Although head trauma is suggested to precipitate hydrocephalus in adult-onset Dandy Walker syndrome (DWS), the clear mechanism is not verified. A 56-year-old female recovered completely after evacuation of acute epidural hematoma until development of dementia 7 days after operation. At day 20, she underwent ventriculo-peritoneal shunting to treat progressive hydrocephalus and has been free from neurologic symptoms for 2 years. The serial CT examinations indicate that after head trauma without subarachnoid hemorrhage, hydrocephalus can develop in a case of previously silent DWS. Rapid changes of intracranial pressure in trauma may affect cerebrospinal fluid outflow through a DWS-related valve mechanism at the foramina of Luschka, which results in hydrocephalus

Kaminogo Makio

Moroki Jiro

Ochi Akira

Shibata Shobu

Nagasaki University's Academic Output SITE: NAOSITE

Acta Med. Nagasaki 44 : 53-57Onset of Dandy-Walker Syndrome in Adult following Head Trauma: A Case Report.Makio KAMINOGO,1) Akira OCHI,1) Jiro MOROKI,2) Shobu SHIBATA 1)1) Department of Neurosurgery, Nagasaki University School of Medicine 2) Department of Neurosurgery, Yamaguchi Central Hospital, Yamaguchi Although head trauma is suggested to precipitate hydro-cephalus in adult-onset Dandy Walker syndrome (DWS), the clear mechanism is not verified. A 56-year-old female recovered completely after evacuation of acute epidural hematoma until development of dementia 7 days after op-eration. At day 20, she underwent ventriculo-peritoneal shunting to treat progressive hydrocephalus and has been free from neurologic symptoms for 2 years. The serial CT examinations indicate that after head trauma without subarachnoid hemorrhage, hydrocephalus can develop in a case of previously silent DWS. Rapid changes of intracranial pressure in trauma may affect cerebrospinal fluid outflow through a DWS-related valve mechanism at the foramina of Luschka, which results in hydrocephalus.Key words : Dandy Walker syndrome, hydrocephalus, head            trauma, and adultIntroduction The Dandy-Walker syndrome (DWS) is developmen-tal anomaly consisting of a posterior fossa cyst (cystic dilation of the fourth ventricle) and hypoplasia of the cerebellar vermis.''" Clinical expression of DWS usually occurs early in life, its major manifestations are caused by hydrocephalus which is considered a consequence, not a part, of the DWS.1-3' Occasionally, patients first become symptomatic with increased intracranial pres-sure in adult life.'-" Head trauma has been suggested to precipitate such hydrocephalus in adults,'' but theAddress Correspondence : Makio Kaminogo, M.D. Department of Neurosurgery, Nagasaki University School of Medicine 1-7-1 Sakamoto, Nagasaki, 852-8501 Japan TEL : +81-95-849-7375, FAX: +81-95-849-7378 E-mail : kaminogo@net.nagasaki-u.ac.jppathophysiology of DWS-associated hydrocephalus in relation to head trauma has not been clarified. We pre-sent an adult with DWS in whom symptomatic hydro-cephalus developed soon after evacuation of an acute epidural hematoma.Case report  A 56-year-old female was referred to us because of altered consciousness (Glasgow Coma Scale: E3, V4, M6) and mild right hemiparesis developing about 12 hrs after head trauma. She had a normal developmental history and had been in good health. She fell down second-floor stairs late one evening and consulted a local phy-sician immediately afterward. No neurologic deficit was evident, and she returned home after treatment of a left parietal scalp laceration. She returned to the physi-cian the next morning because of progressive head-ache and drowsiness, and was referred urgently to us. In addition to the left parietal scalp laceration, a soft tissue mass about 3 cm in diameter was found in the occipital area. Her head circumference measured 57.3 cm. (After full recovery, she indicated that she had been aware of the occipital mass prior to injury but had not asked a physician about it.) Computed tomography (CT) on admission demonstrated a left epidural hematoma, a small contusion in the right temporal lobe, a large posterior fossa cyst, hypoplasia of the vermis, an oc-cipital bone defect, and a cystic scalp mass communi-cating with the posterior fossa cyst through the bone defect. No subarachnoid hemorrhage (SAH) or hydro-cephalus was evident (Fig. 1). The epidural hematoma was evacuated immediately. During surgery the dura mater was opened to examine the brain surface, but no subdural hematoma or SAH was disclosed. Consciousness became clear and left hemiparesis disappeared on the day following surgery, when CT indicated that decom-pression had been obtained both supratentorially andFig. 1 Computed tomograms on admission (12 hrs after head trauma). A left epidural hematoma, a small contusion in the right temporal lobe, a posterior fossa cyst com-municating with the fourth ventricle, hypoplasia of the vermis, an occipital bone defect, and an occipital meningocele com-municating with the posterior fossa cyst were revealed.Fig. 3 Computed tomograms 13 days after head injury revealed hydrocephalus and en-largement of the posterior fossa cyst.Fig. 4 Magnetic resonance imaging shortly before ventriculo-peritoneal shunt placement verified patency of the aqueduct and indicated anterior displacement of the brainstem due to enlargement of the posterior fossa cyst.Fig. 2 Computed tomograms on the day after evacuation of the hematoma. Enlargement of the subdural or subarachnoid space was depicted infratentorially.infratentorially according to enlargement of subdural and/or subarachnoid spaces (Fig. 2). Six days after the head injury, she again began to complain of head-ache and nausea, and disorientation and urinary incon-tinence were noted at about day 10. CT on day 13 re-vealed dilation of lateral and third ventricles (Fig. 3). The posterior fossa cyst also had enlarged. She under-went ventriculo-peritoneal (V-P) shunting on day 20 after patency of the aqueduct had been verified using magnetic resonance imaging (MRI, Fig. 4). Postoperativelyher neurologic symptoms resolved and her occipital scalp mass became smaller. MRI 10 days after shunt-ing revealed patency of the aqueduct, hypoplasia of the corpus callosum, and disappearance of previously noted anterior displacement of the brainstem (Fig. 5). She was discharged with no neurologic deficit 17 days after V-P shunt placement. Follow-up CT demonstrated reduction in size of the lateral ventricles (Fig. 6). The posterior fossa cyst also was smaller. She remained asymptomatic with a normal neurologic examination 2 years after surgery.Fig. 5 Magnetic resonance imaging 10 days after shunt placement revealed patency of the aqueduct somewhat more clearly than Figure 4. Hypoplasia of the corpus callosum and reversal of anterior brainstem displacement also were depicted.Fig. 6 Follow-up computed tomograms 3 months after shunting demonstrated normal size of the anterior horns of the lateral ventricle and of the third ventricle, although the bodies of the lateral ventricles were somewhat large.Discussion  In most cases of DWS, especially in children, symptoms and signs are caused by hydrocephalus,', 10, 11) which re-sults from but is not part of the malformation. 1-3) Late onset of symptomatic hydrocephalus in adult life occa-sionally has been reported, 6, 11, 9 11' these cases follow one of two patterns, either new appearance of hydrocepha-lus with normal head circumference"' or loss of com-pensation in early arrested hydrocephalus with a large head.',',', 13, 14) The etiology of the late-onset cases is not clear, but in some reported cases head trauma had pre-ceded onset of symptomatic hydrocephalus. Lipton et al. reviewed seven cases of adult-onset DWS, pointing out that hydrocephalus appeared to be precipitated by minor head trauma in two of the seven patients.s' On reviewing previous reports of DWS following head trauma, we noted that several cases were discovered incidentally by neuroradiologic examination after head injury. Some of these patients exhibited no neurologic symptoms,"' while others had neurologic signs or symptoms not related to DWS.16-18'  Three well-documented DWS cases exhibited symp-tomatic hydrocephalus after head trauma (Table). Two manifested papilledema 2 to 3 weeks after minor head injury."') The third patient, with pre-existing mild mental retardation, exhibited gradually progressing im-pairment of recent memory and of vision including diplopia.13) However, little documentation of actual onset of hydrocephalus was available in these three cases. Ours is the first report of adult-onset DWS with onset of symptoms of hydrocephalus after head trauma in which serial CT and MRI were performed in the acute posttraumatic stage.  In the present case, head circumference was normal and no developmental delay was evident. CT immedi-ately after evacuation of the hematoma revealed nor-mal size of the third ventricle and the anterior horns of the lateral ventricles, though the bodies of the lat-eral ventricle was slightly enlarged (probably because of the hypoplastic corpus callosum). These findings suggested that hydrocephalus did not develop until after head trauma in our case. Occipital meningocele such as in our patient is not a rare accompanying mal-formation in DWS.19' She had been aware of the occipi-tal mass but did not consult a physician, because it did not progressively enlarge. As it shrank signifi-cantly after V-P shunting and was covered with nor-mal skin and thick subcutaneous tissue, we did not re-pair it.  Posttraumatic hydrocephalus is not common compli-cation of head injuries. Cardoso et al. retrospectively reviewed 2374 severely head injured patients andTable. Adult onset of symptomatic hydrocephalus following head trauma in Dandy-Walker syndromeSource Age/Sex Head trauma Size of head Developmental ICP* Cerebellar or Clinical                                               delay elevation brainstem signs evolutionSahr (8) 16y.o./M neck-strength ? (-) (+) (+) acute                      contest Coleman 17y.o./M collision with relatively (-) (+) (-) acute et al (5) other boy large Stovall 30y.o./M struck by car large (+) (-) (+) chronic et al (13) while walking (67.3 cm)ICP *, intracranial pressurepointed out that hydrocephalus demonstrating large ventricles without enlargement of the sulci and new development of symptoms was found only in 17 pa-tients (0.7%).'0' They also pointed out that SAH and intracranial infection following head injury were the major causes of the traumatic hydrocephalus.") However, intraoperative findings and CT imagings did not indi-cate the apparent SAH to induced subsequent hydro-cephalus. Moreover, there was no evidence of inflam-mation causing hydrocephalus. Since arachnoid cysts can bleed after minor trauma, unsupported vessels around the occipital cyst are postulated to tear easily resulting in hemorrhage into the posterior fossa cyst or the subdural or subarachnoid space in the posterior fossa. Such bleeding may be responsible for distur-bance of cerebrospinal fluid (CSF) outflow from the fourth ventricle to the perimedullary subarachnoid space or CSF flow within the subarachnoid space. Hirsch et al. point out that hydrocephalus develops frequently in the 2 months following birth and postu-late that bleeding at the time of delivery also may rep-resent an important cause of infantile-onset DWS.3' However, no neuroradiologic verification is available for this hypothesis. While the present case is the first in which CT was reported from the acute stage of head trauma in a patient with DWS who subsequently developed symptomatic hydrocephalus, CT did not demonstrate intracranial hemorrhage which would in-terfere with CSF flow. In one of the three reportedcases, CSF was examined by lumbar puncture in the acute posttraumatic stage; again, no subarachnoid hemorrhage was verified." Massive cerebral contusion and cerebral edema can be also proposed as causes of blockage of CSF flow in subarachnoid space, however, serial CT examinations indicated only small cerebral contusion in our patient.  A second explanation is that after head trauma ob-structive mechanisms other than bleeding develop in the CSF outlets from the fourth ventricle to the subarachnoid space. Foramina leading from the ventricular system are not typically patent in patients with DWS; the fo-ramen of Magendie reportedly is obstructed in most cases. CSF is speculated to exit via an undetectable outlet or patent foramina of Luschka.2> Alternatively, the foramina of Luschka may be patent intermittently, with a ball-valve mechanism.") In one adult-onset case related to head trauma, intra.operative inspection indi-cated that neither the foramen of Magendie nor the fo-ramina of Luschka appeared patent.') In that case, head trauma was postulated to directly affect CSF out-flow which previously had been maintained through incompletely occluded foramina. In our case, CT the day after the evacuation of hematoma revealed widen-ing of the subdural or subarachnoid space in the pos-terior fossa, an indication of rapid decompression in the posterior fossa following evacuation of the epidural hematoma. Compression and subsequent decompres-sion of the foramina of Luschka may have occurred withdevelopment of epidural hematoma, and its evacuation, affecting the valve mechanisms in the foramina of Luschka to result in hydrocephalus. Symptoms due to increased intracranial pressure appeared after a neck-strength contest in one reported case." Since increased venous and CSF pressure can occur during exertion, this transient change in ICP also may have interfered with a complicated valvular communication in the fo-ramina of Luschka.  Yet, another explanation for delayed onset of hydro-cephalus is that silent aqueductal stenosis may become symptomatic after head trauma, since aqueductal stenosis has accompanied DWS in some cases."' However, in the present case, MRI disclosed patency of the aque-duct and the occipital meningocele communicating with the occipital cyst shrank after V-P shunt placement, indicating good communication between the lateral ventricles and the occipital cyst.  In summary, the present study cannot precisely demonstrate the cause of DWS-associated hydrocephalus developing after head trauma in adult life. However, CT indicated that hydrocephalus may develop after head trauma without SAH or intracystic hemorrhage in an adult case of previously asymptomatic DWS. Valvular communicating mechanisms in the foramina of Luschka may be vulnerable to transient changes in ICP accompanying head trauma.References1) Gardner E, O'Rahilly R, Prolo D. The Dandy-Walker and Arnold-   Chiari malformations. Clinical, developmental, and teratological    considerations. Arch Neurol 32:393-407, 1975 2) Raybaud C. Cystic malformations of the posterior fossa. Abnormalities    associated with the development of the roof of the fourth ventricle    and adjacent meningeal structures. J Neuroradiology 9:103-133,   19823) Hirsch JF, Pierre-Kahn A, Renier D, Sainte-Rose C, Hoppe-Hirsch E.    The Dandy-Walker malformation. A review of 40 cases. J Neurosurg   61:515-522, 1984 4) Cohen I. Agenesis of the cerebellum (verified by operation). J Mt    Sinai Hosp 8:441-446, 1942 5) Coleman CC, Troland CE. Case reports and technical notes.    Congenital atresia of the foramina of Luschka and Magendie. With    report of two cases of surgical cure. J Neurosurg 5:84-88, 1948 6) Lipton HL, Preziosi TJ, Moses H. Adult onset of the Dandy-Walker    syndrome. Arch Neurol 35:672-674, 1978 7) Maloney AFJ. Two cases of congenital atresia of the foramina of    Magendie and Luschka. J Neurol Neurosurg Psychiat 17:134-138,  1954 8) Sahs AL. Congenital anomaly of the cerebellar vermis. Arch Pathol    32:52-63, 1941 9) Sato K, Kubota T, Nakamura Y. Adult onset of the Dandy-Walker    syndrome. Brit J Neurosurg 10:109-112, 1996 10) Maria BL, Zinreich SJ, Carson BC, Rosenbaum AE, Freeman JM.    Dandy-Walker syndrome revisited. Pediat Neurosci 13:45-51, 198711) Osenbach RK, Menezes AH. Diagnosis and management of the    Dandy-Walker malformation: 30 years of experience. Pediatr    Neurosurg 18:179-189, 1992 12) Engelhard HH, Meyer JR. Adult-onset presentation of Dandy-   Walker variant in siblings. Surg Neurol 44:43-47, 1995 13) Stovall JM, Venkatesh R. Magnetic resonance imaging of an adult    with the Dandy-Walker syndrome. J Natl Med Assoc 80:1241-1247,   1988 14) Walker AE. A case of congenital atresia of the foramina of    Luschka and Magendie. J Neuropathol Exp Neurol 3:368-373, 199415) Yamamoto Y, Waga S. Asymptomatic Dandy-Walker variant in an    adult. Surg Neurol 22:241-242, 1984 16) Cone AM. Head injury in an adult with previously undiagnosed    Dandy-Walker syndrome. A review of the condition and discussion    of its anaesthetic implications. Anaesth Intens Care 23:613-615,   1995 17) Ishimitsu H, Namba S, Nakasone S. An adult case of a Dandy-   Walker syndrome. Brain and Nerve 31:583-589, 1979 (abstract in    English) 18) Whittern CA Jr, Wise BL. Hydrocephalus syndrome. Obstruction of    the foramina of the fourth ventricle. Am J Dis Child 103:87-92,  1962 19) Bindal AK, Storrs BB, McLone DG. Occipital meningoceles in pa-   tients with the Dandy-Walker syndrome. Neurosurgery 28:844-847,   1991 20) Cardoso ER, Galdbraith S. Posttraumatic hydrocephalus. A retro-   spective review. Surg Neurol 23:261-264, 1985 21) Fischer EG. Dandy-Walker syndrome: an evaluation of surgical    treatment. J Neurosurg 39:615-621, 1973

Onset of Dandy-Walker Syndrome in Adult following Head Trauma: A .

https://nagasaki-u.repo.nii.ac.jp/?action=repository_action_common_download&item_id=20189&item_no=1&attribute_id=18&file_no=1

Onset of Dandy-Walker Syndrome in Adult following Head Trauma: A .

Abstract

Similar works

Full text

Available Versions

Nagasaki University's Academic Output SITE: NAOSITE