747
available. Nevertheless, there is nothing in our study to support the contention of Bondl and Stjernswardzthat
early postoperative radiotherapy may be harmful. The only statistically significant difference revealed in either trial is the reduced incidence of local recurrence in those stage-i cases who received early postoperative radiotherapy. Similar findings were obtained in the Cancer Research Campaign Trials an international multicentre trial which took place concurrently with the present study. There is no evidence so far that patients whose radiotherapy is delayed until local recurrence is detected will fare worse in terms of survival than those whose recurrence has been prevented by early radiotherapy. Occult residual tumour in the operation field or regional nodes may be a source of metastases during the
interval between surgery and delayed radiotherapy. If this is so it should be reflected in the long-term survival figures. Radiotherapy was better able to prevent local recurrence of the laterally and medially placed tumours than that of those in the midline of the breast. This is
partly because laterally placed tumours are more likely to give rise to axillary node metastases which can be detected at follow-up. Also, the possibility of incomplete removal is greater with peripheral tumours since they are likely to be excised with a smaller margin of surrounding normal tissue than central ones. Similarly, radiotherapy had a larger influence in preventing recurrence of larger tumours probably because the more advanced carcinomas would be more likely to have spread to lymph-nodes and less likely to have been adequately removed by local mastectomy. The induction of an artificial menopause was offered to patients who were premenopausal or less than three years postmenopausal because a previous trial had indicated that it produced a slight improvement in the reduction in the incidence of was unjustifiable to withhold this possible benefit from all patients although we appreciated that sterilisation would not be acceptable or considered appropriate in every case. We admit that the non-random way in which some patients did not have artificial induction of the menopause might introduce a slight bias to the results. However, it is now believed that the benefit of an artificial menopause is temporary -it delays but does not prevent recurrence or death in those who are destined to die.7 The long-term survival figures are thus unlikely to be influenced by induced menopause. Table n indicates that our results have not been significantly affected by the small number of patients who did not have an artificial menopause. Our study supports the now widely held view that there is little to choose between different methods of conventional treatment by surgery and/or radiotherapy. Postoperative radiotherapy for patients presenting in stage I can safely be delayed until they have evidence of local recurrence or lymph-node metastases. Simple mastectomy with postoperative radiotherapy or radical mastectomy alone are equally effective for treating stage-ii patients-the former is more time-consuming and expensive and the patient may have the unpleasant immediate sequelae of radiotherapy, but the latter produces greater disfigurement. The choice would depend on individual circumstances and preferences of the patient and surgeon. Surgery and/or radiotherapy may control the local survival
rates
and
a
recurrence.6 We felt that it
manifestation of breast cancer but the survival rate depends on the presence or absence of distant metastases and on host resistance. Any improvement on the present results can only be expected from adjuvant systemic therapy of which numerous regimens are being studied. The members of the committee which organised the trial were: Prof. M. R. Alderson (until 1970), Mr A. R. Anscombe, Dr R. Gibb, Mr A. W. Hargreaves (since 1975), Mr N. F. Kirkman, Dr 1. Leck (since 1970), Mr. M. Lentin, Mr J. P. Lythgoe, Dr I. D. H. Todd. Surgeons taking part in the trial were: Mr A. R. Anscombe, Mr M. A. Brennan, Mr A. S. Bullough, Mr H. J. Done, Mr H. M. Goldberg, Mr A. W. Hargreaves, Mr H. Hassall, Mr E. Henderson, Mr G. Ingram, Mr N. C. Keddie, Mr J. H. Kilshaw, Mr N. F. Kirkman, Mr D. K. Lennox, Mr M. Lentin, Mr W. H. Lonsdale, Mr J. P. Lythgoe, Mr J. Magell, Mr J. W. Main, Mr N. Macdonald, Mr A. F. Robinson, Mr J. Y. W. Russell, Mr 1. G. Schraibman, Mr H. F. Smith, Mr W. A. B. Strachan, Mr J. Wilks, Mr A. F. Williams. Radiotherapists taking part in the trial were: Dr J. R. Brown, Dr M. P. Cole, Dr D. P. Deakin, Dr M. B. Duthie, Prof. E. C. Easson, Dr R. Gibb, Dr N. K. Gupta, Dr A. W. Jackson, Dr D. M. Jackson, Dr I. J. Kerby, Dr D. Pearson, Dr R. S. Pointon, Dr G. G. Ribeiro, Dr E. Sherrah-Davis, Dr J. G. Stewart (who has since died), Dr M. L. Sutton, Dr I. D. H. Todd. The study was supported by a grant from the Manchester Regional Hospital Board.
REFERENCES
Bond, W. H. in The Treatment of Carcinoma of the Breast (edited by A. S. Jarrett); p. 24. Amsterdam, 1968. 2. Stjernsward, J. Lancet, 1974, ii, 1285. 3. T.N.M. Classification of Malignant Tumours. U.I.C.C., Geneva, 1968. 4. Peto, R., Pike, M. C., Armitage, P., et al. Br. J. Cancer, 1977, 35, 1. 5. Working Party of the Cancer Research Campaign. Br. med. J. 1976, i, 1035. 6. Paterson, R., Russell, M. H. J. Fac. Radiol. 1959, 10, 130. 7. Cole, M. P. in The Clinical Management of Advanced Breast Cancer— Second Tenovus Workshop (edited by C. A. F. Joslin and E. N. Gleave); p. 2. Cardiff, 1970. 1.
PROTRUDING AURICLE: A NEUROMUSCULAR SIGN DAVID W. SMITH
Department of Pediatrics, University of Washington, Seattle, U.S.A. HIROTADA TAKASHIMA
Department of Pediatrics, University of Washington, and Nihon University, Japan A protruding auricle is usually associated with a defect in development or function of the posterior auricular muscle, which normally draws the pinna towards the calvarium. Hence, a protruding auricle may be a sign of a neuromuscular disorder, as is ptosis.
Summary
Introduction MUSCLE function can influence the shape of cartilage and bone’-for example, ear muscles may affect the shape of the external auricle.2 Man has three extrinsic ear muscles extending from the calvarium to the cartilaginous pinna and six intrinsic pinna muscles.3 The role of these muscles is evident from the hearing-related movements of the external ear in animals such as the dog or cat. Though such movements are seldom noticeable in man, Seiler3 demonstrated by electromyographic techniques that the human ear muscles are responsive to sound stimuli. We have studied the impact of ear mus-
748
cles on gross auricular shape and especially the relation between the posterior auricular muscle and the protruding auricle. Patients and Methods The following groups were studied to determine the relation between the development of the auricle and the posterior auricular muscle: 12 human fetuses; 100 newborn infants; any patient we came across with protruding auricle; patients with specific disorders in which the sign occurs.4
Results In the 12 fetuses (aged 12-22 weeks), all of whom had auricles which were considered to be in normal positions, the posterior auricular muscle was a prominent and easily identifiable structure extending from its origin in the epicranial aponeurosis overlying the mastoid area of the temporal bone to its insertion on the inferior surface of the cartilaginous concha, at the root of the crus helix (fig. 1). The auricle was "held down" toward
Fig. 2-Protruding
auricles in
a
boy with Langer-Giedion syn-
drome. Hall,
B.
D.,
et
al. Birth
Defects orig. Article ser. 1974, 10,
148.
number of established disorders,4 (see accomtable and fig 2). We also found protruding auripanying in cles a number of patients without any known disorder but the majority of these patients had a problem which could have been associated with neuromuscular dysfunction-for example, one patient had idiopathic microcephaly. Eks has attributed protruding auricles in a patient to arthrogryposis multiplex congenita due to a cerebral defect and we see protruding ears as being analogous to multiple joint contractures, that is, as deformities secondary to the neuromuscular dysfunction. Another example of an association between protruding auricles and neuromuscular dysfunction is that of an intelligent 8-year-old boy with congenital muscle hypoplasia and generalized weakness of unknown cause but with good muscular coordination and no apparent deterioration of function (fig. 3). His male first cousin has the ture
in
a
I-Posterior auricular muscle in a 16-week fetus (above) and a 20-week fetus (below). The muscle extends from the mastoid region of the temporal bone to the inferior surface of the conchal cartilage, at the root of the crus helix. The posterior conchal and calvarial skin has been removed, the muscle dissected out, and the auricle pulled forward.
Fig.
Courtesy of Dr Thomas H. Shepard, Central Laboratory for Human Embryology and Pediatrics, University of Washington School of Medicine.
the calvarium in all stages of fetal development by this muscle. None of the 100 consecutive 1-3 day old infants had a protruding auricle-an auricle was considered protruding if the angle between the dorsal surface of the upper helical margin of the auricle and the plane of the pars petrosa of the os temporalis, measured by a transparent protractor, was 40 degrees or.more. Among the patients who had one or two protruding auricles two died in early infancy and their posterior auricular muscles were examined at necropsy. The one who had anencephaly and bilateral protruding ears had no posterior auricular muscle on either side. The other patient, who had died immediately after surgical repair of a cleft lip, was chromosomally normal; idiopathic holoprosencephaly anomaladand facial dysmorphogenesis were confirmed at necropsy. There was no posterior auricular muscle behind the protruding auricle but the muscle was present and inserted at the usual site on the other, normal-looking, auricle. A protruding auricle is an occasional to frequent fea-
Fig.3-8-year-old boy with non-progressive congenital muscle hypoplasia of unknown cause and protruding auricles. The anthelix is also poorly formed. Courtesy of Dr Hossein Massoud, Director of Children’s Hospital, Chattanooga, Tennessee.
Paediatrics, T.
C.
Thompson
disorder (and protruding auricles). The family studies suggest an X-linked recessive pattern of inheritance. The protruding auricles appear to be a consequence of a genetically determined generalised disorder of muscle hypoplasia, but it is not known whether the pathology is primarily neural or muscular. same
Discussion Our findings support the hypothesis that the posterior auricular muscle has a role in the normal positioning of
749
the auricle and that an absence or dysfunction of this muscle allows the auricle to protrude. It follows that the protruding auricle should be interpreted as a sign of a muscular or neurological deficiency of the posterior auricular muscle rather than as a sign of dysmorphogenesis of the auricular cartilage. Thus a protruding auricle can be expected in patients with muscular’disorders such as myotonic dystrophy,4 and there is an obvious relation of protruding auricles with the Moebius anomalad,4 in which there is congenital seventh nerve paralysis, since it is the seventh nerve which innervates the posterior auricular muscle. The absence of the posterior auricular muscle in two patients with protruding auricles and severe defects of brain development (anencephaly and holoprosencephaly) should not necessarily be taken as evidence of a primary muscle deficiency, since a defect of the functional innervation of a muscle early in its development will usually result in hypoplasia, or even absence, of the non-innervated muscle.6 We favour a primary neurological deficiency as the cause of the absence of the posterior auricular muscle in our patients with severe defects of early brain development and protruding auricles. Protruding auricles are associated with a number of disorders in which multiple defects include central nervous-system dysfunction (see accompanying table). The list includes disorders in which the clinical DISORDERS IN WHICH A PROTRUDING AURICLE MAY BE A FEATURE
Fig. 4—Girt with XO Turner syndrome, protruding auricles, ptosis of the left eyelid. Courtesy of Dr Judith G. Hall, Washington School of Medicine.
Division of Medical Genetics,
uncertain in disorders such
as
the XO Turner
and
University
of
syndrome
(fig. 4). Defects of development and/or function of the other extrinsic auricular muscles and of the six intrinsic auricular muscles also need to be studied in relation to other defects of the auricle. two
We thank Dr Thomas H. Shepard, of the Central Laboratory for Human Embryology, for the fetal material; and the department of medical photography and the nursing staff of the University Hospital newborn nursery, Mrs Lyle Harrah (research librarian), and Ms Jane Fowler (secretary) for their help. The work was supported by: the U.S. Bureau of Community Health Services, Health Services Administration, Public Health Service, the Department of Health, Education and Welfare, and the National Institutes of Health; and by Yoshida’s Science and Technology Foundation, Japan.
Requests for reprints should be addressed to D. W. S., Department of Pediatrics, RD-20, University of Washington School of Medicine, Seattle, Washington 98195. REFERENCES
1. Thomson, D’Arcy. On Growth and Form. Cambridge, 1942. 2. Moss, M. L. Birth Defects orig. Article Ser. 1975, 11, 283. 3. Seiler, R. Gegenbaurs morphol. Jahrb. (Leipzig) 1974, 120, 78. 4. Smith, D. W. in Recognizable Patterns of Human Malformation;
p. 485,
Philadelphia, 1976. 5. Ek, J. I. Acta pædiat. scand. 1958, 47, 302. 6. Eastlick, H. L. J. exp. Zool. 1973, 93, 27. 7. 8.
*++= frequent, 20% or more; += occasional, less than 20%.
features do not readily suggest a neural or muscular basis for the protruding auricle, but protruding auricles may be present in an otherwise normal individual and their familial occurrence has been reported.7 A protruding auricle is like ptosis of the eyelid, which may be due to a muscular or a neurological deficiency of the levator palpebrae superioris muscle, may occur by itself or as part of a generalised muscular disorder, or may be a consequence of brain dysfunction. A protruding auricle and ptosis of the eyelid may be present in the same patient. In myotonic dystrophy8 both signs relate muscle dysfunction. However, whether protruding auricles and/or the ptosis of the eyelids are due primarily to muscular or to neurological abnormalities remains to
Becker, O. J. Archs Otolar. 1949, 50, 541. Dodge, P. R., Gamstop, I., Byers, R. K., Russell,
P.
Pediatrics, 1965, 35, 3.
ACID-PHOSPHATASE REACTION IN ACUTE LYMPHOBLASTIC LEUKÆMIA D. CATOVSKY M. F. GREAVES C. PAIN
M. CHERCHI
G. JANOSSY H. E. M. KAY
M.R.C. Leukæmia Unit, Royal Postgraduate Medical School, London W12; Membrane Immunology Laboratory, Imperial Cancer Research Fund, London WC2; and M.R.C. Leukæmia Trials Office, Royal Marsden Hospital, London SW3
Summary
The diagnostic value of the acid-phosphatase reaction was assessed double-
blind in 148 cases of acute lymphoblastic leukæmia classified by surface-membrane markers and entered into the M.R.C. U.K. A.L.L. trials. 90% of cases of T-A.L.L. showed a positive reaction in the majority of blast cells, while only 2% of common-A.L.L. and 10% of
(A.L.L.)
available. Nevertheless, there is nothing in our study to support the contention of Bondl and Stjernswardzthat
early postoperative radiotherapy may be harmful. The only statistically significant difference revealed in either trial is the reduced incidence of local recurrence in those stage-i cases who received early postoperative radiotherapy. Similar findings were obtained in the Cancer Research Campaign Trials an international multicentre trial which took place concurrently with the present study. There is no evidence so far that patients whose radiotherapy is delayed until local recurrence is detected will fare worse in terms of survival than those whose recurrence has been prevented by early radiotherapy. Occult residual tumour in the operation field or regional nodes may be a source of metastases during the
interval between surgery and delayed radiotherapy. If this is so it should be reflected in the long-term survival figures. Radiotherapy was better able to prevent local recurrence of the laterally and medially placed tumours than that of those in the midline of the breast. This is
partly because laterally placed tumours are more likely to give rise to axillary node metastases which can be detected at follow-up. Also, the possibility of incomplete removal is greater with peripheral tumours since they are likely to be excised with a smaller margin of surrounding normal tissue than central ones. Similarly, radiotherapy had a larger influence in preventing recurrence of larger tumours probably because the more advanced carcinomas would be more likely to have spread to lymph-nodes and less likely to have been adequately removed by local mastectomy. The induction of an artificial menopause was offered to patients who were premenopausal or less than three years postmenopausal because a previous trial had indicated that it produced a slight improvement in the reduction in the incidence of was unjustifiable to withhold this possible benefit from all patients although we appreciated that sterilisation would not be acceptable or considered appropriate in every case. We admit that the non-random way in which some patients did not have artificial induction of the menopause might introduce a slight bias to the results. However, it is now believed that the benefit of an artificial menopause is temporary -it delays but does not prevent recurrence or death in those who are destined to die.7 The long-term survival figures are thus unlikely to be influenced by induced menopause. Table n indicates that our results have not been significantly affected by the small number of patients who did not have an artificial menopause. Our study supports the now widely held view that there is little to choose between different methods of conventional treatment by surgery and/or radiotherapy. Postoperative radiotherapy for patients presenting in stage I can safely be delayed until they have evidence of local recurrence or lymph-node metastases. Simple mastectomy with postoperative radiotherapy or radical mastectomy alone are equally effective for treating stage-ii patients-the former is more time-consuming and expensive and the patient may have the unpleasant immediate sequelae of radiotherapy, but the latter produces greater disfigurement. The choice would depend on individual circumstances and preferences of the patient and surgeon. Surgery and/or radiotherapy may control the local survival
rates
and
a
recurrence.6 We felt that it
manifestation of breast cancer but the survival rate depends on the presence or absence of distant metastases and on host resistance. Any improvement on the present results can only be expected from adjuvant systemic therapy of which numerous regimens are being studied. The members of the committee which organised the trial were: Prof. M. R. Alderson (until 1970), Mr A. R. Anscombe, Dr R. Gibb, Mr A. W. Hargreaves (since 1975), Mr N. F. Kirkman, Dr 1. Leck (since 1970), Mr. M. Lentin, Mr J. P. Lythgoe, Dr I. D. H. Todd. Surgeons taking part in the trial were: Mr A. R. Anscombe, Mr M. A. Brennan, Mr A. S. Bullough, Mr H. J. Done, Mr H. M. Goldberg, Mr A. W. Hargreaves, Mr H. Hassall, Mr E. Henderson, Mr G. Ingram, Mr N. C. Keddie, Mr J. H. Kilshaw, Mr N. F. Kirkman, Mr D. K. Lennox, Mr M. Lentin, Mr W. H. Lonsdale, Mr J. P. Lythgoe, Mr J. Magell, Mr J. W. Main, Mr N. Macdonald, Mr A. F. Robinson, Mr J. Y. W. Russell, Mr 1. G. Schraibman, Mr H. F. Smith, Mr W. A. B. Strachan, Mr J. Wilks, Mr A. F. Williams. Radiotherapists taking part in the trial were: Dr J. R. Brown, Dr M. P. Cole, Dr D. P. Deakin, Dr M. B. Duthie, Prof. E. C. Easson, Dr R. Gibb, Dr N. K. Gupta, Dr A. W. Jackson, Dr D. M. Jackson, Dr I. J. Kerby, Dr D. Pearson, Dr R. S. Pointon, Dr G. G. Ribeiro, Dr E. Sherrah-Davis, Dr J. G. Stewart (who has since died), Dr M. L. Sutton, Dr I. D. H. Todd. The study was supported by a grant from the Manchester Regional Hospital Board.
REFERENCES
Bond, W. H. in The Treatment of Carcinoma of the Breast (edited by A. S. Jarrett); p. 24. Amsterdam, 1968. 2. Stjernsward, J. Lancet, 1974, ii, 1285. 3. T.N.M. Classification of Malignant Tumours. U.I.C.C., Geneva, 1968. 4. Peto, R., Pike, M. C., Armitage, P., et al. Br. J. Cancer, 1977, 35, 1. 5. Working Party of the Cancer Research Campaign. Br. med. J. 1976, i, 1035. 6. Paterson, R., Russell, M. H. J. Fac. Radiol. 1959, 10, 130. 7. Cole, M. P. in The Clinical Management of Advanced Breast Cancer— Second Tenovus Workshop (edited by C. A. F. Joslin and E. N. Gleave); p. 2. Cardiff, 1970. 1.
PROTRUDING AURICLE: A NEUROMUSCULAR SIGN DAVID W. SMITH
Department of Pediatrics, University of Washington, Seattle, U.S.A. HIROTADA TAKASHIMA
Department of Pediatrics, University of Washington, and Nihon University, Japan A protruding auricle is usually associated with a defect in development or function of the posterior auricular muscle, which normally draws the pinna towards the calvarium. Hence, a protruding auricle may be a sign of a neuromuscular disorder, as is ptosis.
Summary
Introduction MUSCLE function can influence the shape of cartilage and bone’-for example, ear muscles may affect the shape of the external auricle.2 Man has three extrinsic ear muscles extending from the calvarium to the cartilaginous pinna and six intrinsic pinna muscles.3 The role of these muscles is evident from the hearing-related movements of the external ear in animals such as the dog or cat. Though such movements are seldom noticeable in man, Seiler3 demonstrated by electromyographic techniques that the human ear muscles are responsive to sound stimuli. We have studied the impact of ear mus-
748
cles on gross auricular shape and especially the relation between the posterior auricular muscle and the protruding auricle. Patients and Methods The following groups were studied to determine the relation between the development of the auricle and the posterior auricular muscle: 12 human fetuses; 100 newborn infants; any patient we came across with protruding auricle; patients with specific disorders in which the sign occurs.4
Results In the 12 fetuses (aged 12-22 weeks), all of whom had auricles which were considered to be in normal positions, the posterior auricular muscle was a prominent and easily identifiable structure extending from its origin in the epicranial aponeurosis overlying the mastoid area of the temporal bone to its insertion on the inferior surface of the cartilaginous concha, at the root of the crus helix (fig. 1). The auricle was "held down" toward
Fig. 2-Protruding
auricles in
a
boy with Langer-Giedion syn-
drome. Hall,
B.
D.,
et
al. Birth
Defects orig. Article ser. 1974, 10,
148.
number of established disorders,4 (see accomtable and fig 2). We also found protruding auripanying in cles a number of patients without any known disorder but the majority of these patients had a problem which could have been associated with neuromuscular dysfunction-for example, one patient had idiopathic microcephaly. Eks has attributed protruding auricles in a patient to arthrogryposis multiplex congenita due to a cerebral defect and we see protruding ears as being analogous to multiple joint contractures, that is, as deformities secondary to the neuromuscular dysfunction. Another example of an association between protruding auricles and neuromuscular dysfunction is that of an intelligent 8-year-old boy with congenital muscle hypoplasia and generalized weakness of unknown cause but with good muscular coordination and no apparent deterioration of function (fig. 3). His male first cousin has the ture
in
a
I-Posterior auricular muscle in a 16-week fetus (above) and a 20-week fetus (below). The muscle extends from the mastoid region of the temporal bone to the inferior surface of the conchal cartilage, at the root of the crus helix. The posterior conchal and calvarial skin has been removed, the muscle dissected out, and the auricle pulled forward.
Fig.
Courtesy of Dr Thomas H. Shepard, Central Laboratory for Human Embryology and Pediatrics, University of Washington School of Medicine.
the calvarium in all stages of fetal development by this muscle. None of the 100 consecutive 1-3 day old infants had a protruding auricle-an auricle was considered protruding if the angle between the dorsal surface of the upper helical margin of the auricle and the plane of the pars petrosa of the os temporalis, measured by a transparent protractor, was 40 degrees or.more. Among the patients who had one or two protruding auricles two died in early infancy and their posterior auricular muscles were examined at necropsy. The one who had anencephaly and bilateral protruding ears had no posterior auricular muscle on either side. The other patient, who had died immediately after surgical repair of a cleft lip, was chromosomally normal; idiopathic holoprosencephaly anomaladand facial dysmorphogenesis were confirmed at necropsy. There was no posterior auricular muscle behind the protruding auricle but the muscle was present and inserted at the usual site on the other, normal-looking, auricle. A protruding auricle is an occasional to frequent fea-
Fig.3-8-year-old boy with non-progressive congenital muscle hypoplasia of unknown cause and protruding auricles. The anthelix is also poorly formed. Courtesy of Dr Hossein Massoud, Director of Children’s Hospital, Chattanooga, Tennessee.
Paediatrics, T.
C.
Thompson
disorder (and protruding auricles). The family studies suggest an X-linked recessive pattern of inheritance. The protruding auricles appear to be a consequence of a genetically determined generalised disorder of muscle hypoplasia, but it is not known whether the pathology is primarily neural or muscular. same
Discussion Our findings support the hypothesis that the posterior auricular muscle has a role in the normal positioning of
749
the auricle and that an absence or dysfunction of this muscle allows the auricle to protrude. It follows that the protruding auricle should be interpreted as a sign of a muscular or neurological deficiency of the posterior auricular muscle rather than as a sign of dysmorphogenesis of the auricular cartilage. Thus a protruding auricle can be expected in patients with muscular’disorders such as myotonic dystrophy,4 and there is an obvious relation of protruding auricles with the Moebius anomalad,4 in which there is congenital seventh nerve paralysis, since it is the seventh nerve which innervates the posterior auricular muscle. The absence of the posterior auricular muscle in two patients with protruding auricles and severe defects of brain development (anencephaly and holoprosencephaly) should not necessarily be taken as evidence of a primary muscle deficiency, since a defect of the functional innervation of a muscle early in its development will usually result in hypoplasia, or even absence, of the non-innervated muscle.6 We favour a primary neurological deficiency as the cause of the absence of the posterior auricular muscle in our patients with severe defects of early brain development and protruding auricles. Protruding auricles are associated with a number of disorders in which multiple defects include central nervous-system dysfunction (see accompanying table). The list includes disorders in which the clinical DISORDERS IN WHICH A PROTRUDING AURICLE MAY BE A FEATURE
Fig. 4—Girt with XO Turner syndrome, protruding auricles, ptosis of the left eyelid. Courtesy of Dr Judith G. Hall, Washington School of Medicine.
Division of Medical Genetics,
uncertain in disorders such
as
the XO Turner
and
University
of
syndrome
(fig. 4). Defects of development and/or function of the other extrinsic auricular muscles and of the six intrinsic auricular muscles also need to be studied in relation to other defects of the auricle. two
We thank Dr Thomas H. Shepard, of the Central Laboratory for Human Embryology, for the fetal material; and the department of medical photography and the nursing staff of the University Hospital newborn nursery, Mrs Lyle Harrah (research librarian), and Ms Jane Fowler (secretary) for their help. The work was supported by: the U.S. Bureau of Community Health Services, Health Services Administration, Public Health Service, the Department of Health, Education and Welfare, and the National Institutes of Health; and by Yoshida’s Science and Technology Foundation, Japan.
Requests for reprints should be addressed to D. W. S., Department of Pediatrics, RD-20, University of Washington School of Medicine, Seattle, Washington 98195. REFERENCES
1. Thomson, D’Arcy. On Growth and Form. Cambridge, 1942. 2. Moss, M. L. Birth Defects orig. Article Ser. 1975, 11, 283. 3. Seiler, R. Gegenbaurs morphol. Jahrb. (Leipzig) 1974, 120, 78. 4. Smith, D. W. in Recognizable Patterns of Human Malformation;
p. 485,
Philadelphia, 1976. 5. Ek, J. I. Acta pædiat. scand. 1958, 47, 302. 6. Eastlick, H. L. J. exp. Zool. 1973, 93, 27. 7. 8.
*++= frequent, 20% or more; += occasional, less than 20%.
features do not readily suggest a neural or muscular basis for the protruding auricle, but protruding auricles may be present in an otherwise normal individual and their familial occurrence has been reported.7 A protruding auricle is like ptosis of the eyelid, which may be due to a muscular or a neurological deficiency of the levator palpebrae superioris muscle, may occur by itself or as part of a generalised muscular disorder, or may be a consequence of brain dysfunction. A protruding auricle and ptosis of the eyelid may be present in the same patient. In myotonic dystrophy8 both signs relate muscle dysfunction. However, whether protruding auricles and/or the ptosis of the eyelids are due primarily to muscular or to neurological abnormalities remains to
Becker, O. J. Archs Otolar. 1949, 50, 541. Dodge, P. R., Gamstop, I., Byers, R. K., Russell,
P.
Pediatrics, 1965, 35, 3.
ACID-PHOSPHATASE REACTION IN ACUTE LYMPHOBLASTIC LEUKÆMIA D. CATOVSKY M. F. GREAVES C. PAIN
M. CHERCHI
G. JANOSSY H. E. M. KAY
M.R.C. Leukæmia Unit, Royal Postgraduate Medical School, London W12; Membrane Immunology Laboratory, Imperial Cancer Research Fund, London WC2; and M.R.C. Leukæmia Trials Office, Royal Marsden Hospital, London SW3
Summary
The diagnostic value of the acid-phosphatase reaction was assessed double-
blind in 148 cases of acute lymphoblastic leukæmia classified by surface-membrane markers and entered into the M.R.C. U.K. A.L.L. trials. 90% of cases of T-A.L.L. showed a positive reaction in the majority of blast cells, while only 2% of common-A.L.L. and 10% of
(A.L.L.)
