Etiology, clinical presentation and treatment of congenital hydrocephalus

OBJECTIVE: Intracranial pressure (ICP) monitoring is commonly utilized for identifying pathologic ICP in cases of traumatic brain injury; however, its utility in hydrocephalic children has not been elucidated. Although patients with typical (pressure-active) hydrocephalus present with clear signs and/or symptoms and the need for cerebrospinal fluid (CSF) diversion is often clear, others may have arrested or pressure-compensated hydrocephalus with pathologic ICP elevation masked by ambiguous signs or are completely asymptomatic...

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...

Infection caused by human parvovirus B19 (B19) often has mild yet wide-ranging clinical signs, with the course of disease usually defined as benign. Particularly prevalent in the population of young children, the virus is commonly transmitted to the parents, especially to susceptible mothers. During pregnancy, particularly the first and second trimesters, parvovirus infection can lead to pathology of the fetus: anemia, heart failure, hydrops, and disorders of physical and neurological development. In severe cases, the disease can result in fetal demise...

Congenital hydrocephalus affects approximately one in 1000 newborn children and is fatal in approximately 50% of untreated cases. The currently known management protocols usually necessitate multiple interventions and long-term use of healthcare resources due to a relatively high incidence of complications, and many of them mostly provide a treatment of the effect rather than the cause of cerebrospinal fluid flow reduction or outflow obstruction. Future studies discussing etiology specific hydrocephalus alternative treatments are needed...

Certain congenital or developmental anomalies of adult-onset hydrocephalus often share clinical symptoms with idiopathic normal pressure hydrocephalus(iNPH). These anomalies include long-standing overt ventriculomegaly in adults(LOVA), persistent Break's pouch cyst(PBC)and panventriculomegaly(PaVM). Certain patients with adult-onset hydrocephalus might have congenital or secondary etiologies, such as late-onset idiopathic aqueductal stenosis(LIAS), and syndrome of hydrocephalus in young and middle-aged adults(SHYMA)...

OBJECTIVE: Shunt treatment for hydrocephalus in children should aim for sustainable flexibility in regard to optional, perspective pressure level adjustment during advancing physical and mental development. Gravitation-assisted shunt valves are designed to prevent hydrostatic over-drainage frequently observed in the long course of shunt-treated hydrocephalus. We prospectively studied and analyzed the implication, safety, and feasibility for an adjustable gravitational unit combined with a fixed differential-pressure (DP) valve for neonates and infants primary shunted within the first 12 months of life...

While normal-pressure hydrocephalus (NPH) is most commonly diagnosed in older adulthood, a significant body of literature has accumulated over half a century documenting the clinical phenomenon of an NPH-like syndrome in pediatric patients. As in adult NPH, it is likely that pediatric NPH occurs due to a heterogeneous array of developmental, structural, and neurodegenerative pathologies, ultimately resulting in aberrant cerebrospinal fluid (CSF) flow and distribution within and around the brain. In this review, we aimed to systematically survey the existing clinical evidence supporting the existence of a pediatric form of NPH, dating back to the original recognition of NPH as a clinically significant subtype of communicating hydrocephalus...

PURPOSE: To gain insight into the patient characteristics of surgically treated hydrocephalus in the Netherlands, we report the first data from the Dutch Quality Registry NeuroSurgery (QNRS) database for infants with hydrocephalus requiring surgical intervention. METHODS: We used the prospectively gathered database concerning infants ≤ 2 years of age surgically treated for hydrocephalus. We report data from start of registry, concerning etiology, age, and treatment of patients registered...

OBJECTIVEThe authors describe the demographics and clinical characteristics of the first 517 patients enrolled in the Adult Hydrocephalus Clinical Research Network (AHCRN) during its first 2 years.METHODSAdults ≥ 18 years were nonconsecutively enrolled in a registry at 6 centers. Four categories of adult hydrocephalus were defined: transition (treated before age 18 years), unrecognized congenital (congenital pattern, not treated before age 18 years), acquired (secondary to known risk factors, treated or untreated), and suspected idiopathic normal pressure hydrocephalus (iNPH) (≥ age 65 years, not previously treated)...

OBJECTIVE: Endoscopic third ventriculostomy (ETV) remains the mainstay of treatment for noncommunicating hydrocephalus these days. However, there has been no adequate clue for the intraoperative assessment of ETV efficacy until now. This time, we propose a newly defined finding, "folding sign," which is visible and enables us to confirm penetration of both the third ventricle floor and the Liliequist membrane (LM) during operation. In this report, we describe consecutive ETV cases and discuss the mechanism of folding sign and its clinical meanings...

Central precocious puberty (CPP) is caused by premature activation of the hypothalamo-pituitary-gonadal axis. More than 50% of boys with CPP have an identifiable etiology. Hypothalamic hamartoma (HH), hydrocephalus, tumors, infections, congenital defects, ischemia, radiation, or injury of the brain are the most common causes of secondary CPP. In this report, we present the case of a 2 years and 9 months old male patient who had a 30x40 mm contrast-enhancing suprasellar mass and was histopathologically diagnosed with giant HH...

OBJECT: Endoscopic choroid plexus coagulation (CPC) with or without endoscopic third ventriculostomy (ETV) has been shown to be effective for selected patients with hydrocephalus. However, whether the effect of the coagulation is temporary and the choroid plexus regenerates or can be obliterated has remained largely unknown. The authors evaluate the effectiveness of CPC and report 3 cases of obliteration demonstrated by direct endoscopic observation. METHODS: The authors retrospectively analyzed the surgical results of patients with hydrocephalus primarily treated by CPC with or without ETV...

BACKGROUND: Routinely, hydrocephalus related to fourth ventricular outlet obstruction (FVOO) has been managed with ventriculoperitoneal (VP) shunting or endoscopic third ventriculostomy (ETV). Few reports on Magendie foraminoplasty exist, and Luschka foraminoplasty has not been described. OBJECTIVE: To present an alternative technique in the management of FVOO via an endoscopic transventricular transaqueductal Magendie and Luschka foraminoplasty and to discuss the indications, technique, findings, and outcomes...

OBJECT: Overdrainage of CSF remains an unsolved problem in shunt therapy. The aim of the present study was to evaluate treatment options on overdrainage-related events enabled by the new generation of adjustable gravity-assisted valves. METHODS: The authors retrospectively studied the clinical course of 250 consecutive adult patients with various etiologies of hydrocephalus after shunt insertion for different signs and symptoms of overdrainage. Primary and secondary overdrainage were differentiated...

PURPOSE: The outcome of endoscopic third ventriculostomy (ETV) is worse in children younger than 2 years old and especially in infants, and controversies still exist whether ETV might be superior to shunt placement in this age group. We retrospectively analyzed the data of 23 patients younger than 6 months of age treated with ETV and assessed its feasibility as a first choice of treatment for hydrocephalus. METHODS: Between 1994 and 2008 in our clinic, 23 patients younger than 6 months having presented with obstructive hydrocephalus were treated endoscopically...

INTRODUCTION: Endoscopic third ventriculostomy (ETV) is considered a safe procedure and is a method of choice in treatment of obstructive hydrocephalus nowadays. In case of Sylvian aqueduct stenosis, the success rate reaches 90%. In children younger than 6 to 24 months, respectively, however, some authors report lower effectiveness ranging between 0% and 64%. The reasons of ETV failure are discussed: hyporesorption in patients with obstruction as a consequence of hemorrhage or infection, suboptimal ETV performance, especially in premature newborns, or the theory of different cerebrospinal fluid circulation in newborn babies...

BACKGROUND: Ventriculoperitoneal (VP) shunts are used for intracranial pressure management and temporary cerebrospinal fluid (CSF) drainage. Infection of the central nervous system (CNS) is a major cause of morbidity and mortality in patients with CSF shunts. The aim of the present study was to evaluate the clinical features, pathogens, and outcomes of 22 patients with CSF shunt infections collected over 4 years. METHODS: The patients with shunt insertions were evaluated using; age, sex, etiology of hydrocephalus, shunt infection numbers, biochemical and microbiological parameters, prognosis, clinical infection features and clinical outcome...

OBJECTIVES: Currently, headache, nausea/vomiting, visual changes, and altered mental status are accepted as indications for the evaluation of hydrocephalus in children; while dementia, gait apraxia, and urinary incontinence remain indications in the elderly. The clinical presentation of hydrocephalus in young and middle-aged adults remains poorly described. Hence, middle-aged patients with mild gait, cognitive, or urinary symptoms unaccompanied by clear exam findings often remain undiagnosed and untreated...

OBJECTIVE: To know the etiological factors of cerebral palsy (CP) incident during pregnancy and neonatal/perinatal periods. To know evolution of a child with a CP diagnosis. PATIENTS, MATERIAL AND METHODS: Bibliographical review of paediatric and rehabilitation texts and databases in Internet. Elaboration of a data collecting questionnaire for reviewing clinical records of patients treated in Paediatric Rehabilitation between 1996 and 2000. RESULTS: 250 patients records reviewed...

BACKGROUND: Childhood hypopituitarism may be present at birth or may be acquired. Early diagnosis and treatment promote the best possible outcomes. METHODS: The anatomy, etiologies, clinical presentation, diagnostic testing strategies, and current treatments relevant to childhood hypopituitarism are reviewed. RESULTS: Children with congenital hypopituitarism may present with life-threatening hypoglycemia, abnormal serum sodium concentrations, shock, microphallus in males, and, only later, growth failure...