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Microcephaly associated with massive chronic subdural effusions
246
Brief clinical and laboratory observations
felter's s y n d r o m e a n d h y p o t h y r o i d i s m who h a d elevated concentrations of sweat electrolytes. T h e concentration of the latter r e t u r n e d to n o r m a l after t r e a t m e n t with thyroxine. A l t h o u g h our p a t i e n t did not present with the classical symptoms of infantile hypothyroidism, the decreased p r o t e i n - b o u n d iodine levels a n d the r e m a r k a b l e response to exogenous thyroid speak strongly for the diagnosis. Discontinuation of m e d i c a t i o n and thyroid evaluation should be a t t e m p t e d a n d r e p o r t e d when the p a t i e n t is 2 or 3 years of age. The author expresses his thanks to Dr. A. L. Roby of Jeffersonville, Ind., for providing the follow-up data at 9 months of age.
3licrocephaly associated with massive chronic subdural effusions T h o m a s E. C a d m a n , M.D., ~ Y o u n g B. Lee, M.D., a n d S a m u e l H. T u c k e r , M . D .
PHILADELPHIA,
PA.
T H E ASSOCIATION of chronic subdural effusions with increase h e a d size in infancy is well known; significant e n l a r g e m e n t of the h e a d is recorded as a presenting sign in 30 to 65 per cent of cases. 1-5 I t is not well recognized, however, that on occasion the size of
From the Departments of Neurology and Pediatrics of the University of Pennsylvania School of Medicine and the Children's Hospital of Philadelphia. ~Supported by Speclal Fellowship 2 F l l NB 1481-03 and Trazning Grant No. 2 T I N B 5260-09, National Institute o] Neurological Disease and Blindness, United States Public Health Service. Address: 1740 Bainbridge St., Philadelphia, Pa.
The Journal of Pediatrics August 1968
REFERENCES
1. di Sant'Agnese, P. A., Darling, R. C., Perera, G. A., and Snea, E.: Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas, Pediatrics 12: 549, 1953. 2. di Sant'Agnese, P. A.: Cystic fibrosis of the pancreas, in Nelson, W. E., editor: Textbook of pediatrics, ed. 8, Philadelphia, 1964, W. B. Saunders Company, p. 779. 3. Corm, J. W.: Electrolyte composition of sweat, Arch. Int. Med. 83: 416, 1949. 4. Means, M. A., and Dobson, R. L.: Cytological changes in the sweat gland in hypothyroidism, J. A. M. A. 186: 113, 1963. 5. Grand, R. J., Rosen, S. W., di Sant'Agnese, P. A., and Kirkham, W. R,: Unusual case of XXY Klinefelter's syndrome with pancreatic insufficiency, hypothyroidism, deafness, chronic lung disease, dwarfism and microcephaly, Am. J. Med. 4 h 478, 1966.
the h e a d m a y be smaller than average in infants with subdural collections of fluid. W e are a w a r e of only one r e p o r t in which m e n t i o n was m a d e of small h e a d size in association with a subdural effusion; this was the case in 3 of 45 infants in the series of I n g r a h a m a n d Matson. G W e have recently treated an 8 ~ - m o n t h old infant w h o h a d microcephaly with massive bilateral subdural effusions. CASE REPORT
The patient, a Caucasian girl, was born following a 39 week gestation; the mother was in good health during this her fourth pregnancy. Labor lasted 2 hours and delivery was spontaneous. The birth weight was 3.9 kilograms (ninetieth percentile), and the head circumference was 35.5 centimeters (seventy-fifth percentile). The 1 minute Apgar score was 9; the nursery course was uncomplicated. Growth and development were said to be normal until 5 months, and no routine medical care was sought during this period. At age 5 months the infant either rolled from a table striking her head in the fall, or was deliberately traumatized. Several hours later she vomited blood, and the following day an episode of apnea prompted admission to another hospital, where she was described as semiconscious and cyanotic, with coarse tremors of her arms. Her weight was 5.5 kilograms (below the third
Volume 73 Number 2
Brie/ clinical and laboratory observations
24 7
F3-CZ
C3~Cz
P3-Cz
01 -Cz
F4"Cz
C4-Cz
p4-'Cz
O2-Cz
~__~Sft 1 S|C
MY
Fig. 1. Electroencephalogram showing" suppression of voltage in all leads.
percentile). The anterior fontanel was buiging and tense; the head circumference of 42.5 centimeters was in the fiftieth percentile. The pupils were constricted and reacted sluggishly; the fundi showed no abnormalities. There was spasticity of all extremities, but the deep tendon reflexes were normal. The cerebrospinal fluid contained 1 white blood cell and 125 red blood cells; the protein was 38 mg. per 100 ml. and glucose 64 mg. per 100 ml. Skull roentgenograms were normal, without widening of the sutures (and without the thickening of the vault as demonstrated 3 ~ months later). Subdural taps yielded 40 mI. of dark red blood from the right side and 5 ml. from the left. No fluid was obtained in taps 5 days later. An electroencephalogram 2 weeks after admission showed marked suppression of cortical voltage in all leads. The patient's 2 month hospitalization was complicated by upper intestinal bleeding, anemia, diarrhea, and urinary tract infection. However, when discharged at age 7 months she was alert and aware of her surroundings, the anterior fontanel was not unusual, and the head circumference was 41.4 centimeters (now decreased to below the third percentile). Six weeks following discharge, increasing spasticity and slow psychomotor development suggested the need for further study. The child was admitted to the Children's Hospital of Philadelphia at age 8 ~ months. Her weight was
6.5 kilograms (below the third percentile); height, 68.6 centimeters (fifteenth percentile); and head circumference, 42.5 centimeters (third percentile). She was irritable. The anterior fontanel was flat, though membranous, firm, and slightly pulsating. Transillumination, with a standard 7.5 volt flashlight and rubber head adapter, was negative. The cranial nerve functions were normal, but muscle tone was grossly increased in all extremities, and myotatic reflexes were hyperactive. Gross motor and socioadaptive abilities were those of a child aged 3 months. Skull roentgenograms showed thickening of the vault. The sutures were normal, and there was no intracranial calcification. Marked suppression of voltage in all areas on electroencephalography (Fig. 1) suggested the presence of considerable collections of subdural fluid separating the cerebral cortex from the scalp electrodes. A right carotid arteriogram with cross compression confirmed the presence of massive bilateral subdural masses; the brain was separated from the inner table by 2.6 era. in the parietal regions (Fig. 2). On right and left frontoparietal craniotomies 2 weeks apart, approximately I00 to 150 ml. of xanthochromic fluid was aspirated from each of the deep subdural spaces covering both hemispheres, the fluid being encased in a very thick outer membrane and a thin inner membrane. When the latter was stripped off, the brain appeared small
248
Brief clinical and laboratory observations
The Journal of Pediatrics August 1968
B
Fig. 2. A and B, Right carotid arteriogram (anteroposterior and lateral views). Arrows indicate outer limits of the cortex.
Volume 73 Number 2
Brie[ clinical and laboratory observations
but otherwise normal. Her neurological status did not improve following these procedures.
COMMENT T h e volume of intracranial contents can be fairly accurately computed from measurements on plain skull films. Using the formula of Gordon and associates, 7 we estimated the intracranial volume in our patient to have been, prior to the craniotomies, approximately 660 ml., which is below the fifth percentile for age and is in the microcephalic range. I n a s m u c h as 200 to 300 ml. of subdural fluid was subsequently removed, it would seem that the patient's brain must have been reduced to approximately one third of the size expected for her age. This m a y offer some explanation for the failure of mental or neurologic improvement following decompression. It is not clear why the cranial volume was small despite such large subdural effusions. It may be that brain growth was restricted by the thick inner m e m b r a n e or that post traumatic atrophy had occurred. It is also possible that the brain was small prior to the trauma at age 5 months. T h e fact that the vault was not thickened at that time would suggest that brain growth was not previously
Treatment of EMng's sarcoma with concurrent radiotherapy and chemotherapy
H. Omar Hustu, M.D., Charlene Holton, M.D., David James, Jr., M.D., and Donald Pinkel, M.D. MEMPHIS~
TENN.
249
grossly retarded. It is, however, impossible to make any definite conclusions regarding this factor since we have no reliable information as to neurological development or head size between birth and 5 months. Whatever the pathophysiology, it is important to remember that, although infants with chronic subdural effusions generally have heads larger than normal, microcephaly does not preclude chronic subdural fluid coIlections. We are indebted to Mr. Art Siegal preparation of the photographs.
for
REFERENCES 1. Jackson, I. J., and Thompson, R. K.: Pediatric neurosurgery, Springfield, Ill., 1959, Charles C Thomas, Publisher. 2. Ingraham, F. D., and Matson, D. D.: Subdural hematoma in infancy, Adv. Pediat. 4: 231, 1949. 3. Russell, P. H.: Subdural hematoma in infancy, Brit. M. J. 2: 446, 1965. 4. Guthkelch, A. N.: Subdural effusions in infancy, Brit. M. J. 1: 233, 1953. 5. Elvidge, A. R., and Jackson, I. J.: Subdural hematoma and effusion in infants, Am. J. Dis. Child. 78: 635, 1949. 6. Ingraham, F. D., and Matson, D. D.: Subdural hematoma in infancy, J. PEDIAT.24: 1, 1944. 7. Gordon, Z. R. S.: Measurement of cranial capacity in children, Brit. J. Radiol. 39: 377, 1966.
ALTHOUGH EWlNG'S SARCOMA of bone is most often localized at time of diagnosis, patients with this tumor have usually had a rapidly fatal course, whether treated with amputation or radiotherapy. I n one extensive collection of cases x the disease was reported to be fatal in approximately 85 per cent of patients within 2 years of diagnosis. Only 8 per cent survived 5 years. A more optimistic report was that of a From the St, Jude Children's Research Hospital, and Departments o[ Radiology and Pediatrics, University o[ Tennessee. Supported by United States Public Health Service research project Grants Nos. CA08-480 and CA07594, and training Grant No. CA05176, [rom the National Cancer Institute, and by American Lebanese Syrian Associated Charities (ALSAC)
Brief clinical and laboratory observations
felter's s y n d r o m e a n d h y p o t h y r o i d i s m who h a d elevated concentrations of sweat electrolytes. T h e concentration of the latter r e t u r n e d to n o r m a l after t r e a t m e n t with thyroxine. A l t h o u g h our p a t i e n t did not present with the classical symptoms of infantile hypothyroidism, the decreased p r o t e i n - b o u n d iodine levels a n d the r e m a r k a b l e response to exogenous thyroid speak strongly for the diagnosis. Discontinuation of m e d i c a t i o n and thyroid evaluation should be a t t e m p t e d a n d r e p o r t e d when the p a t i e n t is 2 or 3 years of age. The author expresses his thanks to Dr. A. L. Roby of Jeffersonville, Ind., for providing the follow-up data at 9 months of age.
3licrocephaly associated with massive chronic subdural effusions T h o m a s E. C a d m a n , M.D., ~ Y o u n g B. Lee, M.D., a n d S a m u e l H. T u c k e r , M . D .
PHILADELPHIA,
PA.
T H E ASSOCIATION of chronic subdural effusions with increase h e a d size in infancy is well known; significant e n l a r g e m e n t of the h e a d is recorded as a presenting sign in 30 to 65 per cent of cases. 1-5 I t is not well recognized, however, that on occasion the size of
From the Departments of Neurology and Pediatrics of the University of Pennsylvania School of Medicine and the Children's Hospital of Philadelphia. ~Supported by Speclal Fellowship 2 F l l NB 1481-03 and Trazning Grant No. 2 T I N B 5260-09, National Institute o] Neurological Disease and Blindness, United States Public Health Service. Address: 1740 Bainbridge St., Philadelphia, Pa.
The Journal of Pediatrics August 1968
REFERENCES
1. di Sant'Agnese, P. A., Darling, R. C., Perera, G. A., and Snea, E.: Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas, Pediatrics 12: 549, 1953. 2. di Sant'Agnese, P. A.: Cystic fibrosis of the pancreas, in Nelson, W. E., editor: Textbook of pediatrics, ed. 8, Philadelphia, 1964, W. B. Saunders Company, p. 779. 3. Corm, J. W.: Electrolyte composition of sweat, Arch. Int. Med. 83: 416, 1949. 4. Means, M. A., and Dobson, R. L.: Cytological changes in the sweat gland in hypothyroidism, J. A. M. A. 186: 113, 1963. 5. Grand, R. J., Rosen, S. W., di Sant'Agnese, P. A., and Kirkham, W. R,: Unusual case of XXY Klinefelter's syndrome with pancreatic insufficiency, hypothyroidism, deafness, chronic lung disease, dwarfism and microcephaly, Am. J. Med. 4 h 478, 1966.
the h e a d m a y be smaller than average in infants with subdural collections of fluid. W e are a w a r e of only one r e p o r t in which m e n t i o n was m a d e of small h e a d size in association with a subdural effusion; this was the case in 3 of 45 infants in the series of I n g r a h a m a n d Matson. G W e have recently treated an 8 ~ - m o n t h old infant w h o h a d microcephaly with massive bilateral subdural effusions. CASE REPORT
The patient, a Caucasian girl, was born following a 39 week gestation; the mother was in good health during this her fourth pregnancy. Labor lasted 2 hours and delivery was spontaneous. The birth weight was 3.9 kilograms (ninetieth percentile), and the head circumference was 35.5 centimeters (seventy-fifth percentile). The 1 minute Apgar score was 9; the nursery course was uncomplicated. Growth and development were said to be normal until 5 months, and no routine medical care was sought during this period. At age 5 months the infant either rolled from a table striking her head in the fall, or was deliberately traumatized. Several hours later she vomited blood, and the following day an episode of apnea prompted admission to another hospital, where she was described as semiconscious and cyanotic, with coarse tremors of her arms. Her weight was 5.5 kilograms (below the third
Volume 73 Number 2
Brie/ clinical and laboratory observations
24 7
F3-CZ
C3~Cz
P3-Cz
01 -Cz
F4"Cz
C4-Cz
p4-'Cz
O2-Cz
~__~Sft 1 S|C
MY
Fig. 1. Electroencephalogram showing" suppression of voltage in all leads.
percentile). The anterior fontanel was buiging and tense; the head circumference of 42.5 centimeters was in the fiftieth percentile. The pupils were constricted and reacted sluggishly; the fundi showed no abnormalities. There was spasticity of all extremities, but the deep tendon reflexes were normal. The cerebrospinal fluid contained 1 white blood cell and 125 red blood cells; the protein was 38 mg. per 100 ml. and glucose 64 mg. per 100 ml. Skull roentgenograms were normal, without widening of the sutures (and without the thickening of the vault as demonstrated 3 ~ months later). Subdural taps yielded 40 mI. of dark red blood from the right side and 5 ml. from the left. No fluid was obtained in taps 5 days later. An electroencephalogram 2 weeks after admission showed marked suppression of cortical voltage in all leads. The patient's 2 month hospitalization was complicated by upper intestinal bleeding, anemia, diarrhea, and urinary tract infection. However, when discharged at age 7 months she was alert and aware of her surroundings, the anterior fontanel was not unusual, and the head circumference was 41.4 centimeters (now decreased to below the third percentile). Six weeks following discharge, increasing spasticity and slow psychomotor development suggested the need for further study. The child was admitted to the Children's Hospital of Philadelphia at age 8 ~ months. Her weight was
6.5 kilograms (below the third percentile); height, 68.6 centimeters (fifteenth percentile); and head circumference, 42.5 centimeters (third percentile). She was irritable. The anterior fontanel was flat, though membranous, firm, and slightly pulsating. Transillumination, with a standard 7.5 volt flashlight and rubber head adapter, was negative. The cranial nerve functions were normal, but muscle tone was grossly increased in all extremities, and myotatic reflexes were hyperactive. Gross motor and socioadaptive abilities were those of a child aged 3 months. Skull roentgenograms showed thickening of the vault. The sutures were normal, and there was no intracranial calcification. Marked suppression of voltage in all areas on electroencephalography (Fig. 1) suggested the presence of considerable collections of subdural fluid separating the cerebral cortex from the scalp electrodes. A right carotid arteriogram with cross compression confirmed the presence of massive bilateral subdural masses; the brain was separated from the inner table by 2.6 era. in the parietal regions (Fig. 2). On right and left frontoparietal craniotomies 2 weeks apart, approximately I00 to 150 ml. of xanthochromic fluid was aspirated from each of the deep subdural spaces covering both hemispheres, the fluid being encased in a very thick outer membrane and a thin inner membrane. When the latter was stripped off, the brain appeared small
248
Brief clinical and laboratory observations
The Journal of Pediatrics August 1968
B
Fig. 2. A and B, Right carotid arteriogram (anteroposterior and lateral views). Arrows indicate outer limits of the cortex.
Volume 73 Number 2
Brie[ clinical and laboratory observations
but otherwise normal. Her neurological status did not improve following these procedures.
COMMENT T h e volume of intracranial contents can be fairly accurately computed from measurements on plain skull films. Using the formula of Gordon and associates, 7 we estimated the intracranial volume in our patient to have been, prior to the craniotomies, approximately 660 ml., which is below the fifth percentile for age and is in the microcephalic range. I n a s m u c h as 200 to 300 ml. of subdural fluid was subsequently removed, it would seem that the patient's brain must have been reduced to approximately one third of the size expected for her age. This m a y offer some explanation for the failure of mental or neurologic improvement following decompression. It is not clear why the cranial volume was small despite such large subdural effusions. It may be that brain growth was restricted by the thick inner m e m b r a n e or that post traumatic atrophy had occurred. It is also possible that the brain was small prior to the trauma at age 5 months. T h e fact that the vault was not thickened at that time would suggest that brain growth was not previously
Treatment of EMng's sarcoma with concurrent radiotherapy and chemotherapy
H. Omar Hustu, M.D., Charlene Holton, M.D., David James, Jr., M.D., and Donald Pinkel, M.D. MEMPHIS~
TENN.
249
grossly retarded. It is, however, impossible to make any definite conclusions regarding this factor since we have no reliable information as to neurological development or head size between birth and 5 months. Whatever the pathophysiology, it is important to remember that, although infants with chronic subdural effusions generally have heads larger than normal, microcephaly does not preclude chronic subdural fluid coIlections. We are indebted to Mr. Art Siegal preparation of the photographs.
for
REFERENCES 1. Jackson, I. J., and Thompson, R. K.: Pediatric neurosurgery, Springfield, Ill., 1959, Charles C Thomas, Publisher. 2. Ingraham, F. D., and Matson, D. D.: Subdural hematoma in infancy, Adv. Pediat. 4: 231, 1949. 3. Russell, P. H.: Subdural hematoma in infancy, Brit. M. J. 2: 446, 1965. 4. Guthkelch, A. N.: Subdural effusions in infancy, Brit. M. J. 1: 233, 1953. 5. Elvidge, A. R., and Jackson, I. J.: Subdural hematoma and effusion in infants, Am. J. Dis. Child. 78: 635, 1949. 6. Ingraham, F. D., and Matson, D. D.: Subdural hematoma in infancy, J. PEDIAT.24: 1, 1944. 7. Gordon, Z. R. S.: Measurement of cranial capacity in children, Brit. J. Radiol. 39: 377, 1966.
ALTHOUGH EWlNG'S SARCOMA of bone is most often localized at time of diagnosis, patients with this tumor have usually had a rapidly fatal course, whether treated with amputation or radiotherapy. I n one extensive collection of cases x the disease was reported to be fatal in approximately 85 per cent of patients within 2 years of diagnosis. Only 8 per cent survived 5 years. A more optimistic report was that of a From the St, Jude Children's Research Hospital, and Departments o[ Radiology and Pediatrics, University o[ Tennessee. Supported by United States Public Health Service research project Grants Nos. CA08-480 and CA07594, and training Grant No. CA05176, [rom the National Cancer Institute, and by American Lebanese Syrian Associated Charities (ALSAC)