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Suspected intraaqueductal perinatal hemorrhage in congenital hydrocephalus
Suspected Intraaqueductal Perinatal Hemorrhage in Congenital Hydrocephalus Takao Enomoto, MD, Yutaka Maki, MD, Kunio Nakagawa, MD, Yoshitaka N akada, MD, and Kenzo Hamano, MD
A 8-day-old boy with congenital hydrocephalus was given a shunt operation. Computed tomography (CT) revealed marked internal hydrocephalus and a high density area about the aqueduct of Sylvius, in the fourth ventricle and perhaps partly in the third ventricle which was completely absorbed one month later. Later conray ventriculography demonstrated aqueductal obstruction. Neuroradiologically there was neither neoplastic nor vascular lesion. The patient achieved almost normal motor and mental development at 9 months of age. The cephalopelvic discrepancy and vacuum extraction are thought to be the most likely causes of the hemorrhage. The site of hemorrhage was quite unusual as a neonatal intraventricular hemorrhage. Enomoto T, Maki Y, Nakagawa K, Nakada Y, Hamano K. Suspected intraaqueductal perinatal hemorrhage in congenital hydrocephalus. Brain Dev 1981;3: 93-6
Intraventricular hemorrhage is often associated with premature or low-birth-weight infants. The site of hemorrhage is predominantly the sub ependymal matrix layer of the lateral ventricle or choroid plexus in some cases [1]. The causative factors that contribute to the hemorrhage are still the subject of controversy [2-7]. Obstructive hydrocephalus often develops as a sequela of intraventricular hemorrhage, which is not always responsive even today to a shunt operation [8-11]. The authors report a case with congenital hydrocephalus diagnosed From the Department of Neurological Surgery, The University of Tsukuba School of Medicine, Ibaraki. Received for pUblication: June 26, 1980. Accepted for publication: September 20, 1980.
Key words: Hydrocephalus, intraventricular hemorrhage, aqueductal stenosis, CT-scan. Correspondence address: Dr. Yutaka Maki, Department of Neurological Surgery, The University of Tsukuba School of Medicine, Niihari-gun, Ibaraki 305, Japan.
by CT-scan which disclosed a high density area about the aqueduct of Sylvius, in the fourth ventricle and partly in the third ventricle and treated successfully with a shunt operation. Case Report A 8-day-old boy was admitted to our hospital on February 8, 1978, because of a large head. The pregnancy was uneventful until January 10, 1978, when the mother took ABPC for three days for a common cold. On January 31, 1978, after premature rupture of the amniotic membrane the patient was born with the aid of oxytocin and vacuum extraction at the 33rd week of pregnancy. The Apgar score was 5 at birth. The patient weighed 3,050 g. The chest circumference was 29 cm and that of the head was 40 cm, the latter thereafter increased by 0.5 cm a day. Grand mal seizure appeared on February 1, 1978. Physical examination on admission revealed a somewhat icteric, inactivated and dry infant
with a large scaphocephalic head. All of the sutures except the squamous were widely separated. The anterior fontanelle was tightly distended. The head circumference was 43.7 cm. No abnormalities were noted of the heart, lungs, liver and spleen. There was no signs
indicative of a dysraphic state. Neurological examination revealed an inactivated infant with rigidity of the flexor muscle group especially of upper extremities. Though sucking reflex was poor, other primitive reflexes including Moro, Galant, ATNR and plantar grasp were noted.
Fig I-A CT·scan taken before first operation demonstrating markedly dilated lateral and third ventricles with a high density area about the aqueduct of Sylvius, in the fourth ventricle and partly in the third ventricle (arrow). Dilatation of the lateral ventricle is more marked on the left shifting the falx to the right. Fig I-B CT·scan taken after operation at 8 months of age demonstrating the thickened cerebral mantle with a still enlarged left occipital horn. No abnormally enhanced area can be seen.
94 Brain & Development, Vol 3, tvo 1, 1981
There was the sun setting phenomenon and the Babinski sign was positive. CT scanning carried out on admission revealed marked internal hydrocephalus with a high density area about the aqueduct ofSylvius, in the fourth ventricle and partly in the third ventricle but none in the lateral ventricles (Fig I-A). Laboratory examinations including spinal CSF were not remarkable. But the titer of toxoplasma and cytomegalic inclusion virus were not examined. A ventriculoatrial shunt performed on February 10, 1978, improved movement of the extremities and decreased tension of the fontanelle. But because of subscalp fluid collection, suggesting malfunction of the shunt device, this V-A shunt was replaced by a ventriculoperitoneal shunt on February 16, 1978. On March 10, 1978, about one month after the first record, a CT-scan was taken for the second time, which demonstrated improvement of the hydrocephalus and complete abolishment of the high density area. Though the sunset phenomenon was still noted feeding difficulty was much improved. Retrograde brachial angiography on the right side was carried out on March 26, 1978, to exclude neoplastic or vascular lesion. But the sequelae were all consistent with hydrocephalus. 99ffiTc brain scan performed on April 1, 1978, was also not contributory except for the hydrocephalic change. On the other hand the head again began to grow steadily (Fig 2). The fontanelle was tightly distended. Head lag was noted still. As these clinical signs suggested malfunctioning of the shunting device again, we carried out RI cisternography which showed delayed cortical clearing especially over the left hemisphere but no ventricular reflux. But CT one month after the revision demonstrated the thickened cortical mantle and so re-revision was postponed. Conray ventriculography performed on May 2, 1978, demonstrated the blockade in the upper part of the aqueduct of Sylvius. Thereafter the head circumference gradually fell into the normal range. The patient could gaze and follow an object, and control his head in the vertical position and uttered the simplest words. The patient was discharged on May 8, 1978 and was followed up at outpatient clinics. On October 13, 1978, CT scanning with contrast material was done to exclude neoplastic or vascular lesion but was quite satisfactory except
234567891011
lmonth)
Fig 2 Age and head circumference.
for a still dilated left occipital horn where hydrocephalic process was most prominent from the first (Fig I-B). Physical and neurological state at that time were almost normal. Discussion There is no discrete clue in this case to determine whether hemorrhage was the cause of hydrocephalus or secondary to distress of delivery. Concerning the perinatal intraventricular hemorrhage, several etiological factors are proposed, but as for the site of hemorrhage the sub ependymal matrix layer of the lateral ventricle is the most likely place [12]. No one reported a hemorrhage in the aqueduct or in the fourth ventricle [1,4-8, 13]. This hemorrhage might be the result of parenchymal damage secondary to transient temporal lobe herniation which squashed the midbrain against the tentorial notch. But the paucity of neurological sequelae in this case seems inconsistent with this theory. Several etiologies have been proposed as a cause of congenital hydrocephalus. In this case, though the titer of toxoplasma or cytomegalic inclusion virus was not investigated, the normal fundi and absence of intracranial calcification and hepatosplenomegaly were not indicative of them. There were neither signs indicative of a dysraphic state nor evidence of familial occurrence. Only CT clearly demonstrated the high density area about the aqueductal region, in the fourth ventricle and perhaps partly in the third ventricle. Because there were not any signs of hemorrhage in the lateral ventricles on CT, it
Enomoto et al: Suspected intraaqueductal perinatal hemorrhage 95
seemed not likely that the primary site was in this ventricle, though we have no reasonable explanation why hemorrhage occurred in such a region during perhaps perinatal distress. Other than cephalopelvic discrepancy we could find no other reasons to explain the hemorrhage. We think this kind of lesion may partly elucidate the etiology of aqueductal stenosis. References 1. Leech RW, Kohnen P. Subependymal and intraventricular hemorrhages in the newborn. Am J Pathol1974;77:465-75. 2. Gray OP, Ackerman A, Fraser AJ. Intracranial hemorrhage and clotting defects in low-birthweight infants. Lancet 1968;1:545-8. 3. Grontoft O. Intracerebral and meningeal haemorrhages in perina tally deceased infan ts. I, Intracerebral haemorrhages, a pathologico-anatomical and obstetric study. Acta Obstet Gynecol Scand 1953;32:308-34. 4. Gruenwald P. Subependymal cerebral hemorrhage in premature infants, and its relation to various injurious influences at birth. Am J Obstet Gynecol1951;61:1285-92. 5. Harcke HT, Naeye RL, Storch A, Blanc WA. Perinatal intraventricular hemorrhage. J Pediatr
96 Brain & Development, Vol 3, No 1,1981
1972;80:37-42. 6. Maki Y, Shirai S. Angiographic findings in intraventricular hemorrhage in newborn infants. Acta Radiol rSuppl' 1975;347:167-74. 7. Towbin A. Cerebral intraventricular hemorrhage and subependymal matrix infarction in the fetus and premature newborn. Am J Pathol 1968;52: 121-33. 8. Deonna T, Payot M, Probst A, Prod'hom L-S. Neonatal intracranial hemorrhage in premature infants. Pediatrics 1975;56:1056-64. 9. Korobkin R. The relationship between head circumference and the development of communicating hydrocephalus in infants following intraventricular hemorrhage. Pediatrics 1975;56:74-7. 10. Lorber J, Bhat US. Posthaemorrhagic hydrocephalus. Diagnosis, differential diagnosis, treatment and long-term results. Arch Dis Child 1974; 49:751-62. 11. Volpe JJ, Pasternak JF, Allan WC. Ventricular dilatation preceding rapid head growth following neonatal intracranial hemorrhage. Am J Dis Child 1977; 131 :1212-5. 12. Ross JJ, Dimmette RM. Subependymal cerebral hemorrhage in infancy. Am J Dis Child 1965; 110:531-42. 13. Krishnamoorthy KS, Fernandez RA, Momose KJ, et al. Evaluation of neonatal intracranial hemorrhage by computerized tomography. Pediatrics 1977;59:165-72.
A 8-day-old boy with congenital hydrocephalus was given a shunt operation. Computed tomography (CT) revealed marked internal hydrocephalus and a high density area about the aqueduct of Sylvius, in the fourth ventricle and perhaps partly in the third ventricle which was completely absorbed one month later. Later conray ventriculography demonstrated aqueductal obstruction. Neuroradiologically there was neither neoplastic nor vascular lesion. The patient achieved almost normal motor and mental development at 9 months of age. The cephalopelvic discrepancy and vacuum extraction are thought to be the most likely causes of the hemorrhage. The site of hemorrhage was quite unusual as a neonatal intraventricular hemorrhage. Enomoto T, Maki Y, Nakagawa K, Nakada Y, Hamano K. Suspected intraaqueductal perinatal hemorrhage in congenital hydrocephalus. Brain Dev 1981;3: 93-6
Intraventricular hemorrhage is often associated with premature or low-birth-weight infants. The site of hemorrhage is predominantly the sub ependymal matrix layer of the lateral ventricle or choroid plexus in some cases [1]. The causative factors that contribute to the hemorrhage are still the subject of controversy [2-7]. Obstructive hydrocephalus often develops as a sequela of intraventricular hemorrhage, which is not always responsive even today to a shunt operation [8-11]. The authors report a case with congenital hydrocephalus diagnosed From the Department of Neurological Surgery, The University of Tsukuba School of Medicine, Ibaraki. Received for pUblication: June 26, 1980. Accepted for publication: September 20, 1980.
Key words: Hydrocephalus, intraventricular hemorrhage, aqueductal stenosis, CT-scan. Correspondence address: Dr. Yutaka Maki, Department of Neurological Surgery, The University of Tsukuba School of Medicine, Niihari-gun, Ibaraki 305, Japan.
by CT-scan which disclosed a high density area about the aqueduct of Sylvius, in the fourth ventricle and partly in the third ventricle and treated successfully with a shunt operation. Case Report A 8-day-old boy was admitted to our hospital on February 8, 1978, because of a large head. The pregnancy was uneventful until January 10, 1978, when the mother took ABPC for three days for a common cold. On January 31, 1978, after premature rupture of the amniotic membrane the patient was born with the aid of oxytocin and vacuum extraction at the 33rd week of pregnancy. The Apgar score was 5 at birth. The patient weighed 3,050 g. The chest circumference was 29 cm and that of the head was 40 cm, the latter thereafter increased by 0.5 cm a day. Grand mal seizure appeared on February 1, 1978. Physical examination on admission revealed a somewhat icteric, inactivated and dry infant
with a large scaphocephalic head. All of the sutures except the squamous were widely separated. The anterior fontanelle was tightly distended. The head circumference was 43.7 cm. No abnormalities were noted of the heart, lungs, liver and spleen. There was no signs
indicative of a dysraphic state. Neurological examination revealed an inactivated infant with rigidity of the flexor muscle group especially of upper extremities. Though sucking reflex was poor, other primitive reflexes including Moro, Galant, ATNR and plantar grasp were noted.
Fig I-A CT·scan taken before first operation demonstrating markedly dilated lateral and third ventricles with a high density area about the aqueduct of Sylvius, in the fourth ventricle and partly in the third ventricle (arrow). Dilatation of the lateral ventricle is more marked on the left shifting the falx to the right. Fig I-B CT·scan taken after operation at 8 months of age demonstrating the thickened cerebral mantle with a still enlarged left occipital horn. No abnormally enhanced area can be seen.
94 Brain & Development, Vol 3, tvo 1, 1981
There was the sun setting phenomenon and the Babinski sign was positive. CT scanning carried out on admission revealed marked internal hydrocephalus with a high density area about the aqueduct ofSylvius, in the fourth ventricle and partly in the third ventricle but none in the lateral ventricles (Fig I-A). Laboratory examinations including spinal CSF were not remarkable. But the titer of toxoplasma and cytomegalic inclusion virus were not examined. A ventriculoatrial shunt performed on February 10, 1978, improved movement of the extremities and decreased tension of the fontanelle. But because of subscalp fluid collection, suggesting malfunction of the shunt device, this V-A shunt was replaced by a ventriculoperitoneal shunt on February 16, 1978. On March 10, 1978, about one month after the first record, a CT-scan was taken for the second time, which demonstrated improvement of the hydrocephalus and complete abolishment of the high density area. Though the sunset phenomenon was still noted feeding difficulty was much improved. Retrograde brachial angiography on the right side was carried out on March 26, 1978, to exclude neoplastic or vascular lesion. But the sequelae were all consistent with hydrocephalus. 99ffiTc brain scan performed on April 1, 1978, was also not contributory except for the hydrocephalic change. On the other hand the head again began to grow steadily (Fig 2). The fontanelle was tightly distended. Head lag was noted still. As these clinical signs suggested malfunctioning of the shunting device again, we carried out RI cisternography which showed delayed cortical clearing especially over the left hemisphere but no ventricular reflux. But CT one month after the revision demonstrated the thickened cortical mantle and so re-revision was postponed. Conray ventriculography performed on May 2, 1978, demonstrated the blockade in the upper part of the aqueduct of Sylvius. Thereafter the head circumference gradually fell into the normal range. The patient could gaze and follow an object, and control his head in the vertical position and uttered the simplest words. The patient was discharged on May 8, 1978 and was followed up at outpatient clinics. On October 13, 1978, CT scanning with contrast material was done to exclude neoplastic or vascular lesion but was quite satisfactory except
234567891011
lmonth)
Fig 2 Age and head circumference.
for a still dilated left occipital horn where hydrocephalic process was most prominent from the first (Fig I-B). Physical and neurological state at that time were almost normal. Discussion There is no discrete clue in this case to determine whether hemorrhage was the cause of hydrocephalus or secondary to distress of delivery. Concerning the perinatal intraventricular hemorrhage, several etiological factors are proposed, but as for the site of hemorrhage the sub ependymal matrix layer of the lateral ventricle is the most likely place [12]. No one reported a hemorrhage in the aqueduct or in the fourth ventricle [1,4-8, 13]. This hemorrhage might be the result of parenchymal damage secondary to transient temporal lobe herniation which squashed the midbrain against the tentorial notch. But the paucity of neurological sequelae in this case seems inconsistent with this theory. Several etiologies have been proposed as a cause of congenital hydrocephalus. In this case, though the titer of toxoplasma or cytomegalic inclusion virus was not investigated, the normal fundi and absence of intracranial calcification and hepatosplenomegaly were not indicative of them. There were neither signs indicative of a dysraphic state nor evidence of familial occurrence. Only CT clearly demonstrated the high density area about the aqueductal region, in the fourth ventricle and perhaps partly in the third ventricle. Because there were not any signs of hemorrhage in the lateral ventricles on CT, it
Enomoto et al: Suspected intraaqueductal perinatal hemorrhage 95
seemed not likely that the primary site was in this ventricle, though we have no reasonable explanation why hemorrhage occurred in such a region during perhaps perinatal distress. Other than cephalopelvic discrepancy we could find no other reasons to explain the hemorrhage. We think this kind of lesion may partly elucidate the etiology of aqueductal stenosis. References 1. Leech RW, Kohnen P. Subependymal and intraventricular hemorrhages in the newborn. Am J Pathol1974;77:465-75. 2. Gray OP, Ackerman A, Fraser AJ. Intracranial hemorrhage and clotting defects in low-birthweight infants. Lancet 1968;1:545-8. 3. Grontoft O. Intracerebral and meningeal haemorrhages in perina tally deceased infan ts. I, Intracerebral haemorrhages, a pathologico-anatomical and obstetric study. Acta Obstet Gynecol Scand 1953;32:308-34. 4. Gruenwald P. Subependymal cerebral hemorrhage in premature infants, and its relation to various injurious influences at birth. Am J Obstet Gynecol1951;61:1285-92. 5. Harcke HT, Naeye RL, Storch A, Blanc WA. Perinatal intraventricular hemorrhage. J Pediatr
96 Brain & Development, Vol 3, No 1,1981
1972;80:37-42. 6. Maki Y, Shirai S. Angiographic findings in intraventricular hemorrhage in newborn infants. Acta Radiol rSuppl' 1975;347:167-74. 7. Towbin A. Cerebral intraventricular hemorrhage and subependymal matrix infarction in the fetus and premature newborn. Am J Pathol 1968;52: 121-33. 8. Deonna T, Payot M, Probst A, Prod'hom L-S. Neonatal intracranial hemorrhage in premature infants. Pediatrics 1975;56:1056-64. 9. Korobkin R. The relationship between head circumference and the development of communicating hydrocephalus in infants following intraventricular hemorrhage. Pediatrics 1975;56:74-7. 10. Lorber J, Bhat US. Posthaemorrhagic hydrocephalus. Diagnosis, differential diagnosis, treatment and long-term results. Arch Dis Child 1974; 49:751-62. 11. Volpe JJ, Pasternak JF, Allan WC. Ventricular dilatation preceding rapid head growth following neonatal intracranial hemorrhage. Am J Dis Child 1977; 131 :1212-5. 12. Ross JJ, Dimmette RM. Subependymal cerebral hemorrhage in infancy. Am J Dis Child 1965; 110:531-42. 13. Krishnamoorthy KS, Fernandez RA, Momose KJ, et al. Evaluation of neonatal intracranial hemorrhage by computerized tomography. Pediatrics 1977;59:165-72.