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

Bagnas, Marjorie Anne C.; Rosario-Blasco, Krystle Anne R. Del; Diestro, Jose Danilo Bengzon; Khu, Kathleen Joy

doi:10.1016/j.wneu.2018.09.002

Cited by 10 publications

(5 citation statements)

References 40 publications

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“…According to the classical model of CSF dynamics, PHH and PIH result from obstruction of intraventricular CSF flow and/or dysfunction of extraventricular arachnoid granulations, which causes a decrease in CSF reabsorption10, 16. This model is supported by some PHH case series that reported occlusion of the fourth ventricular outflow tracts by fibrous thickening of the leptomeninges, known as "tetraventricular" PHH 58,59 . Evidence from other PHH case series suggests that blood and its breakdown products acutely obstruct narrow CSF passages such as the cerebral aqueduct 60,61 .…”

Section: The Classical Model Of Pathogenesismentioning

confidence: 96%

Inflammation in acquired hydrocephalus: pathogenic mechanisms and therapeutic targets

et al. 2020

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Publisher's note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Review criteriaWe searched PubMed for articles in all year ranges with multiple combinations of search terms including, "post-haemorrhagic hydrocephalus", "post-infectious hydrocephalus", "worldwide", "epidemiology", "ETV/CPC", "VP Shunt", "NKCC1", "SPAK", "Toll-like receptors" "inflammation", "obstruction", "impaired reabsorption", "CSF hypersecretion", "cerebrospinal fluid". There were no language exclusions and articles chosen were based on relevance to topics covered in this Review. Peer review information Nature Reviews Neurology thanks [Referee# 1 name], [Referee#2 name] and the other, anonymous, reviewer(s) for their contribution to the peer review of this work.

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Section: The Classical Model Of Pathogenesismentioning

confidence: 96%

Inflammation in acquired hydrocephalus: pathogenic mechanisms and therapeutic targets

et al. 2020

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“…It is thought that melanotic infiltration interferes with the capacity of primitive meningeal cells to induce the normal migration of neurons, deposition of extracellular matrix, and development of normal CSF pathways, leading to posterior fossa cyst formation and vermian dysgenesis. Alternatively, Omar et al [ 19 ] suggested that melanosis interferes with the ectodermal-mesodermal interaction, causing an abnormal formation of the fourth ventricle and cerebellum.…”

Section: Discussionmentioning

confidence: 99%

Neurocutaneous melanosis with an intracranial cystic-solid meningeal melanoma in an adult: A case report and review of literature

Liu¹,

Wang²,

Liu³

et al. 2022

WJCC

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BACKGROUND Neurocutaneous melanosis (NCM) is a rare congenital, nonhereditary neurocutaneous syndrome that mainly occurs in children; adult NCM is very rare. Due to its rarity, the clinical features and treatment strategies for NCM remain unclear. The purpose of this study was to explore the clinical features, diagnosis, treatment and prognosis of NCM in adults. Most intracranial meningeal melanomas are solid masses, and cystic-solid malignant melanomas are very rare. Due to the lack of data, the cause of cystic changes and the effect on prognosis are unknown. CASE SUMMARY A 41-year-old woman was admitted to the hospital with intermittent headache for 1 mo. Magnetic resonance imaging (MRI) showed a 4.7 cm × 3.6 cm cystic-solid mass in the left temporal lobe with peritumoral edema. The entire mass was removed, and postoperative pathology indicated malignant melanoma. CONCLUSION MRI is the first-choice imaging approach for diagnosing central nervous system diseases in NCM patients, although cerebrospinal fluid may also be used. At present, there is no optimal treatment plan; gross total resection combined with BRAF inhibitors and MEK inhibitors might be the most beneficial treatment.

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“…Approximately 49 patients with NCM and hydrocephalus have been reported to undergo CSF shunting in the literature, with the most common shunt of choice being the VPS. 45 Patients required multiple shunt revisions or placements to control progression of hydrocephalus. Seventy percent of NCM patients undergoing CSF shunting died within 3 years of shunt insertion due to the malignant progression of melanotic lesions and leptomeningeal spread.…”

Section: Dandy-walker Complexmentioning

confidence: 99%

“…Seventy percent of NCM patients undergoing CSF shunting died within 3 years of shunt insertion due to the malignant progression of melanotic lesions and leptomeningeal spread. 45 Peritoneal seeding may occur in approximately 21% of cases with "metastatic" dissemination to the peritoneal surface through the VPS. 19,45,46 For this reason, shunt insertion with a filter can prevent potential seeding into the abdominal space.…”

Section: Dandy-walker Complexmentioning

confidence: 99%

“…45 Peritoneal seeding may occur in approximately 21% of cases with "metastatic" dissemination to the peritoneal surface through the VPS. 19,45,46 For this reason, shunt insertion with a filter can prevent potential seeding into the abdominal space. 47 In a case reported by Craver et al, a 19-month-old girl with NCM and Dandy-Walker malformation presented with severe respiratory distress requiring multiple thoracenteses and pleuroperitoneal shunts.…”

Section: Dandy-walker Complexmentioning

confidence: 99%

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Neurosurgical management of patients with neurocutaneous melanosis: a systematic review

Rahman

Majmundar

Ghani

et al. 2022

Neurosurgical Focus

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OBJECTIVE Neurocutaneous melanocytosis (NCM), also referred to as neurocutaneous melanosis, is a rare neurocutaneous disorder characterized by excess melanocytic proliferation in the skin, leptomeninges, and cranial parenchyma. NCM most often presents in pediatric patients within the first 2 years of life and is associated with high mortality due to proliferation of melanocytes in the brain. Prognosis is poor, as patients typically die within 3 years of symptom onset. Due to the rarity of NCM, there are no specific guidelines for management. The aims of this systematic review were to investigate approaches toward diagnosis and examine modern neurosurgical management of NCM. METHODS A systematic review was performed using the PubMed database between April and December 2021 to identify relevant articles using PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. Search criteria were created and checked independently among the authors. Inclusion criteria specified unique studies and case reports of NCM patients in which relevant neurosurgical management was considered and/or applied. Exclusion criteria included studies that did not report associated neurological diagnoses and neuroimaging findings, clinical reports without novel observations, and those unavailable in the English language. All articles that met the study inclusion criteria were included and analyzed. RESULTS A total of 26 extracted articles met inclusion criteria and were used for quantitative analysis, yielding a cumulative of 74 patients with NCM. These included 21 case reports, 1 case series, 2 retrospective cohort studies, 1 prospective cohort study, and 1 review. The mean patient age was 16.66 years (range 0.25–67 years), and most were male (76%). Seizures were the most frequently reported symptom (55%, 41/74 cases). Neurological diagnoses associated with NCM included epilepsy (45%, 33/74 cases), hydrocephalus (24%, 18/74 cases), Dandy-Walker malformation (24%, 18/74 cases), and primary CNS melanocytic tumors (23%, 17/74 cases). The most common surgical technique was CSF shunting (43%, 24/56 operations), with tethered cord release (4%, 2/56 operations) being the least frequently performed. CONCLUSIONS Current management of NCM includes CSF shunting to reduce intracranial pressure, surgery, chemotherapy, radiotherapy, immunotherapy, and palliative care. Neurosurgical intervention can aid in the diagnosis of NCM through tissue biopsy and resection of lesions with surgical decompression. Further evidence is required to establish the clinical outcomes of this rare entity and to describe the diverse spectrum of intracranial and intraspinal abnormalities present.

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

Cited by 10 publications

References 40 publications

Inflammation in acquired hydrocephalus: pathogenic mechanisms and therapeutic targets

Inflammation in acquired hydrocephalus: pathogenic mechanisms and therapeutic targets

Neurocutaneous melanosis with an intracranial cystic-solid meningeal melanoma in an adult: A case report and review of literature

Neurosurgical management of patients with neurocutaneous melanosis: a systematic review

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