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Encephaloclastic porencephaly-postnatal evolution
S98
Brief clinical and laboratory observations
Encephaloclastic porencephaly-postnatal evolution Nirmala Kesaree, M.D., Ronald L. Poland, M.D., a n d Zwi H. H a r t , M . D . , * Detroit, Mich.
E N C E P H A L O C L A S T I C P O R E N C E P H A L Y is a f r e q u e n t a u t o p s y finding in cases o f c e r e b r a l palsy. It h a s b e e n a t t r i b u t e d to c e r e b r a l h y p o x i a in utero. 1 This case r e p o r t describes the p o s t n a t a l e v o l u t i o n o f such a cyst.
CASE REPORT Patient C. J. (CHM No. 570823) was the product of a 38-week gestation of a 27-year-old white woman. The pregnancy was complicated by a gain in weight of 47 pounds, mild hypertension, edema and some visual scotomata. The method of delivery was elective cesarean section with the patient under spinal anesthesia. The placenta weighed 1,060 gm, but appeared to be normal. Follow-up of maternal health revealed glucose of 187 mg/dl. Apgar score was 8 at one minute. The birth weight was 3,670 gm (seventy-fifth to ninetieth percentile). Physical findings at birth included: head circumference, 37.5 cm (ninty-seventh percentile) and crown-rump length, 33.5 cm (fiftieth percentile). Fontanelles were not large or bulging.
From the Department of Pediatrics, Children "s Hospital of Michigan. Supported by a grant from the Holden Foundation. *Reprint address: Department of Pediatrics, Children's Hospital of Michigan, 3901 Beaubren Blvd., Detroit, Mich. 48201.
The Journal of Pediatrics April 1976
At 17 hours of age, Patient C. J. was described as flaccid, sucking poorly, and having grunting respirations. At 29 hours o f age, she had a left-sided clonic seizure. The head circumference was 38.5 cm. She was transferred to the Children's Hospital o f Michigan. Physical examination at 36 hours o f age showed an edematous infant. The anterior fontanelle was full. There was audible grunting and marked subcostal retractions. Heart rate was 180 beats per minute. There was no cardiac murmur or third heart sound. The liver was enlarged 4 cm below the costal margin, and the spleen tip was felt. She had a high-pitched cry and increased muscle tone. Transillumination of the skull by two observers was considered to be normal. Lumbar puncture on admission revealed clear fluid, 1 WBC, protein 64 mg/dl, and glucose 106 mg/dl. The following laboratory determinations were normal: blood glucose, electrolytes, BUN, SCOOT, calcium, serum protein including IgM, and routine rarinalysis. Hemoglobin was 11.8 gm/dl. The first four days of life were characterized by respiratory distress, repeated seizures, and deepening jaundice (bilirubin 19 mg/dl). The baby had AO isoimmunization; the Coombs test was positive. An exchange transfusion was performed. Soon afterward the baby lost 12% of her body weight. The hemoglobin rose to 14.8 gm/dl and cardiothoracid ratio fell from 0.54 to 0.50. The edema disappeared and the liver returned to normal size. Skull roentgenograms were normal. The long bones showed slight submetaphyseal rarefaction. A-mode echoencephalogram showed no shift of midline structures and the right and left ventricles measured 14 mm and 12 mm, respectively. At 6 weeks of age, the patient was readmitted because of uncontrolled seizures. Her head circumference was 39 cm. She had a decerebrate posture. Repeat transillumination of the head showed marked penetrance over the right side. Resutts o f a right subdural tap were negative. During an attempted right ventriculogram, a large cyst was encountered which did not communicate
Fig. 1. Left, Attempted right ventriculogram, large porencephalic cyst encountered. Right, Left ventriculogram, moderately dilated ventricles.
Volume 88 Number 4, part 1
with either the subarachnoid space or ventricles (Fig. 1). The fluid was cloudy; numerous lipomacrophages were demonstrated by microscopic examination with the cytocentrifuge technique. The lipomacrophages stained strongly with Sudan IV. A successful ventriculogram was performed through the left ventricle. The fluid was clear with 7 RBC and 0 WBC. Bilateral selective internal carotid arteriography revealed all major vessels patent and free from intrinsic lesions. At 6 months of age Patient C. J. was profoundly retarded, opisthotonic with marked positive right-sided transillumination, and a head circumference of 44 cm. Repeat ventriculogram showed further enlargement of the ventricles and clear acellular fluid in the persistent right porencephalic cyst.
Brief clinical and laboratory observations
599
Cerebral edema and focal areas of necrosis with predilection for the paracentral areas were experimentally induced in term monkeys subjected to partial hypoxia in utero. 4 Significant hemorrhage was not responsible for the devitalized tissue in our case since the lipomacrophages found in the cyst were free of hemosiderin? The authors thank Mrs. Margaret Shepard for the preparation of this report and to Drs. Calvin C. Kay and Richard F. Trevis for the referral of the patient.
REFERENCES DISCUSSION This infant's mother had chemical diabetes. The placenta was abnormally heavy. Fetal and placental hydrops, presumably associated with fetal heart failure, have been described in infants of diabetic mothers. 2 This phenomenon has been attributed to fetal asphyxia and secondary heart failure, owing to failure of placental function3 An attractive speculation is that our patient also suffered cerebral edema and infarction. The infarct, which did not transilluminate, progressed to porencephaly as the necrotic tissue was cleared by macrophages.
A 9/11 translocation in a child with Ph 1negative chronic myelogenous leukemia Dorothy Warburton, Ph.D.,* and Narayan Shah, M.D., N e w York, N. Y.
CHRONIC MYELOGENOUS LEUKEMIA in children can occur either as the adult form or the juvenile form. The distinction between the adult and juvenile forms includes From the Departments of Pediatrics and Human Genetics and Development. Supported by a grant from the National Foundation-March of Dimes, and by a grant from National Institutes of Health R-IO CA 03526-18 and National Institutes of Health CA 13696-03. *Reprint address: Columbia University, College of Physicians and Surgeons, 630 West 168th St., New York, N. Y. 10032.
1. Towbin A: Neonatal injury and malformation, in Pathology of the nervous system. Minkler, 1971, McGraw-Hill Book Company, Vol. 2: 1837. 2. Driscoll SG: Hydrops fetalis, N Engl J Med 275:1432, 1966. 3. Benirschke K, and Driscoll SG: The pathology of the human placenta, New York, 1967, Springer-Verlag, pp 457459. 4. Myers RE: Two patterns of perinatal brain damage and their conditions of occurrance, Am J Obstet Gynecol 112:246, 1972. 5. Dyken PR: Cerebrospinal fluid cytology: Practical clinical usefulness, Neurology 25:210, 1975.
the almost constant findings of a Philadelphia chromosome in the malignant cells of patients with the adult form, and a normal karyotype or nonspecific aneuploidy in the cells of patients with the juvenile form. '-:~ In both children and adults the Ph'-negative cases are more rapidly progressive and respond poorly to busulfan. Some authors believe that these may really be monocytic leukemias with a granulocytic component.:" ~
See related article, p. 596.
Abbreviation used Phi: Philadelphia We report here a case of Ph'-negative chronic myelogenous leukemia, apparently of the adult type, in a ten-yearold girl who is doing well 29 months after diagnosis. Chromosome analysis of bone marrow and spontaneously dividing cells of the peripheral blood showed a translocation between chromosome 9 and 11, but no Ph' chromosome. However, the break point in chromosome 9 was similar to that usually found in association with Ph ~positive chronic myelogenous leukemia.
Brief clinical and laboratory observations
Encephaloclastic porencephaly-postnatal evolution Nirmala Kesaree, M.D., Ronald L. Poland, M.D., a n d Zwi H. H a r t , M . D . , * Detroit, Mich.
E N C E P H A L O C L A S T I C P O R E N C E P H A L Y is a f r e q u e n t a u t o p s y finding in cases o f c e r e b r a l palsy. It h a s b e e n a t t r i b u t e d to c e r e b r a l h y p o x i a in utero. 1 This case r e p o r t describes the p o s t n a t a l e v o l u t i o n o f such a cyst.
CASE REPORT Patient C. J. (CHM No. 570823) was the product of a 38-week gestation of a 27-year-old white woman. The pregnancy was complicated by a gain in weight of 47 pounds, mild hypertension, edema and some visual scotomata. The method of delivery was elective cesarean section with the patient under spinal anesthesia. The placenta weighed 1,060 gm, but appeared to be normal. Follow-up of maternal health revealed glucose of 187 mg/dl. Apgar score was 8 at one minute. The birth weight was 3,670 gm (seventy-fifth to ninetieth percentile). Physical findings at birth included: head circumference, 37.5 cm (ninty-seventh percentile) and crown-rump length, 33.5 cm (fiftieth percentile). Fontanelles were not large or bulging.
From the Department of Pediatrics, Children "s Hospital of Michigan. Supported by a grant from the Holden Foundation. *Reprint address: Department of Pediatrics, Children's Hospital of Michigan, 3901 Beaubren Blvd., Detroit, Mich. 48201.
The Journal of Pediatrics April 1976
At 17 hours of age, Patient C. J. was described as flaccid, sucking poorly, and having grunting respirations. At 29 hours o f age, she had a left-sided clonic seizure. The head circumference was 38.5 cm. She was transferred to the Children's Hospital o f Michigan. Physical examination at 36 hours o f age showed an edematous infant. The anterior fontanelle was full. There was audible grunting and marked subcostal retractions. Heart rate was 180 beats per minute. There was no cardiac murmur or third heart sound. The liver was enlarged 4 cm below the costal margin, and the spleen tip was felt. She had a high-pitched cry and increased muscle tone. Transillumination of the skull by two observers was considered to be normal. Lumbar puncture on admission revealed clear fluid, 1 WBC, protein 64 mg/dl, and glucose 106 mg/dl. The following laboratory determinations were normal: blood glucose, electrolytes, BUN, SCOOT, calcium, serum protein including IgM, and routine rarinalysis. Hemoglobin was 11.8 gm/dl. The first four days of life were characterized by respiratory distress, repeated seizures, and deepening jaundice (bilirubin 19 mg/dl). The baby had AO isoimmunization; the Coombs test was positive. An exchange transfusion was performed. Soon afterward the baby lost 12% of her body weight. The hemoglobin rose to 14.8 gm/dl and cardiothoracid ratio fell from 0.54 to 0.50. The edema disappeared and the liver returned to normal size. Skull roentgenograms were normal. The long bones showed slight submetaphyseal rarefaction. A-mode echoencephalogram showed no shift of midline structures and the right and left ventricles measured 14 mm and 12 mm, respectively. At 6 weeks of age, the patient was readmitted because of uncontrolled seizures. Her head circumference was 39 cm. She had a decerebrate posture. Repeat transillumination of the head showed marked penetrance over the right side. Resutts o f a right subdural tap were negative. During an attempted right ventriculogram, a large cyst was encountered which did not communicate
Fig. 1. Left, Attempted right ventriculogram, large porencephalic cyst encountered. Right, Left ventriculogram, moderately dilated ventricles.
Volume 88 Number 4, part 1
with either the subarachnoid space or ventricles (Fig. 1). The fluid was cloudy; numerous lipomacrophages were demonstrated by microscopic examination with the cytocentrifuge technique. The lipomacrophages stained strongly with Sudan IV. A successful ventriculogram was performed through the left ventricle. The fluid was clear with 7 RBC and 0 WBC. Bilateral selective internal carotid arteriography revealed all major vessels patent and free from intrinsic lesions. At 6 months of age Patient C. J. was profoundly retarded, opisthotonic with marked positive right-sided transillumination, and a head circumference of 44 cm. Repeat ventriculogram showed further enlargement of the ventricles and clear acellular fluid in the persistent right porencephalic cyst.
Brief clinical and laboratory observations
599
Cerebral edema and focal areas of necrosis with predilection for the paracentral areas were experimentally induced in term monkeys subjected to partial hypoxia in utero. 4 Significant hemorrhage was not responsible for the devitalized tissue in our case since the lipomacrophages found in the cyst were free of hemosiderin? The authors thank Mrs. Margaret Shepard for the preparation of this report and to Drs. Calvin C. Kay and Richard F. Trevis for the referral of the patient.
REFERENCES DISCUSSION This infant's mother had chemical diabetes. The placenta was abnormally heavy. Fetal and placental hydrops, presumably associated with fetal heart failure, have been described in infants of diabetic mothers. 2 This phenomenon has been attributed to fetal asphyxia and secondary heart failure, owing to failure of placental function3 An attractive speculation is that our patient also suffered cerebral edema and infarction. The infarct, which did not transilluminate, progressed to porencephaly as the necrotic tissue was cleared by macrophages.
A 9/11 translocation in a child with Ph 1negative chronic myelogenous leukemia Dorothy Warburton, Ph.D.,* and Narayan Shah, M.D., N e w York, N. Y.
CHRONIC MYELOGENOUS LEUKEMIA in children can occur either as the adult form or the juvenile form. The distinction between the adult and juvenile forms includes From the Departments of Pediatrics and Human Genetics and Development. Supported by a grant from the National Foundation-March of Dimes, and by a grant from National Institutes of Health R-IO CA 03526-18 and National Institutes of Health CA 13696-03. *Reprint address: Columbia University, College of Physicians and Surgeons, 630 West 168th St., New York, N. Y. 10032.
1. Towbin A: Neonatal injury and malformation, in Pathology of the nervous system. Minkler, 1971, McGraw-Hill Book Company, Vol. 2: 1837. 2. Driscoll SG: Hydrops fetalis, N Engl J Med 275:1432, 1966. 3. Benirschke K, and Driscoll SG: The pathology of the human placenta, New York, 1967, Springer-Verlag, pp 457459. 4. Myers RE: Two patterns of perinatal brain damage and their conditions of occurrance, Am J Obstet Gynecol 112:246, 1972. 5. Dyken PR: Cerebrospinal fluid cytology: Practical clinical usefulness, Neurology 25:210, 1975.
the almost constant findings of a Philadelphia chromosome in the malignant cells of patients with the adult form, and a normal karyotype or nonspecific aneuploidy in the cells of patients with the juvenile form. '-:~ In both children and adults the Ph'-negative cases are more rapidly progressive and respond poorly to busulfan. Some authors believe that these may really be monocytic leukemias with a granulocytic component.:" ~
See related article, p. 596.
Abbreviation used Phi: Philadelphia We report here a case of Ph'-negative chronic myelogenous leukemia, apparently of the adult type, in a ten-yearold girl who is doing well 29 months after diagnosis. Chromosome analysis of bone marrow and spontaneously dividing cells of the peripheral blood showed a translocation between chromosome 9 and 11, but no Ph' chromosome. However, the break point in chromosome 9 was similar to that usually found in association with Ph ~positive chronic myelogenous leukemia.