The "microcephalic hydrocephalus" paradox as a paradigm of altered neural stem cell biology.

Characterized by enlarged brain ventricles, hydrocephalus is a common neurological disorder classically attributed to a primary defect in cerebrospinal fluid (CSF) homeostasis. Microcephaly ("small head") and hydrocephalus are typically viewed as two mutually exclusive phenomenon, since hydrocephalus is thought of as a fluid "plumbing" disorder leading to CSF accumulation, ventricular dilatation, and resultant macrocephaly. However, some cases of hydrocephalus can be associated with microcephaly. Recent work in the genomics of congenital hydrocephalus (CH) and an improved understanding of the tropism of certain viruses such as Zika and cytomegalovirus are beginning to shed light into the paradox "microcephalic hydrocephalus" by defining prenatal neural stem cells (NSC) as the spatiotemporal "scene of the crime." In some forms of CH and viral brain infections, impaired fetal NSC proliferation leads to decreased neurogenesis, cortical hypoplasia and impaired biomechanical interactions at the CSF-brain interface that collectively engender ventriculomegaly despite an overall and often striking decrease in head circumference. The coexistence of microcephaly and hydrocephalus suggests that these two phenotypes may overlap more than previously appreciated. Continued study of both conditions may be unexpectedly fertile ground for providing new insights into human NSC biology and our understanding of neurodevelopmental disorders.

Multidisciplinary management of people with spina bifida across the lifespan.

The average worldwide prevalence of neural tube defects (NTDs) is 1.0 per 1000 births. Its development is multifactorial due to genetic and non-genetic factors. Spina bifida (SB) is one of main representatives of NTD. The spinal cord lesion level is the main determinant of the level of paralysis, numbness, and difficulties with bladder/bowel functions. Myelomeningocele prenatal repair reduces hydrocephalus and hindbrain herniation and improves motor function. The severity of hydrocephalus is associated with poorer neurodevelopmental outcomes whether operated on prenatally or after birth. People with SB tend to have a lower IQ and cognitive difficulties. Early diagnosis, proactivity, and lifelong multidisciplinary follow-up are key protective issues. Invasive urological interventions should be considered in selected patients after failure of conservative treatment. Transition to adult care should be well planned as it is challenging. Health literacy is directly associated with success at transition. Sexuality and fertility should be addressed before/during puberty. Overall, the rates of fecal and urinary continence and skin breakdown increase with age, whereas the ability to ambulate declines with age. Bowel and urinary incontinence are independent predictors of lower health-related quality of life (HRQoL) in adults with SB. Bowel incontinence has negative impact on HRQoL regardless of frequency or amount. Long-term caregiver support should be offered at diagnosis. Survival at a mean of 50 years is poor, at 32%, due to central nervous system deaths, cancer, urological disease, and sepsis. Challenges to implementation of recommended practices exist, especially in low and middle-income countries.

Abnormal development of transient fetal zones in mild isolated fetal ventriculomegaly.

Mild isolated fetal ventriculomegaly (iFVM) is the most common abnormality of the fetal central nervous system. It is characterized by enlargement of one or both of the lateral ventricles (defined as ventricular width greater than 10 mm, but less than 12 mm). Despite its high prevalence, the pathophysiology of iFVM during fetal brain development and the neurobiological substrate beyond ventricular enlargement remain unexplored. In this work, we aimed to establish the relationships between the structural development of transient fetal brain zones/compartments and increased cerebrospinal fluid volume. For this purpose, we used in vivo structural T2-weighted magnetic resonance imaging of 89 fetuses (48 controls and 41 cases with iFVM). Our results indicate abnormal development of transient zones/compartments belonging to both hemispheres (i.e. on the side with and also on the contralateral side without a dilated ventricle) in fetuses with iFVM. Specifically, compared to controls, we observed enlargement of proliferative zones and overgrowth of the cortical plate in iFVM with associated reduction of volumes of central structures, subplate, and fetal white matter. These results indicate that enlarged lateral ventricles might be linked to the development of transient fetal zones and that global brain development should be taken into consideration when evaluating iFVM.

Hydrocephalus associated with a molar tooth sign: A distinct subtype of Joubert syndrome.

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.

Swedish cohort study found that half of the girls with shunted hydrocephalus had precocious or early puberty.

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.

Positional shunt assist for slit ventricle syndrome.

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.

Navigating the complexities of encephalocraniocutaneous lipomatosis: a case series and review.

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.

Pediatric sellar teratoma - Case report and review of the literature.

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.

Current Status and Associated Factors of Post-Hemorrhagic Hydrocephalus in Infants of 22 to 28 Weeks Gestation With Severe Intraventricular Hemorrhage in Korea: A Nationwide Cohort Study.

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.

Acute Obstructive Hydrocephalus: An Unexpected Cause of Cardiac Arrest.

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.

Percutaneous fetal endoscopic third ventriculostomy for severe isolated cerebral ventriculomegaly.

OBJECTIVE: To demonstrate the feasibility and preliminary results of percutaneous fetal endoscopic third ventriculostomy (ETV) in human fetuses (pfETV) with isolated progressive and/or severe bilateral cerebral ventriculomegaly (IPSBV). METHODS: The initial results of pfETV for IPSBV were described. Perioperative, perinatal and postnatal variables were described. The Ages and Stages Questionnaire (ASQ-3), 3rd edition (ASQ-3) was used for follow-up of all infants. RESULTS: Successful pfETV was performed in 10/11 (91%) fetuses, at a median gestational age (GA) of 28.7 weeks (25.3-30.7). There were no perioperative complications. After pfETV, 70% (7/10) of the fetuses had a decreased or stabilized lateral ventricle atria|lateral ventricle's atria. The median GA at delivery was 38.2 weeks (35.9-39.3). There were no perinatal complications. The postnatal ventriculoperitoneal shunt rate was 80% (8/10). Among neonates/infants who had prenatal stabilization or a decrease in the LVAs, 4 (4/7: 57.1%) had abnormal scores on the ASQ-3. Among neonates/infants that experienced prenatal increases in the LVAs, all of them (3/3: 100%) had abnormal scores on the ASQ-3. CONCLUSION: Percutaneous ETV is feasible in human fetuses with progressive and/or severe cerebral ventriculomegaly and seems to be a safe procedure for both the mother and the fetus.

Postnatal phenobarbital for the prevention of intraventricular haemorrhage in preterm infants.

BACKGROUND: Intraventricular haemorrhage (IVH) is a major complication of preterm birth. Large haemorrhages are associated with a high risk of disability and hydrocephalus. Instability of blood pressure and cerebral blood in the newborn flow are postulated as causative factors. Another mechanism may involve reperfusion damage from oxygen free radicals. It has been suggested that phenobarbital stabilises blood pressure and may protect against free radicals. This is an update of a review first published in 2001 and updated in 2007 and 2013. OBJECTIVES: To assess the benefits and harms of the postnatal administration of phenobarbital in preterm infants at risk of developing IVH compared to control (i.e. no intervention or placebo). SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (CENTRAL), Medline, Embase, CINAHL and clinical trial registries in January 2022. A new, more sensitive search strategy was developed, and searches were conducted without date limits.  SELECTION CRITERIA: We included randomised controlled trials (RCTs) or quasi-RCTs in which phenobarbital was given within the first 24 hours of life to preterm infants identified as being at risk of IVH because of gestational age below 34 weeks, birth weight below 1500 g or respiratory failure. Phenobarbital was compared to no intervention or placebo. We excluded infants with serious congenital malformations. DATA COLLECTION AND ANALYSIS: We used standard Cochrane methods. Our primary outcomes were all grades of IVH and severe IVH (i.e. grade III and IV); secondary outcomes were ventricular dilation or hydrocephalus, hypotension, pneumothorax, hypercapnia, acidosis, mechanical ventilation, neurodevelopmental impairment and death. We used GRADE to assess the certainty of the evidence for each outcome. MAIN RESULTS: We included 10 RCTs (792 infants). The evidence suggests that phenobarbital results in little to no difference in the incidence of IVH of any grade compared with control (risk ratio (RR) 1.00, 95% confidence interval (CI) 0.84 to 1.19; risk difference (RD) 0.00, 95% CI -0.06 to 0.07; I² for RD = 65%; 10 RCTs, 792 participants; low certainty evidence) and in severe IVH (RR 0.88, 95% CI 0.64 to 1.21; 10 RCTs, 792 participants; low certainty evidence). The evidence is very uncertain about the effect of phenobarbital on posthaemorrhagic ventricular dilation or hydrocephalus (RR 0.62, 95% CI 0.31 to 1.26; 4 RCTs, 271 participants; very low certainty evidence), mild neurodevelopmental impairment (RR 0.57, 95% CI 0.15 to 2.17; 1RCT, 101 participants; very low certainty evidence), and severe neurodevelopmental impairment (RR 1.12, 95% CI 0.44 to 2.82; 2 RCTs, 153 participants; very low certainty evidence). Phenobarbital may result in little to no difference in death before discharge (RR 0.88, 95% CI 0.64 to 1.21; 9 RCTs, 740 participants; low certainty evidence) and mortality during study period (RR 0.98, 95% CI 0.72 to 1.33; 10 RCTs, 792 participants; low certainty evidence) compared with control. We identified no ongoing trials. AUTHORS' CONCLUSIONS: The evidence suggests that phenobarbital results in little to no difference in the incidence of IVH (any grade or severe) compared with control (i.e. no intervention or placebo). The evidence is very uncertain about the effects of phenobarbital on ventricular dilation or hydrocephalus and on neurodevelopmental impairment. The evidence suggests that phenobarbital results in little to no difference in death before discharge and all deaths during the study period compared with control. Since 1993, no randomised studies have been published on phenobarbital for the prevention of IVH in preterm infants, and no trials are ongoing. The effects of postnatal phenobarbital might be assessed in infants with both neonatal seizures and IVH, in both randomised and observational studies. The assessment of benefits and harms should include long-term outcomes.

Surgical indications in pediatric arachnoid cysts.

INTRODUCTION: Arachnoid cysts are developmental lesions consisting of CSF collections within the subarachnoid space. There are many theories and hypotheses about their pathogenesis and histopathology and this may also explain the diversities seen in clinical behaviors of these cysts, their natural history and consequently their management where there is a great controversy about selecting patients for surgical intervention. The most common location in pediatrics is the Sylvian or middle cranial fossa and this made it gain more concern and greater debate about its management where its diagnosis is often accidently or associated with nonspecific symptoms. AIM: Our aim in this article was to review the main surgical indications for pediatric arachnoid cysts in the literature. CONCLUSION: We concluded that the decision for surgical intervention for pediatric arachnoid cysts is not a simple one and highly debatable and should be taken cautiously especially with sylvian arachnoid cysts that may reach a large size without symptoms. However, there is a consensus that the occurrence of symptoms definitely correlated to the presence of these cysts or their rupture with consequent subdural hematoma or hygroma are indications for surgical intervention. Large cysts in locations compressing CSF pathways causing hydrocephalus are also candidates for surgical fenestration. The surgical gain from prophylactic surgery is questionable particularly when asymptomatic.

Revision rates of flow- versus pressure-regulated ventricular shunt valves for the treatment of hydrocephalus in neonates following germinal matrix haemorrhage-a retrospective review.

PURPOSE: Neonates with ventricular shunts inserted for hydrocephalus following germinal matrix haemorrhage (GMH) have high rates of shunt revision. The shunt valve plays a key role in regulating the function of the shunt. In this study, we aim to determine if the choice of flow-regulated or pressure-regulated valve used in the initial implantation of a shunt affects the rate of shunt revision. METHODS: A retrospective cohort comparison study was performed on 34 neonates with hydrocephalus following GMH who underwent placement of a ventricular shunt at the Queensland Children's Hospital from November 2014 to June 2020. The primary outcome examined was the need for revision or replacement of the ventricular shunt after successful initial placement within 2 years of implantation. The secondary outcome examined was the survival time of the shunt. RESULTS: 16 patients had placement of a flow-regulated valve, and 18 patients had placement of a pressure-regulated valve. 14 (87.5%) patients with flow-regulated valves required replacement during the follow-up period. 2 (18.18%) patients with a fixed pressure regulated underwent revision, while 2 (28.57%) programmable pressure-regulated shunts required revision. Patients that had a flow-regulated valve had a statistically significant higher rate of revision compared to those who had a pressure-regulated valve, (87.5% flow vs 22.22% pressure) with a P-value of < 0.001. Valve obstruction was also more common in patients with flow-regulated valves than pressure-regulated valves (4 vs 0) with a P-value of 0.010. Overall mean median survival time was 22.06 months, shunts with flow-regulated valves had a shorter median survival time of 3.19 months compared with over 24 months for pressure-regulated valves with a P-value of < 0.001. CONCLUSION: Our study suggests that the initial implantation of flow-regulated valves may carry an increased total rate of shunt revision and valve obstruction within the first 2 years following implantation compared to pressure-regulated valves in patients with hydrocephalus following GMH.

Ventriculosubgaleal shunt: an institutional experience.

BACKGROUND AND OBJECTIVE: Ventriculosubgaleal shunt (VSGS) was first described in 1896 by Von Mickulicz. It allows CSF to flow from the dilated ventricle to the subgaleal pouch developed in the scalp through a small silicon tube. The possibility that the hydrocephalic state in infants caused by hemorrhage/ + infection will only last a short while prompts the search for temporary management options, which must be a closed system in order to be more physiological given that these infants are frequently preterm (LBW) and immunologically immature. VSGS is one such option. To the best of our knowledge, most of the studies done on VSGS are retrospective, and there are very few prospective studies (none on Indian subjects till now). We aim to study the efficacy of VSGS in preventing the placement of VP Shunt in infants with post-hemorrhagic/post-infectious hydrocephalus. METHOD: Our study (prospective observational) comprises 30 infants in whom VSGS was inserted at Sir Gangaram Hospital between August 2017 and October 2020 and who were followed up for 6 months after VSGS insertion. RESULT: Most of the infants had a birth weight between 1501 and 2000 g with a mean gestational age of 31.2 weeks. Thirteen out of 30 patients did not require the placement of VP shunt. The rates were higher in the post-infectious group (50%) in contrary to the post hemorrhagic group where it was 38.8%. Complications encountered with VSGS include CSF leak (13.3%) and wound dehiscence (6.6%). None had VSGS blockage, migration, and infection. CONCLUSION: We conclude that VSGS is a reliable, safe, easy to perform, and temporary treatment option in infants with post-hemorrhagic and post-infectious hydrocephalus and helps in avoiding VP shunt dependency in nearly half of them.

Enterococcus gallinarum group meningitis after transanal migration of the ventriculoperitoneal shunt: a pediatric case report.

In the literature, only 11 Enterococcus gallinarum group meningitis has been reported so far. The Enterococcus gallinarum group was shown for the first time in a pediatric patient presenting with meningitis after bowel perforation, a complication of a ventriculoperitoneal shunt. A 30-month-old male patient presented with vomiting and fever, with the ventriculoperitoneal shunt distal catheter protruding from the anal orifice. The patient was diagnosed with intestinal perforation and meningitis. Enterococcus gallinarum group bacterial yield in cerebrospinal fluid culture. A total of 6 weeks of intravenous antibiotic treatment was given in the hospital. After the treatment, the patient was re-ventriculoperitoneal shunt and was discharged. Among the shunt complications, meningitis with intestinal perforation is rare. It should be kept in mind that meningitis in such patients may be caused by very rare microbiological factors such as Enterococcus gallinarum group. Antibiotherapy should be given according to the culture result. Then planning should be made for a permanent shunt.

Neurodevelopmental outcomes of neonatal posthemorrhagic hydrocephalus and psychological effects on the parents.

BACKGROUND: Neonatal posthemorrhagic hydrocephalus remains a common complication in preterm infants, with high rates of mortality and morbidity, placing parents at high risk of anxiety and depression. We sought to investigate the neurodevelopmental outcomes of infants with posthemorrhagic hydrocephalus who underwent surgery and the psychological effect on their parents. METHODS: We retrospectively analysed all infants with posthemorrhagic hydrocephalus born between 2014 and 2020 in the Children's Hospital of Chongqing Medical University, China. The neurodevelopmental outcomes of 28 patients were evaluated by the Pediatric Stroke Outcome Measure score, and the psychological states of the parents of survivors were assessed by the Hospital Anxiety and Depression Scale. RESULTS: The families of the 28 patients were followed up for a median duration of 3 years; 6 (21.4%) patients died within 6 months after discharge, 12 (42.9%) patients had moderate to severe dysfunction, and only 10 (35.7%) patients had good outcomes. Regarding the 22 parents of the survivors, 5 (22.7%) and 4 (18.2%) had borderline anxiety and depression symptoms, respectively. Two (9.1%) caregivers had exact anxiety and depression symptoms. Leukomalacia after intraventricular haemorrhage was associated with adverse neurological outcomes. The infants' histories of epileptic seizures during the neonatal period were associated with the anxiety of their parents. CONCLUSION: The overall outcome of posthemorrhagic hydrocephalus patients is unsatisfactory, and children with leukomalacia after haemorrhage tend to have poor outcomes. A history of epileptic seizures during the course of the disease may exacerbate the anxiety of the caregivers.

Iniencephaly and long-term survival: a possible association-case report and review of the literature.

BACKGROUND: Iniencephaly is a rare, complex, and severe form of neural tube defect (NTD), usually associated with wide systemic involvement and poor prognosis. The malformation involves the occiput and the inion, variably combined with rachischisis of the upper cervical and thoracic spine. Although most patients are either stillborn or perish within a few hours after birth, there are reports linking iniencephaly and long survival. For the neurosurgeon, associated encephalocele and secondary hydrocephalus represent the main challenges in this setting of patients, together with proper prenatal counseling. MATERIAL AND METHODS: Herein, the authors performed a thorough review of the pertinent literature, looking for reports of long-term survivors. RESULTS: To date, only five long-term survivors have been reported, with surgical repair attempted in four of them. Furthermore, the authors added their personal experience about two children with long-term survival who underwent surgery, thus accurately comparing them with the analogous cases previously reported in literature, with the ultimate aim of providing novel information on the pathology and the therapeutic options needed to offer proper treatment to these patients. CONCLUSIONS: Although no distinctive anatomic differences have been prior identified between long-term survivors and other patients, some variations emerged to occur, regarding age of presentation, the extension of the CNS malformation, the systemic involvement, and the surgical procedures offered. Although the authors shed some light on the topic, further studies are warranted to better define this rare and complex disease, and survival.

Ventriculosubgaleal shunts in the management of neonatal post-haemorrhagic hydrocephalus: technical note.

INTRODUCTION: Germinal matrix / intraventricular haemorrhage (GMIVH) remains a significant complication of prematurity. The more severe grades are associated with parenchymal haemorrhagic infarction (PHI) and hydrocephalus. A temporising procedure is usually the first line in management of neonatal post-haemorrhagic hydrocephalus (nPHH) as the risk of failure of a permanent cerebrospinal fluid (CSF) diversion is higher in the early stage. Our choice of temporising procedure is a ventriculosubgaleal shunt (VSGS). In this technical note, we describe a modification in technique whereby the pocket of the VSGS is fashioned away from the surgical wound. This resulted in lower CSF leak and subsequent infection rates in our centre. METHODS: We conducted a retrospective analysis of all patients who underwent insertion of a VSGS between September 2014 and February 2023. RESULTS: Twenty children were included in our study with a mean gestational age of 31 weeks + 4 days. Post-operatively, 10% of patients did not need a tap, and 10%, 20%, 15%, 25% and 20% respectively had 1, 2, 3, 4 and 5 taps. Two patients experienced CSF leak from their wounds. In both these patients, the pocket was deemed too close to the wound. None of the patients without suspected pre-existing CNS infection at the time of insertion of VSGS had a subsequent VSGS-related infection. VSGS conversion to permanent ventriculoperitoneal shunts (VPS) was required in 15 (75%) of the patients with an average interval duration of 72 days. On reviewing the literature, the infection rate following VSGS is quoted up to 13.5%. In our own centre, 13 patients had undergone VSGS insertion between 2005 and 2013 with a 30.8% infection rate which seemed related to increased leak rates. CONCLUSION: Our modified surgical approach seems to be effective in reducing the risk of infection, which we postulate is a direct result of reduction in the risk of leak from the surgical wound.

Tension pneumocephalus in a patient with NF1 following ventriculoperitoneal shunt-deciphering the cause and proposed management strategy.

INTRODUCTION: Spontaneous pneumocephalus following ventriculoperitoneal shunting is a very unique complication, seen in a handful of patients. Small bony defects form as a result of chronically raised intracranial pressure, which can later lead to pneumocephalus once intracranial pressure decreases following ventriculoperitoneal shunting. CASE REPORT: Here, we present a case of a 15-year-old girl with NF1 who presented to us with pneumocephalus 10 months following shunting and our management strategy along with a literature review of this condition. CONCLUSION: NF1 & hydrocephalus can lead to skull base erosion, which needs to be looked up before proceeding with VP shunting to avoid delayed onset pneumocephalus. SOKHA with the opening of LT is a minimally invasive approach suitable to tackle both problems simultaneously.