Swallowing Disorders After Esophageal Atresia Evaluated With Videomanometry

By Madeleine Montgomery, Hanne Witt, Richard Kuylenstierna, and Bjijrn Frenckner Stockholm, Sweden

Background: Patients with repaired esophageal atresia often show persistent motility disorders of the esophagus.

Methods: In this study, the authors used a newly developed method, videomanometry, to study intraluminal pressures and function of the pharynx and upper esophagus in such a group of patients and compared the results with those in healthy age-matched controls.

Resu/ts:A significant difference was found between timing of the pharynx contraction and upper esophageal sphincter (UES) relaxation, indicating a dyscoordination of swallowing

in the patient group. Transit time of bolus from the pharynx to the esophagus was also shorter for the patient group. Such dyscoordination may be a risk factor that can cause aspira- tion and respiratory symptoms.

Conclusion: UES resting pressure and residual pressure on swallowing, did not differ between the two groups. J Pediatr Surg 33:1219-1223. Copyright o 1998 by W.B. Saunders Company.

INDEX WORDS: Esophageal atresia, manometry, upper esophageal sphincter.

E SOPHAGEAL DYSFUNCTION has been docu- mented by several investigators after successful

repair of esophageal atresia (EA).‘-‘j Symptoms like dysphagia, regurgitation, and respiratory complications are common in patients treated for EA.7,s Motility disorders of the esophagus may be present in both symptomatic and asymptomatic patients. Abnormalities may involve peristalsis of the whole esophagus. Function of the upper esophageal sphincter (UES) has been described as norma12J or almost normal.3,9 Although the etiology of the esophageal dysfunction is not understood, it is at least partly caused by the congenital malformation, because dysfunction has been noted preoperatively in patients with EA,9 and in patients with a tracheoesopha- geal fistula without atresia. lo

Swallowing disorders can be evaluated by different methods such as dynamic cineoradiography and videora- diography, manometry, scintigraphy, ultrasonography, and fiberoptic evaluation. Extensive examination of the motil- ity patterns of the pharynx and esophagus, including evaluation of airway protection and aspiration after barium contrast swallow, can be assessed using a videora- diographic recording during simultaneous solid-state in- traluminal manometry of the pharynx and upper esopha- gus, ie, videomanometry. 11-13 This technique has a number of advantages compared with conventional radiography or manometry. Videomanometry provides both qualita- tive and quantitative information on swallowing, and it can be performed with the patient in an upright physiologi- cal position, eliminating the discomfort of supine swallow- ing and the continuous flow from a perfused manometry catheter.

The aim of this study was to use videomanometry to investigate lower pharynx, UES, and esophageal func- tions. The subjects were adults who had undergone repair of EA with tracheoesophageal fistula in the newborn period and age-matched healthy controls.

MATERIALS AND METHODS

The study protocol was approved by the local Ethics Committee of Karolinska Hospital and Institute. All patients and volunteers gave their informed consent to participation in the study before inclusion.

Eleven patients (aged 23 to 40 years; median, 29 years) who had undergone repair of EA with tracheoesophageal fistula in the neonatal period in Stockholm (Karolinska or Crown Princess Lovisa, St. C&an’s Hospital) entered the study. The patients answered a questionnaire concerning swallowing, dysphagia. gastroesophageal reflux, and respi- ratory symptoms. The control group consisted of 11 healthy volunteers (aged 21 to 42 years: median, 26 years). The healthy controls denied reflux symptoms or dysphagia, and none of them had undergone surgery on the pharynx or the esophagus.

Videomanometry of the Pharynx and Esophagus

The investigation was performed with the subject in an upright sitting position and with videorecording in a lateral projection. The manometry catheter was introduced through the nose, with a small amount of

From the Departments of Pediatric Surgery, Diagnostic Radiology, and Oto Rhino Laryngology, St. Giiran’s/Karolinska Hospital, Karolin- ska Institute, Stockholm, Sweden.

This study was supported by grants from the Crown Princess Lovisa Foundation, “Fdrenade Liv” Mutual Group Life Insurance Company, Stockholm, Sweden and the Swedish Society of Laryngectomees.

Address reprint requests to Madeleine Montgomery, MD, Department of Pediatric Surgery, St. Gtiran WKarolinska Hospital, S-11281 Stock- holm, Sweden.

Copyright o 1998 by WB. Saunders Company 0022-3468/98/3308-0007$03.00/O

Journal of Pediatric Surgery, Vol33, No 8 (August), 1998: pp 1219-1223 1219

1220 MONTGOMERY ET AL

anesthetic lubricant (Xylocain, Astra, Sweden) in the nostril. A solid- state catheter was used (Konigsberg Instruments Inc, Pasadena, CA), which had four pressure transducers, placed 2 cm apart. The two distal sensors were circumferential to allow 360” measurements. The analog signal was converted to a digital signal (Polygraf, Synectics, Stock- holm, Sweden). The data were analyzed with a personal computer using the Polygram Upper-GI-Edition by Gastrosoft InclSynectics Medical (Synectics).

The system was calibrated at room temperature before examination of each individual patient. Initially, the distal sensor was positioned in the UES, which was determined manometrically (Fig 1). The exact position was confirmed and recorded using videoradiography (Fig 2). Intermittent fluoroscopy was used to ensure that the probe position did not change. Each subject swallowed three to five boluses of 10 mL, 60% weight volume barium contrast. Recordings were taken of both the wet swallow (with contrast) and dry swallow (without contrast). With each swallow, the following manometric and radiographic parameters were simultaneously recorded and measured: (1) UES resting pressure (the pressure before and after swallowing), (2) LIES residual pressure during wet and dry swallows, (3) duration of UES relaxation, (4) PC1 (inferior pharynx constrictor) contraction pressure, and (5) timing between the contraction of the PC1 (beginning of the contraction) and the UES relaxation (beginning of the relaxation). The catheter was then moved so that the uppermost transducer was positioned in the UES and the three distal transducers were located in the esophagus. The following parameters were then studied: (1) propagation of the contraction wave (cm/s) from the UES to the upper esophagus (2 cm below the UES) and (2) amplitude of the pressure wave in the upper esophagus.

Presence or absence of misdirected swallowing or aspiration was studied by radiography. The transit time of the bolus from the pharynx to the esophagus (the time interval between passing the faucal isthmus until passage of the UES) was documented with radiography.

Fig 1. Example of a normal videomanometry. The third sensor is in the PCI and the fourth sensor is located in the UES. When peristaltic contraction occurs in PCI, relaxation must be complete in UES. The arrows denote beginning of the contraction of PCI and the beginning of relaxation of UES.

Fig 2. Example of a pathological recording. Time interval between PCI contraction and UES relaxation was 260 ms in this particular patient.

Statistical Analysis

Nonparametric statistics (Mann-Whitney U test) were used for the statistical analysis. A P value of less than .05 was considered significant. The results were expressed as medians and ranges.

RESULTS

Clinical Symptoms

The symptoms reported by the 11 patients with re- paired EA are shown in Table 1. Dysphagia, defined as symptoms (discomfort or pain) arising in conjunction with the propagation of the bolus, occurred in 8 of the 11 patients. However, three had only mild swallowing problems, five had moderate, but none had severe prob-

Table 1. Svmptoms in 11 Patients Wiih Repaired EA

Pneumonia Reflux HOtlWXlC3SS, (No. of

Patient Age Symptoms Nocturnal Epwdes NO. (yr) Dysphagia Regurgitation Asthma Cough Last 5 yrl

1 26 No 2 40 Moderate

3 23 Moderate 4 29 Moderate 5 27 Moderate 6 37 Moderate 7 34 Mild

8 23 Mild 9 36 Mild

10 23 No 11 30 No

No No

Yes No No*

Yes Yes

Yes Yes No No

No Yes

No No Yes

No No

No No

No No

No 1 Yes 0

No 0

No 1 Yes 3 >3

No 0 No 0

No 1

No 0

No 0 No 0

*This patient had no current symptoms of reflux, but had previously undergone antireflux surgery.

VIDEOMANOMETRY AFTER ESOPHAGEAL ATRESIA 1221

lems. Some of the patients had to eat very slowly and avoid certain kinds of food that tended to get stuck in the esophagus. They also had to drink large quantities of liquids with each meal to facilitate swallowing. About half of the patients had symptoms of reflux, like regurgi- tation or heartburn. Asthma and respiratory symptoms occurred in only two patients. Two patients complained of hoarseness or nocturnal cough, indicating reflux.

Videomanometry An example of a normal videomanometry recording is

shown in Fig 1 and another example of a pathological recording is shown in Fig 2. Figure 3 shows a lateral radiograph of the neck with correct positioning of the manometric sensors. The results of the videomanometric

Fig 3. Lateral radiography of the neck shows the correct position- ing of the four manometric sensors. The fourth sensor is in the UES, which is located in the region of the cricoid cartilage (cricopharyngeal musclel.

Table 2. Results of Videomanometry in 11 Patients With Repaired EA Compared With Healthy Controls

Patients Controls

UES resting pressure (mm Hg)

UES residual pressure, wet

(mm Hg) UES residual pressure, dry

(mm Hg) Duration of UES relaxation

(msl Pharynx constrictor pressure

(mm Hg) Timing PCI contractlon, UES

relaxation (ms) Propagation UES, upper

esophagus (cm/s) Transit time from pharynx to

esophagus (ma.)

65 (52.8-120)

-0.9 c-9.7-6.4)

-1.5(-13x-4.7)

685 (327-788)

304 (148464)

327 (228-550)

1.6 (1.42.2)

66.5 (54.8-99.5)

84.8(49-122) NS

2.3 C-3.9-4.2) NS

0.9 (-5.1-1.8) NS

673(535-T/5) NS

293 (195433) NS

508048-593) *

1.8 (1.3-2.2) NS

79.5 (65-87.6) *

‘Significant.

examination are shown in Table 2. There was no differ- ence between the UES resting pressures, residual pres- sures or the duration of the UES relaxation between patients and controls. There was a significant difference between the timing of beginning of the contraction of the PC1 and relaxation of the UES. Timing values were lower in the patients, indicating that the UES relaxes closer to PC1 contraction in the patient group. However, the contraction pressure of PC1 in the patient group was similar to that in the control group.

Propagation of contraction from the UES to the proximal esophagus (2 cm below the UES) did not differ between patients and controls. There was no difference in the amplitude of the pressure wave at this level. Below this level, all the patients showed disturbed motility with a lack of coordination of peristalsis or no peristaltic waves at all. The bolus transit time from the pharynx to the esophagus was significantly shorter in the patient group. Radiographic examination findings showed no case of aspiration.

DISCUSSION

Videomanometry is a recently developed technique that permits simultaneous recordings of pressures and function of the pharynx and upper esophagus.11J2 Solid- state transducers and computerized analysis give reliable recordings of intraluminal pressures. Esophageal motility disorders after EA have been studied with solid-state transducer manometry by Tovar et al6 Videomanometry has not been used before to study swallowing disorders in patients with repaired EA. By using simultaneous fluoros- copy, correct transducer positioning can be maintained during the examination.

The main finding of this study was a difference in timing between contraction of the pharynx and relaxation of the UES, indicating a lack of coordination of swallow-

1222 MONTGOMERY ET AL

ing in patients with repaired EA. The time interval between PC1 contraction and UES relaxation was signifi- cantly shorter in the patients. The transit time of the bolus from the pharynx to the esophagus, in accordance with this observation, was also shorter in the patients. Relax- ation of the UES normally starts before contraction of the PCI begins, and the relaxation must be complete and of sufficient duration. Shorter time intervals indicate that UES relaxation starts closer to PC1 contraction. This could be a potential risk for a misdirection of the bolus that precipitates aspiration. However, in this study, we saw no sign of penetration to the laryngeal inlet or aspiration on the radiography of any of the patients. However, some patients described symptoms, such as hoarseness or nightly cough, which strongly indicate reflux and aspiration at night in the supine position. Such symptoms occurred only in patients who had inadequate timing between pharyngeal contraction and UES relax- ation. Dysphagia, which was the most common symp- tom, occurred in most patients and showed no correlation to a lack of coordination of swallowing.

patients except two, in whom the relaxation was incom- plete. The upper esophageal segment showed total motor incoordination in all the patients.

In our study, we found normal resting pressures and residual pressures of the UES in the patient group compared with the healthy controls. We also observed normal propagation of the peristaltic wave from the UES to the first 2 cm of the upper esophagus. Below this level, motility was poorly coordinated or absent, in accordance with many previous studies.

Many investigators have reported esophageal dysmotil- ity in patients with repaired EA. Aperistalsis, antiperistal- sis, and simultaneous or uncoordinated contractions have been reported. Manometric studies have shown abnormali- ties of the motility in the whole esophagus. However, Burgess et al2 and Shepard et aI5 described an amotile area of the esophagus in the proximity of the anastomosis but normal function of the UES and LES (Lower esophageal sphincter), supporting the view that the operation itself caused the motility disorder. Duranceau et al3 found mostly normal UES function, normal resting pressure, and normal coordination between the pharynx and UES, but in some cases incomplete relaxation of the UES. However, they used a water-perfused catheter, which is widely accepted as less useful in the pharyngo- esophageal segment than the method used in this study.rl-r3 Romeo et al9 studied the motility pattern of the upper esophageal segment preoperatively in 16 newborn pa- tients with EA, with or without fistula. They found normal relaxation and coordination of the UES in all

The etiology of the motility disorders after EA repair remains unclear. Some data indicate a congenital origin, such as disturbed motility seen preoperatively in patients with EA9 or in patients having tracheoesophageal fistula without EA.‘O This opinion is supported by experimental studies in animals, in which transection and anastomosis of the esophagus did not cause motility disorders of the esophagus, as seen in patients with repaired EA.14 Nakazato et alI5 found an abnormal Auerbach plexus in the esophagus and stomach of patients with EA and tracheoesophageal fistula, indicating a congenital func- tional impairment of the upper gastrointestinal tract. However, it has been suggested that extensive mobiliza- tion and denervation of the esophageal segments could aggravate reflux and motility disorders.16 Shono et all7 showed normal peristaltic activity preoperatively in the proximal esophagus in two patients who had EA without a fistula. One patient examined postoperatively showed a disturbed motility pattern. They concluded that the dissec- tion and mobilization during the operation is the major cause of esophageal dysmotility in patients with repaired EA. This conclusion is based on the assumption that the etiology of various types of EA is similar, which is controversial. Kluth and Habenicht’* believe that EA without a fistula and other rare types are of a secondary origin, whereas the usual type of EA with the fistula between the trachea and the distal esophagus represents a primary foregut malformation.

This study shows that the UES resting and residual pressures were normal in adult patients with repaired EA compared with healthy controls. An important finding was a dyscoordination of swallowing in the patient group. The time interval between pharynx contraction and UES relaxation was shorter in patients with repaired EA. The patients also showed a significantly shorter transit time of the bolus from the pharynx to the esophagus. Such poor coordination constitutes a potential risk factor, which could cause aspiration and secondary respiratory compli- cations. The etiology of this disturbance is unclear, but is more likely to be of congenital origin than caused by surgery, because mobilization of the esophageal seg- ments during the operation would not affect the pharyngo- esophageal region. Further investigations are needed in this field.

REFERENCES 1. Lind JF, Blanchard RJ, Gudha H: Esophageal motility in tracheo-

esophageal fistula and esophageal atresia. Surg Gynecol Obstet 123:557- 564,1966

2. Burgess JN, Carlson HC, Ellis FH: Esophageal function after

successful repair of esophageal atresia and tracheoesophageal fistula. A manometric and tine fluorographic study. .I Thorac Cardiovasc Surg 56:667-673, 1968

3. Duranceau A, Fisher SR, Flye MW, et al: Motor function of the

VIDEOMANOMETRY AFTER ESOPHAGEAL ATRESIA 1223

esophagus after repair of esophageal atresia and tracheoesophageal fistula. Surgery 1:116-123, 1977

4. Werlin SL, Dodds WJ, Hogan WJ, et al: Esophageal function in esophageal atresia. Dig Dis Sci 26:796-800, 1981

5. Shepard R, Fenn S, Sieber EK: Evaluation of esophageal function in postoperative esophageal atresia and tracheoesophageal fistula. Surgery 59:608-617, 1966

6. Tovar JA, Diez Pardo JA, Murcia J, et al: Ambulatory 24-hour Manometric and pH metric evidence of permanent impairment of clearance capacity in patients with esophageal atresia. J Pediatr Surg 30:1224-1231

7. Parker AF. Christie DL, Cahill JL: Incidence and significance of gastroesophageal reflux following repair of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 14:5-8, 1979

8. Dudley NE, Phelan PD: Respiratory complications in long-term survivors of esophageal atresia. Arch Dis Child 51:279-282, 1976

9. Romeo G, Zuccarello B, Proietto F, et al: Disorders of the esophageal motor activity in atresia of the esophagus. J Pediatr Surg 22:120-124, 1987

10. Johnston PW, Hastings N: Congenital tracheoesophageal fistula without esophageal atresia. Am J Surg 112:233-240.1966

11. Caste11 JA, Dalton CB, Caste11 DO: Pharyngeal and upper esophageal manometxy in humans. Am J Physiol258:G173-G178,1990

12. Olsson R, Nilsson H, Ekberg 0: Simultaneous videoradiography and pharyngeal solid state manometry (videomanometry) in 25 nondys- phagic volunteers. Dysphagia 10:36-41, 1995

13. Caste11 JA, Dalton CB, Caste11 DO: Effects of body position and bolus consistency on the manometric parameters and coordination of the upper esophageal sphincter and pharynx. Dysphagia 5:179-186, 1990

14. Haller JA, Brooker AF, Talbert J, et al: Esophageal function following resection: Studies in newborn puppies. Ann Thorac Surg 2:180-187,1966

15. Nakazato Y, Landing BH, Wells TR: Abnormal Auerbach plexus in the esophagus and stomach of patients with esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 21:831-837.1986

16. Orringer MB, Kirsch MM, Sloan H: Long-term esophageal function following repair of esophageal atresia. Ann Surg 186:436-443, 1977

17. Shono T, Suita S, Arima T, et al: Motility function of the esophagus before primary anastomosis in esophageal atresia. J Pediatr Surg 28:673-676,1993

18. Kluth D, Habenicht R: The embryology of usual and unusual types of esophageal atresia. Ped Surg Int 2:223-227,1987