Childs Nerv Syst (2002) 18:652–655 DOI 10.1007/s00381-002-0639-3
Takanori Inamura Takato Morioka Shunji Nishio Kiyonobu Ikezaki Hiroki Nonaka Takashi Yoshiura
Received: 13 March 2001 Revised: 24 May 2002 Published online: 4 September 2002 © Springer-Verlag 2002 T. Inamura · T. Morioka (✉) · S. Nishio K. Ikezaki · H. Nonaka Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan e-mail:
[email protected] Tel.: +81-92-6425524 Fax: +81-92-6425526
C A S E R E P O RT
Diverticular enlargement of the foramina of Luschka and congenital hydrocephalus
Abstract Case report: We report on a 9-month-old boy with congenital hydrocephalus involving cystic lesions in the bilateral cerebellopontine angle cisterns. Sequential CT cisternography demonstrated congenital obstruction of the fourth ventricular outlet and diverticular enlargement of the foramina of Luschka. Discussion: The possibility of neuroendoscopic third ventriculostomy as a treatment for these pathologies is discussed.
Keywords CT cisternography · Congenital hydrocephalus · Fourth ventricular outlet obstruction · Foramina of Luschka · Neuroendoscopic third ventriculostomy
T. Yoshiura Department of Radiology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan
Introduction Congenital fourth ventricular outlet obstruction (FVOO) is a rare cause of hydrocephalus [1, 22]. FVOOs occur primarily in children and are the subject of many reports on Dandy-Walker cyst, which is characterized by atresia of the foramina of Luschka and Magendie and hypoplasia or absence of the cerebellar vermis [21]. The present paper describes a 9-month-old boy involving congenital hydrocephalus with cystic lesions of the bilateral cerebellopontine (CP) angle cisterns. Sequential CT cisternography demonstrated congenital FVOO and diverticular enlargement of the foramina of Luschka.
cy, and delivery had been unremarkable. The boy’s birth weight was 3,038 g, the Apgar score was 9, and his head circumference at birth was 32 cm. Physical and mental development were normal until the arrest of mental development at 7 months.
Case report A 9-month-old boy was admitted to our hospital with nausea and vomiting. No abnormalities had been apparent during the pregnan-
Fig. 1 Computed tomography (CT) on admission shows remarked enlargement of all ventricles. Both cerebellopontine (CP) angle cisterns also are enlarged
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On admission, the head circumference was unusually large (47 cm), in association with tense fontanels. A skull radiograph showed many digital impressions, and the dorsum sellae showed osteolytic changes. CT showed dilated ventricles and a bilateral low-density space in the CP angle cistern (Fig. 1). Contrast enhanced CT showed no abnormal enhancement. We inserted an external ventricular drainage system through right anterior horn as an emergency procedure, with the intention of performing neuroendoscopic third ventriculostomy (NTV). The aqueduct was patent, and the floor of the third ventricle was displaced downward. Adhesion of the third ventricular floor to the dorsum sellae caused the attempt at NTV to fail. The external ventricular drain remained in place for 10 days, during which we performed magnetic resonance imaging (MRI) and CT cisternography. MRI again demonstrated the dilated ven-
Fig. 2A–D Magnetic resonance imaging (MRI) findings after placement of a right external ventricular drainage system. A Sagittal T1-weighted image demonstrates enlargement of all ventricles. Downward displacement of the third ventricular floor and compression of the brain stem with anterior displacement by the enlarged fourth ventricle are also seen. B Cine-MRI shows absence of cerebrospinal fluid (CSF) signal through the foramen of Magendie. C A T2-weighted image demonstrates marked periventricular hyperintensity. D Signal intensity in the bilaterally enlarged CP angles is the same as that of the CSF Fig. 3 Sequential CT cisternography. After contrast medium (CM) was introduced by lumbar puncture, sequential CT was performed. CM rapidly entered the cisterns, the left sylvian fissure, and only a small portion of the surrounding cerebral subarachnoid space (top row). CM did not enter other portions of the cerebral subarachnoid space, including the right sylvian fissure (middle two rows). CM also persisted for 48 h after spinal introduction. Ventricular (vent) CM administration demonstrated communications between the ventricles and the cerebellopontine angle cysts (bottom row)
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Fig. 4 MRI 3 months after the shunt placement shows the reduced size of the ventricles and the cysts in the CP angles
tricles with periventricular T2-hyperintensity and cystic enlargement of both CP angle cisterns (Fig. 2A, C, D). Cine-MRI showed absence of any CSF flow signal in the foramen of Magendie (Fig. 2B). CT cisternography with contrast material (CM) introduced by lumbar puncture showed communication between the spinal subarachnoid space and the cerebral cisterns (Fig. 3, upper three rows). CM rapidly entered the cisterns, the left sylvian fissure, and only a small portion of the surrounding cerebral subarachnoid space (Fig. 3, upper row). CM did not enter other portions of the cerebral subarachnoid space, including the right sylvian fissure (Fig. 3, middle two rows). CM also persisted for 48 h after spinal introduction. There was no communication between any of the ventricles or the cystic lesion in the bilateral CP angles and the spinal subarachnoid space and cerebral cisterns. After the CM in the cisterns had largely dissipated, we introduced additional CM via the external ventricular catheter. CT cisternography demonstrated communications between the ventricles and the enlarged space in the CP angles bilaterally (Fig. 3 lower row). The external ventricular drain was replaced by a ventriculoperitoneal shunt. The postoperative course was uneventful, and the baby’s symptoms completely resolved. MRI performed 3 months after the shunt placement showed the reduced size of ventricles and the space bilaterally in the CP angle cisterns (Fig. 4).
Discussion This infant was admitted with rapid development of symptoms of obstructive hydrocephalus. However, the radiographic findings of digital impressions and osteolytic changes in the dorsum sellae indicated long-standing, presumably congenital, hydrocephalus. FVOO, a rare but recognized cause of hydrocephalus [1, 15, 20, 21, 22, 23], was diagnosed in our patient by CT cisternography. Congenital FVOOs were initially described by Dandy [11] and their presence has subsequently been designated Dandy-Walker syndrome [3]. They are also seen in association with such syndromes as Chiari malformation [22], tuberous sclerosis [12], spina bifida and congenital anomalies of the craniovertebral junction, such as platybasia and basilar impression [21]. No such associated abnormality was seen in our patient. Five cases of adult-
onset hydrocephalus with congenital membranous obstruction of the foramen of Magendie have been reported [1, 10, 21]. The presence of semipermeable membranous perforations, or of hinged or irregularly dissected membranes, could explain the delayed onset of symptomatic hydrocephalus in these patients [21]. Semiperforate foramina of Luschka may have been sufficient to allow some CSF flow for prolonged periods of time [21]. In these hydrocephalic patients, the fourth ventricles were markedly enlarged and the CP angle cisterns were usually obliterated. One of the characteristic findings in our patient was the symmetrical, cystic enlargement of the CP angles. And these cystic lesions had communication with the fourth ventricle, but not with the cistern. Furthermore, they had disappeared when MRI was performed 3 months after shunt placement. CP angle cysts are relatively rare and usually unilateral [2, 13, 17]. Thus, they were thought to membranous obstruction and diverticular enlargements of the foramina of Luschka. Although FVOO has recently been treated successfully by NTV [20, 22], this could not be attempted in this case, because the floor of the third ventricle was tightly adherent to the dorsum sellae. This adhesion provided further support for the assumption of long-standing hydrocephalus. Liliequist’s membrane sometimes tightly sequesters the underlying cistern in patients with hydrocephalus, which is one potential cause of failure of NTV [4, 7]. Recently, NTV has come to be regarded as less likely to be effective in patients under 1 year old than in adults, possibly because of insufficient CSF absorption by the arachnoid granulations and/or inadequate CSF circulation in infants with hydrocephalus [5, 6, 7, 9, 16, 18, 19]. On CT cisternography in our patient, CM introduced by lumbar puncture only entered the left sylvian fissure and the surrounding subarachnoid space, without opacification of the entire subarachnoid space of the cerebral convexity, remaining, abnormally, in the cisterns for 3 days after administration. These findings suggest insufficient CSF absorption by the arachnoid granulations in this patient. NTV is widely applied as a treatment for hydrocephalus, and specific indications and contraindications are becoming established [8, 14]. The causes of diminished effectiveness of NTV in infants should be investigated in detail, and CT cisternography has proved to be highly useful. Acknowledgement We thank Ms. Yoshie Hirosawa for her valuable assistance in preparing this manuscript.
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References 1. Amacher AL, Page PK (1971) Hydrocephalus due to membranous obstruction of the fourth ventricle. J Neurosurg 35:672–676 2. Arai H, Sato K (1991) Posterior fossa cysts: clinical, neuroradiological and surgical features. Childs Nerv Syst 7:156–164 3. Benda CE (1954) The Dandy-Walker syndrome or the so called atresia of the foramen of Magendie. J Neuropathol Exp Neurol 13:14–29 4. Buxton N, Vloeberghs M, Punt J (1998) Liliequist’s membrane in minimally invasive endoscopic neurosurgery. Clin Anat 11:187–190 5. Buxton N, Macarthur D, Mallucci C, Punt J, Vloeberghs M (1998) Neuroendoscopic third ventriculostomy in patients less than 1 year old. Pediatr Neurosurg 29:73–76 6. Buxton N, Macarthur D, Mallucci C, Punt J, Vloeberghs M (1998) Neuroendoscopy in the premature population. Childs Nerv Syst 14:649–652 7. Buxton N, Cartmill M, Vloeberghs M (1999) Endoscopic third ventriculostomy: outcome analysis of 100 consecutive procedures. Neurosurgery 45:957–959 8. Cinalli G, Salazar C, Mallucci C, Yada JZ, Zerah M, Sainte-Rose C (1998) The role of endoscopic third ventriculostomy in the management of shunt malfunction. Neurosurgery 43:1323– 1327
9. Cinalli G, Sainte-Rose C, Chumas P, Zerah M, Brunelle F, Lot G, PierreKahn A, Renier D (1999) Failure of third ventriculostomy in the treatment of aqueductal stenosis in children. J Neurosurg 90:448–454 10. Coleman CC, Troland CE (1948) Congenital atresia of the foramina of Luschka and Magendie. With report of two cases of surgical cure. J Neurosurg 5:84–88 11. Dandy WE (1921) The diagnosis and treatment of hydrocephalus due to occlusions of the foramina of Magendie and Luschka. Surg Gynecol Obstet 32:112–124 12. Elliot GB, Wollin DW (1961) Defect of corpus callosum and congenital occlusion of fourth ventricle with tuberous sclerosis. AJR Am J Roentgenol 85:701–705 13. Erdincler P, Kaynar MY, Bozkus H, Ciplak N (1999) Posterior fossa arachnoid cysts. Br J Neurosurg 13:10–17 14. Fukuhara T, Vorster SJ, Luciano MG (2000) Risk factors for failure of endoscopic third ventriculostomy for obstructive hydrocephalus. Neurosurgery 46:1100–1109 15. Glasauer FE (1975) Isotope cisternography and ventriculography in congenital anomalies of the central nervous system. J Neurosurg 43:18–26 16. Hopf NJ, Grunert P, Fries G, Resch KD, Perneczky A (1999) Endoscopic third ventriculostomy: outcome analysis of 100 consecutive procedures. Neurosurgery 44:795–804 17. Jallo GI, Woo HH, Meshki C, Epstein FJ, Wisoff JH (1997) Arachnoid cysts of the cerebellopontine angle: diagnosis and surgery. Neurosurgery 40:31– 37
18. Jones RF, Kwok BC, Stening WA, Vonau M (1996) Third ventriculostomy for hydrocephalus associated with spinal dysraphism: indications and contraindications. Eur J Pediatr Surg 6:5–6 19. Kim SK, Wang KC, Cho BK (2000) Surgical outcome of pediatric hydrocephalus treated by endoscopic III ventriculostomy: prognostic factors and interpretation of postoperative neuroimaging. Childs Nerv Syst 16:161–168 20. Mohanty A, Anandh B, Kolluri VR, Praharaj SS (1999) Neuroendoscopic third ventriculostomy in the management of fourth ventricular outlet obstruction. Minim Invasive Neurosurg 42:18–21 21. Rifkinson-Mann S, Sachdev VP, Huang YP (1987) Congenital fourth ventricular midline outlet obstruction. Report of two cases. J Neurosurg 67:595–599 22. Suehiro T, Inamura T, Natori Y, Sasaki M, Fukui M (2000) Successful neuroendoscopic third ventriculostomy for hydrocephalus and syringomyelia associated with fourth ventricle outlet obstruction. Case report. J Neurosurg 93:326–329 23. Yamada H, Nakamura S, Tanaka Y, Tojima M, Kageyama N (1982) Ventriculography and cisternography with water-soluble contrast media in infants with myelomeningocele. Radiology 143:75–83
