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1.
Nat Rev Dis Primers ; 10(1): 35, 2024 May 16.
Article in English | MEDLINE | ID: mdl-38755194

ABSTRACT

Hydrocephalus is classically considered as a failure of cerebrospinal fluid (CSF) homeostasis that results in the active expansion of the cerebral ventricles. Infants with hydrocephalus can present with progressive increases in head circumference whereas older children often present with signs and symptoms of elevated intracranial pressure. Congenital hydrocephalus is present at or near birth and some cases have been linked to gene mutations that disrupt brain morphogenesis and alter the biomechanics of the CSF-brain interface. Acquired hydrocephalus can develop at any time after birth, is often caused by central nervous system infection or haemorrhage and has been associated with blockage of CSF pathways and inflammation-dependent dysregulation of CSF secretion and clearance. Treatments for hydrocephalus mainly include surgical CSF shunting or endoscopic third ventriculostomy with or without choroid plexus cauterization. In utero treatment of fetal hydrocephalus is possible via surgical closure of associated neural tube defects. Long-term outcomes for children with hydrocephalus vary widely and depend on intrinsic (genetic) and extrinsic factors. Advances in genomics, brain imaging and other technologies are beginning to refine the definition of hydrocephalus, increase precision of prognostication and identify nonsurgical treatment strategies.


Subject(s)
Hydrocephalus , Humans , Hydrocephalus/physiopathology , Hydrocephalus/diagnosis , Hydrocephalus/therapy , Hydrocephalus/etiology , Hydrocephalus/complications , Child , Infant , Ventriculostomy/methods , Cerebrospinal Fluid Shunts/methods , Infant, Newborn
2.
Front Public Health ; 12: 1362465, 2024.
Article in English | MEDLINE | ID: mdl-38577289

ABSTRACT

Background: The underlying mechanism for stroke in patients with tuberculous meningitis (TBM) remains unclear. This study aimed to investigate the predictors of acute ischemic stroke (AIS) in TBM and whether AIS mediates the relationship between inflammation markers and functional disability. Methods: TBM patients admitted to five hospitals between January 2011 and December 2021 were consecutively observed. Generalized linear mixed model and subgroup analyses were performed to investigate predictors of AIS in patients with and without vascular risk factors (VAFs). Mediation analyses were performed to explore the potential causal chain in which AIS may mediate the relationship between neuroimaging markers of inflammation and 90-day functional outcomes. Results: A total of 1,353 patients with TBM were included. The percentage rate of AIS within 30 days after admission was 20.4 (95% CI, 18.2-22.6). A multivariate analysis suggested that age ≥35 years (OR = 1.49; 95% CI, 1.06-2.09; P = 0.019), hypertension (OR = 3.56; 95% CI, 2.42-5.24; P < 0.001), diabetes (OR = 1.78; 95% CI, 1.11-2.86; P = 0.016), smoking (OR = 2.88; 95% CI, 1.68-4.95; P < 0.001), definite TBM (OR = 0.19; 95% CI, 0.06-0.42; P < 0.001), disease severity (OR = 2.11; 95% CI, 1.50-2.90; P = 0.056), meningeal enhancement (OR = 1.66; 95% CI, 1.19-2.31; P = 0.002), and hydrocephalus (OR = 2.98; 95% CI, 1.98-4.49; P < 0.001) were associated with AIS. Subgroup analyses indicated that disease severity (P for interaction = 0.003), tuberculoma (P for interaction = 0.008), and meningeal enhancement (P for interaction < 0.001) were significantly different in patients with and without VAFs. Mediation analyses revealed that the proportion of the association between neuroimaging markers of inflammation and functional disability mediated by AIS was 16.98% (95% CI, 7.82-35.12) for meningeal enhancement and 3.39% (95% CI, 1.22-6.91) for hydrocephalus. Conclusion: Neuroimaging markers of inflammation were predictors of AIS in TBM patients. AIS mediates < 20% of the association between inflammation and the functional outcome at 90 days. More attention should be paid to clinical therapies targeting inflammation and hydrocephalus to directly improve functional outcomes.


Subject(s)
Hydrocephalus , Ischemic Stroke , Tuberculosis, Meningeal , Humans , Adult , Tuberculosis, Meningeal/complications , Tuberculosis, Meningeal/epidemiology , Tuberculosis, Meningeal/drug therapy , Ischemic Stroke/complications , Risk Factors , Inflammation/complications , Hydrocephalus/complications
3.
J Korean Med Sci ; 39(15): e139, 2024 Apr 22.
Article in English | MEDLINE | ID: mdl-38651224

ABSTRACT

BACKGROUND: Post-hemorrhagic hydrocephalus (PHH), a common complication of severe intraventricular hemorrhage (IVH) in very low birth weight (BW) infants, is associated with significant morbidity and poor neurological outcomes. The objective of this study was to assess the current status of PHH and analyze the risk factors associated with the necessity of treatment for PHH in infants born between 22 and 28 weeks of gestation, specifically those with severe IVH (grade 3 or 4). METHODS: The analysis was conducted on 1,097 infants who were born between 22-28 gestational weeks and diagnosed with severe IVH, using data from the Korean Neonatal Network. We observed that the prevalence of PHH requiring treatment was 46.3% in infants with severe IVH. RESULTS: Higher rates of mortality, transfer during admission, cerebral palsy, and ventriculoperitoneal shunt after discharge were higher in infants with PHH than in those without PHH. PHH in severe IVH was associated with a higher rate of pulmonary hemorrhage, seizures, and IVH grade 4 in the entire cohort. In addition, it was associated with a lower rate of small for gestational age and chorioamnionitis. In the subgroup analysis, high BW, outborn status, pulmonary hemorrhage, seizure, sepsis, and IVH grade 4 were associated with a higher incidence of PHH between 22 and 25 gestational weeks (GW). In infants born between 26 and 28 GW, a higher incidence of PHH was associated with seizures and IVH grade 4. CONCLUSION: It is necessary to maintain meticulous monitoring and neurological intervention for infants with PHH not only during admission but also after discharge. In addition, identifying the clinical factors that increase the likelihood of developing PHH from severe IVH is crucial.


Subject(s)
Gestational Age , Hydrocephalus , Humans , Hydrocephalus/complications , Republic of Korea/epidemiology , Infant, Newborn , Female , Male , Risk Factors , Cohort Studies , Cerebral Hemorrhage/complications , Severity of Illness Index , Cerebral Intraventricular Hemorrhage/complications , Ventriculoperitoneal Shunt , Infant , Infant, Very Low Birth Weight
4.
Neurol Clin ; 42(2): 487-496, 2024 May.
Article in English | MEDLINE | ID: mdl-38575261

ABSTRACT

The prevalence of brain tumors in patients with headache is very low; however, 48% to 71% of patients with brain tumors experience headache. The clinical presentation of headache in brain tumors varies according to age; intracranial pressure; tumor location, type, and progression; headache history; and treatment. Brain tumor-associated headaches can be caused by local and distant traction on pain-sensitive cranial structures, mass effect caused by the enlarging tumor and cerebral edema, infarction, hemorrhage, hydrocephalus, and tumor secretion. This article reviews the current findings related to epidemiologic details, clinical manifestations, mechanisms, diagnostic approaches, and management of headache in association with brain tumors.


Subject(s)
Brain Edema , Brain Neoplasms , Hydrocephalus , Humans , Brain Neoplasms/complications , Brain Neoplasms/diagnosis , Headache/diagnosis , Headache/etiology , Headache/therapy , Hydrocephalus/complications
6.
J Med Case Rep ; 18(1): 133, 2024 Mar 31.
Article in English | MEDLINE | ID: mdl-38555457

ABSTRACT

BACKGROUND: Diabetes insipidus is a syndrome characterized by polyuria, which is almost always associated with polydipsia. The most frequent cause is central diabetes insipidus, which is the result of an inadequate secretion of the antidiuretic hormone, and diagnosis involves differentiating it from other causes of polyuria and polydipsia. CASE PRESENTATION: Here, we present a clinical case of a previously healthy 13-year-old Nepali boy, who, in December 2022, was found to have intense polydipsia accompanied by polyuria. He had bilateral lower limb weakness at the time of presentation. Biochemical evaluation demonstrated raised serum sodium (181 mEq/L), serum creatinine (78 µmol/L), and serum uric acid (560 µmol/L) with suppressed serum potassium (2.7 mEq/L), which was the major concern to the clinicians. Further laboratory workup revealed an increased serum osmolarity (393.6 mOsm/kg) with reduced urine osmolarity (222.7 mOsm/kg). On contrast magnetic resonance imaging of the brain, a thick-walled third ventricular cyst with bilateral foramen obstruction, thin membrane-like structure at top of aqueduct of Sylvius with gross obstructive hydrocephalus (inactive), and compressed and thinned pituitary gland with no bright spot was observed. The laboratory findings, radiological findings, and case presentation provided the provisional diagnosis of diabetes insipidus due to hydrocephalus and third ventricular cyst. CONCLUSIONS: Central diabetes insipidus due to hydrocephalus, though rare, can have serious complications including the predilection to develop a deficit of other pituitary hormones. Thus, even if hydrocephalus is dormant with normal intracranial pressure, it must be addressed during investigations of central diabetes insipidus.


Subject(s)
Cysts , Diabetes Insipidus, Neurogenic , Diabetes Insipidus , Hydrocephalus , Male , Humans , Adolescent , Diabetes Insipidus, Neurogenic/complications , Diabetes Insipidus, Neurogenic/diagnosis , Polyuria/complications , Polyuria/diagnosis , Uric Acid , Diabetes Insipidus/complications , Diabetes Insipidus/diagnosis , Vasopressins , Polydipsia/etiology , Polydipsia/complications , Hydrocephalus/complications , Cysts/complications
7.
Theranostics ; 14(5): 1909-1938, 2024.
Article in English | MEDLINE | ID: mdl-38505607

ABSTRACT

Rationale: Hydrocephalus is a substantial complication after intracerebral hemorrhage (ICH) or intraventricular hemorrhage (IVH) that leads to impaired cerebrospinal fluid (CSF) circulation. Recently, brain meningeal lymphatic vessels (mLVs) were shown to serve as critical drainage pathways for CSF. Our previous studies indicated that the degradation of neutrophil extracellular traps (NETs) after ICH/IVH alleviates hydrocephalus. However, the mechanisms by which NET degradation exerts beneficial effects in hydrocephalus remain unclear. Methods: A mouse model of hydrocephalus following IVH was established by infusing autologous blood into both wildtype and Cx3cr1-/- mice. By studying the features and processes of the model, we investigated the contribution of mLVs and NETs to the development and progression of hydrocephalus following secondary IVH. Results: This study observed the widespread presence of neutrophils, fibrin and NETs in mLVs following IVH, and the degradation of NETs alleviated hydrocephalus and brain injury. Importantly, the degradation of NETs improved CSF drainage by enhancing the recovery of lymphatic endothelial cells (LECs). Furthermore, our study showed that NETs activated the membrane protein CX3CR1 on LECs after IVH. In contrast, the repair of mLVs was promoted and the effects of hydrocephalus were ameliorated after CX3CR1 knockdown and in Cx3cr1-/- mice. Conclusion: Our findings indicated that mLVs participate in the development of brain injury and secondary hydrocephalus after IVH and that NETs contribute to acute LEC injury and lymphatic thrombosis. CX3CR1 is a key molecule in NET-induced LEC damage and meningeal lymphatic thrombosis, which leads to mLV dysfunction and exacerbates hydrocephalus and brain injury. NETs may be a critical target for preventing the obstruction of meningeal lymphatic drainage after IVH.


Subject(s)
Brain Injuries , Extracellular Traps , Hydrocephalus , Thrombosis , Mice , Animals , Extracellular Traps/metabolism , Endothelial Cells/metabolism , Cerebral Hemorrhage/complications , Hydrocephalus/complications , Hydrocephalus/metabolism
8.
J Clin Neurosci ; 121: 83-88, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38368659

ABSTRACT

INTRODUCTION: Permanent liquor diversion is associated with a high risk of failure and often requires re-intervention. The ventriculo-gallbladder shunt (VGS) has been recognized as a last-resort alternative for treating hydrocephalus when the peritoneum or other distal sites are no longer suitable for receiving shunts. This article aims to report a case from a neurosurgery referral service in Brazil and review the literature on this issue. METHODS: A systematic literature review was conducted in accordance with the PRISMA statement. The PubMed, Embase, and Web of Science databases were searched for data screening and extraction. The reported case was conducted with ethical approval from the neurosurgical hospital's ethics committee. RESULTS: G.B.S, male, 43 years old, no comorbidities, who has been dealing with a 12-year history of hydrocephalus, with post-surgical chronic fungal meningitis. Two years ago, he underwent a ventriculoatrial shunt (VAS) placement due to multiple ventriculoperitoneal shunt (VPS) failures. Endocarditis was suspected, and the VAS was removed. As an alternative, VGS was implanted 6 months ago, and since then, there has been no need for a new system review. The gallbladder has an absorptive capacity of 1500 cc of liquid daily, which is more than the normal daily production of cerebrospinal fluid (CSF). Therefore, it is a good alternative when the ventriculoperitoneal shunt is not feasible due to postsurgical peritoneal adhesions or when there are contraindications for ventriculoatrial shunts. CONCLUSION: VGS is an alternative for patients who cannot undergo the most common surgical interventions, such as VPS.


Subject(s)
Cerebrospinal Fluid Shunts , Hydrocephalus , Adult , Humans , Male , Gallbladder/surgery , Hydrocephalus/surgery , Hydrocephalus/complications , Prostheses and Implants , Ventriculoperitoneal Shunt/adverse effects
9.
Eur J Med Res ; 29(1): 128, 2024 Feb 16.
Article in English | MEDLINE | ID: mdl-38365795

ABSTRACT

BACKGROUND: Mild fetal ventriculomegaly (VM) is a nonspecific finding common to several pathologies with varying prognosis and is, therefore, a challenge in fetal consultation. We aimed to perform a constant, detailed analysis of prenatal findings and postnatal outcomes in fetuses with early-onset and late-onset mild ventriculomegaly, and provide a new evidence basis and new perspective for prenatal counseling. METHODS: This is a retrospective cohort study of women with a diagnosis of mild fetal VM between January 2018 and October 2020. The population was divided into two groups according to the gestational ages (GAs) at initial diagnosis: the early-onset group (diagnosed at/before 24+6 weeks) and the late-onset group (diagnosed after 24+6 weeks). Clinical data and pregnancy outcomes were obtained from hospital records. The children's neurodevelopment status was assessed using the Ages and Stages Questionnaire, Third Edition (ASQ-3) and telephone interviews. RESULTS: Our study cohort comprised 324 fetuses, out of which 94 (29%) were classified as early-onset group and 230 (71%) late-onset group. Early-onset group was more likely to have concurrent additional abnormalities, whereas in the late-onset group, isolated enlargement was more common (P = 0.01). Unilateral enlargement was more common in the late-onset group (P = 0.05), and symmetrical enlargement in the early-onset group (P < 0.01). In addition, early-onset mild VM cases were more likely to have intrauterine progression (P = 0.03), and many had a higher proportion of complex multisystem abnormalities. Compared with the late-onset group, the early-onset group was more often associated with congenital brain structure malformations. Approximately 11% of fetuses with mild VM had postnatal neurodevelopmental delay/disorders, and the risk was higher in the early-onset group (19.4% vs. 7.4%). Regression analysis showed that the GA at first diagnosis, non-isolated, and intrauterine progression significantly correlated with neurodevelopmental abnormalities. CONCLUSIONS: Early-onset and late-onset mild VM had significantly different ultrasound features and outcomes. Early-onset mild VM may have more complex potential abnormalities and are more likely to predict poor prognosis than the late-onset.


Subject(s)
Hydrocephalus , Child , Pregnancy , Humans , Female , Hydrocephalus/diagnostic imaging , Hydrocephalus/epidemiology , Hydrocephalus/complications , Retrospective Studies , Follow-Up Studies , Ultrasonography, Prenatal , Pregnancy Outcome/epidemiology
10.
Brain Nerve ; 76(2): 101-107, 2024 Feb.
Article in Japanese | MEDLINE | ID: mdl-38351555

ABSTRACT

Idiopathic normal pressure hydrocephalus causes a triad of gait disturbance, dementia, and urinary incontinence in the elderly. All these symptoms may manifest as age-related functional decline or from neurological and non-neurological diseases common in the elderly. In idiopathic normal pressure hydrocephalus, characterized by ataxic-ataxic gait, subcortical dementia, and urge urinary incontinence, it is clinically important to consider these characteristic features. This overview describes the symptomatology of idiopathic normal pressure hydrocephalus.


Subject(s)
Dementia , Hydrocephalus, Normal Pressure , Hydrocephalus , Movement Disorders , Urinary Incontinence , Humans , Aged , Hydrocephalus, Normal Pressure/diagnosis , Hydrocephalus, Normal Pressure/etiology , Hydrocephalus/complications , Dementia/complications , Movement Disorders/complications , Urinary Incontinence/diagnosis , Urinary Incontinence/complications , Gait
11.
J Med Case Rep ; 18(1): 53, 2024 Feb 13.
Article in English | MEDLINE | ID: mdl-38347580

ABSTRACT

BACKGROUND: Neurosarcoidosis occurs symptomatically in 5-10% of patients with sarcoidosis, and hydrocephalus is a rare complication of neurosarcoidosis, with either acute or subacute onset and presenting symptoms related to increased intracranial pressure. It represents a potentially fatal manifestation with a mortality rate of 22% (increased to 75% in case of coexistence of seizures) that requires a prompt initiation of treatment. High-dose intravenous corticosteroid treatment and neurosurgical treatment must be considered in all cases of neurosarcoidosis hydrocephalus. CASE PRESENTATION: Here we present a case of hydrocephalus in neurosarcoidosis, complicated by generalized seizures, in a 29-year-old Caucasian male patient treated with medical treatment only, with optimal response. CONCLUSION: Since neurosurgery treatment can lead to severe complications, this case report underlines the possibility to undergo only medical treatment in selected cases. Further studies are needed to stratify patients and better identify those eligible for only medical approach.


Subject(s)
Central Nervous System Diseases , Hydrocephalus , Sarcoidosis , Humans , Male , Adult , Central Nervous System Diseases/complications , Central Nervous System Diseases/drug therapy , Central Nervous System Diseases/diagnosis , Hydrocephalus/complications , Hydrocephalus/drug therapy , Sarcoidosis/complications , Sarcoidosis/drug therapy , Sarcoidosis/diagnosis , Adrenal Cortex Hormones/therapeutic use , Seizures/complications
12.
Ann Afr Med ; 23(1): 100-103, 2024.
Article in English | MEDLINE | ID: mdl-38358179

ABSTRACT

The pediatric age group with massive hydrocephalus posted for ventriculoperitoneal (VP) shunt presents a lot of confrontation to anesthesiologists due to macrocephalus and associated congenital anomalies. Here, we presented a case report with massive hydrocephalus with aqueduct stenosis, posted for VP shunt placement, and retained a difficult airway.


RésumésLe grouped'âgepédiatrique avec unehydrocéphalie massive affichée pour un shunt ventriculopéritonéal (VP) présente beaucoup de confrontation à l'anesthésisteen raison de la macrocéphalieet des anomalies congénitalesassociées. Ici, nous avonsprésentéun rapport de casd'hydrocéphalie massive avec sténose de l'aqueduc, affiché pour la miseen place d'un shunt VP, et conservéunevoierespiratoire difficile. Mots-clés : Pédiatrique, Voiesrespiratoiresdifficiles, hydrocéphalie, dérivationventriculopéritonéale.


Subject(s)
Anesthetics , Hydrocephalus , Child , Humans , Hydrocephalus/complications , Hydrocephalus/surgery , Ventriculoperitoneal Shunt/adverse effects , Retrospective Studies
13.
Dev Med Child Neurol ; 66(7): 948-957, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38247023

ABSTRACT

Hydrocephalus is rarely described in Joubert-Boltshauser syndrome (JBTS). The aim of this study was to investigate whether this association is a chance occurrence or potentially signifies a new phenotypic subtype. The databases of Wolfson Medical Center, Sourasky Medical Center, and EB's personal collection were reviewed. Records from an additional family were obtained from RG. The patients' medical records, prenatal ultrasounds, and magnetic resonance imaging were assessed. In addition, we reviewed the medical literature for the association of ventriculomegaly/hydrocephalus (VM/HC) in JBTS. Only seven cases (from five families) were found with prenatal onset of VM/HC, diagnosed during the second trimester; three pregnancies were terminated, one was stillborn and three were born, of which one died within a week, and another died at the age of 6 years. Additional central nervous system findings included dysgenesis of the corpus callosum, delayed sulcation, polymicrogyria, and pachygyria. We found 16 publications describing 54 patients with JBTS and VM/HC: only five were diagnosed at birth and three were diagnosed prenatally. Hydrocephalus is extremely rare in JBTS. The recurrence of this association, reported in several publications in multiple family members, suggests that it might represent a new phenotypic subtype of JBTS possibly associated with specific genes or variants. Further genetic studies are needed to confirm this hypothesis. WHAT THIS PAPER ADDS: The association of fetal hydrocephalus with Joubert-Boltshauser syndrome (JBTS) is very rare but not a chance association. This association represents a new phenotypic subtype of JBTS possibly linked to specific genes or variants.


Subject(s)
Abnormalities, Multiple , Cerebellum , Eye Abnormalities , Hydrocephalus , Kidney Diseases, Cystic , Retina , Humans , Hydrocephalus/diagnostic imaging , Hydrocephalus/complications , Cerebellum/abnormalities , Cerebellum/diagnostic imaging , Eye Abnormalities/complications , Eye Abnormalities/diagnostic imaging , Abnormalities, Multiple/diagnostic imaging , Female , Kidney Diseases, Cystic/complications , Kidney Diseases, Cystic/diagnostic imaging , Kidney Diseases, Cystic/genetics , Male , Retina/abnormalities , Retina/diagnostic imaging , Cerebellar Vermis/abnormalities , Cerebellar Vermis/diagnostic imaging , Magnetic Resonance Imaging , Phenotype , Cerebellar Diseases/diagnostic imaging , Cerebellar Diseases/complications , Child , Infant, Newborn
14.
Childs Nerv Syst ; 40(4): 1251-1258, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38217730

ABSTRACT

INTRODUCTION: Encephalocraniocutaneous lipomatosis (ECCL) is a rare congenital syndrome with complex skin, eye, and central nervous system (CNS) symptoms. Diagnosis and treatment are challenging due to its rarity and diverse manifestations. It often involves issues like porencephalic cysts, cortical atrophy, and low-grade gliomas in the CNS, resulting in developmental delays. The spinal cord is frequently affected, leading to problems like medullary compression and radiculopathy, causing back pain and sensory/motor deficits. Surgical interventions are reserved for symptomatic cases to address hydrocephalus or alleviate spinal lipomas. This article reviews a case series to assess surgical risks and neurological outcomes. CASE SERIES: We present a case series ECCL, focusing on the diffuse lipomatosis of the spinal cord and the intricate surgical procedures involved. A multi-stage surgical approach was adopted, with continuous neuromonitoring employed to safeguard motor pathways. We discuss clinical characteristics, imaging studies, and indications for neurosurgical interventions. DISCUSSION: ECCL is a complex syndrome. Diagnosis is challenging and includes clinical evaluation, neuroimaging, and genetic testing. Treatment targets specific symptoms, often requiring surgery for issues like lipomas or cerebral cysts. Surgery involves laminectomies, spinal fusion, and motor pathway monitoring. Thorough follow-up is crucial due to potential CNS complications like low-grade gliomas. Hydrocephalus occurs in some cases, with endoscopic third ventriculostomy (ETV) preferred over ventriculoperitoneal shunt placement. CONCLUSION: Neurosurgery for ECCL is for symptomatic cases. ETV is preferred for hydrocephalus, while the treatment for lipoma is based on the presence of symptoms; the follow-up should assess growth and prevent deformities.


Subject(s)
Cysts , Eye Diseases , Glioma , Hydrocephalus , Lipoma , Lipomatosis , Neurocutaneous Syndromes , Humans , Cysts/complications , Glioma/complications , Hydrocephalus/complications , Lipoma/complications , Lipomatosis/surgery , Neurocutaneous Syndromes/complications
15.
Childs Nerv Syst ; 40(4): 1259-1270, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38276973

ABSTRACT

BACKGROUND: Intracranial teratoma represents a rare neoplasm, occurring predominantly during childhood. Characteristic symptoms depend on the location but are mainly hydrocephalus, visual disturbances, hypopituitarism, and diabetes insipidus. Initial diagnosis can be challenging due to similar radiological features in both teratomas and other lesions such as craniopharyngiomas. Gross total resection is recommended if feasible and associated with a good prognosis. CASE DESCRIPTION: A 10-year-old girl presented with newly diagnosed growth retardation, fatigue, cephalgia and bilateral hemianopia. Further laboratory analysis confirmed central hypothyroidism and hypercortisolism. Cranial magnetic resonance imaging showed a cystic space-occupying lesion in the sellar and suprasellar compartment with compression of the optic chiasm without hydrocephalus present, suspicious of craniopharyngioma. Subsequently, an endonasal endoscopic transsphenoidal near-total tumor resection with decompression of the optic chiasm was performed. During postoperative recovery the patient developed transient diabetes insipidus, the bilateral hemianopia remained unchanged. The patient could be discharged in a stable condition, while hormone replacement for multiple pituitary hormone deficiency was required. Surprisingly, histopathology revealed conspicuous areas of skin with formation of hairs and squamous epithelia, compatible with a mature teratoma. CONCLUSIONS: We present an extremely rare case of pediatric sellar teratoma originating from the pituitary gland and a review of literature focusing on the variation in presentation and treatment. Sellar teratomas are often mistaken for craniopharyngioma due to their similar radiographic appearances. However, the primary goal of treatment for both pathologies is to decompress eloquent surrounding structures such as the optic tract, and if applicable, resolution of hydrocephalus while avoiding damage to the pituitary stalk and especially the hypothalamic structures. If feasible, the aim of surgery should be gross total resection.


Subject(s)
Central Nervous System Neoplasms , Craniopharyngioma , Diabetes Insipidus , Hydrocephalus , Hypopituitarism , Pituitary Neoplasms , Teratoma , Female , Humans , Child , Craniopharyngioma/surgery , Hemianopsia , Pituitary Neoplasms/surgery , Central Nervous System Neoplasms/complications , Teratoma/surgery , Hydrocephalus/complications
16.
Acta Paediatr ; 113(4): 827-832, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38233740

ABSTRACT

AIM: We aimed to evaluate the occurrence of, and risk factors for precocious and early puberty in a retrospective cohort study of girls with shunted infantile hydrocephalus. METHODS: The study population comprised 82 girls with infantile hydrocephalus, born between 1980 and 2002, and treated with a ventriculoperitoneal shunt. Data were available for 39 girls with myelomeningocele and 34 without. Medical records were analysed regarding clinical data and timing of puberty. Precocious and early puberty was defined as the appearance of pubertal signs before 8 years and 0 months and 8 years and 9 months, respectively. RESULTS: Median age at last admission was 15.8 years (range 10.0-18.0). In total, 15 girls (21%) had precocious puberty, and another 21 (29%) had early puberty. Three or more shunt revisions had been performed in 26/36 girls with early or precocious puberty and in 3/37 girls without (p = 0.01). The number of shunt revisions correlated negatively with age at the start of puberty in the girls with myelomeningocele (Spearman's correlation coefficient = -0.512, p = 0.001). CONCLUSION: Girls with shunted infantile hydrocephalus have a high risk of precocious or early puberty. Repeated shunt revisions seemed to be associated with early puberty.


Subject(s)
Hydrocephalus , Meningomyelocele , Puberty, Precocious , Female , Humans , Child , Adolescent , Cohort Studies , Puberty, Precocious/epidemiology , Puberty, Precocious/etiology , Retrospective Studies , Meningomyelocele/complications , Meningomyelocele/surgery , Meningomyelocele/diagnosis , Sweden/epidemiology , Hydrocephalus/surgery , Hydrocephalus/complications , Puberty
18.
J Emerg Med ; 66(2): 139-143, 2024 Feb.
Article in English | MEDLINE | ID: mdl-38228458

ABSTRACT

BACKGROUND: Emergency physicians are well-versed in managing cardiac arrests, including the diagnostic and therapeutic steps after return of spontaneous circulation. Neurologic emergencies are a common cause of out-of-hospital cardiac arrest and must remain high in the differential diagnosis, as such cases often require specific interventions that may deviate from more common care pathways. Performing a noncontrast head computed tomography (NCHCT) scan after cardiac arrest has been found to change management, although the optimal timing of this imaging is unclear. CASE REPORT: This is the case of a young, pregnant woman who presented to the emergency department after cardiac arrest with return of spontaneous circulation in the prehospital setting. She was found to have acute obstructive hydrocephalus on NCHCT, which was later confirmed to be due to a previously undiagnosed colloid cyst of the third ventricle. This acute obstruction resulted in myocardial stunning and, ultimately, cardiac arrest. Although outcomes are often dismal when the cause of arrest is secondary to neurologic catastrophe, this patient survived with completely intact neurologic function. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Although acute obstructive hydrocephalus due to a colloid cyst adjacent to the third ventricle is a rare condition, it is a potentially reversible neurologic cause of out-of-hospital cardiac arrest. However, positive outcomes depend on obtaining the diagnosis rapidly with neurologic imaging and advocating for neurosurgical intervention. This case supports the recommendation that emergency physicians should strongly consider post-cardiac arrest neurologic imaging when another cause is not immediately obvious.


Subject(s)
Cardiopulmonary Resuscitation , Colloid Cysts , Emergency Medical Services , Hydrocephalus , Out-of-Hospital Cardiac Arrest , Female , Humans , Out-of-Hospital Cardiac Arrest/complications , Colloid Cysts/complications , Cardiopulmonary Resuscitation/methods , Hydrocephalus/complications , Tomography, X-Ray Computed , Emergency Medical Services/methods
19.
Childs Nerv Syst ; 40(1): 109-114, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37695368

ABSTRACT

PURPOSE: Hydrocephalus is one of the most common pathologies in pediatric neurosurgery. One of the causes of recurring events of headaches among shunted children is "slit ventricle syndrome" (SVS). Several potential treatments have been proposed, yet SVS often represents a treatment challenge. The goal of the current series is to present our experience with adding a positional shunt-assist (SA) (Miethke, Aesculap) for the treatment of SVS. METHODS: Clinical data was retrospectively collected from all consecutive children with SVS that were treated with SA (Miethke, Aesculap) at our center. Surgical and clinical outcomes as expressed by hospital visits, or need for additional surgery, were evaluated. RESULTS: Nine cases were included. Hydrocephalus etiology included IVH (6), postinfectious (1), and congenital syndromes (2). Average age at first shunt was 4 months. Primary shunt type was differential-pressure-valve in all. Average age at SVS onset was 4 years. Average age at SA placement was 5.5 years. There were no perioperative complications besides a single stich abscess. A 6-month follow-up period after SA was compared to a 6-month period prior to the SA: average hospital visits decreased from 2.4 to 0.6 per patient (p < 0.0002). 4/9 patients needed an LP or shunt revision before the SA surgery, while no procedure was indicated during the immediate 6-month follow-up. At the last follow-up, there was a significant reduction in the rate of ER visits compared to prior to surgery; however, the number of neurosurgical procedures did not significantly differ. CONCLUSION: Using a SA for SVS was associated with a short-term improvement of symptoms in the majority of cases, reduction in hospital visits, and reduced need for SVS-related procedures.


Subject(s)
Hydrocephalus , Slit Ventricle Syndrome , Child , Humans , Infant , Child, Preschool , Slit Ventricle Syndrome/surgery , Slit Ventricle Syndrome/etiology , Slit Ventricle Syndrome/pathology , Cerebrospinal Fluid Shunts/adverse effects , Retrospective Studies , Hydrocephalus/surgery , Hydrocephalus/complications , Neurosurgical Procedures/adverse effects , Ventriculoperitoneal Shunt/adverse effects
20.
World Neurosurg ; 181: e273-e290, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37839574

ABSTRACT

BACKGROUND: The opportunities to treat elderly patients with aneurysmal subarachnoid hemorrhage (aSAH) are increasing globally, but the outcome remains poor. This study seeks to investigate treatment-related factors that can modify functional outcomes in patients with aSAH aged ≥75 years. METHODS: A total of 202 patients with aSAH aged ≥75 years prospectively enrolled in 9 primary stroke centers from 2013 to 2021 were retrospectively analyzed. Clinical variables including treatments for hydrocephalus, angiographic vasospasm, and delayed cerebral ischemia were compared between patients with good (modified Rankin Scale [mRS] score 0-2) and poor (mRS score 3-6) outcomes at 90 days from onset, followed by multivariate analyses to find independent outcome determinants. A modifiable treatment-related variable was evaluated after propensity score matching with adjustments for age, sex, pre-onset mRS score, aSAH severity, and treatment modality. RESULTS: More than half of patients showed World Federation of Neurological Societies grades IV-V on admission. Univariate analyses showed that advanced age, worse pre-onset mRS score, more severe neurologic status on admission, higher modified Fisher grade on admission computed tomography scans, and acute and chronic hydrocephalus were associated with poor outcomes. In contrast, administration of a phosphodiesterase type III inhibitor, cilostazol, was associated with good outcomes in both univariate (P = 0.036) and multivariate analyses (adjusted odds ratio, 0.305; 95% confidence interval, 0.097-0.955; P = 0.042). Propensity score matching analyses showed that patients treated with cilostazol had better outcomes (P = 0.016) with fewer incidences of delayed cerebral infarction (P = 0.008). CONCLUSIONS: Even in patients with aSAH aged ≥75 years, cilostazol administration may lead to better outcomes by suppressing the development of delayed cerebral infarction.


Subject(s)
Hydrocephalus , Subarachnoid Hemorrhage , Vasospasm, Intracranial , Aged , Humans , Cilostazol/therapeutic use , Subarachnoid Hemorrhage/complications , Retrospective Studies , Propensity Score , Cerebral Infarction/etiology , Phosphodiesterase 3 Inhibitors/therapeutic use , Vasospasm, Intracranial/etiology , Hydrocephalus/complications , Treatment Outcome
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