tumor ous
following previpatients in whom the thought to be unresect-
had recurred
surgery and
tumor
was
able. Total tumor control was obtained in 13 of these patients with a median dose of 5,000 rads in 5 1/2 weeks delivered with megavoltage equipment. Such a dose is well tolerated with a minimum of complications and results in useful function of the involved area without mutilation. This article is an example of the growing experience of megavoltage irradiation in desmoid tumors. I would submit that gross excision and postoperative irradiation offer a rea¬ sonable and less morbid alternative to radical surgery in the definitive treatment of desmoid tumors. Stephen D. Sorgen, MD Brooke Army Medical Center Fort Sam Houston, Tex
Figure.
1. Wara
measured four times a day and stabilized around 300 liters/min while he received
only aminophylline. The addition of ephedrine sulfate, 25 mg orally every six hours, was followed by an
immediate rise of his PEFR to 400 liters/min. This improvement lasted for four days only, after which, despite contin¬ ued aminophylline and ephedrine therapy, the PEFR fell to between 150 and 200 liters/min, and physical signs of pulmo¬ nary obstruction were found. Ephedrine therapy was discontinued then, and the PEFR gradually reverted within seven days to around 300 liters/min. No pulse or blood pressure changes occurred. The same
pattern
was
times during that time ephedrine was
repeated three hospitalization, given (Figure).
Comment.—We have
no
more
every
data to
help decide on the mechanism for the observed phenomenon. Its reproducibility in this patient suggests strong¬ ly that it was not chance variation, a suggestion supported also by his asth¬ ma's stability during that hospitaliza¬ tion. Ephedrine might have lost its effectiveness by having depleted nor-
from the type 2 neuro¬ which the drug depends of its effect. On the other
epinephrine nal for
pool,
some
on
hand, if ephedrine had produced a partial blockade of 0-adrenergic re¬
suggested by the studies of Nelson,7 both the loss of improvement ceptors,
as
and the deterioration of asthma would be explained. Unfortunately, we did not compare this patient's responses to other 0-adrenergic agon¬ ists before and after his ephedrine courses.
We have
subsequently almost
en¬
tirely abandoned the use of ephedrine for other orally effective adrenergic
agents and have not again seen the pattern described here. Since ephed-
riñe and pseudoephedrine are still used in asthmatic patients, however, this pattern may be seen again. Pisit Rangsithienchai, MD Richard W. Newcomb, MD La Rabida Children's Hospital and Research Center University of Chicago Pritzker School of Medicine
Chicago
MJ, Glass P: Evaluation of a new beta-2 adrenergic receptor stimulant, terbutaline, in bronchial asthma: II. Oral comparison with ephedrine. Curr Ther Res 15:150-157, 1973. 2. Tashkin DP, Meth R, Simmons DH, et al: Double\x=req-\ blind comparison of acute bronchial and cardiovascular effects of oral terbutaline and ephedrine. Chest 68:155\x=req-\ 161, 1975. 3. Weinberger MM: Use of ephedrine in bronchodilator therapy. Pediatr Clin North Am 22:121-127, 1975. 4. Blumberg MZ, Tinkelman DG, Ginchansky EJ, et al: Terbutaline and ephedrine in asthmatic children. Pediatrics 60:14-19, 1977. 5. Tinkelman DG, Avner SE: Ephedrine therapy in 1. Dulfano
asthmatic children: Clinical tolerance and absence of side effects. JAMA 237:553-557, 1977. 6. Herxheimer H: Dose of ephedrine in the treatment of bronchial asthma in children. Br Med J 1:350-352, 1946. 7. Nelson HS: The effect of
to
epinephrine
in normal
51:191-198, 1973.
ephedrine on the response J Allergy Clin Immunol
men.
Radiotherapy in Desmoid Tumors
To the Editor.\p=m-\Inthe CLINICAL NOTE "Desmoid Tumor Presenting as a Parotid Mass" (239:337, 1978), the authors present an unusual and interesting case of desmoid tumor. In their comments they advocate radical excision of all such tumors and state that radiation therapy is not consistently
helpful.
I would like to draw the authors' and readers' attention to a recent article by Wara et al,1 who review 16 cases of desmoid tumors treated with irradiation. These 16 patients included both patients in whom the
Downloaded From: http://jama.jamanetwork.com/ by a University of Pittsburgh User on 06/18/2015
WM, Phillips TL, Hill D, et al: Desmoid prognosis. Radiology 124:225,
tumors: Treatment and
1977.
Reply.\p=m-\Our statement that roentgenotherapy has not been found to be consistently helpful in the manageIn
ment of desmoid tumors was based on the observations of Enzinger and Shiraki.1 They have reviewed a relatively large series of 30 patients with desmoid tumor followed up for ten or more years. Wara et al have based their findings on a group of 16 cases followed for a period of two to six
years.
However,
most
recurrences
have been reported during the first few months following surgery,1 and a control of disease for two to six years by radiotherapy is great enough to merit further trial in appropriate situations. In our own medical center, a trial has been given to the drug levamisole and levamisole plus testolactone for a similar condition of aggressive fibromatosis. The results have been encouraging (S. A. Wilkins, Jr, personal communication). This discussion indicates that multidisciplinary approach may provide an ultimate answer to the management of neoplastic diseases. Bhagirath Majmudar, MD Nilda Winiarski, MD
Emory University School of Medicine Atlanta
1. Enzinger FM, Shiraki M: Musculo-aponeurotic fibromatosis of the shoulder girdle (extra-abdominal desmoid). Cancer 20:1131-1140, 1967.
Denotation of Dyspnea To the Editor.\p=m-\Ina letter published recently in The Journal (239:928, 1978) E. D. Morton, MD, asks what dyspnea means and whether it refers to a subjective symptom or an objec-
tive sign. Since etymological or historical arguments showing what a word ought to mean are out of style, the answers to these questions should be sought through observation of the word in use. A review of numerous current textbooks, medical dictionaries, and other reference works shows that dyspnea is all but universally understood as a subjective experience. The term is variously defined as labored breathing, difficult breathing, shortness of breath, uncomfortable awareness of one's breathing, a feeling of suffocation, a consciousness of the need for more respiratory effort than seems called for by the level of activity, and a feeling of inability to draw a full breath. The fact that dyspnea can be inferred by an observer from such signs as increase in the rate or depth of respirations, activity of the acces¬ sory respiratory muscles, and flaring of the alae nasi does not make it any less a subjective experience. Parox¬ ysmal nocturnal dyspnea, as often as it turns up in patient histories, is seldom if ever observed by the clini¬ cian. The application of dyspnea to Cheyne-Stokes respirations, cited by Dr Morton from a textbook of medi¬ cine published in 1950, is a decidedly unorthodox use of the term. Most modern authorities would not accept the inference of dyspnea in an uncon¬ scious patient or in one who, though tachypneic at rest, is unaware of any
breathing difficulty. This
subjectiveness, however,
is
etiologic or anatomic breakdown of dyspnea. No one objects on metaphysical grounds to etiologic classifications of such purely subjective phenomena as itch¬ ing, vertigo, chest pain, or blindness. surely
no
obstacle to
an
The mission of the scientist is to observe, record, classify, and inter¬ pret reality. Let us not forget that in medicine the first and fundamental reality is a patient and his troubles as he perceives them. Sometimes the physician, with his cold steel imple¬ ments, loaded questions, and arcane terminology, is at best a well-inten¬ tioned intruder on the scene. John H. Dirckx, MD
University of Dayton Dayton, Ohio
Management of Tic Douloureux To the Editor.\p=m-\TheSPECIAL COMMUNICATION on tic douloureux by Loeser (239:1153, 1978) is to be faulted on several counts. The most important indiscretion is the endorsement of an
experimental procedure for general long before it has even minimal proof of long-term efficacy. Tic douloureux is a notoriously remittent
use
over the last 30 years, those who have had broad experience in the management of this painful condition have seen procedures as well recommended as Jannetta's1 approach fall into oblivion with the passage of time. Secondly, the author fails to place temporizing procedures such as alcoholic injection in their proper role. Permanent interruption of the trigeminal nerve as an initial procedure is frought with the danger of replacing the pain of tic douloureux with unbearable paresthesias, a pitiful, and for the most part an untreatable, state. It is well known2 that facial anesthesia dolorosa can be avoided almost entirely by proper preparation of the patient. Peripheral avulsion or alcoholic injection, giving the patient a long but temporary experience with the paresthesias of nerve interrup¬ tion prior to having a permanent root avulsion, goes a long way in prevent¬ ing the malignant paresthetic state. Thirdly, to say that the results of trigeminal rhizotomy are no better than that of gangliolysis must offend all neurosurgeons truely experienced in the treatment of tic douloureux. about 50% at five "Pain relief years" with gangliolysis hardly com¬ pares with my own experience for over 35 years with close to 100% success in obtaining prolonged pain relief with trigeminal rhizotomy. This substantiates the more extensive ex¬ perience of my teacher, Dr Francis Grant,3 and others of his era who operated under much more trying circumstances. Further, the morbidi¬ ty of trigeminal rhizotomy in experi¬ enced hands is minimal and certainly is comparable with gangliolysis, even when operating on patients older than 80 years of age. Review of the report of Cushing" in 1920 and the reports of Frazier5 and Grant6 from roughly the same era of relatively primitive operating conditions will confirm this statement. Fourthly, to recommend trigeminal tractotomy at this late date, in the context of his short review, is hardly
disease, and
.
.
.
justifiable. My most appreciative patients over a long period of time are the ones I
have treated for tic douloureux by methods condemned by Loeser. The younger generation of neurosurgeons must be encouraged to experiment with new procedures, hoping to improve the previously acceptable
Downloaded From: http://jama.jamanetwork.com/ by a University of Pittsburgh User on 06/18/2015
deficits of root section, but they must measure their success against the record of established treatment and not be given premature approval of their enthusiasm for new methods before these are proved by rigid observation and the passage of time. Henry A. Shenkin, MD Medical College of
Pennsylvania Philadelphia 1. Jannetta PJ: Microsurgical approach to the trigeminal nerve for tic douloureaux. Prog Neurol Surg 7:180\x=req-\ 200, 1975. 2. White JC, Sweet WH: Pain. Springfield, Ill, Charles C Thomas Publisher, 1955, pp 436-438. 3. Grant FC: Results in operative treatment of major trigeminal neuralgia. Ann Surg 107:14-19, 1938. 4.
Cushing H: A method of total extirpation ganglion for trigeminal neuralgia.
Gasserian
of the JAMA
34:1035-1041, 1900. 5. Frazier CH:
Operation for the radical cure of trigeminal neuralgia: Analysis of 500 cases. Ann Surg
88:534-547,1928. 6. Grant FC: relief of pain in
47, 1948.
Complications accompanying surgical trigeminal neuralgia. Am J Surg 75:42\x=req-\
Hemoptysis With Intercourse To the Editor.\p=m-\Recent comment in QUESTIONS AND ANSWERS (239:351,1978) regarding hemoptysis following intercourse prompts the following report. Report of a Case.\p=m-\In1973 a 55-year-old man complained of hemoptysis immedi-
ately following intercourse with orgasm. Similar episodes recurred irregularly
about ten to 12 times over the course of the next two years, sometimes associated with dyspnea and lasting about 15 to 20 minutes. Hemoptysis did not occur following other types of exertion, but there was mild exertional dyspnea. The patient was obese, with repeated attempts at dietary weight reduction having failed in the preceding 15 years when he was under our observation and treatment. Diabetes mellitus was found in 1961 and was treated with oral agents. Control was satisfactory during hospitalizations for other ailments (subtotal pa-
for cyst, 1961; acute epididymitis, 1962; suprapubic prostatectomy for benign hypertrophy, 1967; degenerative disc disease, 1973) but was poor when he followed his own dietary devices. Hyper-
rotidectomy
tension was under treatment with a combination of chlorothiazide and methyl-
dopa. Physical examination showed obesity
and acute and chronic furunculosis but otherwise unremarkable. Blood pres¬ sure was 150/90 mm Hg, but levels prior to treatment had ranged around 200/110 mm Hg. An ECG showed evidence of left ventricular hypertrophy, but there was no cardiomegaly on chest x-ray film, and the lung fields were normal. Blood counts and chemistries and urine analyses were nor¬ mal except for intermittent hyperglycemia and glycosuria of mild degree. Bronchoscopy showed no abnormalities. was
Smoking (one pack
of
cigarettes daily)
discontinued. We substituted furosemide for the chlorothiazide but noted no
was
changes.
following previpatients in whom the thought to be unresect-
had recurred
surgery and
tumor
was
able. Total tumor control was obtained in 13 of these patients with a median dose of 5,000 rads in 5 1/2 weeks delivered with megavoltage equipment. Such a dose is well tolerated with a minimum of complications and results in useful function of the involved area without mutilation. This article is an example of the growing experience of megavoltage irradiation in desmoid tumors. I would submit that gross excision and postoperative irradiation offer a rea¬ sonable and less morbid alternative to radical surgery in the definitive treatment of desmoid tumors. Stephen D. Sorgen, MD Brooke Army Medical Center Fort Sam Houston, Tex
Figure.
1. Wara
measured four times a day and stabilized around 300 liters/min while he received
only aminophylline. The addition of ephedrine sulfate, 25 mg orally every six hours, was followed by an
immediate rise of his PEFR to 400 liters/min. This improvement lasted for four days only, after which, despite contin¬ ued aminophylline and ephedrine therapy, the PEFR fell to between 150 and 200 liters/min, and physical signs of pulmo¬ nary obstruction were found. Ephedrine therapy was discontinued then, and the PEFR gradually reverted within seven days to around 300 liters/min. No pulse or blood pressure changes occurred. The same
pattern
was
times during that time ephedrine was
repeated three hospitalization, given (Figure).
Comment.—We have
no
more
every
data to
help decide on the mechanism for the observed phenomenon. Its reproducibility in this patient suggests strong¬ ly that it was not chance variation, a suggestion supported also by his asth¬ ma's stability during that hospitaliza¬ tion. Ephedrine might have lost its effectiveness by having depleted nor-
from the type 2 neuro¬ which the drug depends of its effect. On the other
epinephrine nal for
pool,
some
on
hand, if ephedrine had produced a partial blockade of 0-adrenergic re¬
suggested by the studies of Nelson,7 both the loss of improvement ceptors,
as
and the deterioration of asthma would be explained. Unfortunately, we did not compare this patient's responses to other 0-adrenergic agon¬ ists before and after his ephedrine courses.
We have
subsequently almost
en¬
tirely abandoned the use of ephedrine for other orally effective adrenergic
agents and have not again seen the pattern described here. Since ephed-
riñe and pseudoephedrine are still used in asthmatic patients, however, this pattern may be seen again. Pisit Rangsithienchai, MD Richard W. Newcomb, MD La Rabida Children's Hospital and Research Center University of Chicago Pritzker School of Medicine
Chicago
MJ, Glass P: Evaluation of a new beta-2 adrenergic receptor stimulant, terbutaline, in bronchial asthma: II. Oral comparison with ephedrine. Curr Ther Res 15:150-157, 1973. 2. Tashkin DP, Meth R, Simmons DH, et al: Double\x=req-\ blind comparison of acute bronchial and cardiovascular effects of oral terbutaline and ephedrine. Chest 68:155\x=req-\ 161, 1975. 3. Weinberger MM: Use of ephedrine in bronchodilator therapy. Pediatr Clin North Am 22:121-127, 1975. 4. Blumberg MZ, Tinkelman DG, Ginchansky EJ, et al: Terbutaline and ephedrine in asthmatic children. Pediatrics 60:14-19, 1977. 5. Tinkelman DG, Avner SE: Ephedrine therapy in 1. Dulfano
asthmatic children: Clinical tolerance and absence of side effects. JAMA 237:553-557, 1977. 6. Herxheimer H: Dose of ephedrine in the treatment of bronchial asthma in children. Br Med J 1:350-352, 1946. 7. Nelson HS: The effect of
to
epinephrine
in normal
51:191-198, 1973.
ephedrine on the response J Allergy Clin Immunol
men.
Radiotherapy in Desmoid Tumors
To the Editor.\p=m-\Inthe CLINICAL NOTE "Desmoid Tumor Presenting as a Parotid Mass" (239:337, 1978), the authors present an unusual and interesting case of desmoid tumor. In their comments they advocate radical excision of all such tumors and state that radiation therapy is not consistently
helpful.
I would like to draw the authors' and readers' attention to a recent article by Wara et al,1 who review 16 cases of desmoid tumors treated with irradiation. These 16 patients included both patients in whom the
Downloaded From: http://jama.jamanetwork.com/ by a University of Pittsburgh User on 06/18/2015
WM, Phillips TL, Hill D, et al: Desmoid prognosis. Radiology 124:225,
tumors: Treatment and
1977.
Reply.\p=m-\Our statement that roentgenotherapy has not been found to be consistently helpful in the manageIn
ment of desmoid tumors was based on the observations of Enzinger and Shiraki.1 They have reviewed a relatively large series of 30 patients with desmoid tumor followed up for ten or more years. Wara et al have based their findings on a group of 16 cases followed for a period of two to six
years.
However,
most
recurrences
have been reported during the first few months following surgery,1 and a control of disease for two to six years by radiotherapy is great enough to merit further trial in appropriate situations. In our own medical center, a trial has been given to the drug levamisole and levamisole plus testolactone for a similar condition of aggressive fibromatosis. The results have been encouraging (S. A. Wilkins, Jr, personal communication). This discussion indicates that multidisciplinary approach may provide an ultimate answer to the management of neoplastic diseases. Bhagirath Majmudar, MD Nilda Winiarski, MD
Emory University School of Medicine Atlanta
1. Enzinger FM, Shiraki M: Musculo-aponeurotic fibromatosis of the shoulder girdle (extra-abdominal desmoid). Cancer 20:1131-1140, 1967.
Denotation of Dyspnea To the Editor.\p=m-\Ina letter published recently in The Journal (239:928, 1978) E. D. Morton, MD, asks what dyspnea means and whether it refers to a subjective symptom or an objec-
tive sign. Since etymological or historical arguments showing what a word ought to mean are out of style, the answers to these questions should be sought through observation of the word in use. A review of numerous current textbooks, medical dictionaries, and other reference works shows that dyspnea is all but universally understood as a subjective experience. The term is variously defined as labored breathing, difficult breathing, shortness of breath, uncomfortable awareness of one's breathing, a feeling of suffocation, a consciousness of the need for more respiratory effort than seems called for by the level of activity, and a feeling of inability to draw a full breath. The fact that dyspnea can be inferred by an observer from such signs as increase in the rate or depth of respirations, activity of the acces¬ sory respiratory muscles, and flaring of the alae nasi does not make it any less a subjective experience. Parox¬ ysmal nocturnal dyspnea, as often as it turns up in patient histories, is seldom if ever observed by the clini¬ cian. The application of dyspnea to Cheyne-Stokes respirations, cited by Dr Morton from a textbook of medi¬ cine published in 1950, is a decidedly unorthodox use of the term. Most modern authorities would not accept the inference of dyspnea in an uncon¬ scious patient or in one who, though tachypneic at rest, is unaware of any
breathing difficulty. This
subjectiveness, however,
is
etiologic or anatomic breakdown of dyspnea. No one objects on metaphysical grounds to etiologic classifications of such purely subjective phenomena as itch¬ ing, vertigo, chest pain, or blindness. surely
no
obstacle to
an
The mission of the scientist is to observe, record, classify, and inter¬ pret reality. Let us not forget that in medicine the first and fundamental reality is a patient and his troubles as he perceives them. Sometimes the physician, with his cold steel imple¬ ments, loaded questions, and arcane terminology, is at best a well-inten¬ tioned intruder on the scene. John H. Dirckx, MD
University of Dayton Dayton, Ohio
Management of Tic Douloureux To the Editor.\p=m-\TheSPECIAL COMMUNICATION on tic douloureux by Loeser (239:1153, 1978) is to be faulted on several counts. The most important indiscretion is the endorsement of an
experimental procedure for general long before it has even minimal proof of long-term efficacy. Tic douloureux is a notoriously remittent
use
over the last 30 years, those who have had broad experience in the management of this painful condition have seen procedures as well recommended as Jannetta's1 approach fall into oblivion with the passage of time. Secondly, the author fails to place temporizing procedures such as alcoholic injection in their proper role. Permanent interruption of the trigeminal nerve as an initial procedure is frought with the danger of replacing the pain of tic douloureux with unbearable paresthesias, a pitiful, and for the most part an untreatable, state. It is well known2 that facial anesthesia dolorosa can be avoided almost entirely by proper preparation of the patient. Peripheral avulsion or alcoholic injection, giving the patient a long but temporary experience with the paresthesias of nerve interrup¬ tion prior to having a permanent root avulsion, goes a long way in prevent¬ ing the malignant paresthetic state. Thirdly, to say that the results of trigeminal rhizotomy are no better than that of gangliolysis must offend all neurosurgeons truely experienced in the treatment of tic douloureux. about 50% at five "Pain relief years" with gangliolysis hardly com¬ pares with my own experience for over 35 years with close to 100% success in obtaining prolonged pain relief with trigeminal rhizotomy. This substantiates the more extensive ex¬ perience of my teacher, Dr Francis Grant,3 and others of his era who operated under much more trying circumstances. Further, the morbidi¬ ty of trigeminal rhizotomy in experi¬ enced hands is minimal and certainly is comparable with gangliolysis, even when operating on patients older than 80 years of age. Review of the report of Cushing" in 1920 and the reports of Frazier5 and Grant6 from roughly the same era of relatively primitive operating conditions will confirm this statement. Fourthly, to recommend trigeminal tractotomy at this late date, in the context of his short review, is hardly
disease, and
.
.
.
justifiable. My most appreciative patients over a long period of time are the ones I
have treated for tic douloureux by methods condemned by Loeser. The younger generation of neurosurgeons must be encouraged to experiment with new procedures, hoping to improve the previously acceptable
Downloaded From: http://jama.jamanetwork.com/ by a University of Pittsburgh User on 06/18/2015
deficits of root section, but they must measure their success against the record of established treatment and not be given premature approval of their enthusiasm for new methods before these are proved by rigid observation and the passage of time. Henry A. Shenkin, MD Medical College of
Pennsylvania Philadelphia 1. Jannetta PJ: Microsurgical approach to the trigeminal nerve for tic douloureaux. Prog Neurol Surg 7:180\x=req-\ 200, 1975. 2. White JC, Sweet WH: Pain. Springfield, Ill, Charles C Thomas Publisher, 1955, pp 436-438. 3. Grant FC: Results in operative treatment of major trigeminal neuralgia. Ann Surg 107:14-19, 1938. 4.
Cushing H: A method of total extirpation ganglion for trigeminal neuralgia.
Gasserian
of the JAMA
34:1035-1041, 1900. 5. Frazier CH:
Operation for the radical cure of trigeminal neuralgia: Analysis of 500 cases. Ann Surg
88:534-547,1928. 6. Grant FC: relief of pain in
47, 1948.
Complications accompanying surgical trigeminal neuralgia. Am J Surg 75:42\x=req-\
Hemoptysis With Intercourse To the Editor.\p=m-\Recent comment in QUESTIONS AND ANSWERS (239:351,1978) regarding hemoptysis following intercourse prompts the following report. Report of a Case.\p=m-\In1973 a 55-year-old man complained of hemoptysis immedi-
ately following intercourse with orgasm. Similar episodes recurred irregularly
about ten to 12 times over the course of the next two years, sometimes associated with dyspnea and lasting about 15 to 20 minutes. Hemoptysis did not occur following other types of exertion, but there was mild exertional dyspnea. The patient was obese, with repeated attempts at dietary weight reduction having failed in the preceding 15 years when he was under our observation and treatment. Diabetes mellitus was found in 1961 and was treated with oral agents. Control was satisfactory during hospitalizations for other ailments (subtotal pa-
for cyst, 1961; acute epididymitis, 1962; suprapubic prostatectomy for benign hypertrophy, 1967; degenerative disc disease, 1973) but was poor when he followed his own dietary devices. Hyper-
rotidectomy
tension was under treatment with a combination of chlorothiazide and methyl-
dopa. Physical examination showed obesity
and acute and chronic furunculosis but otherwise unremarkable. Blood pres¬ sure was 150/90 mm Hg, but levels prior to treatment had ranged around 200/110 mm Hg. An ECG showed evidence of left ventricular hypertrophy, but there was no cardiomegaly on chest x-ray film, and the lung fields were normal. Blood counts and chemistries and urine analyses were nor¬ mal except for intermittent hyperglycemia and glycosuria of mild degree. Bronchoscopy showed no abnormalities. was
Smoking (one pack
of
cigarettes daily)
discontinued. We substituted furosemide for the chlorothiazide but noted no
was
changes.
