262
Brie[ clinical and laboratory observations
T a b l e I I I . S u m m a r y of first a n d last I.Q. tests of 37 phenylketonuric children a r r a n g e d in same m a n n e r as Birch a n d T i z a r d 1~
Mean I.Q.'s Group o[ children Treated Treated Treated Treated
before 2 months (N ~ 9) 3 to 11 months (N ~ 4) 12 to 24 months (N ~ 16) 25 to 60 months (N = 8)
Unaffected siblings (N =- 32)
First ] Last test test 83 32 45 44
82 40 65 64
109
--
results of the first I.Q. test regardless of the age when the diet was started. While the therapeutic value of the lowphenylalanine diet remains to be proven by means of a control study, m we do not believe that any d a t a are available to disprove the clinical impression that the diet has an effect on the I.Q. of children with phenylketonuria, p a r t i c u l a r l y if it is started at a n early age. U n t i l there is definitive proof that the dietary t r e a t m e n t of phenylketonuria is ineffective, patients with this condition should continue to be given the diet. REFERENCES 1. Bickel, H., Gerrard, J., and Hickmans, E. M.: Influences of phenylalanine intake on phenylketonuria, Lancet 2: 812, 1953.
Nonsurgical elevation of depressed skull fracture in an infant Richard Raynor, M.D., and Mohammad Parsa, M.D. NEW
YORK,
N.
Y.
From the Department o[ Neurological Surgery, Columbia University College o[ Physicians and Surgeons, Harlem Hospital Center.
The ]ournal o[ Pediatrics February 1968
2. Knox, W. E.: An evaluation of the treatment of phenylketonuria with diets low in phenylalanine, Pediatrics 26: 1, 1960. 3. Coates, S.: Results of treatment in phenylketonuria, Brit. M. J. 1: 767, 1961. 4. Berman, P. W., Graham, F. K., Eichman, P. L., and Waisman,-H. A.: Psychologic and neurologic status of diet-treated phenylketonuric children and their siblings, Pediatrics 28: 924, 1961. 5. Kang, E. S., Kennedy, J. L., Gates, L., Burwash, I., and McKinnon, A.: Clinical observations in phenylketonuria, Pediatrics 35: 932, 1965. 6. Hsia, D. Y. Y.: Phenylketonuria: A study of human biochemical genetics, Pediatrics 38: 173, 1966. 7. Koch, R., Acosta, P., Fishler, K., Schaeffler, G., and Wohlers, A.: Clinical observation on phenylketonuria, Am. J. Dis. Child. 113: 6, 1967. 8. Berman, P. W., Waisman, H. A., and Graham, F. K.: Intelligence in treated phenylketonuric children: A development study, Child Develop. 37: 731, 1966. 9. Dobson, J, C., et al.: The relationship between cognitive development and dietary treatment in pheny]ketonuria. Presented at the Conference of the American Association of Mental Deficiency, May 19, 1967. 10. Sutherland, B. S., Umbarger, B., and Berry, H. K.: The clinical management of phenylketonuria, GP 35: 93, 1967. 11. Birch, H. G., and Tizard, J.: The dietary treatment of phenylketonuria, Develop. Med. & Child. Neurol. 9: 9, 1967. 12. Hsia, D. Y. Y., Knox, W. E., Quinn, K. V., and Paine, R. S.: A one-year controlled study of the effect of low-phenylalanine diet on phenylketonuria, Pediatrics 21: 178, 1958.
E L E V A T I O N of depressed skull fracture is advisable in infants, since this is a period of r a p i d growth of the b r a i n ; localized pressure from the depressed bone m a y occur a n d so affect cerebral function as to give rise to epileptogenic loci. * This type of lesion, which is sometimes called a "ping p o n g ball" fracture, 2 is similar to the green stick fracture of long bone in which no break in surface continuity occurs, b u t r a t h e r there is an i n w a r d b e n d i n g of the surface. Elevation of such a fracture necessitates a surgical procedure with general anesthesia. Recently a nonsurgical m e t h o d of elevation, new to one of us, was demonstrated. Since it is a sim-
Volume 72 Number 2
ple bedside procedure, we felt it worth reporting. CASE
REPORT
L. W., a 9-week-old male infant, was brought to Harlem Hospital because of a large depression in the left parieto-occipital region. The parents stated that the child had fallen 3 days prior to admission. He had cried vigorously and thrashed about at the time, and seemed perfectly normal. Following this episode, he returned to his normal pattern of behavior and continued to eat well. While feeling the baby's head several days later, the mother noted a depression and brought him to the hospital. Past history was not significant. The baby was full term, and delivery was without complications; no forceps were used. Upon physical examination, the baby was active and alert. He moved all extremities well and withdrew them from painful stimuli. The cranial nerves seemed normal. Reflexes were symmetrical and normal. Upon palpation of the head, a depression was felt in the left parietooccipital region. There appeared to be a small amount of scalp edema present in the arca. Kadiographs of the skull showed a concave
Brief clinical and laboratory observations
263
radiolucent line in the left parieto-occipital region, approximately 5 cm. in diameter and depressed l t/2 cm. below the outer table of the skull (Fig. 1). One of us (M. P.), while a medical student in Iran, had observed while a midwife elevated a depressed fracture in a newborn. A thumb was placed on opposite margins of the depression, and gentle but firm pressure exerted tangentially towards the middle of the depression for several minutes. The area of depression gradually disappeared while pressure was exerted. If this fails to effect elevation, a second operator can exert pressure on the remaining two margins of the lesion. This maneuver was performed on the patient and after several minutes, no depression could be palpated. A roentgenogram was taken and the linear density marking the area of depression had all but disappeared (Fig. 2). The baby tolerated the procedure well. He was observed for 2 days and discharged home. SUMMARY
A m e t h o d of elevating depressed skull fractures in infants is described. A l t h o u g h
Fig. 2
Fig. 1
Fig. 1. Left parietal depressed fracture in a 9-week-old infant. Arrow points to radiolucency caused by depression. Fig. 2. The fracture has been elevated by digital pressure. A faint line of radiolucency is still present (arrow).
2 64
Brie[ clinical and laboratory observations
not used in the United States, this may be standard practice in other areas. Since the procedure can be done at the bedside, it is worth attempting in "ping pong" type fractures in order to avoid, if possible, an operative procedure.
Nepbrotic syndrome and nepbroblastoma Report of a case D. R. Lines, M.B.B.S., M.R.A.C.P. NORTH
ADELAIDE~
SOUTH
AUSTRALIA
N E P H ROBLAS T OMA in association with the nephrotic syndrome has been reported rarely, 1, 2 but a causal relationship has not been shown. The purpose of this paper is to record the clinical and pathological features of a further case of this association, in which the renal pathology was of the minimal change type.
CLINICAL RECORD The patient (A. C. H., case No. BC6835), male, was aged 2 years, 5 months, at the time of his first admission to this hospital for investigation of generalized edema of recent onset. The diagnosis of nephrotic syndrome was confirmed by the finding of heavy proteinuria (2 Gin. per 24 hours) and hypoalbuminemia (0.4 Gin. per 100 ml.). After 6 days of therapy with prednisolone (40 mg. daily) a profound diuresis began and lasted !
From the University o[ Adelaide, Department o[ Child Health, Adelaide Children's Hospital Inc.
The Journal o[ Pediatrics February 1968
REFERENCES i. Ingraham, F. D., and Matson, D. D.: Neurosurgery of infancy and childhood, Springfield, Ill., 1954, Charles C Thomas, Publisher, 456 pages. 2. Jackson, I. A., and Thompson, R. K.: Pediatric neurosurgery, Springfied, Ill., 1959, Charles C Thomas, Publisher, 564 pages.
for 4 days, during which the patient's weight decreased by 3 Kg. The dosage of prednisolone was gradually reduced to 5 rag. per day and he was discharged clinically well. During the next 6 months he suffered 4 episodes of edema and proteinuria, each responding to an increased dose of prednisolone (40 mg. daily). Nine months after the original illness a percutaneous left renal biopsy was performed. In the preparatory intravenous pyelogram a space-occupying lesion was detected in the hilum of the right kidney. At a subsequent taparotomy this appeared to be a nephrobtastoma, and a nephrectomy was performed. PostoperatiVely, cyclophosphamide was given intravenously, and the right renal area was irradiated. Heavy albuminuria, edema, and ascites recurred. This was once again controlled with the administration of prednisolone (40 rag. per day). Because he was hypertensive (blood pressure, 150/120 ram. Hg) and obviously Cushingoid, the dose of prednisolone was gradually reduced and eventually replaced by azothioprine 25 mg. daily. However, the rapid recurrence of edema and the accumulation of a right pleural effusion required the reinstitution of prednisolone therapy. He' was discharged finally on a reducing dose'of prednisolone and azothioprine. During the 14 months since discharge he has suffered one relapse, which was controlled by an increase of prednisolone to 40 rag. daily for 2 weeks. At all other times his 24 hour urinary excretion of protein has