The Management of Hydrocephalus in Midline Posterior Fossa Cystic Collections: Surgical Outcome From a Retrospective Single-Center Case Series of 54 Consecutive Pediatric Patients.

BACKGROUND: Hydrocephalus frequently occurs with midline posterior fossa cystic collections. The classification of this heterogeneous group of developmental anomalies, including Dandy-Walker malformation, persisting Blake's pouch, retrocerebellar arachnoid cysts, and mega cisterna magna, is subject of debate. The absence of diagnostic criteria is confusing regarding the ideal management of PFCC-related hydrocephalus. OBJECTIVE: To decipher the surgical strategy for the treatment of children with PFCC-related hydrocephalus through a retrospective analysis of the surgical outcome driven by their clinical and radiological presentation. METHODS: This study enrolled patients operated of symptomatic PFCC-related hydrocephalus. Clinical and MRI features were examined, as well as the surgical outcome. Unbiased subgroup classification of the patients was performed with multiple component analysis as a function of imaging characteristics and hierarchical clustering on principal component. Outcome was assessed with binomial logistic regression and Kaplan-Meier analysis. RESULTS: Fifty-four patients were included between 2007 and 2021. Multiple component analysis suggested that cerebellar and vermian hypoplasia, vermian rotation, basal-tentorial angle, and fastigial angle were strongly correlated. Hierarchical clustering and the distribution of the patients in the bidimensional plot showed the clear segregation of 3 major clusters, which correlated with the radiological diagnosis ( P < .01). Binomial logistic regression and survival analysis showed that endoscopic third ventriculostomy was an effective treatment for patients with persisting Blake's pouch, while failing to control hydrocephalus in most of patients with Dandy-Walker malformation. CONCLUSION: Preoperative MRI in patients with PFCC-related hydrocephalus is essential to better define the diagnosis. The choice of treatment strategy notably relies on correct radiological diagnosis.

Endoscopic ultrasonic aspiration of posterior fossa abscess in Dandy-Walker malformation: case report.

BACKGROUND: Dandy-Walker malformation (DWM) is a posterior fossa malformation characterized by a huge posterior fossa cyst in communication with the fourth ventricle. Hydrocephalus is associated with more than 80% of cases and is usually treated by shunting. Despite infection being a common complication of the shunt, abscess formation within the cyst was reported only once. CASE REPORT: A neonate affected by DWM developed a posterior fossa abscess following a shunt infection. The purulent collection was refractory to standard treatment (antibiotics and burr hole drainage); therefore, an endoscopic approach was performed in order to remove the purulent collection under direct vision. This material was aspirated with the help of an endoscopic ultrasonic aspirator. The outcome was favorable, with a resolution of infection and re-implantation of the ventriculo-peritoneal shunt. Surprisingly, post-operative radiological examination showed substantial modification of the anatomy of the posterior fossa with disappearing of the Dandy-Walker cyst. To the best of our knowledge, this is the first documented report of a true Dandy-Walker malformation that modified its anatomical appearance over time. DISCUSSION AND CONCLUSION: Endoscopic aspiration of intracranial purulent collection should be considered a valid option to manage complicated cases. An endoscopic ultrasonic aspirator may make the procedure more effective and faster.

Lateral ventricle pleomorphic xanthoastrocytoma concurrent with Dandy-Walker complex: A case report.

INTRODUCTION: Dandy-Walker complex and pleomorphic xanthoastrocytomas are both rare disease entities that typically manifest early in life and are associated with congenital etiological factors. Dandy-Walker complex is a cerebellar malformation associated with a series of anatomical changes. The disease onset is usually at birth or during infancy. Late onset in adulthood is uncommon. Pleomorphic xanthoastrocytoma is a rare WHO grade II astrocytic tumor affecting mainly young adults. Concomitant occurrence of Dandy-Walker complex and pleomorphic xanthoastrocytoma has not been previously reported. PATIENT CONCERNS AND DIAGNOSIS: A 30-year-old woman with a previous history of unconfirmed resected lateral ventricle meningioma presented with severe headache for 1 day. Imaging examination revealed a mass in the right lateral ventricle with heterogeneous signal patterns, changes in the posterior fossa corresponding to a Dandy-Walker variant, and mild hydrocephalus. INTERVENTIONS AND OUTCOMES: Surgical complete resection of the mass was achieved. postoperative histopathological examination confirmed WHO grade II pleomorphic xanthoastrocytoma. Three years postsurgery, ventriculoperitoneal shunt was performed due to worsening of hydrocephalus. The patient has since remained symptom-free. CONCLUSION: This is the first report of concomitant occurrence of Dandy-Walker complex and pleomorphic xanthoastrocytoma. The association of neurological congenital malformation with intracranial neoplasms may be multifactorial, with underlying role of genetic mutations or chromosome alterations.

Occipital encephalocele associated with Dandy-Walker malformation: a case-based review.

INTRODUCTION: Occipital encephalocele is a brain malformation that has been remotely associated with Dandy-Walker; only case reports and very small series have been published so far; therefore, their behavior and management are still under investigation. The goal of the present case-based review is to provide a summary of the state of the art in this association. METHODS AND RESULTS: The pertinent literature has been reviewed, and an exemplary case has been reported (an 11-month-old female with Dandy-Walker malformation and occipital encephalocele). So far, 33 cases have been described, with a mean age at surgery of 5, 1 day). The majority of the cases tend to present with hydrocephalus. There are no specific surgery approaches or global consensus about this association. The management possibly relies on surgery with shunt or encephalocele excision but without a dedicated protocol yet. CONCLUSIONS: The clinical research on occipital encephalocele in association with Dandy-Walker malformation is just at the beginning. New targets and wide-ranging clinical trials are needed to get an optimal management protocol.

Management and Outcome of a Neurologically Intact Infant with Pierre Robin Sequence and Dandy-Walker Variant Diagnosed with Large Hemispheric Brain Abscess ­ Case Report

Pierre Robin sequence (PRS) is a condition consisting of three essential components: micrognathia or retrognathia, cleft palate, and glossoptosis. It can be part of multiple congenital anomalies. We present the case and outcome of a 3-month-old clinically stable patient who has PRS with Dandy-Walker variant ­ which is a rare presentation in the literature ­ with a large right hemispheric brain abscess, treated with multiple minimally-invasive surgical drainage procedures with adjuvant antibiotics.

Shunt Valve Rupture in Ventriculoperitoneal Shunt Failure.

BACKGROUND: Shunt complications are common despite advances in surgical techniques and shunting technology. Proximal and/or distal catheter malfunctions are detected in pediatric and adult patients. However, valve dysfunction is rare in such cases. CASE DESCRIPTION: A 24-year-old woman presented with a history of ventriculostomy and ventriculoperitoneal shunt (VPS) secondary to hydrocephalus concomitant with Dandy-Walker syndrome. She has had undulant headache and vision loss episodes in both eyes for 15 days. Her VPS valve was normal when manually checked, and the VPS was observed as intact on x-ray and computed tomography scan. She had high-grade papilledema in both eyes with an optical coherence tomography scan value of 55/99. Lumbar puncture was performed. Cerebrospinal fluid opening pressure was 560 mm H2O under sedation. VPS exploration surgery was performed. There was a tiny defect over the shunt valve from where clear cerebrospinal fluid was leaking. We revised the old VPS valve with a new valve of 1.5 regular pressure. Her vision improved shortly after the surgery. CONCLUSIONS: This case is a very rare example of shunt valve dysfunction that required further investigation and a new valve replacement even though the preoperative imaging was normal.

An incidental finding of Dandy-Walker malformation.

ABSTRACT: Dandy-Walker malformation is a rare congenital anomaly affecting the posterior fossa, occurring in one in 30,000 births. Its hallmark characteristics include hypoplasia of the vermis, dilation of the fourth ventricle, and an enlarged posterior fossa. This case study describes a finding of Dandy-Walker malformation during a workup of encephalopathy in a patient on veno-venous extracorporeal membrane oxygenation for acute respiratory distress syndrome.

Dandy-Walker syndrome associated with syringomyelia in an adult: a case report and literature review.

Dandy-Walker syndrome associated with syringomyelia is a rare condition, with few reports of adult cases. We describe an adult case of Dandy-Walker syndrome with concomitant syringomyelia. A 33-year-old man presented with a 3-month history of walking instability, numbness in the hands, memory deterioration, and urinary incontinence. A physical examination showed a positive Romberg sign. Brain computed tomography and magnetic resonance imaging showed hydrocephalus, a cyst in the posterior fossa, absence of the cerebellar vermis, hypoplasia of the corpus callosum and cerebella, and syringomyelia. All of these symptoms were consistent with the diagnosis of Dandy-Walker syndrome. Surgery involving arachnoid adhesiolysis and endoscopic third ventriculostomy was performed. At the 6-month follow-up, the symptoms were completely relieved. Magnetic resonance imaging showed that syringomyelia was greatly reduced and the hydrocephalus remained unchanged. Dandy-Walker syndrome with concomitant syringomyelia in adults is exceedingly rare. Early diagnosis and appropriate surgical treatment of this condition should be highlighted. Combined arachnoid adhesiolysis and endoscopic third ventriculostomy may be an effective approach.

Shunt Surgery for Neurocutaneous Melanosis with Hydrocephalus: Case Report and Review of the Literature.

BACKGROUND: Neurocutaneous melanosis is a rare phakomatosis characterized by large or multiple pigmented nevi and melanosis of the leptomeninges. It is often complicated by hydrocephalus due to melanotic deposits interfering with cerebrospinal fluid reabsorption in the basal cisterns or causing foraminal or aqueductal obstruction. In 10% of cases, it will be associated with the Dandy-Walker complex. CASE DESCRIPTION: We present the case of a 6-year-old girl with multiple congenital hairy nevi presenting with generalized tonic-clonic seizures, headache, and vomiting. Neuroimaging showed a communicating hydrocephalus associated with the Dandy-Walker variant, melanotic deposits in the amygdalae, thalami, and cortical sulci, and abnormal leptomeningeal enhancement. After undergoing ventriculoperitoneal shunt insertion, the symptoms of increased intracranial pressure abated. However, she again showed deterioration 1 month postoperatively due to progressive leptomeningeal spread suspicious for malignant degeneration. CONCLUSION: The results of the present case and the findings from a review of related data suggest that shunt insertion (ventriculoperitoneal or cystoperitoneal) is an effective palliative measure for patients with neurocutaneous melanosis with associated hydrocephalus. Despite treatment, however, the prognosis of these patients remains poor owing to malignant progression and leptomeningeal spread of lesions, in particular, in cases associated with the Dandy-Walker complex.

Intradiploic Cerebrospinal Fluid Cyst Following Occipital Encephalocele Surgery in Patient with Dandy-Walker Malformation.

Intradiploic cerebrospinal fluid cysts are rare entities that have been reported to occur following trauma and surgery. We present a case of a 53-year-old female patient with an incidental intradipoloic cerebrospinal fluid cyst in communication with a Dandy-Walker malformation, likely related to a remote history of childhood surgery for occipital encephalocele.

Dandy-Walker malformation and syringomyelia: a rare association.

PURPOSE: Dandy-Walker malformation is a rare condition due to imperforation of the Blake's pouch during intrauterine brain development, usually leading to early severe hydrocephalus. The association with holocord syringomyelia is rare, and from the Gardner's first report in 1957, only 23 cases have been described, mostly from autopsy series and pre-MRI period. Besides a worsening of clinical picture, its occurrence generates some concern about the best surgical treatment that varies widely among the literature reports. METHODS: An 11-year-old girl with Dandy-Walker malformation presented with a holocord syrinx due to the herniation of the lower pole of the posterior fossa cyst through the foramen magnum. RESULTS: After an unsuccessful shunt revision, she underwent a cystoperitoneal shunt with regression of the syrinx and of neurological symptoms at the 12-month follow-up. CONCLUSIONS: Previous literature about pathogenesis, treatment, and follow-up is discussed and summarized.

The outcome of cochlear implantation among children with genetic syndromes.

OBJECTIVE: To assess the outcome and efficacy of cochlear implantation in children with genetic syndromes. METHOD: Study design: case-control study. SETTING: A cochlear implantation tertiary referral center. PATIENTS: All pediatric cochlear implantation recipients with Waardenburg syndrome, Usher syndrome, Dandy-Walker syndrome, or albinism. A control group was appropriately matched to the syndromic group with regard to age at implantation and duration of device use. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Subjects' auditory abilities, speech intelligibility, and pure tone thresholds were compared between the syndromic and non-syndromic group. RESULTS: A total of 25 subjects (13 syndromic and 12 non-syndromic) participated in the study. Neither auditory ability nor speech intelligibility scores differed significantly by group. The final PTA of both the groups showed normal-to-mild hearing loss: 26 dB HL in the syndromic group and 23 dB HL for the control group. CONCLUSIONS: Cochlear implant recipients with genetic syndromes achieved similar levels auditory perception and speech intelligibility as their peers with a genetic syndrome. The presence of any of the genetic syndromes described herein should not be a contraindication to cochlear implant provision, as it would have a positive impact on the patients' sensory perception and lifestyle.

Congenital Melanocytic Nevus Syndrome: A Case Series. / Síndrome del nevus melanocítico congénito. Serie de casos.

Congenital melanocytic nevus syndrome (CMNS) is the result of an abnormal proliferation of melanocytes in the skin and central nervous system caused by progenitor-cell mutations during embryonic development. Mutations in the NRAS gene have been detected in many of these cells. We present 5 cases of giant congenital melanocytic nevus, 3 of them associated with CMNS; NRAS gene mutation was studied in these 3 patients. Until a few years ago, surgery was the treatment of choice, but the results have proved unsatisfactory because aggressive interventions do not improve cosmetic appearance and only minimally reduce the risk of malignant change. In 2013, trametinib was approved for use in advanced melanoma associated with NRAS mutations. This drug, which acts on the intracellular RAS/RAF/MEK/pERK/MAPK cascade, could be useful in pediatric patients with CMNS. A better understanding of this disease will facilitate the development of new strategies.

Syringomyelia caused by an arachnoid web in a patient with shunted Dandy-Walker malformation.

INTRODUCTION: Dandy-Walker malformation (DWM) is a congenital brain anomaly characterized by dysgenesis of the cerebellar vermis and the presence of a posterior fossa cyst. The association of syringomyelia with DWM is extremely rare. CASE REPORT: A 10-year-old patient who was diagnosed with DWM in infancy presented with progressive scoliosis and fecal incontinence. He had been treated with cystoventriculoperitoneal shunting with a Y-connection during infancy, which was followed by a revision 6 years later. During the revision surgery, intraventricular bleeding occurred and was managed conservatively. Imaging studies for the current visit revealed syringomyelia along the cervicothoracic spinal cord and a membranous structure around the cervicomedullary junction. Phase-contrast cine magnetic resonance imaging (MRI) revealed disturbed cerebrospinal fluid (CSF) flow across the membrane. We excised the arachnoid web that was tethering the brainstem and blocking CSF flow. Postoperatively, the patient experienced symptom relief, and the follow-up imaging study demonstrated a dramatic decrease in the size of the syringomyelia. DISCUSSION: We suggest that syrinx formation in this patient was possibly caused by disturbed CSF flow and tethering of the brainstem. CONCLUSION: We experienced an unusual case of DWM with syringomyelia which was caused by an arachnoid web blocking CSF flow and tethering the brainstem. The arachnoid web seems to be formed by previous bleeding which occurred at the time of shunt revision. After excision of the arachnoid web, the patient showed good outcome.

Challenges and outcome of cranial neuroendoscopic surgery in a resource constrained developing African country.

BACKGROUND: Cranial neuroendoscopy has been safely applied to the surgical treatment of different lesions of the brain in our center since its introduction in September 2009. This article summarizes our experience with neuroendoscopy, highlighting the salient challenges and outcome. METHODS: A single institution, retrospective analysis of prospectively acquired cases over a 2.5-year period (September 2010 to February 2013). Challenges experienced during the course of patient care as well as complications and outcomes were recorded and analyzed using SPSS (SPSS Inc. Chicago IL, USA) version 17. Tests of statistical significance were set at 95% level. RESULTS: Of the 291 cranial procedures performed during the study period, 37 (12.7%) were neuroendoscopic interventions. Patients were between the ages of 0.25 years and 25 years with a mean of 5.7 ± 1.5 years (95% confidence interval (CI)). Aqueductal stenosis was the most common indication for endoscopic intervention in 22 (59.5%) patients. Endoscopic third ventriculostomy was the most commonly performed neuroendoscopic procedure in 21 patients (56.7%). Major challenges experienced were patient dependent in 28 ± 1.0 patients (95% CL), learning curve related in 21 ± 0.4 patients, and poor endoscopy support infrastructure in 15 ± 0.5 patients. Complications were significantly more common in the first 6 months of neuroendoscopy (χ2= 7.57, df = 1, P< 0.05). Overall, 30 (81.1%) patients in our study experienced a positive outcome. The permanent morbidity and mortality rates in our series were 2.7% and 8.5%, respectively. CONCLUSION: Highlighted are the myriad obstacles which interface the successful set up of neuroendoscopy service especially in resource-constrained settings. Endoscopic procedures become safer with experience and complications reduce significantly after a steep learning curve.

[Intravenous Sedation and Repeated "the Same Day General Anesthesia" for a School-age Boy with Dandy-Walker Syndrome and Dentinogenesis].

Dandy-Walker syndrome (DWS) is characterized by perfect or partial defect of the cerebellum vermis and cystic dilatation of the posterior fossa communicating with the fourth ventricle. Common clinical signs are mental retardation, cerebellar ataxia, and those of increased intracranial pressure (ICP). Associated congenital anomalies are craniofacial, cardiac, renal, and skeletal abnormalities. We experienced a case of intravenous sedation and six times of "the same day" general anesthesia for a school-aged boy (10-13 years old) with DWS and hypodentinogenesis. The patient underwent an examination and dental treatments. We had to pay attention to airway management tracheal tube selection and control of ICP. In addition, we should prevent tooth injuries through mishaps during tracheal intubations, since all-tooth-hypoplasia with fragile dental crowns was strongly suggested in this case. Detailed postoperative care is also required for general anesthesia afflicted with DWS.

Perioperative management of a patient with Dandy Walker malformation with tetralogy of Fallot undergoing total correction and fresh homologous pericardial pulmonary valve conduit implantation: Report of a rare case.

Perioperative management of a patient with Dandy-Walker malformation (DWM) with tetralogy of Fallot (TOF), patent ductus arteriosus, and pulmonary artery stenosis is a great challenge to the anesthesiologist. Anesthetic management in such patients can trigger tet spells that might rapidly increase intracranial pressure (ICP), conning and even death. The increase in ICP can precipitate tet spells and further brain hypoxia. To avoid an increase in ICP during TOF corrective surgery ventriculo-peritoneal (VP) shunt should be performed before cardiac surgery. We present the first case report of a 11-month-old male baby afflicted with DWM and TOF who underwent successful TOF total corrective surgery and fresh autologous pericardial pulmonary valve conduit implantation under cardiopulmonary bypass after 1 week of VP shunt insertion.

Dandy-Walker Malformation: A Clinical and Surgical Outcome Analysis.

OBJECTIVE: To determine the clinical presentations, complications and mortality in patients with Dandy-Walker Malformation (DWM) after surgery i.e., shunt with y-connector. STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: Neurosurgery Ward, JPMC, Karachi, from January 2009 to December 2013. METHODOLOGY: Cases of DWM, with associated hydrocephalus, further confirmed on CT scan of brain, were admitted through OPD. Those who were previously operated, those with other associated co-morbid or anomalies were excluded from this study. Combined drainage of the ventricular system and posterior fossa cyst, via dual shunt i.e. cystoperitoneal and ventriculoperitoneal shunt with y-connector was performed in all patients. Complications and mortality after surgical intervention in these patients were noted upto one month after surgery. The data analysis for descriptive statistics was done on SPSS version 20. RESULTS: In this study of 70 patients, majority of the patients were female aged between 1 - 2 years. Hydrocephalus was the predominant symptom as being present in all patients, followed by cerebellar signs in 60 (86%), and other in 5 (7.14%) patients. Complications of surgery were infection and shunt fracture dislocation in 7 (10%) each, malpositioning and shunt blockage in 6 (8.5%) each within one month of surgery, intracranial haemorrhage in 5 (7.14%) patients. Only one patient (1.42%) expired after surgical intervention. CONCLUSION: In DWM, the commonest presentation is that of hydrocephalus. Shunt malfunction and infection are the commonest complication after shunting. Dual shunt with y-connector has the lowest mortality when compared with other methods for treatment of shunt with y-connector.

Dandy-Walker syndrome with severe velopharyngeal dysfunction: a contraindication for Le Fort I surgery?

Dandy-Walker syndrome is a rare congenital brain deformation. Most symptoms are related with fourth ventricle and skull base malformations. Quite often, symptoms develop from infancy or progress rapidly. Cerebellar dysfunction, lack of muscle coordination, and skull deformities involving eye movement might be present. There are several Dandy-Walker syndrome complex types. We present a 23-year-old patient who had a severe dentofacial deformity with mandibular prognathism and extremely undeveloped maxillary bone resulting in palatopharyngeal and velopharyngeal dysfunction with complete lack of soft palate function resulting in increased speech tone and volume. Performing Le Fort I osteotomy in this case is greatly controversial and might result in even greater loss of function or even its total lack. Velopharyngeal complex is very important, and every surgeon should consider its value while planning Le Fort I osteotomies.