Conference at the Mount Sinai Hospital of New York

Conference at the Mount Sinai Hospital of New York

Clinical Conference C O N F E R E N C E A T T H E M O U N T S I N A I H O S P I T A L OP N E W Y O R K DR. HORACE L. HODES, PEDIATRICIAN, AND DR. SAMU...

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Clinical Conference C O N F E R E N C E A T T H E M O U N T S I N A I H O S P I T A L OP N E W Y O R K DR. HORACE L. HODES, PEDIATRICIAN, AND DR. SAMUEL KARELITZ, 2~tSSOCIATE PEDIATRICIAN o d a y ' s clinic we have selected F fORo u r teases, each with central nervous system involvement. Two patients had neoplastic disease proved in one instance by surgery a n d in the other at post-mortem examination. The other two patients suffered f r o m infection probably originating in the middle ears in one and in the frontal sinuses in the other. I n neither of the latter two eases can we be absolutely certain of our clinical diagnoses although t h e r a p y based on these .impressions resulted in a p p a r e n t cure.

Case 1. Congenital Brain Tumor in an Infant One l~onth of Age Dm SAMUEL SAPIN (Resident Pediatrician). A 2 8 9 white girl was admitted to The Mount Sinai Hospital because of left facial paralysis a n d vomiting. The infant was delivered without difficulty a f t e r a normal p r e g n a n c y and weighed 6 pounds, 5 ounces. The family history was not remarkable. The baby was breast fed and progressed normally until 4 weeks of age when, following a severe cold without fever, she began to regurgitate her feedings in a nonprojcctile m a n n e r and would choke while feeding. A left facial weakness was noted. I t was" subsequently noted t h a t her head was held constantly t u r n e d to the right. The i n f a n t became apathetic, her cry became weaker, she no longer followed

light, and weight gain ceased. Convulsions were not noted. Physical examination on admission revealed a small, chronically ill, 21/2month-old girl who could not follow light and could not lift her head. The temperature, pulse rate, r e s p i r a t o r y rate, and blood pressure were normal. H e r weight was 9 pounds, 10 ounces; length, 23 inches; head circumference, 16 88 inches; chest circumference, 141fi~ inches~ H e r cry was weak a n d she a p p e a r e d to have great difficulty in swallowing. The head was held t u r n e d to the right and the right side of the head was flattened. The anterior fontanel was slightly bulging, tense, and ~heasured 2 x 1 ~ inches. The sutures of the skull were separated. The scalp veins were slightly dilated. Slight b l u r r i n g of the margins of both optic discs was the only fundal abnormality. There were no meningeal signs. A left facial palsy of the peripheral type was present. The gag reflex was somewhat diminished. Tonic neck reflexes were present. There was a poor grasp reflex, but no weakness of the extremities. The deep tendon reflexes were equal .and active. The remainder of the examination was not remarkable. The urine and blood counts were normal. X - r a y examination of the skull revealed widely separated sutures but showed no evidence of intracranial calcification. An electroencephalogram

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showed no abnormalities. No fluid was obtained on bilateral subdural punctures done on admission, but lumbar p u n c t u r e revealed slightly xanthochromic spinal fluid under considerably increased pressure. The fluid contained 130 fresh red blood cells and 10 lymphoeytes per cubic millimeter and a protein concentration of 63 rag. per cent. Following spinal tap, the infant became considerably more alert and the left facial palsy disappeared ahnost completely. This improvement, however, lasted only two days. A therapeutic dose of Prostigmine Methylsulfate, 0.3 rag. intramuscularly, to rule out myasthenia gravis did not affect her condition. A ventriculogram done ten gays a f t e r admission revealed a thin cerebral cortex and massive dilatation of both lateral ventricles. The third and f o u r t h ventricles were not visualized. This picture was interpreted as being consistent with a spaceoccupying lesion in the posterior fossa or with atresia of the aqueduct. Sixty cubic centimeters of fluid had bden removed and 50 c.c. of air were injected. The infant swallowed and sucked poorly and was fed by gavage. She continued to regurgitate through her mouth and nose. The head grew larger, and twelve days a f t e r admission, measured 18 inches. F e v e r app e a r e d on the t h i r t i e t h hospital d a y and the patient expired two days later. Post-mortem examination revealed partial atelectasis of all lobes of the lungs and f a t t y degeneration of the liver. The brain weighed 650 grams, a p p e a r e d voluminous and was collapsed. There was a m a r k e d internal hydrocephalus; the third ventricle and aqueduete were enlarged and the optic ehiasm was stretched. A large, brownish gray, highly vascular t u m o r was

found in the cerebe]lopontine angle. This mass occupied most of the left side of the pons and extended out into the cerebellar hemisphere (Figs. ] and 2). The posterior lobe of this half of the cerebellum was almost completely disintegrated by the tumor. On microscopic e x a m i n a t i o n the t u m o r was found to be a very primitive gliagenous neoplasm which was classified by Dr. J. H. Globus as a neuroepithelioma. A space-occupying lesion, probably a brain tumor, was considered to be the most likely diagnosis on admission. The child's course in the hospital together with the results of m a n y studies confirmed this impression and excluded other diagnostic possibilities. These conditions included meningitis with subsequent hydrocephalus and facial palsy, subdural hematoma, toxoplasmosis and hydrocephalus due to eongenital obstruction of the aqueduct: The absence of retinitis and the lack of intracranial calcification were eonsidered to exclude toxoplasmosis. Failure to obtain fluid on bilateral subdural p u n c t u r e eliminated the possibility of subdural hematoma. The incidence of brain tumors in early infancy is low. I n one series of 100 eases of brain tumors in children reported by Bailey, Buchanan, and Buoy ~ only 5 per cent occurred in children u n d e r one year of age. I n K e i t h ' s 2 large series of 427 eases of brain tumors in children, only 1.3 p e r cent occurred in infants under one y e a r of age. B r a i n t u m o r s in children differ f r o m adults both in location and t y p e 2 I n children about 65 to 70 per eent of brain tumors occur in the infratentoriaI region. In infants this localization is not so frequent. About 75 to 85 per cent o2 all brain tumors in children are gliomas.

CLINICAL

Unlike adults, there are relatively fe~meningiomas, p i t u i t a r y adenomas, and neurinomas. Neuroepitheliomas arise from a very primitive and undifferentiated type of parent cell and are therefore highly malignant. It is difficult to obtain statistical data regarding the incidence of this tumor in in-

Fig.

].--Ventral

Fig.

2.--Cross

surface

section

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wise other diagnostic aids, such as disturbances of gait, are missing since they have not yet learned to stand or walk. The earliest sign is usually enlargement of tile head accompanied by tenseness of the fontanel and widening of the sutures of the skull. Ophthalmoscopic examination may reveal papil-

of the lower brain stem and cerebellum, showing tumor as presenting itself on the surface.

of brain

stem and cerebellum, showing depth of affected structures.

rants and children because the histogenetic classification of brain tmnors varies among neuropatho]ogists. I t may be stated with assurance, however, that this is a very rare tumor. The symptoms of brain tumors in infancy differ from those of older children. Infants are too young to indicate headache and visual disturbance. Like-

the

extent

the

extent

of the tumor

of the

into the

ledema, but in many cases tlie fundi are entirely normal. Nonspeeifie symptoms, such as vomiting and apathy, are frequent. As illustrated in this patient, vomiting is not necessarily projectile in character. Not infrequently fever is present, a finding which may lead to an erroneous diagnosis of meningitis or encephalitis. Convul-

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sions are more commonly encountered in infants with subdural hematomas than in patients with brain tumors. However convulsions occur more often with brain tumors than with congenital hydrocephalus. Convulsions are particularly rare with infratentorial tumors. The spinal fluid of children with brain tumors m a y contain an increased number of lymphocytes and an increased concentration of protein. These, too, m a y lead to the suspicion of an inflamm a t o r y process. I n b r a i n tumors the s y m p t o m s m a y be i n t e r m i t t e n t a n d thus postpone a definite diagnosis. DISCUSSION

QUESTION.--Is the electroencephalog r a m of a n y help in the diagnosis of brain tumors in children ? DR. HODES.--It is k n o w n t h a t t h e

interpretation of electroencephalograms in infants is difficult. I n a recent r e p o r t 4 of such tracings done on t h i r t y children with posterior fossa brain tumors, 70 per cent were said to show bilateral abnormalities in leads f r o m the occipital and adjacent areas. The electroencephalogram, therefore, m a y be of some help but is not to be relied upon if it is negative. QUESTION.~WI~at is the prognosis in brain tumors in children ? Dm S. W. GRoss.--The over-all outlook is grave. The only favorable tumor which one sees in children is the astroeytoma. I f this t u m o r can be removed surgically, in toto, p e r m a n e n t cure can be expected. Medulloblastomas respond to x - r a y therapy. In a large series of over 400 brain tumors in children, only 13 p e r cent of the total n u m b e r lived five years or longer and most of these patients had astroeytomas.

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PEDIATRICS

QuEsTmN.--Should a l u m b a r puncture be done if the existence of a brain t u m o r is suspected? DR. GRoss.--There have been m a n y warnings about the danger of spinal punctures in the presence of brain tumors, and m a n y agree t h a t if there is evidence of m a r k e d increase of int r a e r a n i a l pressure, a spinal tap is hazardous. I f on clinical grounds the pressure is believed to be only moderately increased, careful removal of a small amount of spinal fluid m a y not be h a r m f u l and m a y be of value in establishing a diagnosis. QuEsTIo~.--Might not a ventricular t a p have been safer than a spinal tap in this baby ? DR. GRoss.--Yes. QUESTION.--Why was s u r g e r y not p e r f o r m e d in this b a b y and w h a t are the results of surgery for brain tumors in infants? DR. GROSS.--During hospitalization the i n f a n t was never in adequate condition for extensive surgery. 0UESTION.--Are these brain tumors of congenital origin? Are the t u m o r cells present but lying d o r m a n t since birth ? DR. GROSS.--Perhaps Dr. Globus would p r e f e r to answer this question. D~. J. H. G L o s i s . - - T h e histogenetic basis of classifying p r i m a r y neuroectodermal brain tumors was advanced by me a little over t h i r t y years ago. Subsequently it was accepted b y others interested in the field. I n accordance with this e0ncept, all p r i m a r y true brain tumors take their origin from embryonal residues with the m a j o r i t y of their cells duplicating some phase in the evolution of cell forms, those

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which ultimately differentiate into the specific constituents of the nervous system. Some of the true brain tumors, for obvious reasons, are best classified as neuroepitheliomas. They are those which consist p r e d o m i n a n t l y of primifive epithelial cells, among which there are encountered cells of more m a t u r i n g type. I n line with the accepted concept t h a t the more primitive the cell the more m a l i g n a n t it is, it m a y be concluded t h a t a neuroepithelioma, consisting, as it does, of the most primitive n e u r o e c t o d e r m a l cell forms, is therefore most malignant and, for the same reason, quite rare.

REFERENCES I. Bailey, P.~ Buchanan, D. N, and Bucy, P.C.: Intracranial, Tumors of ]~nfancy and Childhood, Chicag% 1939, University of Chicago. Press. 2. ]~eith, H. M., Winchell, l~[cI<. C., and ~ernohan, J.W.: Brain Tmnors in Children, Pediatrics 3: 839, 1949. 3. Gross, S. W." B r a i n Tumors in Infancy, Am. J. Dis. Child. 48: 739, 1934. 4. Drumheller, J. W., I
Case 2. Congenital Neuroblastoma of the Spinal Canal

DR. GROSs.--Medulloblastoma is the most radiosensitive of these. Patients m a y go on for two to five years a f t e r a course of radiotherapy. Survival of ten years is known:

DR. AVRUM LABE KATCtIER (Assistant R e s i d e n t ) . - - A n 8-day-old female i n f a n t was admitted to the hospital because of failure to move h e r lower extremities. P r e g n a n c y had been norma] and the delivery spontaneous and uncomplicated. The child weighed 7 pounds, 9 ounces at birth. On the first day of life both legs were noted to be flaccid, and only very feeble movements of the lower extremities were observed a f t e r that t i m c

COMMENT BY DR. GROSS.--Brain t u m o r was the most likely lesion in this patient and that was the admission diagnosis. I n infancy, vomiting, listlessness, and r a p i d enlargement of the head should always lead to a suspicion of a brain t u m o r and should p r o m p t the medical .attendant to c a r r y out further procedures to establish the correct diagnosis. Subdural taps are done. I f these arc negative, v e n t r i c u l o g r a p h y should be carried out without delay. In this ease the diagnosis seemed certain a f t e r ventrieulography. Surgical intervention was delayed in the hope t h a t the i n f a n t ' s condition could be improved. However, in spite of adequate supportive t h e r a p y she never improved sufficiently to w a r r a n t exploration of the posterior fossa.

Physical examination o n admission to the hospital revealed the following: T e m p e r a t u r e was 98 ~ F., pulse 110, respirations 30, and blood pressure 85/40. The child was a well-developed and well-nourished infant who did not seem ill or in distress. The head was normal in size a n d shape. The anterior fontanel was ] • 1 cm. in size, soft, and flat. The posterior fontanel was closed. There was slight overriding of the sutures. There was a small hemorrhage on the se]era of the left eye medial to the limbus. The fundi were normal. The ears, nose, and throat were not remarkable. The chest was clear to percussion and auscultation. The heart was not remarkable. The abdomen was soft and nontender. The liver edge was 3 cm. below the costal margin.

QUESTmN.--Are a n y of these tumors radiosensitive ?