Hydrosyringomyelia associated with Dandy-Walker malformation — Is it really rare or undiagnosed? (original) (raw)
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Hydrocephalus in Dandy–Walker malformation
Child's Nervous System, 2011
Introduction Even if the first description of Dandy-Walker dates back 1887, difficulty in the establishment of correct diagnosis, especially concerning differential diagnosis with other types of posterior fossa CSF collection, still persists. Further confusion is added by the inclusion, in some classification, of different malformations with different prognosis and therapeutic strategy under the same label of "Dandy-Walker". Methods An extensive literature review concerning embryologic, etiologic, pathogenetic, clinical and neuroradiological aspects has been performed. Therapeutic options, prognosis and intellectual outcome are also reviewed. Conclusion The correct interpretation of the modern neuroradiologic techniques, including CSF flow MR imaging, may help in identifying a "real" Dandy-Walker malformation. Among therapeutical strategies, single shunting (ventriculo-peritoneal or cyst-peritoneal shunts) appears effective in the control of both ventricle and cyst size. Endoscopic third ventriculostomy may be considered an acceptable alternative, especially in older children, with the aim to reduce the shunt-related problems. Prognosis and intellectual outcome mostly depend on the presence of associated malformations, the degree of vermian malformation and the adequate control of hydrocephalus.
Journal of neurosurgery. Pediatrics, 2009
Patients with symptomatic Chiari malformation Type I (CM-I) typically exhibit a chronic, slowly progressive disease course with evolution of symptoms. However, some authors have reported acute neurological deterioration in the setting of CM-I and acquired Chiari malformations. Although brainstem dysfunction has been documented in patients with CM-II and hydrocephalus or shunt malfunction, to the authors' knowledge only 1 report describing ventriculoperitoneal (VP) shunt malfunction causing neurological deterioration in a patient with CM-I exists. The authors report on their experience with the treatment of previously asymptomatic CM-I in 2 children who experienced quite different manifestations of acute neurological deterioration secondary to VP shunt malfunction. Presumably, VP shunt malfunction created a positive rostral pressure gradient across a stenotic foramen magnum, resulting in tetraparesis from foramen magnum syndrome in 1 patient and acute ataxia and cranial nerve def...
Hydrocephalus and Dandy- Walker Malformation: a review
2020
Introduction: Dandy Walker malformation (DWM) is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle leading to an increase of the posterior fossa and superior dislocation of the lateral sinus, tentorium and torcula. Although it is the commonest posterior fossa malformation, its pathogenesis is still not fully understood, making the differential diagnosis with other posterior fossa malformations difficult and as a result the choice of therapeutic strategy. Material and methods: An extensive review of the literature relating to Dandy Walker malformation was performed. Historical, genetic, embryologic, epidemiologic, clinical and radiological presentation, treatment and prognosis were revised. Conclusion: The correct diagnosis of Dandy Walker malformation can be made through careful interpretation of magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) flow studies. The choice of hydrocephalus treatment depends on wheth...
Journal of Neurosurgery: Pediatrics, 2009
Object Hydrocephalus is a notorious neurosurgical disease that carries the adage “once a shunt always a shunt.” This study was conducted to review the treatment results of pediatric hydrocephalus. Methods Pediatric patients who underwent ventriculoperitoneal shunt surgery over the past 14 years were reviewed for shunt revisions. Variables studied included age at shunt placement, revision, or replacement; programmable shunts; infection; obstruction; and diagnosis (congenital, posthemorrhagic, craniospinal dysraphism, and others including trauma, tumors, and infection). Multiple regression analysis methods were used to determine independent risk factors for shunt failure and the number of shunt revisions. The Kaplan-Meier method of survival analysis was used to compare etiologies on the 5-year survival (revision-free) rate and the median 5-year survival time. Results A total of 253 patients were studied with an almost equal sex distribution. There were 92 patients with congenital hydr...
Shunt malfunction in patients with hydrocephalus: complications revisited Images in
DESCRIPTION The ventriculoperitoneal shunt has been the mainstay for definitive treatment of hydrocephalus since time immemorial. As such, several case reports describing the complications of this procedure have been documented in the literature over the past few decades. The spectrum ranges from ventricular catheter dislodgement to abdominal catheter end perforating the stomach and causing intussuscep-tion; several case illustrations have depicted the possible aftermath of this blind procedure. After the advent of neuronavigation and planning procedures on CT scans, the complication rates have significantly reduced. Shunt malfunction, secondary to migration of the abdominal end, remains as one of the most common complications following ventriculoperito-neal shunting. 1 Large bowel perforation is a rare complication with an incidence of 0.1-0.7%. 2 In rare instances, there have been cases of herniation of the peritoneum along with the distal end of the catheter through a lax posterior rectus sheath. 3 The unprecedented cases we describe, the first of a child and the other of an elderly woman, mandate a separate mention in the literature. We present our institutional experience, treatment dilemma, surgical management and outcomes in both the patients. Case 1: A 1½-year-old boy presented with intermittent appearance of the distal end of a ventricu-loperitoneal shunt tube at the anus, while attempting to pass stools. The mother said that the child had been treated for obstructive hydroceph-alus with a medium pressure Chhabra shunt, 9 months prior. On neurological examination, the child was active and alert; he had acquired appropriate milestones for his age and had no meningeal signs. The shunt chamber seemed to be functioning well. He was investigated with a shunt series X-ray, which revealed the distal end of the shunt tube traversing the entire lumen of the large intestine and ending in the sigmoid colon (figure 1A). A CT scan (brain CT) was ordered, which showed a significant Figure 1 (A) Shunt series X-ray showing the distal end of the ventriculoperitoneal shunt traversing the caecum and the ascending colon into the transverse colon with the tip at the rectum. (B) Postoperative shunt series X-ray showing the revised distal end within the peritoneal cavity. Hegde A, et al. BMJ Case Rep 2016.
Journal of Medical Case Reports, 2012
Introduction: Aqueductal stenosis may be caused by a number of etiologies including congenital stenosis, tumor, inflammation, and, very rarely, vascular malformation. However, aqueductal stenosis caused by a developmental venous anomaly presenting as congenital hydrocephalus is even more rare, and, to the best of our knowledge, has not yet been reported in the literature. In this study, we review the literature and report the first case of congenital hydrocephalus associated with aqueductal stenosis from a developmental venous anomaly. Case presentation: The patient is a three-day-old, African-American baby girl with a prenatal diagnosis of hydrocephalus. She presented with a full fontanelle, splayed sutures, and macrocephaly. Postnatal magnetic resonance imaging showed triventricular hydrocephalus, suggesting aqueductal stenosis. Examination of the T1weighted sagittal magnetic resonance imaging enhanced with gadolinium revealed a developmental venous anomaly passing through the orifice of the aqueduct. We treated the patient with a ventriculoperitoneal shunt. Conclusions: Ten cases of aqueductal stenosis due to venous lesions have been reported and, although these venous angiomas and developmental venous anomalies are usually considered congenital lesions, all 10 cases became symptomatic as older children and adults. Our case is the first in which aqueductal stenosis caused by a developmental venous anomaly presents as congenital hydrocephalus. We hope adding to the literature will improve understanding of this very uncommon cause of hydrocephalus and, therefore, will aid in treatment.
Hydrodynamics in vein of Galen malformations
Childs Nervous System, 1992
Forty-three patients with vein of Galen aneurysmal malformations (VGAM) referred to us for endovascular treatment between 1985 and 1990 and 335 additional cases published in the literature were reviewed with particular attention to the presence of ventricular enlargement and outcome after shunting. Hydrocephalus was the second most frequent symptom (46.8%); it is more frequent in infants (73%) than in children, adults (30%) or neonates (15%). Of the patients reported in the literature, 17.9% had undergone shunting. Within the shunted population there was an overall morbidity of 41% and a mortality of 10% (especially in the infant group). In our series 17 patients (39.5%) were shunted and a significant difference in the clinical outcome was noted between the shunted and the nonshunted group. Of the nonshunted patients, 66.6% were free of any neurological deficit or mental retardation and fewer than 5% presented with significant mental retardation. On the other hand, only 33.3% of the shunted patients had a favorable outcome and more than 15% developed significant mental retardation. Among the various causes of hydrocephalus in patients with VGAMs, such as obstruction of the aqueduct, subarachnoid hemorrhage, or ex vacuo hydrocephalus, high venous pressure may be of particular importance. In this article a physiopathological interpretation of the hydrodynamics in VGAMs is developed and a speculative explanation for CSF disorders related to ventricular shunting proposed. Treatment of hydrocephalus in VGAMs can be achieved through obliteration of the malformation or at least diminishing the venous pressure; surgical ventricular shunting does not have to be the first treatment of hydrodynamic disorders associated with VGAMs, especially in infants.
Bilateral Foraminal Stenosis in Patient with Dandy-Walker Malformation : Hiding Pathology
The Egyptian Journal of Hospital Medicine, 2018
Background: Etiologies of hydrocephalus are many and sometimes it can be attributed to be multifactorial in origin. Presence of known cause of hydrocephalus like fourth ventricular pathology may mask foramenal stenosis. Diagnosis of unilateral and bilateral stenosis of foramen of Monro should be included in the treatment plan in hydrocephalus A case report of a 9 months-old boy presented with delayed milestones of development and an increase in head circumference. The patient has been diagnosed as hydrocephalus and associated Dandy-Walker malformation. Ventriculoperitoneal shunt was inserted successfully but drained only one lateral ventricle. The association of isolated lateral ventricle with Dandy-Walker malformation is due to stenosis of foramen of Monro. Our case report is unique due to association between foramen occlusion and Dandy-Walker malformation.