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Journal of Clinical Neuromuscular... Mar 2021Spinal muscular atrophy (SMA) is a common rare neuromuscular disease responsible for very high mortality during infancy and high morbidity during childhood and...
OBJECTIVE
Spinal muscular atrophy (SMA) is a common rare neuromuscular disease responsible for very high mortality during infancy and high morbidity during childhood and adolescence. It is caused by autosomal recessive mutations in the survival motor neuron gene. In 2016, the Food and Drug Administration approved the first disease modifying therapy for use in all patients of any age. Nusinersen is an antisense oligonucleotide that showed dramatic benefits with achievement of motor milestones in infants and improved gross motor function in children.
METHODS
This was a retrospective chart review of all SMA patients seen at a single site between 2016 and 2020 for treatment with nusinersen.
RESULTS
We report 8 patients who underwent placement of an Ommaya reservoir and lumbosacral catheter for drug delivery. Complications included infection and revisions due to catheter separation. One patient required fluoroscopy for injections because of location of port site.
CONCLUSION
We conclude that placement of an Ommaya port is a viable option for patients who have challenges for access to intrathecal space. Practical innovations have the potential to control administration costs, achieve therapeutic value, and promote patient safety.
Topics: Adolescent; Catheters, Indwelling; Child; Female; Humans; Lumbosacral Region; Male; Oligonucleotides; Retrospective Studies; Spinal Muscular Atrophies of Childhood
PubMed: 33595996
DOI: 10.1097/CND.0000000000000333 -
Journal of Visualized Experiments : JoVE Jan 2021Leptomeningeal disease (LMD) is an uncommon type of central nervous system (CNS) metastasis to the cerebral spinal fluid (CSF). The most common cancers that cause LMD...
Leptomeningeal disease (LMD) is an uncommon type of central nervous system (CNS) metastasis to the cerebral spinal fluid (CSF). The most common cancers that cause LMD are breast and lung cancers and melanoma. Patients diagnosed with LMD have a very poor prognosis and generally survive for only a few weeks or months. One possible reason for the lack of efficacy of systemic therapy against LMD is the failure to achieve therapeutically effective concentrations of drug in the CSF because of an intact and relatively impermeable blood-brain barrier (BBB) or blood-CSF barrier across the choroid plexus. Therefore, directly administering drugs intrathecally or intraventricularly may overcome these barriers. This group has developed a model that allows for the effective delivery of therapeutics (i.e., drugs, antibodies, and cellular therapies) chronically and the repeated sampling of CSF to determine drug concentrations and target modulation in the CSF (when the tumor microenvironment is targeted in mice). The model is the murine equivalent of a magnetic resonance imaging-compatible Ommaya reservoir, which is used clinically. This model, which is affixed to the skull, has been designated as the "Murine Ommaya." As a therapeutic proof of concept, human epidermal growth factor receptor 2 antibodies (clone 7.16.4) were delivered into the CSF via the Murine Ommaya to treat mice with LMD from human epidermal growth factor receptor 2-positive breast cancer. The Murine Ommaya increases the efficiency of drug delivery using a miniature access port and prevents the wastage of excess drug; it does not interfere with CSF sampling for molecular and immunological studies. The Murine Ommaya is useful for testing novel therapeutics in experimental models of LMD.
Topics: Animals; Breast Neoplasms; Central Nervous System Diseases; Drug Delivery Systems; Female; Heterografts; Injections, Intraventricular; Meningeal Neoplasms; Mice; Models, Biological; Neoplasm Metastasis; Neoplastic Cells, Circulating; Prognosis
PubMed: 33586709
DOI: 10.3791/62033 -
Child's Nervous System : ChNS :... Feb 2021To report our experience and management strategies during 10 years for 137 childhood craniopharyngiomas treated at a single institution.
PURPOSE
To report our experience and management strategies during 10 years for 137 childhood craniopharyngiomas treated at a single institution.
METHODS
Medical records of children with craniopharyngioma treated at Children's Cancer Hospital Egypt (CCHE-57357) from July 2007 to December 2017 were retrospectively reviewed. Beta-catenin as an immunohistochemical marker was assessed also in available specimens.
RESULTS
Our registry included 137 patients. Headache (n = 122), visual failure (n = 118), and hypothyroidism(n = 78) were the most common findings on presentation. Three management protocols were identified; 65 patients were primarily followed up after surgery, 71 patients had radiotherapy after surgery, and one patient underwent surgery for Ommaya insertion with intracystic interferon injection. Overall, gross total resection/near total resection was achieved in 48 cases (35.04%), subtotal resection was achieved in 58 patients (42.33%), 29 (21.16%) had biopsy and Ommaya reservoir, and two patients with calcified lesions had no operations. Fifty-four patients showed recurrence/progression of their lesions. Allover, 5-year progression-free survival (PFS) was 52.3%, while it was 34.49% and 72.25% for the follow-up group and the radiotherapy group, respectively. Beta-catenin mutations were positive in 61/95 patients; 5-year PFS for beta-catenin negative and positive cases was 65.5% and 39.4% respectively (p = 0.087). Mortality was reported in eight patients. Intraoperative endoscopy-assisted assessment was the cornerstone of tailored decision-making.
CONCLUSION
The concepts of conservative surgery and multimodal management should be applied to reach the perfect balance between the quality of life and the best tumor control rates. Beta-catenin mutations more than 5% are associated with statistically trending aggressive clinical behavior. The CCHE-57357 algorithm of individualized management protocol was presented.
Topics: Child; Craniopharyngioma; Egypt; Humans; Neoplasm Recurrence, Local; Pituitary Neoplasms; Quality of Life; Retrospective Studies; Treatment Outcome
PubMed: 32712862
DOI: 10.1007/s00381-020-04833-x -
Clinical and Translational Radiation... Jul 2020Large cystic craniopharyngioma management combining cyst puncture through an Ommaya reservoir with hypofractionated stereotactic radiotherapy was evaluated. The planning...
Dosimetric and volumetric outcomes of combining cyst puncture through an Ommaya reservoir with index-optimized hypofractionated stereotactic radiotherapy in the treatment of craniopharyngioma.
Large cystic craniopharyngioma management combining cyst puncture through an Ommaya reservoir with hypofractionated stereotactic radiotherapy was evaluated. The planning optimization was focused on the gradient and selectivity. Punctured and filled cyst treatment plans were compared with a retrospective analysis of volumetric and functional outcomes.
PubMed: 32490217
DOI: 10.1016/j.ctro.2020.05.003 -
Outcomes and Costs Following Ommaya Placement with Thrombocytopenia Among U.S. Patients with Cancer.World Neurosurgery Mar 2020Placement of Ommaya reservoirs for the administration of intrathecal chemotherapy may be complicated by comorbid thrombocytopenia among patients with hematologic or...
BACKGROUND
Placement of Ommaya reservoirs for the administration of intrathecal chemotherapy may be complicated by comorbid thrombocytopenia among patients with hematologic or leptomeningeal disease. Aggregated data on risks of Ommaya placement among thrombocytopenic patients are lacking. This study assesses complications, revision rates, and costs associated with Ommaya placement among patients with thrombocytopenia in a large population sample.
METHODS
Using a national administrative database, this retrospective study identifies a cohort of adult patients with cancer who underwent Ommaya placement between 2007 and 2016. Preoperative thrombocytopenia was defined as diagnosis of secondary thrombocytopenia, bleeding event, procedure to control bleeding, or platelet transfusion, within 30 days before index admission. Univariate and multivariate analyses were performed to assess costs, 30-day complications, readmissions, and revisions among patients with and without preoperative thrombocytopenia.
RESULTS
The analytic cohort included 1652 patients, of whom 29.3% met criteria for preoperative thrombocytopenia. In-hospital mortality rates were 7.7% among patients thrombocytopenia with versus 1.2% among patients without thrombocytopenia (P < 0.001). Preoperative thrombocytopenia was associated with 14.5 times greater hazard of intracranial hemorrhage within 30 days following Ommaya placement, occurring in 25.6% versus 2.0% of patients with and without thrombocytopenia, respectively (P < 0.014). Revision rates did not differ significantly between patients with and without thrombocytopenia. Thrombocytopenia was associated with longer length of stay (7.4 vs. 13.9 days, P < 0.001) and additional $10,000 per patient in costs of index hospitalization (P < 0.001).
CONCLUSIONS
This is the largest study to date documenting costs and complication rates of Ommaya placement in patients with and without thrombocytopenia.
Topics: Aged; Antineoplastic Agents; Catheters, Indwelling; Costs and Cost Analysis; Drug Delivery Systems; Female; Humans; Insurance Coverage; Insurance, Health; Male; Neoplasms; Retrospective Studies; Thrombocytopenia; Treatment Outcome; United States
PubMed: 31866457
DOI: 10.1016/j.wneu.2019.12.063 -
Neurology India 2020External ventricular drainage (EVD) is one of the commonest procedures in neurosurgical practice to manage acute hydrocephalus. We evaluated the infectious and...
BACKGROUND AND AIMS
External ventricular drainage (EVD) is one of the commonest procedures in neurosurgical practice to manage acute hydrocephalus. We evaluated the infectious and non-infectious complications associated with a modified technique for EVD using an Ommaya reservoir.
METHODS
Ommaya reservoir was placed in all patients who required EVD placement for CSF drainage. CSF drainage was achieved using a needle placed in a non-coring fashion percutaneously into the Ommaya reservoir to achieve CSF drainage externally. CSF was monitored for signs of infection regularly using CSF biochemistry and cultures. CSF infection was defined by a positive culture or a secondary infection in patients with already infected CSF.
RESULTS
59 patients required continuous CSF drainage during the study period from January 2014 to June 2017. 46 (77.96%) patients had non-infected CSF at time of starting drainage and 13 (22.03%) patients required external CSF drainage for primarily infected CSF. The study period had a total of 793 CSF drainage days (Range 3-64 days) with an average of 13.4 days per patient. The cumulative rate of new infection was 5.08%. No ventricular catheter blockage or dislodgement was seen in any of the patients.
CONCLUSIONS
External ventricular drainage through an Ommaya chamber is a safe and effective method and can be used to reduce the catheter related complications like infection, catheter blockage and dislodgement.
Topics: Acute Disease; Adolescent; Adult; Aged; Brain Neoplasms; Catheter-Related Infections; Catheters, Indwelling; Cerebral Intraventricular Hemorrhage; Cerebral Ventriculitis; Child; Child, Preschool; Drainage; Equipment and Supplies; Female; Humans; Hydrocephalus; Infant; Male; Middle Aged; Prosthesis Implantation; Scalp; Subarachnoid Hemorrhage; Ventriculostomy; Young Adult
PubMed: 32415024
DOI: 10.4103/0028-3886.284354 -
Journal of Clinical Neuroscience :... Feb 2020The efficacy of the endoscopic transcortical transventricular approach (ETTA) for craniopharyngioma in the third ventricle with hydrocephalus has been reported focusing...
Cyst fenestration and Ommaya reservoir placement in endoscopic transcortical transventricular approach for recurrent suprasellar cystic craniopharyngioma without ventriculomegaly.
The efficacy of the endoscopic transcortical transventricular approach (ETTA) for craniopharyngioma in the third ventricle with hydrocephalus has been reported focusing on its reduced invasiveness. On the other hand, suprasellar craniopharyngioma without ventriculomegaly is generally surgically managed by craniotomy or the endoscopic endonasal approach (EEA). Here, we report an elderly patient who received cyst fenestration and Ommaya reservoir placement in ETTA for recurrent suprasellar cystic craniopharyngioma without ventriculomegaly. The ETTA as a less invasive procedure is feasible in patients not only with intraventricular craniopharyngioma but also with suprasellar craniopharyngioma without hydrocephalus provided a navigational system is applied and the surgeon has ample experience with transcranial endoscopic procedures.
Topics: Aged; Brain Neoplasms; Central Nervous System Cysts; Craniopharyngioma; Craniotomy; Cysts; Drug Delivery Systems; Female; Humans; Hydrocephalus; Male; Neuroendoscopy; Nose; Otologic Surgical Procedures; Pituitary Neoplasms; Prostheses and Implants; Third Ventricle
PubMed: 31926662
DOI: 10.1016/j.jocn.2019.12.060 -
Child's Nervous System : ChNS :... Sep 2021A 13-year-old female patient was diagnosed with a tectal glioma (TG), a subgroup of astrocytoma that can result in obstructive hydrocephalus secondary to aqueductal... (Review)
Review
BACKGROUND
A 13-year-old female patient was diagnosed with a tectal glioma (TG), a subgroup of astrocytoma that can result in obstructive hydrocephalus secondary to aqueductal stenosis. Endoscopic third ventriculostomy (ETV) is used to treat this type of hydrocephalus with a good success rate. Our institution performs ETV and Ommaya reservoir (OR) placement in these cases. The OR allows measurement of intracranial pressure (ICP) and cerebrospinal fluid (CSF) access and a method for performing ventricular dye studies to evaluate third ventricular stoma (TVS) patency. In this case, a porencephalic cyst (PC) developed around the OR's ventricular catheter (OVC) two and a half months after surgery.
CONCLUSION
The PC is thought to have developed in association with TVS stoma closure and resolved after ETV revision.
Topics: Adolescent; Cerebrospinal Fluid Shunts; Cysts; Female; Humans; Hydrocephalus; Neuroendoscopy; Third Ventricle; Treatment Outcome; Ventriculostomy
PubMed: 33442758
DOI: 10.1007/s00381-021-05042-w -
International Medical Case Reports... 2022, a common component of the skin and mucosal microbiota of both immunocompetent and immunocompromised individuals, has become an emerging pathogen, colonizing indwelling...
, a common component of the skin and mucosal microbiota of both immunocompetent and immunocompromised individuals, has become an emerging pathogen, colonizing indwelling medical devices and causing infections at multiple sites. A 3-year-old boy with an Ommaya reservoir in the right ventricle and a medical history of grade 3 intraventricular hemorrhage, Hirschsprung disease, catheter-related methicillin-resistant bacteremia, and congenital central hypoventilation syndrome was hospitalized for Ommaya reservoir infection with . He was treated with ampicillin, to which the initial isolate was susceptible. may have acquired multiple-drug resistance during the antibiotic treatment due to biofilm production. The Ommaya reservoir was replaced by external ventricular drainage. Cultures of the removed Ommaya reservoir, and cerebrospinal fluid samples grew , which was susceptible to meropenem and vancomycin and resistant to other antibiotics. The antibiotic was switched to vancomycin to treat this new multidrug-resistant strain. After 8 days of vancomycin treatment, the cerebrospinal fluid culture obtained by a lumbar puncture was negative for . In cases of device-associated infections caused by biofilm-producing bacteria, it is desirable to remove the device as soon as possible.
PubMed: 35535175
DOI: 10.2147/IMCRJ.S361505 -
Frontiers in Surgery 2022Therapy for large or deep cystic brain metastases is a troublesome procedure in clinical departments. Stereotactic cyst aspiration, combined with Gamma Knife...
BACKGROUND
Therapy for large or deep cystic brain metastases is a troublesome procedure in clinical departments. Stereotactic cyst aspiration, combined with Gamma Knife radiosurgery, can be an effective treatment for cystic brain metastases. However, there is still a possibility that a reaccumulation of cystic fluid may lead to poor efficacy or even reoperation.
CASE PRESENTATION
We present a case of a 67-year-old man who was diagnosed with lung cancer brain metastasis. The intracranial lesion seen on imaging appeared to be cystic and located deep inside the brain with associated limb dysfunction. The patient did not respond well to chemotherapy and underwent cyst aspiration with Ommaya reservoir implantation under neuronavigation. Repeated cystic fluid reaccumulation and exacerbation of symptoms occurred during treatment. We performed repeated aspiration via the Ommaya reservoir to control the symptoms and combined it with radiotherapy. During the follow-up period of 14 months, the intracranial tumor was effectively and satisfactorily controlled.
CONCLUSIONS
We highlight that Ommaya reservoir implantation during stereotactic cyst aspiration is necessary to prevent fluid reaccumulation, thereby avoiding the need for a second surgical procedure.
PubMed: 35651693
DOI: 10.3389/fsurg.2022.901674