to those of other patients. Another child with Down's syndrome had symptoms since the age of 6 months. Barium meal revealed in all cases a bird beak distal thinning of a very dilated and aperistaltic esophagus and endoscopy ruled out any organic obstruction.

Standard pull-through manometry of the LES and stationary evaluation of esophageal body function were carried but in 13 patients according to previously reported techniques [1] using different equipments that evolved along the years. In all we measured the LES pressure, the presence and/or completeness of deglutitory relaxations and the presence and proportion of peristaltic contractions of the esophageal body. In the last 3 patients circadian esophageal body function was also evaluated by means of ambulatory 24-hour manometry according to techniques described in detail elsewhere [2]. We analyzed data on the total number of motor sequences, the proportion of peristaltic waves and the proportion of those that were ineffective (low amplitude, non-progressive and/or incomplete).
Table II. Results of manometric studies in child

Figure 1. Stationary recording in an 11-year-old-boy. Channels: 2, deglutition, 3, mid esophageal body, 4, LES, 5, stomach and 6, respiration. Only minimal sphincteric relaxation, with resting pressures above 10 mmHg, after deglutitions d(7), d(S) and d(9).
428f1

Figure 2. Rapid pull-through recording in a 13-year-old patient who had high-pressure LES (1) and in a 5-year-old boy who had normal LES pressure (2). None of them had deglutitory relaxations (E = sphincter).
428f2

Six patients were treated with forceful dilatations under anesthesia (11 sessions in all) although all but one had to undergo, aside with the remaining 10 children, transabdominal esophageal myotomy accompanied or followed (1 case) by an antireflux procedure in 12/16 (2 Nissen, 10 Thal) in order to prevent postoperative gastroesophageal reflux (GER).
Table III. Ambulatory esophageal manometry in th

Figure 3. Esophageal body tracings in a 3-year-old patient who had a clinical picture of dysphagia and regurgitation, a typical radiological picture of achalasia and a pattern of non-progressive vigorous con-tractions. Channel 3 = upper esophagus, channel 4 = mid esophagus and channel 5 = lower esophagus.
428f3

Figure 4. 6-h segment of the twenty four-hour ambulatory manometry tracing of patient from Figure 1. He had only 192 motor sequences in 24 hours and they were uniformly non propulsive. This part of the tracing shows the complete absence of motor sequences during the night. Upper tracing = upper esophagus, center tracing = middle esophagus and lower tracing = lower esophagus.
428f4

Results

All patients but one (including the child with Allgrove's syndrome) had the same functional pattern at diagnosis consisting of lack or incompleteness of LES relaxation (Figure 1) and practical absence of peristaltic waves (in 9/12 there were no waves at all) in the esophageal body. LES pressure was above 35 mmHg in only 4/13, the highest one being 80 mmHg (Table II and Figure 2). In only one case we could appreciate a pattern consistent with that of vigorous achalasia in adults: achalasic sphincter and very strong (maximum amplitude of 102.8 mmHg) but non-progressive waves in the esophageal body (Figure 3). As far as the circadian function is concerned, a uniform dismotility pattern was documented in the three patients studied who had very scarce motor sequences in 24 hours and almost no peristaltic movements or no waves at all (Table III and Figure 4).

One patient responded dramatically to forceful balloon dilation (precisely the one with vigorous achalasia pattern) but all the remaining children had to be operated upon after several trials with inconsistent temporary relief. The results of surgery were uniformly satisfactory since immediate relief of dysphagia was obtained. However, most patients continued to suffer occasionaly from episodes of mild dysphagia in the long-term follow-up. pH-monitoring testing of the antireflux barrier showed that all 4 patients in whom an antireflux procedure had not been added to the myotomy, and the one cured by forceful dilation, have excessive acid exposure. In one, we had to perform a secondary Nissen fundoplication, and the remaining two, having only mild symptoms, are not willing to accept additional procedures.

Discussion

Achalasia is rare in infancy and childhood and only relatively short series from individual institutions [3-19] and two collective reviews (one worldwide including 175 children [20] and the other limited to Switzerland with 16 cases [21]) have been reported. The clinical symptoms described suggest that, irrespective of the age of onset, the functional disturbances might be similar to those of adult patients. However, only some reports contain manometric data [6, 8, 10, 11, 13, 16-20, 22], very rarely detailed [10, 20], making difficult to assess to which extent the disorder is the same.

Our own data demonstrate that all patients studied manometrically (including the one with "triple A" association) showed the cornerstone sphincteric disturbance found in adults, i.e. an incomplete or absent relaxation during deglutition. Not all patients had increased sphincteric pressure at rest as it has been reported in adult clinical material, but the absolute value of this finding is questionable in view of the evolving equipment and techniques used for manometric studies along so many years. As far as the absence of peristaltic waves in the esophagus is concerned, our patients behaved also as adults by this respect since not a single one produced organized, progressive waves. The finding of a vigorous pattern in one case further illustrates that the motor disturbance is very probably the same in adults and children. The dramatic result of forceful balloon dilation of the cardia in this single patient (by the way, quite unique in the pediatric literature) does not authorize speculation on a particularly favorable response associated with this pattern but it is obviously intriguing.

 

In conclusion, according to our experience, the sphinteric and esophageal motor patterns observed in children with achalasia are identical to those well known in adults. Taking into account the usual long delays before diagnosis and the relatively late onset common in the pediatric form of the disease, it seems reasonable to admit that we are probably dealing with the same and identical disturbance.

Acknowledgements

Prof. A. Delgado, Dr. J. Albisu and Dr. P. Fernandez referred their patients to us for confirmation of diagnosis and treatment. Our nursing staff made the collection of functional data possible with outstanding skill and patience. We acknowledge their contribution and thank them all very warmly.

This work was supported in part by FIS 84/1049, 90/572 and 94/286 Grants.

References

1. Arana J, Tovar JA. Motor efficiency of the refluxing esophagus in basal conditions and after acid challenge. J Pediatr Surg 1989;24:1049-1054.

2. Tovar JA, Diez Pardo JA, Murcia J, Prieto G, Molina M, Polanco I. Ambulatory 24-hour manometric and pH metric evidence of permanent impairment of clearance capacity in patients with esophageal atresia. J Pediatr Surg 1995;30:1224-1231.

3. Sigge W. Hellersche Myotomie und Thalsche Fundic Patch Operation zur Behandlung der Kardiaachalasie des Kindes. Z Kinderchir 1976;18:44-55.

4. Soulier Y, Lefort J, Borde J, Valayer J. Le mégaœsophage idiopathique de l'enfant. A propos d'une série de 27 cas opérés. Ann Chir Inf 1979;20:311-316.

5. Azizkhan RG, Tapper D, Eraklis A. Achalasia in childhood: a 20-year experience. J Pediatr Surg 1980;15:452-456.

6. Ballantine TVN, Fitzgerald JF, Grosfeld JL. Transabdominal esophagomyotomy for achalasia in children. J Pediatr Surg 1980;15:457-461.

7. Saied H, Gharbi H, Hentati M. Le mégaœsophage idiopathique chez l'enfant. A propos de 8 observations. Ann Chir Inf 1980;21:309-312.

8. Höllwarth M, Graf D, Foter R: Megaösophagus. Differentialdiagnose und Therapie. Z Kinderchir 1981;32:37-46.

9. Vaysse P, Guitard J, Moscovici J, Cao Van C, Juskiewenski S. Mégaœsophage par achalasie chez l'enfant. A propos de 7 observations. Chir Pediatr 1982;23:81-86.

10. Berquist WE, Byrne WJ, Ament ME, Fonkalsrud EW, Euler AR. Achalasia: diagnosis, management and clinical course in 16 children. Pediatrics 1983;71:798-805.

11. Koch A, Bettex M, Tschäppeler H, König W. Die Funktion des Ösophagus nach Kardiomyotomie bei der Kindlichen Achalasie. Z Kinderchir 1983;38:206-210.

12. Buick RG, Spitz L. Achalasia of the cardia in children. Br J Surg 1985;72:341-343.

13. Lemmer JH, Coran AG, Wesley JR, Polley TZ Jr, Byrne WJ. Achalasia in children: treatment by anterior esophageal myotomy (modified Heller operation). J Pediatr Surg 1985;20: 333-338.

14. Donahue PE, Samelson S, Schlesinger PK, Bombeck CT, Nyhus LM. Achalasia of the esophagus. Treatment controversies and the method of choice. Ann Surg 1986;203:505-511.

15. Volpi ML, Perlasca E, Balossi P, Cerizzi A. Megaesophagus in childhood. Considerations on 4 cases. Minerva Pediatr 1986;38:1065-1068.

16. Nakayama DK, Shorter NA, Boyle JT, Watkins JB, O'Neill JA Jr. Pneumatic dilation and operative treatment of achalasia in children. J Pediatr Surg 1987;22:619-622.

17. Vane DW, Cosby K, West K, Grosfeld JL. Late results following esophagomyotomy in children with achalasia. J Pediatr Surg 1988;23:515-519.

18. Nihoul-Fekete C, Bawab F, Lortat-Jacob S, Arhan P, Pellerin D. Achalasia of the esophagus in childhood: surgical treatment in 35 cases with special reference to familial cases and glucocorticoid deficiency association. J Pediatr Surg 1989;24:1060-1063.

19. Allen KB, Ricketts RR. Surgery for achalasia of the cardia in children: the Dor-Gavriliu procedure. J Pediatr Surg 1992;27:1418-1421.

20. Myers NA, Jolley SG, Taylor R. Achalasia of the cardia in children: a worldwide survey. J Pediatr Surg 1994;29:1375-1379.

21. Illi OE, Stauffer UG. Achalasia in childhood and adolescence. Eur J Pediatr Surg 1994;4:214-217.

22. Emblem R, Stringer MD, Hall CM, Spitz L. Current results of surgery for achalasia of the cardia. Arch Dis Child 1993;68:749-751.

 


Publication date: May 1998 OESO©2015