surgery in oculopharyngeal muscular dystrophy
TRANSCRIPT
Surgery in Oculopharyngeal Muscular Dystrophy
Andre? Duranceau, MD, Montreal, Canada
Marcelle D. Forand, BSc, Montreal, Canada
Jean P. Fauteux, MD, Montreal, Canada
The oculopharyngeal muscular dystrophy syndrome wa3 transmitted in an autosomal dominant fashion
through 11 generations of French Canadians. In Barbeau’s study of more than 160 patients, the mode
of presentation was palpebral ptosis and orophar- yngeal dysphagia. Because the nature of the disorder is unknown, the patients were often diagnosed as having cervical esophageal cancer, and they fre-
quently died of starvation or from repetitive aspira- tion [2,21.
Oropharyngeal dysphagia is usually progressive and starts at about age 40 years. This dysphagia is responsible for repetitive pharyngooral and phar- yngonasal regurgitations. Alimentation time is in-
creased and weight loss frequently occurs. In the advanced stage of the disease laryngeal, tracheo- hrcmchial and pulmonary symptoms predominate,
resulting from the aspiration of food at mealtime and of saliva during t.he night [9].
A number of esophageal motility studies were
performed in patients with this disorder, with various
results and interpretations [.‘j-IO]. Poor contraction
of i.he pharynx with normal or abnormal function of
the upper esophageal sphincter was described. Sur- gery for oropharyngeal dysphagia related to this disorder was also reported in several cases [3,~1,7,N,ll.I~]. Th e condition of most patients is
improved by cricopharyngeal myotomy.
‘I’he purposes of this study are (1) to document
dysfunction of the pharynx and upper esophageal sphincter in patients with oculopharyngeal muscular dys,trophy, and (‘L) to document the effects of surgery on the function of’ these areas.
From the Department of Surgery, Universit6 de MontrBal, HBtel-Dieu de Monk&l, Quebec, Canada.
Reprint requests should be addressed to Andre Duranceau, MD, Depart- ment of Surgery, HBtel-Dieu de Montr6al3840, St-Urbain, Mont&al. Qukbec H2VI’ IT& Canada.
Presented at the 20th Annual Meeting of the Society for Surgery of the Alimentary Tract, New Orleans, Louisiana, May 22-23, 1979.
Material and Methods
Motor function of the pharynx and upper esophageal sphincter was studied in 15 patients with oculopharyngeal
muscular dystrophy and in 15 asymptomatic volunteers. The results in both groups were initially compared, and
then the oculopharyngeal muscular dystrophy group was
used as its own control before and after cricopharyngeal
myotomy. The oculopharyngeal muscular dystrophy group included six men and nine women aged 50 to 74 years (mean 63.9). The control group included 10 men and 5
women aged 20 to 41 years (mean 26.6). Manometric Evaluation. Esophageal motility studies
were performed using a 55 cm triple lumen polyethylene tube (USC1 no. 1100). All three lumens had an internal diameter of 20.32 mm (0.08 inch) and ended in a lateral
position 5 cm from each other with an orientation of the orifice at 120” one from each other.
The lumen recording pharyngeal function was water- filled but not perfused. When recording the upper esoph- ageal sphincter area the lumen was perfused at a rate of 7.5
cc/min and pressures were recorded in an anteroposterior position. A Harvard pump (model 954) was used for per- fusion.
Pressures from the columns of water within the lumen
were transmitted to external transducers (Hewlett/Pack- ard model 1280) and recorded on a four channel physiog-
raph (Hewlett/Packard model 7754A). Pressures were re- corded in mm Hg with atmospheric pressure as the 0 ref- erence point. Manometric calibration was performed before and after each recording, and no medication was given before any of the studies.
Manometric Interpretation. Once the pharyngeal and upper esophageal sphincter areas were located manome- trically, they were studied for 10 consecutive swallows. Pharyngeal function was examined for the peak, the du- ration and the frequency of its contraction. The upper esophageal sphincter area was studied for its resting and contracting pressure, relaxation, relaxation time and coordination. Relaxation was interpreted as normal when maximal relaxation was within 5 mm Hg of resting cervical esophageal pressure. Total relaxation time was recorded. The upper esophageal sphincter was considered coordi-
Volume 139. January 1980 33
Duranceau et al
Figure 1. Finction of the pharynx. OPMD = oculopharyngeat muscular dystrophy.
nated .with pharyngeal contraction when peak pharyngeal contraction corresponded to the nadir of upper esophageal sphincter relaxation and when upper esophageal sphincter relaxation entirely encompassed the time of pharyngeal contraction. A statistical analysis of the variables studied by two observers were computed and compared with the control group using Student’s t test for unpaired values. The variables observed in the patients with oculopharyn- geal muscular dystrophy before and after surgery were computed and averaged using Student’s t test for paired values. P <0.05 was considered statistically significant.
Surgery. All 15 patients with oculopharyngeal muscular dystrophy underwent cricopharyngeal myotomy. Surgery was performed under general anesthesia through a left cervical oblique incision. The proximal esophagus and the distal pharynx were isolated. A no. 42 mercury bougie was passed through the upper esophageal sphincter area into the cervical esophagus. A 5 cm cricopharyngeal myotomy
including the distal pharynx, the upper esophageal
% % 100
90
80
70
60
50
40
30
20
10
0 NS NS NS NS
34 The American Journal of Surgery
DS DS DS
1 1 1
Figure 2. Pharyngeat function in oculopharyngeal muscular dystrophy (case 73): for a single bolus the contractions are weak, prolonged and must be repeated. DS = dry swallow.
sphincter area and the proximal esophagus was carried out on the longitudinal axis. The mucosa was dissected from the muscular layer over approximately 40 percent of the circumference of the esophagus. The muscular layer was then transected proximally and distally in the transverse axis. The strip of muscle resulting from this transection was then either removed for histologic analysis or fixed ante- riorly to the thyroid capsule. After the procedure, a naso- gastric tube was used for 24 hours. The patient was dis- charged on a soft diet 3 to 4 days after surgery.
Results
Pharynx. Figure 1 illustrates pharyngeal function in the oculopharyngeal muscular dystrophy group before and after cricopharyngeal myotomy and in the
control group. Pharyngeal contraction in a typical patient with oculopharyngeal muscular dystrophy
M yi 25
20
15
10
05
0 1 P
80
70
60
50
40
30
20
10
”
NS PC02 NS P<Ol Figure 3. Function of the upper esophageal sphincter. OPMD = oc- ulopharyngeal muscular dystrophy.
Oculopharyngeal Muscular Dystrophy
DS
UES 40
Figure 4. Function of the upper esophageal sphincter (case 13). As seen in the recording, repetitive relaxations rep- resent attempts of the powerless pharynx to push the bolus through the sphincter area. Only one of the three waves observed in the cervical esophagus is propulsive. UES = upper esophageal sphincter; WS = wet swallow.
is illustrated in Figure 2. All parameters of pharyn-
geal function differed significantly in the two groups
and remained unaffected by cricopharyngeal myot- omy. Peak contraction pressure was 32.1 f 2.1 mm
Hg in the control group but 5.0 f 1.1 mm Hg in the
oculopharyngeal muscular dystrophy group (p <O.OOl). After cricopharyngeal myotomy the mean pressure of the pharynx during contraction was 6.1 f 1.2 mm Hg (difference not significant). The con- traction time in normal subjects (0.467 f 0.047) dif-
fered significantly from that in the oculopharyngeal muscular dystrophy group (I .053 f 0.125 seconds)
(p (0.001). After surgery the contraction tjme in the
oculopharyngeal muscular dystrophy group was 0.833 f 0.094 seconds (difference not significant). The nol,mal group showed a single contraction peak of the pharynx ( 1 .O f 0). With every bolus given, the ocu- lopharyngeal muscular dystrophy patient needed 2.8,?7 f 0.256 pharyngeal contractions to help swal- loming (p <O.OOl). After surgery the patient still needed to swallow 2.733 f 0.248 times for every water bolus (difference not, significant).
ilpper Esophageal Sphincter. Manometric find- ings at the level of the proximal esophageal sphincter are summarized in Figure 3 and illustrated in Figures 4 and 5. IJpper esophageal sphincter function did not difi’er significantly between the oculopharyngeal muscular dystrophy group and the control group.
Pharynx
UES
40
20
0
5 set
Figure 5. Recordings in a patient with oculopharyngeal muscular dystrophy (case 73) show weak contraction of the pharynx, normal function of the upper esophageal sphincter ( UES) and normal initiation of peristalsis in the esophagus. DS = dry s wall0 w.
Cricopharyngeal myotomy significantly lowered
upper esophageal sphincter resting and closing
pressure while relaxation time returned toward
normal values. Resting pressure in t,he cont.rol group (51.13 f 3.84 mm Hg) did not vary significantly from
that in the oculopharyngeal muscular dystrophy group (52.14 f 9.01 mm Hg). Similarly, the con-
traction pressure of the upper esophageal sphincter area was 88.2 f 7.9 mm Hg in the control group and
70.8 f 10.6 mm Hg in the oculopharyngeal muscular dystrophy group (difference not significant). Resting and contraction pressure at the upper esophageal sphincter level were significantly altered by crico- pharyngeal myotomy: resting pressure decreased to 21.46 f 3.87 (p <0.02) while contraction peaked at 32.80 f 5.42 mm Hg (p <O.Ol). Normal relaxation in control subjects (97.33 f 1.53 percent) did not vary significantly from that observed in the oculophar- yngeal muscular dystrophy patients (88.26 f 5.38 percent). After surgery normal relaxation was not significantly altered, with values of 68.0 & 8.81 per- cent. Relaxation time was 1.18 f 0.12 seconds in normal subjects and 2.43 f 0.18 seconds in the ocu- lopharyngeal muscular dystrophy group (p <O.OOl).
35
Duranceau eta1
TABLE I Postoperative Results in Patients With Oculopharyngeal Muscular Dystrophy
Follow- Symptom Weight Regurgitation UP
Case Age (yr) Duration Loss Dysphagia Pharyngooral Pharyngonasal Aspiration Time No. &Sex (yr) (kg[poundsl) Preop Postop Preop Postop Preop Postop Preop Postop (mo)
1 65M 12 0 ++++ ++ ++ + + + + + 23 2 61M 10 0 ++++ 0 ++ 0 +++ 0 ++++ 0 30 3 71F 10 13.6(30) ++++ 0 ++ 0 ++ 0 ++ + 14 4 58F 11 6.8(15) ++++ 0 + 0 + 0 ++++ + 14 5 50F 6 :.8(15) ++++ ++ ++ 0 ++ 0 ++++ 0 32 6 54F 5 ++++ + + 0 ++ 0 ++++ 0 13 7 64M 4 18.2(40) ++++ 0 +++ 0 +++ 0 ++++ 0 29 8 74F 20 0 ++++ 0 + 0 0 0 ++++ 0 22 9 69F 25 9.1(20) ++++ 0 ++ 0 ++ 0 ++++ + 21 10 52F 5 0 ++t+ 0 0 0 t 0 + 0 21 11 68M 13 0 ++++ 0 0 0 t++ 0 ++++ 0 25 12 86M 13 13.6(30) +t++ 0 ++ 0 ++ 0 ++++ 0 19 13 74M 15 18.2(40) +ttt ++ +t 0 ++ 0 ++++ 0 20 14 74F 2 ++++ + + 0 t 0 t+t+ 0 42 15 59F 15
:.8(15) ++tt 0 + 0 ++ 0 ++ + 9
0 = none; -I- = rare; -I+ = moderate; -H-i-i- = severe; preop = preoperative; postop = postoperative.
After surgery relaxation time was brought back toward normal with values of 1.87 f 0.158 seconds, which differed significantly from the preoperative values (p <0.05). Upper esophageal sphincter coor- dination was interpreted as normal in 98.0 f JO.07 percent of the control group and in 94.26 f 2.255 percent of the oculopharyngeal muscular dystrophy patients (difference not significant). After surgery normal cocrdination decreased slightly to 83.8 f 6.4 percent (difference not significant).
Subjective Results of Surgery. Table I summarizes the clinical results after surgery on the upper
esophageal sphincter in patients with oculopharyn- geal muscular dystrophy. The results were obtained 9 to 42 months after surgery for a mean follow-up of 22.2 months. The symptoms were present for 2 to 25 years with a mean duration of 11.06 years. Weight loss was observed in 9 of 15 patients. Four patients had lost 13.6 kg (30 pounds) or more. Dysphagia was controlled in 10 of 15 patients. Three patients con- tinue to have moderate difficulty in swallowing and two still have intermittent dysphagia. Pharyngooral regurgitation occurred in 14 of 15 patients. All pa- tients were significantly helped by the operation. One
Figure 6. Left, preoperative radiologic evaluation of the upper esophageal sphincter area in an advanced case of ocutopharyngeal muscular dys- trophy shows the pseudo-tumor effect of the sphincter; tracheal aspiration can be observed. Right, after crico- pharyngeat myotomy a wide opening of the upper esophageal sphincter area, which lowers resistance to bolus passage, can be observed.
36 The American Journal of Surgery
Oculopharyngeal Muscular Dystrophy
patient occasionally has pharyngonasal regurgitation during meals. Episodes of aspiration at mealtime,
during sleep or during motility studies disappeared
completely after surgery in 10 of 15 patients. Two patient,s continue to have episodes of aspiration during meals, and three patients have occasional episodes of salivary aspiration during the night. The
radiologic findings before and after surgery are shown
in Figure 6.
Comments
‘i’he natural history of oculopharyngeal muscular
dy:,trophy was initially described in 1915 by Taylor I13], who realized that the condition eventually leads
to ,Seath by starvation or aspiration if the patient reaches the fifth decade of life. The largest concen- t ration of’ patients with this disorder is in the prov- ince c)t’ Que bet [I ,2]. Oculopharyngeal muscular
dystrophy also occurs in patients of other ethnic or- igins 11 -.‘I, IO].
The clinical presentation of the patient with ocu-
lopharyngeal muscular dystrophy is usually charac-
t er ized by dysphagia and ptosis of the eyelids. These
syrnpt.oms begin around age 40 years and progress slo*;vly. Bilateral progressive ptosis is usually the first
symptom, hut dysphagia may appear before the ptclsis. The oropharyngeal dysphagia is initially ob-
served with dry solids (vegetables, fruit and meats), and af’ter several years the patient notices dysphagia with liquids. In more than half of our initial group this dysphagia increased when the patient felt, anx-
ious or drank cold liquids. Pharyngooral and phar- yngonasnl regurgitations then appear, signaling the incompetence of the velopharyngeal muscles. These symptoms emharass t.he patient, and he often stops eating in public or even with his family. Alimentation
time increases significantly and weight loss ensues.
The abnormalities in transport through the pharynx
and upper esophageal sphincter lead to repetitive bouts of aspiration. Tracheobronchial symptoms are
ini-:ially intermit~tent, although in severe cases tra-
cheal soilage occurs at every meal. Aspiration also occ urs at night: stasis of food particles and saliva in t hr pharynx and larynx during sleep leads to chronic aspiration. Aspiration pneumonia was observed in L’o~r of’ our patients, all of whom had severe symp- tor?s. This syndrome has been identified through 11 generut.ions of French Canadians, both male and f’e- male II-~.‘1,.3,10, IYJ. All patients are traceable t,o the same two ancestors, and the children are affected in half’ of’ thp cases. Oculopharyngeal muscular dys- trophy is a purely autosomal dominant trait.
Motility studies have been performed in patients with oculopharyngeal muscular dystrophy using
various techniques, with great differences in the in-
terpretation of results. In some studies the tracings
are interpreted as normal [4,5,Y]. Marked weakness of the striated muscle in the upper esophagus is de-
scribed hy some. Other studies report weakness of pharyngeall contraction, low cricopharyngeal basal
pressure and normal function of the upper esopha-
geal sphincter [ 7). Differences in recording t.ech- niques probably explain the differences in reported
observations. In 1968 Murphy and 1)rachman 151
reported motility studies performed in three patients. In two of those patients pharyngeal t’rmct ion was not
mentioned and upper esophageal sphincter function was considered normal. The author ~~~nc~lucles that only the pharynx, the hypopharynx and t,he upper third of the esophagus were afl’ect.ed. Lewis [s]
Bender IX] and Roberts and Bamfort h IIo] reported complete motor paralysis of the pharynx. In their studies pressures at the level of the upper esophageal sphincter were considered normal Or tlec,reased. Sphincter contraction was interpreted as lweak or
absent. Bender noted severe relaxat.ion and coordi-
nation problems in one patient. Our study was designed to accurately measure
motor function of both the pharynx and the proximal esophageal sphincter. In patients with oculophar-
yngeal muscular dystrophy, peak pharyngeal con- traction was significantly weaker than in the control group. These contractions were of’ longer duration and always repetitive. Because of’ the veloc.ity and
amplitude of movement in the pharynx, the non-
perfused system used in our study I~tlderest,imates
these pressure abnormalities. Manometric recording 01’ upper esophageal
sphincter function was performed with a rapid per-
fusion technique. When compared with I he control
group, upper esophageal sphincter function was normal in the oculopharyngeal muscular dystrophy
group. Resting pressure, contracting pressure, coordination and relaxation did not differ signifi- cantly in the two groups. Relaxation time was longer in the oculopharyngeal muscular dystrophy group than in the normal group. ‘Theoretically a clear pharyngeal pressure signal is necessary to initiate relaxation of the upper esophageal sphinct*er. The results in this study show that even a weak pharyn- geal contraction causes normal relaxation of’ the upper esophageal sphincter. ‘l’he patient,s with ocu- lopharyngeal dystrophy had difficulty clearing the pharynx and larynx of their cont.ents arid they did so by repeated swallowing. Function of’ thr upper esophageal1 sphincter was normal, hut transport through th.e upper sphincter zone was altered becbause of t,he powerless pharynx.
Volwne 139. January 1990 37
Duranceau et al
Cricopharyngeal myotomy is known to decrease resting sphincteric pressures in patients with Zenk-
er’s diverticula [14]. Surgery on the upper esophageal sphincter has been reported in a small number of
patients with oculopharyngeal muscular dystrophy.
Peterman et al [4] referred to one patient of French Canadian origin who underwent cricopharyngeal
myotomy and obtained 10 months of complete relief of symptoms. Dysphagia recurred and a second my-
otomy using a contralateral approach was performed with no significant benefit. In 1971 Montgomery and
Lynch [22] reported on eight patients affected with
the same condition. Every patient presented with severe dysphagia and underwent extensive myotomy of the proximal esophageal sphincter area. In their
well illustrated paper the authors describe the sharp dissection of the muscularis from the mucosa of the
cervical esophagus. The muscle is then cut
transversely, proximally and distally and sutured
anteriorly. The follow-up in these eight patients was 18 months to 4 years, and the results were uniformly
excellent with complete relief of dysphagia. Aspira- tion disappeared clinically but radiologic studies
continued to show contraction abnormalities of the
palatal and hypopharyngeal musculature, together with retained secretions in the pyriform sinuses. In
1974 Nanson [II] reported on two patients of un-
known ancestry with oculopharyngeal muscular dystrophy, both of whom were much improved by cricopharyngeal myotomy. Blakeley et al [15] de-
scribed two cases of oculopharyngeal muscular dys- trophy with the typical clinical presentation. Both
patients were completely cured of their symptoms by cricopharyngeal myotomy. Hurwitz et al [7] had one
patient with oculopharyngeal muscular dystrophy who improved clinically after surgery as demon-
strated by amelioration of symptoms and weight gain. In 1978 we reported our initial experience with 11 patients who underwent surgery for oropharyngeal dysphagia secondary to oculopharyngeal muscular dystrophy [3]. Eight of the 11 patients had excellent results and 3 were significantly improved. Bender [8] describes one patient with oculopharyngeal muscular
dystrophy who underwent cricopharyngeal myotomy for dysphagia. Despite a weak and dilated hypo- pharynx, 6 weeks later the patient was improved clinically.
The effects of operation on the upper sphincter and pharynx in patients with oculopharyngeal muscular dystrophy are poorly documented. Hurwitz et al [7] and Bender [8] report that upper esophageal function is unchanged after myotomy. In our 15 pa- tients pharyngeal function remained unchanged after extensive myotomy distal to the pharyngeal pouch: peak contraction pressure, contraction duration and
the number of pharyngeal contractions were un- changed by the operation. However, resting and contracting pressures. of the upper esophageal
sphincter were lowered by more than 50 percent. Clinically dysphagia, pharyngooral and pharyngo- nasal regurgitations and repetitive aspirations
completely disappeared in the majority of patients. Relaxation time was significantly shortened toward
normal by operation. The unchanged function of the pharynx suggests that improvement results from the
lower resistance in the upper esophageal sphincter area which permits passage of the bolus propelled by
the weakened pharynx.
Clinically an abnormal temporal relation certainly exists between the weak pharyngeal contraction and
the response of the upper esophageal sphincter. The manometric technique in our study probably does not allow full recording and interpretation of the
abnormalities present in both the pharynx and upper esophageal sphincter. A study establishing a corre-
lation between radiologic and manometric findings
in these patients is needed.
Summary
Oculopharyngeal muscular dystrophy is an auto- somal dominant inherited condition seen mainly in
patients of French Canadian origin. Fifteen patients with an established diagnosis of the disease were
evaluated clinically, manometrically and radiologi- cally before and after cricopharyngeal myotomy. All
patients presented with oropharyngeal dysphagia to
solids and liquids, pharyngooral and pharyngonasal
regurgitation, frequent aspiration at mealtime and chronic aspiration of saliva during the night. When compared with a control group, significant differ- ences were observed in the pressure, duration and
frequency of pharyngeal contraction. Cricopharyn- geal myotomy did not alter pharyngeal function. At the level of the upper esophageal sphincter, resting and contraction pressure relaxation, relaxation time and coordination were examined. Relaxation time was the only significant abnormality observed be-
tween patients with oculopharyngeal muscular dys- trophy and control subjects. Surgery significantly lowered the resting and contracting pressures of the sphincter. Relaxation time was brought toward normal. All patients’ symptoms were significantly improved by cricopharyngeal myotomy.
References
1. Barbeau A. The syndrome of hereditary late onset ptosis and dysphagia in French Canada. Wilhelm Erb Symposium, Dee 1, 1965 (Heidelberg). In: Kuhn E. Progressive muskeldys- trophie. myotonie. myasthenie. Berlin, Springer-Verlag. 1966.
38 The American Journal of Surgery
Oculopharyngeal Muscular Dystrophy
2 l3arbeau A. La myopathie oculaire au Canada franqais. Compte
rendu ler Congr& International Neuro-GBnBtique et Neuro-
Ophtalmologie. Basel, New York: S Karger, 1968, p 49-
55 3. Duranceau A, Letendre J, Clermont RJ, LBvesque HP, Barbeau
A. Oropharyngeal dysphagia in patients with oculopharyngeal
muscular dystrophy. Can J Surg 1978;21:236-329.
4. Peterman AF, Lillington GA, Jamplis RW. Progressive muscular
dystrophy with ptosis and dysphagia. Arch Neurol 1964;
10:52-55. 5. Murphy SF, Drachman DB. The oculopharyngeal syndrome.
JAMA 1968:203:99-104. 6. Lewis I Late onset muscle dystrophy: oculopharyngoesopha-
geal variety. Can Med Assoc J 1966;95:146-150.
7. Hurwitz AL, Nelson JA, Haddad JK. Oropharyngeal dysphagia
manometric and tine esophagraphic findings. Am J Dig Dis 1975;20:313-23.
8. Bender MD. Esophageal manometry in oculopharyngeal dys-
trophy Am J Gastroenterol 1976;62:215-21.
9. Bray GM, Kaarso M, Ross RT. Ocular myopathy with dysphagia.
Neurology 1965; 15678-84. 10. Roberts AH, Bamforth J. The pharynx and esophagus in ocular
muscular dystrophy. Neurology 1968;18:645-52. 11. Nanson EM. Achalasia of cricopharyngeus muscle and pha-
ryngeal diverticulum. NZ Med J 1974;520:41-48. 12. Montgomery WW, Lynch JP. Oculopharyngeal muscular dys-
trophy treated by inferior constructor myotomy. Trans Am
Acad Ophthalmol Otolaryngol 1971;75:986-93. 13. Taylor EW. Progressive vagus-glossopharyngeal paralysis with
ptosis. A contribution to the group of family diseases. J Nerv Ment Dis 1915;42:129-39.
14. Ellis FH. Shlegel JF, Lynch VP. Cricopharyngeal myotomy for
pharyngoesophageal diverticulum. Ann Surg 1969;170: 340-49.
15. Blakeley WR, Garety FJ, Smith DE. Section of the cricophar-
yngeus muscle for dysphagia. Arch Surg 1968;96:745- 62.
Discussion
Dr. ttoseman (San IXego, CA): We have been interested in t lis at Scripps Memorial Hospital and have done about six myotomies in selected patients. We have found that the cintsesophagogram is a major determinant for select,ivity. It is also an caxcellent objective way to follow improvement
because pressure measurements may be misleading, as they
vary on conventional manometry. I wonder if the:se patients
have had both clinical improvement and documented im-
provement on cineesophagograms?
Alfred Hurwitz (San <Jose, CA): Thank you for an ex- cellent study. I should point out the extreme difficulty of getting these patients to cooperate during the study be-
cause oft he choking and gagging that often occurs during the procedure. It is not an easy study to perform.
Do you think these patients have a mixed defect of
pharyngeal and upper esophageal dysfunction? Do you think the defect is mainly pharyngeal and that you mostly alleviated the barrier of the upper esophageal sphincter? Is this mainly a pharyngeal problem?
Andre Chranceau (closing): In answer to Dr. Rose- man’s question, we did perform some cineesophagograms
in all of the patients, who were classified clinically into four groups. Mo:;t patients are 40 to 45 years old at t.he time of their initial symptoms, which are usually mild. As men- tioned in the paper. the symptoms progress with age so that
when patients reach 60 or 65 years of age they usually have much more severe dysphagia. The patients present with severe oropharyngeal dysphagia, pharyngooral and phar- yngonasal regurgitation, and repetitive aspiration, and some of them have aspiration pneumonia. We operated on 15 patients.. all of’ whom had very severe symptoms; we knew that they had intact propulsion of the tongue, which is probably one reason we achieved very successful results in controlling symptoms.
In response to Dr. Hurwitz, I think that t~he pharyngeal abnormalities are evident from the manometric point of view, but that the actual recording techniques of the manometric system do not record all of the abn’ormalities present in the patients. We are attempting to correlate the abnormalities demonstrated on t,he tine studies,with those on the manometric st,udies. As Dr. Koseman mentioned, I think that we will find more abnormalit~ies on t,he tine
studies.
Volu ne 139, January 1980 39