Hydranencephaly Following Elevated Hematocrit Values in a Newly Born Infant

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770 PEDIATRICS Vol. 54 No. 6 December 1974

Hydranencephaly

Followi ng Elevated

Hematocrit

Values

in a Newly

Born

Infant

Herbert Koffler, M.D., William J. Keenan, M.D., and James M. Sutherland, M.D.

Fromthe DepartmentofPediatrics, UniversityofCincinnatiCollegeofMedicine; the CrosleyMernorialNursery

of the Cincinnati General Hospital; and the Children ‘s hospital Research Foundation, Cincinnati, Ohio.

ABSTRACT. A newly born infant with a hematocrit value of 78% was subsequently found to have hydranencephaly. Polycythemia produces hyperviscosity which, in turn, re-suIts in decreased tissue perfusion. It is possible that

corn-promised cerebral circulation produced brain ischemia

with consequent massive destruction of cerebral tissue. Pe-diatrics, 54:770, 1974, POLYCYTHEMIA, HYPERVISCOSITY,

HYI)RANENCEPIIALY, BRAIN TISSUE I)ESTRUCTION, BRAIN

ISCIIEM IA.

Polycythemia is now recognized as a problem in the newly born infant. The relationship be-tween hematocrit value and blood viscosity has been documented in this age group.” Signs and symptoms referable to the cardiorespiratory sys-tem and central nervous system have been the pri-mary abnormalities associated with the polycy-themia-hyperviscosity syndrome in the newborn period.

Infants born of a multiple pregnancy4 and in-fants who are small for gestational age’ alert the clinician to anticipate polycythemia. In addition, polycythemia may occur during the first day of life with maternal-fetal transfusions6 and with placental transfusion such as that associated with delayed clamping of the cord at delivery.7

This is a report of a patient with elevated he-matocrit values in the newborn period and proba-ble disturbed cerebral circulation who subse-quently developed hydranencephaly.

CASE REPORT

R.A. (*185560), a full-term white girl, was referred to Cincinnati Children’s Hospital on November 27, 1967 at 6 hours of age with a history of cyanosis first noticed upon

her arrival in the nursery. Apgar score had been 9 at one minute and 9 at five minutes. Examination at 7 hours of age revealed a 2,890-gm (25th percentile) infant with a length of 49 cm (50th percentile) and an occipitofrontal circurnfer-ence of 33 cm (25th to 50th percentile). Rectal temperature

was 33 C, respiratory rate was 80 per minute, and pulse rate

was 108 per minute. Her skin was pale; her hands, feet, lips, and perioral region were cyanotic. There were petechiae on the back, face, groin, and axilla. There was molding of the head but no over-riding of the sutures. On auscultation, the lungs were clear; there were no cardiac murmurs. Pe-ripheral pulses were present but weak. There were no ab-normalities on abdominal examination. The infant was lethargic and hypotonic and responded to stimulation with a high-pitched cry. Grasp and suck were poor; Moro

re-sponse was fair.

Laboratory examination revealed: capillary hematocrit value, 78%; white blood cell count, 12,600/cu mm, with 72% neutrophils, 24% lymphocytes, and 4% monocytes. The platelet count was 588,000/cu mm. Blood glucose was 72 mg/100 ml. Cerebrospinal fluid glucose and protein deter-minations were not obtained. Blood, urine, and cerebrospi-nal fluid cultures were done and were negative after incu-bation for three days. Electrocardiographic tracing was normal. Chest roentgenogram showed multiple densities scattered throughout both lung fields and fluid within the major and minor fissures. This was interpreted as

repre-senting the picture of pulmonary edema.

The patient was treated with antibiotics and oxygen and placed in an incubator to maintain warmth. Oral feedings were begun at 14 hours of age. The next day her respiratory rate was 40 to 50 per minute, oxygen requirements were

de-(Received May 21; revision accepted for publication July 3,

1974.)

Supported in part by U.S. Public Health Service training grant, Project MCT 174.

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Research Foundation, Elland and Bethesda Avenues,

Cm-cinnati, Ohio 45229.

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ARTICLES 771 creased, and a chest roentgenogram was almost completely

clear. Her capillary hematocrit value was 70%. Urine out-put was scanty. During the next three days she began eat-ing well, became vigorous, and no longer required addition-al oxygen. Her capillary hematocrit value was 62% on the fifth postnatal day. She continued to do well and was dis-charged at 9 days of age with a capillary hematocrit value

of 57%.

On readmission, 54 days later, the mother related that the infant had been a “poor eater” and had developed drill-culty opening her left eye since she had been home. There was a four-day history of lethargy and hypotonia. She weighed only 3,800 gm, less than the third percentile for 2 months of age, and had grown only 1 cm in length. Her head circumference was 39 cm, 75th percentile for her age. Her anterior fontanel was bulging. There was a left facial

weakness and left hemiparesis. The grasp and suck were

poor, and Moro response absent. The head transilluminated

throughout. Electroencephalographic tracing revealed

lit-tie evidence of recordable potential, and ventriculographic study confirmed the diagnosis of hydranencephaly. The

pa-tient was institutionalized and died four months later. An

autopsy was not performed.

DISCUSSION

The relationship between hematocrit value and viscosity has been examined in the newborn pe-riod. Of the many factors which influence blood viscosity,8 red blood cell number is considered to be the most important in the newly born infant.”2 With a venous hematocrit value of 65% or great-er, small changes in hematocrit produce large in-creases in viscosity. The signs

and

symptoms of the polycythemia-hyperviscosity syndrome ap-pear to be directly related to the hyperviscous

Central nervous system signs

and

symptoms de-scribed during the nursery couise of newly born infants have included excessive irritability, stiff-ness, jitteriness, and seizures, or, conversely, leth-argy, poor suck, and marked hypotonia at times associated with cyanosis

and

apnea. Gross et all

evaluated 18 symptomatic polycythemic-hyper-viscous infants whose venous hematocrit values ranged from 63% to 77%. Fourteen of these 18 pa-tients had neurological signs and symptoms. Three of the six infants with abnormal electroen-cephabograms had seizures. Follow-up examina-tion at 23 months of age revealed four children with signfficant motor and/or mental retardation. Two of the four had been treated with a partial exchange transfusion.

Despite reports of neurological symptoms and findings associated

with

the polycythemia-hyper-viscosity syndrome, there is no report of morpho-logical cerebral changes. The patient presented in this case report had hydranencephaly following symptoms associated with elevated hematocrit values. Hydranencephaly results from destruction of cerebral hemispheres within intact meninges

and skull; the resulting cavity is a fluid-filled membranous sac. Diverse circumstances lead to hydranencephaly; the link with vascular compro-mise in at beast a portion of the patients has been strengthened by the data of a number of workers. For instance, after bilateral ligation of the com-mon carotid artery

and

jugular vein in fetal mon-keys, Myers9 has demonstrated liquefaction ne-crosis of large areas of cerebral tissue with

dis-appearance and removal of necrotic tissue and conversion into a fluid-filled cavity in conti-nuity with the ventricular system. Becker1#{176} showed that paraffin injected into the carotid ar-tery of newborn puppies first produces multiple cysts, and then liquification of the cerebral hem-isphere in the latter stages of necrosis. Spatz” has shown that the brains of newborn animals liquify and disappear when the blood supply has been in-terrupted. Lindenberg

and

Swanson’2 describe five children with cerebral infarction associated with compromised carotid artery blood flow. The distribution and extent of damage resembled hy-dranencephaly except that the walls of the ventri-des and some of the white matter were preserved.

The increased viscosity in polycythemia has been thought to result in cerebral dysfunction be-cause of a viscosity-dependent decrease in cere-bral blood flow. Nelson and Fazekas13 studied an adult with polycythemia vera before and after several cerebral thrombotic episodes. These studies indicated that following cerebral ischemia and phlebotomy, cerebral tissue was freely per-fused but that metabolic work was not being done normally by injured and, therefore, malfunction-ing tissue. At autopsy the patient’s brain showed encephabomalacia. Muir,14 Lyon and Robain,1’ and Aicardi and co-workers16 suggested on the basis of their patients that a general reduction in cerebral blood flow could give rise to a spectrum of pathology from porencephaly, through poby-porencephaly (encephabomalacia), to hydranen-cephaly.

It is hypothesized that the patient reported here had a hematocrit value high enough to pro-duce hyperviscosity and a subsequent compro-mise in cerebral circulation that led to wide-spread infarction and liquifaction necrosis. Dur-ing her initial hospitalization it was not recog-nized that her signs and symptoms were related to her elevated hematocrit. Without a clear under-standing of the infant’s problem, she was initially treated with antibiotics and oxygen. By 5 days of age she was vigorous, eating well, no longer on an-tibiotic therapy, and did not require supplemen-tal oxygen. The patient was felt to be normal on discharge at 9 days of age. In hydranencephaly

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772 HYDRANENCEPHALY

there may be well-preserved subcortical tissue and function. Signs of retardation only are appar-ent when cerebral cortical function becomes more obvious at 7 to 8 weeks of age.’7

Our patient had minimal symptoms in the first day after birth. Asymptomatic infants with hema-tocrit values in the range of those infants with the polycythemia-hyperviscosity syndrome have also been described. Gatte et al’8 comment that 25 of 629 (3.9%) newly born infants had capillary hema-tocrit values higher than 75% and had neither cya-nosis nor cardiorespiratory distress. During a one-month period, 34% of 255 capillary hematocrit values done on the first day of life in a well-baby nursery at the Cincinnati General Hospital were 65% or greater, 1 1.7% were 70% or greater, and 3.5% were 75% or greater. Despite the fact that hematocrit values determined from capillary blood are invariably higher than corresponding values obtained on blood samples from large yes-sels (venapuncture),’ a notable proportion of in-fants have hematocrit values in an excessively high range. Thus, if polycythemia represents a hazard to normal cerebral development, a signifi-cant number of newly born infants can be consid-ered to be at risk. Furthermore, hematocrit as well as viscosity can be brought to a normal range by a modified exchange transfusion”9 though the utility of this procedure in improving quality of survivorship is not documented.

It is estimated that 3% of the population born annually has cerebral dysfunction.2#{176} The infants with the polycythemia-hyperviscosity syndrome may contribute to that population. Infants with polycythemia should be considered to be a high risk population and should be followed closely.

SUMMARY

A newly born infant with a hematocrit value of 78% was subsequently found to have hydranen-cephaly. Polycythemia produces hyperviscosity which, in turn, results in decreased tissue perfu-sion. It is possible that compromised cerebral cir-culation produced brain ischemia with conse-quent massive destruction of cerebral tissue.

REFERENCES

1. (;ross, G. P., Hathaway, W. E., and McCaughey, H. R.;

Ilyperviscosity in the neonate. J. Pediatr., 82: 1(X)4, 1973.

2. Mackintosh, T. F., and Walker, C. H. M.: Blood viscosi-ty in the newborn. Arch. Dis. Child., 48:547, 1973. 3. Kolltras, S. B.: Polycythemia and hyperviscosity

syn-drornes iii infants and children. Pediatr. Clin. North Am., 19:919, 1972.

4. Rausen, A. R, Seki, M., and Strauss, L.: Twin

transfu-siOfl syndrome: A review of 19 cases studied at one

institution, J. Pediatr., 66:613, 1965.

5. Humbert, J.R., Abelson, H., Hathaway, W. E., and Bat-taglia, F. C.: Polycythemia in small for gestational age infants. J. Pediatr., 75:812, 1969.

6. Michael, A. F., Jr., and Mauer, A. M.: Maternal-fetal transfusion as a cause of plethora in the neonatal period. Pediatrics, 28:458, 1961.

7. Oh, W., and Lind, J.: Venous and capillary hematocnt in newborn infants and placental transfusion. Acta

Paediatr. Scand., 55:38, 1966.

8. Dintenfass, L.: Blood viscosity, internal fluidity of the red cell, dynamic coagulation and the critical cap-illary radius as factors in the physiology and pa-thology of circulation and microcirculation. Med.

J. Aust., 1:688, 1968.

9. Myers, R. E.: Brain pathology following fetal vascular

occlusion: An experimental study. Invest.

Ophthalmol., 8:41, 1969.

10. Becker, H., cited by Halsey, J. H., Jr., Allen, N., and Chamberlin, H. R.: The morphogenesis of hydran-encephaly. J. Neurol. Sci., 12:187, 1971.

11. Spatz, cited by Watson, E. H.: Hydranencephaly: Re-port of two cases which combine features of hy-drocephalus and anencephaly. Am. J. Dis. Child., 67:282, 1944.

12. Lmndenberg, R., and Swanson, P. D.: Infantile hydran-encephaly: A report of 5 cases of infarction of both cerebral hemispheres in infancy. Brain, 90:839, 1967.

13. Nelson, D., and Fazekas, J. F.: Cerebral blood flow in polycythemia vera. Arch. Intern. Med., 98:328, 1956.

14. Muir, C. S.: Hydranencephaly and allied disorders. Arch. Dis. Child., 34:231, 1959.

15. Lyon, G., and Robain, 0.: Etude comparative

disence-phalopathies circulatories prenatales et

parana-tales (hydranencephalus, porencephalies et ence-phalomalacies kystiques de Ia substance blanche). Acta Neuropathol., 9:79, 1967.

16. Aicardi, J., Goutieres, F., and Hodebourg, D. A.: Multi-cystic encephalomalacia of infants and its relation to abnormal gestation and hydranencephaly. J.

Neurol., Sci. 15:357, 1972.

17. Crome, L.: Hydraencephaly. Dev. Med. Child Neurol., 14:224, 1972.

18. Gatte, R. A., Muster, A. J., Cole, R. B., and Paul, M. H.: Neonatal polycythemia with transient cyanosis and cardiorespiratory abnormalities. J. Pediatr., 69:1063, 1966.

19. Benfield, D. G., Lubbe, R. J., and Sutherland, J. M.:

Therapeutic consequences of partial exchange transfusion in symptomatic polycythemic neo-nates, abstracted. Pediatr. Res., 6:406, 1972.

20. Niswander, K. R., and Gordon, M.: Introduction. In,

The Women and Their Pregnancies. The

Collabora-tive Perinatal Study of the National Institute of

Neurological Diseases and Stroke. Philadelphia:

W. B. Saunders Company, 1972, p. 2

ACKNOWLEDGMENT

We are indebted to Drs. Jean Steichen and Irwin Light for their assistance in translating the literature and

re-viewing the manuscript and to Miss Finita Peck and Mrs. Jean Sennett for their assistance in preparing the manu-script.

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1974;54;770

Pediatrics

Herbert Koffler, William J. Keenan and James M. Sutherland